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Related to primary amyloidosis: secondary amyloidosis, familial amyloidosis


Deposition of amyloid in one or more organs of the body.



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.


References in periodicals archive ?
sup][2] Pulmonary amyloidosis is usually due to primary amyloidosis with deposition of immunoglobulin light-chain fragments.
Primary amyloidosis with high grade transitional cell carcinoma of bladder: a rare case report.
Primary amyloidosis of the renal pelvis and upper ureter.
Long-term survival (10 years or more) in 30 patients with primary amyloidosis.
Primary amyloidosis occurs when the body produces abnormal protein fibers, which join together to form amyloid deposits in different organs, including the kidneys, where they cause serious damage (NIDDK, 2003).
Fushimi reported the first case with primary amyloidosis of ureter successfully treated by oral administration of prednisolone.
A Monoclonal gammopathy of undetermined significance (MGUS) is the term used to denote the presence of a monoclonal protein (M-protein, myeloma protein, or paraprotein) in persons without evidence of plasma-cell myeloma, Waldenstrom macroglobulinemia, primary amyloidosis, or related disorders.
Primary amyloidosis occurs spontaneously in the absence of apparent predisposing illness and often affects tissues of mesodermal origin such as muscle, heart, skin, spleen, liver, kidney, thyroid, tongue etc.
This assay, which first became commercially available in 2001, is used to diagnose and monitor light chain myeloma, primary amyloidosis, and related light chain diseases.
Therefore, we declared a diagnosis of primary amyloidosis of the right external auditory canal and middle ear.
8-10) An enlarged tongue resulting in apertognathia and tooth indentations along the lateral border can be the first clinical sign of primary amyloidosis.

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