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March 31, 2026 7:00 am
C Jarasvaraparn, et al. J Pediatr Gastroenterol Nutr. 2026;82:751–759. Clinical presentation, management, and outcomes for noncirrhotic portal hypertension in children
Background: Noncirrhotic portal hypertension (NCPH) is a rare condition marked by elevated portal venous pressure in the absence of cirrhosis and is caused by intrahepatic and extrahepatic pathways. NCPH is more common in Eastern countries than in Western countries. In the East, infectious diseases, particularly schistosomiasis in Africa, and poor socioeconomic conditions leading to chronic abdominal infections, are significant contributing factors. On the other hand, Western countries see a higher prevalence of NCPH linked to hypercoagulable disorders and potential genetic predispositions.
Methods: This was a single-center retrospective study of 63 pediatric patients form 2000-2024.
Key findings:

My take: More than two decades ago, a patient was referred to me with dyspnea as the pulmonologist suspected that this was due to portal hypertension/hepatopulmonary syndrome. Ultimately, after evaluation by a colleague, she was diagnosed with idiopathic noncirrhotic portal hypertension and underwent a successful liver transplantation. This condition is very difficult to diagnose if you are unaware of its existence.
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Categories: Pediatric Gastroenterology Intestinal Disorder
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