Bile ; metabolism ; Cholestasis ; metabolism ; pathology ; Humans

Idiopathic adulthood ductopenia (IAD) is a chronic cholestatic liver disease of unknown etiology characterized by adult onset, an absence of autoantibodies, inflammatory bowel disease, and a loss of interlobular bile ducts. In the present report, a case fulfilling the IAD criteria is described. A 19-year-old man was admitted to the hospital for persistent elevation of transaminases and alkaline phosphatase without clinical symptoms. Viral hepatitis markers and autoantibodies were absent. The patient had a normal extrahepatic biliary tree and had no evidence of inflammatory bowel disease. A liver biopsy specimen showed absence of interlobular bile ducts from 58% of the portal tracts. He was diagnosed with IAD and was treated with ursodeoxycholic acid.
Adult ; Cholestasis, Intrahepatic/*diagnosis/etiology/pathology ; Chronic Disease ; Humans ; Male

OBJECTIVETo assess the efficacy and safety of bilateral versus unilateral biliary drainage in malignant hilar obstruction.

METHODSTopically relevant studies,regardless of randomized or observational design, were searched for in PubMed, EmBase and the Cochrane Library database. Risk ratios (RRs) with 95% confidence intervals (CIs) were calculated to compare the effect of the two treatments.

RESULTSThree randomized trials and 7 observational studies were included, involving 894 patients with malignant hilar obstruction. The meta-analysis assessment of primary outcomes showed that the stent patency rate was better in bilateral drainage than in unilateral drainage (Rr=2.03,95% CI [1.16-3.56], P=0.01), but there were no significant differences in successful drainage rate (Rr=1.07,95% CI [0.97-1.18], P=0.20) and patient survival rate (Rr=-0.16,95% CI [-0.40-0.08], P=0.20). In the analysis of secondary outcomes,there were also no significant differences in the technical success rate (Rr=1.05,95% CI [0.98-1.17], P=0.34),the early complication rate (Rr=1.15, 95% CI [0.75-1.76], P=0.52), late complication rate (Rr=1.09,95% CI [0.75-1.60], P=0.60) and 30-day mortality rate (Rr=0.68,95% CI [0.38-1.23], P=0.20).

CONCLUSIONAlthough the cumulative stent patency was better for the bilateral than the unilateral drainage approach, based on the available data, there is not enough data to support bilateral drainage for malignant hilar obstruction. Well-designed randomized controlled trials are necessary to confirm it.

Biliary Tract Neoplasms ; pathology ; Cholestasis ; therapy ; Drainage ; methods ; Humans

As a secondary endocrine organ, the liver is closely related to the endocrine system. Liver involvement is not uncommon in endocrine diseases, such as hyper/hypothyroidism, diabetes, dysfunction of adrenal and gonadal. It can be manifested in a variety of forms, including hepatocyte injury (elevated transaminase), bile duct injury (cholestasis), hepatocyte steatosis, vascular injury and liver tumor. Direct and indirect liver injury caused by abnormal hormone levels and side effects of drugs for the treatment of endocrine diseases are common pathogenesis. In addition, endocrine diseases can be concomitant with liver diseases, such as autoimmune thyroiditis and autoimmune hepatitis. Systemic diseases can also involve the endocrine system and liver at the same time, such as systemic lupus erythematosus and IgG4 related diseases. For patients with unexplained liver injury, endocrine system diseases should be considered as the differential diagnosis.
Cholestasis/pathology* ; Endocrine System Diseases/pathology* ; Hepatitis, Autoimmune/pathology* ; Humans ; Liver/pathology* ; Liver Diseases/pathology*

Progressive familial intrahepatic cholestasis type 3 (PFIC3) is an autosomal recessive disorder of cholestasis of hepatocellular origin, typically seen in infancy or childhood caused by a defect in the ABCB4 located on chromosome 7. Here we report on an older patient, aged 15, who presented with biochemical testing that led to an initial consideration of a diagnosis of Wilson disease (WD) resulting in a delayed diagnosis of PFIC3. Diagnosis of PFIC3 was later confirmed by molecular studies that identified novel mutations in the ABCB4 gene. Cholestasis due to PFIC3 can cause elevated hepatic copper and increased urine copper excretion that overlap with current diagnostic criteria for WD. Molecular diagnostics are very useful for establishing the diagnosis of PFIC3. Ursodeoxycholic acid ameliorates cholestasis in PFIC3, and may help mediate a reduction in hepatic copper content in response to treatment.
Cholestasis ; Cholestasis, Intrahepatic* ; Chromosomes, Human, Pair 7 ; Copper ; Delayed Diagnosis ; Diagnosis* ; Hepatolenticular Degeneration* ; Humans ; Molecular Biology* ; Pathology, Molecular ; Ursodeoxycholic Acid

OBJECTIVETo study the features of histopathologic and ultrastructural pathologic changes of liver biopsy in patients with infantile cholestatic disease, and to investigate its diagnostic significance combining with the clinical data.

METHODSThirty-six children diagnosed as infantile cholestatic disease and received liver biopsy in Chongqing Medical University Children's Hospital from Jun 2007 to Oct 2008 were enrolled and the pathologic and ultrastructural pathologic changes of liver were analyzed.

RESULTSMorphologic changes under light microscope in liver tissues included hepatocyte swelling, hepatocyte denaturation, hepatocyte necrosis, multinucleated giant cell formation, bile duct proliferation, fiber tissues proliferation and inflammatory cells infiltration in liver lobules and portal regions. The characteristics of cholestasis including intralobular cholestasis, acinus formation, feather-like cytoplasmic filaments and bile stasis in bile canaliculi were observed. The morphologic changes of biliary atresia were observed in 7 cases whose image investigations showed no obstruction of biliary tract. Nuclear changes, resolution of cytoplasm, inflammatory cell infiltration, collagen fiber proliferation and increased number of lysosomes were observed under electromicroscope. Two cases of glycogen storage disease, 1 case of Niemann-Pick disease and 1 case of lipid storage disease with unknown cause were confirmed by the combination of histological changes and clinical manifestations.

CONCLUSIONCommon pathologic changes of liver tissues existed under light microscope or electroscope. The diagnosis of hereditary metabolic disorders could be made increasingly by application of these two technologies in clinical practice. It is difficult to diagnose biliary atresia in early childhood by image investigations and the pathological changes of liver tissues are helpful.

Cholestasis ; diagnosis ; etiology ; pathology ; Female ; Humans ; Infant ; Liver ; pathology ; Liver Diseases ; diagnosis ; etiology ; pathology ; Male

Cholestasis, Intrahepatic ; diagnosis ; pathology ; therapy ; Female ; Humans ; Pregnancy ; Pregnancy Complications ; diagnosis ; pathology ; therapy

Chemical and Drug Induced Liver Injury ; etiology ; immunology ; pathology ; Cholestasis ; etiology ; pathology ; Humans

Drug-induced bile duct injury is a specific kind of drug-induced liver injury that has two main pathological types, namely ductopenia, or vanishing bile duct syndrome, and secondary sclerosing cholangitis. However, in recent years, the reports of new drugs that cause bile duct injury have been constantly increasing, and these drugs have different clinicopathological features and a novel pathogenesis. Therefore, this paper summarizes and analyzes the progress and challenges in the etiology, pathogenesis, diagnosis and treatment, and other aspects of drug-induced bile duct injury.
Humans ; Cholestasis/chemically induced* ; Cholangitis, Sclerosing/diagnosis* ; Chemical and Drug Induced Liver Injury/pathology* ; Bile Ducts/pathology*

To assess the extent of microfilaments in cholestatic liver diseases we examined the cytoplasmic microfilaments in intrahepatic and extrahepatic cholestasis in man by electron microscopy. Study subjects were two patients with drug-induced intrahepatic cholestasis, three patients with intrahepatic cholestasis due to viral hepatitis, four patients with extrahepatic cholestasis due to stones of the common bile duct and two patients with primary biliary cirrhosis. Two biopsied specimens from patients without clinical or histological evidence of liver disease served as noncholestatic controls. The microfilaments in hepatocytes and biliary ductular cells were significantly increased in cholestasis compared with those in non-cholestatic controls. Well developed bundles of microfilaments were noted around the pericanalicular ectoplasm and seemed to be parallel to plasma membrane of the hepatocytes in cholestasis. In cholestasis, there were increased bundles of microfilaments around the periluminal region, lateral cell wall, and nucleus of biliary ductular cells. Two patterns of microfilaments bundles (fine microfilamentous network and spindle-shaped dense or clusters of microfilaments) were associated with cholestasis. The clustered form of microfilaments also seemed to be clearly associated with intracytoplasmic vacuoles containing bile salts. In conclusion, the increase of microfilaments in hepatocytes and biliary ductular cells may be the consequence of various forms of cholestasis. Further studies are needed to clarify the functional significance of increased microfilaments in cholestasis.
Bile Canaliculi/*pathology/ultrastructure ; Biopsy ; Cholestasis, Intrahepatic/*pathology ; Hepatocytes/*pathology/ultrastructure ; Humans ; Microfilaments/*pathology/ultrastructure ; Microscopy, Electron