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Primary biliary cholangitis

ORPHA:186 · Disease · Disorder

HPO 表現型（共 41 項）

Very frequent (99-80%)(6)

  • Antimitochondrial antibody positivityHP:0030167
  • Biliary cirrhosisHP:0002613
  • CirrhosisHP:0001394
  • Conjugated hyperbilirubinemiaHP:0002908
  • Dermatographic urticariaHP:0011971
  • Hyperpigmentation of the skinHP:0000953

Frequent (79-30%)(20)

  • Abnormal circulating lipid concentrationHP:0003119
  • Abnormal intrahepatic bile duct morphologyHP:0011040
  • Abnormality of the thyroid glandHP:0000820
  • Antinuclear antibody positivityHP:0003493
  • AutoimmunityHP:0002960
  • Elevated circulating alkaline phosphatase concentrationHP:0003155
  • Elevated gamma-glutamyltransferase levelHP:0030948
  • Hepatic failureHP:0001399
  • Hepatic fibrosisHP:0001395
  • Hepatocellular carcinomaHP:0001402
  • HepatomegalyHP:0002240
  • HypercholesterolemiaHP:0003124
  • Increased circulating IgM concentrationHP:0003496
  • JaundiceHP:0000952
  • Orthostatic hypotensionHP:0001278
  • Portal hypertensionHP:0001409
  • PruritusHP:0000989
  • Recurrent fungal infectionsHP:0002841
  • Unusual fungal nail infectionHP:0012203
  • XanthelasmaHP:0001114

Occasional (29-5%)(14)

  • Abdominal distentionHP:0003270
  • AscitesHP:0001541
  • Celiac diseaseHP:0002608
  • Esophageal varixHP:0002040
  • Excessive daytime somnolenceHP:0001262
  • FatigueHP:0012378
  • Hepatic encephalopathyHP:0002480
  • HepatitisHP:0012115
  • HypoalbuminemiaHP:0003073
  • Increased circulating IgA concentrationHP:0003261
  • OsteoporosisHP:0000939
  • Sleep disturbanceHP:0002360
  • SplenomegalyHP:0001744
  • SteatorrheaHP:0002570

Very rare (<4-1%)(1)

  • Gastrointestinal inflammationHP:0004386