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1.(MeSH)A malabsorption syndrome resulting from extensive operative resection of the SMALL INTESTINE, the absorptive region of the GASTROINTESTINAL TRACT.
Short Bowel Syndrome (n.) [MeSH]
|Short bowel syndrome|
|Classification and external resources|
Short bowel syndrome (SBS, also short gut syndrome or simply short gut) is a malabsorption disorder caused by the surgical removal of the small intestine, or rarely due to the complete dysfunction of a large segment of bowel. Most cases are acquired, although some children are born with a congenital short bowel. It usually does not develop unless more than two thirds of the small intestine have been removed.
The symptoms of short bowel syndrome can include:
Patients with short bowel syndrome may have complications caused by malabsorption of vitamins and minerals, such as deficiencies in vitamins A, D, E, K, and B12, calcium, magnesium, iron, folic acid, and zinc. These may appear as anemia, hyperkeratosis (scaling of the skin), easy bruising, muscle spasms, poor blood clotting, and bone pain.
Short bowel syndrome in adults is usually caused by surgery for:
In healthy adults, the small intestine has an average length of approximately 6 meters (19.7 feet). Short bowel syndrome usually develops when there is less than 2 meters (6.6 feet) of the small intestine left to absorb sufficient nutrients.
Short bowel syndrome caused by the surgical removal of a portion of the bowel may be a temporary condition, due to the adaptive property of the small intestine.
In a process called intestinal adaptation, physiological changes to the remaining portion of the small intestine occur to increase its absorptive capacity. These changes include:
Symptoms of short bowel syndrome are usually addressed by prescription medicine. These include:
In 2004, the FDA approved a therapy that reduces the frequency and volume of TPN, comprising:
NutreStoreTM [L-glutamine for oral solution] and Zorbtive [somatropin (rDNA origin) for injection] together with a specialized oral diet. Byrne, TA, Wilmore DW, Iyer K, et al. Growth hormone, glutamine, and an optimal diet reduces parenteral nutrition in patients with short bowel syndrome. Ann Surg. 2005;242;655-661
Surgical procedures to lengthen dilated bowel include the Bianchi procedure (where the bowel is cut in half and one end is sewn to the other) and a newer procedure called serial transverse enteroplasty (STEP—where the bowel is cut and stapled in a zigzag pattern). Heung Bae Kim, MD, and Tom Jaksic, MD, both of Children's Hospital Boston, devised the STEP procedure in the early 2000s. The procedure lengthens the bowel of children with SBS and may allow children to avoid the need for intestinal transplantation. As of June 2009, Kim and Jaksic have performed 18 STEP procedures.
The Bianchi and STEP procedures are usually performed by pediatric surgeons at quaternary hospitals who specialize in small bowel surgery.
There is no cure for short bowel syndrome. In newborn infants, the 4-year survival rate on parenteral nutrition is approximately 70%. In newborn infants with less than 10% of expected intestinal length, 5 year survival is approximately 20%. Some studies suggest that much of the mortality is due to a complication of the TPN, especially chronic liver disease. Much hope is vested in Omegaven, a type of lipid TPN feed, in which recent case reports suggest the risk of liver disease is much lower.
Although promising, small intestine transplant has a mixed success rate, with postoperative mortality rate of up to 30%. One-year and 4-year survival rate are 90% and 60%, respectively.