Continuous Glucose Monitoring in Children with Glycogen Storage Disease Type I

Overview

Journal J Inherit Metab Dis

Publisher Wiley

Date 2002 Mar 28

PMID 11916320

Citations 12

Authors

E Hershkovitz

A Rachmel

M Phillip

Affiliations

Soon will be listed here.

Abstract

Glycogen storage disease type I (GSD I) is characterized by impaired production of glucose from glycogenolysis and gluconeogenesis resulting in severe fasting hypoglycaemia. The aim of the present study was to examine the efficacy of a continuous subcutaneous glucose monitoring system (CGMS MiniMed), to determine the magnitude and significance of hypoglycaemia in GSD I and to evaluate the efficacy of its dietary treatment. Four children with GSD I were studied over a 72-h period. Results indicated that the values recorded with continuous subcutaneous glucose monitoring were highly correlated with paired blood glucose values measured by glucometer. Significant periods of asymptomatic hypoglycaemia were noted, especially during night-time. The study suggests that repeated continuous subcutaneous glucose monitoring may serve as a useful tool for the assessment of the long-term management of GSD I patients.

Citing Articles

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Bedtime extended release cornstarch improves biochemical profile and sleep quality for patients with glycogen storage disease type Ia.

Hsu R, Chen H, Chien Y, Hwu W, Lin J, Weng H Mol Genet Genomic Med. 2023; 11(10):e2221.

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Dynamic Methods for Childhood Hypoglycemia Phenotyping: A Narrative Review.

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Role of continuous glucose monitoring in the management of glycogen storage disorders.

Herbert M, Pendyal S, Rairikar M, Halaby C, Benjamin R, Kishnani P J Inherit Metab Dis. 2018; 41(6):917-927.

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A preliminary study of telemedicine for patients with hepatic glycogen storage disease and their healthcare providers: from bedside to home site monitoring.

Hoogeveen I, Peeks F, de Boer F, Lubout C, De Koning T, Te Boekhorst S J Inherit Metab Dis. 2018; 41(6):929-936.

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