the liver transplantation program was established at shiraz nemazee hospital in 1993. shortage of cadaver organ supply due to various social and legal issues urged us to develop a living-related liver transplantation (lrlt) program. so far 7 (6 males, 1 female) living-related liver transplantations have been performed at this center. the mean age of patients was 8.21 years (+/- 4.16), with a ra...

Abdominal wall hernias are common problems found in patients with cirrhosis because of persistently high intra-abdominal pressure. When abdominal hernias are neglected in such patients, they may become larger and could result in cosmetic problems and pressure effects that are also difficult to treat. We found a voluminous mass in the anterior abdominal wall of a 40-year-old patient with cirrhos...

Spontaneous bacterial peritonitis (SBP) is a common cause of morbidity and mortality in patients with advanced cirrhosis and portal hypertension. While gram-negative rods and Enterococcus species are the common offending organisms, Salmonella has also been recognized as a rare and atypical offending organism. Atypical features of Salmonella SBP include both its occurrence in cirrhotic patients ...

Familial Mediaterranian Fever (FMF) is an ethnically related genetic disease, characterized by spontaneously resolving episodes of fever and pain. Amyloidosis of the amyloid A type is the most important manifestation of FMF, affecting many organs, including the kidneys, adrenal glands, intestines, spleen, thyroid, heart, lungs and liver. There is an increasing concern about the association of n...

AIM To characterize natural history of cryptogenic cirrhosis (CC) and compare its clinical features and outcomes to those of hepatitis C virus (HCV)-related cirrhosis. METHODS A prospective cohort of 102 consecutive patients at their first diagnosis of CC were enrolled in this study. The clinical data and outcomes were compared to an age- and Child-Pugh class-matched cohort of 110 patients wi...

Liver transplantation (LT) is the treatment of choice for patients with end-stage liver disease (ESLD). Chronic liver disease due to many causes is prevalent in a significant percentage of the Bangladeshi population. Until recently, liver transplantation facilities were not available, and ESLD patients were dying without treatment. Liver transplantation is a complex procedure that requires inte...

Congenital hepatic fibrosis is an uncommon fibrocystic disorder affecting the intrahepatic bile ducts. It has autosomal recessive inheritance. The main consequence of this condition is portal hypertension and it is often misdiagnosed as cirrhosis. Patients with congenital hepatic fibrosis usually present during childhood or early adolescence with oesophageal variceal bleeding. Portosystemic shu...

Celiac disease (CD) is a systemic immune-mediated disorder triggered by dietary gluten in genetically predisposed individuals. The typical symptoms are anemia, diarrhea, fatigue, weight loss, and abdominal pain. CD has been reported in patients with primary sclerosing cholangitis, primary biliary cholangitis, autoimmune hepatitis, aminotransferase elevations, nonalcoholic fatty liver disease, h...

Clinical and histological features provide evidence that immunological reactions of the cell-mediated type are abnormal in some forms of chronic liver disease. By using the leucocyte migration test of Søborg and Bendixen (1967), a correlate of cellular hypersensitivity in vitro, the authors studied the production of the migration inhibitory factor by lymphocytes exposed to a fetal liver homogen...

Primary Biliary Cirrhosis (PBC) is the result of long-standing damage to intra-hepatic biliary channels as a result of immune mechanisms leading to cholestasis and its associated complications. It can end up in chronic liver disease or cirrhosis. It usually manifests as pruritis that then proceeds to fatigue, jaundice and other features of chronic liver disease. It is detected by raised serolog...