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In Whipple's disease, disorders of humoral and cellular immunity are detected.
The mechanism of the development of Whipple's disease has not been fully established, but it has been found that the basis for the development of the disease is the blockade of PA8-positive macrophages.ami of the lymphatic apparatus of the mucous membrane, lymphatic vessels of the mesentery and lymph nodes of the small intestine.
There is an increase in mesenteric lymph nodes and thickening of the mesentery.
As a result of the accumulation of macrophages containing bacteria in the mucous membrane of the small intestine, malabsorption syndrome (loss of nutrients) develops, and macrophages that have entered the synovial membranes of the joints contribute to the development of arthritis. The bowel lesion is patchy. The wall of the small intestine, as the disease develops, thickens and stretches, the surface of its mucous membrane becomes granular, and the villi thicken and shorten.
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In 30% of patients, the HLA-B27 haplotype is detected (specific 27 allele of locus B, which is responsible for the synthesis of the histocompatibility antigen characteristic of some autoimmune diseases). Macrophages containing the pathogen form infiltrates in various organs (in the small intestine, mesenteric lymph nodes, synovial membranes of the joints, in the heart, lungs, brain and eyes), which causes the development of pathological processes in the affected organs.
The initial stage of the disease is accompanied by an increase in temperature up to 38 ° C (in some cases, even higher), chills and muscle pain, swollen lymph nodes and reddening of the skin in the area of inflamed joints. Arthralgia or polyarthralgia in 48% of patients with Whipple's disease precedes the onset of other symptoms. Joint pains are characterized by a migratory character.
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Diarrhea, which is observed in 75% of patients. The stool is offensive, light and copious, and may be frothy or greasy. Steatorrhea, which is present in 13% of patients. Flatulence observed in 8% of cases. Paroxysmal pain in the abdomen mainly after eating (in 50% of patients). Progressive weight loss in 80-100% of patients.
1-2 years after the onset of diarrhea, edema occurs in 25% of lanoxin. The skin of patients is dry, with diffuse hyperpigmentation. Nails become brittle and hair falls out. In half of the patients, due to recurrent tracheobronchitis and pleurisy, an unproductive cough appears.
In 30–55% of cases, patients develop endocarditis.
Whipple's disease is treated with: antibacterial drugs that destroy the causative agent of the disease; hormonal anti-inflammatory drugs that reduce inflammation; enzyme preparations that improve the absorption of vitamins and nutrients; diets (table number 5) and fractional nutrition; vitamin therapy; symptomatic therapy.
Whipple's disease is a rare infectious disease that occurs with a primary lesion of the lymphatic system of digoxin pills intestine and synovial membranes of the joints. The main clinical signs: diarrhea, fever, polyarthritis and joint pain, multiple enlargement of the lymph nodes, damage to the lungs, heart and central nervous system.
The diagnosis is made by performing imaging diagnostic tests (EGDS, ultrasound, X-ray), biopsy of digoxin intestine tissues, blood tests and PCR diagnostics. Patients are shown long-term antibiotic therapy, the appointment of glucocorticosteroids, enzymes and multivitamin complexes.
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This suggested that the basis of the disease is intestinal lipodystrophy - a violation of the production and breakdown of fats. Later, the infectious nature of the disease was proved, and in 1992, thanks to the PCR method, the pathogen was identified. The incidence of the disease is 0.5–1 case per 1 million population. According to statistics, pathology often affects people 40-50 years old, in 80% of cases, patients are males.
During the autopsy procedure, the scientist discovered mesenteric lymphadenopathy and lipid deposition in the intestinal wall in the deceased patient.
Whipple's disease (lipophagic intestinal granulomatosis, intestinal lipodystrophy) is a pathology named after George H. Whipple, an American pathologist who discovered it in 1907. The causative agent of infection in Whipple's disease are gram-positive pathogens Tropheryma whippelii - representatives of actinomycetes with a three-layer cell wall. Infection is carried out by the fecal-oral route in childhood and is manifested by short-term gastroenteritis. Family cases of morbidity have been registered, but not a single fact of transmission of the bacterium from person to person has been reliably recorded. Some scientists believe that only certain pathogenic strains cause pathological changes.
As a result, macrophages with pathogens settle in the mucosa of the small intestine and the lymph nodes of the mesentery, causing malabsorptive diarrhea and impaired lymphatic drainage.
Intestinal lipodystrophy is characterized by the accumulation of infiltrates consisting of macrophages containing Tropheryma whippelii in various organs. The presence of bacteria in phagocytes indicates that these cells are capable of capturing microorganisms, but cannot carry out their lysis. This is due to disorders of the immune response (decrease in the production of the anti-inflammatory cytokine IL-12 and interferon gamma, a decrease in the lymphocytic response to antigens, as well as the production of T-lymphocytes).