Abstract
Congenital hepatic fibrosis is a rare autosomal recessive disease. Only a few such cases have been described worldwide, but the exact incidence of the disease is unknown. The diagnosis is sometimes difficult to establish and one of the main diagnostic method is the histological evaluation. The management and prognosis of congenital hepatic fibrosis is dependent on alimentary tract bleeding secondary to portal hypertension. In late childhood, the abdominal pain, cholangitis, and features of hypersplenism complicate the problem. Herein we present the case report of patient with congenital hepatic fibrosis. Our choice of treatment was the living donor liver transplantation. This procedure is very difficult but it is the only life-saving option for the patients with congenital hepatic fibrosis.
Highlights
Congenital hepatic fibrosis is a rare autosomal recessive disease
The management and prognosis of congenital hepatic fibrosis is dependent on alimentary tract bleeding secondary to portal hypertension
3. Часто единственным и жизнесохраняющим методом лечения Врожденный фиброз печени (ВФП) является ортотопическая трансплантация печени
Summary
Congenital hepatic fibrosis is a rare autosomal recessive disease. Only a few such cases have been described worldwide, but the exact incidence of the disease is unknown. Mamedov R.A., Bayramov N.Yu. First living donor liver transplantation for congenital hepatic fibrosis in Azerbaijan. Врожденный фиброз печени (ВФП) представляет собой необычное состояние, при котором портальная гипертензия (ПГ) возникает без значительных нарушений функции печени или почек и характеризуется гистологически дефектным ремоделированием пластинок протока. В данной работе мы сообщаем о случае трансплантации родственной донорской печени пациенту с ВФП.
Sign-in/Register to view highlights & summary Sign-in/Register to access full text options 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a call
