https://www.ncbi.nlm.nih.gov/pubmed/27906797

Sood V, Lal BB, Khanna R, Rawat D, Sharma CB, Alam S. J Pediatr Gastroenterol Nutr. 2016 Nov 30.

Abstract

BACKGROUND:

Non-cirrhotic portal fibrosis (NCPF) has been classically described as a disease of young to middle age with limited literature regarding its occurrence, onset or clinical presentation in children. We hereby present a series of 19 patients diagnosed and managed as NCPF in pediatric age group.

METHODS:

A retrospective review of all the patients presenting to the pediatric hepatology department (age < 18 years) and diagnosed as NCPF was done and data was evaluated.

RESULTS:

A total of 19 patients were diagnosed as NCPF with median age at onset of symptoms and diagnosis as 10 years and 13.8 years respectively. Majority presented with left upper quadrant discomfort or mass. Laboratory parameters showed hypersplenism in majority with preserved liver synthetic functions. Median values for hepatic venous pressure gradient (HVPG) and liver stiffness measurement (LSM) were 13.5 mm Hg and 10.6 kilopascals respectively. Classical hepatic histopathological features seen were maintained lobular architecture, atretic portal tracts, approximation of portal-portal and portal-central areas and aberrant peripheral portal channels. During follow up, majority of the patients did not show disease progression.

CONCLUSIONS:

NCPF is not an uncommon entity in pediatric population with age of onset in early second decade. Hepatic histopathology must be used to exclude cirrhosis and to confirm the diagnosis. HVPG and LSM values, in some cases, may overlap with those in cirrhotic patients and may not be diagnostic in isolation. Any patient presenting with evidence of portal hypertension with preserved hepatic functions, irrespective of the age, should be evaluated for possible NCPF.