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Amyloidosis is a group of diseases in which abnormal proteins, known as amyloid fibrils, build up in tissue. [4] There are several non-specific and vague signs and symptoms associated with amyloidosis. [5] These include fatigue, peripheral edema, weight loss, shortness of breath, palpitations, and feeling faint with standing. [5]
The AA protein is mainly deposited in the liver, spleen and kidney, and AA amyloidosis can lead to nephrotic syndrome and ESRD. [ 17 ] [ 18 ] Natural history studies show, however, that it is the kidney involvement that drives the progression of the disease.
AL amyloidosis is caused by the deposition of abnormal antibody free light chains. The abnormal light chains are produced by monoclonal plasma cells, and, although AL amyloidosis can occur without diagnosis of another disorder, it is often associated with other plasma cell disorders, such as multiple myeloma and Waldenström's macroglobulinemia. [6]
Amyloidosis is the accumulation of misfolded protein fibers in the body that can be associated with many chronic illnesses. Even though amyloidosis is common in chronic kidney disease (CKD) patients receiving chronic regular dialysis, it has also been reported in a patient with chronic kidney failure but who never received dialysis.
LECT2 Amyloidosis (ALECT2) is a form of amyloidosis caused by the LECT2 protein. It was found to be the third most common (~3% of total) cause of amyloidosis in a set of more than 4,000 individuals studied at the Mayo Clinic; the first and second most common forms the disorder were AL amyloidosis and AA amyloidosis, respectively.
Familial renal amyloidosis is a form of amyloidosis primarily presenting in the kidney. [1] It is associated most commonly with congenital mutations in the fibrinogen alpha chain and classified as a dysfibrinogenemia (see Hereditary Fibrinogen Aα-Chain Amyloidosis). [2] [3] and, less commonly, with congenital mutations in apolipoprotein A1 [4 ...
Amyloidosis is the most common form of MGRS, with the AL subtype being the most common type. [3] Extra-renal manifestations, such as heart failure, gastrointestinal symptoms, secondary neuropathy (carpal tunnel syndrome), liver involvement and peripheral neuropathy are very common with amyloidosis related MGRS. [3]
Increased bone resorption leads to hypercalcemia and causes nephrocalcinosis, thereby contributing to kidney failure. Amyloidosis is a distant third in the causation. People with amyloidosis have high levels of amyloid protein that can be excreted through the kidneys and cause damage to the kidneys and other organs. [28] [29]
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