First living donor liver transplantation for congenital hepatic fi brosis in Azerbaijan
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 manag...
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| Format: | Article |
| Language: | English |
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N.V. Sklifosovsky Research Institute for Emergency Medicine of Moscow Healthcare Department
2017-06-01
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| Series: | Трансплантология (Москва) |
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| Online Access: | https://www.jtransplantologiya.ru/jour/article/view/165 |
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| author | R. A. Mamedov N. Yu. Bayramov |
| author_facet | R. A. Mamedov N. Yu. Bayramov |
| author_sort | R. A. Mamedov |
| collection | DOAJ |
| description | 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. |
| format | Article |
| id | doaj-art-ab5628c20e30497399850065c390807b |
| institution | Kabale University |
| issn | 2074-0506 2542-0909 |
| language | English |
| publishDate | 2017-06-01 |
| publisher | N.V. Sklifosovsky Research Institute for Emergency Medicine of Moscow Healthcare Department |
| record_format | Article |
| series | Трансплантология (Москва) |
| spelling | doaj-art-ab5628c20e30497399850065c390807b2025-08-20T03:35:07ZengN.V. Sklifosovsky Research Institute for Emergency Medicine of Moscow Healthcare DepartmentТрансплантология (Москва)2074-05062542-09092017-06-019211311710.23873/2074-0506-2017-9-2-113-117159First living donor liver transplantation for congenital hepatic fi brosis in AzerbaijanR. A. Mamedov0N. Yu. Bayramov1Teaching Surgical Clinic of Azerbaijan Medical UniversityTeaching Surgical Clinic of Azerbaijan Medical UniversityCongenital 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.https://www.jtransplantologiya.ru/jour/article/view/165liver fibrosisliving donor liver transplantationcongenital hepatic fibrosischronic liver failure |
| spellingShingle | R. A. Mamedov N. Yu. Bayramov First living donor liver transplantation for congenital hepatic fi brosis in Azerbaijan Трансплантология (Москва) liver fibrosis living donor liver transplantation congenital hepatic fibrosis chronic liver failure |
| title | First living donor liver transplantation for congenital hepatic fi brosis in Azerbaijan |
| title_full | First living donor liver transplantation for congenital hepatic fi brosis in Azerbaijan |
| title_fullStr | First living donor liver transplantation for congenital hepatic fi brosis in Azerbaijan |
| title_full_unstemmed | First living donor liver transplantation for congenital hepatic fi brosis in Azerbaijan |
| title_short | First living donor liver transplantation for congenital hepatic fi brosis in Azerbaijan |
| title_sort | first living donor liver transplantation for congenital hepatic fi brosis in azerbaijan |
| topic | liver fibrosis living donor liver transplantation congenital hepatic fibrosis chronic liver failure |
| url | https://www.jtransplantologiya.ru/jour/article/view/165 |
| work_keys_str_mv | AT ramamedov firstlivingdonorlivertransplantationforcongenitalhepaticfibrosisinazerbaijan AT nyubayramov firstlivingdonorlivertransplantationforcongenitalhepaticfibrosisinazerbaijan |