Orphanet: Wrodzona dysplazja naczyń pęcherzyków płucnych

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Congenital alveolar capillary dysplasia

Disease definition

Congenital alveolar capillary dysplasia (ACD) is a rare and fatal developmental lung disease characterized by respiratory distress in neonates due to refractory hypoxemia and severe pulmonary arterial hypertension.


Classification level: Disorder
  • Synonym(s):
    • ACDMPV
    • Alveolar capillary dysplasia with misalignment of pulmonary veins
    • Alveolar capillary dysplasia with misalignment of pulmonary vessels
  • Prevalence: <1 / 1 000 000
  • Inheritance: Autosomal dominant 
  • Age of onset: Infancy, Neonatal
  • ICD-10: -
  • OMIM: 265380
  • UMLS: C2677362
  • MeSH: -
  • GARD: 8644
  • MedDRA: -
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