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Primary sclerosing cholangitis

ORPHA:171 · Disease · Disorder

HPO 表現型（共 55 項）

Very frequent (99-80%)(3)

  • Abnormal biliary tract morphologyHP:0012440
  • AutoimmunityHP:0002960
  • CholestasisHP:0001396

Frequent (79-30%)(16)

  • Abnormal large intestine physiologyHP:0012700
  • AscitesHP:0001541
  • CirrhosisHP:0001394
  • Dilated superficial abdominal veinsHP:0030168
  • Elevated alkaline phosphatase of hepatic originHP:0010638
  • Elevated circulating hepatic transaminase concentrationHP:0002910
  • FeverHP:0001945
  • Hepatic fibrosisHP:0001395
  • HepatomegalyHP:0002240
  • HepatosplenomegalyHP:0001433
  • Palmar telangiectasiaHP:0100869
  • Portal hypertensionHP:0001409
  • Spider hemangiomaHP:0012522
  • SplenomegalyHP:0001744
  • Ulcerative colitisHP:0100279
  • Weight lossHP:0001824

Occasional (29-5%)(28)

  • Abdominal painHP:0002027
  • Adenocarcinoma of the large intestineHP:0040275
  • Celiac diseaseHP:0002608
  • CholangiocarcinomaHP:0030153
  • CholelithiasisHP:0001081
  • Chronic hepatic failureHP:0100626
  • Congestive heart failureHP:0001635
  • Decreased circulating vitamin D concentrationHP:0100512
  • Decreased circulating vitamin E concentrationHP:0100513
  • Decreased circulating vitamin K concentrationHP:0011892
  • DepressionHP:0000716
  • FatigueHP:0012378
  • Generalized amyotrophyHP:0003700
  • HepatitisHP:0012115
  • Hepatocellular carcinomaHP:0001402
  • HypoalbuminemiaHP:0003073
  • JaundiceHP:0000952
  • OsteopeniaHP:0000938
  • OsteoporosisHP:0000939
  • PancreatitisHP:0001733
  • Pleural effusionHP:0002202
  • Polyclonal elevation of circulating IgM concentrationHP:0003459
  • Prolonged prothrombin timeHP:0008151
  • PruritusHP:0000989
  • Reduced circulating vitamin A concentrationHP:0004905
  • Renal insufficiencyHP:0000083
  • ThyroiditisHP:0100646
  • Type I diabetes mellitusHP:0100651

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

  • Acute hepatic failureHP:0006554
  • EncephalopathyHP:0001298
  • Neoplasm of the gallbladderHP:0100575
  • UveitisHP:0000554

Excluded (0%)(4)

  • Abnormal eosinophil morphologyHP:0001879
  • Amyloid depositionHP:0011034
  • HistiocytosisHP:0100727
  • Recurrent systemic pyogenic infectionsHP:0005429