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

More general terms

• genetic disease of the gastrointestinal tract

Database

• OMIM: https://mirror.omim.org/entry/300048