Small Bowel Disorders: Whipple's Disease and Short-Bowel Syndrome
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Small Bowel Disorders: Whipple's Disease and Short-Bowel Syndrome

WhippleÂ’s disease typically presents as fever of unknown origin, chronic diarrhea, and joint pain. In addition to causing malabsorption, WhippleÂ’s disease can affect a variety of organs including the brain. Short-bowel syndrome is characterized by malabsorption when less than 180 cm of functional small bowel remains.

Whipple’s Disease

• A very rare systemic infectious disease that mainly affects white men between age 40 and 50. The disease is caused by Tropheryma whipplei.

• Whipple’s disease typically presents as fever of unknown origin, chronic diarrhea, and joint pain. In addition to causing malabsorption, Whipple’s disease can affect a variety of organs including the brain (cognitive deficit, movement disorder, seizures), heart and lungs (pleuritis, pericarditis, endocarditis), joints (migratory asymmetric arthralgia), and eyes (vitreous opacities and hemorrhage, ophthalmoplegia, and central scotoma). Untreated Whipple’s disease can be fatal.

• The diagnosis is confirmed by a small intestine (or any other involved organ) biopsy, which shows periodic acid-Schiff (PAS)–positive macrophage inclusions. Polymerase chain reaction (PCR) testing for T. whipplei also confirms the diagnosis.

• Treatment with antibiotics should last for more than a year to prevent relapse. One regimen involves the use of doxycycline with hydroxychloroquine for 12 to 18 months. Sulfonamides cross the blood-brain barrier and should be added for treatment of neurologic symptoms.

Short-Bowel Syndrome

• Short-bowel syndrome is characterized by malabsorption when less than 180 cm of functional\ small bowel remains. The loss of small bowel can be anatomical because of surgery (resection, bowel bypass), or functional because of small-bowel disease (Crohn’s disease, ischemic injury). After bowel resection, some physiologic adaptation occurs in the remaining bowel to increase absorptive capacity. Changes can occur for up to 1 year after surgery and include slowing of motility, increase in bowel diameter, and lengthening of the villi.

• Symptoms of short-bowel syndrome include chronic diarrhea and weight loss, fatigue, abdominal pain, and edema. In addition to malabsorption, other factors contributing to the diarrhea include bacterial overgrowth if the ileocecal valve is removed and colonic irritation if bile acid reabsorption is decreased. Symptoms and signs of vitamin and mineral deficiencies can be present including anemia, metabolic bone disease, skin changes, bleeding, and muscle cramps.

• Treatment aims at establishing adequate caloric intake, preferably orally. Medium-chain triglycerides (which do not require digestion), high carbohydrate (except lactose), and a high fiber diet are recommended. Antidiarrheal agents and tincture of opium can be used, while cholestyramine is added to bind bile acids if colonic diarrhea is suspected. Antibiotic treatment for bacterial overgrowth is considered if the ileocecal valve is not present. Vitamins and minerals should be supplemented; and when oral intake is not adequate, total parenteral nutrition (TPN) can become necessary. Surgical solutions such as intestinal lengthening and bowel transplant are promising but not yet reliable.

 

Related keywords: human tapeworms
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