Effect of dietary interventions in the maintenance of normoglycaemia in glycogen storage disease type 1a: a systematic review and meta-analysis

Background Untreated glycogen storage disease (GSD)‐1a patients experience hypoglycaemia and growth retardation. The present study examined the effects of dietary interventions on the maintenance of normoglycaemia. Methods Clinical trials were identified from EMBASE (January 1980 to November 2011),...

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Bibliographic Details
Published in:Journal of human nutrition and dietetics 2013-08, Vol.26 (4), p.329-339
Main Authors: Shah, K. K., O'Dell, S. D.
Format: Article
Language:English
Subjects:
Blood Glucose - metabolism
Cholesterol - blood
clinical nutrition
Dietary Carbohydrates - pharmacology
Dietary Carbohydrates - therapeutic use
dietary intervention
Glucose - pharmacology
Glucose - therapeutic use
Glycogen Storage Disease Type I - blood
Glycogen Storage Disease Type I - complications
Glycogen Storage Disease Type I - diet therapy
Humans
Hypoglycemia - blood
Hypoglycemia - prevention & control
Insulin - blood
Lactic Acid - blood
metabolic disorders
Starch - pharmacology
Starch - therapeutic use
systematic review
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Summary:Background Untreated glycogen storage disease (GSD)‐1a patients experience hypoglycaemia and growth retardation. The present study examined the effects of dietary interventions on the maintenance of normoglycaemia. Methods Clinical trials were identified from EMBASE (January 1980 to November 2011), MEDLINE (January 1948 to November 2011) and the Cochrane Central Register of Controlled Trials (2011, Issue 4). The intermittent administration of uncooked cornstarch was compared with: (i) continuous nocturnal feeding of dextrose; (ii) modified uncooked cornstarch; and (iii) dextrose and an uncooked cornstarch–dextrose mixture. One author extracted the data, and assessed the trial eligibility and risk of bias. Quality assessment and data extraction were conducted and checked independently. Results Of 41 articles retrieved, five controlled trials (49 participants) were identified with follow‐up at 2 days to 14 years. Results from three nonrandomised controlled trials comparing uncooked cornstarch with continuous nocturnal feeding of dextrose were pooled in a meta‐analysis based on a fixed‐effect model. Twenty‐six participants (three trials) receiving uncooked cornstarch showed a significant increase in blood glucose concentration: mean difference (MD) 0.62 mmol L−1 [95% confidence interval (CI) = 0.23–1.00] (P = 0.002), 21 (two trials) increased serum insulin: MD 62.37 pmol L−1 (95% CI = 32.19–92.55) (P 
ISSN:0952-3871
1365-277X
DOI:10.1111/jhn.12030