Paul Wales

1343 statements · 13 topics

Abdominal Wall Defects · guest expert Colorectal / ARM & Hirschsprung · guest expert Etiologies (Gastroschisis/NEC/Atresia/Volvulus) · guest expert Intestinal Failure · guest expert Intestinal Rehab · guest expert Motility / Pseudo-obstruction · guest expert Short Bowel Syndrome · guest expert

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One thing I just want to say about oral feeding is that even in the scenario where it's really non-nutritive. I think it's important for people to sort of look at that oral feeding, not so much as an internal nutrition perspective, but more of a skill development perspective that they never learn how to eat by mouth and suck and swallow and process, then they won't eat solids later when you want them to, and you're stuck basically with tube feeding on an older child.
quote · Intestinal Failure
You're right, and they're not isocaloric, and so 1 mL of PN is not 1 mL of formula. So if you keep going beyond 100 per kilo or 120 per kilo, you, you have a problem. And kids, not only will they take a calorie protein hit, they'll take, you start to get into problems with sodium and calcium as well, because the composition in the milk is not the same as what's in the parental solution.
quote · Intestinal Failure
So, um, an intestinal rehabilitation program, as we defined also in these Aspen guidelines that just came out, is a multidisciplinary or interdisciplinary collaborative patient care paradigm that that brings coordinated care for children with intestinal failure through comprehensive management of Their specialized nutrition and other associated needs that they have.
quote · Abdominal Wall Defects
There's literature that shows the improvement of outcomes with programs in general. You see an improved survival related to liver dysfunction, decreased septic episodes, reduction in central line complications, reduced ICU admissions, and the patients that were listed for transplant are coming off the list because of improved status. Mortality has gone down.
quote · Crohn's Disease
In short bowel syndrome, we have 3 anatomical subtypes. Type 1 is the high L piginostomy. Type 2, where you've lost your distal small bowel, your ileum, and part of your colon, and there's a small bowel colonic anastomosis. And then type 3 where you've lost the mid-part of your small bowel, but you still have some retained ileum and an intact colon.
quote · Crohn's Disease
It it's, it's really a functional problem. Essentially, when someone's gut for whatever the diagnosis, whatever the reason, their gut function is insufficient to absorb enough nutrients, fluids, calories to support survival and in the in the case of children, growth, which is a big distinction between adults and children.
quote · Abdominal Wall Defects

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Paul's statements about Abdominal Wall Defects 40 statements

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Intestinal rehabilitation: What is intestinal rehab? - Episode 1

Quotation Well, let's go back one step before that and sort of define what is intestinal failure because that sort of leads into who's going to benefit from an intestinal rehab program. Open
Statement Until recently, there was no standardized definition for intestinal failure. Open
Statement Intestinal failure is when gut function is insufficient to absorb enough nutrients, fluids, and calories to support survival and, in children, growth. Open
Quotation It it's, it's really a functional problem. Essentially, when someone's gut for whatever the diagnosis, whatever the reason, their gut function is insufficient to absorb enough nutrients, fluids, calories to support survival and in the in the case of children, growth, which is a big distinction between adults and children. Open
Quotation So, um, an intestinal rehabilitation program, as we defined also in these Aspen guidelines that just came out, is a multidisciplinary or interdisciplinary collaborative patient care paradigm that that brings coordinated care for children with intestinal failure through comprehensive management of Their specialized nutrition and other associated needs that they have. Open
Statement An intestinal rehabilitation program is a multidisciplinary collaborative patient care paradigm that brings coordinated care for children with intestinal failure through comprehensive management of their specialized nutrition and other associated needs, as defined in recent ASPEN guidelines. Open
Statement The intestinal rehabilitation approach streamlines care and improves communication with families and between care providers. Open
Quotation Patients that are gonna come to intestinal rehab program have intestinal failure, and we can divide the causes of intestinal failure into three categories. Open
Statement Causes of intestinal failure divide into three categories: short bowel syndrome, motility disorders, and congenital enteropathies. Open
Statement Short bowel syndrome is by far the most common category of intestinal failure in pediatric patients. Open
Quotation There's short bowel syndrome, which by far is the most common category in pediatric patients with intestinal failure, and causes of short bowel syndrome are usually related to diagnoses of or, or disorders of the neonate. Open
Statement Causes of short bowel syndrome include congenital anomalies (intestinal atresia, malrotation, volvulus, gastroschisis, long segment Hirschsprung disease) and acquired neonatal diseases (necrotizing enterocolitis). Open
Quotation So if we think of the intestine like a pipe, and that pipe is made of muscle that contracts in a coordinated way to push food and stool from one end to the other, you can have abnormalities of the muscle itself, or of the nerves that control that muscle. And the bowel is unable to push things through in a coordinated way. Open
Statement Motility disorders occur when abnormalities of intestinal muscle or the nerves controlling that muscle prevent coordinated propulsion of food and stool. Open
Statement Congenital enteropathies are conditions where patients have all their bowel but the mucosal lining does not digest or absorb properly. Open
Quotation Enteropathies or the congenital diarrheas, and these are conditions where the patient has all of their bowel, but the mucosa, the inside lining that, that absorbs, uh, digests and absorbs doesn't work. Open
Quotation The reason it's important to at least identify these three categories is that some patients will have elements of 12, or 3 of the categories, the way they present. Open
Statement Some patients have elements of two or all three categories of intestinal failure in their presentation. Open
Statement Most intestinal failure patients are infants or babies, but some older pediatric patients develop intestinal failure from inflammatory bowel disease, Crohn disease complications, trauma, malignancy, or vascular thrombosis. Open
Statement Long-term survivors now develop chronic comorbidities not previously seen to the same extent because patients did not live long enough, including renal dysfunction, metabolic bone disease, neurocognitive issues, and quality of life problems. Open
Quotation But now what we're seeing is we have kids that have developed chronic comorbidities, that comorbidities that never, we didn't really see to the same extent because they didn't live long enough. Open

Intestinal Rehabilitation, Episode 1: What is intestinal rehabilitation?

Quotation Well, let's go back one step before that and sort of define what is intestinal failure because that sort of leads into who's going to benefit from an intestinal rehab program. Open
Statement Until recently, there was no standardized definition for intestinal failure. Open
Statement Intestinal failure is a functional problem where gut function is insufficient to absorb enough nutrients, fluids, and calories to support survival and, in children, growth. Open
Quotation It it's, it's really a functional problem. Essentially, when someone's gut for whatever the diagnosis, whatever the reason, their gut function is insufficient to absorb enough nutrients, fluids, calories to support survival and in the in the case of children, growth, which is a big distinction between adults and children. Open
Quotation So, um, an intestinal rehabilitation program, as we defined also in these Aspen guidelines that just came out, is a multidisciplinary or interdisciplinary collaborative patient care paradigm that that brings coordinated care for children with intestinal failure through comprehensive management of Their specialized nutrition and other associated needs that they have. Open
Statement An intestinal rehabilitation program is a multidisciplinary collaborative patient care paradigm that brings coordinated care for children with intestinal failure through comprehensive management of their specialized nutrition and other associated needs, as defined in recent ASPEN guidelines. Open
Statement Intestinal rehabilitation streamlines care and improves communication between family and care providers through an amalgamation of experts providing holistic, comprehensive, coordinated care. Open
Quotation Patients that are gonna come to intestinal rehab program have intestinal failure, and we can divide the causes of intestinal failure into three categories. Open
Quotation There's short bowel syndrome, which by far is the most common category in pediatric patients with intestinal failure, and causes of short bowel syndrome are usually related to diagnoses of or, or disorders of the neonate. Open
Statement Short bowel syndrome is by far the most common category of intestinal failure in pediatric patients. Open
Statement Causes of short bowel syndrome are usually related to neonatal disorders including congenital anomalies (intestinal atresia, malrotation, volvulus, gastroschisis, long segment Hirschsprung disease) or acquired diseases (necrotizing enterocolitis). Open
Statement Motility disorders occur when abnormalities of the intestinal muscle itself or the nerves controlling that muscle prevent coordinated propulsion of food and stool, making patients dependent on intravenous support. Open
Quotation So if we think of the intestine like a pipe, and that pipe is made of muscle that contracts in a coordinated way to push food and stool from one end to the other, you can have abnormalities of the muscle itself, or of the nerves that control that muscle. And the bowel is unable to push things through in a coordinated way. Open
Quotation And they are therefore dependent on intravenous support. Open
Quotation Enteropathies or the congenital diarrheas, and these are conditions where the patient has all of their bowel, but the mucosa, the inside lining that, that absorbs, uh, digests and absorbs doesn't work. Open
Statement Enteropathies or congenital diarrheas are conditions where the patient has all of their bowel but the mucosal lining that digests and absorbs does not work, leading to hypersecretion and profuse fluid losses. Open
Statement Some patients will have elements of two or all three categories of intestinal failure; for example, gastroschisis can involve short bowel, inflammation affecting absorption, and motility issues. Open
Statement Although most intestinal failure patients are infants or babies, older pediatric patients can develop intestinal failure from inflammatory bowel disease, Crohn's disease complications, trauma, malignancy, or vascular thrombosis leading to gut loss. Open
Statement Improved survival has revealed chronic comorbidities not previously seen to the same extent because patients did not live long enough, including renal dysfunction, metabolic bone disease, neurocognitive issues, and quality of life concerns. Open
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Intestinal Rehabilitation, Episode 6: Cholestasis

Statement Cincinnati Children's Hospital institutionally defined cholestasis as conjugated bilirubin greater than 3 mg/dL or 50 micromoles/L sustained for 2 weeks and not associated with a septic event. Open
Quotation Institutionally defined it as a conjugated bilirubin greater than 3 mg per deciliter or 50 micromoles per liter sustained for 2 weeks and not associated with a septic event. Open
Statement A 2021 JPN publication defined cholestasis as conjugated bilirubin around 2 mg/dL or 34 micromoles/L for 2 weeks, not associated with a septic event. Open
Statement In young children, intestinal failure-associated liver disease presents as cholestatic liver disease, whereas in adolescents and adults it tends to be steatosis (fatty deposition). Open
Quotation Now, prematurity, we as clinicians, as a team, we don't have much control over that. But the other factors are modifiable, and we can have an impact on that. Open
Statement Prematurity is not modifiable by the clinical team, but lack of enteral feeding, sepsis, and TPN components are modifiable risk factors. Open
Statement Prevention of cholestasis requires aggressive introduction of enteral feeding to establish enterohepatic circulation and surgical procedures to optimize anatomy for feed delivery. Open
Statement Two strategies for reversing cholestasis are dose restriction and change of lipid composition. Open
Statement Conventional intralipid (soybean-based) produces prostaglandins and eicosanoids that are pro-inflammatory when metabolized. Open
Statement SMOF lipid promotes bile flow, is hepatoprotective, and can be delivered at conventional dose to achieve somatic growth and provide neurologic nutrients while protecting the liver. Open
Statement SMOF lipid does not contain enough arachidonic acid, so dose restriction of SMOF can lead to essential fatty acid deficiency. Open
Statement When SMOF is delivered at conventional dosing, no patients develop essential fatty acid deficiency. Open
Statement Conventional dosing for lipids is settling around 2.5 g/kg/day, but nutrition guidelines for preterm infants and babies state 3-4 g/kg/day. Open
Quotation My opinion is that a billy of 2 is not by any stretch advanced liver disease, and it's not dangerous, and it's usually transient. So I personally would not change the lipid emulsion out of Billy to. Open
Statement A bilirubin of 2 mg/dL is not advanced liver disease, is not dangerous, and is usually transient; Dr. Wales would not change lipid emulsion at this threshold. Open
Statement During secondary surgical or autologous reconstruction procedures, a liver biopsy is commonly taken to provide an up-to-date microscopic snapshot. Open
Statement Liver function and biochemistry are followed routinely as inpatient and at outpatient clinic visits. Open
Statement Elastography (FibroScan) is available for monitoring but is good for mild/no fibrosis or very advanced fibrosis; it is not as sensitive for patients with intermediate fibrosis. Open
Statement Follow-up visit frequency for children on TPN at home ranges from every 1-4 months depending on patient stability; actively adapting patients may be seen more frequently due to rapid changes being made. Open
Paul's statements about Colorectal / ARM & Hirschsprung 40 statements

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Intestinal rehabilitation: What is intestinal rehab? - Episode 1

Quotation Well, let's go back one step before that and sort of define what is intestinal failure because that sort of leads into who's going to benefit from an intestinal rehab program. Open
Statement Until recently, there was no standardized definition for intestinal failure. Open
Quotation It it's, it's really a functional problem. Essentially, when someone's gut for whatever the diagnosis, whatever the reason, their gut function is insufficient to absorb enough nutrients, fluids, calories to support survival and in the in the case of children, growth, which is a big distinction between adults and children. Open
Statement Intestinal failure is when gut function is insufficient to absorb enough nutrients, fluids, and calories to support survival and, in children, growth. Open
Statement An intestinal rehabilitation program is a multidisciplinary collaborative patient care paradigm that brings coordinated care for children with intestinal failure through comprehensive management of their specialized nutrition and other associated needs, as defined in recent ASPEN guidelines. Open
Quotation So, um, an intestinal rehabilitation program, as we defined also in these Aspen guidelines that just came out, is a multidisciplinary or interdisciplinary collaborative patient care paradigm that that brings coordinated care for children with intestinal failure through comprehensive management of Their specialized nutrition and other associated needs that they have. Open
Statement The intestinal rehabilitation approach streamlines care and improves communication with families and between care providers. Open
Quotation Patients that are gonna come to intestinal rehab program have intestinal failure, and we can divide the causes of intestinal failure into three categories. Open
Statement Causes of intestinal failure divide into three categories: short bowel syndrome, motility disorders, and congenital enteropathies. Open
Statement Short bowel syndrome is by far the most common category of intestinal failure in pediatric patients. Open
Quotation There's short bowel syndrome, which by far is the most common category in pediatric patients with intestinal failure, and causes of short bowel syndrome are usually related to diagnoses of or, or disorders of the neonate. Open
Statement Causes of short bowel syndrome include congenital anomalies (intestinal atresia, malrotation, volvulus, gastroschisis, long segment Hirschsprung disease) and acquired neonatal diseases (necrotizing enterocolitis). Open
Quotation So if we think of the intestine like a pipe, and that pipe is made of muscle that contracts in a coordinated way to push food and stool from one end to the other, you can have abnormalities of the muscle itself, or of the nerves that control that muscle. And the bowel is unable to push things through in a coordinated way. Open
Statement Motility disorders occur when abnormalities of intestinal muscle or the nerves controlling that muscle prevent coordinated propulsion of food and stool. Open
Statement Congenital enteropathies are conditions where patients have all their bowel but the mucosal lining does not digest or absorb properly. Open
Quotation Enteropathies or the congenital diarrheas, and these are conditions where the patient has all of their bowel, but the mucosa, the inside lining that, that absorbs, uh, digests and absorbs doesn't work. Open
Statement Some patients have elements of two or all three categories of intestinal failure in their presentation. Open
Quotation The reason it's important to at least identify these three categories is that some patients will have elements of 12, or 3 of the categories, the way they present. Open
Statement Most intestinal failure patients are infants or babies, but some older pediatric patients develop intestinal failure from inflammatory bowel disease, Crohn disease complications, trauma, malignancy, or vascular thrombosis. Open
Statement Long-term survivors now develop chronic comorbidities not previously seen to the same extent because patients did not live long enough, including renal dysfunction, metabolic bone disease, neurocognitive issues, and quality of life problems. Open
Quotation But now what we're seeing is we have kids that have developed chronic comorbidities, that comorbidities that never, we didn't really see to the same extent because they didn't live long enough. Open

Intestinal Rehabilitation, Episode 1: What is intestinal rehabilitation?

Quotation Well, let's go back one step before that and sort of define what is intestinal failure because that sort of leads into who's going to benefit from an intestinal rehab program. Open
Statement Until recently, there was no standardized definition for intestinal failure. Open
Quotation It it's, it's really a functional problem. Essentially, when someone's gut for whatever the diagnosis, whatever the reason, their gut function is insufficient to absorb enough nutrients, fluids, calories to support survival and in the in the case of children, growth, which is a big distinction between adults and children. Open
Statement Intestinal failure is a functional problem where gut function is insufficient to absorb enough nutrients, fluids, and calories to support survival and, in children, growth. Open
Quotation So, um, an intestinal rehabilitation program, as we defined also in these Aspen guidelines that just came out, is a multidisciplinary or interdisciplinary collaborative patient care paradigm that that brings coordinated care for children with intestinal failure through comprehensive management of Their specialized nutrition and other associated needs that they have. Open
Statement An intestinal rehabilitation program is a multidisciplinary collaborative patient care paradigm that brings coordinated care for children with intestinal failure through comprehensive management of their specialized nutrition and other associated needs, as defined in recent ASPEN guidelines. Open
Statement Intestinal rehabilitation streamlines care and improves communication between family and care providers through an amalgamation of experts providing holistic, comprehensive, coordinated care. Open
Quotation Patients that are gonna come to intestinal rehab program have intestinal failure, and we can divide the causes of intestinal failure into three categories. Open
Statement Causes of short bowel syndrome are usually related to neonatal disorders including congenital anomalies (intestinal atresia, malrotation, volvulus, gastroschisis, long segment Hirschsprung disease) or acquired diseases (necrotizing enterocolitis). Open
Quotation There's short bowel syndrome, which by far is the most common category in pediatric patients with intestinal failure, and causes of short bowel syndrome are usually related to diagnoses of or, or disorders of the neonate. Open
Statement Short bowel syndrome is by far the most common category of intestinal failure in pediatric patients. Open
Statement Motility disorders occur when abnormalities of the intestinal muscle itself or the nerves controlling that muscle prevent coordinated propulsion of food and stool, making patients dependent on intravenous support. Open
Quotation So if we think of the intestine like a pipe, and that pipe is made of muscle that contracts in a coordinated way to push food and stool from one end to the other, you can have abnormalities of the muscle itself, or of the nerves that control that muscle. And the bowel is unable to push things through in a coordinated way. Open
Quotation And they are therefore dependent on intravenous support. Open
Statement Enteropathies or congenital diarrheas are conditions where the patient has all of their bowel but the mucosal lining that digests and absorbs does not work, leading to hypersecretion and profuse fluid losses. Open
Quotation Enteropathies or the congenital diarrheas, and these are conditions where the patient has all of their bowel, but the mucosa, the inside lining that, that absorbs, uh, digests and absorbs doesn't work. Open
Statement Some patients will have elements of two or all three categories of intestinal failure; for example, gastroschisis can involve short bowel, inflammation affecting absorption, and motility issues. Open
Statement Although most intestinal failure patients are infants or babies, older pediatric patients can develop intestinal failure from inflammatory bowel disease, Crohn's disease complications, trauma, malignancy, or vascular thrombosis leading to gut loss. Open
Statement Improved survival has revealed chronic comorbidities not previously seen to the same extent because patients did not live long enough, including renal dysfunction, metabolic bone disease, neurocognitive issues, and quality of life concerns. Open
Paul's statements about Crohn's Disease 123 statements

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Intestinal rehabilitation: What is intestinal rehab? - Episode 1

Quotation Well, let's go back one step before that and sort of define what is intestinal failure because that sort of leads into who's going to benefit from an intestinal rehab program. Open
Statement Until recently, there was no standardized definition for intestinal failure. Open
Statement Intestinal failure is when gut function is insufficient to absorb enough nutrients, fluids, and calories to support survival and, in children, growth. Open
Quotation It it's, it's really a functional problem. Essentially, when someone's gut for whatever the diagnosis, whatever the reason, their gut function is insufficient to absorb enough nutrients, fluids, calories to support survival and in the in the case of children, growth, which is a big distinction between adults and children. Open
Quotation So, um, an intestinal rehabilitation program, as we defined also in these Aspen guidelines that just came out, is a multidisciplinary or interdisciplinary collaborative patient care paradigm that that brings coordinated care for children with intestinal failure through comprehensive management of Their specialized nutrition and other associated needs that they have. Open
Statement An intestinal rehabilitation program is a multidisciplinary collaborative patient care paradigm that brings coordinated care for children with intestinal failure through comprehensive management of their specialized nutrition and other associated needs, as defined in recent ASPEN guidelines. Open
Statement The intestinal rehabilitation approach streamlines care and improves communication with families and between care providers. Open
Quotation Patients that are gonna come to intestinal rehab program have intestinal failure, and we can divide the causes of intestinal failure into three categories. Open
Statement Causes of intestinal failure divide into three categories: short bowel syndrome, motility disorders, and congenital enteropathies. Open
Quotation There's short bowel syndrome, which by far is the most common category in pediatric patients with intestinal failure, and causes of short bowel syndrome are usually related to diagnoses of or, or disorders of the neonate. Open
Statement Short bowel syndrome is by far the most common category of intestinal failure in pediatric patients. Open
Statement Causes of short bowel syndrome include congenital anomalies (intestinal atresia, malrotation, volvulus, gastroschisis, long segment Hirschsprung disease) and acquired neonatal diseases (necrotizing enterocolitis). Open
Quotation So if we think of the intestine like a pipe, and that pipe is made of muscle that contracts in a coordinated way to push food and stool from one end to the other, you can have abnormalities of the muscle itself, or of the nerves that control that muscle. And the bowel is unable to push things through in a coordinated way. Open
Statement Motility disorders occur when abnormalities of intestinal muscle or the nerves controlling that muscle prevent coordinated propulsion of food and stool. Open
Statement Congenital enteropathies are conditions where patients have all their bowel but the mucosal lining does not digest or absorb properly. Open
Quotation Enteropathies or the congenital diarrheas, and these are conditions where the patient has all of their bowel, but the mucosa, the inside lining that, that absorbs, uh, digests and absorbs doesn't work. Open
Quotation The reason it's important to at least identify these three categories is that some patients will have elements of 12, or 3 of the categories, the way they present. Open
Statement Some patients have elements of two or all three categories of intestinal failure in their presentation. Open
Statement Most intestinal failure patients are infants or babies, but some older pediatric patients develop intestinal failure from inflammatory bowel disease, Crohn disease complications, trauma, malignancy, or vascular thrombosis. Open
Statement Long-term survivors now develop chronic comorbidities not previously seen to the same extent because patients did not live long enough, including renal dysfunction, metabolic bone disease, neurocognitive issues, and quality of life problems. Open
Quotation But now what we're seeing is we have kids that have developed chronic comorbidities, that comorbidities that never, we didn't really see to the same extent because they didn't live long enough. Open

Intestinal Rehabilitation, Episode 1: What is intestinal rehabilitation?

Quotation Well, let's go back one step before that and sort of define what is intestinal failure because that sort of leads into who's going to benefit from an intestinal rehab program. Open
Statement Until recently, there was no standardized definition for intestinal failure. Open
Statement Intestinal failure is a functional problem where gut function is insufficient to absorb enough nutrients, fluids, and calories to support survival and, in children, growth. Open
Quotation It it's, it's really a functional problem. Essentially, when someone's gut for whatever the diagnosis, whatever the reason, their gut function is insufficient to absorb enough nutrients, fluids, calories to support survival and in the in the case of children, growth, which is a big distinction between adults and children. Open
Quotation So, um, an intestinal rehabilitation program, as we defined also in these Aspen guidelines that just came out, is a multidisciplinary or interdisciplinary collaborative patient care paradigm that that brings coordinated care for children with intestinal failure through comprehensive management of Their specialized nutrition and other associated needs that they have. Open
Statement An intestinal rehabilitation program is a multidisciplinary collaborative patient care paradigm that brings coordinated care for children with intestinal failure through comprehensive management of their specialized nutrition and other associated needs, as defined in recent ASPEN guidelines. Open
Statement Intestinal rehabilitation streamlines care and improves communication between family and care providers through an amalgamation of experts providing holistic, comprehensive, coordinated care. Open
Quotation Patients that are gonna come to intestinal rehab program have intestinal failure, and we can divide the causes of intestinal failure into three categories. Open
Statement Short bowel syndrome is by far the most common category of intestinal failure in pediatric patients. Open
Statement Causes of short bowel syndrome are usually related to neonatal disorders including congenital anomalies (intestinal atresia, malrotation, volvulus, gastroschisis, long segment Hirschsprung disease) or acquired diseases (necrotizing enterocolitis). Open
Quotation There's short bowel syndrome, which by far is the most common category in pediatric patients with intestinal failure, and causes of short bowel syndrome are usually related to diagnoses of or, or disorders of the neonate. Open
Statement Motility disorders occur when abnormalities of the intestinal muscle itself or the nerves controlling that muscle prevent coordinated propulsion of food and stool, making patients dependent on intravenous support. Open
Quotation So if we think of the intestine like a pipe, and that pipe is made of muscle that contracts in a coordinated way to push food and stool from one end to the other, you can have abnormalities of the muscle itself, or of the nerves that control that muscle. And the bowel is unable to push things through in a coordinated way. Open
Quotation And they are therefore dependent on intravenous support. Open
Statement Enteropathies or congenital diarrheas are conditions where the patient has all of their bowel but the mucosal lining that digests and absorbs does not work, leading to hypersecretion and profuse fluid losses. Open
Quotation Enteropathies or the congenital diarrheas, and these are conditions where the patient has all of their bowel, but the mucosa, the inside lining that, that absorbs, uh, digests and absorbs doesn't work. Open
Statement Some patients will have elements of two or all three categories of intestinal failure; for example, gastroschisis can involve short bowel, inflammation affecting absorption, and motility issues. Open
Statement Although most intestinal failure patients are infants or babies, older pediatric patients can develop intestinal failure from inflammatory bowel disease, Crohn's disease complications, trauma, malignancy, or vascular thrombosis leading to gut loss. Open
Statement Improved survival has revealed chronic comorbidities not previously seen to the same extent because patients did not live long enough, including renal dysfunction, metabolic bone disease, neurocognitive issues, and quality of life concerns. Open

Advancements in Pediatric Intestinal Failure: Innovative Therapies and Improved Outcomes

Quotation They go anywhere from mild cholestasis to more profound steatosis, which we see in older children or adolescents and adults, with ongoing inflammation, fibrosis, and ultimately cirrhosis leading to death or the need for transplantation. Open
Statement Liver disease associated with intestinal failure ranges from mild cholestasis to profound steatosis (in older children/adolescents/adults), with progression through inflammation, fibrosis, and ultimately cirrhosis leading to death or transplantation. Open
Quotation There's some patient factors related to prematurity, uh, lack of enteral feeding, the inability to feed because of the anatomy or recurrent infections, sepsis, which can affect the liver directly and indirectly. And the components of TPN. Open
Statement Risk factors for intestinal failure-associated liver disease include prematurity, lack of enteral feeding, inability to feed due to anatomy, recurrent infections/sepsis, and components of TPN. Open
Quotation The term intestinal rehab program was coined in the year 2000. Open
Statement The term 'intestinal rehabilitation program' was coined in the year 2000. Open
Quotation Intestinal rehab teams improve the outcome of children because of integration of care, improved communication, better continuity. Open
Statement Intestinal rehabilitation teams improve outcomes through integration of care, improved communication, and better continuity. Open
Quotation We have a large reliance on speech, language, and occupational therapy. Open
Statement Intestinal rehabilitation programs rely heavily on speech, language, and occupational therapy specialists. Open
Quotation While our patients are on TPN, we're trying to mitigate the progression of liver disease, sepsis, vascular complications. Open
Statement While patients are on TPN, the goal is to mitigate progression of liver disease, sepsis, and vascular complications. Open
Quotation Having a dedicated unit with dedicated staff all contributes to better outcomes. Open
Quotation What we ask our Families to do at home is significant. These patients go home on TPN. The families run the pump, add vitamins to the TPN, program the pump, record ins and outs, manage the G tube, manage the stoma. Open
Statement Families of intestinal failure patients manage significant tasks at home including running TPN pumps, adding vitamins, programming pumps, recording ins and outs, managing G-tubes, and managing stomas. Open
Statement High-quality families demonstrate evidence of burnout over time despite education and empowerment efforts. Open
Quotation We educate and empower our parents because we've all seen high-quality families that over time demonstrate evidence of burnout. Open
Statement Literature shows intestinal rehabilitation programs improve survival related to liver dysfunction, decrease septic episodes, reduce central line complications, reduce ICU admissions, and enable patients listed for transplant to come off the list due to improved status. Open
Quotation There's literature that shows the improvement of outcomes with programs in general. You see an improved survival related to liver dysfunction, decreased septic episodes, reduction in central line complications, reduced ICU admissions, and the patients that were listed for transplant are coming off the list because of improved status. Mortality has gone down. Open
Statement Mortality in intestinal failure has decreased with intestinal rehabilitation programs. Open
Quotation Lipids are a source of essential fatty acids. They're a non-protein energy source important for growth. Fatty acids have a major role in cellular pathways. Open
Statement Lipids in parenteral nutrition are a source of essential fatty acids, a non-protein energy source important for growth, and fatty acids have a major role in cellular pathways. Open
Statement Historically, most lipid emulsions in North America are soybean-based with high phytosterol content, high omega-6 long-chain polyunsaturated fatty acid content, and low antioxidant content. Open
Quotation Historically, most of the lipid emulsions in North America are soybean-based, so they have a high phytosterol content. A high omega 6 long chain polyunsaturated fatty acid content and low antioxidant content. Open
Statement Prolonged soybean-based lipid exposure is associated with deterioration of liver function, specifically cholestasis. Open
Quotation It is associated with deterioration of liver dysfunction, i.e., cholestasis. Open
Quotation This is a paper that we wrote several years ago that showed that for every day that an infant is exposed to soybean lipid dosed at greater than 2.5 g per kilo per day, which is more conventional dosing. That is associated with a 3% increase in the odds ratio of developing advanced liver disease. Open
Statement For every day an infant is exposed to soybean lipid dosed at greater than 2.5 g/kg/day, there is a 3% increase in the odds ratio of developing advanced liver disease. Open
Statement First-generation lipid emulsions were soybean-based, rich in omega-6 PUFA (such as Intralipid), and have been in use for 40 years. Open
Quotation The first generation were basically the soybean-based lipids, rich in omega 6UUFA. That would be intralipids, and that's been around for 40 years and it's saved a lot of lives. Open
Statement Third-generation lipid emulsions reduce omega-6 component and change the omega-6 to omega-3 ratio by adding omega-3 lipids. Open
Quotation The 3rd generation lipids attempt to not only reduce the omega 6 component, but to change the omega 6 to the omega 3 ratio with the addition of omega 3 lipids. Open
Statement Omega-6 lipids are metabolized to arachidonic acid, which produces leukotrienes and prostaglandins with a more pro-inflammatory profile. Open
Quotation Omega 6 lipids are metabolized to arachidonic acid. Which produce leukotrienes and prostaglandins of the more pro-inflammatory profile. The omega 3 lipids are metabolized through EPA and DHA and lead to the production of cytokines that have a less inflammatory profile. Open
Statement Omega-3 lipids are metabolized through EPA and DHA, leading to production of cytokines with a less inflammatory profile. Open
Statement Omega-3 lipids improve bile flow by decreasing lithogenicity of bile, decreasing steatosis, stimulating improved beta-oxidation and clearance, lowering oxidative stress, and supporting immune function. Open
Quotation Omega 3 lipids improve bile flow because they decrease lithogenicity of the bile. They decrease steatosis. They stimulate improved beta oxidation and clearance, and they lower oxidative stress and support immune function. Open
Statement The two main strategies for improving lipids in parenteral nutrition are lipid minimization and change in composition. Open
Quotation One is lipid minimization, and then the second strategy is a change in composition. Open
Quotation There's a lot of literature dating back even decades that show that if you restrict exposure to soybean lipid, you improve cholestasis. When you dose the lipid at 1 g per kilo per day or less. Open
Statement Literature dating back decades shows that restricting exposure to soybean lipid improves cholestasis, particularly when dosed at 1 g/kg/day or less. Open
Statement In preterm babies, fat is important for growth, especially for neurocognitive development, creating a risk of essential fatty acid deficiency if lipid dosing is restricted too much. Open
Quotation The problem with this in preterm babies is that fat is important for growth, especially for neurocognitive development. So there is the risk of essential fatty acid deficiency if we restrict lipid dosing too much. Open
Statement DHA and arachidonic acid are important for retinal and brain development. Open
Quotation DHA and arachidonic acid are important for retinal and brain development. None of the lipid emulsions today were designed for premature babies. They were all designed for adults in a critical care setting. Open
Statement None of the current lipid emulsions were designed for premature babies; they were all designed for adults in critical care settings. Open
Quotation We're currently in work with industry to create a lipid emulsion that's more specifically tailored to preterm babies that has a higher content of arachidonic acid. Open
Statement Work is currently underway with industry to create a lipid emulsion specifically tailored to preterm babies with higher arachidonic acid content. Open
Statement Central lines are the lifeline for intestinal failure patients, and without venous access, families cannot be supported. Open
Quotation So our patients with central lines, it's literally their lifeline. And without venous access, we can't support our families. They are at risk for complications, infections, blockages, thrombosis, and breakage. Open
Statement Central lines are at risk for complications including infections, blockages, thrombosis, and breakage. Open
Quotation In the United States, there's about 240,000 collapses in the United States on an annual basis. The cost of one of these is about $30,000 about $2 billion a year nationwide. Open
Statement In the United States, there are approximately 240,000 central line-associated bloodstream infections (CLABSIs) annually, with each costing about $30,000, totaling approximately $2 billion per year nationwide. Open
Quotation We landed on 4% tetrosodium EDTA or kite lock as it's known in the trade name, which is an antithrombolytic, anti-fibrinolytic, and antimicrobial. So, it satisfies all three criteria that are important for a good lock solution. Open
Statement The team pivoted to 4% tetrasodium EDTA (Taurolock/Kitelock) when ethanol became too expensive and difficult to obtain. Open
Statement 4% tetrasodium EDTA (Kitelock) is antithrombotic, anti-fibrinolytic, and antimicrobial, satisfying all three criteria important for a good lock solution. Open
Statement Kitelock is licensed in Europe and Australia but not available in the United States. Open
Quotation It's licensed in Europe, it's licensed in Australia. It's not available in the United States. Open
Quotation I'm happy to say that we have finally launched our multi-center randomized trial of kitelock versus heparin, which went live 4 weeks ago, and we're actively recruiting patients. In the next year, we'll have the results of the trial, and we can get the kitelock into the United States. Open
Statement A multi-center randomized trial of Kitelock versus heparin went live 4 weeks ago and is actively recruiting patients, with results expected within the next year. Open
Statement In short bowel syndrome, there are three anatomical subtypes: Type 1 (high jejunostomy), Type 2 (loss of distal small bowel/ileum and part of colon with small bowel-colonic anastomosis), and Type 3 (loss of mid-small bowel with retained ileum and intact colon). Open
Quotation In short bowel syndrome, we have 3 anatomical subtypes. Type 1 is the high L piginostomy. Type 2, where you've lost your distal small bowel, your ileum, and part of your colon, and there's a small bowel colonic anastomosis. And then type 3 where you've lost the mid-part of your small bowel, but you still have some retained ileum and an intact colon. Open
Statement The most common anatomical subtype of short bowel syndrome in pediatrics is Type 2. Open
Quotation The most common anatomical subtype in pediatrics is type 2. These patients struggle. Type 1 and type 2 struggle because they lack an ileum. Open
Statement Type 1 and Type 2 short bowel syndrome patients struggle because they lack an ileum. Open
Quotation The problem is that the native hormone has a half-life of 7 minutes. So it's not practical. So it's been altered, one amino acid alteration to an analog called taglutide. That has a half-life of 2 hours and it's given once a day subcutaneous injection. Open
Statement Native GLP-2 hormone has a half-life of 7 minutes, making it impractical for clinical use. Open
Statement GLP-2 has been altered by one amino acid to create an analog called teduglutide, which has a half-life of 2 hours and is given once daily by subcutaneous injection. Open
Statement A multi-center phase 3 study published in 2020 at Cincinnati Children's showed patients receiving teduglutide had a 40% reduction in TPN fluid and calorie requirements over a six-month period. Open
Quotation What we see in patients that received taglutide, a 40% reduction in TPN fluid and calorie requirements over a six-month period. And 70% of patients that received the analog achieved the study endpoint, which was a 20% reduction in TPN requirements. Open
Statement 70% of patients who received teduglutide achieved the study endpoint of a 20% reduction in TPN requirements. Open
Quotation It's licensed for children that are greater than 1 year of age. Already, it's made a big difference in our practice and even kids with very extreme anatomy have been able to get off TPN as a result of being able to replace this hormone. Open
Statement Teduglutide is licensed for children greater than 1 year of age. Open
Statement Teduglutide has made a big difference in practice, with even children with very extreme anatomy able to get off TPN. Open
Statement Another GLP-2 analog, apraglutide, has a longer half-life and can be given once weekly. Open
Quotation Another GLP-2 analog, a proglutide, has a longer half-life and can be given once a week. That's currently being studied. And what we've shown is that it actually works even better than taglutide. You get more bowel lengthening with a proglutide compared to glutide. Open
Statement Apraglutide works even better than teduglutide, producing more bowel lengthening. Open
Quotation These interventions have led to a decrease in transplantation since 2008. In large part because of the successes of intestinal rehabilitation, the outcomes of transplant at 5 years are about 65%. Whatever we can do to avoid transplant and lifelong immunosuppression, that is our goal. Open
Statement These interventions (optimized lipids, central line infection prevention, GLP-2 analogs) have led to a decrease in transplantation since 2008. Open
Statement Outcomes of intestinal transplant at 5 years are approximately 65%. Open
Statement The decrease in transplantation is in large part because of the successes of intestinal rehabilitation. Open
Statement The goal is to avoid transplant and lifelong immunosuppression whenever possible. Open
Quotation We have lots of exciting medical and surgical options available to us, but this should all be done under the umbrella of a multidisciplinary team. Open
Paul's statements about Etiologies (Gastroschisis/NEC/Atresia/Volvulus) 49 statements

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Intestinal rehabilitation: What is intestinal rehab? - Episode 1

Quotation Well, let's go back one step before that and sort of define what is intestinal failure because that sort of leads into who's going to benefit from an intestinal rehab program. Open
Statement Until recently, there was no standardized definition for intestinal failure. Open
Statement Intestinal failure is when gut function is insufficient to absorb enough nutrients, fluids, and calories to support survival and, in children, growth. Open
Quotation It it's, it's really a functional problem. Essentially, when someone's gut for whatever the diagnosis, whatever the reason, their gut function is insufficient to absorb enough nutrients, fluids, calories to support survival and in the in the case of children, growth, which is a big distinction between adults and children. Open
Statement An intestinal rehabilitation program is a multidisciplinary collaborative patient care paradigm that brings coordinated care for children with intestinal failure through comprehensive management of their specialized nutrition and other associated needs, as defined in recent ASPEN guidelines. Open
Quotation So, um, an intestinal rehabilitation program, as we defined also in these Aspen guidelines that just came out, is a multidisciplinary or interdisciplinary collaborative patient care paradigm that that brings coordinated care for children with intestinal failure through comprehensive management of Their specialized nutrition and other associated needs that they have. Open
Statement The intestinal rehabilitation approach streamlines care and improves communication with families and between care providers. Open
Quotation Patients that are gonna come to intestinal rehab program have intestinal failure, and we can divide the causes of intestinal failure into three categories. Open
Statement Causes of intestinal failure divide into three categories: short bowel syndrome, motility disorders, and congenital enteropathies. Open
Quotation There's short bowel syndrome, which by far is the most common category in pediatric patients with intestinal failure, and causes of short bowel syndrome are usually related to diagnoses of or, or disorders of the neonate. Open
Statement Short bowel syndrome is by far the most common category of intestinal failure in pediatric patients. Open
Statement Causes of short bowel syndrome include congenital anomalies (intestinal atresia, malrotation, volvulus, gastroschisis, long segment Hirschsprung disease) and acquired neonatal diseases (necrotizing enterocolitis). Open
Statement Motility disorders occur when abnormalities of intestinal muscle or the nerves controlling that muscle prevent coordinated propulsion of food and stool. Open
Quotation So if we think of the intestine like a pipe, and that pipe is made of muscle that contracts in a coordinated way to push food and stool from one end to the other, you can have abnormalities of the muscle itself, or of the nerves that control that muscle. And the bowel is unable to push things through in a coordinated way. Open
Statement Congenital enteropathies are conditions where patients have all their bowel but the mucosal lining does not digest or absorb properly. Open
Quotation Enteropathies or the congenital diarrheas, and these are conditions where the patient has all of their bowel, but the mucosa, the inside lining that, that absorbs, uh, digests and absorbs doesn't work. Open
Statement Some patients have elements of two or all three categories of intestinal failure in their presentation. Open
Quotation The reason it's important to at least identify these three categories is that some patients will have elements of 12, or 3 of the categories, the way they present. Open
Statement Most intestinal failure patients are infants or babies, but some older pediatric patients develop intestinal failure from inflammatory bowel disease, Crohn disease complications, trauma, malignancy, or vascular thrombosis. Open
Statement Long-term survivors now develop chronic comorbidities not previously seen to the same extent because patients did not live long enough, including renal dysfunction, metabolic bone disease, neurocognitive issues, and quality of life problems. Open
Quotation But now what we're seeing is we have kids that have developed chronic comorbidities, that comorbidities that never, we didn't really see to the same extent because they didn't live long enough. Open

Intestinal Rehabilitation, Episode 1: What is intestinal rehabilitation?

Quotation Well, let's go back one step before that and sort of define what is intestinal failure because that sort of leads into who's going to benefit from an intestinal rehab program. Open
Statement Until recently, there was no standardized definition for intestinal failure. Open
Quotation It it's, it's really a functional problem. Essentially, when someone's gut for whatever the diagnosis, whatever the reason, their gut function is insufficient to absorb enough nutrients, fluids, calories to support survival and in the in the case of children, growth, which is a big distinction between adults and children. Open
Statement Intestinal failure is a functional problem where gut function is insufficient to absorb enough nutrients, fluids, and calories to support survival and, in children, growth. Open
Statement An intestinal rehabilitation program is a multidisciplinary collaborative patient care paradigm that brings coordinated care for children with intestinal failure through comprehensive management of their specialized nutrition and other associated needs, as defined in recent ASPEN guidelines. Open
Quotation So, um, an intestinal rehabilitation program, as we defined also in these Aspen guidelines that just came out, is a multidisciplinary or interdisciplinary collaborative patient care paradigm that that brings coordinated care for children with intestinal failure through comprehensive management of Their specialized nutrition and other associated needs that they have. Open
Statement Intestinal rehabilitation streamlines care and improves communication between family and care providers through an amalgamation of experts providing holistic, comprehensive, coordinated care. Open
Quotation Patients that are gonna come to intestinal rehab program have intestinal failure, and we can divide the causes of intestinal failure into three categories. Open
Statement Short bowel syndrome is by far the most common category of intestinal failure in pediatric patients. Open
Quotation There's short bowel syndrome, which by far is the most common category in pediatric patients with intestinal failure, and causes of short bowel syndrome are usually related to diagnoses of or, or disorders of the neonate. Open
Statement Causes of short bowel syndrome are usually related to neonatal disorders including congenital anomalies (intestinal atresia, malrotation, volvulus, gastroschisis, long segment Hirschsprung disease) or acquired diseases (necrotizing enterocolitis). Open
Quotation So if we think of the intestine like a pipe, and that pipe is made of muscle that contracts in a coordinated way to push food and stool from one end to the other, you can have abnormalities of the muscle itself, or of the nerves that control that muscle. And the bowel is unable to push things through in a coordinated way. Open
Statement Motility disorders occur when abnormalities of the intestinal muscle itself or the nerves controlling that muscle prevent coordinated propulsion of food and stool, making patients dependent on intravenous support. Open
Quotation And they are therefore dependent on intravenous support. Open
Statement Enteropathies or congenital diarrheas are conditions where the patient has all of their bowel but the mucosal lining that digests and absorbs does not work, leading to hypersecretion and profuse fluid losses. Open
Quotation Enteropathies or the congenital diarrheas, and these are conditions where the patient has all of their bowel, but the mucosa, the inside lining that, that absorbs, uh, digests and absorbs doesn't work. Open
Statement Some patients will have elements of two or all three categories of intestinal failure; for example, gastroschisis can involve short bowel, inflammation affecting absorption, and motility issues. Open
Statement Although most intestinal failure patients are infants or babies, older pediatric patients can develop intestinal failure from inflammatory bowel disease, Crohn's disease complications, trauma, malignancy, or vascular thrombosis leading to gut loss. Open
Statement Improved survival has revealed chronic comorbidities not previously seen to the same extent because patients did not live long enough, including renal dysfunction, metabolic bone disease, neurocognitive issues, and quality of life concerns. Open

Intestinal Rehabilitation, Episode 2: Overwhelming intestinal damage, Part 1

Statement The outlook for preterm infants with short bowel syndrome has changed over the last two decades as a result of advances in medical and surgical care. Open
Statement A more aggressive approach to surgical resection has accompanied improved outcomes in short bowel syndrome. Open
Statement Intestinal transplant is not experimental therapy; it is part of the continuum of therapy for a child with short bowel syndrome. Open
Statement In the acute phase with infarcted gut, the clinical picture is usually one of overwhelming sepsis, and even after resection leaving ultra-short bowel, patients are rocky and unstable due to sepsis, SIRS, or septic experience. Open
Statement In the very acute time, the primary cause of death is sepsis and multi-organ failure. Open
Statement In the intermediate and longer term, complications include intestinal failure-associated liver disease, recurrent sepsis, or line problems, which historically led to death or transplant. Open
Statement Much of the success seen in the last two decades is because clinicians are way better at preventing sepsis and liver disease. Open
Statement Babies born with duodenal atresia or very proximal jejunal atresia are born with elevated direct or conjugated bilirubins, supporting the concept that an undecompressed foregut affects liver function. Open
Statement Liver function is affected by multiple factors: prematurity, sepsis, choice of TPN, and presence or absence of enteral nutrition. Open
Paul's statements about Gastroschisis 28 statements

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Intestinal Rehabilitation, Episode 3: Enteral Autonomy, Part 1

Quotation That remaining bowel, that residual intestine goes through this process of adaptation and where the bowel is trying to compensate to reestablish function, to absorb enough nutrients and fluids to maintain survival. Open
Statement Adaptation is driven by intraluminal nutrients and their interaction with pancreatic biliary secretions and trophic gut peptides Open
Statement Structural changes during adaptation include mucosal hypertrophy with increased villous length, increased blood supply through angiogenesis, bowel dilation, and gut lengthening in younger children, all increasing surface area for absorption Open
Statement Functional changes during adaptation include slowed motility to allow more contact time and upregulation of enterocyte transporters Open
Quotation So, each, each cell is working harder, if you will, to try to, to, Function better and our job as a team is to try to promote that the best we can. Open
Statement Until recently there was no standardized definition for enteral autonomy Open
Statement TPN complications include line infections, liver disease, and vascular thrombosis Open
Statement In the past, patients were lost to complications such as liver disease before they could reach their adaptive potential; management of these complications has improved significantly Open
Statement Current ASPEN guidelines define enteral autonomy as independence of parenteral support for 12 weeks with maintenance of adequate growth and hydration Open
Quotation It's one thing to turn off parenteral support, but, you know, you're not really off TPN or off parental support unless you can actually grow off TPN. Open
Statement A 2012 PIFCO paper by Squires showed 50% of patients achieved enteral autonomy over 5-6 years, 25% died, and 25% got transplanted Open
Statement Recent papers in the last 5-6 years show 60-80% of patients achieve enteral autonomy, with a higher proportion surviving to reach autonomy Open
Statement Small bowel length is an independently significant variable for adaptation capacity Open
Statement The ileum has a much greater capacity to adapt than the jejunum Open
Statement At 5 years old, a child has about 425 centimeters of small bowel, with the steepest growth rate between 35 weeks gestation to about 6 months postnatal Open
Statement The ileocecal valve itself is not the important factor; most people who lose their ileocecal valve also lose their terminal ileum, which is the bigger factor for adaptive potential Open
Statement If a patient has the majority of their small bowel, it almost does not matter how much colon they have; probability of enteral autonomy is 85-100% Open

CAPS - The use of urine sodium to creatinine ratio as a marker of total body sodium in infants with intestinal failure - Sara Choi

Quotation This is often an underappreciated problem. Open
Statement Patients with stomas, even those without intestinal failure, run the risk of sodium depletion, which has a significant impact on their growth. Open
Statement Stomas and high stool losses are a large source of sodium bicarbonate as well as magnesium loss. Open
Statement Sodium status needs to be thought of, especially in the setting of a baby experiencing poor weight gain in the exposure to what is believed to be adequate calories. Open
Quotation It's important to remember that when we replace sodium, it does help restore growth, but it doesn't allow catch up growth. Open
Statement When sodium is replaced, it helps restore growth, but it doesn't allow catch up growth. Open
Statement It's important to track these patients serially so that a trend can be detected and sodium depletion can be avoided in the first place. Open
Statement If it becomes part of practice to order urine electrolytes at the time that someone's doing their TPN blood work, it's easy to calculate the fractional excretion of sodium ratio. Open
Statement Having potassium, chloride, and osmolality added to the urine electrolytes in addition to just the sodium gives a better picture of whether someone is truly sodium depleted. Open
Statement If potassium level is higher than sodium level in urine, that often indicates the aldosterone pathway is turned on. Open
Quotation If we see their potassium level higher than their sodium level, that often will tell you that the aldosterone pathway is turned on. Open
Paul's statements about Intestinal Failure 364 statements

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Intestinal rehabilitation: What is intestinal rehab? - Episode 1

Quotation Well, let's go back one step before that and sort of define what is intestinal failure because that sort of leads into who's going to benefit from an intestinal rehab program. Open
Statement Until recently, there was no standardized definition for intestinal failure. Open
Quotation It it's, it's really a functional problem. Essentially, when someone's gut for whatever the diagnosis, whatever the reason, their gut function is insufficient to absorb enough nutrients, fluids, calories to support survival and in the in the case of children, growth, which is a big distinction between adults and children. Open
Statement Intestinal failure is when gut function is insufficient to absorb enough nutrients, fluids, and calories to support survival and, in children, growth. Open
Quotation So, um, an intestinal rehabilitation program, as we defined also in these Aspen guidelines that just came out, is a multidisciplinary or interdisciplinary collaborative patient care paradigm that that brings coordinated care for children with intestinal failure through comprehensive management of Their specialized nutrition and other associated needs that they have. Open
Statement An intestinal rehabilitation program is a multidisciplinary collaborative patient care paradigm that brings coordinated care for children with intestinal failure through comprehensive management of their specialized nutrition and other associated needs, as defined in recent ASPEN guidelines. Open
Statement The intestinal rehabilitation approach streamlines care and improves communication with families and between care providers. Open
Quotation Patients that are gonna come to intestinal rehab program have intestinal failure, and we can divide the causes of intestinal failure into three categories. Open
Statement Causes of intestinal failure divide into three categories: short bowel syndrome, motility disorders, and congenital enteropathies. Open
Quotation There's short bowel syndrome, which by far is the most common category in pediatric patients with intestinal failure, and causes of short bowel syndrome are usually related to diagnoses of or, or disorders of the neonate. Open
Statement Short bowel syndrome is by far the most common category of intestinal failure in pediatric patients. Open
Statement Causes of short bowel syndrome include congenital anomalies (intestinal atresia, malrotation, volvulus, gastroschisis, long segment Hirschsprung disease) and acquired neonatal diseases (necrotizing enterocolitis). Open
Quotation So if we think of the intestine like a pipe, and that pipe is made of muscle that contracts in a coordinated way to push food and stool from one end to the other, you can have abnormalities of the muscle itself, or of the nerves that control that muscle. And the bowel is unable to push things through in a coordinated way. Open
Statement Motility disorders occur when abnormalities of intestinal muscle or the nerves controlling that muscle prevent coordinated propulsion of food and stool. Open
Quotation Enteropathies or the congenital diarrheas, and these are conditions where the patient has all of their bowel, but the mucosa, the inside lining that, that absorbs, uh, digests and absorbs doesn't work. Open
Statement Congenital enteropathies are conditions where patients have all their bowel but the mucosal lining does not digest or absorb properly. Open
Quotation The reason it's important to at least identify these three categories is that some patients will have elements of 12, or 3 of the categories, the way they present. Open
Statement Some patients have elements of two or all three categories of intestinal failure in their presentation. Open
Statement Most intestinal failure patients are infants or babies, but some older pediatric patients develop intestinal failure from inflammatory bowel disease, Crohn disease complications, trauma, malignancy, or vascular thrombosis. Open
Statement Long-term survivors now develop chronic comorbidities not previously seen to the same extent because patients did not live long enough, including renal dysfunction, metabolic bone disease, neurocognitive issues, and quality of life problems. Open
Quotation But now what we're seeing is we have kids that have developed chronic comorbidities, that comorbidities that never, we didn't really see to the same extent because they didn't live long enough. Open

Intestinal Rehabilitation, Episode 1: What is intestinal rehabilitation?

Quotation Well, let's go back one step before that and sort of define what is intestinal failure because that sort of leads into who's going to benefit from an intestinal rehab program. Open
Statement Until recently, there was no standardized definition for intestinal failure. Open
Quotation It it's, it's really a functional problem. Essentially, when someone's gut for whatever the diagnosis, whatever the reason, their gut function is insufficient to absorb enough nutrients, fluids, calories to support survival and in the in the case of children, growth, which is a big distinction between adults and children. Open
Statement Intestinal failure is a functional problem where gut function is insufficient to absorb enough nutrients, fluids, and calories to support survival and, in children, growth. Open
Statement An intestinal rehabilitation program is a multidisciplinary collaborative patient care paradigm that brings coordinated care for children with intestinal failure through comprehensive management of their specialized nutrition and other associated needs, as defined in recent ASPEN guidelines. Open
Quotation So, um, an intestinal rehabilitation program, as we defined also in these Aspen guidelines that just came out, is a multidisciplinary or interdisciplinary collaborative patient care paradigm that that brings coordinated care for children with intestinal failure through comprehensive management of Their specialized nutrition and other associated needs that they have. Open
Statement Intestinal rehabilitation streamlines care and improves communication between family and care providers through an amalgamation of experts providing holistic, comprehensive, coordinated care. Open
Quotation Patients that are gonna come to intestinal rehab program have intestinal failure, and we can divide the causes of intestinal failure into three categories. Open
Statement Causes of short bowel syndrome are usually related to neonatal disorders including congenital anomalies (intestinal atresia, malrotation, volvulus, gastroschisis, long segment Hirschsprung disease) or acquired diseases (necrotizing enterocolitis). Open
Statement Short bowel syndrome is by far the most common category of intestinal failure in pediatric patients. Open
Quotation There's short bowel syndrome, which by far is the most common category in pediatric patients with intestinal failure, and causes of short bowel syndrome are usually related to diagnoses of or, or disorders of the neonate. Open
Statement Motility disorders occur when abnormalities of the intestinal muscle itself or the nerves controlling that muscle prevent coordinated propulsion of food and stool, making patients dependent on intravenous support. Open
Quotation So if we think of the intestine like a pipe, and that pipe is made of muscle that contracts in a coordinated way to push food and stool from one end to the other, you can have abnormalities of the muscle itself, or of the nerves that control that muscle. And the bowel is unable to push things through in a coordinated way. Open
Quotation And they are therefore dependent on intravenous support. Open
Statement Enteropathies or congenital diarrheas are conditions where the patient has all of their bowel but the mucosal lining that digests and absorbs does not work, leading to hypersecretion and profuse fluid losses. Open
Quotation Enteropathies or the congenital diarrheas, and these are conditions where the patient has all of their bowel, but the mucosa, the inside lining that, that absorbs, uh, digests and absorbs doesn't work. Open
Statement Some patients will have elements of two or all three categories of intestinal failure; for example, gastroschisis can involve short bowel, inflammation affecting absorption, and motility issues. Open
Statement Although most intestinal failure patients are infants or babies, older pediatric patients can develop intestinal failure from inflammatory bowel disease, Crohn's disease complications, trauma, malignancy, or vascular thrombosis leading to gut loss. Open
Statement Improved survival has revealed chronic comorbidities not previously seen to the same extent because patients did not live long enough, including renal dysfunction, metabolic bone disease, neurocognitive issues, and quality of life concerns. Open

Intestinal Rehabilitation, Episode 2: Overwhelming intestinal damage, Part 1

Statement The outlook for preterm infants with short bowel syndrome has changed over the last two decades as a result of advances in medical and surgical care. Open
Statement A more aggressive approach to surgical resection has accompanied improved outcomes in short bowel syndrome. Open
Statement Intestinal transplant is not experimental therapy; it is part of the continuum of therapy for a child with short bowel syndrome. Open
Statement In the acute phase with infarcted gut, the clinical picture is usually one of overwhelming sepsis, and even after resection leaving ultra-short bowel, patients are rocky and unstable due to sepsis, SIRS, or septic experience. Open
Statement In the very acute time, the primary cause of death is sepsis and multi-organ failure. Open
Statement In the intermediate and longer term, complications include intestinal failure-associated liver disease, recurrent sepsis, or line problems, which historically led to death or transplant. Open
Statement Much of the success seen in the last two decades is because clinicians are way better at preventing sepsis and liver disease. Open
Statement Babies born with duodenal atresia or very proximal jejunal atresia are born with elevated direct or conjugated bilirubins, supporting the concept that an undecompressed foregut affects liver function. Open
Statement Liver function is affected by multiple factors: prematurity, sepsis, choice of TPN, and presence or absence of enteral nutrition. Open

Intestinal Rehabilitation, Episode 2: Overwhelming intestinal damage, Part 2

Quotation The goalposts have moved over the last two decades. Open
Quotation Like in Toronto, we, we classified that as less than 20% of expected for age. Open
Statement In Toronto, ultra-short gut is classified as less than 20% of expected bowel length for age; in a term baby that corresponds to 20–30 centimeters. Open
Statement Overall survival in the ultra-short gut population in the current era of management is over 90%, actually 90–95%. Open
Statement Ultra-short gut patients who reached enteral autonomy required multiple nutritional supplements but were able to grow within normal parameters. Open
Quotation There should be more optimism in general with this population just because our ability to mitigate those long term, you know, risks. Of death from liver disease or sepsis have been transformed in the current era of management. Open
Statement Long-term risks of death from liver disease or sepsis in ultra-short gut patients have been transformed in the current era of management. Open
Statement Ultra-short gut patients who did better tended to have some remnant ileum and longer colonic remnants. Open
Statement Neonatologists see some of the survivors—ex-premature infants with intestinal failure—return to their follow-up clinics, which informs their perspective. Open
Statement Not every child needs to die with a laparotomy decision; the data will tell whether the unoperated septic/inflammatory response leads to negative outcomes. Open
Quotation I don't subscribe to the notion that Every kid needs to die with a laparotomy indecision. Open
Statement Long-term neurocognitive tracking of this population is part of the program's responsibility and is beneficial to patients. Open
Statement Lipid restriction as a way of controlling liver disease was never a practice subscribed to by the speakers. Open
Quotation You know, that was never really a practice that we subscribe to. Open
Statement The surgeon's intraoperative decisions—what is done and what is not done—have a lifelong impact and are critically important; these decisions can determine whether the child stays on or gets off TPN or whether they survive. Open
Quotation The surgeon's role in the patient with short bowel syndrome is, is obviously significant. Partly for what we do and partly for what we don't do, right? Open
Quotation Those intraoperative decisions have a lifelong impact. Open
Quotation So, those, those decisions that you make at the time that you see an intraabdominal catastrophe can make the difference between whether this kid will get, stay on or get off TPN or whether they survive or don't. Open
Statement Traditional teaching is to remove obviously dead and infarcted bowel, leave suspicious or questionable bowel, and return in 24–48 hours to let it demarcate; the approach described is a departure from that teaching. Open
Statement Some of these children look worse over the next 24–36 hours, then start to stabilize—they may not get better, but they stop getting worse. Open
Quotation Often, some of these kids do get, they kind of look worse over the next 24, 36 hours, and then they start to, you know, they may not even be getting better, they just stop getting worse. Open

Intestinal Rehabilitation, Episode 3: Enteral Autonomy, Part 1

Statement In short gut syndrome, residual intestine undergoes adaptation to compensate and reestablish function to absorb enough nutrients and fluids to maintain survival. Open
Quotation That remaining bowel, that residual intestine goes through this process of adaptation and where the bowel is trying to compensate to reestablish function, to absorb enough nutrients and fluids to maintain survival. Open
Statement In short gut syndrome, residual intestine undergoes adaptation to compensate and reestablish function to absorb enough nutrients and fluids to maintain survival. Open
Quotation That remaining bowel, that residual intestine goes through this process of adaptation and where the bowel is trying to compensate to reestablish function, to absorb enough nutrients and fluids to maintain survival. Open
Statement The adaptive process is driven by the presence of intraluminal nutrients and their interaction with gut secretions (pancreatic, biliary) and trophic gut peptides. Open
Statement The adaptive process is driven by the presence of intraluminal nutrients and their interaction with gut secretions (pancreatic, biliary) and trophic gut peptides. Open
Quotation The process is driven by The presence of intraluminal nutrients and their interaction with both gut secretions, you know, pancreatic biliary secretions, as well as the impact on the relationship with trophic gut peptides. Open
Quotation The process is driven by The presence of intraluminal nutrients and their interaction with both gut secretions, you know, pancreatic biliary secretions, as well as the impact on the relationship with trophic gut peptides. Open
Statement Structural changes during adaptation include mucosal hypertrophy (increased villous length), increased blood supply through angiogenesis, bowel dilation, and in younger children, gut lengthening—all increasing surface area for nutrient absorption. Open
Statement Structural changes during adaptation include mucosal hypertrophy (increased villous length), increased blood supply through angiogenesis, bowel dilation, and in younger children, gut lengthening—all increasing surface area for nutrient absorption. Open
Quotation What all of those things have in common is that they increase surface area for absorption absorption of nutrients, right? Open
Quotation What all of those things have in common is that they increase surface area for absorption absorption of nutrients, right? Open
Statement Functional changes during adaptation include slowed motility to allow more contact time and up-regulation of enterocyte transporters to move nutrients across cells more efficiently. Open
Statement Functional changes during adaptation include slowed motility to allow more contact time and up-regulation of enterocyte transporters to move nutrients across cells more efficiently. Open
Quotation So, each, each cell is working harder, if you will, to try to, to, Function better and our job as a team is to try to promote that the best we can. Open
Quotation So, each, each cell is working harder, if you will, to try to, to, Function better and our job as a team is to try to promote that the best we can. Open
Statement Until very recently, there was no standardized definition for enteral autonomy, and most intestinal failure outcomes have poor definitions. Open
Quotation Up until very recently, there actually was no standardized definition for that. Open
Statement Until very recently, there was no standardized definition for enteral autonomy, and most intestinal failure outcomes have poor definitions. Open
Quotation Up until very recently, there actually was no standardized definition for that. Open
Statement Higher proportions of patients are now surviving to have the ability to reach enteral autonomy due to better management of TPN complications such as line infections, liver disease, and vascular thrombosis. Open
Statement Higher proportions of patients are now surviving to have the ability to reach enteral autonomy due to better management of TPN complications such as line infections, liver disease, and vascular thrombosis. Open
Statement In the past, patients were lost to complications such as liver disease before they could reach their adaptive potential; current management is much better at preventing these complications. Open
Statement In the past, patients were lost to complications such as liver disease before they could reach their adaptive potential; current management is much better at preventing these complications. Open
Quotation So the current Aspen guidelines that just came out with standardized definitions, the independence of uh parental support for 12 weeks and with maintenance of adequate growth and hydration during that time period. Open
Statement The current ASPEN guidelines define enteral autonomy as independence from parenteral support for 12 weeks with maintenance of adequate growth and hydration during that time period. Open
Statement The current ASPEN guidelines define enteral autonomy as independence from parenteral support for 12 weeks with maintenance of adequate growth and hydration during that time period. Open
Quotation So the current Aspen guidelines that just came out with standardized definitions, the independence of uh parental support for 12 weeks and with maintenance of adequate growth and hydration during that time period. Open
Statement A patient is not truly off TPN unless they can actually grow off TPN; stopping TPN without achieving growth is a mistake. Open
Quotation Because it's one thing to turn off parenteral support, but, you know, you're not really off TPN or off parental support unless you can actually grow off TPN. Open
Statement A patient is not truly off TPN unless they can actually grow off TPN; stopping TPN without achieving growth is a mistake. Open
Quotation Because it's one thing to turn off parenteral support, but, you know, you're not really off TPN or off parental support unless you can actually grow off TPN. Open
Statement In the 2012 Squires/PIFCO paper, 50% of patients achieved enteral autonomy over 5-6 years, 25% died, and 25% received transplants. Open
Statement In the 2012 Squires/PIFCO paper, 50% of patients achieved enteral autonomy over 5-6 years, 25% died, and 25% received transplants. Open
Quotation Basically, 50% of patients achieved dental autonomy over 56 years, 25% died and 25% got transplanted. Open
Quotation Basically, 50% of patients achieved dental autonomy over 56 years, 25% died and 25% got transplanted. Open
Statement Recent papers from the last 5-6 years show that 60-80% of patients now achieve enteral autonomy, representing improved outcomes compared to historical data. Open
Statement Recent papers from the last 5-6 years show that 60-80% of patients now achieve enteral autonomy, representing improved outcomes compared to historical data. Open
Quotation Has increased between, depending on who you read, anywhere between 60 to 80%. Open
Quotation Has increased between, depending on who you read, anywhere between 60 to 80%. Open
Statement Small bowel length is an independently significant variable predicting adaptive capacity, which is intuitive since the majority of nutrient digestion and fluid absorption occurs in the small bowel. Open
Statement Small bowel length is an independently significant variable predicting adaptive capacity, which is intuitive since the majority of nutrient digestion and fluid absorption occurs in the small bowel. Open
Quotation You know, the ileum has a much greater capacity to adapt than the jejunum does. Open
Statement The ileum has a much greater capacity to adapt than the jejunum; patients with predominant ileal anatomy do better than those with predominant jejunal anatomy. Open
Statement The ileum has a much greater capacity to adapt than the jejunum; patients with predominant ileal anatomy do better than those with predominant jejunal anatomy. Open
Quotation You know, the ileum has a much greater capacity to adapt than the jejunum does. Open
Statement A full-term baby is born with approximately 160 centimeters of small bowel, which grows to about 425 centimeters by age 5 years, with the steepest growth rate between 35 weeks gestation and 6 months postnatal. Open
Statement A full-term baby is born with approximately 160 centimeters of small bowel, which grows to about 425 centimeters by age 5 years, with the steepest growth rate between 35 weeks gestation and 6 months postnatal. Open
Quotation When you're 5 years old, you have about 425 centimeters of small bowel, and the rate of growth on the curve is steepest between 35 weeks gestation to about 6 months postnatal. Open
Quotation When you're 5 years old, you have about 425 centimeters of small bowel, and the rate of growth on the curve is steepest between 35 weeks gestation to about 6 months postnatal. Open
Statement The presence or absence of the ileocecal valve is a predictor of adaptation, though the valve itself may not be the important factor; rather, loss of the valve typically accompanies loss of the terminal ileum, which is the bigger factor affecting adaptive potential. Open
Statement The presence or absence of the ileocecal valve is a predictor of adaptation, though the valve itself may not be the important factor; rather, loss of the valve typically accompanies loss of the terminal ileum, which is the bigger factor affecting adaptive potential. Open
Quotation Most people that lose their ileocecal valve also lose their terminal ileum, and I think that is the bigger factor as far as, uh, adaptive potential. Open
Quotation Most people that lose their ileocecal valve also lose their terminal ileum, and I think that is the bigger factor as far as, uh, adaptive potential. Open
Quotation So we've presented data that shows that if you've got the majority of your small bowel, then it almost doesn't matter how much colon you have, you have a probability of ventral autonomy somewhere between 85 to 100%. Open
Statement When a patient has the majority of their small bowel, it almost does not matter how much colon they have—probability of enteral autonomy is 85-100%. Open
Statement When a patient has the majority of their small bowel, it almost does not matter how much colon they have—probability of enteral autonomy is 85-100%. Open
Quotation So we've presented data that shows that if you've got the majority of your small bowel, then it almost doesn't matter how much colon you have, you have a probability of ventral autonomy somewhere between 85 to 100%. Open
Statement When small bowel remnant is less than 50% of expected length, the colon becomes vitally important, assuming an increasing role in energy absorption from short-chain fatty acids and fluid/salt absorption. Open
Statement When small bowel remnant is less than 50% of expected length, the colon becomes vitally important, assuming an increasing role in energy absorption from short-chain fatty acids and fluid/salt absorption. Open

CAPS - The use of urine sodium to creatinine ratio as a marker of total body sodium in infants with intestinal failure - Sara Choi

Quotation This is often an underappreciated problem. Open
Statement Patients with stomas, even those without intestinal failure, run the risk of sodium depletion, which has a significant impact on their growth. Open
Statement Stomas and high stool losses are a large source of sodium bicarbonate as well as magnesium loss. Open
Statement Sodium status needs to be thought of, especially in the setting of a baby experiencing poor weight gain in the exposure to what is believed to be adequate calories. Open
Statement When sodium is replaced, it helps restore growth, but it doesn't allow catch up growth. Open
Quotation It's important to remember that when we replace sodium, it does help restore growth, but it doesn't allow catch up growth. Open
Statement It's important to track these patients serially so that a trend can be detected and sodium depletion can be avoided in the first place. Open
Statement If it becomes part of practice to order urine electrolytes at the time that someone's doing their TPN blood work, it's easy to calculate the fractional excretion of sodium ratio. Open
Statement Having potassium, chloride, and osmolality added to the urine electrolytes in addition to just the sodium gives a better picture of whether someone is truly sodium depleted. Open
Statement If potassium level is higher than sodium level in urine, that often indicates the aldosterone pathway is turned on. Open
Quotation If we see their potassium level higher than their sodium level, that often will tell you that the aldosterone pathway is turned on. Open

Pediatric Vascular Access in Brief: Preoperative, Operative, and Postoperative Considerations

Statement Traditional locking solutions are predominantly antibiotic locks, but they lead to development of bacterial resistance. Open
Quotation Antimicrobial, it doesn't have any resistance, and it'll kill bacteria that are both planktonic, meaning floating around in the, in the lumen, or sessile, meaning that they're embedded in a biofilm along the wall of the catheter. Open
Statement Ethanol locks are antimicrobial without resistance development and kill both planktonic bacteria (floating in the lumen) and sessile bacteria (embedded in biofilm along the catheter wall). Open
Statement Kite lock is a 4% tetrasodium EDTA solution that is antimicrobial without resistance, and has antifibrinolytic and antithrombotic properties. Open
Statement Kite lock is licensed for pediatric use in Canada, Europe, and Australia, but availability varies by region. Open

Intestinal Rehabilitation, Episode 4: Surgical Management, Part 1

Statement The majority of pediatric short bowel syndrome cases present at birth due to neonatal causes: congenital GI anomalies or acquired conditions like necrotizing enterocolitis. Open
Quotation If you are thinking of reestablishing continuity, I feel very strongly that you have to deal with the size discrepancy. Open
Statement When reestablishing bowel continuity, size discrepancy between bowel ends must be addressed or a functional obstruction will result even if the anastomosis is patent. Open
Statement Size discrepancy can be managed by resection to a more appropriate caliber, tapering enteroplasty, or serial transverse enteroplasty (STEP) depending on available bowel length. Open
Statement Gastroschisis patients tend to not do as well with STEP procedures regardless of whether atresia is present; among STEP procedures performed over the years, gastroschisis cases consistently had worse outcomes. Open
Quotation In general, gastroschisis, they don't tend to do as well regardless. Open
Quotation If, and if I look at the step procedures that I've done over the years, the ones that tend to not do as well or tend to always be the gastroschisis. Open
Quotation I don't usually do that operation anyway unless the bowel is an adequate diameter to make it worthwhile, and in my head over the years, it's always been about 5 centimeters anyway. Open
Statement STEP procedure is not usually performed unless bowel is at least 5 centimeters dilated to make it worthwhile. Open
Statement In scenarios where bowel is not adequately dilated, options include resecting bulbs with tapering enteroplasty, establishing continuity if possible, or leaving bowel disconnected with distal feeding tube access to grow the distal bowel before reconnection. Open
Statement The STEP procedure was originally described by HP Kim and Tom Jackson. Open
Statement For STEP, a transverse supraumbilical abdominal incision is used, all adhesions are lysed, and the entire intestinal tract is laid out from top to bottom to understand anatomy and maximize mobility. Open
Statement The anti-mesenteric surface of the bowel is marked with surgical pen after drying with a lap sponge; maintaining this alignment prevents longitudinal twisting of the bowel during stapling. Open
Statement Bowel length is measured before the procedure using 30 silk dipped in mineral oil; caliber and length of the dilated segment to be stepped are also recorded. Open
Statement An endo-GIA stapler is preferred over an open GIA stapler because the smaller cartridge head is more amenable in smaller patients. Open
Statement Staple lines are applied perpendicular to the mesentery at 90° and 270° (3 o'clock and 9 o'clock positions if mesenteric vessels are at 0°). Open
Statement A vascular (white) cartridge with 2.5 mm staples that crimp to 1 mm is used; this is more appropriate for younger patients' bowel thickness and prevents leaks that occurred with classic blue loads in early experience. Open
Statement Target caliber for STEP is 1.5 cm in babies and 2 to 2.5 cm in older infants or children; making it too narrow risks obstruction, especially in patients with borderline motility. Open
Statement Before firing the stapler, measurements are taken to both the end of the bowel and to the side to ensure proper caliber is maintained throughout the length. Open
Statement A U-stitch with 4-0 PDS is placed in the crotch of each staple line to prevent potential leaks at that point. Open
Statement The procedure is performed with a partner, alternating sides of the bowel, working down the length; 45 mm cartridges are most versatile, but 30 mm and 60 mm cartridges may be needed depending on bowel diameter. Open
Statement At the top and bottom of the STEP segment, an additional oblique staple line is fired to taper the transition and avoid a 'dog ear' blind loop that can dilate over time. Open
Statement The STEP procedure is performed with staple lines perpendicular to the mesentery (90° and 270°), not in the anti-mesenteric to mesenteric orientation (180° and 0°). Open
Statement The duodenum is not stepped; the STEP procedure starts where the bowel begins to dilate, usually distal to the duodenum. Open
Statement Avoiding staple lines in the duodenum allows proximal access if emergency reoperation is needed for a leak. Open
Statement If duodenal narrowing is needed, a stapler is used to partially narrow the lateral side away from the bile duct and ampulla; plication sutures are not used because they usually fail. Open

Intestinal Rehabilitation, Episode 4: Surgical Management, Part 1

Statement The majority of pediatric short bowel syndrome cases present at birth due to neonatal causes: congenital GI anomalies or acquired conditions like necrotizing enterocolitis. Open
Quotation If you are thinking of reestablishing continuity, I feel very strongly that you have to deal with the size discrepancy. Open
Statement When reestablishing bowel continuity, size discrepancy between bowel ends must be addressed or a functional obstruction will result even if the anastomosis is patent. Open
Statement Size discrepancy can be managed by resection to a more appropriate caliber, tapering enteroplasty, or serial transverse enteroplasty (STEP) depending on available bowel length. Open
Statement Gastroschisis patients tend to not do as well with STEP procedures regardless of whether atresia is present; among STEP procedures performed over the years, gastroschisis cases consistently had worse outcomes. Open
Quotation In general, gastroschisis, they don't tend to do as well regardless. Open
Quotation If, and if I look at the step procedures that I've done over the years, the ones that tend to not do as well or tend to always be the gastroschisis. Open
Statement STEP procedure is not usually performed unless bowel is at least 5 centimeters dilated to make it worthwhile. Open
Quotation I don't usually do that operation anyway unless the bowel is an adequate diameter to make it worthwhile, and in my head over the years, it's always been about 5 centimeters anyway. Open
Statement In scenarios where bowel is not adequately dilated, options include resecting bulbs with tapering enteroplasty, establishing continuity if possible, or leaving bowel disconnected with distal feeding tube access to grow the distal bowel before reconnection. Open
Statement The STEP procedure was originally described by HP Kim and Tom Jackson. Open
Statement For STEP, a transverse supraumbilical abdominal incision is used, all adhesions are lysed, and the entire intestinal tract is laid out from top to bottom to understand anatomy and maximize mobility. Open
Statement The anti-mesenteric surface of the bowel is marked with surgical pen after drying with a lap sponge; maintaining this alignment prevents longitudinal twisting of the bowel during stapling. Open
Statement Bowel length is measured before the procedure using 30 silk dipped in mineral oil; caliber and length of the dilated segment to be stepped are also recorded. Open
Statement An endo-GIA stapler is preferred over an open GIA stapler because the smaller cartridge head is more amenable in smaller patients. Open
Statement Staple lines are applied perpendicular to the mesentery at 90° and 270° (3 o'clock and 9 o'clock positions if mesenteric vessels are at 0°). Open
Statement A vascular (white) cartridge with 2.5 mm staples that crimp to 1 mm is used; this is more appropriate for younger patients' bowel thickness and prevents leaks that occurred with classic blue loads in early experience. Open
Statement Target caliber for STEP is 1.5 cm in babies and 2 to 2.5 cm in older infants or children; making it too narrow risks obstruction, especially in patients with borderline motility. Open
Statement Before firing the stapler, measurements are taken to both the end of the bowel and to the side to ensure proper caliber is maintained throughout the length. Open
Statement A U-stitch with 4-0 PDS is placed in the crotch of each staple line to prevent potential leaks at that point. Open
Statement The procedure is performed with a partner, alternating sides of the bowel, working down the length; 45 mm cartridges are most versatile, but 30 mm and 60 mm cartridges may be needed depending on bowel diameter. Open
Statement At the top and bottom of the STEP segment, an additional oblique staple line is fired to taper the transition and avoid a 'dog ear' blind loop that can dilate over time. Open
Statement The STEP procedure is performed with staple lines perpendicular to the mesentery (90° and 270°), not in the anti-mesenteric to mesenteric orientation (180° and 0°). Open
Statement The duodenum is not stepped; the STEP procedure starts where the bowel begins to dilate, usually distal to the duodenum. Open
Statement Avoiding staple lines in the duodenum allows proximal access if emergency reoperation is needed for a leak. Open
Statement If duodenal narrowing is needed, a stapler is used to partially narrow the lateral side away from the bile duct and ampulla; plication sutures are not used because they usually fail. Open

Intestinal Rehabilitation, Episode 4: Surgical Management, Part 2

Quotation I think we have to remember that the biggest benefit of the step, for instance, is the fact that you're tapering the bowel and reestablishing a more normal caliber to improve motility. Open
Statement The biggest benefit of the STEP procedure is tapering the bowel and reestablishing a more normal caliber to improve motility. Open
Statement It can take up to 6 months before you actually start to see a significant improvement in absorptive capacity after STEP. Open
Quotation It can take up to 6 months before you actually start to see a significant improvement in absorptive capacity. Open
Statement Improvements in absorptive capacity are measured with fecal fat, alpha-1 antitrypsin clearance, xylose absorption, and citrulline levels as they rise over time. Open
Statement The reason absorptive improvement takes time is that inflamed, sick, leaky mucosa in the setting of bacterial overgrowth needs to heal. Open
Statement Bleeding at STEP staple lines is an underreported complication that is really difficult to manage in some cases. Open
Quotation My hypothesis is that this is a microbiome problem. I think you have a pro-inflammatory environment. Open
Statement Staple line bleeding is hypothesized to be a microbiome problem occurring in a pro-inflammatory environment. Open
Statement Staple line bleeding tends to occur in type 2 anatomy, which is small bowel to colonic remnant in the absence of an intact colon ileocecal valve. Open
Statement Pathology of staple line ulcers shows non-specific inflammation with no vasculitis, no viral elements, and no obvious ischemia. Open
Quotation It's non-specific inflammation. There's no vasculitis, there's no viral elements, there's no obvious ischemia, and the management can be super difficult. Open
Statement Most referrals for intestinal rehabilitation have had multiple operations before coming to the specialist center. Open
Statement Optimizing a child's anatomy may not be conducive to just one operation; you have to set yourself up sometimes planning for the next case. Open
Quotation Keep your eye on the horizon, which is what can we do to try to optimize this kid's anatomy. And if you think about that plan, it may not be conducive to just one operation today. Open

Intestinal Rehabilitation, Episode 6: Cholestasis

Quotation Institutionally defined it as a conjugated bilirubin greater than 3 mg per deciliter or 50 micromoles per liter sustained for 2 weeks and not associated with a septic event. Open
Statement Cincinnati Children's Hospital institutionally defined cholestasis as conjugated bilirubin greater than 3 mg/dL or 50 micromoles/L sustained for 2 weeks and not associated with a septic event. Open
Statement A 2021 JPN publication defined cholestasis as conjugated bilirubin around 2 mg/dL or 34 micromoles/L for 2 weeks, not associated with a septic event. Open
Statement In young children, intestinal failure-associated liver disease presents as cholestatic liver disease, whereas in adolescents and adults it tends to be steatosis (fatty deposition). Open
Statement Prematurity is not modifiable by the clinical team, but lack of enteral feeding, sepsis, and TPN components are modifiable risk factors. Open
Quotation Now, prematurity, we as clinicians, as a team, we don't have much control over that. But the other factors are modifiable, and we can have an impact on that. Open
Statement Prevention of cholestasis requires aggressive introduction of enteral feeding to establish enterohepatic circulation and surgical procedures to optimize anatomy for feed delivery. Open
Statement Two strategies for reversing cholestasis are dose restriction and change of lipid composition. Open
Statement Conventional intralipid (soybean-based) produces prostaglandins and eicosanoids that are pro-inflammatory when metabolized. Open
Statement SMOF lipid promotes bile flow, is hepatoprotective, and can be delivered at conventional dose to achieve somatic growth and provide neurologic nutrients while protecting the liver. Open
Statement SMOF lipid does not contain enough arachidonic acid, so dose restriction of SMOF can lead to essential fatty acid deficiency. Open
Statement When SMOF is delivered at conventional dosing, no patients develop essential fatty acid deficiency. Open
Statement Conventional dosing for lipids is settling around 2.5 g/kg/day, but nutrition guidelines for preterm infants and babies state 3-4 g/kg/day. Open
Quotation My opinion is that a billy of 2 is not by any stretch advanced liver disease, and it's not dangerous, and it's usually transient. So I personally would not change the lipid emulsion out of Billy to. Open
Statement A bilirubin of 2 mg/dL is not advanced liver disease, is not dangerous, and is usually transient; Dr. Wales would not change lipid emulsion at this threshold. Open
Statement During secondary surgical or autologous reconstruction procedures, a liver biopsy is commonly taken to provide an up-to-date microscopic snapshot. Open
Statement Liver function and biochemistry are followed routinely as inpatient and at outpatient clinic visits. Open
Statement Elastography (FibroScan) is available for monitoring but is good for mild/no fibrosis or very advanced fibrosis; it is not as sensitive for patients with intermediate fibrosis. Open
Statement Follow-up visit frequency for children on TPN at home ranges from every 1-4 months depending on patient stability; actively adapting patients may be seen more frequently due to rapid changes being made. Open

Intestinal Rehabilitation, Episode 7: Refeeding in a neonatal patient

Statement One goal in intestinal failure management is to establish normal feeding habits that promote gut function and optimize quality of life including social aspects of eating. Open
Quotation Even if a kid has a complicated disease process, one of our goals as a team is to try to establish the most normal feeding habits we can that not only promote gut function and everything else, but also to optimize quality of life and the importance of the social aspects of eating that we all experience with our families and friends. Open
Statement When initiating feeds in patients with high stoma output, losses increase initially, but this must be pushed through in a hospital setting where volume replacement is available. Open
Statement In short bowel syndrome, protein absorption is fairly well preserved, so the benefit of completely broken-down protein formulas (free amino acids or hydrolysates) is primarily from an allergy perspective. Open
Quotation In the world of short bowel syndrome, protein absorption is actually fairly well preserved. So it seems like the benefit is from an allergy perspective. Open
Statement Many formulas have moved from predominant long-chain fat to increasing MCT components, but long-chain fat is a stronger driver for adaptation. Open
Quotation Over the last several years, a lot of the formulas have moved from predominant long chain fat to increasing in MCT components. But long chain fat is a stronger driver for adaptation, and I personally haven't really bought into that. Open
Statement One milliliter of parenteral nutrition is not isocaloric with one milliliter of formula, and advancing beyond 100-120 per kilo creates problems with not only calories and protein but also sodium and calcium due to compositional differences. Open
Quotation You're right, and they're not isocaloric, and so 1 mL of PN is not 1 mL of formula. So if you keep going beyond 100 per kilo or 120 per kilo, you, you have a problem. And kids, not only will they take a calorie protein hit, they'll take, you start to get into problems with sodium and calcium as well, because the composition in the milk is not the same as what's in the parental solution. Open
Statement At some point during feeding advancement, fortification is necessary. Open
Statement Feeding options include bolus (oral or gastric via tube), continuous (post-pyloric tube or surgical J-tube), or a hybrid model with gastric bolus component plus continuous component (gastric or post-pyloric). Open
Statement Bolus feeds are preferred as the default approach; patients should fail bolus feeds before being placed on continuous feeds as the sole delivery method. Open
Quotation Because I like bolus feeds as a default, I'd like, I like to see a patient fail bolus feeds before they end up on continuous feeds as a sole delivery remote. Open
Statement Continuous feeds can be used as a supplemental approach, with bolus feeds during the day and continuous supplementation overnight. Open
Statement Even when oral feeding is non-nutritive, it is important for skill development; children who never learn to suck, swallow, and process food by mouth will not eat solids later and will remain dependent on tube feeding. Open
Quotation One thing I just want to say about oral feeding is that even in the scenario where it's really non-nutritive. I think it's important for people to sort of look at that oral feeding, not so much as an internal nutrition perspective, but more of a skill development perspective that they never learn how to eat by mouth and suck and swallow and process, then they won't eat solids later when you want them to, and you're stuck basically with tube feeding on an older child. Open
Quotation Yeah, and it, it goes to the point that we've made in a previous podcast about having a plan and and resisting the temptation to get it all done in one. Operation. It's all about optimizing the patient for the next step, but you don't have to do it all in one go. Open
Statement The technical benefit of distal feeding is that size discrepancy at anastomosis is significantly improved because the bowel has been used. Open
Statement The hole from a removed G-tube closes very quickly. Open
Statement A G-tube provides versatility for nutrition supplementation, medication delivery, and venting for gassy or bloated children to improve enteral tolerance. Open

Intestinal Rehabilitation Episode 8: Refeeding of an Older Patient

Quotation The benefit in the curse in a patient like that is that they do know how to eat. They've established that that behavior. Open
Statement The benefit and curse in older patients with bowel loss is that they already know how to eat and have established that behavior. Open
Statement Even if you cannot cure the patient and get them off TPN, it is important to make compromises to optimize quality of life. Open
Quotation So even if you can't cure this patient and get them off TPN. It's important to make compromises to optimize quality of life the best you can. Open
Quotation The general strategy is you want to push the macronutrient modules of protein and fat are well tolerated. Most of these kids don't tolerate simple sugars very well. Open
Statement The general strategy is to push the macronutrient modules of protein and fat, which are well tolerated. Open
Statement Most patients with short bowel do not tolerate simple sugars very well. Open
Quotation So the general concept of pushing solids. And minimizing fluid intake helps. They don't tend to dump as much smaller meals more frequently of solids separated from their liquids to a certain extent, and minimization of simple sugars. Open
Statement The general concept of pushing solids and minimizing fluid intake helps prevent dumping. Open
Statement Smaller meals more frequently of solids separated from liquids, with minimization of simple sugars, reduces dumping tendency. Open
Statement Patients who have transitioned off TPN often come back with problems when diet history reveals they have gotten loose with diet choices, particularly increased sugars. Open
Statement Minimizing sugars reduces symptoms related to bacterial overgrowth, making patients less bloated and gassy. Open
Statement In older children with fluid losses (by stoma or other source), if they are on parenteral support, some fluid can be replaced IV. Open
Statement As you try to get patients off IV support, keeping them hydrated by replenishing losses through enteral replacements is important. Open
Quotation For transport requires sodium and glucose, and so your solution that you're replacing with has to contain some glucose and some salt. Open
Statement Transport requires sodium and glucose, so the rehydration solution must contain some glucose and some salt. Open
Statement There are homemade recipes for oral rehydration solutions and over-the-counter preparations available. Open
Quotation Some people will resort to things like Gatorade and stuff like that, but that doesn't always work that well. It's actually too much sugar in it. Open
Statement Gatorade does not always work well for rehydration because it has too much sugar in it. Open
Statement It is all about normal growth parameters and getting patients through puberty. Open
Statement Some kids end up back on parenteral support to get through puberty. Open
Quotation It is true some kids end up back on parental support, you know, to get through puberty. And then when you're not growing anymore as an adult, often that borderline or marginal gut function is enough to sustain them. Open
Statement When patients are not growing anymore as adults, borderline or marginal gut function is often enough to sustain them. Open
Statement Patients need to be monitored for micronutrient deficiencies after getting off TPN, as this is when they often get into trouble. Open
Statement Long-term growth and outcome need to be followed, highlighting the importance of multidisciplinary teams. Open
Statement Weight is tracked as a major metric for growth, but it has to be balanced with height. Open
Statement What is commonly seen in this population is round babies where their weight for height is elevated. Open
Quotation What we commonly see in this type of population is. Round babies where their weight for height is elevated Open
Statement There is increasing data looking at quality of weight: how much is fat weight versus lean body mass. Open

Advancements in Pediatric Intestinal Failure: Innovative Therapies and Improved Outcomes

Quotation They go anywhere from mild cholestasis to more profound steatosis, which we see in older children or adolescents and adults, with ongoing inflammation, fibrosis, and ultimately cirrhosis leading to death or the need for transplantation. Open
Statement Liver disease associated with intestinal failure ranges from mild cholestasis to profound steatosis (in older children/adolescents/adults), with progression through inflammation, fibrosis, and ultimately cirrhosis leading to death or transplantation. Open
Quotation There's some patient factors related to prematurity, uh, lack of enteral feeding, the inability to feed because of the anatomy or recurrent infections, sepsis, which can affect the liver directly and indirectly. And the components of TPN. Open
Statement Risk factors for intestinal failure-associated liver disease include prematurity, lack of enteral feeding, inability to feed due to anatomy, recurrent infections/sepsis, and components of TPN. Open
Statement The term 'intestinal rehabilitation program' was coined in the year 2000. Open
Quotation The term intestinal rehab program was coined in the year 2000. Open
Quotation Intestinal rehab teams improve the outcome of children because of integration of care, improved communication, better continuity. Open
Statement Intestinal rehabilitation teams improve outcomes through integration of care, improved communication, and better continuity. Open
Quotation We have a large reliance on speech, language, and occupational therapy. Open
Statement Intestinal rehabilitation programs rely heavily on speech, language, and occupational therapy specialists. Open
Quotation While our patients are on TPN, we're trying to mitigate the progression of liver disease, sepsis, vascular complications. Open
Statement While patients are on TPN, the goal is to mitigate progression of liver disease, sepsis, and vascular complications. Open
Quotation Having a dedicated unit with dedicated staff all contributes to better outcomes. Open
Statement Families of intestinal failure patients manage significant tasks at home including running TPN pumps, adding vitamins, programming pumps, recording ins and outs, managing G-tubes, and managing stomas. Open
Quotation What we ask our Families to do at home is significant. These patients go home on TPN. The families run the pump, add vitamins to the TPN, program the pump, record ins and outs, manage the G tube, manage the stoma. Open
Quotation We educate and empower our parents because we've all seen high-quality families that over time demonstrate evidence of burnout. Open
Statement High-quality families demonstrate evidence of burnout over time despite education and empowerment efforts. Open
Quotation There's literature that shows the improvement of outcomes with programs in general. You see an improved survival related to liver dysfunction, decreased septic episodes, reduction in central line complications, reduced ICU admissions, and the patients that were listed for transplant are coming off the list because of improved status. Mortality has gone down. Open
Statement Literature shows intestinal rehabilitation programs improve survival related to liver dysfunction, decrease septic episodes, reduce central line complications, reduce ICU admissions, and enable patients listed for transplant to come off the list due to improved status. Open
Statement Mortality in intestinal failure has decreased with intestinal rehabilitation programs. Open
Statement Lipids in parenteral nutrition are a source of essential fatty acids, a non-protein energy source important for growth, and fatty acids have a major role in cellular pathways. Open
Quotation Lipids are a source of essential fatty acids. They're a non-protein energy source important for growth. Fatty acids have a major role in cellular pathways. Open
Quotation Historically, most of the lipid emulsions in North America are soybean-based, so they have a high phytosterol content. A high omega 6 long chain polyunsaturated fatty acid content and low antioxidant content. Open
Statement Historically, most lipid emulsions in North America are soybean-based with high phytosterol content, high omega-6 long-chain polyunsaturated fatty acid content, and low antioxidant content. Open
Statement Prolonged soybean-based lipid exposure is associated with deterioration of liver function, specifically cholestasis. Open
Quotation It is associated with deterioration of liver dysfunction, i.e., cholestasis. Open
Statement For every day an infant is exposed to soybean lipid dosed at greater than 2.5 g/kg/day, there is a 3% increase in the odds ratio of developing advanced liver disease. Open
Quotation This is a paper that we wrote several years ago that showed that for every day that an infant is exposed to soybean lipid dosed at greater than 2.5 g per kilo per day, which is more conventional dosing. That is associated with a 3% increase in the odds ratio of developing advanced liver disease. Open
Quotation The first generation were basically the soybean-based lipids, rich in omega 6UUFA. That would be intralipids, and that's been around for 40 years and it's saved a lot of lives. Open
Statement First-generation lipid emulsions were soybean-based, rich in omega-6 PUFA (such as Intralipid), and have been in use for 40 years. Open
Statement Third-generation lipid emulsions reduce omega-6 component and change the omega-6 to omega-3 ratio by adding omega-3 lipids. Open
Quotation The 3rd generation lipids attempt to not only reduce the omega 6 component, but to change the omega 6 to the omega 3 ratio with the addition of omega 3 lipids. Open
Statement Omega-6 lipids are metabolized to arachidonic acid, which produces leukotrienes and prostaglandins with a more pro-inflammatory profile. Open
Quotation Omega 6 lipids are metabolized to arachidonic acid. Which produce leukotrienes and prostaglandins of the more pro-inflammatory profile. The omega 3 lipids are metabolized through EPA and DHA and lead to the production of cytokines that have a less inflammatory profile. Open
Statement Omega-3 lipids are metabolized through EPA and DHA, leading to production of cytokines with a less inflammatory profile. Open
Quotation Omega 3 lipids improve bile flow because they decrease lithogenicity of the bile. They decrease steatosis. They stimulate improved beta oxidation and clearance, and they lower oxidative stress and support immune function. Open
Statement Omega-3 lipids improve bile flow by decreasing lithogenicity of bile, decreasing steatosis, stimulating improved beta-oxidation and clearance, lowering oxidative stress, and supporting immune function. Open
Quotation One is lipid minimization, and then the second strategy is a change in composition. Open
Statement The two main strategies for improving lipids in parenteral nutrition are lipid minimization and change in composition. Open
Statement Literature dating back decades shows that restricting exposure to soybean lipid improves cholestasis, particularly when dosed at 1 g/kg/day or less. Open
Quotation There's a lot of literature dating back even decades that show that if you restrict exposure to soybean lipid, you improve cholestasis. When you dose the lipid at 1 g per kilo per day or less. Open
Statement In preterm babies, fat is important for growth, especially for neurocognitive development, creating a risk of essential fatty acid deficiency if lipid dosing is restricted too much. Open
Quotation The problem with this in preterm babies is that fat is important for growth, especially for neurocognitive development. So there is the risk of essential fatty acid deficiency if we restrict lipid dosing too much. Open
Quotation DHA and arachidonic acid are important for retinal and brain development. None of the lipid emulsions today were designed for premature babies. They were all designed for adults in a critical care setting. Open
Statement DHA and arachidonic acid are important for retinal and brain development. Open
Statement None of the current lipid emulsions were designed for premature babies; they were all designed for adults in critical care settings. Open
Statement Work is currently underway with industry to create a lipid emulsion specifically tailored to preterm babies with higher arachidonic acid content. Open
Quotation We're currently in work with industry to create a lipid emulsion that's more specifically tailored to preterm babies that has a higher content of arachidonic acid. Open
Statement Central lines are the lifeline for intestinal failure patients, and without venous access, families cannot be supported. Open
Quotation So our patients with central lines, it's literally their lifeline. And without venous access, we can't support our families. They are at risk for complications, infections, blockages, thrombosis, and breakage. Open
Statement Central lines are at risk for complications including infections, blockages, thrombosis, and breakage. Open
Statement In the United States, there are approximately 240,000 central line-associated bloodstream infections (CLABSIs) annually, with each costing about $30,000, totaling approximately $2 billion per year nationwide. Open
Quotation In the United States, there's about 240,000 collapses in the United States on an annual basis. The cost of one of these is about $30,000 about $2 billion a year nationwide. Open
Statement 4% tetrasodium EDTA (Kitelock) is antithrombotic, anti-fibrinolytic, and antimicrobial, satisfying all three criteria important for a good lock solution. Open
Statement The team pivoted to 4% tetrasodium EDTA (Taurolock/Kitelock) when ethanol became too expensive and difficult to obtain. Open
Quotation We landed on 4% tetrosodium EDTA or kite lock as it's known in the trade name, which is an antithrombolytic, anti-fibrinolytic, and antimicrobial. So, it satisfies all three criteria that are important for a good lock solution. Open
Statement Kitelock is licensed in Europe and Australia but not available in the United States. Open
Quotation It's licensed in Europe, it's licensed in Australia. It's not available in the United States. Open
Statement A multi-center randomized trial of Kitelock versus heparin went live 4 weeks ago and is actively recruiting patients, with results expected within the next year. Open
Quotation I'm happy to say that we have finally launched our multi-center randomized trial of kitelock versus heparin, which went live 4 weeks ago, and we're actively recruiting patients. In the next year, we'll have the results of the trial, and we can get the kitelock into the United States. Open
Statement In short bowel syndrome, there are three anatomical subtypes: Type 1 (high jejunostomy), Type 2 (loss of distal small bowel/ileum and part of colon with small bowel-colonic anastomosis), and Type 3 (loss of mid-small bowel with retained ileum and intact colon). Open
Quotation In short bowel syndrome, we have 3 anatomical subtypes. Type 1 is the high L piginostomy. Type 2, where you've lost your distal small bowel, your ileum, and part of your colon, and there's a small bowel colonic anastomosis. And then type 3 where you've lost the mid-part of your small bowel, but you still have some retained ileum and an intact colon. Open
Quotation The most common anatomical subtype in pediatrics is type 2. These patients struggle. Type 1 and type 2 struggle because they lack an ileum. Open
Statement The most common anatomical subtype of short bowel syndrome in pediatrics is Type 2. Open
Statement Type 1 and Type 2 short bowel syndrome patients struggle because they lack an ileum. Open
Statement Native GLP-2 hormone has a half-life of 7 minutes, making it impractical for clinical use. Open
Quotation The problem is that the native hormone has a half-life of 7 minutes. So it's not practical. So it's been altered, one amino acid alteration to an analog called taglutide. That has a half-life of 2 hours and it's given once a day subcutaneous injection. Open
Statement GLP-2 has been altered by one amino acid to create an analog called teduglutide, which has a half-life of 2 hours and is given once daily by subcutaneous injection. Open
Statement A multi-center phase 3 study published in 2020 at Cincinnati Children's showed patients receiving teduglutide had a 40% reduction in TPN fluid and calorie requirements over a six-month period. Open
Quotation What we see in patients that received taglutide, a 40% reduction in TPN fluid and calorie requirements over a six-month period. And 70% of patients that received the analog achieved the study endpoint, which was a 20% reduction in TPN requirements. Open
Statement 70% of patients who received teduglutide achieved the study endpoint of a 20% reduction in TPN requirements. Open
Statement Teduglutide is licensed for children greater than 1 year of age. Open
Quotation It's licensed for children that are greater than 1 year of age. Already, it's made a big difference in our practice and even kids with very extreme anatomy have been able to get off TPN as a result of being able to replace this hormone. Open
Statement Teduglutide has made a big difference in practice, with even children with very extreme anatomy able to get off TPN. Open
Statement Another GLP-2 analog, apraglutide, has a longer half-life and can be given once weekly. Open
Quotation Another GLP-2 analog, a proglutide, has a longer half-life and can be given once a week. That's currently being studied. And what we've shown is that it actually works even better than taglutide. You get more bowel lengthening with a proglutide compared to glutide. Open
Statement Apraglutide works even better than teduglutide, producing more bowel lengthening. Open
Statement These interventions (optimized lipids, central line infection prevention, GLP-2 analogs) have led to a decrease in transplantation since 2008. Open
Quotation These interventions have led to a decrease in transplantation since 2008. In large part because of the successes of intestinal rehabilitation, the outcomes of transplant at 5 years are about 65%. Whatever we can do to avoid transplant and lifelong immunosuppression, that is our goal. Open
Statement Outcomes of intestinal transplant at 5 years are approximately 65%. Open
Statement The decrease in transplantation is in large part because of the successes of intestinal rehabilitation. Open
Statement The goal is to avoid transplant and lifelong immunosuppression whenever possible. Open
Quotation We have lots of exciting medical and surgical options available to us, but this should all be done under the umbrella of a multidisciplinary team. Open
Paul's statements about Intestinal Rehab 468 statements

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Intestinal rehabilitation: What is intestinal rehab? - Episode 1

Quotation Well, let's go back one step before that and sort of define what is intestinal failure because that sort of leads into who's going to benefit from an intestinal rehab program. Open
Statement Until recently, there was no standardized definition for intestinal failure. Open
Quotation It it's, it's really a functional problem. Essentially, when someone's gut for whatever the diagnosis, whatever the reason, their gut function is insufficient to absorb enough nutrients, fluids, calories to support survival and in the in the case of children, growth, which is a big distinction between adults and children. Open
Statement Intestinal failure is when gut function is insufficient to absorb enough nutrients, fluids, and calories to support survival and, in children, growth. Open
Statement An intestinal rehabilitation program is a multidisciplinary collaborative patient care paradigm that brings coordinated care for children with intestinal failure through comprehensive management of their specialized nutrition and other associated needs, as defined in recent ASPEN guidelines. Open
Quotation So, um, an intestinal rehabilitation program, as we defined also in these Aspen guidelines that just came out, is a multidisciplinary or interdisciplinary collaborative patient care paradigm that that brings coordinated care for children with intestinal failure through comprehensive management of Their specialized nutrition and other associated needs that they have. Open
Statement The intestinal rehabilitation approach streamlines care and improves communication with families and between care providers. Open
Statement Causes of intestinal failure divide into three categories: short bowel syndrome, motility disorders, and congenital enteropathies. Open
Quotation Patients that are gonna come to intestinal rehab program have intestinal failure, and we can divide the causes of intestinal failure into three categories. Open
Quotation There's short bowel syndrome, which by far is the most common category in pediatric patients with intestinal failure, and causes of short bowel syndrome are usually related to diagnoses of or, or disorders of the neonate. Open
Statement Short bowel syndrome is by far the most common category of intestinal failure in pediatric patients. Open
Statement Causes of short bowel syndrome include congenital anomalies (intestinal atresia, malrotation, volvulus, gastroschisis, long segment Hirschsprung disease) and acquired neonatal diseases (necrotizing enterocolitis). Open
Statement Motility disorders occur when abnormalities of intestinal muscle or the nerves controlling that muscle prevent coordinated propulsion of food and stool. Open
Quotation So if we think of the intestine like a pipe, and that pipe is made of muscle that contracts in a coordinated way to push food and stool from one end to the other, you can have abnormalities of the muscle itself, or of the nerves that control that muscle. And the bowel is unable to push things through in a coordinated way. Open
Statement Congenital enteropathies are conditions where patients have all their bowel but the mucosal lining does not digest or absorb properly. Open
Quotation Enteropathies or the congenital diarrheas, and these are conditions where the patient has all of their bowel, but the mucosa, the inside lining that, that absorbs, uh, digests and absorbs doesn't work. Open
Quotation The reason it's important to at least identify these three categories is that some patients will have elements of 12, or 3 of the categories, the way they present. Open
Statement Some patients have elements of two or all three categories of intestinal failure in their presentation. Open
Statement Most intestinal failure patients are infants or babies, but some older pediatric patients develop intestinal failure from inflammatory bowel disease, Crohn disease complications, trauma, malignancy, or vascular thrombosis. Open
Quotation But now what we're seeing is we have kids that have developed chronic comorbidities, that comorbidities that never, we didn't really see to the same extent because they didn't live long enough. Open
Statement Long-term survivors now develop chronic comorbidities not previously seen to the same extent because patients did not live long enough, including renal dysfunction, metabolic bone disease, neurocognitive issues, and quality of life problems. Open

Intestinal Rehabilitation, Episode 1: What is intestinal rehabilitation?

Quotation Well, let's go back one step before that and sort of define what is intestinal failure because that sort of leads into who's going to benefit from an intestinal rehab program. Open
Statement Until recently, there was no standardized definition for intestinal failure. Open
Quotation It it's, it's really a functional problem. Essentially, when someone's gut for whatever the diagnosis, whatever the reason, their gut function is insufficient to absorb enough nutrients, fluids, calories to support survival and in the in the case of children, growth, which is a big distinction between adults and children. Open
Statement Intestinal failure is a functional problem where gut function is insufficient to absorb enough nutrients, fluids, and calories to support survival and, in children, growth. Open
Quotation So, um, an intestinal rehabilitation program, as we defined also in these Aspen guidelines that just came out, is a multidisciplinary or interdisciplinary collaborative patient care paradigm that that brings coordinated care for children with intestinal failure through comprehensive management of Their specialized nutrition and other associated needs that they have. Open
Statement An intestinal rehabilitation program is a multidisciplinary collaborative patient care paradigm that brings coordinated care for children with intestinal failure through comprehensive management of their specialized nutrition and other associated needs, as defined in recent ASPEN guidelines. Open
Statement Intestinal rehabilitation streamlines care and improves communication between family and care providers through an amalgamation of experts providing holistic, comprehensive, coordinated care. Open
Quotation Patients that are gonna come to intestinal rehab program have intestinal failure, and we can divide the causes of intestinal failure into three categories. Open
Statement Causes of short bowel syndrome are usually related to neonatal disorders including congenital anomalies (intestinal atresia, malrotation, volvulus, gastroschisis, long segment Hirschsprung disease) or acquired diseases (necrotizing enterocolitis). Open
Statement Short bowel syndrome is by far the most common category of intestinal failure in pediatric patients. Open
Quotation There's short bowel syndrome, which by far is the most common category in pediatric patients with intestinal failure, and causes of short bowel syndrome are usually related to diagnoses of or, or disorders of the neonate. Open
Statement Motility disorders occur when abnormalities of the intestinal muscle itself or the nerves controlling that muscle prevent coordinated propulsion of food and stool, making patients dependent on intravenous support. Open
Quotation So if we think of the intestine like a pipe, and that pipe is made of muscle that contracts in a coordinated way to push food and stool from one end to the other, you can have abnormalities of the muscle itself, or of the nerves that control that muscle. And the bowel is unable to push things through in a coordinated way. Open
Quotation And they are therefore dependent on intravenous support. Open
Quotation Enteropathies or the congenital diarrheas, and these are conditions where the patient has all of their bowel, but the mucosa, the inside lining that, that absorbs, uh, digests and absorbs doesn't work. Open
Statement Enteropathies or congenital diarrheas are conditions where the patient has all of their bowel but the mucosal lining that digests and absorbs does not work, leading to hypersecretion and profuse fluid losses. Open
Statement Some patients will have elements of two or all three categories of intestinal failure; for example, gastroschisis can involve short bowel, inflammation affecting absorption, and motility issues. Open
Statement Although most intestinal failure patients are infants or babies, older pediatric patients can develop intestinal failure from inflammatory bowel disease, Crohn's disease complications, trauma, malignancy, or vascular thrombosis leading to gut loss. Open
Statement Improved survival has revealed chronic comorbidities not previously seen to the same extent because patients did not live long enough, including renal dysfunction, metabolic bone disease, neurocognitive issues, and quality of life concerns. Open

Intestinal Rehabilitation, Episode 2: Overwhelming intestinal damage, Part 1

Statement The outlook for preterm infants with short bowel syndrome has changed over the last two decades as a result of advances in medical and surgical care. Open
Statement A more aggressive approach to surgical resection has accompanied improved outcomes in short bowel syndrome. Open
Statement Intestinal transplant is not experimental therapy; it is part of the continuum of therapy for a child with short bowel syndrome. Open
Statement In the acute phase with infarcted gut, the clinical picture is usually one of overwhelming sepsis, and even after resection leaving ultra-short bowel, patients are rocky and unstable due to sepsis, SIRS, or septic experience. Open
Statement In the very acute time, the primary cause of death is sepsis and multi-organ failure. Open
Statement In the intermediate and longer term, complications include intestinal failure-associated liver disease, recurrent sepsis, or line problems, which historically led to death or transplant. Open
Statement Much of the success seen in the last two decades is because clinicians are way better at preventing sepsis and liver disease. Open
Statement Babies born with duodenal atresia or very proximal jejunal atresia are born with elevated direct or conjugated bilirubins, supporting the concept that an undecompressed foregut affects liver function. Open
Statement Liver function is affected by multiple factors: prematurity, sepsis, choice of TPN, and presence or absence of enteral nutrition. Open

Intestinal Rehabilitation, Episode 2: Overwhelming intestinal damage, Part 2

Quotation The goalposts have moved over the last two decades. Open
Quotation The goalposts have moved over the last two decades. Open
Statement In Toronto, ultra-short gut is classified as less than 20% of expected bowel length for age; in a term baby that corresponds to 20–30 centimeters. Open
Statement In Toronto, ultra-short gut is classified as less than 20% of expected bowel length for age; in a term baby that corresponds to 20–30 centimeters. Open
Quotation Like in Toronto, we, we classified that as less than 20% of expected for age. Open
Quotation Like in Toronto, we, we classified that as less than 20% of expected for age. Open
Statement Overall survival in the ultra-short gut population in the current era of management is over 90%, actually 90–95%. Open
Statement Overall survival in the ultra-short gut population in the current era of management is over 90%, actually 90–95%. Open
Statement Ultra-short gut patients who reached enteral autonomy required multiple nutritional supplements but were able to grow within normal parameters. Open
Statement Ultra-short gut patients who reached enteral autonomy required multiple nutritional supplements but were able to grow within normal parameters. Open
Quotation There should be more optimism in general with this population just because our ability to mitigate those long term, you know, risks. Of death from liver disease or sepsis have been transformed in the current era of management. Open
Quotation There should be more optimism in general with this population just because our ability to mitigate those long term, you know, risks. Of death from liver disease or sepsis have been transformed in the current era of management. Open
Statement Long-term risks of death from liver disease or sepsis in ultra-short gut patients have been transformed in the current era of management. Open
Statement Long-term risks of death from liver disease or sepsis in ultra-short gut patients have been transformed in the current era of management. Open
Statement Ultra-short gut patients who did better tended to have some remnant ileum and longer colonic remnants. Open
Statement Ultra-short gut patients who did better tended to have some remnant ileum and longer colonic remnants. Open
Statement Neonatologists see some of the survivors—ex-premature infants with intestinal failure—return to their follow-up clinics, which informs their perspective. Open
Statement Neonatologists see some of the survivors—ex-premature infants with intestinal failure—return to their follow-up clinics, which informs their perspective. Open
Statement Not every child needs to die with a laparotomy decision; the data will tell whether the unoperated septic/inflammatory response leads to negative outcomes. Open
Statement Not every child needs to die with a laparotomy decision; the data will tell whether the unoperated septic/inflammatory response leads to negative outcomes. Open
Quotation I don't subscribe to the notion that Every kid needs to die with a laparotomy indecision. Open
Quotation I don't subscribe to the notion that Every kid needs to die with a laparotomy indecision. Open
Statement Long-term neurocognitive tracking of this population is part of the program's responsibility and is beneficial to patients. Open
Statement Long-term neurocognitive tracking of this population is part of the program's responsibility and is beneficial to patients. Open
Statement Lipid restriction as a way of controlling liver disease was never a practice subscribed to by the speakers. Open
Statement Lipid restriction as a way of controlling liver disease was never a practice subscribed to by the speakers. Open
Quotation You know, that was never really a practice that we subscribe to. Open
Quotation You know, that was never really a practice that we subscribe to. Open
Statement The surgeon's intraoperative decisions—what is done and what is not done—have a lifelong impact and are critically important; these decisions can determine whether the child stays on or gets off TPN or whether they survive. Open
Quotation The surgeon's role in the patient with short bowel syndrome is, is obviously significant. Partly for what we do and partly for what we don't do, right? Open
Quotation The surgeon's role in the patient with short bowel syndrome is, is obviously significant. Partly for what we do and partly for what we don't do, right? Open
Statement The surgeon's intraoperative decisions—what is done and what is not done—have a lifelong impact and are critically important; these decisions can determine whether the child stays on or gets off TPN or whether they survive. Open
Quotation Those intraoperative decisions have a lifelong impact. Open
Quotation Those intraoperative decisions have a lifelong impact. Open
Quotation So, those, those decisions that you make at the time that you see an intraabdominal catastrophe can make the difference between whether this kid will get, stay on or get off TPN or whether they survive or don't. Open
Quotation So, those, those decisions that you make at the time that you see an intraabdominal catastrophe can make the difference between whether this kid will get, stay on or get off TPN or whether they survive or don't. Open
Statement Traditional teaching is to remove obviously dead and infarcted bowel, leave suspicious or questionable bowel, and return in 24–48 hours to let it demarcate; the approach described is a departure from that teaching. Open
Statement Traditional teaching is to remove obviously dead and infarcted bowel, leave suspicious or questionable bowel, and return in 24–48 hours to let it demarcate; the approach described is a departure from that teaching. Open
Statement Some of these children look worse over the next 24–36 hours, then start to stabilize—they may not get better, but they stop getting worse. Open
Statement Some of these children look worse over the next 24–36 hours, then start to stabilize—they may not get better, but they stop getting worse. Open
Quotation Often, some of these kids do get, they kind of look worse over the next 24, 36 hours, and then they start to, you know, they may not even be getting better, they just stop getting worse. Open
Quotation Often, some of these kids do get, they kind of look worse over the next 24, 36 hours, and then they start to, you know, they may not even be getting better, they just stop getting worse. Open

Intestinal Rehabilitation, Episode 3: Enteral Autonomy, Part 1

Quotation That remaining bowel, that residual intestine goes through this process of adaptation and where the bowel is trying to compensate to reestablish function, to absorb enough nutrients and fluids to maintain survival. Open
Statement In short gut syndrome, residual intestine undergoes adaptation to compensate and reestablish function to absorb enough nutrients and fluids to maintain survival. Open
Quotation That remaining bowel, that residual intestine goes through this process of adaptation and where the bowel is trying to compensate to reestablish function, to absorb enough nutrients and fluids to maintain survival. Open
Statement In short gut syndrome, residual intestine undergoes adaptation to compensate and reestablish function to absorb enough nutrients and fluids to maintain survival. Open
Quotation The process is driven by The presence of intraluminal nutrients and their interaction with both gut secretions, you know, pancreatic biliary secretions, as well as the impact on the relationship with trophic gut peptides. Open
Statement The adaptive process is driven by the presence of intraluminal nutrients and their interaction with gut secretions (pancreatic, biliary) and trophic gut peptides. Open
Statement The adaptive process is driven by the presence of intraluminal nutrients and their interaction with gut secretions (pancreatic, biliary) and trophic gut peptides. Open
Quotation The process is driven by The presence of intraluminal nutrients and their interaction with both gut secretions, you know, pancreatic biliary secretions, as well as the impact on the relationship with trophic gut peptides. Open
Statement Structural changes during adaptation include mucosal hypertrophy (increased villous length), increased blood supply through angiogenesis, bowel dilation, and in younger children, gut lengthening—all increasing surface area for nutrient absorption. Open
Statement Structural changes during adaptation include mucosal hypertrophy (increased villous length), increased blood supply through angiogenesis, bowel dilation, and in younger children, gut lengthening—all increasing surface area for nutrient absorption. Open
Quotation What all of those things have in common is that they increase surface area for absorption absorption of nutrients, right? Open
Quotation What all of those things have in common is that they increase surface area for absorption absorption of nutrients, right? Open
Statement Functional changes during adaptation include slowed motility to allow more contact time and up-regulation of enterocyte transporters to move nutrients across cells more efficiently. Open
Statement Functional changes during adaptation include slowed motility to allow more contact time and up-regulation of enterocyte transporters to move nutrients across cells more efficiently. Open
Quotation So, each, each cell is working harder, if you will, to try to, to, Function better and our job as a team is to try to promote that the best we can. Open
Quotation So, each, each cell is working harder, if you will, to try to, to, Function better and our job as a team is to try to promote that the best we can. Open
Statement Until very recently, there was no standardized definition for enteral autonomy, and most intestinal failure outcomes have poor definitions. Open
Statement Until very recently, there was no standardized definition for enteral autonomy, and most intestinal failure outcomes have poor definitions. Open
Quotation Up until very recently, there actually was no standardized definition for that. Open
Quotation Up until very recently, there actually was no standardized definition for that. Open
Statement Higher proportions of patients are now surviving to have the ability to reach enteral autonomy due to better management of TPN complications such as line infections, liver disease, and vascular thrombosis. Open
Statement Higher proportions of patients are now surviving to have the ability to reach enteral autonomy due to better management of TPN complications such as line infections, liver disease, and vascular thrombosis. Open
Statement In the past, patients were lost to complications such as liver disease before they could reach their adaptive potential; current management is much better at preventing these complications. Open
Statement In the past, patients were lost to complications such as liver disease before they could reach their adaptive potential; current management is much better at preventing these complications. Open
Quotation So the current Aspen guidelines that just came out with standardized definitions, the independence of uh parental support for 12 weeks and with maintenance of adequate growth and hydration during that time period. Open
Statement The current ASPEN guidelines define enteral autonomy as independence from parenteral support for 12 weeks with maintenance of adequate growth and hydration during that time period. Open
Quotation So the current Aspen guidelines that just came out with standardized definitions, the independence of uh parental support for 12 weeks and with maintenance of adequate growth and hydration during that time period. Open
Statement The current ASPEN guidelines define enteral autonomy as independence from parenteral support for 12 weeks with maintenance of adequate growth and hydration during that time period. Open
Statement A patient is not truly off TPN unless they can actually grow off TPN; stopping TPN without achieving growth is a mistake. Open
Quotation Because it's one thing to turn off parenteral support, but, you know, you're not really off TPN or off parental support unless you can actually grow off TPN. Open
Statement A patient is not truly off TPN unless they can actually grow off TPN; stopping TPN without achieving growth is a mistake. Open
Quotation Because it's one thing to turn off parenteral support, but, you know, you're not really off TPN or off parental support unless you can actually grow off TPN. Open
Statement In the 2012 Squires/PIFCO paper, 50% of patients achieved enteral autonomy over 5-6 years, 25% died, and 25% received transplants. Open
Statement In the 2012 Squires/PIFCO paper, 50% of patients achieved enteral autonomy over 5-6 years, 25% died, and 25% received transplants. Open
Quotation Basically, 50% of patients achieved dental autonomy over 56 years, 25% died and 25% got transplanted. Open
Quotation Basically, 50% of patients achieved dental autonomy over 56 years, 25% died and 25% got transplanted. Open
Statement Recent papers from the last 5-6 years show that 60-80% of patients now achieve enteral autonomy, representing improved outcomes compared to historical data. Open
Statement Recent papers from the last 5-6 years show that 60-80% of patients now achieve enteral autonomy, representing improved outcomes compared to historical data. Open
Quotation Has increased between, depending on who you read, anywhere between 60 to 80%. Open
Quotation Has increased between, depending on who you read, anywhere between 60 to 80%. Open
Statement Small bowel length is an independently significant variable predicting adaptive capacity, which is intuitive since the majority of nutrient digestion and fluid absorption occurs in the small bowel. Open
Statement Small bowel length is an independently significant variable predicting adaptive capacity, which is intuitive since the majority of nutrient digestion and fluid absorption occurs in the small bowel. Open
Quotation You know, the ileum has a much greater capacity to adapt than the jejunum does. Open
Statement The ileum has a much greater capacity to adapt than the jejunum; patients with predominant ileal anatomy do better than those with predominant jejunal anatomy. Open
Statement The ileum has a much greater capacity to adapt than the jejunum; patients with predominant ileal anatomy do better than those with predominant jejunal anatomy. Open
Quotation You know, the ileum has a much greater capacity to adapt than the jejunum does. Open
Statement A full-term baby is born with approximately 160 centimeters of small bowel, which grows to about 425 centimeters by age 5 years, with the steepest growth rate between 35 weeks gestation and 6 months postnatal. Open
Statement A full-term baby is born with approximately 160 centimeters of small bowel, which grows to about 425 centimeters by age 5 years, with the steepest growth rate between 35 weeks gestation and 6 months postnatal. Open
Quotation When you're 5 years old, you have about 425 centimeters of small bowel, and the rate of growth on the curve is steepest between 35 weeks gestation to about 6 months postnatal. Open
Quotation When you're 5 years old, you have about 425 centimeters of small bowel, and the rate of growth on the curve is steepest between 35 weeks gestation to about 6 months postnatal. Open
Statement The presence or absence of the ileocecal valve is a predictor of adaptation, though the valve itself may not be the important factor; rather, loss of the valve typically accompanies loss of the terminal ileum, which is the bigger factor affecting adaptive potential. Open
Statement The presence or absence of the ileocecal valve is a predictor of adaptation, though the valve itself may not be the important factor; rather, loss of the valve typically accompanies loss of the terminal ileum, which is the bigger factor affecting adaptive potential. Open
Quotation Most people that lose their ileocecal valve also lose their terminal ileum, and I think that is the bigger factor as far as, uh, adaptive potential. Open
Quotation Most people that lose their ileocecal valve also lose their terminal ileum, and I think that is the bigger factor as far as, uh, adaptive potential. Open
Statement When a patient has the majority of their small bowel, it almost does not matter how much colon they have—probability of enteral autonomy is 85-100%. Open
Quotation So we've presented data that shows that if you've got the majority of your small bowel, then it almost doesn't matter how much colon you have, you have a probability of ventral autonomy somewhere between 85 to 100%. Open
Statement When a patient has the majority of their small bowel, it almost does not matter how much colon they have—probability of enteral autonomy is 85-100%. Open
Quotation So we've presented data that shows that if you've got the majority of your small bowel, then it almost doesn't matter how much colon you have, you have a probability of ventral autonomy somewhere between 85 to 100%. Open
Statement When small bowel remnant is less than 50% of expected length, the colon becomes vitally important, assuming an increasing role in energy absorption from short-chain fatty acids and fluid/salt absorption. Open
Statement When small bowel remnant is less than 50% of expected length, the colon becomes vitally important, assuming an increasing role in energy absorption from short-chain fatty acids and fluid/salt absorption. Open

CAPS - The use of urine sodium to creatinine ratio as a marker of total body sodium in infants with intestinal failure - Sara Choi

Quotation This is often an underappreciated problem. Open
Statement Patients with stomas, even those without intestinal failure, run the risk of sodium depletion, which has a significant impact on their growth. Open
Statement Stomas and high stool losses are a large source of sodium bicarbonate as well as magnesium loss. Open
Statement Sodium status needs to be thought of, especially in the setting of a baby experiencing poor weight gain in the exposure to what is believed to be adequate calories. Open
Statement When sodium is replaced, it helps restore growth, but it doesn't allow catch up growth. Open
Quotation It's important to remember that when we replace sodium, it does help restore growth, but it doesn't allow catch up growth. Open
Statement It's important to track these patients serially so that a trend can be detected and sodium depletion can be avoided in the first place. Open
Statement If it becomes part of practice to order urine electrolytes at the time that someone's doing their TPN blood work, it's easy to calculate the fractional excretion of sodium ratio. Open
Statement Having potassium, chloride, and osmolality added to the urine electrolytes in addition to just the sodium gives a better picture of whether someone is truly sodium depleted. Open
Statement If potassium level is higher than sodium level in urine, that often indicates the aldosterone pathway is turned on. Open
Quotation If we see their potassium level higher than their sodium level, that often will tell you that the aldosterone pathway is turned on. Open

Pediatric Vascular Access in Brief: Preoperative, Operative, and Postoperative Considerations

Statement Traditional locking solutions are predominantly antibiotic locks, but they lead to development of bacterial resistance. Open
Quotation Antimicrobial, it doesn't have any resistance, and it'll kill bacteria that are both planktonic, meaning floating around in the, in the lumen, or sessile, meaning that they're embedded in a biofilm along the wall of the catheter. Open
Statement Ethanol locks are antimicrobial without resistance development and kill both planktonic bacteria (floating in the lumen) and sessile bacteria (embedded in biofilm along the catheter wall). Open
Statement Kite lock is a 4% tetrasodium EDTA solution that is antimicrobial without resistance, and has antifibrinolytic and antithrombotic properties. Open
Statement Kite lock is licensed for pediatric use in Canada, Europe, and Australia, but availability varies by region. Open

Intestinal Rehabilitation, Episode 4: Surgical Management, Part 1

Statement The majority of pediatric short bowel syndrome cases present at birth due to neonatal causes: congenital GI anomalies or acquired conditions like necrotizing enterocolitis. Open
Quotation If you are thinking of reestablishing continuity, I feel very strongly that you have to deal with the size discrepancy. Open
Statement When reestablishing bowel continuity, size discrepancy between bowel ends must be addressed or a functional obstruction will result even if the anastomosis is patent. Open
Statement Size discrepancy can be managed by resection to a more appropriate caliber, tapering enteroplasty, or serial transverse enteroplasty (STEP) depending on available bowel length. Open
Statement Gastroschisis patients tend to not do as well with STEP procedures regardless of whether atresia is present; among STEP procedures performed over the years, gastroschisis cases consistently had worse outcomes. Open
Quotation In general, gastroschisis, they don't tend to do as well regardless. Open
Quotation If, and if I look at the step procedures that I've done over the years, the ones that tend to not do as well or tend to always be the gastroschisis. Open
Quotation I don't usually do that operation anyway unless the bowel is an adequate diameter to make it worthwhile, and in my head over the years, it's always been about 5 centimeters anyway. Open
Statement STEP procedure is not usually performed unless bowel is at least 5 centimeters dilated to make it worthwhile. Open
Statement In scenarios where bowel is not adequately dilated, options include resecting bulbs with tapering enteroplasty, establishing continuity if possible, or leaving bowel disconnected with distal feeding tube access to grow the distal bowel before reconnection. Open
Statement The STEP procedure was originally described by HP Kim and Tom Jackson. Open
Statement For STEP, a transverse supraumbilical abdominal incision is used, all adhesions are lysed, and the entire intestinal tract is laid out from top to bottom to understand anatomy and maximize mobility. Open
Statement The anti-mesenteric surface of the bowel is marked with surgical pen after drying with a lap sponge; maintaining this alignment prevents longitudinal twisting of the bowel during stapling. Open
Statement Bowel length is measured before the procedure using 30 silk dipped in mineral oil; caliber and length of the dilated segment to be stepped are also recorded. Open
Statement An endo-GIA stapler is preferred over an open GIA stapler because the smaller cartridge head is more amenable in smaller patients. Open
Statement Staple lines are applied perpendicular to the mesentery at 90° and 270° (3 o'clock and 9 o'clock positions if mesenteric vessels are at 0°). Open
Statement A vascular (white) cartridge with 2.5 mm staples that crimp to 1 mm is used; this is more appropriate for younger patients' bowel thickness and prevents leaks that occurred with classic blue loads in early experience. Open
Statement Target caliber for STEP is 1.5 cm in babies and 2 to 2.5 cm in older infants or children; making it too narrow risks obstruction, especially in patients with borderline motility. Open
Statement Before firing the stapler, measurements are taken to both the end of the bowel and to the side to ensure proper caliber is maintained throughout the length. Open
Statement A U-stitch with 4-0 PDS is placed in the crotch of each staple line to prevent potential leaks at that point. Open
Statement The procedure is performed with a partner, alternating sides of the bowel, working down the length; 45 mm cartridges are most versatile, but 30 mm and 60 mm cartridges may be needed depending on bowel diameter. Open
Statement At the top and bottom of the STEP segment, an additional oblique staple line is fired to taper the transition and avoid a 'dog ear' blind loop that can dilate over time. Open
Statement The STEP procedure is performed with staple lines perpendicular to the mesentery (90° and 270°), not in the anti-mesenteric to mesenteric orientation (180° and 0°). Open
Statement The duodenum is not stepped; the STEP procedure starts where the bowel begins to dilate, usually distal to the duodenum. Open
Statement Avoiding staple lines in the duodenum allows proximal access if emergency reoperation is needed for a leak. Open
Statement If duodenal narrowing is needed, a stapler is used to partially narrow the lateral side away from the bile duct and ampulla; plication sutures are not used because they usually fail. Open

Intestinal Rehabilitation, Episode 4: Surgical Management, Part 1

Statement The majority of pediatric short bowel syndrome cases present at birth due to neonatal causes: congenital GI anomalies or acquired conditions like necrotizing enterocolitis. Open
Statement When reestablishing bowel continuity, size discrepancy between bowel ends must be addressed or a functional obstruction will result even if the anastomosis is patent. Open
Quotation If you are thinking of reestablishing continuity, I feel very strongly that you have to deal with the size discrepancy. Open
Statement Size discrepancy can be managed by resection to a more appropriate caliber, tapering enteroplasty, or serial transverse enteroplasty (STEP) depending on available bowel length. Open
Statement Gastroschisis patients tend to not do as well with STEP procedures regardless of whether atresia is present; among STEP procedures performed over the years, gastroschisis cases consistently had worse outcomes. Open
Quotation In general, gastroschisis, they don't tend to do as well regardless. Open
Quotation If, and if I look at the step procedures that I've done over the years, the ones that tend to not do as well or tend to always be the gastroschisis. Open
Statement STEP procedure is not usually performed unless bowel is at least 5 centimeters dilated to make it worthwhile. Open
Quotation I don't usually do that operation anyway unless the bowel is an adequate diameter to make it worthwhile, and in my head over the years, it's always been about 5 centimeters anyway. Open
Statement In scenarios where bowel is not adequately dilated, options include resecting bulbs with tapering enteroplasty, establishing continuity if possible, or leaving bowel disconnected with distal feeding tube access to grow the distal bowel before reconnection. Open
Statement The STEP procedure was originally described by HP Kim and Tom Jackson. Open
Statement For STEP, a transverse supraumbilical abdominal incision is used, all adhesions are lysed, and the entire intestinal tract is laid out from top to bottom to understand anatomy and maximize mobility. Open
Statement The anti-mesenteric surface of the bowel is marked with surgical pen after drying with a lap sponge; maintaining this alignment prevents longitudinal twisting of the bowel during stapling. Open
Statement Bowel length is measured before the procedure using 30 silk dipped in mineral oil; caliber and length of the dilated segment to be stepped are also recorded. Open
Statement An endo-GIA stapler is preferred over an open GIA stapler because the smaller cartridge head is more amenable in smaller patients. Open
Statement Staple lines are applied perpendicular to the mesentery at 90° and 270° (3 o'clock and 9 o'clock positions if mesenteric vessels are at 0°). Open
Statement A vascular (white) cartridge with 2.5 mm staples that crimp to 1 mm is used; this is more appropriate for younger patients' bowel thickness and prevents leaks that occurred with classic blue loads in early experience. Open
Statement Target caliber for STEP is 1.5 cm in babies and 2 to 2.5 cm in older infants or children; making it too narrow risks obstruction, especially in patients with borderline motility. Open
Statement Before firing the stapler, measurements are taken to both the end of the bowel and to the side to ensure proper caliber is maintained throughout the length. Open
Statement A U-stitch with 4-0 PDS is placed in the crotch of each staple line to prevent potential leaks at that point. Open
Statement The procedure is performed with a partner, alternating sides of the bowel, working down the length; 45 mm cartridges are most versatile, but 30 mm and 60 mm cartridges may be needed depending on bowel diameter. Open
Statement At the top and bottom of the STEP segment, an additional oblique staple line is fired to taper the transition and avoid a 'dog ear' blind loop that can dilate over time. Open
Statement The STEP procedure is performed with staple lines perpendicular to the mesentery (90° and 270°), not in the anti-mesenteric to mesenteric orientation (180° and 0°). Open
Statement The duodenum is not stepped; the STEP procedure starts where the bowel begins to dilate, usually distal to the duodenum. Open
Statement Avoiding staple lines in the duodenum allows proximal access if emergency reoperation is needed for a leak. Open
Statement If duodenal narrowing is needed, a stapler is used to partially narrow the lateral side away from the bile duct and ampulla; plication sutures are not used because they usually fail. Open

Intestinal Rehabilitation, Episode 4: Surgical Management, Part 2

Quotation I think we have to remember that the biggest benefit of the step, for instance, is the fact that you're tapering the bowel and reestablishing a more normal caliber to improve motility. Open
Statement The biggest benefit of the STEP procedure is tapering the bowel and reestablishing a more normal caliber to improve motility. Open
Statement It can take up to 6 months before you actually start to see a significant improvement in absorptive capacity after STEP. Open
Quotation It can take up to 6 months before you actually start to see a significant improvement in absorptive capacity. Open
Statement Improvements in absorptive capacity are measured with fecal fat, alpha-1 antitrypsin clearance, xylose absorption, and citrulline levels as they rise over time. Open
Statement The reason absorptive improvement takes time is that inflamed, sick, leaky mucosa in the setting of bacterial overgrowth needs to heal. Open
Statement Bleeding at STEP staple lines is an underreported complication that is really difficult to manage in some cases. Open
Quotation My hypothesis is that this is a microbiome problem. I think you have a pro-inflammatory environment. Open
Statement Staple line bleeding is hypothesized to be a microbiome problem occurring in a pro-inflammatory environment. Open
Statement Staple line bleeding tends to occur in type 2 anatomy, which is small bowel to colonic remnant in the absence of an intact colon ileocecal valve. Open
Quotation It's non-specific inflammation. There's no vasculitis, there's no viral elements, there's no obvious ischemia, and the management can be super difficult. Open
Statement Pathology of staple line ulcers shows non-specific inflammation with no vasculitis, no viral elements, and no obvious ischemia. Open
Statement Most referrals for intestinal rehabilitation have had multiple operations before coming to the specialist center. Open
Statement Optimizing a child's anatomy may not be conducive to just one operation; you have to set yourself up sometimes planning for the next case. Open
Quotation Keep your eye on the horizon, which is what can we do to try to optimize this kid's anatomy. And if you think about that plan, it may not be conducive to just one operation today. Open

Intestinal Rehabilitation, Episode 6: Cholestasis

Statement Cincinnati Children's Hospital institutionally defined cholestasis as conjugated bilirubin greater than 3 mg/dL or 50 micromoles/L sustained for 2 weeks and not associated with a septic event. Open
Quotation Institutionally defined it as a conjugated bilirubin greater than 3 mg per deciliter or 50 micromoles per liter sustained for 2 weeks and not associated with a septic event. Open
Statement A 2021 JPN publication defined cholestasis as conjugated bilirubin around 2 mg/dL or 34 micromoles/L for 2 weeks, not associated with a septic event. Open
Statement In young children, intestinal failure-associated liver disease presents as cholestatic liver disease, whereas in adolescents and adults it tends to be steatosis (fatty deposition). Open
Statement Prematurity is not modifiable by the clinical team, but lack of enteral feeding, sepsis, and TPN components are modifiable risk factors. Open
Quotation Now, prematurity, we as clinicians, as a team, we don't have much control over that. But the other factors are modifiable, and we can have an impact on that. Open
Statement Prevention of cholestasis requires aggressive introduction of enteral feeding to establish enterohepatic circulation and surgical procedures to optimize anatomy for feed delivery. Open
Statement Two strategies for reversing cholestasis are dose restriction and change of lipid composition. Open
Statement Conventional intralipid (soybean-based) produces prostaglandins and eicosanoids that are pro-inflammatory when metabolized. Open
Statement SMOF lipid promotes bile flow, is hepatoprotective, and can be delivered at conventional dose to achieve somatic growth and provide neurologic nutrients while protecting the liver. Open
Statement SMOF lipid does not contain enough arachidonic acid, so dose restriction of SMOF can lead to essential fatty acid deficiency. Open
Statement When SMOF is delivered at conventional dosing, no patients develop essential fatty acid deficiency. Open
Statement Conventional dosing for lipids is settling around 2.5 g/kg/day, but nutrition guidelines for preterm infants and babies state 3-4 g/kg/day. Open
Statement A bilirubin of 2 mg/dL is not advanced liver disease, is not dangerous, and is usually transient; Dr. Wales would not change lipid emulsion at this threshold. Open
Quotation My opinion is that a billy of 2 is not by any stretch advanced liver disease, and it's not dangerous, and it's usually transient. So I personally would not change the lipid emulsion out of Billy to. Open
Statement During secondary surgical or autologous reconstruction procedures, a liver biopsy is commonly taken to provide an up-to-date microscopic snapshot. Open
Statement Liver function and biochemistry are followed routinely as inpatient and at outpatient clinic visits. Open
Statement Elastography (FibroScan) is available for monitoring but is good for mild/no fibrosis or very advanced fibrosis; it is not as sensitive for patients with intermediate fibrosis. Open
Statement Follow-up visit frequency for children on TPN at home ranges from every 1-4 months depending on patient stability; actively adapting patients may be seen more frequently due to rapid changes being made. Open

Intestinal Rehabilitation, Episode 7: Refeeding in a neonatal patient

Statement One goal in intestinal failure management is to establish normal feeding habits that promote gut function and optimize quality of life including social aspects of eating. Open
Quotation Even if a kid has a complicated disease process, one of our goals as a team is to try to establish the most normal feeding habits we can that not only promote gut function and everything else, but also to optimize quality of life and the importance of the social aspects of eating that we all experience with our families and friends. Open
Statement When initiating feeds in patients with high stoma output, losses increase initially, but this must be pushed through in a hospital setting where volume replacement is available. Open
Statement In short bowel syndrome, protein absorption is fairly well preserved, so the benefit of completely broken-down protein formulas (free amino acids or hydrolysates) is primarily from an allergy perspective. Open
Quotation In the world of short bowel syndrome, protein absorption is actually fairly well preserved. So it seems like the benefit is from an allergy perspective. Open
Quotation Over the last several years, a lot of the formulas have moved from predominant long chain fat to increasing in MCT components. But long chain fat is a stronger driver for adaptation, and I personally haven't really bought into that. Open
Statement Many formulas have moved from predominant long-chain fat to increasing MCT components, but long-chain fat is a stronger driver for adaptation. Open
Statement One milliliter of parenteral nutrition is not isocaloric with one milliliter of formula, and advancing beyond 100-120 per kilo creates problems with not only calories and protein but also sodium and calcium due to compositional differences. Open
Quotation You're right, and they're not isocaloric, and so 1 mL of PN is not 1 mL of formula. So if you keep going beyond 100 per kilo or 120 per kilo, you, you have a problem. And kids, not only will they take a calorie protein hit, they'll take, you start to get into problems with sodium and calcium as well, because the composition in the milk is not the same as what's in the parental solution. Open
Statement At some point during feeding advancement, fortification is necessary. Open
Statement Feeding options include bolus (oral or gastric via tube), continuous (post-pyloric tube or surgical J-tube), or a hybrid model with gastric bolus component plus continuous component (gastric or post-pyloric). Open
Quotation Because I like bolus feeds as a default, I'd like, I like to see a patient fail bolus feeds before they end up on continuous feeds as a sole delivery remote. Open
Statement Bolus feeds are preferred as the default approach; patients should fail bolus feeds before being placed on continuous feeds as the sole delivery method. Open
Statement Continuous feeds can be used as a supplemental approach, with bolus feeds during the day and continuous supplementation overnight. Open
Quotation One thing I just want to say about oral feeding is that even in the scenario where it's really non-nutritive. I think it's important for people to sort of look at that oral feeding, not so much as an internal nutrition perspective, but more of a skill development perspective that they never learn how to eat by mouth and suck and swallow and process, then they won't eat solids later when you want them to, and you're stuck basically with tube feeding on an older child. Open
Statement Even when oral feeding is non-nutritive, it is important for skill development; children who never learn to suck, swallow, and process food by mouth will not eat solids later and will remain dependent on tube feeding. Open
Quotation Yeah, and it, it goes to the point that we've made in a previous podcast about having a plan and and resisting the temptation to get it all done in one. Operation. It's all about optimizing the patient for the next step, but you don't have to do it all in one go. Open
Statement The technical benefit of distal feeding is that size discrepancy at anastomosis is significantly improved because the bowel has been used. Open
Statement The hole from a removed G-tube closes very quickly. Open
Statement A G-tube provides versatility for nutrition supplementation, medication delivery, and venting for gassy or bloated children to improve enteral tolerance. Open

Intestinal Rehabilitation Episode 8: Refeeding of an Older Patient

Quotation The benefit in the curse in a patient like that is that they do know how to eat. They've established that that behavior. Open
Statement The benefit and curse in older patients with bowel loss is that they already know how to eat and have established that behavior. Open
Statement Even if you cannot cure the patient and get them off TPN, it is important to make compromises to optimize quality of life. Open
Quotation So even if you can't cure this patient and get them off TPN. It's important to make compromises to optimize quality of life the best you can. Open
Statement The general strategy is to push the macronutrient modules of protein and fat, which are well tolerated. Open
Quotation The general strategy is you want to push the macronutrient modules of protein and fat are well tolerated. Most of these kids don't tolerate simple sugars very well. Open
Statement Most patients with short bowel do not tolerate simple sugars very well. Open
Statement The general concept of pushing solids and minimizing fluid intake helps prevent dumping. Open
Quotation So the general concept of pushing solids. And minimizing fluid intake helps. They don't tend to dump as much smaller meals more frequently of solids separated from their liquids to a certain extent, and minimization of simple sugars. Open
Statement Smaller meals more frequently of solids separated from liquids, with minimization of simple sugars, reduces dumping tendency. Open
Statement Patients who have transitioned off TPN often come back with problems when diet history reveals they have gotten loose with diet choices, particularly increased sugars. Open
Statement Minimizing sugars reduces symptoms related to bacterial overgrowth, making patients less bloated and gassy. Open
Statement In older children with fluid losses (by stoma or other source), if they are on parenteral support, some fluid can be replaced IV. Open
Statement As you try to get patients off IV support, keeping them hydrated by replenishing losses through enteral replacements is important. Open
Quotation For transport requires sodium and glucose, and so your solution that you're replacing with has to contain some glucose and some salt. Open
Statement Transport requires sodium and glucose, so the rehydration solution must contain some glucose and some salt. Open
Statement There are homemade recipes for oral rehydration solutions and over-the-counter preparations available. Open
Statement Gatorade does not always work well for rehydration because it has too much sugar in it. Open
Quotation Some people will resort to things like Gatorade and stuff like that, but that doesn't always work that well. It's actually too much sugar in it. Open
Statement It is all about normal growth parameters and getting patients through puberty. Open
Statement Some kids end up back on parenteral support to get through puberty. Open
Quotation It is true some kids end up back on parental support, you know, to get through puberty. And then when you're not growing anymore as an adult, often that borderline or marginal gut function is enough to sustain them. Open
Statement When patients are not growing anymore as adults, borderline or marginal gut function is often enough to sustain them. Open
Statement Patients need to be monitored for micronutrient deficiencies after getting off TPN, as this is when they often get into trouble. Open
Statement Long-term growth and outcome need to be followed, highlighting the importance of multidisciplinary teams. Open
Statement Weight is tracked as a major metric for growth, but it has to be balanced with height. Open
Quotation What we commonly see in this type of population is. Round babies where their weight for height is elevated Open
Statement What is commonly seen in this population is round babies where their weight for height is elevated. Open
Statement There is increasing data looking at quality of weight: how much is fat weight versus lean body mass. Open

Advancements in Pediatric Intestinal Failure: Innovative Therapies and Improved Outcomes

Statement Liver disease associated with intestinal failure ranges from mild cholestasis to profound steatosis (in older children/adolescents/adults), with progression through inflammation, fibrosis, and ultimately cirrhosis leading to death or transplantation. Open
Quotation They go anywhere from mild cholestasis to more profound steatosis, which we see in older children or adolescents and adults, with ongoing inflammation, fibrosis, and ultimately cirrhosis leading to death or the need for transplantation. Open
Quotation They go anywhere from mild cholestasis to more profound steatosis, which we see in older children or adolescents and adults, with ongoing inflammation, fibrosis, and ultimately cirrhosis leading to death or the need for transplantation. Open
Statement Liver disease associated with intestinal failure ranges from mild cholestasis to profound steatosis (in older children/adolescents/adults), with progression through inflammation, fibrosis, and ultimately cirrhosis leading to death or transplantation. Open
Statement Risk factors for intestinal failure-associated liver disease include prematurity, lack of enteral feeding, inability to feed due to anatomy, recurrent infections/sepsis, and components of TPN. Open
Statement Risk factors for intestinal failure-associated liver disease include prematurity, lack of enteral feeding, inability to feed due to anatomy, recurrent infections/sepsis, and components of TPN. Open
Quotation There's some patient factors related to prematurity, uh, lack of enteral feeding, the inability to feed because of the anatomy or recurrent infections, sepsis, which can affect the liver directly and indirectly. And the components of TPN. Open
Quotation There's some patient factors related to prematurity, uh, lack of enteral feeding, the inability to feed because of the anatomy or recurrent infections, sepsis, which can affect the liver directly and indirectly. And the components of TPN. Open
Quotation The term intestinal rehab program was coined in the year 2000. Open
Quotation The term intestinal rehab program was coined in the year 2000. Open
Statement The term 'intestinal rehabilitation program' was coined in the year 2000. Open
Statement The term 'intestinal rehabilitation program' was coined in the year 2000. Open
Quotation Intestinal rehab teams improve the outcome of children because of integration of care, improved communication, better continuity. Open
Statement Intestinal rehabilitation teams improve outcomes through integration of care, improved communication, and better continuity. Open
Quotation Intestinal rehab teams improve the outcome of children because of integration of care, improved communication, better continuity. Open
Statement Intestinal rehabilitation teams improve outcomes through integration of care, improved communication, and better continuity. Open
Statement Intestinal rehabilitation programs rely heavily on speech, language, and occupational therapy specialists. Open
Statement Intestinal rehabilitation programs rely heavily on speech, language, and occupational therapy specialists. Open
Quotation We have a large reliance on speech, language, and occupational therapy. Open
Quotation We have a large reliance on speech, language, and occupational therapy. Open
Quotation While our patients are on TPN, we're trying to mitigate the progression of liver disease, sepsis, vascular complications. Open
Statement While patients are on TPN, the goal is to mitigate progression of liver disease, sepsis, and vascular complications. Open
Quotation While our patients are on TPN, we're trying to mitigate the progression of liver disease, sepsis, vascular complications. Open
Statement While patients are on TPN, the goal is to mitigate progression of liver disease, sepsis, and vascular complications. Open
Quotation Having a dedicated unit with dedicated staff all contributes to better outcomes. Open
Quotation Having a dedicated unit with dedicated staff all contributes to better outcomes. Open
Quotation What we ask our Families to do at home is significant. These patients go home on TPN. The families run the pump, add vitamins to the TPN, program the pump, record ins and outs, manage the G tube, manage the stoma. Open
Quotation What we ask our Families to do at home is significant. These patients go home on TPN. The families run the pump, add vitamins to the TPN, program the pump, record ins and outs, manage the G tube, manage the stoma. Open
Statement Families of intestinal failure patients manage significant tasks at home including running TPN pumps, adding vitamins, programming pumps, recording ins and outs, managing G-tubes, and managing stomas. Open
Statement Families of intestinal failure patients manage significant tasks at home including running TPN pumps, adding vitamins, programming pumps, recording ins and outs, managing G-tubes, and managing stomas. Open
Statement High-quality families demonstrate evidence of burnout over time despite education and empowerment efforts. Open
Statement High-quality families demonstrate evidence of burnout over time despite education and empowerment efforts. Open
Quotation We educate and empower our parents because we've all seen high-quality families that over time demonstrate evidence of burnout. Open
Quotation We educate and empower our parents because we've all seen high-quality families that over time demonstrate evidence of burnout. Open
Quotation There's literature that shows the improvement of outcomes with programs in general. You see an improved survival related to liver dysfunction, decreased septic episodes, reduction in central line complications, reduced ICU admissions, and the patients that were listed for transplant are coming off the list because of improved status. Mortality has gone down. Open
Quotation There's literature that shows the improvement of outcomes with programs in general. You see an improved survival related to liver dysfunction, decreased septic episodes, reduction in central line complications, reduced ICU admissions, and the patients that were listed for transplant are coming off the list because of improved status. Mortality has gone down. Open
Statement Literature shows intestinal rehabilitation programs improve survival related to liver dysfunction, decrease septic episodes, reduce central line complications, reduce ICU admissions, and enable patients listed for transplant to come off the list due to improved status. Open
Statement Literature shows intestinal rehabilitation programs improve survival related to liver dysfunction, decrease septic episodes, reduce central line complications, reduce ICU admissions, and enable patients listed for transplant to come off the list due to improved status. Open
Statement Mortality in intestinal failure has decreased with intestinal rehabilitation programs. Open
Statement Mortality in intestinal failure has decreased with intestinal rehabilitation programs. Open
Statement Lipids in parenteral nutrition are a source of essential fatty acids, a non-protein energy source important for growth, and fatty acids have a major role in cellular pathways. Open
Statement Lipids in parenteral nutrition are a source of essential fatty acids, a non-protein energy source important for growth, and fatty acids have a major role in cellular pathways. Open
Quotation Lipids are a source of essential fatty acids. They're a non-protein energy source important for growth. Fatty acids have a major role in cellular pathways. Open
Quotation Lipids are a source of essential fatty acids. They're a non-protein energy source important for growth. Fatty acids have a major role in cellular pathways. Open
Quotation Historically, most of the lipid emulsions in North America are soybean-based, so they have a high phytosterol content. A high omega 6 long chain polyunsaturated fatty acid content and low antioxidant content. Open
Quotation Historically, most of the lipid emulsions in North America are soybean-based, so they have a high phytosterol content. A high omega 6 long chain polyunsaturated fatty acid content and low antioxidant content. Open
Statement Historically, most lipid emulsions in North America are soybean-based with high phytosterol content, high omega-6 long-chain polyunsaturated fatty acid content, and low antioxidant content. Open
Statement Historically, most lipid emulsions in North America are soybean-based with high phytosterol content, high omega-6 long-chain polyunsaturated fatty acid content, and low antioxidant content. Open
Quotation It is associated with deterioration of liver dysfunction, i.e., cholestasis. Open
Statement Prolonged soybean-based lipid exposure is associated with deterioration of liver function, specifically cholestasis. Open
Statement Prolonged soybean-based lipid exposure is associated with deterioration of liver function, specifically cholestasis. Open
Quotation It is associated with deterioration of liver dysfunction, i.e., cholestasis. Open
Statement For every day an infant is exposed to soybean lipid dosed at greater than 2.5 g/kg/day, there is a 3% increase in the odds ratio of developing advanced liver disease. Open
Quotation This is a paper that we wrote several years ago that showed that for every day that an infant is exposed to soybean lipid dosed at greater than 2.5 g per kilo per day, which is more conventional dosing. That is associated with a 3% increase in the odds ratio of developing advanced liver disease. Open
Quotation This is a paper that we wrote several years ago that showed that for every day that an infant is exposed to soybean lipid dosed at greater than 2.5 g per kilo per day, which is more conventional dosing. That is associated with a 3% increase in the odds ratio of developing advanced liver disease. Open
Statement For every day an infant is exposed to soybean lipid dosed at greater than 2.5 g/kg/day, there is a 3% increase in the odds ratio of developing advanced liver disease. Open
Quotation The first generation were basically the soybean-based lipids, rich in omega 6UUFA. That would be intralipids, and that's been around for 40 years and it's saved a lot of lives. Open
Statement First-generation lipid emulsions were soybean-based, rich in omega-6 PUFA (such as Intralipid), and have been in use for 40 years. Open
Statement First-generation lipid emulsions were soybean-based, rich in omega-6 PUFA (such as Intralipid), and have been in use for 40 years. Open
Quotation The first generation were basically the soybean-based lipids, rich in omega 6UUFA. That would be intralipids, and that's been around for 40 years and it's saved a lot of lives. Open
Quotation The 3rd generation lipids attempt to not only reduce the omega 6 component, but to change the omega 6 to the omega 3 ratio with the addition of omega 3 lipids. Open
Statement Third-generation lipid emulsions reduce omega-6 component and change the omega-6 to omega-3 ratio by adding omega-3 lipids. Open
Statement Third-generation lipid emulsions reduce omega-6 component and change the omega-6 to omega-3 ratio by adding omega-3 lipids. Open
Quotation The 3rd generation lipids attempt to not only reduce the omega 6 component, but to change the omega 6 to the omega 3 ratio with the addition of omega 3 lipids. Open
Quotation Omega 6 lipids are metabolized to arachidonic acid. Which produce leukotrienes and prostaglandins of the more pro-inflammatory profile. The omega 3 lipids are metabolized through EPA and DHA and lead to the production of cytokines that have a less inflammatory profile. Open
Statement Omega-6 lipids are metabolized to arachidonic acid, which produces leukotrienes and prostaglandins with a more pro-inflammatory profile. Open
Statement Omega-6 lipids are metabolized to arachidonic acid, which produces leukotrienes and prostaglandins with a more pro-inflammatory profile. Open
Quotation Omega 6 lipids are metabolized to arachidonic acid. Which produce leukotrienes and prostaglandins of the more pro-inflammatory profile. The omega 3 lipids are metabolized through EPA and DHA and lead to the production of cytokines that have a less inflammatory profile. Open
Statement Omega-3 lipids are metabolized through EPA and DHA, leading to production of cytokines with a less inflammatory profile. Open
Statement Omega-3 lipids are metabolized through EPA and DHA, leading to production of cytokines with a less inflammatory profile. Open
Statement Omega-3 lipids improve bile flow by decreasing lithogenicity of bile, decreasing steatosis, stimulating improved beta-oxidation and clearance, lowering oxidative stress, and supporting immune function. Open
Quotation Omega 3 lipids improve bile flow because they decrease lithogenicity of the bile. They decrease steatosis. They stimulate improved beta oxidation and clearance, and they lower oxidative stress and support immune function. Open
Statement Omega-3 lipids improve bile flow by decreasing lithogenicity of bile, decreasing steatosis, stimulating improved beta-oxidation and clearance, lowering oxidative stress, and supporting immune function. Open
Quotation Omega 3 lipids improve bile flow because they decrease lithogenicity of the bile. They decrease steatosis. They stimulate improved beta oxidation and clearance, and they lower oxidative stress and support immune function. Open
Quotation One is lipid minimization, and then the second strategy is a change in composition. Open
Statement The two main strategies for improving lipids in parenteral nutrition are lipid minimization and change in composition. Open
Statement The two main strategies for improving lipids in parenteral nutrition are lipid minimization and change in composition. Open
Quotation One is lipid minimization, and then the second strategy is a change in composition. Open
Statement Literature dating back decades shows that restricting exposure to soybean lipid improves cholestasis, particularly when dosed at 1 g/kg/day or less. Open
Statement Literature dating back decades shows that restricting exposure to soybean lipid improves cholestasis, particularly when dosed at 1 g/kg/day or less. Open
Quotation There's a lot of literature dating back even decades that show that if you restrict exposure to soybean lipid, you improve cholestasis. When you dose the lipid at 1 g per kilo per day or less. Open
Quotation There's a lot of literature dating back even decades that show that if you restrict exposure to soybean lipid, you improve cholestasis. When you dose the lipid at 1 g per kilo per day or less. Open
Quotation The problem with this in preterm babies is that fat is important for growth, especially for neurocognitive development. So there is the risk of essential fatty acid deficiency if we restrict lipid dosing too much. Open
Statement In preterm babies, fat is important for growth, especially for neurocognitive development, creating a risk of essential fatty acid deficiency if lipid dosing is restricted too much. Open
Statement In preterm babies, fat is important for growth, especially for neurocognitive development, creating a risk of essential fatty acid deficiency if lipid dosing is restricted too much. Open
Quotation The problem with this in preterm babies is that fat is important for growth, especially for neurocognitive development. So there is the risk of essential fatty acid deficiency if we restrict lipid dosing too much. Open
Quotation DHA and arachidonic acid are important for retinal and brain development. None of the lipid emulsions today were designed for premature babies. They were all designed for adults in a critical care setting. Open
Statement DHA and arachidonic acid are important for retinal and brain development. Open
Quotation DHA and arachidonic acid are important for retinal and brain development. None of the lipid emulsions today were designed for premature babies. They were all designed for adults in a critical care setting. Open
Statement DHA and arachidonic acid are important for retinal and brain development. Open
Statement None of the current lipid emulsions were designed for premature babies; they were all designed for adults in critical care settings. Open
Statement None of the current lipid emulsions were designed for premature babies; they were all designed for adults in critical care settings. Open
Quotation We're currently in work with industry to create a lipid emulsion that's more specifically tailored to preterm babies that has a higher content of arachidonic acid. Open
Statement Work is currently underway with industry to create a lipid emulsion specifically tailored to preterm babies with higher arachidonic acid content. Open
Statement Work is currently underway with industry to create a lipid emulsion specifically tailored to preterm babies with higher arachidonic acid content. Open
Quotation We're currently in work with industry to create a lipid emulsion that's more specifically tailored to preterm babies that has a higher content of arachidonic acid. Open
Quotation So our patients with central lines, it's literally their lifeline. And without venous access, we can't support our families. They are at risk for complications, infections, blockages, thrombosis, and breakage. Open
Statement Central lines are the lifeline for intestinal failure patients, and without venous access, families cannot be supported. Open
Statement Central lines are the lifeline for intestinal failure patients, and without venous access, families cannot be supported. Open
Quotation So our patients with central lines, it's literally their lifeline. And without venous access, we can't support our families. They are at risk for complications, infections, blockages, thrombosis, and breakage. Open
Statement Central lines are at risk for complications including infections, blockages, thrombosis, and breakage. Open
Statement Central lines are at risk for complications including infections, blockages, thrombosis, and breakage. Open
Quotation In the United States, there's about 240,000 collapses in the United States on an annual basis. The cost of one of these is about $30,000 about $2 billion a year nationwide. Open
Statement In the United States, there are approximately 240,000 central line-associated bloodstream infections (CLABSIs) annually, with each costing about $30,000, totaling approximately $2 billion per year nationwide. Open
Statement In the United States, there are approximately 240,000 central line-associated bloodstream infections (CLABSIs) annually, with each costing about $30,000, totaling approximately $2 billion per year nationwide. Open
Quotation In the United States, there's about 240,000 collapses in the United States on an annual basis. The cost of one of these is about $30,000 about $2 billion a year nationwide. Open
Quotation We landed on 4% tetrosodium EDTA or kite lock as it's known in the trade name, which is an antithrombolytic, anti-fibrinolytic, and antimicrobial. So, it satisfies all three criteria that are important for a good lock solution. Open
Statement The team pivoted to 4% tetrasodium EDTA (Taurolock/Kitelock) when ethanol became too expensive and difficult to obtain. Open
Statement 4% tetrasodium EDTA (Kitelock) is antithrombotic, anti-fibrinolytic, and antimicrobial, satisfying all three criteria important for a good lock solution. Open
Quotation We landed on 4% tetrosodium EDTA or kite lock as it's known in the trade name, which is an antithrombolytic, anti-fibrinolytic, and antimicrobial. So, it satisfies all three criteria that are important for a good lock solution. Open
Statement 4% tetrasodium EDTA (Kitelock) is antithrombotic, anti-fibrinolytic, and antimicrobial, satisfying all three criteria important for a good lock solution. Open
Statement The team pivoted to 4% tetrasodium EDTA (Taurolock/Kitelock) when ethanol became too expensive and difficult to obtain. Open
Quotation It's licensed in Europe, it's licensed in Australia. It's not available in the United States. Open
Statement Kitelock is licensed in Europe and Australia but not available in the United States. Open
Statement Kitelock is licensed in Europe and Australia but not available in the United States. Open
Quotation It's licensed in Europe, it's licensed in Australia. It's not available in the United States. Open
Quotation I'm happy to say that we have finally launched our multi-center randomized trial of kitelock versus heparin, which went live 4 weeks ago, and we're actively recruiting patients. In the next year, we'll have the results of the trial, and we can get the kitelock into the United States. Open
Statement A multi-center randomized trial of Kitelock versus heparin went live 4 weeks ago and is actively recruiting patients, with results expected within the next year. Open
Statement A multi-center randomized trial of Kitelock versus heparin went live 4 weeks ago and is actively recruiting patients, with results expected within the next year. Open
Quotation I'm happy to say that we have finally launched our multi-center randomized trial of kitelock versus heparin, which went live 4 weeks ago, and we're actively recruiting patients. In the next year, we'll have the results of the trial, and we can get the kitelock into the United States. Open
Quotation In short bowel syndrome, we have 3 anatomical subtypes. Type 1 is the high L piginostomy. Type 2, where you've lost your distal small bowel, your ileum, and part of your colon, and there's a small bowel colonic anastomosis. And then type 3 where you've lost the mid-part of your small bowel, but you still have some retained ileum and an intact colon. Open
Quotation In short bowel syndrome, we have 3 anatomical subtypes. Type 1 is the high L piginostomy. Type 2, where you've lost your distal small bowel, your ileum, and part of your colon, and there's a small bowel colonic anastomosis. And then type 3 where you've lost the mid-part of your small bowel, but you still have some retained ileum and an intact colon. Open
Statement In short bowel syndrome, there are three anatomical subtypes: Type 1 (high jejunostomy), Type 2 (loss of distal small bowel/ileum and part of colon with small bowel-colonic anastomosis), and Type 3 (loss of mid-small bowel with retained ileum and intact colon). Open
Statement In short bowel syndrome, there are three anatomical subtypes: Type 1 (high jejunostomy), Type 2 (loss of distal small bowel/ileum and part of colon with small bowel-colonic anastomosis), and Type 3 (loss of mid-small bowel with retained ileum and intact colon). Open
Quotation The most common anatomical subtype in pediatrics is type 2. These patients struggle. Type 1 and type 2 struggle because they lack an ileum. Open
Statement The most common anatomical subtype of short bowel syndrome in pediatrics is Type 2. Open
Statement The most common anatomical subtype of short bowel syndrome in pediatrics is Type 2. Open
Quotation The most common anatomical subtype in pediatrics is type 2. These patients struggle. Type 1 and type 2 struggle because they lack an ileum. Open
Statement Type 1 and Type 2 short bowel syndrome patients struggle because they lack an ileum. Open
Statement Type 1 and Type 2 short bowel syndrome patients struggle because they lack an ileum. Open
Statement Native GLP-2 hormone has a half-life of 7 minutes, making it impractical for clinical use. Open
Statement Native GLP-2 hormone has a half-life of 7 minutes, making it impractical for clinical use. Open
Quotation The problem is that the native hormone has a half-life of 7 minutes. So it's not practical. So it's been altered, one amino acid alteration to an analog called taglutide. That has a half-life of 2 hours and it's given once a day subcutaneous injection. Open
Quotation The problem is that the native hormone has a half-life of 7 minutes. So it's not practical. So it's been altered, one amino acid alteration to an analog called taglutide. That has a half-life of 2 hours and it's given once a day subcutaneous injection. Open
Statement GLP-2 has been altered by one amino acid to create an analog called teduglutide, which has a half-life of 2 hours and is given once daily by subcutaneous injection. Open
Statement GLP-2 has been altered by one amino acid to create an analog called teduglutide, which has a half-life of 2 hours and is given once daily by subcutaneous injection. Open
Quotation What we see in patients that received taglutide, a 40% reduction in TPN fluid and calorie requirements over a six-month period. And 70% of patients that received the analog achieved the study endpoint, which was a 20% reduction in TPN requirements. Open
Statement A multi-center phase 3 study published in 2020 at Cincinnati Children's showed patients receiving teduglutide had a 40% reduction in TPN fluid and calorie requirements over a six-month period. Open
Quotation What we see in patients that received taglutide, a 40% reduction in TPN fluid and calorie requirements over a six-month period. And 70% of patients that received the analog achieved the study endpoint, which was a 20% reduction in TPN requirements. Open
Statement A multi-center phase 3 study published in 2020 at Cincinnati Children's showed patients receiving teduglutide had a 40% reduction in TPN fluid and calorie requirements over a six-month period. Open
Statement 70% of patients who received teduglutide achieved the study endpoint of a 20% reduction in TPN requirements. Open
Statement 70% of patients who received teduglutide achieved the study endpoint of a 20% reduction in TPN requirements. Open
Quotation It's licensed for children that are greater than 1 year of age. Already, it's made a big difference in our practice and even kids with very extreme anatomy have been able to get off TPN as a result of being able to replace this hormone. Open
Statement Teduglutide is licensed for children greater than 1 year of age. Open
Statement Teduglutide is licensed for children greater than 1 year of age. Open
Quotation It's licensed for children that are greater than 1 year of age. Already, it's made a big difference in our practice and even kids with very extreme anatomy have been able to get off TPN as a result of being able to replace this hormone. Open
Statement Teduglutide has made a big difference in practice, with even children with very extreme anatomy able to get off TPN. Open
Statement Teduglutide has made a big difference in practice, with even children with very extreme anatomy able to get off TPN. Open
Statement Another GLP-2 analog, apraglutide, has a longer half-life and can be given once weekly. Open
Quotation Another GLP-2 analog, a proglutide, has a longer half-life and can be given once a week. That's currently being studied. And what we've shown is that it actually works even better than taglutide. You get more bowel lengthening with a proglutide compared to glutide. Open
Quotation Another GLP-2 analog, a proglutide, has a longer half-life and can be given once a week. That's currently being studied. And what we've shown is that it actually works even better than taglutide. You get more bowel lengthening with a proglutide compared to glutide. Open
Statement Another GLP-2 analog, apraglutide, has a longer half-life and can be given once weekly. Open
Statement Apraglutide works even better than teduglutide, producing more bowel lengthening. Open
Statement Apraglutide works even better than teduglutide, producing more bowel lengthening. Open
Statement These interventions (optimized lipids, central line infection prevention, GLP-2 analogs) have led to a decrease in transplantation since 2008. Open
Quotation These interventions have led to a decrease in transplantation since 2008. In large part because of the successes of intestinal rehabilitation, the outcomes of transplant at 5 years are about 65%. Whatever we can do to avoid transplant and lifelong immunosuppression, that is our goal. Open
Quotation These interventions have led to a decrease in transplantation since 2008. In large part because of the successes of intestinal rehabilitation, the outcomes of transplant at 5 years are about 65%. Whatever we can do to avoid transplant and lifelong immunosuppression, that is our goal. Open
Statement These interventions (optimized lipids, central line infection prevention, GLP-2 analogs) have led to a decrease in transplantation since 2008. Open
Statement Outcomes of intestinal transplant at 5 years are approximately 65%. Open
Statement The decrease in transplantation is in large part because of the successes of intestinal rehabilitation. Open
Statement Outcomes of intestinal transplant at 5 years are approximately 65%. Open
Statement The decrease in transplantation is in large part because of the successes of intestinal rehabilitation. Open
Statement The goal is to avoid transplant and lifelong immunosuppression whenever possible. Open
Statement The goal is to avoid transplant and lifelong immunosuppression whenever possible. Open
Quotation We have lots of exciting medical and surgical options available to us, but this should all be done under the umbrella of a multidisciplinary team. Open
Quotation We have lots of exciting medical and surgical options available to us, but this should all be done under the umbrella of a multidisciplinary team. Open
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Intestinal Rehabilitation, Episode 2: Overwhelming intestinal damage, Part 2

Quotation The goalposts have moved over the last two decades. Open
Statement In Toronto, ultra-short gut is classified as less than 20% of expected bowel length for age; in a term baby that corresponds to 20–30 centimeters. Open
Quotation Like in Toronto, we, we classified that as less than 20% of expected for age. Open
Statement Overall survival in the ultra-short gut population in the current era of management is over 90%, actually 90–95%. Open
Statement Ultra-short gut patients who reached enteral autonomy required multiple nutritional supplements but were able to grow within normal parameters. Open
Quotation There should be more optimism in general with this population just because our ability to mitigate those long term, you know, risks. Of death from liver disease or sepsis have been transformed in the current era of management. Open
Statement Long-term risks of death from liver disease or sepsis in ultra-short gut patients have been transformed in the current era of management. Open
Statement Ultra-short gut patients who did better tended to have some remnant ileum and longer colonic remnants. Open
Statement Neonatologists see some of the survivors—ex-premature infants with intestinal failure—return to their follow-up clinics, which informs their perspective. Open
Quotation I don't subscribe to the notion that Every kid needs to die with a laparotomy indecision. Open
Statement Not every child needs to die with a laparotomy decision; the data will tell whether the unoperated septic/inflammatory response leads to negative outcomes. Open
Statement Long-term neurocognitive tracking of this population is part of the program's responsibility and is beneficial to patients. Open
Statement Lipid restriction as a way of controlling liver disease was never a practice subscribed to by the speakers. Open
Quotation You know, that was never really a practice that we subscribe to. Open
Statement The surgeon's intraoperative decisions—what is done and what is not done—have a lifelong impact and are critically important; these decisions can determine whether the child stays on or gets off TPN or whether they survive. Open
Quotation The surgeon's role in the patient with short bowel syndrome is, is obviously significant. Partly for what we do and partly for what we don't do, right? Open
Quotation Those intraoperative decisions have a lifelong impact. Open
Quotation So, those, those decisions that you make at the time that you see an intraabdominal catastrophe can make the difference between whether this kid will get, stay on or get off TPN or whether they survive or don't. Open
Statement Traditional teaching is to remove obviously dead and infarcted bowel, leave suspicious or questionable bowel, and return in 24–48 hours to let it demarcate; the approach described is a departure from that teaching. Open
Statement Some of these children look worse over the next 24–36 hours, then start to stabilize—they may not get better, but they stop getting worse. Open
Quotation Often, some of these kids do get, they kind of look worse over the next 24, 36 hours, and then they start to, you know, they may not even be getting better, they just stop getting worse. Open

Intestinal Rehabilitation Episode 8: Refeeding of an Older Patient

Statement The benefit and curse in older patients with bowel loss is that they already know how to eat and have established that behavior. Open
Quotation The benefit in the curse in a patient like that is that they do know how to eat. They've established that that behavior. Open
Statement Even if you cannot cure the patient and get them off TPN, it is important to make compromises to optimize quality of life. Open
Quotation So even if you can't cure this patient and get them off TPN. It's important to make compromises to optimize quality of life the best you can. Open
Quotation The general strategy is you want to push the macronutrient modules of protein and fat are well tolerated. Most of these kids don't tolerate simple sugars very well. Open
Statement The general strategy is to push the macronutrient modules of protein and fat, which are well tolerated. Open
Statement Most patients with short bowel do not tolerate simple sugars very well. Open
Quotation So the general concept of pushing solids. And minimizing fluid intake helps. They don't tend to dump as much smaller meals more frequently of solids separated from their liquids to a certain extent, and minimization of simple sugars. Open
Statement The general concept of pushing solids and minimizing fluid intake helps prevent dumping. Open
Statement Smaller meals more frequently of solids separated from liquids, with minimization of simple sugars, reduces dumping tendency. Open
Statement Patients who have transitioned off TPN often come back with problems when diet history reveals they have gotten loose with diet choices, particularly increased sugars. Open
Statement Minimizing sugars reduces symptoms related to bacterial overgrowth, making patients less bloated and gassy. Open
Statement In older children with fluid losses (by stoma or other source), if they are on parenteral support, some fluid can be replaced IV. Open
Statement As you try to get patients off IV support, keeping them hydrated by replenishing losses through enteral replacements is important. Open
Statement Transport requires sodium and glucose, so the rehydration solution must contain some glucose and some salt. Open
Quotation For transport requires sodium and glucose, and so your solution that you're replacing with has to contain some glucose and some salt. Open
Statement There are homemade recipes for oral rehydration solutions and over-the-counter preparations available. Open
Statement Gatorade does not always work well for rehydration because it has too much sugar in it. Open
Quotation Some people will resort to things like Gatorade and stuff like that, but that doesn't always work that well. It's actually too much sugar in it. Open
Statement It is all about normal growth parameters and getting patients through puberty. Open
Statement Some kids end up back on parenteral support to get through puberty. Open
Quotation It is true some kids end up back on parental support, you know, to get through puberty. And then when you're not growing anymore as an adult, often that borderline or marginal gut function is enough to sustain them. Open
Statement When patients are not growing anymore as adults, borderline or marginal gut function is often enough to sustain them. Open
Statement Patients need to be monitored for micronutrient deficiencies after getting off TPN, as this is when they often get into trouble. Open
Statement Long-term growth and outcome need to be followed, highlighting the importance of multidisciplinary teams. Open
Statement Weight is tracked as a major metric for growth, but it has to be balanced with height. Open
Statement What is commonly seen in this population is round babies where their weight for height is elevated. Open
Quotation What we commonly see in this type of population is. Round babies where their weight for height is elevated Open
Statement There is increasing data looking at quality of weight: how much is fat weight versus lean body mass. Open
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Intestinal rehabilitation: What is intestinal rehab? - Episode 1

Quotation Well, let's go back one step before that and sort of define what is intestinal failure because that sort of leads into who's going to benefit from an intestinal rehab program. Open
Statement Until recently, there was no standardized definition for intestinal failure. Open
Quotation It it's, it's really a functional problem. Essentially, when someone's gut for whatever the diagnosis, whatever the reason, their gut function is insufficient to absorb enough nutrients, fluids, calories to support survival and in the in the case of children, growth, which is a big distinction between adults and children. Open
Statement Intestinal failure is when gut function is insufficient to absorb enough nutrients, fluids, and calories to support survival and, in children, growth. Open
Statement An intestinal rehabilitation program is a multidisciplinary collaborative patient care paradigm that brings coordinated care for children with intestinal failure through comprehensive management of their specialized nutrition and other associated needs, as defined in recent ASPEN guidelines. Open
Quotation So, um, an intestinal rehabilitation program, as we defined also in these Aspen guidelines that just came out, is a multidisciplinary or interdisciplinary collaborative patient care paradigm that that brings coordinated care for children with intestinal failure through comprehensive management of Their specialized nutrition and other associated needs that they have. Open
Statement The intestinal rehabilitation approach streamlines care and improves communication with families and between care providers. Open
Statement Causes of intestinal failure divide into three categories: short bowel syndrome, motility disorders, and congenital enteropathies. Open
Quotation Patients that are gonna come to intestinal rehab program have intestinal failure, and we can divide the causes of intestinal failure into three categories. Open
Statement Short bowel syndrome is by far the most common category of intestinal failure in pediatric patients. Open
Quotation There's short bowel syndrome, which by far is the most common category in pediatric patients with intestinal failure, and causes of short bowel syndrome are usually related to diagnoses of or, or disorders of the neonate. Open
Statement Causes of short bowel syndrome include congenital anomalies (intestinal atresia, malrotation, volvulus, gastroschisis, long segment Hirschsprung disease) and acquired neonatal diseases (necrotizing enterocolitis). Open
Quotation So if we think of the intestine like a pipe, and that pipe is made of muscle that contracts in a coordinated way to push food and stool from one end to the other, you can have abnormalities of the muscle itself, or of the nerves that control that muscle. And the bowel is unable to push things through in a coordinated way. Open
Statement Motility disorders occur when abnormalities of intestinal muscle or the nerves controlling that muscle prevent coordinated propulsion of food and stool. Open
Statement Congenital enteropathies are conditions where patients have all their bowel but the mucosal lining does not digest or absorb properly. Open
Quotation Enteropathies or the congenital diarrheas, and these are conditions where the patient has all of their bowel, but the mucosa, the inside lining that, that absorbs, uh, digests and absorbs doesn't work. Open
Statement Some patients have elements of two or all three categories of intestinal failure in their presentation. Open
Quotation The reason it's important to at least identify these three categories is that some patients will have elements of 12, or 3 of the categories, the way they present. Open
Statement Most intestinal failure patients are infants or babies, but some older pediatric patients develop intestinal failure from inflammatory bowel disease, Crohn disease complications, trauma, malignancy, or vascular thrombosis. Open
Statement Long-term survivors now develop chronic comorbidities not previously seen to the same extent because patients did not live long enough, including renal dysfunction, metabolic bone disease, neurocognitive issues, and quality of life problems. Open
Quotation But now what we're seeing is we have kids that have developed chronic comorbidities, that comorbidities that never, we didn't really see to the same extent because they didn't live long enough. Open

Intestinal Rehabilitation, Episode 1: What is intestinal rehabilitation?

Quotation Well, let's go back one step before that and sort of define what is intestinal failure because that sort of leads into who's going to benefit from an intestinal rehab program. Open
Statement Until recently, there was no standardized definition for intestinal failure. Open
Quotation It it's, it's really a functional problem. Essentially, when someone's gut for whatever the diagnosis, whatever the reason, their gut function is insufficient to absorb enough nutrients, fluids, calories to support survival and in the in the case of children, growth, which is a big distinction between adults and children. Open
Statement Intestinal failure is a functional problem where gut function is insufficient to absorb enough nutrients, fluids, and calories to support survival and, in children, growth. Open
Statement An intestinal rehabilitation program is a multidisciplinary collaborative patient care paradigm that brings coordinated care for children with intestinal failure through comprehensive management of their specialized nutrition and other associated needs, as defined in recent ASPEN guidelines. Open
Quotation So, um, an intestinal rehabilitation program, as we defined also in these Aspen guidelines that just came out, is a multidisciplinary or interdisciplinary collaborative patient care paradigm that that brings coordinated care for children with intestinal failure through comprehensive management of Their specialized nutrition and other associated needs that they have. Open
Statement Intestinal rehabilitation streamlines care and improves communication between family and care providers through an amalgamation of experts providing holistic, comprehensive, coordinated care. Open
Quotation Patients that are gonna come to intestinal rehab program have intestinal failure, and we can divide the causes of intestinal failure into three categories. Open
Statement Causes of short bowel syndrome are usually related to neonatal disorders including congenital anomalies (intestinal atresia, malrotation, volvulus, gastroschisis, long segment Hirschsprung disease) or acquired diseases (necrotizing enterocolitis). Open
Quotation There's short bowel syndrome, which by far is the most common category in pediatric patients with intestinal failure, and causes of short bowel syndrome are usually related to diagnoses of or, or disorders of the neonate. Open
Statement Short bowel syndrome is by far the most common category of intestinal failure in pediatric patients. Open
Statement Motility disorders occur when abnormalities of the intestinal muscle itself or the nerves controlling that muscle prevent coordinated propulsion of food and stool, making patients dependent on intravenous support. Open
Quotation So if we think of the intestine like a pipe, and that pipe is made of muscle that contracts in a coordinated way to push food and stool from one end to the other, you can have abnormalities of the muscle itself, or of the nerves that control that muscle. And the bowel is unable to push things through in a coordinated way. Open
Quotation And they are therefore dependent on intravenous support. Open
Quotation Enteropathies or the congenital diarrheas, and these are conditions where the patient has all of their bowel, but the mucosa, the inside lining that, that absorbs, uh, digests and absorbs doesn't work. Open
Statement Enteropathies or congenital diarrheas are conditions where the patient has all of their bowel but the mucosal lining that digests and absorbs does not work, leading to hypersecretion and profuse fluid losses. Open
Statement Some patients will have elements of two or all three categories of intestinal failure; for example, gastroschisis can involve short bowel, inflammation affecting absorption, and motility issues. Open
Statement Although most intestinal failure patients are infants or babies, older pediatric patients can develop intestinal failure from inflammatory bowel disease, Crohn's disease complications, trauma, malignancy, or vascular thrombosis leading to gut loss. Open
Statement Improved survival has revealed chronic comorbidities not previously seen to the same extent because patients did not live long enough, including renal dysfunction, metabolic bone disease, neurocognitive issues, and quality of life concerns. Open
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Intestinal Rehabilitation, Episode 2: Overwhelming intestinal damage, Part 2

Quotation The goalposts have moved over the last two decades. Open
Statement In Toronto, ultra-short gut is classified as less than 20% of expected bowel length for age; in a term baby that corresponds to 20–30 centimeters. Open
Quotation Like in Toronto, we, we classified that as less than 20% of expected for age. Open
Statement Overall survival in the ultra-short gut population in the current era of management is over 90%, actually 90–95%. Open
Statement Ultra-short gut patients who reached enteral autonomy required multiple nutritional supplements but were able to grow within normal parameters. Open
Statement Long-term risks of death from liver disease or sepsis in ultra-short gut patients have been transformed in the current era of management. Open
Quotation There should be more optimism in general with this population just because our ability to mitigate those long term, you know, risks. Of death from liver disease or sepsis have been transformed in the current era of management. Open
Statement Ultra-short gut patients who did better tended to have some remnant ileum and longer colonic remnants. Open
Statement Neonatologists see some of the survivors—ex-premature infants with intestinal failure—return to their follow-up clinics, which informs their perspective. Open
Quotation I don't subscribe to the notion that Every kid needs to die with a laparotomy indecision. Open
Statement Not every child needs to die with a laparotomy decision; the data will tell whether the unoperated septic/inflammatory response leads to negative outcomes. Open
Statement Long-term neurocognitive tracking of this population is part of the program's responsibility and is beneficial to patients. Open
Statement Lipid restriction as a way of controlling liver disease was never a practice subscribed to by the speakers. Open
Quotation You know, that was never really a practice that we subscribe to. Open
Quotation The surgeon's role in the patient with short bowel syndrome is, is obviously significant. Partly for what we do and partly for what we don't do, right? Open
Statement The surgeon's intraoperative decisions—what is done and what is not done—have a lifelong impact and are critically important; these decisions can determine whether the child stays on or gets off TPN or whether they survive. Open
Quotation Those intraoperative decisions have a lifelong impact. Open
Quotation So, those, those decisions that you make at the time that you see an intraabdominal catastrophe can make the difference between whether this kid will get, stay on or get off TPN or whether they survive or don't. Open
Statement Traditional teaching is to remove obviously dead and infarcted bowel, leave suspicious or questionable bowel, and return in 24–48 hours to let it demarcate; the approach described is a departure from that teaching. Open
Statement Some of these children look worse over the next 24–36 hours, then start to stabilize—they may not get better, but they stop getting worse. Open
Quotation Often, some of these kids do get, they kind of look worse over the next 24, 36 hours, and then they start to, you know, they may not even be getting better, they just stop getting worse. Open

Intestinal Rehabilitation, Episode 3: Enteral Autonomy, Part 1

Statement In short gut syndrome, residual intestine undergoes adaptation to compensate and reestablish function to absorb enough nutrients and fluids to maintain survival. Open
Quotation That remaining bowel, that residual intestine goes through this process of adaptation and where the bowel is trying to compensate to reestablish function, to absorb enough nutrients and fluids to maintain survival. Open
Quotation The process is driven by The presence of intraluminal nutrients and their interaction with both gut secretions, you know, pancreatic biliary secretions, as well as the impact on the relationship with trophic gut peptides. Open
Statement The adaptive process is driven by the presence of intraluminal nutrients and their interaction with gut secretions (pancreatic, biliary) and trophic gut peptides. Open
Statement Structural changes during adaptation include mucosal hypertrophy (increased villous length), increased blood supply through angiogenesis, bowel dilation, and in younger children, gut lengthening—all increasing surface area for nutrient absorption. Open
Quotation What all of those things have in common is that they increase surface area for absorption absorption of nutrients, right? Open
Statement Functional changes during adaptation include slowed motility to allow more contact time and up-regulation of enterocyte transporters to move nutrients across cells more efficiently. Open
Quotation So, each, each cell is working harder, if you will, to try to, to, Function better and our job as a team is to try to promote that the best we can. Open
Statement Until very recently, there was no standardized definition for enteral autonomy, and most intestinal failure outcomes have poor definitions. Open
Quotation Up until very recently, there actually was no standardized definition for that. Open
Statement Higher proportions of patients are now surviving to have the ability to reach enteral autonomy due to better management of TPN complications such as line infections, liver disease, and vascular thrombosis. Open
Statement In the past, patients were lost to complications such as liver disease before they could reach their adaptive potential; current management is much better at preventing these complications. Open
Quotation So the current Aspen guidelines that just came out with standardized definitions, the independence of uh parental support for 12 weeks and with maintenance of adequate growth and hydration during that time period. Open
Statement The current ASPEN guidelines define enteral autonomy as independence from parenteral support for 12 weeks with maintenance of adequate growth and hydration during that time period. Open
Quotation Because it's one thing to turn off parenteral support, but, you know, you're not really off TPN or off parental support unless you can actually grow off TPN. Open
Statement A patient is not truly off TPN unless they can actually grow off TPN; stopping TPN without achieving growth is a mistake. Open
Statement In the 2012 Squires/PIFCO paper, 50% of patients achieved enteral autonomy over 5-6 years, 25% died, and 25% received transplants. Open
Quotation Basically, 50% of patients achieved dental autonomy over 56 years, 25% died and 25% got transplanted. Open
Statement Recent papers from the last 5-6 years show that 60-80% of patients now achieve enteral autonomy, representing improved outcomes compared to historical data. Open
Quotation Has increased between, depending on who you read, anywhere between 60 to 80%. Open
Statement Small bowel length is an independently significant variable predicting adaptive capacity, which is intuitive since the majority of nutrient digestion and fluid absorption occurs in the small bowel. Open
Statement The ileum has a much greater capacity to adapt than the jejunum; patients with predominant ileal anatomy do better than those with predominant jejunal anatomy. Open
Quotation You know, the ileum has a much greater capacity to adapt than the jejunum does. Open
Statement A full-term baby is born with approximately 160 centimeters of small bowel, which grows to about 425 centimeters by age 5 years, with the steepest growth rate between 35 weeks gestation and 6 months postnatal. Open
Quotation When you're 5 years old, you have about 425 centimeters of small bowel, and the rate of growth on the curve is steepest between 35 weeks gestation to about 6 months postnatal. Open
Statement The presence or absence of the ileocecal valve is a predictor of adaptation, though the valve itself may not be the important factor; rather, loss of the valve typically accompanies loss of the terminal ileum, which is the bigger factor affecting adaptive potential. Open
Quotation Most people that lose their ileocecal valve also lose their terminal ileum, and I think that is the bigger factor as far as, uh, adaptive potential. Open
Quotation So we've presented data that shows that if you've got the majority of your small bowel, then it almost doesn't matter how much colon you have, you have a probability of ventral autonomy somewhere between 85 to 100%. Open
Statement When a patient has the majority of their small bowel, it almost does not matter how much colon they have—probability of enteral autonomy is 85-100%. Open
Statement When small bowel remnant is less than 50% of expected length, the colon becomes vitally important, assuming an increasing role in energy absorption from short-chain fatty acids and fluid/salt absorption. Open
Paul's statements about Short Bowel Syndrome 66 statements

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Intestinal rehabilitation: What is intestinal rehab? - Episode 1

Quotation Well, let's go back one step before that and sort of define what is intestinal failure because that sort of leads into who's going to benefit from an intestinal rehab program. Open
Statement Until recently, there was no standardized definition for intestinal failure. Open
Quotation It it's, it's really a functional problem. Essentially, when someone's gut for whatever the diagnosis, whatever the reason, their gut function is insufficient to absorb enough nutrients, fluids, calories to support survival and in the in the case of children, growth, which is a big distinction between adults and children. Open
Statement Intestinal failure is when gut function is insufficient to absorb enough nutrients, fluids, and calories to support survival and, in children, growth. Open
Quotation So, um, an intestinal rehabilitation program, as we defined also in these Aspen guidelines that just came out, is a multidisciplinary or interdisciplinary collaborative patient care paradigm that that brings coordinated care for children with intestinal failure through comprehensive management of Their specialized nutrition and other associated needs that they have. Open
Statement An intestinal rehabilitation program is a multidisciplinary collaborative patient care paradigm that brings coordinated care for children with intestinal failure through comprehensive management of their specialized nutrition and other associated needs, as defined in recent ASPEN guidelines. Open
Statement The intestinal rehabilitation approach streamlines care and improves communication with families and between care providers. Open
Statement Causes of intestinal failure divide into three categories: short bowel syndrome, motility disorders, and congenital enteropathies. Open
Quotation Patients that are gonna come to intestinal rehab program have intestinal failure, and we can divide the causes of intestinal failure into three categories. Open
Statement Short bowel syndrome is by far the most common category of intestinal failure in pediatric patients. Open
Quotation There's short bowel syndrome, which by far is the most common category in pediatric patients with intestinal failure, and causes of short bowel syndrome are usually related to diagnoses of or, or disorders of the neonate. Open
Statement Causes of short bowel syndrome include congenital anomalies (intestinal atresia, malrotation, volvulus, gastroschisis, long segment Hirschsprung disease) and acquired neonatal diseases (necrotizing enterocolitis). Open
Statement Motility disorders occur when abnormalities of intestinal muscle or the nerves controlling that muscle prevent coordinated propulsion of food and stool. Open
Quotation So if we think of the intestine like a pipe, and that pipe is made of muscle that contracts in a coordinated way to push food and stool from one end to the other, you can have abnormalities of the muscle itself, or of the nerves that control that muscle. And the bowel is unable to push things through in a coordinated way. Open
Quotation Enteropathies or the congenital diarrheas, and these are conditions where the patient has all of their bowel, but the mucosa, the inside lining that, that absorbs, uh, digests and absorbs doesn't work. Open
Statement Congenital enteropathies are conditions where patients have all their bowel but the mucosal lining does not digest or absorb properly. Open
Statement Some patients have elements of two or all three categories of intestinal failure in their presentation. Open
Quotation The reason it's important to at least identify these three categories is that some patients will have elements of 12, or 3 of the categories, the way they present. Open
Statement Most intestinal failure patients are infants or babies, but some older pediatric patients develop intestinal failure from inflammatory bowel disease, Crohn disease complications, trauma, malignancy, or vascular thrombosis. Open
Quotation But now what we're seeing is we have kids that have developed chronic comorbidities, that comorbidities that never, we didn't really see to the same extent because they didn't live long enough. Open
Statement Long-term survivors now develop chronic comorbidities not previously seen to the same extent because patients did not live long enough, including renal dysfunction, metabolic bone disease, neurocognitive issues, and quality of life problems. Open

Intestinal Rehabilitation, Episode 1: What is intestinal rehabilitation?

Quotation Well, let's go back one step before that and sort of define what is intestinal failure because that sort of leads into who's going to benefit from an intestinal rehab program. Open
Statement Until recently, there was no standardized definition for intestinal failure. Open
Quotation It it's, it's really a functional problem. Essentially, when someone's gut for whatever the diagnosis, whatever the reason, their gut function is insufficient to absorb enough nutrients, fluids, calories to support survival and in the in the case of children, growth, which is a big distinction between adults and children. Open
Statement Intestinal failure is a functional problem where gut function is insufficient to absorb enough nutrients, fluids, and calories to support survival and, in children, growth. Open
Quotation So, um, an intestinal rehabilitation program, as we defined also in these Aspen guidelines that just came out, is a multidisciplinary or interdisciplinary collaborative patient care paradigm that that brings coordinated care for children with intestinal failure through comprehensive management of Their specialized nutrition and other associated needs that they have. Open
Statement An intestinal rehabilitation program is a multidisciplinary collaborative patient care paradigm that brings coordinated care for children with intestinal failure through comprehensive management of their specialized nutrition and other associated needs, as defined in recent ASPEN guidelines. Open
Statement Intestinal rehabilitation streamlines care and improves communication between family and care providers through an amalgamation of experts providing holistic, comprehensive, coordinated care. Open
Quotation Patients that are gonna come to intestinal rehab program have intestinal failure, and we can divide the causes of intestinal failure into three categories. Open
Quotation There's short bowel syndrome, which by far is the most common category in pediatric patients with intestinal failure, and causes of short bowel syndrome are usually related to diagnoses of or, or disorders of the neonate. Open
Statement Short bowel syndrome is by far the most common category of intestinal failure in pediatric patients. Open
Statement Causes of short bowel syndrome are usually related to neonatal disorders including congenital anomalies (intestinal atresia, malrotation, volvulus, gastroschisis, long segment Hirschsprung disease) or acquired diseases (necrotizing enterocolitis). Open
Quotation So if we think of the intestine like a pipe, and that pipe is made of muscle that contracts in a coordinated way to push food and stool from one end to the other, you can have abnormalities of the muscle itself, or of the nerves that control that muscle. And the bowel is unable to push things through in a coordinated way. Open
Statement Motility disorders occur when abnormalities of the intestinal muscle itself or the nerves controlling that muscle prevent coordinated propulsion of food and stool, making patients dependent on intravenous support. Open
Quotation And they are therefore dependent on intravenous support. Open
Quotation Enteropathies or the congenital diarrheas, and these are conditions where the patient has all of their bowel, but the mucosa, the inside lining that, that absorbs, uh, digests and absorbs doesn't work. Open
Statement Enteropathies or congenital diarrheas are conditions where the patient has all of their bowel but the mucosal lining that digests and absorbs does not work, leading to hypersecretion and profuse fluid losses. Open
Statement Some patients will have elements of two or all three categories of intestinal failure; for example, gastroschisis can involve short bowel, inflammation affecting absorption, and motility issues. Open
Statement Although most intestinal failure patients are infants or babies, older pediatric patients can develop intestinal failure from inflammatory bowel disease, Crohn's disease complications, trauma, malignancy, or vascular thrombosis leading to gut loss. Open
Statement Improved survival has revealed chronic comorbidities not previously seen to the same extent because patients did not live long enough, including renal dysfunction, metabolic bone disease, neurocognitive issues, and quality of life concerns. Open

Intestinal Rehabilitation, Episode 4: Surgical Management, Part 1

Statement The majority of pediatric short bowel syndrome cases present at birth due to neonatal causes: congenital GI anomalies or acquired conditions like necrotizing enterocolitis. Open
Quotation If you are thinking of reestablishing continuity, I feel very strongly that you have to deal with the size discrepancy. Open
Statement When reestablishing bowel continuity, size discrepancy between bowel ends must be addressed or a functional obstruction will result even if the anastomosis is patent. Open
Statement Size discrepancy can be managed by resection to a more appropriate caliber, tapering enteroplasty, or serial transverse enteroplasty (STEP) depending on available bowel length. Open
Quotation In general, gastroschisis, they don't tend to do as well regardless. Open
Statement Gastroschisis patients tend to not do as well with STEP procedures regardless of whether atresia is present; among STEP procedures performed over the years, gastroschisis cases consistently had worse outcomes. Open
Quotation If, and if I look at the step procedures that I've done over the years, the ones that tend to not do as well or tend to always be the gastroschisis. Open
Statement STEP procedure is not usually performed unless bowel is at least 5 centimeters dilated to make it worthwhile. Open
Quotation I don't usually do that operation anyway unless the bowel is an adequate diameter to make it worthwhile, and in my head over the years, it's always been about 5 centimeters anyway. Open
Statement In scenarios where bowel is not adequately dilated, options include resecting bulbs with tapering enteroplasty, establishing continuity if possible, or leaving bowel disconnected with distal feeding tube access to grow the distal bowel before reconnection. Open
Statement The STEP procedure was originally described by HP Kim and Tom Jackson. Open
Statement For STEP, a transverse supraumbilical abdominal incision is used, all adhesions are lysed, and the entire intestinal tract is laid out from top to bottom to understand anatomy and maximize mobility. Open
Statement The anti-mesenteric surface of the bowel is marked with surgical pen after drying with a lap sponge; maintaining this alignment prevents longitudinal twisting of the bowel during stapling. Open
Statement Bowel length is measured before the procedure using 30 silk dipped in mineral oil; caliber and length of the dilated segment to be stepped are also recorded. Open
Statement An endo-GIA stapler is preferred over an open GIA stapler because the smaller cartridge head is more amenable in smaller patients. Open
Statement Staple lines are applied perpendicular to the mesentery at 90° and 270° (3 o'clock and 9 o'clock positions if mesenteric vessels are at 0°). Open
Statement A vascular (white) cartridge with 2.5 mm staples that crimp to 1 mm is used; this is more appropriate for younger patients' bowel thickness and prevents leaks that occurred with classic blue loads in early experience. Open
Statement Target caliber for STEP is 1.5 cm in babies and 2 to 2.5 cm in older infants or children; making it too narrow risks obstruction, especially in patients with borderline motility. Open
Statement Before firing the stapler, measurements are taken to both the end of the bowel and to the side to ensure proper caliber is maintained throughout the length. Open
Statement A U-stitch with 4-0 PDS is placed in the crotch of each staple line to prevent potential leaks at that point. Open
Statement The procedure is performed with a partner, alternating sides of the bowel, working down the length; 45 mm cartridges are most versatile, but 30 mm and 60 mm cartridges may be needed depending on bowel diameter. Open
Statement At the top and bottom of the STEP segment, an additional oblique staple line is fired to taper the transition and avoid a 'dog ear' blind loop that can dilate over time. Open
Statement The STEP procedure is performed with staple lines perpendicular to the mesentery (90° and 270°), not in the anti-mesenteric to mesenteric orientation (180° and 0°). Open
Statement The duodenum is not stepped; the STEP procedure starts where the bowel begins to dilate, usually distal to the duodenum. Open
Statement Avoiding staple lines in the duodenum allows proximal access if emergency reoperation is needed for a leak. Open
Statement If duodenal narrowing is needed, a stapler is used to partially narrow the lateral side away from the bile duct and ampulla; plication sutures are not used because they usually fail. Open
Paul's statements about Thrombosis 5 statements

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Pediatric Vascular Access in Brief: Preoperative, Operative, and Postoperative Considerations

Statement Traditional locking solutions are predominantly antibiotic locks, but they lead to development of bacterial resistance. Open
Statement Ethanol locks are antimicrobial without resistance development and kill both planktonic bacteria (floating in the lumen) and sessile bacteria (embedded in biofilm along the catheter wall). Open
Quotation Antimicrobial, it doesn't have any resistance, and it'll kill bacteria that are both planktonic, meaning floating around in the, in the lumen, or sessile, meaning that they're embedded in a biofilm along the wall of the catheter. Open
Statement Kite lock is a 4% tetrasodium EDTA solution that is antimicrobial without resistance, and has antifibrinolytic and antithrombotic properties. Open
Statement Kite lock is licensed for pediatric use in Canada, Europe, and Australia, but availability varies by region. Open