Successful live related renal transplant in a case of tuberous sclerosis.

Tuberous sclerosis (TS) is an autosomal dominant multisystemic disease involving primarily the skin, the brain and the kidneys. Inspite of the kidney being involved in 40-80% of patients with this disease, the incidence of end stage renal disease is only about 1%. There are only 34 reported cases of successful renal transplantation in tuberous sclerosis patients with end stage renal disease. We report a case of successful renal transplantation in a patient of tuberous sclerosis with bilateral polycystic kidneys presenting with renal failure who also underwent bilateral native nephrectomies on follow up.

Results of liver transplantation in fulminant hepatic failure.

Liver transplantation has dramatically changed the management of patients with fulminant hepatic failure and subacute hepatic failure. A wide range of survival rates (55% to 90%) reported from different centers performing liver transplantation for fulminant hepatic failure reflects variations in patient selection and the centers' experience. Results of transplantation appear to be improving, with survival rates approaching 80% at 1 year in some series. The improved results are attributed to vigorous perioperative management, using intracranial pressure monitoring and continuous arteriovenous hemofiltration when required, early listing of patients with organ-sharing network, and exclusion of patients who are unlikely to recover neurologically after successful transplantation. Use of piggyback hepatectomy to minimize hemodynamic alterations during surgery has also contributed to better results in critically ill patients. Patients with acetaminophen toxicity have a more favorable outcome than those with viral hepatitis or non-acetaminophen drug hepatotoxicity. Because of limited availability of cadaveric organs, emergency living-related liver transplantation, use of ABO incompatible and marginal livers need consideration. Auxiliary liver transplantation is desirable for those who have a chance of spontaneous recovery, thus obviating life-long immunosuppression after recovery of the native liver.

Fibrosing cholestatic hepatitis-like syndrome in a hepatitis B virus and hepatitis C virus-negative renal transplant recipient: a case report with autopsy findings.

We report a patient with fibrosing cholestatic hepatitis (FCH)-like syndrome in renal transplant recipient, who was negative for hepatitis-B and C-virus infection. The patient presented initially with extrahepatic biliary obstruction due to stricture at the lower end of the common bile duct. Cholestasis persisted inspite of effective biliary drainage. He was operated for empyema of the gallbladder and histological examination showed the presence of cytomegalovirus inclusions in the wall of the gallbladder. The patient died inspite of aggressive management; autopsy examination of the liver revealed evidence of FCH-like changes.