Вроджена вада розвитку легеневих дихальних шляхів
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