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Autoimmune pulmonary alveolar proteinosis

ORPHA:747 · Disease · Disorder

HPO 表現型（共 20 項）

Very frequent (99-80%)(1)

  • Intraalveolar phospholipid accumulationHP:0006517

Frequent (79-30%)(11)

  • Abnormal circulating protein concentrationHP:0010876
  • Abnormality of the upper respiratory tractHP:0002087
  • Autoimmune antibody positivityHP:0030057
  • ClubbingHP:0001217
  • CyanosisHP:0000961
  • Decreased DLCOHP:0045051
  • DyspneaHP:0002094
  • Foam cellsHP:0003651
  • HypoxemiaHP:0012418
  • Increased circulating lactate dehydrogenase concentrationHP:0025435
  • Restrictive ventilatory defectHP:0002091

Occasional (29-5%)(3)

  • CoughHP:0012735
  • CracklesHP:0030830
  • Crazy paving patternHP:0025391

Very rare (<4-1%)(5)

  • Chest painHP:0100749
  • FatigueHP:0012378
  • FeverHP:0001945
  • HemoptysisHP:0002105
  • Weight lossHP:0001824