Congenital pulmonary airway malformation

Orpha code: 2444OMIM code:

Definition

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.

Disease data
Classification

Malformation syndrome

Synonyms
CCAM
CCAM
CPAM
Congenital cystic adenomatoid malformation of the lung
Congenital cystic adenomatous malformation of the lung
Congenital cystic disease of the lung
ORPHA code
2444
OMIM code
-
ICD10 code
Q33.0
ICD11 code
LA75.4

No additional description.

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