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Whipple's illness

Whipple's illness (intestinal lipodystrophy) — rather rare disease of perhaps infectious etiology, is characterized by blockade of absorbent vessels of a small bowel mukopolisakharidny complexes of a bacterial origin; proceeds with an arthralgia, diarrhea and exhaustion.

Epidemiology
Now in the world literature over 300 cases of this illness, the first message on which made in 1907 the American pathologist of G. H. Whipple, are described. In 80% of cases men at the age of 40 — 49 are ill.

Etiology and pathogeny
Blockade of the lymphatic device of a mucous membrane, absorbent vessels of a mesentery and lymph nodes of a small bowel PAS positive macrophages is the cornerstone of a disease. By means of a submicroscopy it is found out that these mukopolisakharidny complexes are either bacteria, or the products of phagocytal activity having a bacterial origin. The cells containing these substances find at this disease not only in a small bowel now, but also in other bodies and fabrics, than and systemacity of defeat speaks.

Classification
Detailed classification of an illness of Whipple is not developed. It is conditionally possible to allocate 3 stages of a disease (see below).
Approximate formulation of the diagnosis:
1. Whipple's illness, second stage. A syndrome of the broken absorption of the III degree with disturbance of protein metabolism, a vitamin deficiency.
2. Whipple's illness, third stage. A syndrome of the broken absorption of the III degree. Anasarca. The expressed polnartralgichesky syndrome.

Preliminary diagnosis
In the first stage abenteric symptoms dominate. Polyarthralgias, an uncertain abdominal pain, bronchitis, subfebrile temperature concern to them. This stage of an illness can proceed many years.

In the second stage signs of a heavy syndrome of the broken absorption with diarrhea, a steatorrhea, weight loss appear. The second stage is characterized by also various abenteric symptoms: lymph nodes increase, appear on hemorrhage skin, a nodal erythema. Persons of young age have a weight loss and even exhaustion can progress against a normal chair and even locks though at attentive studying of the anamnesis and survey of patients it is possible to reveal polyexcrements and a steatorrhea.

The third stage of an illness is characterized by development of a cachexia and a further pregressirovaniye of systemic lesions: paikardita, polyserositis, splenomegaly and neurologic defects. 6 — 10% of patients have paresis of oculomotor muscles, a hearing disorder and sight, a headache, an ataxy, a nystagmus, a tremor, there are symptoms of dementia, the sleep is interrupted, the polydipsia and a polyphagia develop. In rare instances the neurologic symptomatology can also precede intestinal symptoms that does differential diagnosis extremely difficult.

At patients SOE is considerably raised, hemoglobin level is reduced, the number of leukocytes and thrombocytes is increased. At the same time concentration of protein, iron, calcium, cholesterol in blood serum decreases. Functional absorbing tests with xylose, iodine - potassium test, test with loading glucose, etc. are positive. The steatorrhea is characteristic: loss of fat reaches 50 g/days and above.

Verification of the diagnosis, differential diagnosis
At endoscopic research the mucous membrane of a duodenum is edematous, hyperemic, with reinforced folds. A relief of a mucous membrane uneven because of the numerous slightly towering formations of light yellow color. The final diagnosis is established on the basis of data of histologic and especially ultrastructural research of a mucous membrane of the small bowel received during a duodenofibroskopiya. Histologic research allows to reveal the deformed, often club-shaped vorsina of a small bowel because of a large number of a lymph. A pathognomonic sign is sharply expressed infiltration of own layer of a mucous membrane the PAS positive macrophages who are located in polygonal cells with granular protoplasm. By means of a submicroscopy it is found out that PAS positive material of cells belongs to decomposition products of the bacterial nature or gram-positive bacteria, fagotsitirovanny these cells. A variety of abenteric symptoms, system nature of defeat and late development of the main signs of the broken absorption do the diagnosis of an illness of Whipple exclusively difficult. Successful early diagnosis of an illness of Whipple is possible under following conditions: a) knowledge of the main symptoms of an illness specialists of related clinical subjects (cardiologists, neuropathologists, rheumatologists, oculists, etc.); b) uses for diagnosis of a method of an enterobiopsiya; c) uses for final diagnosis of a method of a submicroscopy. It is clear, that two last conditions can be satisfied only in a specialized gastroenterological hospital where it is necessary to direct patients with the presumable diagnosis of an illness of Whipple.

Differential diagnosis of an illness of Whipple with other diseases of a small bowel presents great difficulties.

Treatment, prevention
Replacement of usual fats by average and korotkotsepochny (fatty products like portalak, etc.) leads to fast considerable reduction of a steatorrhea and simultaneous improvement owing to a body weight increase.

Antibiotics (tetracycline, doxycycline, clindamycin, etc.) apply is long — from 6 months to one year. Doses of drugs sredneterapevtichesky. In case of intolerance of antibiotics of a tetracycline row apply penicillin in a combination with streptomycin intramusculary. The dose of penicillin has to be not less than 1 200 000 PIECES/days, streptomycin — 1 g/days. Course of treatment of 10 — 14 days. Further antibiotics are appointed inside to the long term determined by results of repeated histologic researches of a mucous membrane of a small bowel and a condition of the patient.

At decrease in concentration of protein in blood serum appoint transfusions of amino-acid mixes on 250 ml daily or every other day during 2 — 4 weeks. Frustration of a water-salt, vitamin exchange liquidate the same drugs that at chronic enteritis.
At Whipple's illness patients are disabled, transfer them to disability of the I group. They have to be under supervision of specialized gastroenterological institutions as the forecast at this disease serious and often adverse.

 
"An illness heavy and - chains and a lymphoma of a small bowel   Variable immunodeficiency"