technetium-tc-99m-sulfur-colloid and Cholestasis--Intrahepatic

Byler's syndrome is a rare form of autosomal recessive intrahepatic cholestasis that is fatal in children. A 10-year-old girl diagnosed with Byler's syndrome underwent reduced-size liver transplantation using lateral segments of her living mother's liver. The donor's and the recipient's liver functions after transplantation were evaluated using Tc-99m disofenin and Tc-99m SC to investigate morphology, liver perfusion, and hepatobiliary function.

Topics: Child; Cholestasis, Intrahepatic; Female; Genes, Recessive; Hepatectomy; Humans; Imino Acids; Liver Transplantation; Organotechnetium Compounds; Radionuclide Imaging; Syndrome; Technetium Tc 99m Disofenin; Technetium Tc 99m Sulfur Colloid; Tissue Donors

Topics: Aged; Aged, 80 and over; Cholestasis, Intrahepatic; Female; Humans; Radionuclide Imaging; Technetium Tc 99m Sulfur Colloid

Intrahepatic cholelithiasis is commonly associated with suppurative cholangitis and occurs equally in males and females. Patients usually present with a history of recurrent symptoms and a differential diagnosis which includes viral hepatitis, pancreatitis, myocardial infarction and perforated ulcer. Cholangiography including stereocholangiography is essential for localizing intrahepatic stones and ductal strictures. The guiding operative principle is to drain all infected bile distal to ducts obstructed with stones or stricture. This may also necessitate a bilioenterostomy with or without partial hepatic resection. Although these procedures are formidable in an acutely ill patient, a more simple but inappropriate choledocholithotomy and t-tube drainage done proximal to an obstructed intrahepatic duct will be ineffective and may result in continued sepsis and death.

Topics: Adult; Bile Ducts, Intrahepatic; Cholangiography; Cholangitis; Cholelithiasis; Cholestasis, Intrahepatic; Diagnosis, Differential; Drainage; Female; Humans; Male; Technetium Tc 99m Sulfur Colloid; Ultrasonography

Topics: Biopsy; Cholangiopancreatography, Endoscopic Retrograde; Cholestasis, Extrahepatic; Cholestasis, Intrahepatic; Hepatitis; Humans; Hyperbilirubinemia; Hyperbilirubinemia, Hereditary; Jaundice; Liver Function Tests; Sulfur; Technetium; Technetium Tc 99m Sulfur Colloid; Ultrasonography