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Pediatric Nephrology
Published in: Pediatric Nephrology 9/2022

18-01-2022 | Hemolytic Uremic Syndrome | Review

Thrombotic microangiopathy in children

Authors: Lilian Monteiro P. Palma, Maria Helena Vaisbich-Guimarães, Meera Sridharan, Cheryl L. Tran, Sanjeev Sethi

Published in: Pediatric Nephrology | Issue 9/2022

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Abstract

The syndrome of thrombotic microangiopathy (TMA) is a clinical-pathological entity characterized by microangiopathic hemolytic anemia, thrombocytopenia, and end organ involvement. It comprises a spectrum of underlying etiologies that may differ in children and adults. In children, apart from ruling out shigatoxin-associated hemolytic uremic syndrome (HUS) and other infection-associated TMA like Streptococcus pneumoniae-HUS, rare inherited causes including complement-associated HUS, cobalamin defects, and mutations in diacylglycerol kinase epsilon gene must be investigated. TMA should also be considered in the setting of solid organ or hematopoietic stem cell transplantation. In this review, acquired and inherited causes of TMA are described with a focus on particularities of the main causes of TMA in children. A pragmatic approach that may help the clinician tailor evaluation and management is provided. The described approach will allow for early initiation of treatment while waiting for the definitive diagnosis of the underlying TMA.
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Metadata
Title
Thrombotic microangiopathy in children
Authors
Lilian Monteiro P. Palma
Maria Helena Vaisbich-Guimarães
Meera Sridharan
Cheryl L. Tran
Sanjeev Sethi
Publication date
18-01-2022
Publisher
Springer Berlin Heidelberg
Published in
Pediatric Nephrology / Issue 9/2022
Print ISSN: 0931-041X
Electronic ISSN: 1432-198X
DOI
https://doi.org/10.1007/s00467-021-05370-8

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