ABSTRACT
CD is a rare autosomal recessive disease, characterized by multifocal cystic dilatation of intrahepatic bile ducts. The course of the disease is characterized by intrahepatic cholelithiasis, recurrent episodes of cholangitis, because of cholelithiasis, hepatic abscesses often ending in death caused by uncontrolled infection. Other conditions such as choledochal cyst and renal cystic disease are frequently associated, and patients have a higher risk for the development of cholangiocarcinoma. Endoscopic drainage of the bile duct is palliative and ineffective. OLT appears to be the treatment of choice. In monolobar cases partial liver resection has been shown to be a curative therapeutic option. We report on the course of disease in a Turkish girl who was diagnosed with CD in the neonatal period. At the age of 8.2 yr, she received OLT and is in good health 57 months post-transplantation.
Subject(s)
Caroli Disease/therapy , Liver Transplantation/methods , Bile Ducts, Intrahepatic/pathology , Biopsy , Child , Female , Humans , Immunosuppressive Agents/therapeutic use , Kidney/pathology , Liver/diagnostic imaging , Liver/pathology , Tomography, X-Ray Computed/methods , Treatment OutcomeABSTRACT
Primary pancreatic lymphoma (PPL) is a rare form of extranodal lymphoma. So far, only in adults has a coincidence with diabetes mellitus (DM) been described. We report a nearly 9-year-old German boy presenting with a Burkitt lymphoma associated with a temporary insulin-dependent DM and exocrine insufficiency of the pancreas. After the patient went into remission, a spontaneous recovery of the endocrine pancreatic function was observed. On immunophenotyping, the lymphoma cells revealed expression of FasL suggesting a potentially apoptotic effect on pancreatic tissue. Beta cell impairment may have been caused by FasL resembling autoimmune diabetes.
