congenital idiopathic intestinal pseudo-obstruction
CIIP, pediatric intestinal pseudo-obstruction, IPO
Chronic idiopathic intestinal pseudoobstruction (CIIP) is caused by severe abnormality of gastrointestinal motility.
Etiology
primary CIIP (defects of enteric neuronal cells)
- autosomal recessive inheritance
- syndrome associating short small bowel, malrotation, and pyloric hypertrophy associated with morphologic defects of argyrophil neurons in the myenteric plexus (MIM.243180)
- X-linked inheritance
- L1CAM germline mutations at Xq28
secondary CIIP
Associations
hydrocephalus with stenosis of the aqueduct of Sylvius (HSAS) (15368500)
References
Park SH, Min H, Chi JG, Park KW, Yang HR, Seo JK. Immunohistochemical studies of pediatric intestinal pseudo-obstruction: bcl2, a valuable biomarker to detect immature enteric ganglion cells. Am J Surg Pathol. 2005 Aug;29(8):1017-24. PMID: 16006795