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ORPHA:2444· ICD-10 Q33.0· Congenital pulmonary airway malformation

Визначення(English summary)

A rare respiratory malformation characterized by a hamartomatous mass of non-functioning lung tissue of variable extent and with variable degrees of cystic or adenomatoid change. Clinical presentation, prognosis, and presence of associated abnormalities depend on the subtype of the lesion. Based on histopathological findings, five subtypes (types 0 to 4) can be differentiated.

Поширеність
1-9 / 100 000
Успадкування
Not applicable
Вік початку
Adolescent, Adult, Antenatal, Neonatal