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Diseases

Genetic and Rare Diseases Information Center (GARD)

Intestinal pseudoobstruction neuronal chronic idiopathic X-linked


Other Names for this Disease
  • Chronic intestinal pseudoobstruction
  • CIIP
  • CIIP X-linked
  • CIIPX
  • Congenital idiopathic intestinal pseudoobstruction
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Overview

Intestinal pseudo-obstruction is a condition characterized by impairment of the muscle contractions that move food through the digestive tract. The condition may arise from abnormalities of the gastrointestinal muscles themselves (myogenic) or from problems with the nerves that control the muscle contractions (neurogenic). When intestinal pseudo-obstruction occurs by itself, it is called primary or idiopathic (unknown cause) intestinal pseudo-obstruction. The disorder can also develop as a complication of another medical condition; in these cases, it is called secondary intestinal pseudo-obstruction. The affected people have symptoms that resemble those of an intestinal blockage (obstruction) but without any obstruction. It may be acute or chronic and is characterized by the presence of dilation of the bowel on imaging. The causes may be unknown or due to alterations (mutations) in the FLNA gene, other genes or are secondary to other conditions. It may be inherited in some cases. The intestinal pseudoobstruction neuronal chronic idiopathic X-linked is caused by alterations (mutations) in the FLNA gene which is located in the X chromosome.[1][2] There is no specific treatment but several medications and procedures may be used to treat the symptoms, depending also in the cause of this condition.[3]
Last updated: 7/17/2015

References

  1. Intestinal pseudo-obstruction. Genetics Home Reference. October, 2010; http://ghr.nlm.nih.gov/condition/intestinal-pseudo-obstruction#definition. Accessed 7/17/2015.
  2. Camilleri M. Chronic intestinal pseudo-obstruction. UpToDate. July 29, 2013; http://www.uptodate.com/contents/chronic-intestinal-pseudo-obstruction. Accessed 7/17/2015.
  3. Angkathunyakul N, Treepongkaruna S, Molagool S & Ruangwattanapaisarn N. Abnormal layering of muscularis propria as a cause of chronic intestinal pseudo-obstruction: A case report and literature review. World J Gastroenterol. June 14, 2015; 21(22):7059-7064. http://www-ncbi-nlm-nih-gov.ezproxy.nihlibrary.nih.gov/pmc/articles/PMC4462749/. Accessed 7/17/2015.
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Basic Information

  • MedlinePlus was designed by the National Library of Medicine to help you research your health questions, and it provides more information about this topic.

In Depth Information

  • Online Mendelian Inheritance in Man (OMIM) is a catalog of human genes and genetic disorders. Each entry has a summary of related medical articles. It is meant for health care professionals and researchers. OMIM is maintained by Johns Hopkins University School of Medicine. 
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Intestinal pseudoobstruction neuronal chronic idiopathic X-linked. Click on the link to view a sample search on this topic.
Other Names for this Disease
  • Chronic intestinal pseudoobstruction
  • CIIP
  • CIIP X-linked
  • CIIPX
  • Congenital idiopathic intestinal pseudoobstruction
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.