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Cardiac Manifestations in Children with Inborn Errors of Metabolism.

ΤίτλοςCardiac Manifestations in Children with Inborn Errors of Metabolism.
Publication TypeJournal Article
Year of Publication2017
AuthorsPapadopoulou-Legbelou, K., Gogou M., & Evangeliou A.
JournalIndian Pediatr
Volume54
Issue8
Pagination667-673
Date Published2017 Aug 15
ISSN0974-7559
Λέξεις κλειδιάCardiomyopathies, Child, Preschool, Humans, Metabolism, Inborn Errors
Abstract

NEED AND PURPOSE: Cardiac involvement is a part of many inborn errors of metabolism, but has not been systematically studied. This review focuses on studies describing cardiac manifestations of inborn errors of metabolism in childhood.METHODS: Two independent reviewers searched the topic using PubMed database. Studies published within 20 years were considered, without applying any restrictions related to study design. Despite the small number of existing systematic studies on the topic, several case series/reports were identified.CONCLUSIONS: Cardiomyopathy is the most frequent heart disorder in most metabolic defects. Heart rhythm disorders are mainly encountered in mitochondrial disorders and acidemias, whereas valvular dysfunction is a prominent finding in storage disorders. Cardiac involvement in mitochondrial disorders, congenital disorders of glycosylation and acidemias usually constitute an early symptom. On the contrary, in storage disorders, heart problems are revealed in later stages during routine multisystemic evaluation, with the exception of Pompe disease. As a variety of cardiac manifestations can be found in inborn errors of metabolism, these children should be systematically screened for heart problems during their follow-up.

Alternate JournalIndian Pediatr
PubMed ID28891481

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