Description of the disease * EnglishPolish Pobierz sekcję do PDF Definition A rare subtype of congenital pulmonary airway malformation characterized by global arrest of lung development with small, solid appearing lungs with a diffusely granular surface, histologically featuring bronchus-like structures with smooth muscle, glands, and numerous cartilage plates, embedded in loose, vascular mesenchymal tissue. The condition presents at birth and is incompatible with life. Disease data Classification Clinical subtype Synonyms CPAM type 0 CPAM typu 0 Wrodzona torbielowatość gruczolakowata płuc typu 0 Congenital cystic adenomatoid malformation of the lung type 0 Congenital cystic adenomatous malformation of the lung type 0 ORPHA code 280827 OMIM code - ICD10 code Q33.0 ICD11 code - *Soruce Extended description of the disease Pobierz sekcję do PDF No additional description. Orphanet - interntowa baza danych dotyczących rzadkich chorób i sierochych leków. ©INSERM 1999 - Dostępna na stronie www.orphanet.pl