Amyloidosis HY
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This document discusses amyloidosis, a condition caused by the extracellular deposition of insoluble protein fibrils. There are several types of amyloidosis, including AL amyloidosis produced by clonal plasma cells, AA amyloidosis secondary to chronic inflammation, ATTR amyloidosis which can be age-related or hereditary, and AB2-m amyloidosis related to dialysis. Clinical presentations of amyloidosis include nephrotic syndrome for AL and AA types, hepatomegaly and splenomegaly for AL and AA types, restrictive cardiomyopathy for AL and ATTR types, and carpal tunnel syndrome and scapulohumeral periarthritis for AB2-m type. Other presentations include peripheral neuropathy, autonomic neuropathy,
Amyloidosis HY
- 1. Amyloidosis Etiology Extracellular deposition of insoluble protein fibrils AL: produced by clonal plasma cells AA: secondary to chronic inflammation ATTR: age-related or hereditary AB2-m: dialysis-related Clinical Presentation Nephrotic syndrome (AL & AA) Hepatomegaly with/without splenomegaly (AL & AA) Restrictive cardiomyopathy (AL & ATTR) CTS, scapulohumeral periarthritis (AB2-m) Peripheral/autonomic neuropathy Periorbital purpura, waxy skin, macroglossia