congenital idiopathic intestinal pseudoobstruction, X-linked

congenital idiopathic intestinal pseudoobstruction, X-linked

An X-linked condition (OMIM:300048) characterised by severely compromised gastrointestinal motility due to qualitative defects of enteric ganglia and nerve fibres, resulting in recurrent intestinal pseudo-obstruction in the absence of detectable mechanical lesions.

Molecular pathology
Defects in FLNA cause X-linked congenital idiopathic intestinal pseudoobstruction.