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Modified Atkins ketogenic diet improves heart and skeletal muscle function in glycogen storage disease type III.
Acta Myol 2019; 38(1):17-20AM

Abstract

Glycogen storage disease type III (GSDIII) management in adult patients includes a high-protein diet with cornstarch supplementation to maintain a normal level of glucose in the blood. This regimen can prevent hypoglycaemia but does not seem to improve skeletal muscle and heart function. A 34 years-old patient with GSD IIIa with hypertrophic cardiomyopathy was then treated with a modified Atkins ketogenic diet. After 12 months of treatment ejection fraction raised from 30 to 45%, liver enzymes were reduced and CK plasma level dropped from 568 to 327 U/l. Physical activity increased from about 1300 to 2800 steps per day and health-related quality of life assessment ameliorated. An increase in uric acid triglycerides plasma level was observed. This data obtained in an adult patient confirm previous reports evidencing the effectiveness of ketogenic diets in improving cardiac and muscular manifestations in children with GSDIII.

Authors+Show Affiliations

Department of Medicine, University of Padua, Italy.Department of Medicine, University of Padua, Italy.Department of Medicine, University of Padua, Italy.

Pub Type(s)

Journal Article

Language

eng

PubMed ID

31309177

Citation

Francini-Pesenti, Francesco, et al. "Modified Atkins Ketogenic Diet Improves Heart and Skeletal Muscle Function in Glycogen Storage Disease Type III." Acta Myologica : Myopathies and Cardiomyopathies : Official Journal of the Mediterranean Society of Myology, vol. 38, no. 1, 2019, pp. 17-20.
Francini-Pesenti F, Tresso S, Vitturi N. Modified Atkins ketogenic diet improves heart and skeletal muscle function in glycogen storage disease type III. Acta Myol. 2019;38(1):17-20.
Francini-Pesenti, F., Tresso, S., & Vitturi, N. (2019). Modified Atkins ketogenic diet improves heart and skeletal muscle function in glycogen storage disease type III. Acta Myologica : Myopathies and Cardiomyopathies : Official Journal of the Mediterranean Society of Myology, 38(1), pp. 17-20.
Francini-Pesenti F, Tresso S, Vitturi N. Modified Atkins Ketogenic Diet Improves Heart and Skeletal Muscle Function in Glycogen Storage Disease Type III. Acta Myol. 2019;38(1):17-20. PubMed PMID: 31309177.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Modified Atkins ketogenic diet improves heart and skeletal muscle function in glycogen storage disease type III. AU - Francini-Pesenti,Francesco, AU - Tresso,Silvia, AU - Vitturi,Nicola, Y1 - 2019/06/01/ PY - 2019/7/17/entrez PY - 2019/7/17/pubmed PY - 2019/7/17/medline KW - glycogen storage disease type III KW - hypertrophic cardiomyopathy KW - ketogenic diet SP - 17 EP - 20 JF - Acta myologica : myopathies and cardiomyopathies : official journal of the Mediterranean Society of Myology JO - Acta Myol VL - 38 IS - 1 N2 - Glycogen storage disease type III (GSDIII) management in adult patients includes a high-protein diet with cornstarch supplementation to maintain a normal level of glucose in the blood. This regimen can prevent hypoglycaemia but does not seem to improve skeletal muscle and heart function. A 34 years-old patient with GSD IIIa with hypertrophic cardiomyopathy was then treated with a modified Atkins ketogenic diet. After 12 months of treatment ejection fraction raised from 30 to 45%, liver enzymes were reduced and CK plasma level dropped from 568 to 327 U/l. Physical activity increased from about 1300 to 2800 steps per day and health-related quality of life assessment ameliorated. An increase in uric acid triglycerides plasma level was observed. This data obtained in an adult patient confirm previous reports evidencing the effectiveness of ketogenic diets in improving cardiac and muscular manifestations in children with GSDIII. SN - 2532-1900 UR - https://www.unboundmedicine.com/medline/citation/31309177/Modified_Atkins_ketogenic_diet_improves_heart_and_skeletal_muscle_function_in_glycogen_storage_disease_type_III L2 - https://www.ncbi.nlm.nih.gov/pmc/articles/pmid/31309177/ DB - PRIME DP - Unbound Medicine ER -