Intestinal lipodystrophy

Intestinal lipodystrophy (Whipple's disease, idiopathic steatorrhoea, etc.) is a systemic disease with a primary lesion of the small intestine and a violation of fat absorption. A rare disease that occurs mainly in middle-aged and elderly men.

Etiology and pathogenesis are unknown.

The main symptoms are persistent diarrhea (up to 5-15 times a day), general exhaustion, colic pain in the abdomen, weakness. The chair is plentiful, light, foamy or ointment, sometimes peculiar "chile". Often observed migratory polyarthralgia, dry cough, subfebrile fever is possible. Skin is dry and hyperpigmented. Peripheral lymph nodes are enlarged, dense, but painless. Sometimes signs of polyserositis are determined. The abdomen is swollen, soft, in some cases, palpation can determine the focal seal at the attachment site of the mesentery of the small intestine, formed by a conglomerate of enlarged lymph nodes. There may be hypoproteinemic edema on the legs.

The blood determines a moderate neutrophilic leukocytosis (in some cases eoeinophilia), hypoproteinemia, hypocholesterolemia, hypocalcemia. A characteristic feature is the detection in the bowel movements of a large amount of neutral fat, crystals of fatty acids, undigested muscle fibers. Radiologic examination shows a thickening of the folds of the small intestine mucosa, a decrease in its tone and peristalsis.

Diagnosis is established with aspiration biopsy of the small intestine mucosa: histological examination of biopsies reveals atrophy of intestinal villi and reveals macrophages specific for this disease with foamy protoplasm, which is pink in color with Schiff's reagent (Siraca's body). Differential diagnosis is carried out with chronic enteritis, enterocolitis, European and tropical sprue, disaccharidase-deficient and gluten enteropathy, exudative enteropathy, chronic pancreatitis.

Flow. The disease begins imperceptibly, gradually, with polyarthralgia or moderate diarrhea, but then quickly progresses, there are pronounced violations of intestinal digestion and absorption. The average life expectancy of patients is 2 years.

Treatment during periods of exacerbation of the process is stationary. Assign a diet with a high content of protein, vitamins and fat restriction. Drug therapy is carried out with glucocorticosteroid hormones (20-30 mg of prednisolone per day) and antibiotics. In addition, parenterally, it is better to prescribe vitamins, especially fat-soluble (A, E, K), the absorption of which is most severe in this disease, as well as intra-enzyme preparations (festal, panzinorm, etc.), astringents (tanalbine, kaolin, etc. .). After discharge from the hospital, supportive therapy with corticosteroid hormones in small doses, preparations of digestive enzymes.