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Open Access 01-12-2021 | Obesity | Research

Cardiometabolic outcomes in children and adolescents with West syndrome

Authors: Inbar Gilboa, Galit Israeli, Avivit Brener, Michal Yackobovitch-Gavan, Uri Kramer, Shimrit Uliel-Sibony, Yael Lebenthal

Published in: BMC Pediatrics | Issue 1/2021

Abstract

Background

West syndrome is a convulsive disorder of infancy with unique seizures and a characteristic background electroencephalograph pattern. Adrenocorticotropic hormone (ACTH) is effective in spasm cessation, yet metabolic consequences of this therapeutic agent in childhood have not been published.

Methods

In this observational study we explored the cardiometabolic outcomes of 117 children with West syndrome (78 ACTH-treated and 39 non-ACTH-treated) monitored at a single medical center from 1995 to 2019 (median follow-up 7.2 years). Outcomes included the prevalence of cardiometabolic derangements (obesity, hypertension, and dyslipidemia) during infancy (< 2 years), early childhood (2–6 years), and childhood/adolescence (6–18 years).

Results

The rates of metabolic derangements during infancy in the West syndrome cohort were high compared to childhood/adolescence (obesity 27.3 % vs. 3.3 %, [p = 0.010], diastolic hypertension 48.8 % vs. 5.1 % [p < 0.001], hypertriglyceridemia 71 % vs. 40 % [p = 0.008], low high-density lipoprotein cholesterol [HDL-c] 54.2 % vs. 12.9 % [p = 0.001], and elevated triglycerides/HDL-c ratios 62.5 % vs. 12.9 % [p < 0.001]). The proportion of systolic and/or diastolic blood pressure levels categorized as hypertensive was 58.5 % during infancy, 48.1 % during early childhood, and 26.3 % during childhood/adolescence. ACTH-treated patients had higher weight and weight-to-length z-scores and higher triglyceride levels during infancy compared to non-ACTH-treated patients (p = 0.008, p = 0.001, and p = 0.037, respectively), and higher triglyceride levels during early childhood (p = 0.050), with no significant group differences during childhood/adolescence.

Conclusions

Children with West syndrome apparently have an increased prevalence of cardiometabolic derangements more pronounced in infants and in ACTH-treated patients. These findings highlight the need to monitor these children for cardiometabolic derangements, even though these cardiometabolic abnormalities are transitory and tend to decrease with time. The health implications of cardiometabolic derangements during critical windows of growth and development warrant further investigation.

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Title: Cardiometabolic outcomes in children and adolescents with West syndrome
Authors: Inbar Gilboa
Galit Israeli
Avivit Brener
Michal Yackobovitch-Gavan
Uri Kramer
Shimrit Uliel-Sibony
Yael Lebenthal
Publication date: 01-12-2021
Publisher: BioMed Central
Keywords: Obesity
Obesity
Dyslipidemia
Salaam-Tic
Hypertension
Hypertension
Published in: BMC Pediatrics / Issue 1/2021
Electronic ISSN: 1471-2431
DOI: https://doi.org/10.1186/s12887-021-02871-1

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Cardiometabolic outcomes in children and adolescents with West syndrome

Abstract

Background

Methods

Results

Conclusions

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Abstract

Background

Methods

Results

Conclusions

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