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Congenital pulmonary airway malformation type 0

MedGen UID:
1776488
•Concept ID:
C5437764
•
Congenital Abnormality
Synonyms: Congenital cystic adenomatoid malformation of the lung type 0; congenital cystic adenomatoid malformation of the lung type 0; Congenital cystic adenomatous malformation of the lung type 0; congenital cystic adenomatous malformation of the lung type 0; congenital pulmonary airway malformation type 0; CPAM type 0
SNOMED CT: Congenital pulmonary airway malformation type 0 (890394003)
 
Monarch Initiative: MONDO:0017248
Orphanet: ORPHA280827

Definition

Congenital pulmonary airway malformation type 0 originates in the trachea or bronchi with acinar dysgenesis, cartilage, smooth muscles, and glands separated by mesenchyme. It is fatal after birth. [from SNOMEDCT_US]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVCongenital pulmonary airway malformation type 0

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