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Related to ATTR amyloidosis: Hereditary 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 ?
Systemic wild-type ATTR amyloidosis (formerly age-related or senile systemic amyloidosis) refers to the deposition of unmutated transthyretin in tissues, often in the myocardium, in elderly individuals.
Specifically, Alnylam will obtain global development and commercialization rights to its investigational RNAi therapeutics programs for the treatment of ATTR amyloidosis, including patisiran and ALN-TTRsc02.
Alnylam Pharmaceuticals, a biopharmaceutical company, is halting the development of revusiran, a therapeutic intended for treating hereditary ATTR amyloidosis with cardiomyopathy (hATTR-CM), it was reported yesterday.
M2 PHARMA-November 27, 2017-Alnylam Secures Breakthrough Therapy Designation from US FDA for Patisiran for Treatment of Hereditary ATTR Amyloidosis with Polyneuropathy