You refer these patients to liver transplant programs much earlier. And this is why we see so many more patients nowadays than perhaps when Fred and Maria were running the program, uh 15 years ago because teams are are trained to recognize the tumors and send them to a center that offers transplant um um or or can and can do aggressive resection.
You refer these patients to liver transplant programs much earlier. And this is why we see so many more patients nowadays than perhaps when Fred and Maria were running the program, uh 15 years ago because teams are are trained to recognize the tumors and send them to a center that offers transplant um um or or can and can do aggressive resection.
You refer these patients to liver transplant programs much earlier. And this is why we see so many more patients nowadays than perhaps when Fred and Maria were running the program, uh 15 years ago because teams are are trained to recognize the tumors and send them to a center that offers transplant um um or or can and can do aggressive resection.
You refer these patients to liver transplant programs much earlier. And this is why we see so many more patients nowadays than perhaps when Fred and Maria were running the program, uh 15 years ago because teams are are trained to recognize the tumors and send them to a center that offers transplant um um or or can and can do aggressive resection.
The NASPGHAN guidelines, which is what most of the North American pediatric GI people are going to, in theory, follow, actually recommends not to get a HIDA scan because there's always a concern, especially in a 50-day-old, that if you tried to load the patient with phenobarb, you delay treatment by potentially five, six days.
The NASPGHAN guidelines, which is what most of the North American pediatric GI people are going to, in theory, follow, actually recommends not to get a HIDA scan because there's always a concern, especially in a 50-day-old, that if you tried to load the patient with phenobarb, you delay treatment by potentially five, six days.
epidemiologicalIdiopathic neonatal hepatitis has decreased from 70% of cholestatic infants in the 1970s-1980s to approximately 10% currently due to recognition of inborn errors of bile acid metabolism and genotyping of progressive familial intrahepatic cholestasis (PFIC).↗
▶Ep 2 · 1:43
quoteIf you actually looked at studies from the 70s and 1980s, idiopathic neonatal hepatitis would be about 70% of the patients. Now it's probably down to 10%↗
▶Ep 2 · 3:25
clinicalThe jaundice chip is a microarray gene chip that tests for PFIC abnormalities (PFIC 1, 2, 3), alpha-1 antitrypsin, and JAG-1 mutation, but takes 4-6 weeks to return results.↗
▶Ep 2 · 4:51
clinicalThe absence of a gallbladder on ultrasound does not conclusively show biliary atresia, and the presence of a gallbladder does not exclude biliary atresia due to sophisticated ultrasound probe sensitivity.↗
▶Ep 2 · 4:51
quoteThe absence of A gallbladder on ultrasound does not conclusively show biliary atresia. The corollary to that, of course, is the presence of a gallbladder doesn't exclude biliary atresia↗
▶Ep 2 · 5:14
clinicalThe triangular cord sign is a radiographic finding representing the fibrous plate above the portal vein where bile ducts were supposed to be, now showing inflammatory fibrous scar.↗
▶Ep 2 · 5:41
opinionAt Cincinnati Children's Hospital, HIDA scans are not routinely used because the 3-5 day phenobarbital loading period can delay intervention into a time period potentially detrimental to Kasai outcome.↗
▶Ep 2 · 5:41
quoteAt our hospital we actually don't use HIDA scans and in fact if we order a HIDA scan, Dr. Ballesterri, our senior most hepatologists, looks upon it quite poorly.↗
▶Ep 2 · 8:23
clinicalPathognomonic findings on liver biopsy for biliary atresia include significant bile duct proliferation in portal triads and bile plugs within bile ducts, with likelihood of biliary atresia exceeding 90% when these are present.↗
▶Ep 2 · 8:34
quoteIf you see that, the confirmatory, I mean the likelihood of it being biliotresia is well over 90+%. It's very rare that you will have a misdiagnosis at this stage.↗
▶Ep 2 · 8:50
clinicalAt Cincinnati, liver biopsies are reviewed with pathologists before taking patients to the operating room, making intraoperative decision-making easier and sometimes reducing the necessity of intraoperative cholangiogram.↗
▶Ep 2 · 10:28
clinicalAlagille syndrome patients do not have the same bile duct proliferation as biliary atresia patients and will not have the pathognomonic finding of a bile duct plug.↗
▶Ep 2 · 11:27
clinicalAt Cincinnati, the workup for biliary atresia is limited to liver biopsy, labs, and ultrasound before posting the patient for surgery.↗
▶Ep 2 · 12:07
clinicalThe extended hilar dissection technique, going to the first bifurcation of vessels beyond the portal vein, is the proper way Kasai originally described the procedure.↗
▶Ep 2 · 12:26
quoteThis is what the Japanese will call an extended dissection, where they'll go to the first bifurcation of the vessels. This is actually the proper way or the way Kasai. Described it↗
▶Ep 2 · 12:35
opinionJapanese centers uniformly perform extended dissections, which may explain why their Kasai outcomes are better than those in the United States.↗
▶Ep 2 · 13:26
clinicalPolysplenia syndrome patients with biliary atresia are the most difficult cases because they often have pre-duodenal portal veins and frequently have no bile duct remnant, making Kasai nearly impossible in this population.↗
▶Ep 2 · 14:57
clinicalData from England by Mark Davenport showed that cystic variants of biliary atresia have better outcomes if intervention occurs before 30 days of age.↗
▶Ep 2 · 15:14
clinicalCholedochal cyst patients will have pigmented stool, while cystic variant biliary atresia patients will become acholic, providing a simple clinical differentiation.↗
▶Ep 2 · 15:42
clinicalA Roux limb length of 20 cm is inadequate because 5-8 cm of length is almost always lost during hilar dissection at the time of transplant.↗
▶Ep 2 · 15:47
quote20 is not good for a transplant team because almost always you'll lose 5 to 78 centimeters of length on your root at the time of the hilar dissection.↗
▶Ep 2 · 16:09
clinicalThe target Roux limb length should be 30-40 cm to preserve adequate length for transplant teams, as one advantage of transplanting biliary atresia patients is having a pre-existing Roux limb.↗
▶Ep 2 · 16:32
clinicalAnti-refluxing valves for the Roux limb have not been proven effective.↗
▶Ep 2 · 17:11
quoteIt's pretty clear cut that when they localized or focused on the group just 3 centers doing their biliatresis, that the 3 centers that did more than 5 cases a year had. Much better outcomes than those who had less.↗
▶Ep 2 · 17:11
epidemiologicalUK data from approximately 15 years ago showed that centers performing more than 5 biliary atresia cases per year had statistically significantly better outcomes (approximately 20% difference) than those performing fewer cases.↗
▶Ep 2 · 17:38
guidelineThe British health service focused all Kasai procedures at three high-volume centers, which are the only centers that can be reimbursed for the procedure.↗
▶Ep 2 · 18:03
clinicalTwo European studies (King's Group using 2 mg/kg steroids and Klaus Peterson's German group using 10 mg/kg for 5 days) showed no benefit from postoperative corticosteroids in biliary atresia.↗
▶Ep 2 · 18:22
quoteThe Children's Network, which is the national biliotreia Research Consortium, did a multi. Center trial in which they had about 70 patients enrolled in two different arms and they showed clearly that there was no improvement of drainage in terms of corticosteroid use between those who got it and those who didn't.↗
▶Ep 2 · 18:22
clinicalThe Children's Network biliary atresia Research Consortium multi-center trial with approximately 70 patients in two arms showed no improvement in bile drainage with corticosteroid use and a slight increase in adverse events (primarily medical rather than surgical complications).↗
▶Ep 2 · 18:57
opinionDespite negative trial results, there is a subset of biliary atresia patients with an inflammatory gene profile in which steroids might prove beneficial.↗
▶Ep 2 · 19:39
clinicalCincinnati does not use MRCP for biliary atresia diagnosis due to having a high-capacity pathology team.↗
▶Ep 2 · 20:12
quoteIf you cassa and Alis patients, you will shorten their natural. History of that liver staying with them and they will be transplanted sooner. That's actually fairly clear cut.↗
▶Ep 2 · 20:12
clinicalPerforming Kasai on Alagille syndrome patients will shorten the natural history of their native liver and result in earlier transplantation.↗
▶Ep 2 · 20:21
clinicalFor patients with cholestasis where biliary atresia diagnosis is uncertain, workup includes echocardiogram to rule out pulmonic stenosis, spine X-rays to rule out butterfly vertebrae, and eye exams, all to evaluate for Alagille syndrome.↗
Biliary Atresia with Dr. Greg Tiao
▶Ep 12 · 0:40
quoteBiliaryresia is an uncommon disease, results in an obstructive cholangiopathy of the biliary system.↗
▶Ep 12 · 0:40
clinicalBiliary atresia is an uncommon disease that results in an obstructive cholangiopathy of the biliary system↗
quoteBiliaryresia is an uncommon disease, results in an obstructive cholangiopathy of the biliary system.↗
▶Ep 12 · 0:40
clinicalBiliary atresia is an uncommon disease that results in an obstructive cholangiopathy of the biliary system↗
▶Ep 12 · 0:40
epidemiologicalBiliary atresia is the most common cause for end-stage liver disease in children↗
▶Ep 12 · 0:54
clinicalThe etiology of biliary atresia is uncertain↗
▶Ep 12 · 0:54
clinicalThe etiology of biliary atresia is uncertain↗
▶Ep 12 · 0:56
clinicalBiliary atresia causes a biliary epithelial injury process that causes biliary obstruction↗
▶Ep 12 · 0:56
clinicalBiliary atresia causes a biliary epithelial injury process that causes biliary obstruction↗
▶Ep 12 · 1:10
clinicalUntreated biliary atresia causes progressive cholestasis, portal fibrosis, eventually cirrhosis, portal hypertension, and can cause death by the age of 2 years↗
▶Ep 12 · 1:10
quoteThe infant develops progressive cholestasis, portal fibrosis, eventually cirrhosis, and then the manifestations of that, which include portal hypertension, and if untreated, can cause death by the age of 2 years of age.↗
▶Ep 12 · 1:10
quoteThe infant develops progressive cholestasis, portal fibrosis, eventually cirrhosis, and then the manifestations of that, which include portal hypertension, and if untreated, can cause death by the age of 2 years of age.↗
▶Ep 12 · 1:10
clinicalUntreated biliary atresia causes progressive cholestasis, portal fibrosis, eventually cirrhosis, portal hypertension, and can cause death by the age of 2 years↗
▶Ep 12 · 1:31
epidemiologicalIn the Far East, biliary atresia incidence is estimated at 1 in 8000 live births↗
▶Ep 12 · 1:31
quoteIn the Far East, it's been estimated that 1 in 8000 live births will result in a patient with biliatresia.↗
▶Ep 12 · 1:31
epidemiologicalIn the Far East, biliary atresia incidence is estimated at 1 in 8000 live births↗
▶Ep 12 · 1:31
quoteIn the Far East, it's been estimated that 1 in 8000 live births will result in a patient with biliatresia.↗
▶Ep 12 · 1:43
epidemiologicalIn the United States, biliary atresia incidence is 1 in 15,000 live births↗
▶Ep 12 · 1:43
quoteIn the West, or especially in the United States, it's less common where the incidence is 1 in 15,000 live births.↗
▶Ep 12 · 1:43
quoteIn the West, or especially in the United States, it's less common where the incidence is 1 in 15,000 live births.↗
▶Ep 12 · 1:43
epidemiologicalIn the United States, biliary atresia incidence is 1 in 15,000 live births↗
▶Ep 12 · 2:02
quoteThe child or the infant is typically jaundiced. That's the first clinical manifestation.↗
▶Ep 12 · 2:02
clinicalInfants with biliary atresia typically present with jaundice as the first clinical manifestation↗
▶Ep 12 · 2:02
quoteThe child or the infant is typically jaundiced. That's the first clinical manifestation.↗
▶Ep 12 · 2:02
clinicalInfants with biliary atresia typically present with jaundice as the first clinical manifestation↗
▶Ep 12 · 2:08
clinicalPatients with biliary atresia have acholic or very pale gray stools↗
▶Ep 12 · 2:08
clinicalPatients with biliary atresia have acholic or very pale gray stools↗
▶Ep 12 · 2:13
clinicalPatients with biliary atresia have dark urine↗
▶Ep 12 · 2:13
clinicalPatients with biliary atresia have dark urine↗
▶Ep 12 · 2:14
clinicalThe liver in biliary atresia becomes quite firm and is usually palpable in the right costochondral margin↗
▶Ep 12 · 2:14
clinicalThe liver in biliary atresia becomes quite firm and is usually palpable in the right costochondral margin↗
▶Ep 12 · 2:21
clinicalBiliary atresia typically presents between 1 to 2 months of age↗
▶Ep 12 · 2:21
clinicalBiliary atresia typically presents between 1 to 2 months of age↗
▶Ep 12 · 2:26
clinicalScreening can pick up biliary atresia at an earlier stage and has been demonstrated to improve outcomes↗
▶Ep 12 · 2:26
clinicalScreening can pick up biliary atresia at an earlier stage and has been demonstrated to improve outcomes↗
▶Ep 12 · 2:26
quoteAnd that screening has, has been demonstrated to improve the outcome of patients who are detected at an earlier age.↗
▶Ep 12 · 2:26
quoteAnd that screening has, has been demonstrated to improve the outcome of patients who are detected at an earlier age.↗
▶Ep 12 · 2:46
clinicalThe most common reason an infant has jaundice is physiologic jaundice of the newborn↗
▶Ep 12 · 2:46
clinicalThe most common reason an infant has jaundice is physiologic jaundice of the newborn↗
clinicalDirect hyperbilirubinemia indicates a more pathologic process↗
▶Ep 12 · 2:58
quoteIf it's a direct hyperbilirubinemia, then you actually have to be worried that there's a more pathologic process underway.↗
▶Ep 12 · 2:58
quoteIf it's a direct hyperbilirubinemia, then you actually have to be worried that there's a more pathologic process underway.↗
▶Ep 12 · 2:58
clinicalDirect hyperbilirubinemia indicates a more pathologic process↗
▶Ep 12 · 3:18
clinicalAnatomic causes of direct hyperbilirubinemia include biliary atresia, choledochal cysts, and inspissated bile syndrome↗
▶Ep 12 · 3:18
clinicalAnatomic causes of direct hyperbilirubinemia include biliary atresia, choledochal cysts, and inspissated bile syndrome↗
▶Ep 12 · 3:39
clinicalHepatocellular causes of direct hyperbilirubinemia include viral hepatitis, sepsis, PFIC syndromes, alpha-1 antitrypsin deficiency, tyrosinemia, and transport abnormalities↗
▶Ep 12 · 3:39
clinicalHepatocellular causes of direct hyperbilirubinemia include viral hepatitis, sepsis, PFIC syndromes, alpha-1 antitrypsin deficiency, tyrosinemia, and transport abnormalities↗
▶Ep 12 · 4:23
clinicalWorkup for biliary atresia includes liver profile with GGT, alpha-1 antitrypsin genotype, and TORCH workup↗
▶Ep 12 · 4:23
clinicalWorkup for biliary atresia includes liver profile with GGT, alpha-1 antitrypsin genotype, and TORCH workup↗
▶Ep 12 · 5:00
clinicalPatients are screened for cystic fibrosis, hypothyroidism, and galactosemia as part of perinatal screening↗
▶Ep 12 · 5:00
clinicalPatients are screened for cystic fibrosis, hypothyroidism, and galactosemia as part of perinatal screening↗
▶Ep 12 · 5:13
clinicalMatrix metalloproteinase 7 (MMP-7) is a new biochemical marker with very high diagnostic sensitivity and specificity for biliary atresia↗
▶Ep 12 · 5:13
quoteMore recently, from a laboratory standpoint, there's been some New studies showing that the protein MMP 7, matrix metallorotinase 7, is a new biochemical marker that has very high diagnostic sensitivity and specificity for biliary.↗
▶Ep 12 · 5:13
quoteMore recently, from a laboratory standpoint, there's been some New studies showing that the protein MMP 7, matrix metallorotinase 7, is a new biochemical marker that has very high diagnostic sensitivity and specificity for biliary.↗
▶Ep 12 · 5:13
clinicalMatrix metalloproteinase 7 (MMP-7) is a new biochemical marker with very high diagnostic sensitivity and specificity for biliary atresia↗
▶Ep 12 · 5:32
clinicalUltrasound is the first imaging test typically obtained for biliary atresia workup↗
▶Ep 12 · 5:32
clinicalUltrasound is the first imaging test typically obtained for biliary atresia workup↗
▶Ep 12 · 5:42
clinicalHIDA scan is fairly sensitive but has quite low specificity for biliary atresia and can delay workup and diagnosis↗
▶Ep 12 · 5:42
quoteThe HIDA scan was used for many years, but that test is fairly sensitive, but its specificity is quite low.↗
▶Ep 12 · 5:42
quoteThe HIDA scan was used for many years, but that test is fairly sensitive, but its specificity is quite low.↗
▶Ep 12 · 5:42
clinicalHIDA scan is fairly sensitive but has quite low specificity for biliary atresia and can delay workup and diagnosis↗
▶Ep 12 · 5:50
quoteAnd so it's usually not utilized because it can actually delay the workup. And diagnosis of bilioresia.↗
▶Ep 12 · 5:50
quoteAnd so it's usually not utilized because it can actually delay the workup. And diagnosis of bilioresia.↗
▶Ep 12 · 6:09
clinicalLiver biopsy is the gold standard for diagnosing biliary atresia↗
▶Ep 12 · 6:09
clinicalLiver biopsy is the gold standard for diagnosing biliary atresia↗
▶Ep 12 · 6:09
quoteAnd then of course the gold standard for biliaryresia is a liver biopsy.↗
▶Ep 12 · 6:09
quoteAnd then of course the gold standard for biliaryresia is a liver biopsy.↗
▶Ep 12 · 6:18
quoteThe classic findings of biliotresia on that biopsy is expansion of the perioral space with mononuclear cells.↗
▶Ep 12 · 6:18
clinicalBile duct plugs are the pathognomonic finding of biliary atresia on biopsy↗
▶Ep 12 · 6:18
quoteThe classic findings of biliotresia on that biopsy is expansion of the perioral space with mononuclear cells.↗
▶Ep 12 · 6:18
clinicalClassic biopsy findings of biliary atresia include expansion of the periportal space with mononuclear cells, bile duct proliferation, and bile duct plugs within proliferating periportal biliary ducts↗
▶Ep 12 · 6:18
clinicalClassic biopsy findings of biliary atresia include expansion of the periportal space with mononuclear cells, bile duct proliferation, and bile duct plugs within proliferating periportal biliary ducts↗
▶Ep 12 · 6:18
clinicalBile duct plugs are the pathognomonic finding of biliary atresia on biopsy↗
▶Ep 12 · 6:27
quoteYou'll see bile duct proliferation and then the pathomnemonic finding of bilioresia is bile duct plugs within those proliferating perioral biliary ducts.↗
▶Ep 12 · 6:27
quoteYou'll see bile duct proliferation and then the pathomnemonic finding of bilioresia is bile duct plugs within those proliferating perioral biliary ducts.↗
▶Ep 12 · 6:45
clinicalTreatment options for biliary atresia are surgical and include Kasai portoenterostomy or liver transplantation↗
▶Ep 12 · 6:45
clinicalTreatment options for biliary atresia are surgical and include Kasai portoenterostomy or liver transplantation↗
▶Ep 12 · 7:00
clinicalAs long as the patient reaches the OR before 70-75 days, the success rate of Kasai procedure justifies the small morbidity risks↗
▶Ep 12 · 7:00
quoteMost teams will find that as long as they get the patient to the OR before 70, 75 days, the success rate of the procedure justifies the small morbidity risks of the procedure.↗
▶Ep 12 · 7:00
quoteMost teams will find that as long as they get the patient to the OR before 70, 75 days, the success rate of the procedure justifies the small morbidity risks of the procedure.↗
▶Ep 12 · 7:00
clinicalAs long as the patient reaches the OR before 70-75 days, the success rate of Kasai procedure justifies the small morbidity risks↗
▶Ep 12 · 7:12
clinicalIn older patients with manifestations of cirrhosis including ascites and coagulopathy, consideration for primary transplant is warranted↗
▶Ep 12 · 7:12
clinicalIn older patients with manifestations of cirrhosis including ascites and coagulopathy, consideration for primary transplant is warranted↗
▶Ep 12 · 7:34
clinicalBoth Kasai and transplant procedures require an intraoperative cholangiogram to establish anatomy and visualize patency of the common hepatic duct and intrahepatic components↗
▶Ep 12 · 7:34
clinicalBoth Kasai and transplant procedures require an intraoperative cholangiogram to establish anatomy and visualize patency of the common hepatic duct and intrahepatic components↗
▶Ep 12 · 7:55
clinicalIn Kasai procedure, the gallbladder is mobilized to identify the common bile duct and common hepatic duct, with dissection carried laterally to where arteries branch↗
▶Ep 12 · 7:55
clinicalIn Kasai procedure, the gallbladder is mobilized to identify the common bile duct and common hepatic duct, with dissection carried laterally to where arteries branch↗
▶Ep 12 · 8:08
clinicalThe hilar plate in biliary atresia has a triangle-like appearance extending from the common hepatic duct to a broader base↗
▶Ep 12 · 8:08
clinicalThe hilar plate in biliary atresia has a triangle-like appearance extending from the common hepatic duct to a broader base↗
▶Ep 12 · 8:25
clinicalThe hilar plate is divided in one sharp transection right proximal to Glisson's capsule↗
▶Ep 12 · 8:25
clinicalThe hilar plate is divided in one sharp transection right proximal to Glisson's capsule↗
▶Ep 12 · 8:34
clinicalA 30 to 35 centimeter Roux-en-Y limb is created and brought up in a retrocolic fashion for hilar plate reconstruction↗
▶Ep 12 · 8:34
clinicalA 30 to 35 centimeter Roux-en-Y limb is created and brought up in a retrocolic fashion for hilar plate reconstruction↗
▶Ep 12 · 9:04
clinicalThe most worrisome complication of Kasai is vascular injury to the portal vein or hepatic artery↗
▶Ep 12 · 9:04
clinicalThe most worrisome complication of Kasai is vascular injury to the portal vein or hepatic artery↗
▶Ep 12 · 9:11
opinionDuring Kasai, anything that is easily demonstrable should not be divided; if you think it's a vessel, don't divide it because it may be a segmental artery of importance↗
▶Ep 12 · 9:11
quoteWe really try not to divide anything that is easily demonstrable. What I mean by that is if you think it's a vessel, don't divide it because you'd be surprised how oftentimes you think it's nothing and it turns out to be a segmental artery of importance.↗
▶Ep 12 · 9:11
quoteWe really try not to divide anything that is easily demonstrable. What I mean by that is if you think it's a vessel, don't divide it because you'd be surprised how oftentimes you think it's nothing and it turns out to be a segmental artery of importance.↗
▶Ep 12 · 9:11
opinionDuring Kasai, anything that is easily demonstrable should not be divided; if you think it's a vessel, don't divide it because it may be a segmental artery of importance↗
▶Ep 12 · 9:27
clinicalOther complications of Kasai include bowel obstruction and wound issues↗
▶Ep 12 · 9:27
clinicalOther complications of Kasai include bowel obstruction and wound issues↗
▶Ep 12 · 9:34
clinicalCholangitis is a longer-term concern after Kasai, but requires adequate bile flow to occur↗
▶Ep 12 · 9:34
clinicalCholangitis is a longer-term concern after Kasai, but requires adequate bile flow to occur↗
▶Ep 12 · 9:34
quoteOne of the concerns we have longer term is cholangitis. But of course, for cholangitis, you actually have to have adequate bile flow to even begin to have that.↗
▶Ep 12 · 9:34
quoteOne of the concerns we have longer term is cholangitis. But of course, for cholangitis, you actually have to have adequate bile flow to even begin to have that.↗
▶Ep 12 · 9:42
clinicalCholangitis after Kasai is presumed to be an ascending process and can be suppressed with antibiotics↗
▶Ep 12 · 9:42
clinicalCholangitis after Kasai is presumed to be an ascending process and can be suppressed with antibiotics↗
▶Ep 12 · 9:57
clinicalIn the United States, most Kasai patients are discharged home within 5 to 7 days after the procedure↗
▶Ep 12 · 9:57
clinicalIn the United States, most Kasai patients are discharged home within 5 to 7 days after the procedure↗
▶Ep 12 · 10:06
clinicalPost-Kasai patients require multidisciplinary care with both surgeon and gastroenterologist follow-up↗
▶Ep 12 · 10:06
clinicalPost-Kasai patients require multidisciplinary care with both surgeon and gastroenterologist follow-up↗
▶Ep 12 · 10:14
clinicalPost-Kasai patients are on prophylactic antibiotics, Actigall, and require nutritional support including fat-soluble vitamins and elemental diets↗
▶Ep 12 · 10:14
clinicalPost-Kasai patients are on prophylactic antibiotics, Actigall, and require nutritional support including fat-soluble vitamins and elemental diets↗
▶Ep 12 · 10:28
clinicalA successful Kasai is defined as direct bilirubin under 2 at 3 months of age↗
▶Ep 12 · 10:28
quoteThe definition of a successful cassai is a direct bilirubin under 2 at 3 months of age.↗
▶Ep 12 · 10:28
quoteThe definition of a successful cassai is a direct bilirubin under 2 at 3 months of age.↗
▶Ep 12 · 10:28
clinicalA successful Kasai is defined as direct bilirubin under 2 at 3 months of age↗
▶Ep 12 · 10:35
clinicalImmediate post-operative success indicator is a pigmented stool, which indicates the patient is draining↗
▶Ep 12 · 10:35
quoteWhat we're looking for immediately post-op is a pigmented stool. If we see that, we know the patient is draining↗
▶Ep 12 · 10:35
quoteWhat we're looking for immediately post-op is a pigmented stool. If we see that, we know the patient is draining↗
▶Ep 12 · 10:35
clinicalImmediate post-operative success indicator is a pigmented stool, which indicates the patient is draining↗
Biliary Atresia Part I
▶Ep 13 · 6:06
clinicalA high GGT is more consistent with an obstructive process and helps guide toward eliminating PFIC (progressive familial intrahepatic cholestasis) diagnoses.↗
▶Ep 13 · 6:06
clinicalA high GGT is more consistent with an obstructive process and helps guide toward eliminating PFIC (progressive familial intrahepatic cholestasis) diagnoses.↗
▶Ep 13 · 6:37
clinicalAt 50 days of age, physiologic jaundice from the newborn period would have resolved.↗
▶Ep 13 · 6:37
clinicalAt 50 days of age, physiologic jaundice from the newborn period would have resolved.↗
▶Ep 13 · 8:16
quoteWe're fortunate in Cincinnati that we have an excellent ultrasound team and they will look for a triangle chord sign, but we really don't find that to be too consistent.↗
▶Ep 13 · 8:16
quoteWe're fortunate in Cincinnati that we have an excellent ultrasound team and they will look for a triangle chord sign, but we really don't find that to be too consistent.↗
▶Ep 13 · 9:45
clinicalCincinnati Children's Hospital has not used HIDA scans for biliary atresia diagnosis in approximately 25 years.↗
▶Ep 13 · 9:45
clinicalCincinnati Children's Hospital has not used HIDA scans for biliary atresia diagnosis in approximately 25 years.↗
▶Ep 13 · 13:46
guidelineNASPGHAN guidelines recommend not obtaining HIDA scans because phenobarbital loading delays treatment by 5-6 days, pushing closer to the window where Kasai efficacy deteriorates.↗
▶Ep 13 · 13:46
quoteThe NASPGHAN guidelines, which is what most of the North American pediatric GI people are going to, in theory, follow, actually recommends not to get a HIDA scan because there's always a concern, especially in a 50-day-old, that if you tried to load the patient with phenobarb, you delay treatment by potentially five, six days.↗
▶Ep 13 · 13:46
host_summaryNASPGHAN guidelines recommend not obtaining HIDA scans because phenobarbital loading delays treatment by 5-6 days, pushing closer to the window where Kasai efficacy deteriorates.↗
▶Ep 13 · 13:46
quoteThe NASPGHAN guidelines, which is what most of the North American pediatric GI people are going to, in theory, follow, actually recommends not to get a HIDA scan because there's always a concern, especially in a 50-day-old, that if you tried to load the patient with phenobarb, you delay treatment by potentially five, six days.↗
▶Ep 13 · 16:28
clinicalSignificant fibrosis on liver biopsy reflects disease progression and may guide surgical decision-making.↗
▶Ep 13 · 16:28
clinicalSignificant fibrosis on liver biopsy reflects disease progression and may guide surgical decision-making.↗
▶Ep 13 · 20:26
quoteI've seen some of the consequences of that, where you just seem to see a little bit more dense adhesions. And again, depending on the stage of the liver disease patients, those things can become quite vascularized.↗
▶Ep 13 · 20:26
clinicalLiver exteriorization during Kasai portoenterostomy can create denser, more vascularized adhesions that complicate future liver transplantation.↗
▶Ep 13 · 20:26
clinicalLiver exteriorization during Kasai portoenterostomy can create denser, more vascularized adhesions that complicate future liver transplantation.↗
▶Ep 13 · 20:26
quoteI've seen some of the consequences of that, where you just seem to see a little bit more dense adhesions. And again, depending on the stage of the liver disease patients, those things can become quite vascularized.↗
▶Ep 13 · 24:31
quoteThe only chance this kid has to avoid transplant early on is a well-done Kasai. And so, that's where I think you really have to be thinking about the patient's best interest when you consider this.↗
▶Ep 13 · 24:31
host_summaryPeterson ran a prospective randomized trial comparing laparoscopic to open Kasai and stopped the trial early.↗
▶Ep 13 · 24:31
opinionThe only chance a child with biliary atresia has to avoid early transplant is a well-done Kasai portoenterostomy.↗
▶Ep 13 · 24:31
clinicalEven in the best hands, long-term drainage rate after Kasai portoenterostomy is only 70-75%.↗
▶Ep 13 · 24:31
quoteThe problem with Kasai is even open, that because the drainage rate is not so ideal, even in the best hands, it's only 70%, 75% long-term.↗
▶Ep 13 · 24:31
quoteThe only chance this kid has to avoid transplant early on is a well-done Kasai. And so, that's where I think you really have to be thinking about the patient's best interest when you consider this.↗
▶Ep 13 · 24:31
quoteThe problem with Kasai is even open, that because the drainage rate is not so ideal, even in the best hands, it's only 70%, 75% long-term.↗
▶Ep 13 · 24:31
clinicalEven in the best hands, long-term drainage rate after Kasai portoenterostomy is only 70-75%.↗
▶Ep 13 · 24:31
opinionThe only chance a child with biliary atresia has to avoid early transplant is a well-done Kasai portoenterostomy.↗
▶Ep 13 · 24:31
clinicalPeterson ran a prospective randomized trial comparing laparoscopic to open Kasai and stopped the trial early.↗
▶Ep 13 · 25:50
clinicalIn the Children's Network surgical committee, approximately one-third of centers cut into the liver during Kasai, while the rest transect at 1-2 mm of fibrous remnant.↗
▶Ep 13 · 25:50
quoteWe do not dig into the liver. I think Dr. Neo wrote a paper just a few years ago where he described the different depth of the hilar plate transection.↗
▶Ep 13 · 25:50
clinicalDamage to hepatic artery branches during Kasai dissection can exacerbate the underlying liver disease process.↗
▶Ep 13 · 25:50
clinicalThe hilar plate should be transected at the level of Glisson's capsule, leaving it intact, without cutting into the liver parenchyma itself.↗
▶Ep 13 · 25:50
clinicalDr. Neo published a paper describing different depths of hilar plate transection in Kasai portoenterostomy.↗
▶Ep 13 · 25:50
clinicalDamage to hepatic artery branches during Kasai dissection can exacerbate the underlying liver disease process.↗
▶Ep 13 · 25:50
clinicalIn the Children's Network surgical committee, approximately one-third of centers cut into the liver during Kasai, while the rest transect at 1-2 mm of fibrous remnant.↗
▶Ep 13 · 25:50
host_summaryDr. Neo published a paper describing different depths of hilar plate transection in Kasai portoenterostomy.↗
▶Ep 13 · 25:50
clinicalThe hilar plate should be transected at the level of Glisson's capsule, leaving it intact, without cutting into the liver parenchyma itself.↗
▶Ep 13 · 25:50
quoteWe do not dig into the liver. I think Dr. Neo wrote a paper just a few years ago where he described the different depth of the hilar plate transection.↗
▶Ep 13 · 30:10
clinicalFor the portoenterostomy anastomosis, 6-0 Maxon suture is used with all knots tied on the outside.↗
▶Ep 13 · 30:10
opinionThe Roux anastomosis suture choice does not make a difference in outcomes.↗
▶Ep 13 · 30:10
clinicalFor the portoenterostomy anastomosis, 6-0 Maxon suture is used with all knots tied on the outside.↗
▶Ep 13 · 30:10
opinionThe Roux anastomosis suture choice does not make a difference in outcomes.↗
Biliary Atresia Part II
▶Ep 14 · 8:57
quoteI think you've got to be very careful when you make those kind of statements. Because it was a properly designed randomized trial. I mean, Georgians work to do this. It's not a small number. It's 110 patients in each arm.↗
▶Ep 14 · 8:57
quoteI think you've got to be very careful when you make those kind of statements. Because it was a properly designed randomized trial. I mean, Georgians work to do this. It's not a small number. It's 110 patients in each arm.↗
▶Ep 14 · 12:31
clinicalCincinnati molecular profiling identified two biliary atresia phenotypes: inflammatory (potentially steroid-responsive) and fibrotic (less likely to respond)↗
▶Ep 14 · 12:31
clinicalCincinnati molecular profiling identified two biliary atresia phenotypes: inflammatory (potentially steroid-responsive) and fibrotic (less likely to respond)↗
▶Ep 14 · 19:48
host_summarySTART trial antibiotic protocol: treatment dose for 2 weeks, then prophylaxis for 6 months↗
▶Ep 14 · 19:48
clinicalCincinnati antibiotic protocol: cephalosporin until taking PO, then Bactrim or amoxicillin for minimum 3 months post-op↗
▶Ep 14 · 19:48
clinicalCincinnati antibiotic protocol: cephalosporin until taking PO, then Bactrim or amoxicillin for minimum 3 months post-op↗
▶Ep 14 · 19:48
clinicalSTART trial antibiotic protocol: treatment dose for 2 weeks, then prophylaxis for 6 months↗
▶Ep 14 · 24:14
clinicalCincinnati approach: clinical assessment with consideration of biopsy fibrosis; will explore surgically unless significant cirrhosis on pre-op biopsy; some 120-day patients have done well while some 60-day patients had advanced disease↗
▶Ep 14 · 24:14
quoteI've done 120 day olds who actually used liver was actually reasonable and the patients are still training.↗
▶Ep 14 · 24:14
quoteI've done 120 day olds who actually used liver was actually reasonable and the patients are still training.↗
▶Ep 14 · 24:14
clinicalCincinnati approach: clinical assessment with consideration of biopsy fibrosis; will explore surgically unless significant cirrhosis on pre-op biopsy; some 120-day patients have done well while some 60-day patients had advanced disease↗
▶Ep 14 · 27:21
clinicalCholangitis management: early recognition critical, families instructed to seek immediate medical attention for fever, not wait at home↗
▶Ep 14 · 27:21
clinicalCincinnati cholangitis protocol: IV antibiotics, consider short course of steroids if no response after several days↗
▶Ep 14 · 27:21
clinicalCincinnati cholangitis protocol: IV antibiotics, consider short course of steroids if no response after several days↗
▶Ep 14 · 27:21
clinicalCholangitis management: early recognition critical, families instructed to seek immediate medical attention for fever, not wait at home↗
▶Ep 14 · 27:21
quoteIf they have an episode of cholangitis, which is the biggest complication that can affect the long-term efficacy of a KASAI, we would bring them in early for antibiotics.↗
▶Ep 14 · 27:21
quoteIf they have an episode of cholangitis, which is the biggest complication that can affect the long-term efficacy of a KASAI, we would bring them in early for antibiotics.↗
▶Ep 14 · 29:07
clinicalCincinnati performs revision Kasai only in highly selected patients: those who previously cleared jaundice and normalized bilirubin, then became acholic after cholangitis; 25-patient series showed native liver salvage possible, some patients 5-10 years out with native liver↗
▶Ep 14 · 29:07
clinicalCincinnati performs revision Kasai only in highly selected patients: those who previously cleared jaundice and normalized bilirubin, then became acholic after cholangitis; 25-patient series showed native liver salvage possible, some patients 5-10 years out with native liver↗
▶Ep 14 · 31:22
clinicalTransplant referral timing: if bilirubin has not dropped below 2 at 3 months post-Kasai, refer to transplant center for evaluation even if not immediately listing↗
▶Ep 14 · 31:22
clinicalTransplant referral timing: if bilirubin has not dropped below 2 at 3 months post-Kasai, refer to transplant center for evaluation even if not immediately listing↗
▶Ep 14 · 32:54
clinicalCincinnati mouse model identified peptide sequence common to rotavirus, CMV, and EBV that may govern host immune response in biliary atresia↗
▶Ep 14 · 32:54
clinicalCincinnati mouse model identified peptide sequence common to rotavirus, CMV, and EBV that may govern host immune response in biliary atresia↗
quoteWhat we're gonna try to touch on is a concept which is a timely diagnosis of biliatresia is critical to prolong the native liver survival.↗
▶Ep 24 · 0:53
clinicalTimely diagnosis of biliary atresia is critical to prolong native liver survival↗
▶Ep 24 · 0:53
quoteWhat we're gonna try to touch on is a concept which is a timely diagnosis of biliatresia is critical to prolong the native liver survival.↗
▶Ep 24 · 0:53
clinicalTimely diagnosis of biliary atresia is critical to prolong native liver survival↗
▶Ep 24 · 2:47
clinicalCincinnati Children's stopped doing HIDA scans about 25 years ago↗
▶Ep 24 · 2:47
clinicalCincinnati Children's stopped doing HIDA scans about 25 years ago↗
▶Ep 24 · 2:50
clinicalHIDA scan adds about 5 days to the diagnostic workup↗
▶Ep 24 · 2:50
clinicalHIDA scan adds about 5 days to the diagnostic workup↗
▶Ep 24 · 3:17
clinicalDoctor Bezarra screened about 1000 proteins in the Children's Network registry and found 70 or so that were elevated in the biliary atresia population, of which MMP-7 was the most clear↗
▶Ep 24 · 3:17
clinicalDoctor Bezarra screened about 1000 proteins in the Children's Network registry and found 70 or so that were elevated in the biliary atresia population, of which MMP-7 was the most clear↗
▶Ep 24 · 4:22
clinicalMMP-7 is now a validated biomarker for biliary atresia but is not perfect↗
▶Ep 24 · 4:22
clinicalMMP-7 is now a validated biomarker for biliary atresia but is not perfect↗
epidemiologicalIdiopathic neonatal hepatitis has decreased from 70% of cholestatic infants in the 1970s-1980s to approximately 10% currently due to recognition of inborn errors of bile acid metabolism and genotyping of progressive familial intrahepatic cholestasis (PFIC).↗
▶Ep 2 · 1:42
epidemiologicalIdiopathic neonatal hepatitis has decreased from 70% of cholestatic infants in the 1970s-1980s to approximately 10% currently due to recognition of inborn errors of bile acid metabolism and genotyping of progressive familial intrahepatic cholestasis (PFIC).↗
▶Ep 2 · 1:43
quoteIf you actually looked at studies from the 70s and 1980s, idiopathic neonatal hepatitis would be about 70% of the patients. Now it's probably down to 10%↗
▶Ep 2 · 1:43
quoteIf you actually looked at studies from the 70s and 1980s, idiopathic neonatal hepatitis would be about 70% of the patients. Now it's probably down to 10%↗
▶Ep 2 · 3:25
clinicalThe jaundice chip is a microarray gene chip that tests for PFIC abnormalities (PFIC 1, 2, 3), alpha-1 antitrypsin, and JAG-1 mutation, but takes 4-6 weeks to return results.↗
▶Ep 2 · 3:25
clinicalThe jaundice chip is a microarray gene chip that tests for PFIC abnormalities (PFIC 1, 2, 3), alpha-1 antitrypsin, and JAG-1 mutation, but takes 4-6 weeks to return results.↗
▶Ep 2 · 4:51
quoteThe absence of A gallbladder on ultrasound does not conclusively show biliary atresia. The corollary to that, of course, is the presence of a gallbladder doesn't exclude biliary atresia↗
▶Ep 2 · 4:51
clinicalThe absence of a gallbladder on ultrasound does not conclusively show biliary atresia, and the presence of a gallbladder does not exclude biliary atresia due to sophisticated ultrasound probe sensitivity.↗
▶Ep 2 · 4:51
quoteThe absence of A gallbladder on ultrasound does not conclusively show biliary atresia. The corollary to that, of course, is the presence of a gallbladder doesn't exclude biliary atresia↗
▶Ep 2 · 4:51
clinicalThe absence of a gallbladder on ultrasound does not conclusively show biliary atresia, and the presence of a gallbladder does not exclude biliary atresia due to sophisticated ultrasound probe sensitivity.↗
▶Ep 2 · 5:14
clinicalThe triangular cord sign is a radiographic finding representing the fibrous plate above the portal vein where bile ducts were supposed to be, now showing inflammatory fibrous scar.↗
▶Ep 2 · 5:14
clinicalThe triangular cord sign is a radiographic finding representing the fibrous plate above the portal vein where bile ducts were supposed to be, now showing inflammatory fibrous scar.↗
▶Ep 2 · 5:41
opinionAt Cincinnati Children's Hospital, HIDA scans are not routinely used because the 3-5 day phenobarbital loading period can delay intervention into a time period potentially detrimental to Kasai outcome.↗
▶Ep 2 · 5:41
quoteAt our hospital we actually don't use HIDA scans and in fact if we order a HIDA scan, Dr. Ballesterri, our senior most hepatologists, looks upon it quite poorly.↗
▶Ep 2 · 5:41
quoteAt our hospital we actually don't use HIDA scans and in fact if we order a HIDA scan, Dr. Ballesterri, our senior most hepatologists, looks upon it quite poorly.↗
▶Ep 2 · 5:41
opinionAt Cincinnati Children's Hospital, HIDA scans are not routinely used because the 3-5 day phenobarbital loading period can delay intervention into a time period potentially detrimental to Kasai outcome.↗
▶Ep 2 · 8:23
clinicalPathognomonic findings on liver biopsy for biliary atresia include significant bile duct proliferation in portal triads and bile plugs within bile ducts, with likelihood of biliary atresia exceeding 90% when these are present.↗
▶Ep 2 · 8:23
clinicalPathognomonic findings on liver biopsy for biliary atresia include significant bile duct proliferation in portal triads and bile plugs within bile ducts, with likelihood of biliary atresia exceeding 90% when these are present.↗
▶Ep 2 · 8:34
quoteIf you see that, the confirmatory, I mean the likelihood of it being biliotresia is well over 90+%. It's very rare that you will have a misdiagnosis at this stage.↗
▶Ep 2 · 8:34
quoteIf you see that, the confirmatory, I mean the likelihood of it being biliotresia is well over 90+%. It's very rare that you will have a misdiagnosis at this stage.↗
▶Ep 2 · 8:50
clinicalAt Cincinnati, liver biopsies are reviewed with pathologists before taking patients to the operating room, making intraoperative decision-making easier and sometimes reducing the necessity of intraoperative cholangiogram.↗
▶Ep 2 · 8:50
clinicalAt Cincinnati, liver biopsies are reviewed with pathologists before taking patients to the operating room, making intraoperative decision-making easier and sometimes reducing the necessity of intraoperative cholangiogram.↗
▶Ep 2 · 10:28
clinicalAlagille syndrome patients do not have the same bile duct proliferation as biliary atresia patients and will not have the pathognomonic finding of a bile duct plug.↗
▶Ep 2 · 10:28
clinicalAlagille syndrome patients do not have the same bile duct proliferation as biliary atresia patients and will not have the pathognomonic finding of a bile duct plug.↗
▶Ep 2 · 11:27
clinicalAt Cincinnati, the workup for biliary atresia is limited to liver biopsy, labs, and ultrasound before posting the patient for surgery.↗
▶Ep 2 · 11:27
clinicalAt Cincinnati, the workup for biliary atresia is limited to liver biopsy, labs, and ultrasound before posting the patient for surgery.↗
▶Ep 2 · 12:07
clinicalThe extended hilar dissection technique, going to the first bifurcation of vessels beyond the portal vein, is the proper way Kasai originally described the procedure.↗
▶Ep 2 · 12:07
clinicalThe extended hilar dissection technique, going to the first bifurcation of vessels beyond the portal vein, is the proper way Kasai originally described the procedure.↗
▶Ep 2 · 12:26
quoteThis is what the Japanese will call an extended dissection, where they'll go to the first bifurcation of the vessels. This is actually the proper way or the way Kasai. Described it↗
▶Ep 2 · 12:26
quoteThis is what the Japanese will call an extended dissection, where they'll go to the first bifurcation of the vessels. This is actually the proper way or the way Kasai. Described it↗
▶Ep 2 · 12:35
opinionJapanese centers uniformly perform extended dissections, which may explain why their Kasai outcomes are better than those in the United States.↗
▶Ep 2 · 12:35
opinionJapanese centers uniformly perform extended dissections, which may explain why their Kasai outcomes are better than those in the United States.↗
▶Ep 2 · 13:26
clinicalPolysplenia syndrome patients with biliary atresia are the most difficult cases because they often have pre-duodenal portal veins and frequently have no bile duct remnant, making Kasai nearly impossible in this population.↗
▶Ep 2 · 13:26
clinicalPolysplenia syndrome patients with biliary atresia are the most difficult cases because they often have pre-duodenal portal veins and frequently have no bile duct remnant, making Kasai nearly impossible in this population.↗
▶Ep 2 · 14:57
clinicalData from England by Mark Davenport showed that cystic variants of biliary atresia have better outcomes if intervention occurs before 30 days of age.↗
▶Ep 2 · 14:57
host_summaryData from England by Mark Davenport showed that cystic variants of biliary atresia have better outcomes if intervention occurs before 30 days of age.↗
▶Ep 2 · 15:14
clinicalCholedochal cyst patients will have pigmented stool, while cystic variant biliary atresia patients will become acholic, providing a simple clinical differentiation.↗
▶Ep 2 · 15:14
clinicalCholedochal cyst patients will have pigmented stool, while cystic variant biliary atresia patients will become acholic, providing a simple clinical differentiation.↗
▶Ep 2 · 15:42
clinicalA Roux limb length of 20 cm is inadequate because 5-8 cm of length is almost always lost during hilar dissection at the time of transplant.↗
▶Ep 2 · 15:42
clinicalA Roux limb length of 20 cm is inadequate because 5-8 cm of length is almost always lost during hilar dissection at the time of transplant.↗
▶Ep 2 · 15:47
quote20 is not good for a transplant team because almost always you'll lose 5 to 78 centimeters of length on your root at the time of the hilar dissection.↗
▶Ep 2 · 15:47
quote20 is not good for a transplant team because almost always you'll lose 5 to 78 centimeters of length on your root at the time of the hilar dissection.↗
▶Ep 2 · 16:09
clinicalThe target Roux limb length should be 30-40 cm to preserve adequate length for transplant teams, as one advantage of transplanting biliary atresia patients is having a pre-existing Roux limb.↗
▶Ep 2 · 16:09
clinicalThe target Roux limb length should be 30-40 cm to preserve adequate length for transplant teams, as one advantage of transplanting biliary atresia patients is having a pre-existing Roux limb.↗
▶Ep 2 · 16:32
clinicalAnti-refluxing valves for the Roux limb have not been proven effective.↗
▶Ep 2 · 16:32
clinicalAnti-refluxing valves for the Roux limb have not been proven effective.↗
▶Ep 2 · 17:11
epidemiologicalUK data from approximately 15 years ago showed that centers performing more than 5 biliary atresia cases per year had statistically significantly better outcomes (approximately 20% difference) than those performing fewer cases.↗
▶Ep 2 · 17:11
host_summaryUK data from approximately 15 years ago showed that centers performing more than 5 biliary atresia cases per year had statistically significantly better outcomes (approximately 20% difference) than those performing fewer cases.↗
▶Ep 2 · 17:11
quoteIt's pretty clear cut that when they localized or focused on the group just 3 centers doing their biliatresis, that the 3 centers that did more than 5 cases a year had. Much better outcomes than those who had less.↗
▶Ep 2 · 17:11
quoteIt's pretty clear cut that when they localized or focused on the group just 3 centers doing their biliatresis, that the 3 centers that did more than 5 cases a year had. Much better outcomes than those who had less.↗
▶Ep 2 · 17:38
guidelineThe British health service focused all Kasai procedures at three high-volume centers, which are the only centers that can be reimbursed for the procedure.↗
▶Ep 2 · 17:38
guidelineThe British health service focused all Kasai procedures at three high-volume centers, which are the only centers that can be reimbursed for the procedure.↗
▶Ep 2 · 18:03
clinicalTwo European studies (King's Group using 2 mg/kg steroids and Klaus Peterson's German group using 10 mg/kg for 5 days) showed no benefit from postoperative corticosteroids in biliary atresia.↗
▶Ep 2 · 18:03
host_summaryTwo European studies (King's Group using 2 mg/kg steroids and Klaus Peterson's German group using 10 mg/kg for 5 days) showed no benefit from postoperative corticosteroids in biliary atresia.↗
▶Ep 2 · 18:22
clinicalThe Children's Network biliary atresia Research Consortium multi-center trial with approximately 70 patients in two arms showed no improvement in bile drainage with corticosteroid use and a slight increase in adverse events (primarily medical rather than surgical complications).↗
▶Ep 2 · 18:22
quoteThe Children's Network, which is the national biliotreia Research Consortium, did a multi. Center trial in which they had about 70 patients enrolled in two different arms and they showed clearly that there was no improvement of drainage in terms of corticosteroid use between those who got it and those who didn't.↗
▶Ep 2 · 18:22
quoteThe Children's Network, which is the national biliotreia Research Consortium, did a multi. Center trial in which they had about 70 patients enrolled in two different arms and they showed clearly that there was no improvement of drainage in terms of corticosteroid use between those who got it and those who didn't.↗
▶Ep 2 · 18:22
host_summaryThe Children's Network biliary atresia Research Consortium multi-center trial with approximately 70 patients in two arms showed no improvement in bile drainage with corticosteroid use and a slight increase in adverse events (primarily medical rather than surgical complications).↗
▶Ep 2 · 18:57
opinionDespite negative trial results, there is a subset of biliary atresia patients with an inflammatory gene profile in which steroids might prove beneficial.↗
▶Ep 2 · 18:57
opinionDespite negative trial results, there is a subset of biliary atresia patients with an inflammatory gene profile in which steroids might prove beneficial.↗
▶Ep 2 · 19:39
clinicalCincinnati does not use MRCP for biliary atresia diagnosis due to having a high-capacity pathology team.↗
▶Ep 2 · 19:39
clinicalCincinnati does not use MRCP for biliary atresia diagnosis due to having a high-capacity pathology team.↗
▶Ep 2 · 20:12
quoteIf you cassa and Alis patients, you will shorten their natural. History of that liver staying with them and they will be transplanted sooner. That's actually fairly clear cut.↗
▶Ep 2 · 20:12
clinicalPerforming Kasai on Alagille syndrome patients will shorten the natural history of their native liver and result in earlier transplantation.↗
▶Ep 2 · 20:12
clinicalPerforming Kasai on Alagille syndrome patients will shorten the natural history of their native liver and result in earlier transplantation.↗
▶Ep 2 · 20:12
quoteIf you cassa and Alis patients, you will shorten their natural. History of that liver staying with them and they will be transplanted sooner. That's actually fairly clear cut.↗
▶Ep 2 · 20:21
clinicalFor patients with cholestasis where biliary atresia diagnosis is uncertain, workup includes echocardiogram to rule out pulmonic stenosis, spine X-rays to rule out butterfly vertebrae, and eye exams, all to evaluate for Alagille syndrome.↗
▶Ep 2 · 20:21
clinicalFor patients with cholestasis where biliary atresia diagnosis is uncertain, workup includes echocardiogram to rule out pulmonic stenosis, spine X-rays to rule out butterfly vertebrae, and eye exams, all to evaluate for Alagille syndrome.↗
Urologic Fetal Intervention: Cincinnati Fetal Center
▶Ep 3 · 7:38
clinicalWhen a vesicoamniotic shunt is placed, the bladder often collapses and upper tracts decompress significantly, though residual hydroureteronephrosis may persist.↗
▶Ep 3 · 7:38
clinicalWhen a vesicoamniotic shunt is placed, the bladder often collapses and upper tracts decompress significantly, though residual hydroureteronephrosis may persist.↗
▶Ep 3 · 11:35
epidemiologicalFetoscopic cystoscopy altered the diagnosis in 25–33% of fetuses with suspected LUTO, according to a review by Katie Morris.↗
▶Ep 3 · 11:35
host_summaryFetoscopic cystoscopy altered the diagnosis in 25–33% of fetuses with suspected LUTO, according to a review by Katie Morris.↗
▶Ep 3 · 11:58
guidelineFetoscopic cystoscopy must be considered experimental for LUTO until properly evaluated in a rigorous trial.↗
▶Ep 3 · 11:58
guidelineFetoscopic cystoscopy must be considered experimental for LUTO until properly evaluated in a rigorous trial.↗
▶Ep 3 · 12:09
epidemiologicalA French-Brazilian-Houston study of 111 LUTO fetuses found that only cystoscopy (not shunting) may prevent renal function impairment at 6 months of age.↗
▶Ep 3 · 12:09
host_summaryA French-Brazilian-Houston study of 111 LUTO fetuses found that only cystoscopy (not shunting) may prevent renal function impairment at 6 months of age.↗
▶Ep 3 · 12:55
host_summaryIn a Barcelona-Leuven series of 20 fetoscopic valve ablations, bladder size and amniotic fluid normalized in 80% of cases; among livebirths, there was no pulmonary hypoplasia and three-quarters had normal renal function.↗
▶Ep 3 · 12:55
epidemiologicalIn a Barcelona-Leuven series of 20 fetoscopic valve ablations, bladder size and amniotic fluid normalized in 80% of cases; among livebirths, there was no pulmonary hypoplasia and three-quarters had normal renal function.↗
▶Ep 3 · 13:36
clinicalFetoscopic valve ablation carries a 9–10% risk of urethral fistula and a 6% risk of recurrent severe LUTO.↗
▶Ep 3 · 13:36
host_summaryFetoscopic valve ablation carries a 9–10% risk of urethral fistula and a 6% risk of recurrent severe LUTO.↗
▶Ep 3 · 14:06
clinicalVesicoamniotic shunt complications include migration, blockage, and (rarely) iatrogenic gastroschisis when placed at 17 weeks.↗
▶Ep 3 · 14:06
clinicalVesicoamniotic shunt complications include migration, blockage, and (rarely) iatrogenic gastroschisis when placed at 17 weeks.↗
▶Ep 3 · 34:57
epidemiologicalIn Mark Johnson's historical studies, the third vesicocentesis provided fresher urine that correlated strongly with histologic renal damage; the first and second taps were not predictive.↗
▶Ep 3 · 34:57
epidemiologicalIn Mark Johnson's historical studies, the third vesicocentesis provided fresher urine that correlated strongly with histologic renal damage; the first and second taps were not predictive.↗
quoteThe major renal morbidity comes postnatally.↗
▶Ep 3 · 37:04
quoteThe major renal morbidity comes postnatally.↗
▶Ep 3 · 43:12
opinionGreg Ryan argues there is no role for open fetal surgery in LUTO, especially in the worst-prognosis group, given the maternal and fetal risks and the experimental nature of the procedure.↗
▶Ep 3 · 43:12
opinionGreg Ryan argues there is no role for open fetal surgery in LUTO, especially in the worst-prognosis group, given the maternal and fetal risks and the experimental nature of the procedure.↗
▶Ep 3 · 43:18
quoteI would argue that there is absolutely no role for open surgery for a fetus, particularly the ones in the group you've identified, and I think your own results are simply supporting that.↗
▶Ep 3 · 43:18
quoteI would argue that there is absolutely no role for open surgery for a fetus, particularly the ones in the group you've identified, and I think your own results are simply supporting that.↗
▶Ep 3 · 49:58
epidemiologicalIn a long-term follow-up series, one-third of shunted LUTO survivors developed end-stage renal disease requiring transplant after age 5 years, despite having acceptable discharge creatinine.↗
▶Ep 3 · 49:58
epidemiologicalIn a long-term follow-up series, one-third of shunted LUTO survivors developed end-stage renal disease requiring transplant after age 5 years, despite having acceptable discharge creatinine.↗
▶Ep 3 · 51:24
epidemiologicalPosterior urethral valve patients required transplant at median age 10–12 years; prune belly/urethral hypoplasia at ~4.5 years; urethral atresia earlier.↗
▶Ep 3 · 51:24
epidemiologicalPosterior urethral valve patients required transplant at median age 10–12 years; prune belly/urethral hypoplasia at ~4.5 years; urethral atresia earlier.↗
▶Ep 3 · 52:13
clinicalProgressive renal injury in shunted LUTO survivors is driven by repeated urinary tract infections, vesicoureteral reflux, and valve bladder dysfunction.↗
▶Ep 3 · 52:13
clinicalProgressive renal injury in shunted LUTO survivors is driven by repeated urinary tract infections, vesicoureteral reflux, and valve bladder dysfunction.↗
▶Ep 3 · 52:49
quoteThese MFM crazy people are trying to give us survivors of what would have otherwise been a lethal disease.↗
▶Ep 3 · 52:49
quoteThese MFM crazy people are trying to give us survivors of what would have otherwise been a lethal disease.↗
▶Ep 3 · 54:16
quoteWe may be giving you pretty good kids, but we really have to figure out how to protect those kidneys after these kids are born.↗
▶Ep 3 · 54:16
quoteWe may be giving you pretty good kids, but we really have to figure out how to protect those kidneys after these kids are born.↗
▶Ep 3 · 58:46
opinionMark Johnson's closing pearl: patient selection is the most critical factor in fetal intervention for LUTO.↗
▶Ep 3 · 58:46
opinionMark Johnson's closing pearl: patient selection is the most critical factor in fetal intervention for LUTO.↗
opinionGreg Ryan's closing pearl: just because a procedure can be done does not mean it should be done; honest counseling about outcomes is essential.↗
▶Ep 3 · 1:00:04
opinionGreg Ryan's closing pearl: just because a procedure can be done does not mean it should be done; honest counseling about outcomes is essential.↗
▶Ep 3 · 1:00:42
quoteJust because we can do them doesn't always mean we should do them.↗
▶Ep 3 · 1:00:42
quoteJust because we can do them doesn't always mean we should do them.↗
epidemiologicalThere are around 250 new cases of hepatoblastoma per year.↗
▶Ep 4 · 0:15
quoteSo, Hepatoblastoma, there's around 250 new cases a year. Huge changes in terms of the treatment algorithm over the last 20 years.↗
▶Ep 4 · 0:51
quoteHepatoblastoma is the most common malignant liver tumor in children, but it's nowhere near the most common liver tumor or the most common abdominal tumor.↗
▶Ep 4 · 1:41
epidemiologicalHemangioma is the most common liver lesion in children.↗
▶Ep 4 · 1:41
quoteThe most common lesion is hemangioma. Even though hepatoblastoma is the most common malignant liver tumor in children, it's still pretty rare with about 250 cases per year.↗
▶Ep 4 · 1:41
quoteCommon things being common, hemangioma should be pretty high on the list.↗
▶Ep 4 · 3:00
quoteEOVIST is an MRI contrast agent that is taken up by the hepatocytes and then excreted. It can provide the best definition when looking at the liver on imaging.↗
▶Ep 4 · 3:00
clinicalEOVIST is an MRI contrast agent that is taken up by hepatocytes and then excreted, providing the best definition when looking at the liver on imaging and distinguishing biliary anatomy.↗
▶Ep 4 · 4:08
clinicalCouinaud labeled the liver segments in a counterclockwise fashion after the districts of Paris, by injecting portal vessels.↗
▶Ep 4 · 4:08
quoteHe injected them into portal vessels. And so that's really how these are labeled. But he labeled them in this counterclockwise fashion after, I can never pronounce this, but the districts of Paris are done in the same fashion.↗
▶Ep 4 · 4:45
clinicalThe right hepatic vein differentiates the anterior sections (segments 5 and 8) from the posterior sections (segments 6 and 7) of the right liver.↗
▶Ep 4 · 4:45
clinicalThe right portal vein separates superior segments (7 and 8) from inferior segments (5 and 6) in the right liver.↗
▶Ep 4 · 7:15
quotePretext 3 and 4 is the big game changer. This change in practice is what improves survival rates for patients with hepatoblastoma from less than 30% in the 70s and 80s to between 80 and 90% today.↗
▶Ep 4 · 7:15
epidemiologicalThe change in practice for PRETEXT 3 and 4 tumors improved survival rates for patients with hepatoblastoma from less than 30% in the 1970s and 1980s to between 80 and 90% today.↗
▶Ep 4 · 7:31
guidelinePRETEXT 3 tumors should generally be biopsied at diagnosis and then started on neoadjuvant chemotherapy, with readiness to send patients to a transplant center or a center with expertise in advanced liver resections depending on response.↗
▶Ep 4 · 7:31
guidelineFor PRETEXT 3 or 4 liver disease, specific indications for transplant include unresectable disease, unsafe resection, or resection that would leave the patient with an inadequate liver remnant.↗
▶Ep 4 · 8:19
clinicalTeams are now trained to recognize hepatoblastoma tumors and send them to centers that offer transplant or can do aggressive resection, resulting in more patient referrals than 15 years ago.↗
▶Ep 4 · 8:19
quoteYou refer these patients to liver transplant programs much earlier. And this is why we see so many more patients nowadays than perhaps when Fred and Maria were running the program 15 years ago. Because teams are trained to recognize tumors and send them to a center that offers transplant or can do aggressive resection.↗
▶Ep 4 · 9:06
clinicalThe FIT study has been ongoing for three years.↗
▶Ep 4 · 9:06
quoteThe FIT study has been going on now for three years. We've had the privilege of contributing to it in terms of its development.↗
Hepatoblastoma with Dr. Greg Tiao
▶Ep 5 · 0:46
epidemiologicalHepatoblastoma is nowhere near the most common liver tumor or the most common abdominal tumor in children↗
▶Ep 5 · 0:46
quoteHepatoblastoma is the most common malignant liver tumor in children, but it's nowhere near the most common liver tumor or the most common abdominal tumor.↗
▶Ep 5 · 2:20
clinicalHepatoblastoma can present as an asymptomatic abdominal mass, tumor rupture, or respiratory distress due to tumor size↗
▶Ep 5 · 3:13
clinicalMost experts rely on MRI to delineate anatomy and evaluate the extent of liver involvement in hepatoblastoma↗
▶Ep 5 · 3:13
clinicalEovist is an MRI contrast agent taken up by hepatocytes and then excreted, providing the best definition when looking at the liver on imaging↗
▶Ep 5 · 4:25
clinicalAggressive resections include trisectionectomy, mesohepatectomy, resection with additional procedure in remnant liver, and liver transplantation↗
▶Ep 5 · 5:41
clinicalThe right hepatic vein differentiates anterior and posterior sections of the right liver↗
▶Ep 5 · 6:02
clinicalThe right portal vein separates superior and inferior segments of the right liver↗
▶Ep 5 · 6:51
clinicalThe pre-text staging system classifies tumors based on the number of contiguous liver sections free of disease (minus four equals pre-text stage)↗
▶Ep 5 · 6:51
clinicalPre-text 1 has three contiguous sections free of disease, pre-text 2 has two, pre-text 3 has one, and pre-text 4 has no free sections or diffuse tumor↗
▶Ep 5 · 8:15
clinicalPre-text 3 and 4 tumors represent a big game changer in practice that improved survival from less than 30% in the 1970s-80s to 80-90% today↗
▶Ep 5 · 8:15
clinicalPre-text 3 tumors should generally be biopsied at diagnosis, started on neoadjuvant chemotherapy, then referred to transplant center or center with expertise in advanced liver resections↗
▶Ep 5 · 9:35
quoteYou refer these patients to liver transplant programs much earlier. And this is why we see so many more patients nowadays than perhaps when Fred and Maria were running the program, uh 15 years ago because teams are are trained to recognize the tumors and send them to a center that offers transplant um um or or can and can do aggressive resection.↗
▶Ep 5 · 9:35
clinicalIt is important to consult a transplant center early for pre-text 3 and 4 tumors↗
▶Ep 5 · 10:27
clinicalThe PHITT (Pediatric Hepatic International Tumor Trial) is an ongoing multinational trial to identify high and low risk features for each pre-text stage↗
▶Ep 5 · 10:27
clinicalThe PHITT study has been ongoing for three years↗
quoteSo, Hepatoblastoma, there's around 250 new cases a year. Huge changes in terms of the treatment algorithm over the last 20 years.↗
▶Ep 4 · 0:15
epidemiologicalThere are around 250 new cases of hepatoblastoma per year.↗
▶Ep 4 · 0:15
quoteSo, Hepatoblastoma, there's around 250 new cases a year. Huge changes in terms of the treatment algorithm over the last 20 years.↗
▶Ep 4 · 0:15
epidemiologicalThere are around 250 new cases of hepatoblastoma per year.↗
▶Ep 4 · 0:51
quoteHepatoblastoma is the most common malignant liver tumor in children, but it's nowhere near the most common liver tumor or the most common abdominal tumor.↗
▶Ep 4 · 0:51
quoteHepatoblastoma is the most common malignant liver tumor in children, but it's nowhere near the most common liver tumor or the most common abdominal tumor.↗
▶Ep 4 · 1:41
quoteThe most common lesion is hemangioma. Even though hepatoblastoma is the most common malignant liver tumor in children, it's still pretty rare with about 250 cases per year.↗
▶Ep 4 · 1:41
quoteThe most common lesion is hemangioma. Even though hepatoblastoma is the most common malignant liver tumor in children, it's still pretty rare with about 250 cases per year.↗
▶Ep 4 · 1:41
epidemiologicalHemangioma is the most common liver lesion in children.↗
▶Ep 4 · 1:41
quoteCommon things being common, hemangioma should be pretty high on the list.↗
▶Ep 4 · 1:41
epidemiologicalHemangioma is the most common liver lesion in children.↗
▶Ep 4 · 1:41
quoteCommon things being common, hemangioma should be pretty high on the list.↗
▶Ep 4 · 3:00
clinicalEOVIST is an MRI contrast agent that is taken up by hepatocytes and then excreted, providing the best definition when looking at the liver on imaging and distinguishing biliary anatomy.↗
▶Ep 4 · 3:00
quoteEOVIST is an MRI contrast agent that is taken up by the hepatocytes and then excreted. It can provide the best definition when looking at the liver on imaging.↗
▶Ep 4 · 3:00
quoteEOVIST is an MRI contrast agent that is taken up by the hepatocytes and then excreted. It can provide the best definition when looking at the liver on imaging.↗
▶Ep 4 · 3:00
clinicalEOVIST is an MRI contrast agent that is taken up by hepatocytes and then excreted, providing the best definition when looking at the liver on imaging and distinguishing biliary anatomy.↗
▶Ep 4 · 4:08
quoteHe injected them into portal vessels. And so that's really how these are labeled. But he labeled them in this counterclockwise fashion after, I can never pronounce this, but the districts of Paris are done in the same fashion.↗
▶Ep 4 · 4:08
clinicalCouinaud labeled the liver segments in a counterclockwise fashion after the districts of Paris, by injecting portal vessels.↗
▶Ep 4 · 4:08
quoteHe injected them into portal vessels. And so that's really how these are labeled. But he labeled them in this counterclockwise fashion after, I can never pronounce this, but the districts of Paris are done in the same fashion.↗
▶Ep 4 · 4:08
clinicalCouinaud labeled the liver segments in a counterclockwise fashion after the districts of Paris, by injecting portal vessels.↗
▶Ep 4 · 4:45
clinicalThe right hepatic vein differentiates the anterior sections (segments 5 and 8) from the posterior sections (segments 6 and 7) of the right liver.↗
▶Ep 4 · 4:45
clinicalThe right portal vein separates superior segments (7 and 8) from inferior segments (5 and 6) in the right liver.↗
▶Ep 4 · 4:45
clinicalThe right portal vein separates superior segments (7 and 8) from inferior segments (5 and 6) in the right liver.↗
▶Ep 4 · 4:45
clinicalThe right hepatic vein differentiates the anterior sections (segments 5 and 8) from the posterior sections (segments 6 and 7) of the right liver.↗
▶Ep 4 · 7:15
epidemiologicalThe change in practice for PRETEXT 3 and 4 tumors improved survival rates for patients with hepatoblastoma from less than 30% in the 1970s and 1980s to between 80 and 90% today.↗
▶Ep 4 · 7:15
quotePretext 3 and 4 is the big game changer. This change in practice is what improves survival rates for patients with hepatoblastoma from less than 30% in the 70s and 80s to between 80 and 90% today.↗
▶Ep 4 · 7:15
quotePretext 3 and 4 is the big game changer. This change in practice is what improves survival rates for patients with hepatoblastoma from less than 30% in the 70s and 80s to between 80 and 90% today.↗
▶Ep 4 · 7:15
epidemiologicalThe change in practice for PRETEXT 3 and 4 tumors improved survival rates for patients with hepatoblastoma from less than 30% in the 1970s and 1980s to between 80 and 90% today.↗
▶Ep 4 · 7:31
guidelineFor PRETEXT 3 or 4 liver disease, specific indications for transplant include unresectable disease, unsafe resection, or resection that would leave the patient with an inadequate liver remnant.↗
▶Ep 4 · 7:31
guidelinePRETEXT 3 tumors should generally be biopsied at diagnosis and then started on neoadjuvant chemotherapy, with readiness to send patients to a transplant center or a center with expertise in advanced liver resections depending on response.↗
▶Ep 4 · 7:31
guidelineFor PRETEXT 3 or 4 liver disease, specific indications for transplant include unresectable disease, unsafe resection, or resection that would leave the patient with an inadequate liver remnant.↗
▶Ep 4 · 7:31
guidelinePRETEXT 3 tumors should generally be biopsied at diagnosis and then started on neoadjuvant chemotherapy, with readiness to send patients to a transplant center or a center with expertise in advanced liver resections depending on response.↗
▶Ep 4 · 8:19
quoteYou refer these patients to liver transplant programs much earlier. And this is why we see so many more patients nowadays than perhaps when Fred and Maria were running the program 15 years ago. Because teams are trained to recognize tumors and send them to a center that offers transplant or can do aggressive resection.↗
▶Ep 4 · 8:19
quoteYou refer these patients to liver transplant programs much earlier. And this is why we see so many more patients nowadays than perhaps when Fred and Maria were running the program 15 years ago. Because teams are trained to recognize tumors and send them to a center that offers transplant or can do aggressive resection.↗
▶Ep 4 · 8:19
clinicalTeams are now trained to recognize hepatoblastoma tumors and send them to centers that offer transplant or can do aggressive resection, resulting in more patient referrals than 15 years ago.↗
▶Ep 4 · 8:19
clinicalTeams are now trained to recognize hepatoblastoma tumors and send them to centers that offer transplant or can do aggressive resection, resulting in more patient referrals than 15 years ago.↗
▶Ep 4 · 9:06
quoteThe FIT study has been going on now for three years. We've had the privilege of contributing to it in terms of its development.↗
▶Ep 4 · 9:06
clinicalThe FIT study has been ongoing for three years.↗
▶Ep 4 · 9:06
quoteThe FIT study has been going on now for three years. We've had the privilege of contributing to it in terms of its development.↗
▶Ep 4 · 9:06
clinicalThe FIT study has been ongoing for three years.↗
Hepatoblastoma with Dr. Greg Tiao
▶Ep 5 · 0:46
epidemiologicalHepatoblastoma is nowhere near the most common liver tumor or the most common abdominal tumor in children↗
▶Ep 5 · 0:46
quoteHepatoblastoma is the most common malignant liver tumor in children, but it's nowhere near the most common liver tumor or the most common abdominal tumor.↗
▶Ep 5 · 2:20
clinicalHepatoblastoma can present as an asymptomatic abdominal mass, tumor rupture, or respiratory distress due to tumor size↗
▶Ep 5 · 3:13
clinicalEovist is an MRI contrast agent taken up by hepatocytes and then excreted, providing the best definition when looking at the liver on imaging↗
▶Ep 5 · 3:13
clinicalMost experts rely on MRI to delineate anatomy and evaluate the extent of liver involvement in hepatoblastoma↗
▶Ep 5 · 4:25
clinicalAggressive resections include trisectionectomy, mesohepatectomy, resection with additional procedure in remnant liver, and liver transplantation↗
▶Ep 5 · 5:41
clinicalThe right hepatic vein differentiates anterior and posterior sections of the right liver↗
▶Ep 5 · 6:02
clinicalThe right portal vein separates superior and inferior segments of the right liver↗
▶Ep 5 · 6:51
clinicalPre-text 1 has three contiguous sections free of disease, pre-text 2 has two, pre-text 3 has one, and pre-text 4 has no free sections or diffuse tumor↗
▶Ep 5 · 6:51
clinicalThe pre-text staging system classifies tumors based on the number of contiguous liver sections free of disease (minus four equals pre-text stage)↗
▶Ep 5 · 8:15
clinicalPre-text 3 and 4 tumors represent a big game changer in practice that improved survival from less than 30% in the 1970s-80s to 80-90% today↗
▶Ep 5 · 8:15
clinicalPre-text 3 tumors should generally be biopsied at diagnosis, started on neoadjuvant chemotherapy, then referred to transplant center or center with expertise in advanced liver resections↗
▶Ep 5 · 9:35
quoteYou refer these patients to liver transplant programs much earlier. And this is why we see so many more patients nowadays than perhaps when Fred and Maria were running the program, uh 15 years ago because teams are are trained to recognize the tumors and send them to a center that offers transplant um um or or can and can do aggressive resection.↗
▶Ep 5 · 9:35
clinicalIt is important to consult a transplant center early for pre-text 3 and 4 tumors↗
▶Ep 5 · 10:27
clinicalThe PHITT (Pediatric Hepatic International Tumor Trial) is an ongoing multinational trial to identify high and low risk features for each pre-text stage↗
▶Ep 5 · 10:27
clinicalThe PHITT study has been ongoing for three years↗
Urologic Fetal Intervention: Cincinnati Fetal Center
▶Ep 2 · 7:38
clinicalWhen a vesicoamniotic shunt is placed, the bladder often collapses and upper tracts decompress significantly, though residual hydroureteronephrosis may persist.↗
▶Ep 2 · 11:35
host_summaryFetoscopic cystoscopy altered the diagnosis in 25–33% of fetuses with suspected LUTO, according to a review by Katie Morris.↗
▶Ep 2 · 11:58
guidelineFetoscopic cystoscopy must be considered experimental for LUTO until properly evaluated in a rigorous trial.↗
▶Ep 2 · 12:09
host_summaryA French-Brazilian-Houston study of 111 LUTO fetuses found that only cystoscopy (not shunting) may prevent renal function impairment at 6 months of age.↗
▶Ep 2 · 12:55
host_summaryIn a Barcelona-Leuven series of 20 fetoscopic valve ablations, bladder size and amniotic fluid normalized in 80% of cases; among livebirths, there was no pulmonary hypoplasia and three-quarters had normal renal function.↗
▶Ep 2 · 13:36
host_summaryFetoscopic valve ablation carries a 9–10% risk of urethral fistula and a 6% risk of recurrent severe LUTO.↗
▶Ep 2 · 14:06
clinicalVesicoamniotic shunt complications include migration, blockage, and (rarely) iatrogenic gastroschisis when placed at 17 weeks.↗
▶Ep 2 · 34:57
epidemiologicalIn Mark Johnson's historical studies, the third vesicocentesis provided fresher urine that correlated strongly with histologic renal damage; the first and second taps were not predictive.↗
quoteThe major renal morbidity comes postnatally.↗
▶Ep 2 · 43:12
opinionGreg Ryan argues there is no role for open fetal surgery in LUTO, especially in the worst-prognosis group, given the maternal and fetal risks and the experimental nature of the procedure.↗
▶Ep 2 · 43:18
quoteI would argue that there is absolutely no role for open surgery for a fetus, particularly the ones in the group you've identified, and I think your own results are simply supporting that.↗
▶Ep 2 · 49:58
epidemiologicalIn a long-term follow-up series, one-third of shunted LUTO survivors developed end-stage renal disease requiring transplant after age 5 years, despite having acceptable discharge creatinine.↗
▶Ep 2 · 51:24
epidemiologicalPosterior urethral valve patients required transplant at median age 10–12 years; prune belly/urethral hypoplasia at ~4.5 years; urethral atresia earlier.↗
▶Ep 2 · 52:13
clinicalProgressive renal injury in shunted LUTO survivors is driven by repeated urinary tract infections, vesicoureteral reflux, and valve bladder dysfunction.↗
▶Ep 2 · 52:49
quoteThese MFM crazy people are trying to give us survivors of what would have otherwise been a lethal disease.↗
▶Ep 2 · 54:16
quoteWe may be giving you pretty good kids, but we really have to figure out how to protect those kidneys after these kids are born.↗
▶Ep 2 · 58:46
opinionMark Johnson's closing pearl: patient selection is the most critical factor in fetal intervention for LUTO.↗
opinionGreg Ryan's closing pearl: just because a procedure can be done does not mean it should be done; honest counseling about outcomes is essential.↗
▶Ep 2 · 1:00:42
quoteJust because we can do them doesn't always mean we should do them.↗
epidemiologicalHepatoblastoma is nowhere near the most common liver tumor or the most common abdominal tumor in children↗
▶Ep 9 · 0:46
quoteHepatoblastoma is the most common malignant liver tumor in children, but it's nowhere near the most common liver tumor or the most common abdominal tumor.↗
▶Ep 9 · 2:20
clinicalHepatoblastoma can present as an asymptomatic abdominal mass, tumor rupture, or respiratory distress due to tumor size↗
▶Ep 9 · 3:13
clinicalMost experts rely on MRI to delineate anatomy and evaluate the extent of liver involvement in hepatoblastoma↗
▶Ep 9 · 3:13
clinicalEovist is an MRI contrast agent taken up by hepatocytes and then excreted, providing the best definition when looking at the liver on imaging↗
▶Ep 9 · 4:25
clinicalAggressive resections include trisectionectomy, mesohepatectomy, resection with additional procedure in remnant liver, and liver transplantation↗
▶Ep 9 · 5:41
clinicalThe right hepatic vein differentiates anterior and posterior sections of the right liver↗
▶Ep 9 · 6:02
clinicalThe right portal vein separates superior and inferior segments of the right liver↗
▶Ep 9 · 6:51
clinicalPre-text 1 has three contiguous sections free of disease, pre-text 2 has two, pre-text 3 has one, and pre-text 4 has no free sections or diffuse tumor↗
▶Ep 9 · 6:51
clinicalThe pre-text staging system classifies tumors based on the number of contiguous liver sections free of disease (minus four equals pre-text stage)↗
▶Ep 9 · 8:15
clinicalPre-text 3 and 4 tumors represent a big game changer in practice that improved survival from less than 30% in the 1970s-80s to 80-90% today↗
▶Ep 9 · 8:15
clinicalPre-text 3 tumors should generally be biopsied at diagnosis, started on neoadjuvant chemotherapy, then referred to transplant center or center with expertise in advanced liver resections↗
▶Ep 9 · 9:35
quoteYou refer these patients to liver transplant programs much earlier. And this is why we see so many more patients nowadays than perhaps when Fred and Maria were running the program, uh 15 years ago because teams are are trained to recognize the tumors and send them to a center that offers transplant um um or or can and can do aggressive resection.↗
▶Ep 9 · 9:35
clinicalIt is important to consult a transplant center early for pre-text 3 and 4 tumors↗
▶Ep 9 · 10:27
clinicalThe PHITT study has been ongoing for three years↗
▶Ep 9 · 10:27
clinicalThe PHITT (Pediatric Hepatic International Tumor Trial) is an ongoing multinational trial to identify high and low risk features for each pre-text stage↗
Urologic Fetal Intervention: Cincinnati Fetal Center
▶Ep 2 · 7:38
clinicalWhen a vesicoamniotic shunt is placed, the bladder often collapses and upper tracts decompress significantly, though residual hydroureteronephrosis may persist.↗
▶Ep 2 · 11:35
host_summaryFetoscopic cystoscopy altered the diagnosis in 25–33% of fetuses with suspected LUTO, according to a review by Katie Morris.↗
▶Ep 2 · 11:58
guidelineFetoscopic cystoscopy must be considered experimental for LUTO until properly evaluated in a rigorous trial.↗
▶Ep 2 · 12:09
host_summaryA French-Brazilian-Houston study of 111 LUTO fetuses found that only cystoscopy (not shunting) may prevent renal function impairment at 6 months of age.↗
▶Ep 2 · 12:55
host_summaryIn a Barcelona-Leuven series of 20 fetoscopic valve ablations, bladder size and amniotic fluid normalized in 80% of cases; among livebirths, there was no pulmonary hypoplasia and three-quarters had normal renal function.↗
▶Ep 2 · 13:36
host_summaryFetoscopic valve ablation carries a 9–10% risk of urethral fistula and a 6% risk of recurrent severe LUTO.↗
▶Ep 2 · 14:06
clinicalVesicoamniotic shunt complications include migration, blockage, and (rarely) iatrogenic gastroschisis when placed at 17 weeks.↗
▶Ep 2 · 34:57
epidemiologicalIn Mark Johnson's historical studies, the third vesicocentesis provided fresher urine that correlated strongly with histologic renal damage; the first and second taps were not predictive.↗
quoteThe major renal morbidity comes postnatally.↗
▶Ep 2 · 43:12
opinionGreg Ryan argues there is no role for open fetal surgery in LUTO, especially in the worst-prognosis group, given the maternal and fetal risks and the experimental nature of the procedure.↗
▶Ep 2 · 43:18
quoteI would argue that there is absolutely no role for open surgery for a fetus, particularly the ones in the group you've identified, and I think your own results are simply supporting that.↗
▶Ep 2 · 49:58
epidemiologicalIn a long-term follow-up series, one-third of shunted LUTO survivors developed end-stage renal disease requiring transplant after age 5 years, despite having acceptable discharge creatinine.↗
▶Ep 2 · 51:24
epidemiologicalPosterior urethral valve patients required transplant at median age 10–12 years; prune belly/urethral hypoplasia at ~4.5 years; urethral atresia earlier.↗
▶Ep 2 · 52:13
clinicalProgressive renal injury in shunted LUTO survivors is driven by repeated urinary tract infections, vesicoureteral reflux, and valve bladder dysfunction.↗
▶Ep 2 · 52:49
quoteThese MFM crazy people are trying to give us survivors of what would have otherwise been a lethal disease.↗
▶Ep 2 · 54:16
quoteWe may be giving you pretty good kids, but we really have to figure out how to protect those kidneys after these kids are born.↗
▶Ep 2 · 58:46
opinionMark Johnson's closing pearl: patient selection is the most critical factor in fetal intervention for LUTO.↗
opinionGreg Ryan's closing pearl: just because a procedure can be done does not mean it should be done; honest counseling about outcomes is essential.↗
▶Ep 2 · 1:00:42
quoteJust because we can do them doesn't always mean we should do them.↗
quoteHepatoblastoma is the most common malignant liver tumor in children, but it's nowhere near the most common liver tumor or the most common abdominal tumor.↗
▶Ep 11 · 0:46
epidemiologicalHepatoblastoma is nowhere near the most common liver tumor or the most common abdominal tumor in children↗
▶Ep 11 · 2:20
clinicalHepatoblastoma can present as an asymptomatic abdominal mass, tumor rupture, or respiratory distress due to tumor size↗
▶Ep 11 · 3:13
clinicalMost experts rely on MRI to delineate anatomy and evaluate the extent of liver involvement in hepatoblastoma↗
▶Ep 11 · 3:13
clinicalEovist is an MRI contrast agent taken up by hepatocytes and then excreted, providing the best definition when looking at the liver on imaging↗
▶Ep 11 · 4:25
clinicalAggressive resections include trisectionectomy, mesohepatectomy, resection with additional procedure in remnant liver, and liver transplantation↗
▶Ep 11 · 5:41
clinicalThe right hepatic vein differentiates anterior and posterior sections of the right liver↗
▶Ep 11 · 6:02
clinicalThe right portal vein separates superior and inferior segments of the right liver↗
▶Ep 11 · 6:51
clinicalThe pre-text staging system classifies tumors based on the number of contiguous liver sections free of disease (minus four equals pre-text stage)↗
▶Ep 11 · 6:51
clinicalPre-text 1 has three contiguous sections free of disease, pre-text 2 has two, pre-text 3 has one, and pre-text 4 has no free sections or diffuse tumor↗
▶Ep 11 · 8:15
clinicalPre-text 3 and 4 tumors represent a big game changer in practice that improved survival from less than 30% in the 1970s-80s to 80-90% today↗
▶Ep 11 · 8:15
clinicalPre-text 3 tumors should generally be biopsied at diagnosis, started on neoadjuvant chemotherapy, then referred to transplant center or center with expertise in advanced liver resections↗
▶Ep 11 · 9:35
quoteYou refer these patients to liver transplant programs much earlier. And this is why we see so many more patients nowadays than perhaps when Fred and Maria were running the program, uh 15 years ago because teams are are trained to recognize the tumors and send them to a center that offers transplant um um or or can and can do aggressive resection.↗
▶Ep 11 · 9:35
clinicalIt is important to consult a transplant center early for pre-text 3 and 4 tumors↗
▶Ep 11 · 10:27
clinicalThe PHITT (Pediatric Hepatic International Tumor Trial) is an ongoing multinational trial to identify high and low risk features for each pre-text stage↗
▶Ep 11 · 10:27
clinicalThe PHITT study has been ongoing for three years↗