Congenital pulmonary airway malformation type 0

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Congenital pulmonary airway malformation type 0

Synonyms: CPAM type 0 | Congenital cystic adenomatoid malformation of the lung type 0 | Congenital cystic adenomatous malformation of the lung type 0

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.

Data from Orphanet are used to provide information on a disease's name, synonym(s), and overview.

Reference: Access aggregated data from Orphanet at Orphadata.

Orphadata: Free access data from Orphanet. © INSERM 1999. Available on http://www.orphadata.org. Data version March 2024

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Congenital pulmonary airway malformation type 0?

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