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Whipple's Disease

Whipple's disease

Definition

Whipple's disease is a rare infectious disorder that can affect many areas of the body, including the gastrointestinal and central nervous systems. Caused by the bacteria Tropheryma whipplei, it is typically diagnosed from malabsorption symptoms such as diarrhea and weight loss. If the central nervous system is infected, Whipple's disease can cause impairment of mental faculties and lead to dementia . It can be treated successfully with antibiotic therapy, but up to a third of patients suffer relapse.

Description

Whipple's disease, also known as intestinal lipodystrophy, was first reported in 1907 by George Hoyt Whipple (18781976). An autopsy on a thirty-seven year old male missionary revealed a granular accumulation of fatty acids in the walls of the small intestine and lymph nodes.

Historically, Whipple's disease has been considered an gastro-intestinal disorder, however, in the 1960s it was realized that other organs could be involved, with or without intestinal infection. It is now considered a systemic infection with a wide range of possible symptoms.

Demographics

The disorder typically affects middle-aged men of European descent. Most cases have been reported in North America and Europe. Many texts suggest the disorder affects eight times as many males as females, although there is some evidence to suggest the rate in females is rising.

The disease is extremely rare and no reliable estimate of incidence is known. Farmers and other rural people are most often diagnosed with Whipple's disease, but as yet, no specific environmental factors have been linked to the disorder.

Causes and symptoms

The bacterium that causes Whipple's disease was only successfully cultured in 1997. Tropheryma whipplei belongs to the high G+C phylum of gram-positive bacteria, and its genome was sequenced in 2003.

Whipple's disease has traditionally been regarded as a malabsorption disease of the small intestine, but in most cases the first symptoms are arthritic joints, which can precede the malabsorption symptoms of Whipple's disease by many years. Commonly, the disease progresses to the small intestine. Symptoms then include diarrhea, anemia, weight loss, and there is often fat present in the stool, all due to the bacteria disrupting absorption of fat and nutrients. If untreated, other malabsorption problems, such as reductions in the levels of calcium and magnesium, may result. Fever and night sweats are common, as well as general weakness. There are many further possible symptoms depending on the organs affected.

In cases where the central nervous system is affected, there may be a decrease in intellectual abilities, insomnia, hearing loss or tinnitus (ringing in the ears), and uncontrolled muscle movements (ataxia ) or eye movements. If untreated, the disorder can lead to dementia and progressive brain cell death, leading to coma and death over a period of months to years.

Diagnosis

Diagnosis of Whipple's disease is difficult, and is commonly suspected only if the patient presents with malabsorption symptoms. Then, a small-bowel biopsy can be made to locate the presence of the bacteria and confirm the diagnosis. However, symptoms can vary greatly depending on the areas of the body that are affected, and up to a third of sufferers do not present with malabsorption ailments.

Treatment team

Once diagnosed, the treatment of Whipple's disease is often straightforward, and can be monitored with minor hospital procedures. However, due to the rarity of the disease and the recent developments in studying the disorder it is recommended that contact be made with specialized centers of research or a neurologist .

Treatment

Whipple's disease generally responds well to antibiotic therapy. The recommended treatment is two weeks of intravenous antibiotics followed by a year or more of oral antibiotics. If the malabsorption symptoms are pronounced, the patient may require intravenous fluids and electrolytes, and other dietary supplements. A diet high in calories and protein is often recommended, and should be monitored by a physician.

Recovery and rehabilitation

When treated, symptoms such as diarrhea and fever can resolve within days, and most symptoms typically improve within a few weeks. In most cases, symptoms of the disorder are lessened or ameliorated by treatment. The progress of therapy can be checked by biopsy of the small intestine. In about one third of cases, the disease relapses and is more likely to affect the central nervous system than the initial infection. Periodic monitoring over several years, therefore, is essential to prevent neurological damage.

Clinical trials

Although as of early 2004, there were no ongoing clinical trials in the United States specific for Whipple's disease, the National Institute of Diabetes and Digestive and Kidney Diseases supports research for similar disorders.

Prognosis

If untreated, Whipple's disease can be fatal, but when treated with antibiotic therapy most patients experience rapid recovery and lasting remission. However, up to a third of patients may suffer a relapse.

Special concerns

Knowledge of Whipple's disease is rapidly evolving, and there have been many recent developments that may lead to new diagnostic options and new treatments in the near future.

Resources

PERIODICALS

Marth, Thomas, and Dider Raoult. "Whipple's disease," Lancet 361, no. 9353 (January 18, 2003): 239247.

OTHER

"NINDS Whipple's Disease Information Page." National Institute of Neurological Disorders and Stroke. (March 10, 2004). <http://www.ninds.nih.gov/health_and_medical/disorders/whipples.htm>.

"Whipple's Disease." National Digestive Diseases Information Clearinghouse. (March 10, 2004). <http://digestive.niddk.nih.gov/ddiseases/pubs/whipple/index.htm>.

ORGANIZATIONS

National Organization for Rare Disorders (NORD). P.O. Box 1968 (55 Kenosia Avenue), Danbury, CT 06813-1968. (203) 7440100 or (800) 999NORD (6673); Fax: (203) 7982291. orphan@rarediseases.org. <http://www.rarediseases.org>.

David Tulloch,

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Whipple's disease

Whipple's disease (wip-ŭlz) n. a rare disease, occurring only in males, in which there is malabsorption, usually accompanied by skin pigmentation and arthritis. [ G. H. Whipple (1878–1976), US pathologist]

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