scholarly journals A case report. Middle-aged congenital hepatic fibrosis followed up as idiopathic portal hypertension.

Kanzo ◽  
1992 ◽  
Vol 33 (8) ◽  
pp. 643-648 ◽  
Author(s):  
Noriko KOJIMAHARA ◽  
Etsuko HASHIMOTO ◽  
Makiko TANIAI ◽  
Kiyomasa KOBAYASHI ◽  
Hiroaki OKUDA ◽  
...  
Keyword(s):  
Case Report ◽  
Portal Hypertension ◽  
Hepatic Fibrosis ◽  
Idiopathic Portal Hypertension ◽  
Congenital Hepatic Fibrosis ◽  
Middle Aged

A case of systemic sclerosis complicated by idiopathic portal hypertension: case report and literature review

2006 ◽  
Vol 16 (3) ◽  
pp. 183-187 ◽  
Author(s):  
Kenji Takagi ◽  
Shinichiro Nishio ◽  
Kimiko Akimoto ◽  
Takumi Yoshino ◽  
Shinichi Kawai
Keyword(s):  
Case Report ◽  
Portal Hypertension ◽  
Systemic Sclerosis ◽  
Literature Review ◽  
Idiopathic Portal Hypertension

scholarly journals Its Congenital Hepatic Fibrosis; Not Cirrhosis At All

2018 ◽  
Vol 1 (2) ◽  
pp. 124-126
Author(s):  
Ananta Shrestha ◽  
Mamun Al-Mahtab ◽  
Salimur Rahman ◽  
Jahangir Sarkar ◽  
Thupten K Lama
Keyword(s):  
Portal Hypertension ◽  
Hepatic Fibrosis ◽  
Autosomal Recessive ◽  
Rare Condition ◽  
Recessive Trait ◽  
Congenital Hepatic Fibrosis ◽  
Life Threatening ◽  
Hepatic Histology ◽  
Upper Abdomen ◽  
Medical University

Congenital hepatic fibrosis is a rare condition characterized by extensive fibrosis of liver but with preserved normal lobular architecture inherited as autosomal recessive trait. We report a 19 year-old-female admitted to Bangabandhu Sheikh Mujib Medical University with the complaints of lump in upper abdomen since last 13 years and episodes of fever and abdominal pain for same duration. She was diagnosed with hepatic TB on hepatic histology. Congenital hepatic fibrosis is a rare cause of portal hypertension that presents during childhood. Prognosis of congenital hepatic fibrosis is good. Life threatening events in these patients are related with variceal bleeding and episodes of cholangitis. Owing to relatively good liver function these patients tolerate portosystemic shunt surgeries quite well.Though rare, congenital hepatic fibrosis should be included in the differential diagnosis of portal hypertension in early life.

scholarly journals Focal nodular hyperplasia-like lesion of progressive type in a patient with congenital hepatic fibrosis: a case report

Kanzo ◽  
2015 ◽  
Vol 56 (9) ◽  
pp. 461-468 ◽  
Author(s):  
Mie Arakawa ◽  
Koichi Honda ◽  
Masataka Seike ◽  
Tomoko Tokumaru ◽  
Kosiro Tsutsumi ◽  
...  
Keyword(s):  
Case Report ◽  
Hepatic Fibrosis ◽  
Focal Nodular Hyperplasia ◽  
Congenital Hepatic Fibrosis ◽  
Progressive Type ◽  
Nodular Hyperplasia
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