Increased Elastase and Matrix Metalloproteinase Levels in the Pulmonary Arteries of Infants With Congenital Diaphragmatic Hernia

jpedsurg.org

Pulmonary vascular disease (PVD) complicated with pulmonary hypertension (PH) is a leading cause of mortality in congenital diaphragmatic hernia (CDH). Unfortunately, CDH patients are often resistant to PH therapy. Using the nitrogen CDH rat model, we previously demonstrated that CDH-associated PVD involves an induction of elastase and matrix metalloproteinase (MMP) activities, increased osteopontin and epidermal growth factor (EGF) levels, and enhanced smooth muscle cell (SMC) proliferation. Here, we aimed to determine whether the levels of the key members of this proteinase-induced pathway are also elevated in the pulmonary arteries (PAs) of CDH patients.

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Article · Jan 2024 · 1 min read

In brief

This study investigates the molecular mechanisms underlying pulmonary hypertension in infants with congenital diaphragmatic hernia, focusing on elevated elastase and matrix metalloproteinase levels in pulmonary arteries. Building on rat model findings, the research examines whether similar proteinase pathway activation occurs in human CDH patients, potentially explaining therapy resistance and high mortality rates.

Pulmonary vascular disease with pulmonary hypertension is a leading cause of death in CDH infants and often resists standard therapy
Rat CDH models show elevated elastase, MMP activity, osteopontin, and EGF levels driving smooth muscle cell proliferation in pulmonary vessels
This study validates whether the proteinase-induced vascular remodeling pathway identified in animal models also occurs in human CDH patients
Understanding elastase and MMP upregulation in CDH pulmonary arteries may reveal novel therapeutic targets beyond conventional PH treatments

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