Amyloidosis (Kidney)
Overview
Plain-Language Overview
Amyloidosis (Kidney) is a condition where abnormal proteins called amyloid build up in the kidneys. These proteins are misfolded and deposit in the kidney tissues, especially in the filtering units called glomeruli. This buildup interferes with the kidney's ability to filter blood properly, leading to problems like proteinuria (excess protein in urine) and swelling in the body. Over time, the damage can cause the kidneys to lose function, potentially leading to kidney failure. The condition affects the urinary system and can have serious effects on overall health due to impaired waste removal and fluid balance.
Clinical Definition
Amyloidosis (Kidney) is characterized by the extracellular deposition of insoluble amyloid fibrils composed of misfolded protein precursors in the renal parenchyma, predominantly within the glomeruli, blood vessels, and interstitium. The most common forms affecting the kidney are AL amyloidosis, caused by monoclonal light chains from plasma cell dyscrasias, and AA amyloidosis, secondary to chronic inflammatory states with elevated serum amyloid A protein. These deposits disrupt normal kidney architecture and function, leading to nephrotic syndrome, progressive renal insufficiency, and eventual end-stage renal disease. Diagnosis is clinically significant due to the systemic nature of amyloidosis and its association with underlying disorders such as multiple myeloma or chronic infections.
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