TY - JOUR
T1 - Dietary lipids in glycogen storage disease type III
T2 - A systematic literature study, case studies, and future recommendations
AU - Rossi, Alessandro
AU - Hoogeveen, Irene J.
AU - Bastek, Vanessa B.
AU - de Boer, Foekje
AU - Montanari, Chiara
AU - Meyer, Uta
AU - Maiorana, Arianna
AU - Bordugo, Andrea
AU - Dianin, Alice
AU - Campana, Carmen
AU - Rigoldi, Miriam
AU - Kishnani, Priya S.
AU - Pendyal, Surekha
AU - Strisciuglio, Pietro
AU - Gasperini, Serena
AU - Parenti, Giancarlo
AU - Parini, Rossella
AU - Paci, Sabrina
AU - Melis, Daniela
AU - Derks, Terry G.J.
N1 - Funding Information:
The authors would like to thank Margreet van Rijn, metabolic dietician from Groningen who was involved in the initiation of this project. The authors would also like to acknowledge Ellen Wagenaar who was responsible for the organisation of the dietary networking session at the IGSD2017. M.R., S.G., and R.P. gratefully acknowledge Roberta Pretese, metabolic dietician in Monza, who thoroughly followed all GSDIII patients of the centre. This project was funded by Junior Scientific Masterclass by University Medical Center Groningen (MD‐PhD 15‐16 grant to I.J.H. and Dr T.G.J.D.). The stay of A.R. at University of Groningen was financially supported by University of Naples “Federico II” and Compagnia di San Paolo, in the frame of Programme STAR.
Publisher Copyright:
© 2020 The Authors. Journal of Inherited Metabolic Disease published by John Wiley & Sons Ltd on behalf of SSIEM
PY - 2020/7/1
Y1 - 2020/7/1
N2 - A potential role of dietary lipids in the management of hepatic glycogen storage diseases (GSDs) has been proposed, but no consensus on management guidelines exists. The aim of this study was to describe current experiences with dietary lipid manipulations in hepatic GSD patients. An international study was set up to identify published and unpublished cases describing hepatic GSD patients with a dietary lipid manipulation. A literature search was performed according to the Cochrane Collaboration methodology through PubMed and EMBASE (up to December 2018). All delegates who attended the dietetics session at the IGSD2017, Groningen were invited to share unpublished cases. Due to multiple biases, only data on GSDIII were presented. A total of 28 cases with GSDIII and a dietary lipid manipulation were identified. Main indications were cardiomyopathy and/or myopathy. A high fat diet was the most common dietary lipid manipulation. A decline in creatine kinase concentrations (n = 19, P <.001) and a decrease in cardiac hypertrophy in paediatric GSDIIIa patients (n = 7, P <.01) were observed after the introduction with a high fat diet. This study presents an international cohort of GSDIII patients with different dietary lipid manipulations. High fat diet may be beneficial in paediatric GSDIIIa patients with cardiac hypertrophy, but careful long-term monitoring for potential complications is warranted, such as growth restriction, liver inflammation, and hepatocellular carcinoma development.
AB - A potential role of dietary lipids in the management of hepatic glycogen storage diseases (GSDs) has been proposed, but no consensus on management guidelines exists. The aim of this study was to describe current experiences with dietary lipid manipulations in hepatic GSD patients. An international study was set up to identify published and unpublished cases describing hepatic GSD patients with a dietary lipid manipulation. A literature search was performed according to the Cochrane Collaboration methodology through PubMed and EMBASE (up to December 2018). All delegates who attended the dietetics session at the IGSD2017, Groningen were invited to share unpublished cases. Due to multiple biases, only data on GSDIII were presented. A total of 28 cases with GSDIII and a dietary lipid manipulation were identified. Main indications were cardiomyopathy and/or myopathy. A high fat diet was the most common dietary lipid manipulation. A decline in creatine kinase concentrations (n = 19, P <.001) and a decrease in cardiac hypertrophy in paediatric GSDIIIa patients (n = 7, P <.01) were observed after the introduction with a high fat diet. This study presents an international cohort of GSDIII patients with different dietary lipid manipulations. High fat diet may be beneficial in paediatric GSDIIIa patients with cardiac hypertrophy, but careful long-term monitoring for potential complications is warranted, such as growth restriction, liver inflammation, and hepatocellular carcinoma development.
KW - dietary intervention
KW - glycogen storage diseases
KW - high fat
KW - medium-chain triglycerides
KW - metabolic control
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U2 - 10.1002/jimd.12224
DO - 10.1002/jimd.12224
M3 - Article
C2 - 32064649
AN - SCOPUS:85080101501
VL - 43
SP - 770
EP - 777
JO - Journal of Inherited Metabolic Disease
JF - Journal of Inherited Metabolic Disease
SN - 0141-8955
IS - 4
ER -