出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2014/08/07 04:03:44」(JST)
| Short bowel syndrome | |
|---|---|
| Classification and external resources | |
| ICD-10 | K91.8 |
| ICD-9 | 579.3 |
| DiseasesDB | 12026 |
| MedlinePlus | 000237 |
| eMedicine | med/2746 ped/2088 |
| MeSH | D012778 |
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. SBS has been designated a rare disease by the EMA,[1] and is eligible for orphan drug designation according to the U.S. Food and Drug Administration (FDA).[2] There are roughly 3,500 adults and 9,000 children with SBS in the USA alone.
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 and children 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 USFDA approved a therapy that reduces the frequency and volume of TPN, comprising: NutreStore (oral solution of glutamine) and Zorbtive (growth hormone, of recombinant DNA origin, for injection) together with a specialized oral diet.[3] Eight years later, in 2012, an advisory panel to the USFDA voted unanimously to approve for treatment of SBS the agent teduglutide, a glucagon-like peptide-2 analog developed by NPS Pharmaceuticals, who intend to market the agent in the United States under the brandname Gattex.[4] Teduglutide had been previously approved for use in Europe and is marketed under the brand Revestive by Nycomed.[4]
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.[5]
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%.[6] Some studies suggest that much of the mortality is due to a complication of the TPN, especially chronic liver disease.[7] 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.[8]
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.
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| 関連記事 | 「short」「gut」「syndrome」「g」 |