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Chronic intestinal pseudoobstruction


Información en español Title


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Other Names:
CIPO
This disease is grouped under:
ACTG2-related disorders

Summary Summary


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Chronic intestinal pseudo-obstruction (CIPO) is a rare disease characterized by repetitive episodes or continuous symptoms of bowel obstruction when no blockage exists. Problems with nerves, muscles, or interstitial cells of Cajal (the cells that set the pace of intestinal contractions) prevent normal contractions and cause problems with the movement of food, fluid, and air through the intestines. The most common symptoms are abdominal swelling or bloating (distention), vomiting, abdominal pain, failure to thrive, diarrhea, constipation, feeding intolerance and urinary symptoms. CIPO can occur in people of any age. It may be primary or secondary. Primary or idiopathic (where the cause is unknown) CIPO occurs by itself. Secondary CIPO develops as a complication of another medical condition. In some people with CIPO, the condition is caused by variations (mutations) affecting the FLNA or ACTG2 gene. Before making the diagnosis other conditions with similar symptoms should be ruled out.[1][2][3] 

Treatment aims to restore the normal bowel movements and to correct nutritional deficiencies. Treatment may include antibiotics, prokinetic medications (metoclopramide, cisapride), surgical excision of intestinal segments in cases of localized disease, and parenteral nutrition. Intestinal transplantation has been successful in some cases. Several specialists may be needed for better management of the disease.[4]
Last updated: 3/28/2018

Symptoms Symptoms


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This table lists symptoms that people with this disease may have. For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. This information comes from a database called the Human Phenotype Ontology (HPO) . The HPO collects information on symptoms that have been described in medical resources. The HPO is updated regularly. Use the HPO ID to access more in-depth information about a symptom.

Showing of 5 |
Medical Terms Other Names
Learn More:
HPO ID
30%-79% of people have these symptoms
Abnormal nervous system morphology
Abnormal shape of nervous system
0012639
Intestinal malrotation 0002566
Pyloric stenosis 0002021
5%-29% of people have these symptoms
Abnormal platelet morphology
Abnormal shape of platelets
0011875
Patent ductus arteriosus 0001643
Showing of 5 |
Do you have more information about symptoms of this disease? We want to hear from you.
Last updated: 2/1/2021
Do you have updated information on this disease? We want to hear from you.

Organizations Organizations


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Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Organizations Supporting this Disease

  • Gastroparesis Patient Association for Cures and Treatments, Inc. (G-PACT)
    185-132 Newberry Commons
    Etters, PA 17319
    Toll-free: 1-888-874-7228
    E-mail: contact@g-pact.org
    Website: https://www.g-pact.org/

Organizations Providing General Support

  • Association of Gastrointestinal Motility Disorders (AGMD)
    140 Pleasant Street
    Lexington, MA 02421
    Telephone: +1-781-275-1300
    E-mail: info@agmdhope.org
    Website: https://www.agmdhope.org/
  • International Foundation for Functional Gastrointestinal Disorders (IFFGD)
    3015 Dunes West Blvd. Suite 512
    Mount Pleasant, SC 29466
    Telephone: +1-414-964-1799
    Website: https://iffgd.org/
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Learn More Learn More


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These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

Where to Start

  • MedlinePlus was designed by the National Library of Medicine to help you research your health questions, and it provides more information about this topic.
  • Genetics Home Reference (GHR) contains information on Chronic intestinal pseudoobstruction. This website is maintained by the National Library of Medicine.
  • The National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK) provides information on this topic.
  • The National Organization for Rare Disorders (NORD) has a report for patients and families about this condition. NORD is a patient advocacy organization for individuals with rare diseases and the organizations that serve them.

In-Depth Information

  • Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
  • The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
  • Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.

News & Events News & Events


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News

  • Rare Disease Day at NIH 2021
    March 1, 2021

GARD Answers GARD Answers


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Questions sent to GARD may be posted here if the information could be helpful to others. We remove all identifying information when posting a question to protect your privacy. If you do not want your question posted, please let us know.

Have a question? Contact a GARD Information Specialist.

References References


  1. Gfroerer S & Rolle U. Pediatric intestinal motility disorders. World J Gastroenterol. September 7, 2015; 21(33):9683–9687. http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4562951/.
  2. Carnillereri M & Friedman LS. Chronic intestinal pseudo-obstruction. UpToDate. 2016; http://www.uptodate.com/contents/topic.do?topicKey=GAST/2639.
  3. Intestinal Pseudo-obstruction. National Institute of Diabetes and Digestive and Kidney Diseases. February 26, 2014.; http://www.niddk.nih.gov/health-information/health-topics/digestive-diseases/intestinal-pseudo-obstruction/Pages/facts.aspx#sup1.
  4. Chronic intestinal pseudoobstruction. Orphanet. 2007; http://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=2978.
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