systemic disease, resulting in failure of various organs, sclerosis of the parenchyma and atrophy, characterized by deposition in tissues of amyloid (a complex protein-polysaccharide complex). Rare disease, common in the elderly after age 60.
- Primary amyloidosis is often caused by changes of plasma cells in multiple myeloma, monoclonal HyperGamma-globulinemia or disease valdenstroma. The complex protein-polysaccharide complex formed from light chains of Ig, and when the above-mentioned diseases, the synthesis of such circuits is increasing rapidly.
Secondary amyloidosis is a chronic inflammatory diseases (this can be
, bronchiectasis and
or leprosy). In this case, the amyloid is composed of fibrillar protein amyloid a and serum amyloid protein (decay products).
Idiopathic amyloidosis. Is usually congenital enzyme defect. Distinguish some forms of familial amyloidosis, e.g., amyloidosis of the skin with Mediterranean fever (paroxysmal polyserositis, manifested by fever, abdominal pain, also
, pleurisy, and lesions on the skin surface).
- Or senile amyloidosis in old age.
- Dialysis amyloidosis occurs when the conduct of extrarenal blood purification in cases of renal failure.
Causes various lesions of human organs kidneys, bowels or skin are not yet known to science. The final diagnosis is affected by electron microscopy.
The symptoms and course of the disease are different and depend on the extent and localization of amyloid deposits in the organs from the time course of the disease and presence of complications. So, there is a whole complex of symptoms that are associated with a lesion of some organs.
- lose some parts of the digestive system causes amyloidosis of the esophagus characterized by dysphagia when swallowing solid or dense dry food, also sometimes belching
- amyloidosis of the stomach often occurs together with amyloidosis of the intestine and some other organs. Symptoms are dyspepsia and a feeling of heaviness after a meal
- intestinal amyloidosis common disease that results in the discomfort and severity, sometimes expressed in blunt abdominal pain, constipation and diarrhea. Isolated tumour-like intestinal amyloidosis occurs in the form of the tumor with pain and intestinal obstruction, and learn of him, often right on the operating table
amyloidosis of the liver occurs also often. In this type of the disease is a thickening and enlargement of the liver, by palpation palpation a smooth edge, but painless. Rarely is the disease with right upper quadrant pain or dyspepsia, splenomegaly,
or hemorrhagic syndrome
amyloidosis of the pancreas manifests itself in chronic
accompanied by a dull pain in the left upper quadrant and dyspeptic symptoms
amyloidosis of the heart leads to the deposition of protein-polysaccharide complex in the tissue of the heart, which is expressed later in hypertrophy of the myocardium, a violation of his cuts in the development of chronic circulatory failure,
renal amyloidosis occurs when any form of amyloidosis. So, the main symptom of renal amyloidosis proteinuria, growing in all its forms, but typical of secondary disease. Patients often long time have no complaints. And later, with the appearance of swelling,
also thrombosis of the renal vein, accompanied by pain and anuria, lead patients with renal amyloidosis in the hospital for a consultation with a doctor
also, when amyloidosis affects other systems and organs. Disrupts the function of the cardiovascular system, skin translucent nodules on the skin, manifested
Diagnosis of amyloidosis is carried out laboratory studies and other special studies.
In recognition of amyloidosis laboratory studies reveal anemia in the blood, leukocytosis, increased erythrocyte sedimentation rate (sed rate), hypoproteinemia and hyperglobulinemia, hyponatremia, hypocalcaemia and hypoprothrombinemia. When liver disease is manifested hypercholesterolemia, and occasionally hyperbilirubinemia. Evaluate the functions of the thyroid gland and kidneys. Examine the urine sediment revealing not only protein, but also leukocytes and red blood cells, cylinders. Increased content of amyloid in the blood and urine specifies the primary amyloidosis. The detection of signs of inflammatory disease manifested secondary amyloidosis. Is scatological study with the identification of steatorrhea and amenorei, creatorii.
Additional research to identify amyloidosis is echocardiography, x-ray, in which
- gipotonica the esophagus, and peristalsis weakened, while horizontal position of the patient suspension of barium lingers in the esophagus is the esophageal amyloidosis
- the evacuation of the contents from the stomach, smoothed the folds of the mucous membrane, weakened peristalsis amyloidosis of the stomach
- observed the flattening the relief of the intestinal mucosa and intense intestinal loops, thickening of folds of intestinal amyloidosis.
When lesions of the gastrointestinal tract see
of the stomach and chronic
Carpal tunnel syndrome and peripheral polyneuropathy, restrictive cardiomyopathy,
the urinary tract obstruction, acute tubular necrosis, symmetrical polyarthritis, diseases of the lungs, toxic effects on the kidneys,
when multiple infarctions of the brain lead to amyloidosis.
Patients with amyloidosis shows
- outpatient mode if not observed the serious condition of the patient
- long eating raw liver for two years 100120 grams per day
- the restriction of the use of salt and protein in food
- the treatment of the disease, symptoms of which are similar to amyloidosis. After the treatment the symptoms disappear. This is secondary amyloidosis
- if amyloidosis of the intestine is manifested by diarrhea, prescribed astringents, for example, basic bismuth nitrate, or adsorbents.
Surgical intervention when the disease
The improvement of the human condition is possible by means of splenectomy, which reduces the amount of amyloid, obrazuemogo in the body of the patient. When amyloidosis isolated tumor of the digestive tract, you need surgery.
In primary amyloidosis requires frequent and periodic determination of the level of amyloid proteins, to evaluate the correctness and effectiveness of treatment of the disease. Also pay attention to control kidney function to watch for treatment and effectiveness of therapy.
In secondary amyloidoses and dialysis you should know that even with ongoing treatment there is chronic renal insufficiency and heart, affected joints, occurs polyneuropathy and interstitial lung disease.
Complications of amyloidosis can be considered as a perforation of ulcers, amyloid ulcers of the esophagus, stomach, esophageal and gastro-intestinal bleeding, intestinal stenosis.
Possible hypoproteinemia in severe form as a consequence of changes in the processes of absorption in the intestine.
The course and prognosis of the disease
If you develop chronic renal failure in primary amyloidosis, these people do not live more than a year. If heart failure developed, these patients to live remains no more than four months.
In General, the average survival rate is around 1214 months, but according to statistics women live longer than men.
In secondary amyloidosis the prognosis for recovery and cure it a little bit better. It also is determined by identifying the underlying disease and its treatment.
When family and dialysis amyloidoses forecast may be different. Worse of all, this disease occurs in the elderly.