| Short bowel syndrome | |
|---|---|
| Other names | Short gut syndrome, short gut, intestinal failure |
 | |
| A piece of diseasedileum following removal by surgery. | |
| Specialty | Gastroenterology |
| Symptoms | Diarrhea,dehydration,malnutrition,weight loss[1] |
| Complications | Anemia,kidney stones[2] |
| Causes | Surgical removal of a large portion of thesmall intestine[1] |
| Risk factors | Crohn's disease,necrotising enterocolitis[2] |
| Treatment | Specific diet, medications, surgery[1] |
| Medication | Antibiotics,antacids,loperamide,teduglutide,growth hormone[1] |
| Prognosis | Depends on amount of small bowel remaining[2] |
| Frequency | 3 per million per year, in which is a rare disorder[1] |
Short bowel syndrome (SBS, or simplyshort gut) is a raremalabsorption disorder caused by a lack of functionalsmall intestine.[3] The primary symptom isdiarrhea, which can result indehydration,malnutrition, andweight loss.[1] Other symptoms may include bloating,heartburn, feeling tired,lactose intolerance, and foul-smelling stool.[1] Complications can includeanemia andkidney stones.[2]
Most cases are due to the surgical removal of a large portion of the small intestine.[1] This is most often required due toCrohn's disease in adults andnecrotising enterocolitis in young children.[2] A recent national study showed the prevalence of SBS was 1% among patients with Crohn's disease.[4] Other causes include damage to the small intestine from other means and being born with an abnormally short intestine.[1] It usually does not develop until less than 2 m (6.6 ft) of the normally 6.1 m (20 ft) small intestine remains.[1][3]
Treatment may include a specific diet, medications, or surgery.[1] The diet may includeslightly salty and slightly sweet liquids, vitamin and mineral supplements, small frequent meals, and the avoidance of high fat food.[1] Occasionally, nutrients need to be given through anintravenous line, known asparenteral nutrition.[1] Medications used may includeantibiotics,antacids,loperamide,teduglutide, andgrowth hormone.[1] The success rate ofTeduglutide, defined as at least a 30% reduction inParenteral nutrition, exceeded 50% of treated patients.[5]Different types of surgery, including anintestinal transplant, may help some people.[1]
Short bowel syndrome newly occurs in about three per million people each year.[1] There are estimated to be about 15,000 people with the condition in the United States.[2] The prevalence in the United States is approximately 30 cases per million and in Europe it is approximately 1.4 cases per million (but the rate varies widely between countries).[6] The prevalence of short bowel syndrome has increased by more than 2 fold in the last 40 years.[6] It is classified as arare disease by theEuropean Medicines Agency.[7] Outcomes depend on the amount of bowel remaining and whether or not the small bowel remains connected with the large bowel.[2]
The symptoms of short bowel syndrome can include:[8][9]
Persons with short bowel syndrome may have complications caused by malabsorption of vitamins and minerals, such asdeficiencies in vitaminsA,D,E,K,B9 (folic acid), andB12,calcium,magnesium,iron, andzinc. These may appear asanemia,hyperkeratosis (scaling of the skin), easybruising, musclespasms, poorblood clotting, andbone pain.[citation needed]
Short bowel syndrome in adults and children is most commonly caused by surgery (intestinal resection).[6] In those who undergo intestinal resection, approximately 15% eventually develop small bowel syndrome (75% of those due to 1 large resection and 25% due to multiple separate intestinal resections).[6] This surgery may be done for:
Some children are also born with an abnormally short small intestine, known as congenital short bowel.[1]
Surgical complications, requiring re-surgery, are a common cause of small bowel syndrome, contributing up to 50% of cases based on some estimates.[6] These surgical complications includeinternal hernias,volvuli,ischemia orprofound hypotension.[6]
The length of the small intestine can vary greatly, from as short as 2.75 m (9.0 ft) to as long as 10.49 m (34.4 ft).[10] On average it is about 6.1 m (20 ft).[1] Due to this variation it is recommended that following surgery the amount of bowel remaining be specified rather than the amount removed.[10]
Short bowel syndrome usually develops when there is less than 2 meters (6.6 feet) of the small intestine left to absorb sufficientnutrients.[citation needed]
The resection of specific areas of the small bowel can lead to distinct symptoms in short bowel syndrome. The resection of the ileum leads to a malabsorption ofvitamin B12,bile acids and the fat soluble vitaminsA,D,E andK.[6] Loss of the distal ileum also leads to loss of inhibitory hormones; leading to gastric hypersecretion, intestinal hypermotility (decreases in the intestinal transit time) leading to secretory diarrhea and macronutrient, micronutrient, vitamin and mineral deficiencies.[6] Loss of theileocecal valve leads tosmall intestinal bacterial overgrowth (SIBO) as bacterial flora normally found in the large intestines migrate proximally and colonize the small intestines leading to further malabsorption.[6] SIBO leads to malabsorption as the bacteria colonizing the small intestine metabolize nutrients, directly competing with the intestinal absorption of nutrients. The bacteria colonizing the small intestines in SIBO may also cause bile acid deconjugation leading to malabsorption of lipids.[6]
In a process called intestinal adaptation, physiological changes to the remaining portion of the small intestine occur to increase its absorptive capacity. These changes usually take place over 1–2 years.[11] These changes include:
Osteoporosis is a very common comorbidity in people with short bowel syndrome who are on parenteral nutrition, with an estimated prevalence of 57-67%.[6] The contributing factors to the osteoporosis include malnutrition, vitamin D deficiency due to malabsorption and vitamin D deficiency due to scarce sunlight exposure due to chronic disability.[6]
Intestinal failure is decreased intestinal function such that nutrients, water, and electrolytes are not sufficiently absorbed. Short bowel syndrome is when there is less than 2 m (6.6 ft) of working bowel and is the most common cause of intestinal failure.[3]
Symptoms of short bowel syndrome are usually addressed with medication. These include:[citation needed]
In 2004, theUSFDA approved a therapy that reduces the frequency and volume oftotal parenteral nutrition (TPN), comprising:NutreStore (oral solution of glutamine) and Zorbtive (growth hormone, ofrecombinant DNA origin, for injection) together with a specialized oral diet.[12] After 24 weeks of successful Phase III patient treatment trials, Teduglutide was shown to be relatively safe and effective with varying degrees of benefits and adverse effects per patient.[13] Adequate safety evaluations prove to be difficult due to a limited sample size available for study, however.[13] In 2012, an advisory panel to the USFDA voted unanimously to approve for treatment of SBS the agentteduglutide, aglucagon-like peptide-2 analog developed byNPS Pharmaceuticals, who intend to market the agent in the United States under the brandname Gattex.[14] Teduglutide had been previously approved for use in Europe and is marketed under the brand Revestive byNycomed.[14]
Antiperistaltic transverse coloplasty, proposed as aprophylactic measure against short bowel syndrome, retains transverse colon function after extensive colectomies, promoting improved stool consistency and quality of life. This technique involves repositioning the colon to simulate its original placement, potentially averting short bowel syndrome-related complications and benefiting patient outcomes.[15]
Surgical procedures to lengthen dilated bowel include theBianchi procedure, where the bowel is cut in half and one end is sewn to the other, and a newer procedure calledserial 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.[16] The Bianchi and STEP procedures are usually performed by pediatric surgeons atquaternary hospitals who specialize in small bowel surgery.[citation needed]
After resection, having a remnant small bowel length of less than 75 cm (30 in) and a remaining large bowel length of less than 57% of the original length are both associated with subsequent dependence on parenteral nutrition.[6] There is no cure for short bowel syndrome excepttransplant. 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%.[17] Some studies suggest that much of the mortality is due to a complication of the total parenteral nutrition (TPN), especiallychronic liver disease.[18] As of 2006, much hope has been vested inOmegaven, a type of lipid TPN feed, for which recent case reports suggest the risk of liver disease is much lower.[19]
Although promising,small intestine transplant has a mixed success rate, with a postoperative mortality rate of up to 30%. One-year and 4-year survival rates are 90% and 60%, respectively.[citation needed]
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