Amyloidosis

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amyloidosis

[‚am·ə·loi′dō·səs]
(medicine)
Deposition of amyloid in one or more organs of the body.

Amyloidosis

 

or amyloid dystrophy, a disorder of protein metabolism accompanied by the formation within tissues of a specific protein polysaccharide complex known as amyloid. The progress of the disease is associated with distortion of the protein-synthesizing function of the reticuloendothelial system and with accumulation of anomalous proteins in the blood plasma which act as autoantigens and also stimulate the formation of autoantibodies. As a result of antigen-antibody interaction, there is deposition of widely dispersed proteins which figure in the formation of amyloid. Once deposited in tissues, such as vascular and gland walls, the amyloid displaces functionally specialized elements of the organ; this process leads to the destruction of the organ.

Several types of amyloidosis are recognized: primary, secondary, and senile amyloidosis; amyloidosis with multiple myeloma; and localized tumorlike amyloidosis. Primary amyloidosis has no connection with any other disease; it affects mainly the cardiovascular system, alimentary tract, muscles, and skin. Secondary amyloidosis develops subsequent to other diseases which are accompanied by prolonged suppuration and tissue breakdown, such as tuberculosis, syphilis, and rheumatoid arthritis. It most often affects the spleen, liver, kidneys, adrenal glands, and intestines. Senile amyloidosis usually involves the heart. Amyloidosis may be either general or localized. Im-munodepressive and hepatic preparations are used in its treatment.

V. V. SEROV

References in periodicals archive ?
It also is possible that the circulating monoclonal light chains in these patients were extremely amyloidogenic, leading to renal amyloidosis, even in very small, undetectable quantities in the serum and their absence in the urine.
13) Like albumin, other proteins should also not be found in the urine, however during renal amyloidosis, amyloid proteins accumulate in the kidney causing proteinuria.
The effective dosage reported by Livneh et al in a study of 68 FMF patients who also had complications of secondary renal amyloidosis is > 1.
These data suggest that renal amyloidosis may increase mortality for patients whose health is already compromised by Familial Mediterranean Fever," said Dr.
Renal amyloidosis is a chronic and fatal disease marked by the deposit of amyloid fibrils in the kidney.
Nevertheless, 2 other forms of renal amyloidosis have also been described: the vascular and the interstitial forms.
5,26-35) Annual average incidence of renal amyloidosis in our unit on native kidney biopsies is found 6.
However, tubular casts containing amyloid as the only manifestation of renal amyloidosis have been reported in rare cases (Figure 24).
Amyloid derived from a mutant fibrinogen (AFib) causes isolated renal amyloidosis, and ALys derived from a mutant lysozyme is associated with visceral involvement.