Short bowel syndrome

From WikiMD

Short bowel syndrome is a disorder characterized by malabsorption of nutrients due to problems involving the small intestine. Short bowel syndrome may be mild, moderate, or severe, depending on how well the small intestine is working.

People with short bowel syndrome cannot absorb enough water, vitamins, minerals, protein, fat, calories, and other nutrients from food. What nutrients the small intestine has trouble absorbing depends on which section of the small intestine has been damaged or removed.

Risk factors

Short bowel syndrome typically occurs in people who have

  • had at least half of their small intestine removed and sometimes all or part of their large intestine removed
  • significant damage of the small intestine
  • poor motility, or movement, inside the intestines.

Cause

The main cause of short bowel syndrome is surgery to remove a portion of the small intestine. This surgery can treat intestinal diseases, injuries, or birth defects.

Some children are born with an abnormally short small intestine or with part of their bowel missing, which can cause short bowel syndrome. In infants, short bowel syndrome most commonly occurs following surgery to treat necrotizing enterocolitis, a condition in which part of the tissue in the intestines is destroyed. Short bowel syndrome may also occur following surgery to treat conditions such as

  • cancer and damage to the intestines caused by cancer treatment
  • Crohn's disease, a disorder that causes inflammation, or swelling, and irritation of any part of the digestive tract
  • gastroschisis, which occurs when the intestines stick out of the body through one side of the umbilical cord
  • internal hernia, which occurs when the small intestine is displaced into pockets in the abdominal lining
  • intestinal atresia, which occurs when a part of the intestines doesn't form completely
  • intestinal injury from loss of blood flow due to a blocked blood vessel
  • intestinal injury from trauma
  • intussusception, in which one section of either the large or small intestine folds into itself, much like a collapsible telescope
  • meconium ileus, which occurs when the meconium, a newborn's first stool, is thicker and stickier than normal and blocks the ileum
  • midgut volvulus, which occurs when blood supply to the middle of the small intestine is completely cut off
  • omphalocele, which occurs when the intestines, liver, or other organs stick out through the navel, or belly button

Even if a person does not have surgery, disease or injury can damage the small intestine.

Signs and symptoms

The main symptom of short bowel syndrome is diarrhea—loose, watery stools. Diarrhea can lead to dehydration, malnutrition, and weight loss. Other signs and symptoms may include

  • bloating
  • cramping
  • fatigue, or feeling tired
  • foul-smelling stool
  • heartburn
  • too much gas
  • vomiting
  • weakness

People with short bowel syndrome are also more likely to develop food allergies and sensitivities, such as lactose intolerance. Lactose intolerance is a condition in which people have digestive symptoms—such as bloating, diarrhea, and gas—after eating or drinking milk or milk products.

Diagnosis

A health care provider diagnoses short bowel syndrome based on

A medical and family history:Taking a medical and family history may help a health care provider diagnose short bowel syndrome. He or she will ask the patient about symptoms and may request a history of past operations. A physical exam:A physical exam may help diagnose short bowel syndrome. During a physical exam, a health care provider usually

examines a patient's body, looking for muscle wasting or weight loss and signs of vitamin and mineral deficiencies uses a stethoscope to listen to sounds in the abdomen taps on specific areas of the patient's body

Blood tests:Blood tests can show mineral and vitamin levels and measure complete blood count.

Fecal fat tests:A fecal fat test measures the body's ability to break down and absorb fat. For this test, a patient provides a stool sample at a health care provider's office.

An x-ray of the small and large intestines:An x-ray is a picture created by using radiation and recorded on film or on a computer. The amount of radiation used is small.

Upper gastrointestinal (GI) series:Upper GI series, also called a barium swallow, uses x rays and fluoroscopy to help diagnose problems of the upper GI tract. Fluoroscopy is a form of x ray that makes it possible to see the internal organs and their motion on a video monitor

Computerized tomography (CT) scan:Computerized tomography scans use a combination of x-rays and computer technology to create images.

Treatment

Treatment may include

  • nutritional support
  • medications
  • surgery
  • intestinal transplant

Nutritional Support

The main treatment for short bowel syndrome is nutritional support, which may include the following:

Oral rehydration: Adults should drink water, sports drinks, sodas without caffeine, and salty broths. Children should drink oral rehydration solutions—special drinks that contain salts and minerals to prevent dehydration—such as Pedialyte, Naturalyte, Infalyte, and CeraLyte, which are sold in most grocery stores and drugstores. Parenteral nutrition:This treatment delivers fluids, electrolytes, and liquid vitamins and minerals into the bloodstream through an intravenous (IV) tube—a tube placed into a vein. Health care providers give parenteral nutrition to people who cannot or should not get their nutrition or enough fluids through eating. Enteral nutritionl: This treatment delivers liquid food to the stomach or small intestine through a feeding tube—a small, soft, plastic tube placed through the nose or mouth into the stomach. Gallstones—small, pebblelike substances that develop in the gallbladder—are a complication of enteral nutrition. Vitamin and mineral supplements: A person may need to take vitamin and mineral supplements during or after parenteral or enteral nutrition. Special diet: A health care provider can recommend a specific diet plan for the patient that may include small, frequent feedings avoiding foods that can cause diarrhea, such as foods high in sugar, protein, and fiber avoiding high-fat foods

Medications

A health care provider may prescribe medications to treat short bowel syndrome, including

Surgery

The goal of surgery is to increase the small intestine's ability to absorb nutrients. Approximately half of the patients with short bowel syndrome need surgery.

Intestinal Transplant

An intestinal transplant is surgery to remove a diseased or an injured small intestine and replace it with a healthy small intestine from a person who has just died, called a donor. Sometimes a living donor can provide a segment of his or her small intestine.

The medication(s) listed below have been approved by the Food and Drug Administration (FDA) as orphan products for treatment of this condition.

  • Teduglutide [rDNA origin] (Brand name: Gattex)Treatment of adult patients with short bowel syndrome (SBS) who are dependent on parenteral support
  • Glutamine (Brand name: NutreStore™) Treatment of short bowel syndrome in patients receiving specialized nutritional support when used in conjunction with a rercombinant human growth hormone that is approved for this indication.
  • Follitropin alfa, recombinant (Brand name: Zorbtive)Treatment of short bowel syndrome in patients receiving specialized nutritional support.

Epidemiology

Short bowel syndrome is a rare condition. Each year, short bowel syndrome affects about three out of every million people.

Prognosis

There is no cure for short bowel syndrome except transplant. 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%.

NIH genetic and rare disease info

Short bowel syndrome is a rare disease.

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