Amyloidosis

Amyloidosis - a systemic disease affecting multiple organs and different tissue structures, characterized by the disturbance of protein metabolism and extracellular deposition in these complex protein-polysaccharide complex. There are the following basic forms of amyloidosis: 1) primary amyloidosis, apparently representing fermentopathy innate, inherited in an autosomal dominant manner (perhaps there are several forms of congenital amyloidosis); 2) secondary, occurring against a background of long-term chronic diseases characterized by the collapse of tissues and absorption decay products (in tuberculosis of the lungs and other organs, bronchiectasis, chronic osteomyelitis and other diseases) or significant immunopathological disorders (ulcerative colitis, serum sickness, and Dr. T. ).; 3) idiopathic; 4) senile amyloidosis.

The defeat of the digestive system found in any form of amyloidosis, especially of some of its departments (language - macroglossia, intestines, liver). In most cases, the defeat of the digestive system is just one of the many locations of the process (at the same time there is a renal amyloidosis, skin, skeletal muscles, nervous system and other organs and tissues).

Amyloidosis is more common esophageal lesions simultaneously other parts of the digestive system. Characterized dysphagia swallowing thick and dry food, especially when eating, lying down, belching. When X-ray esophagus gipotonichen study peristalsis weakened, if a patient study in a horizontal position barium suspension of long delays in the esophagus. Complications: amyloid ulceration of the esophagus and esophageal bleeding.

Amyloidosis of the stomach is usually combined with amyloidosis of the intestine and other organs. The clinical picture: a feeling of heaviness in the epigastric region after eating, dyspepsia; X-ray examination - smoothed the folds of mucous membrane, the weakening of peristalsis and emptying of the stomach. Complications: amyloid stomach ulcers, stomach bleeding, perforation of the ulcer. The differential diagnosis spend with chronic gastritis, gastric ulcer, rarely - tumor. Crucial have biopsy data (identification amyloidosis).

-Part Amyloidosis intestinal localization of the disease. It manifests a sense of discomfort, heaviness, dull or less moderate spastic abdominal pain, stool disorders: constipation or persistent diarrhea. Koprologmcheskoe study reveals expressed steatorrhea, amiloreyu, Creators. In the blood, anemia, leukocytosis, increased erythrocyte sedimentation rate, hypoproteinemia (due to hypoalbuminemia), hyperglobulinaemia, hyponatremia, hypoprothrombinemia, hypocalcemia. Special research methods reveal a violation of membrane digestion and absorption in the intestine (see. Failure absorption syndrome). When X-ray is characteristic deployed ( "vzdybpennost") intestinal loops, thickened folds, and flattening of intestinal mucosa relief, slowing or speeding up the passage of barium suspension through the intestines. A biopsy of the mucous membrane of small and large intestine confirms the diagnosis and allows to make a differential diagnosis with enteritis and colitis, especially ulcerative colitis. Isolated tumor intestinal amyloidosis occurs under the guise of the tumor (pain, ileus) and is usually found already on the operating table. Complication: severe hypoproteinemia due to malabsorption processes in the gut, polyhypovitaminosis, intestinal stenosis, amyloid ulcers, intestinal bleeding, perforation.

Liver Amyloidosis occurs relatively frequently. Characterized by an increase in liver and seal palpation edge of her smooth, painless. Not rare syndrome of portal hypertension, ascites. Less common are pain in the right upper quadrant, dyspeptic symptoms, splenomegaly, jaundice, hemorrhagic syndrome. Laboratory tests determine the change in protein-sediment samples hyperglobulinemia, hypercholesterolemia, in some cases - giperbiliru-Binh, increased activity of alkaline phosphatase, aminotransferase serum; positive test with bromsulfaleinom. Crucial in the diagnosis of a hepatic biopsy. Complications: hepatic failure (7%).

Amyloidosis pancreas is rarely diagnosed (takes place under the guise of chronic pancreatitis); characterized by a dull pain in the left upper quadrant, dyspeptic symptoms, pancreatogenic diarrhea, steatorrhea. Investigation reveals duodenal contents exocrine pancreatic insufficiency. In severe cases, secondary diabetes develops.

The diagnosis of amyloidosis of the digestive system. Of great importance is the identification of lesions of other organs and systems: kidneys, heart, skin, spleen, etc.,. the most reliable method for diagnosing amyloidosis is an organ biopsy, in which punctate detect clumps of amyloid with Congo red staining sections, methylene or gentian violet. In the secondary form of the disease it is important to identify chronic disease, which is usually complicated by amyloidosis (tuberculosis, bronchiectasis, empyema, and so on. D.). Applied also functional clinical trials: with Congo red and methylene blue (rapid disappearance of colors by intravenous administration of the serum amyloid due to the sharp decrease in allocation of fixing their kidneys in the urine). However, primary amyloidosis they give inconsistent results. The diagnosis of primary amyloidosis is based on a thorough genetic analysis (identification of amyloidosis with relatives). Laboratory blood tests reveal Dysproteinemia (hypoalbuminemia, gipergammaglo-bulinemiya; in later stage -increase content ap-F-2 and serum betaglobulinov), increased erythrocyte sedimentation rate, in some cases - hyperfibrinogenemia, elevated levels of cholesterol and blood betalipidov. However, these changes are not specific for amyloidosis. The course progresses. As the disease progresses, patients disabling.

Prognosis is poor. Death occurs from exhaustion patients, renal, cardiac failure.

Treatment. In secondary amyloidosis in the first place, treatment of the underlying diseases (tuberculosis, osteomyelitis, chronic empyema, etc.), After which the cure is often disappear and symptoms of amyloidosis. From the specific means of treatment of amyloidosis using im munodepressanty corticosteroids. With early treatment may reverse the development of amyloidosis. Symptomatic therapy: a multivitamin (parenteral), diuretics (when expressed edema), plasma transfusion. When liver failure - appropriate symptomatic therapy. When intestinal amyloidosis, occurring with persistent diarrhea, -vyazhuschie means (bismuth nitrate basic, adsorbents). When the isolated tumor-amyloidosis of the gastrointestinal tract - surgical treatment.