Adolescent ; Bile Ducts, Intrahepatic ; abnormalities ; Cholestasis, Intrahepatic ; etiology ; Humans ; Male

We report 13 children fulfilling criteria of Alagille syndrome. All had chronic cholestasis secondary to paucity of intrahepatic bile ducts and triangular facies. Eight children had associated congenital heart disease (six pulmonic stenosis, one each tetralogy of Fallot and patent ductus arteriosus), seven with butterfly vertebrae and one with posterior embryotoxon. Seven of the 13 children are alive and jaundice-free but three with concomitant hypercholesterolemia; the six other children died of liver-related complications.
Butterflies ; Bile Ducts, Intrahepatic ; Jaundice ; Musculoskeletal Abnormalities ; Spine

Adult ; Bile Ducts, Intrahepatic ; abnormalities ; Cholestasis, Intrahepatic ; diagnosis ; etiology ; therapy ; Female ; Humans

Adolescent ; Bile Duct Diseases ; congenital ; Bile Ducts, Intrahepatic ; abnormalities ; Dilatation, Pathologic ; Female ; Humans

Congenital hepatic fibrosis is an inherited, congenital disorder of the liver, and is occasionally associated with cystic disease of the liver and kidney. We present a case of congenital hepatic fibrosis with Caroli's disease. A 21-year-old woman had suffered from an episodic fever with headaches for 3 years. In laboratory examination, the liver function test was within the normal limits. Esophageal varix was noted by an endoscopic examination. Hepatosplenomegaly and multiple dilated bile ducts were seen by abdominal CT scanning. An orthotopic whole liver transplantation was done. The liver was fibrotic and enlarged. Multiple cystically dilated intrahepatic ducts were noted. Microscopically, diffuse portal fibrosis and widening with proliferation of bile ductules were seen. Intrahepatic bile ducts were markedly dilated and tortuous. The liver cell cords were well preserved.
Bile ; Bile Ducts ; Bile Ducts, Intrahepatic ; Caroli Disease* ; Congenital, Hereditary, and Neonatal Diseases and Abnormalities ; Esophageal and Gastric Varices ; Female ; Fever ; Fibrosis* ; Headache ; Humans ; Kidney ; Liver ; Liver Function Tests ; Liver Transplantation ; Tomography, X-Ray Computed ; Young Adult

Primary biliary cirrhosis is a chronic, progressive liver disease characterized by inflammatory destruction of septal and intralobular bile ducts which results in intrahepatic cholestasis. Although the cause remains obscure, it is frequently associated with a variety of disorders presumed to be autoimmune in nature. We report a case of early primary biliary cirrhosis which was anteceded by rheumatoid arthritis. The patient was a 54-year-old female who was admitted due to arthralgia and joint deformity. She had been diagnosed as having rheumatoid arthritis 10 years before. On admission, she had elevated serum ALT, AST, alkaline phosphatase, gamma-GTP and positive serum antimitochondrial antibody test. Microscopic findings of the liver were consistent with the early stage of primary bi]iary cirrhosis.
Alkaline Phosphatase ; Arthralgia ; Arthritis, Rheumatoid* ; Bile Ducts ; Cholestasis, Intrahepatic ; Congenital Abnormalities ; Female ; Fibrosis ; Humans ; Joints ; Liver ; Liver Cirrhosis, Biliary* ; Liver Diseases ; Middle Aged

Combined hepatocellular carcinoma (HCC) and cholangiocarcinoma (CC) is sometimes found in resected livers, however, cases with double cancer of HCC and CC are very rare. As well, the rarity of CC arising in von Meyenburg complexes (VMCs) is appreciated. We report the case of a 74-year-old man found to have intrahepatic double cancer composed of well-differentiated HCC and CC which exhibited a histologic progression from VMCs to adenomatous lesions and CC. To our knowledge, this is the first case report published in the literature of a double HCC and CC associated with multiple VMCs. The pathogenesis and previous associated reports of these lesions are discussed.
Aged ; Bile Ducts, Intrahepatic/abnormalities* ; Carcinoma, Hepatocellular/pathology* ; Carcinoma, Hepatocellular/etiology ; Case Report ; Cholangiocarcinoma/pathology* ; Cholangiocarcinoma/etiology ; HumanLiver Neoplasms/pathology* ; Liver Neoplasms/etiology ; Male ; Neoplasms, Multiple Primary/pathology* ; Neoplasms, Multiple Primary/etiology