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idiopathic congenital intestinal pseudo-obstruction
Monday 4 April 2005
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
- Chagas disease
- myxedema
- Duchenne muscular dystrophy
Associations
hydrocephalus with stenosis of the aqueduct of Sylvius (HSAS) (15368500)
- germline mutations of the L1CAM coding for L1 cell adhesion molecule at Xq28 (15368500)
References
Normal oxidative phosphorylation in intestinal smooth muscle of childhood chronic intestinal pseudo-obstruction. Galmiche L, Jaubert F, Sauvat F, Sarnacki S, Goulet O, Assouline Z, Vedrenne V, Lebre AS, Boddaert N, Brousse N, Chrétien D, Munnich A, Rötig A. Neurogastroenterol Motil. 2011 Jan;23(1):24-e1. PMID: 20879992
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
