| Autor: |
Zamarra, Giovanni Battista, Sandu, Marina, Caione, Nicholas, Di Pasquale, Gabriele, Di Berardino, Alessio, Di Ludovico, Armando, La Bella, Saverio, Chiarelli, Francesco, Cattivera, Valentina, Colella, Jacopo, Di Donato, Giulio |
| Zdroj: |
Journal of Clinical Medicine; Nov2024, Vol. 13 Issue 22, p6682, 18p |
| Abstrakt: |
Amyloidosis is a rare multisystem disorder characterized by extracellular accumulation of insoluble fibrils in various organs and tissues. The most common subtype in the pediatric population is systemic reactive amyloidosis, typically developing secondary to chronic inflammatory conditions and resulting in deposition of serum amyloid A protein in association with apolipoprotein HDL3. Clinical presentation is highly variable and is mostly influenced by specific organs involved, precursor protein type, and extent of amyloid deposition, often closely reflecting clinical features of the underlying disease. The most critical determinants of prognosis are cardiac and renal involvement. Diagnosis of amyloidosis is confirmed by tissue biopsy, which remains the gold standard, followed by precise amyloid fibril typing. The primary therapeutic approach is directed towards controlling underlying disease and reducing serum levels of precursor proteins to prevent further amyloid deposition. This study aims to highlight the main clinical characteristics of amyloidosis with onset in childhood, emphasizing the key differences compared to adult form. [ABSTRACT FROM AUTHOR] |
| Databáze: |
Complementary Index |
| Externí odkaz: |
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