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Short Bowel Syndrome

Abstract

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NORD is very grateful to Jon A. Vanderhoof, MD, Pediatric Gastroenterologist, Boys Town National Research Hospital, and Rosemary Pauley-Hunter, APRN, MS, CCRP, Pediatric Gastroenterology Nurse Practitioner, Boys Town National Research Hospital, for assistance in the preparation of this report.

Synonyms of Short Bowel Syndrome

  • SBS

Disorder Subdivisions

  • No subdivisions found.

General Discussion

Summary
Short bowel syndrome is a complex disease that occurs due to the physical loss or the loss of function of a portion of the small and/or large intestine. Consequently, individuals with short bowel syndrome often have a reduced ability to absorb nutrients such as fats, carbohydrates (sugars) vitamins, minerals, trace elements and fluids (malabsorption). The specific symptoms and severity of short bowel syndrome vary from one person to another. Diarrhea is common, often severe and can cause dehydration, which can even be life threatening. Short bowel syndrome can lead to malnutrition, unintended weight loss and additional symptoms may be due to the loss of essential vitamins and minerals. There is no cure, but the disorder usually can be treated effectively. However, in severe cases, short bowel syndrome can lead to severe, disabling and life-threatening complications. Short bowel syndrome is most commonly associated with the surgical removal (resection) of half or more of the small intestine. Such surgery is performed to treat intestinal diseases such as Crohn's disease, injury or trauma to the small bowel, or congenital birth defects. The presence or absence of the large intestine (colon) also plays an important role in the genesis and/or treatment of the short bowel syndrome.

Introduction
Through the years, the definition of short bowel syndrome in the medical literature has varied. This has led to confusion. Although some medical sources seem to reserve the name short bowel syndrome for cases caused by surgical resection of a portion of the small intestine, other sources have noted that the disorder can result from any disease, injury or condition that hinders or prevents the proper function of the small intestine even if the length of the bowel is unaffected. Short bowel syndrome may be classified as a cause or subcategory of intestinal failure. In rare cases, infants are born with a short bowel (congenital short bowel syndrome). Although these congenital cases are often associated with malrotation of the small intestine, the exact cause of congenital short bowel syndrome is unknown.

Short Bowel Syndrome Resources

Please note that some of these organizations may provide information concerning certain conditions potentially associated with this disorder.

NORD Member Organizations:

(To become a member of NORD, an organization must meet established criteria and be approved by the NORD Board of Directors. If you're interested in becoming a member, please contact Susan Olivo, Membership Manager, at solivo@rarediseases.org.)

Other Organizations:

The information in NORD’s Rare Disease Database is for educational purposes only. It should never be used for diagnostic or treatment purposes. If you have questions regarding a medical condition, always seek the advice of your physician or other qualified health professional. NORD’s reports provide a brief overview of rare diseases. For more specific information, we encourage you to contact your personal physician or the agencies listed as “Resources” on this report.

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