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01-08-2011 | Educational Review

Renal amyloidosis in children

Authors: Yelda Bilginer, Tekin Akpolat, Seza Ozen

Published in: Pediatric Nephrology | Issue 8/2011

Abstract

Renal amyloidosis is a detrimental disease caused by the deposition of amyloid fibrils. A child with renal amyloidosis may present with proteinuria or nephrotic syndrome. Chronic renal failure may follow. Amyloid fibrils may deposit in other organs as well. The diagnosis is through the typical appearance on histopathology. Although chronic infections and chronic inflammatory diseases used to be the causes of secondary amyloidosis in children, the most frequent cause is now autoinflammatory diseases. Among this group of diseases, the most frequent one throughout the world is familial Mediterranean fever (FMF). FMF is typically characterized by attacks of clinical inflammation in the form of fever and serositis and high acute-phase reactants. Persisting inflammation in inadequately treated disease is associated with the development of secondary amyloidosis. The main treatment is colchicine. A number of other monogenic autoinflammatory diseases have also been identified. Among them cryopyrin-associated periodic syndrome (CAPS) is outstanding with its clinical features and the predilection to develop secondary amyloidosis in untreated cases. The treatment of secondary amyloidosis mainly depends on the treatment of the disease. However, a number of new treatments for amyloid per se are in the pipeline.

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Title: Renal amyloidosis in children
Authors: Yelda Bilginer
Tekin Akpolat
Seza Ozen
Publication date: 01-08-2011
Publisher: Springer Berlin Heidelberg
Published in: Pediatric Nephrology / Issue 8/2011
Print ISSN: 0931-041X
Electronic ISSN: 1432-198X
DOI: https://doi.org/10.1007/s00467-011-1797-x

At a glance: The STEP trials

Springer Medicine

Renal amyloidosis in children

Abstract

At a glance: The STEP trials

Springer Medicine

Abstract

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