Gastrointestinal protein loss in children with portal hypertension
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Gastrointestinal protein loss in children with portal hypertension Kornchanok Chindaratana 1 & Pornthep Tanpowpong 1 Suporn Treepongkaruna 1
&
Chatmanee Lertudomphonwanit 1 &
Received: 25 April 2020 / Accepted: 15 July 2020 # Indian Society of Gastroenterology 2020
Abstract 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. Keywords Biliary atresia . Children . Chronic liver disease . Cirrhosis . Enteropathy . Gastrointestinal . Liver transplantation . Malabsorption . Protein loosing enteropathy
Introduction Protein-losing enteropathy (PLE) is characterized by serum protein loss into the lumen of gastrointestinal (GI) tract, which can be caused by various GI diseases. Three main pathophysiologic mechanisms are the followings: (1) erosive diseases such as inflammatory bowel disease, severe food allergy; (2) non-erosive diseases such as enteric infections; and (3) increased central venous pressure or mesenteric lymphatic obstruction resulting in direct leakage of protein-rich lymph [1, 2]. Increasing pressure in the veins can also cause transmitted back pressure into the intestinal lymphatic system that leads to * Pornthep Tanpowpong [email protected] 1
Division of Gastroenterology, Department of Pediatrics, Faculty of Medicine Ramathibodi Hospital, Mahidol University, Bangkok, Thailand
lacteal dilatation and rupture such as the condition seen in patients after the Fontan procedure [1]. In portal hypertension, the markedly increased portal venous pressure may lead to an increased thoracic duct pressure and downstrea
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