Gastrointestinal protein loss in children with portal hypertension

Portal hypertension increases pressure in lymphatic ducts, which may lead to gastrointestinal (GI) protein loss. Reports have shown that adults with portal hypertension had resolution of protein-losing enteropathy after transplantation; but studies in children are very limited. We therefore aimed to evaluate GI protein loss in children with portal hypertension and defined changes after liver transplantation. Children aged 3 months to 18 years with portal hypertension and the age-matched healthy controls were enrolled during August 2018 to September 2019. Random fecal alpha-1 antitrypsin measurements were obtained at the initial visit, 3 months later, 1 week before, and 3 months after liver transplantation (if applicable). One or more positive test (> 0.795 mg/dL) was interpreted as a positive result. We enrolled 76 children (n = 38 in each group) with a median age of 15.5 months (interquartile range [IQR], 11.2–41.7), female 51%, and 92% with biliary atresia in the portal hypertension group. We noted GI protein loss in 4/38 children (10.5%) with portal hypertension, while none in the controls (p = 0.11). We found no significant differences on the markers of severity of liver disease and serum albumin between patients with vs. the ones without GI protein loss (p > 0.05). After liver transplantation, 2/4 patients with GI protein loss had undetectable loss. We found that, in a small group of children, 10.5% with portal hypertension had notable GI protein loss without significant relationships with the severity of liver disease.