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Short bowel syndrome (NORD)



Patient care

Information for patients and families

The Primary School
Glut1 Deficiency Foundation
The National Organization for Rare Disorders (NORD)
Narcolepsy (NORD)
Adrenoleukodystrophy (NORD)
Zellweger spectrum disorders (NORD)
Glycogen storage disease type II (NORD)
Metachromatic leukodystrophy (NORD)
Gaucher disease (NORD)
Niemann-Pick disease types A and B (NORD)
Fabry disease (NORD)
Tay-Sachs disease (NORD)
Mucopolysaccharide storage disease type 1 (Hurler syndrome) (NORD)
Mucopolysaccharide storage disease type 2 (Hunter syndrome) (NORD)
Phenylketonuria (NORD)
Cystinuria (NORD)
Aromatic L-amino acid decarboxylase deficiency (NORD)
Cerebrotendinous xanthomatosis (NORD)
Classical homocystinuria (NORD)
Autoimmune polyglandular syndrome type 1 (NORD)
Thyroid eye disease (NORD)
Opsoclonus myoclonus syndrome (NORD)
Thyroid eye disease (NORD)
Short bowel syndrome (NORD)
Bile synthesis disorders (NORD)
Eosinophilic esophagitis (NORD)
Cyclic vomiting syndrome (NORD)
Polycythemia vera (NORD)
Essential thrombocythemia (NORD)
Myelofibrosis (NORD)
Warm autoimmune hemolytic anemia and cold agglutinin (NORD)
Sickle cell disease (NORD)
Waldenstrom macroglobulinemia (NORD)
Post-transplant lymphoproliferative disorders (NORD)
Cytomegalovirus infection after transplant (NORD)
Congenital athymia (NORD)
Severe chronic neutropenia (NORD)
Mycobacterium avium complex (NORD)
Congenital cytomegalovirus (NORD)
Opsoclonus myoclonus syndrome (NORD)
Spinocerebellar ataxia (NORD)
Early infantile epileptic encephalopathy (NORD)
IgA nephropathy (NORD)
Focal segmental glomerulosclerosis (NORD)
Alagille syndrome (NORD)
NGLY1 deficiency (NORD)
PIK3CA-related overgrowth spectrum (NORD)
Recurrent pericarditis (NORD)
Gorlin Syndrome Alliance

Content Reviewers:

Rishi Desai, MD, MPH

In short bowel syndrome, bowel is another word for the intestines.

It’s a condition that occurs when either the small intestine and/or the large intestine become physically shorter when a portion is removed by surgery, or functionally shorter, when a portion is damaged in a way that makes it nonfunctional.

This can lead to poor absorption of water and vital nutrients from food.

Normally, digestion begins when food is chewed and travels into the stomach where it’s dissolved by stomach acid, enzymes, and physical churning.

Then, it’s sent to the first portion of the small intestine, which is called the duodenum.

This is where more enzymes are added from the pancreas to digest macronutrients, including proteins, carbohydrates, and fats, while bile is added from the liver and gallbladder to help absorb fats.

It’s also where the absorption of some minerals like calcium, iron, and magnesium begins.

The next section is called the jejunum, and it has long projections on its surface, giving it a large surface area for absorption.

It plays the biggest role in the digestion and absorption of most nutrients, including the breakdown-products of macronutrients, zinc, water-soluble vitamins, and fat soluble vitamins, namely A, D, E, and K.

The third section is called the ileum, and it has tight intercellular junctions, allowing it to efficiently absorb fluid and begin concentrating the intestinal contents.

Unlike the jejunum, the ileum is also capable of undergoing structural and functional adaptations to compensate for the jejunum if needed.

The final portion is called the terminal ileum, and it absorbs vitamin B12 and bile salts which are recycled.

It ends with the ileocecal valve, which prevents intestinal contents from going into the large intestine too quickly and the backward flow of material.

The large intestine functions to absorb water, electrolytes like sodium and potassium, short-chain fatty acids, and bacteria within it produce vitamin K.

Since each part of the small intestine has a specialized function, as does the large intestine, the manifestations of short bowel syndrome depend on which portion is no longer functioning normally.

Short bowel syndrome manifests if there are less than 100 centimeters of functioning intestine remaining, and is a malabsorption disorder caused by the surgical removal or complete dysfunction of a large segment of bowel. Problems include production of a large amount of liquid stool, steatorrhea, malnutrition, loss of electrolytes, vitamins, and minerals, and weight loss. Diagnosis is usually apparent; patients with Crohn's disease tend to be most commonly affected. Management is with total parenteral nutrition in the early stages, then vitamin and mineral replacement. Prolonged parenteral fluid support may be required. Surgical procedure are a last resort, and include transplant and non-transplant options.