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Related to 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 ?
The explanation for this finding is that the full benefits of delaying amyloidosis in terms of reduced AD prevalence are not realized for many years because of the long lag time between amyloidosis and clinical AD.
Breast amyloidosis may result from localized or systemic disease.
Morphologically, 20 patients (53%) were diagnosed as papular amyloidosis (Figure 1), 11 (29%) as macular (Figure 2) and 7 (18%) as biphasic.
Radiologic findings of abdominal involvement in systemic amyloidosis are nonspecific and can present in a variety of ways.
All proceeds for the CD will go to the UCL Amyloidosis Research Fund, which receives donations from patients, relatives and other supporters.
Systemic amyloidosis is a syndrome of amyloid fibril extracellular deposition, which is a combination of more than thirty precursor proteins with a core structure of [sz] strands and various nonfibrillary constituents including glycosaminoglycans and serum amyloid P (SAP).
The most common forms of amyloidosis include systemic AL amyloidosis (formerly primary amyloidosis), systemic AA amyloidosis (formerly secondary amyloidosis), systemic wild-type ATTR amyloidosis (formerly age-related or senile systemic amyloidosis), systemic hereditary ATTR amyloidosis (formerly familial amyloid polyneuropathy), and localized AL amyloidosis (Table 1).
Amyloidosis may involve the respiratory system in larynx, trachea, bronchus, lung, and mediastinal lymph nodes.
99m)Tc-Pyrophosphate scintigraphy for differentiating light-chain cardiac amyloidosis from the transthyretin-related familial and senile cardiac amyloidoses.
4) The diagnosis of laryngeal amyloidosis should trigger evaluation for systemic amyloidosis.
Her medical history was positive for familial amyloidosis diagnosed based on abdominal and rectal biopsies when she was in second grade.
USPRwire, Tue Apr 05 2016] Global Markets Direct's, 'Amyloidosis - Pipeline Review, H1 2016', provides an overview of the Amyloidosis pipeline landscape.