title: [Histopathological features of 27 cases of primary biliary cirrhosis].
abstract: OBJECTIVE
  To explore the clinical and histopathological features of primary biliary cirrhosis (PBC).
  
  
  METHODS
  The clinical, laboratory, as well as histological features of 27 cases of PBC were retrospectively analyzed.
  
  
  RESULTS
  The male to female ratio was 1:8 (3:24), aged from 22 to 69 years. The main clinical manifestations included: fatigue (62.9%, 17/27), jaundice (59.2%, 16/27) and pruritus (29.6%, 8/27), with all of patients having markedly elevated serum alkaline phosphatase and gamma-glutamyl transpeptidase and 95.8% (23/27) of the patients being positive for anti-mitochondrial antibody (AMA). The main histopathological changes were: necroinflammation of interlobular bile ducts (100%, 27/27), lymphocyte aggregation or lymphocyte follicles (15%, 4/27), granuloma (26%, 7/27), decreased number of interlobular bile ducts and smaller bile duct proliferation (55%, 15/27), feathery degeneration of hepatocytes (59%, 16/27); bilirubinostasis in hepatocytes and/or canaliculi (52%, 14/27); fibrosis and distortion of lobules (26%, 7/27), pseudolobular formation (11%, 3/27).
  
  
  CONCLUSIONS
  The main clinical features of PBS are fatigue and pruritus, markedly elevated phosphatase and gamma-glutamyl transpeptidase, and positive AMA with or without jaundice. Its histopathological hallmarks are (1)necroinflammmation and ductopenia involved mainly in interlobular bile ducts; (2)lymphocyte aggregation, granuloma formation and bile ductular proliferation in the portal area; and (3)feathery degeneration of hepatocytes.
authors.affiliation: Beijing Friendship Hospital, Capital University of Medical Sciences, Beijing 100050, China.
authors.forename: Xiaoyan
authors.initials: X
authors.lastname: Shi
authors.forename: Changhuai
authors.initials: C
authors.lastname: Zhang
authors.forename: Jidong
authors.initials: J
authors.lastname: Jia
authors.forename: Fukui
authors.initials: F
authors.lastname: Zhang
authors.forename: Linxue
authors.initials: L
authors.lastname: Qian
authors.forename: Baoen
authors.initials: B
authors.lastname: Wang
mesh_terms: D000328:Adult; D000368:Aged; D001653:Bile Ducts, Intrahepatic; D005221:Fatigue; D005260:Female; D022781:Hepatocytes; D006801:Humans; D008105:Liver Cirrhosis, Biliary / Q000503:physiopathology*; D008297:Male; D008875:Middle Aged; D008928:Mitochondria; D011537:Pruritus; D055815:Young Adult; D005723:gamma-Glutamyltransferase
chemical_list: D005723:gamma-Glutamyltransferase