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X-linked congenital idiopathic intestinal pseudoobstruction (CIIPX)
Pathology:
- primary qualitative defects of enteric ganglia & nerve fibers
Genetics:
- associated with defects in FLNA
Clinical manifestations:
- severe abnormality of gastrointestinal motility
- affected individuals manifest recurrent signs of intestinal obstruction in the absence of any mechanical lesion
General
genetic disease of the gastrointestinal tract
Database Correlations
OMIM 300048
References
OMIM :accession 300048