title: Lipids in hepatic glycogen storage diseases: pathophysiology, monitoring of dietary management and future directions.
abstract: Hepatic glycogen storage diseases (GSD) underscore the intimate relationship between carbohydrate and lipid metabolism. The hyperlipidemias in hepatic GSD reflect perturbed intracellular metabolism, providing biomarkers in blood to monitor dietary management. In different types of GSD, hyperlipidemias are of a different origin. Hypertriglyceridemia is most prominent in GSD type Ia and associated with long-term outcome morbidity, like pancreatitis and hepatic adenomas. In the ketotic subtypes of GSD, hypertriglyceridemia reflects the age-dependent fasting intolerance, secondary lipolysis and increased mitochondrial fatty acid oxidation. The role of high protein diets is established for ketotic types of GSD, but non-traditional dietary interventions (like medium-chain triglycerides and the ketogenic diet) in hepatic GSD are still controversial and necessitate further studies. Patients with these rare inherited disorders of carbohydrate metabolism meet several criteria of the metabolic syndrome, therefore close monitoring for cardiovascular diseases in ageing GSD patients may be justified.
authors: Section of Metabolic Diseases, Beatrix Children's Hospital, University of Groningen, University Medical Center Groningen, PO Box 30 001, 9700 RB, Groningen, The Netherlands, t.g.j.derks@umcg.nl.
authors: Terry G J
authors: TG
authors: Derks
authors: Margreet
authors: M
authors: van Rijn
mesh_terms: D015415:Biomarkers; D005953:Glycogen Storage Disease Type I / Q000150:complications* / Q000178:diet therapy; D006801:Humans; D006949:Hyperlipidemias / Q000503:physiopathology*; D015228:Hypertriglyceridemia / Q000503:physiopathology*; D014280:Triglycerides / Q000097:blood*
chemical_list: D015415:Biomarkers; D014280:Triglycerides
references: J Inherit Metab Dis. 2010 Feb;33(1):25-31
references: 20127282
references: Am J Clin Nutr. 1969 May;22(5):617-27
references: 5254184
references: J Inherit Metab Dis. 2011 Jun;34(3):631-42
references: 21556835
references: Arch Dis Child. 2014 Dec;99(12):1098-102
references: 24996789
references: Eur J Pediatr. 2002 Oct;161 Suppl 1:S62-4
references: 12373574
references: J Inherit Metab Dis. 2008 Dec;31(6):729-32
references: 18709545
references: Pediatr Res. 2011 Dec;70(6):638-41
references: 21857385
references: Eur J Pediatr. 2002 Oct;161 Suppl 1:S20-34
references: 12373567
references: J Pediatr. 2009 Apr;154(4):588-91
references: 19101686
references: Eur J Clin Nutr. 2014 Jan;68(1):101-5
references: 24149443
references: JIMD Rep. 2014;17:91-5
references: 25308556
references: J Pediatr. 2011 Sep;159(3):442-6
references: 21481415
references: Pediatr Res. 1969 Mar;3(2):160-70
references: 5253123
references: Appl Spectrosc. 2014;68(4):428-33
references: 24694699
references: Biochem J. 2010 Dec 1;432(2):249-54
references: 20854262
references: Pediatr Res. 2008 Jun;63(6):702-7
references: 18520334
references: J Pediatr. 1974 Dec;85(6):792-5
references: 4213651
references: Pediatr Res. 1984 Sep;18(9):879-81
references: 6384910
references: Pediatrics. 1970 Dec;46(6):925-9
references: 5275632
references: J Hum Nutr Diet. 2013 Aug;26(4):329-39
references: 23294025
references: J Inherit Metab Dis. 2015 May;38(3):545-50
references: 25164784
references: J Inherit Metab Dis. 2001 Dec;24(8):863-9
references: 11916320
references: Mol Genet Metab. 2013 May;109(1):1-2
references: 23480859
references: N Engl J Med. 1976 Feb 19;294(8):423-5
references: 813144
references: Cell Mol Life Sci. 2014 Apr;71(8):1453-67
references: 24196749
references: Eur J Pediatr. 2002 Oct;161 Suppl 1:S112-9
references: 12373584
references: Diabetes. 1977 Nov;26(11):1016-23
references: 913891
references: Ann Nutr Metab. 2010;56(3):225-32
references: 20357432
references: Acta Paediatr Scand. 1974 Sep;63(5):695-8
references: 4528774
references: J Inherit Metab Dis. 2015 May;38(3):521-7
references: 25164786
references: Eur J Pediatr. 2007 Oct;166(10):1009-16
references: 17206455
references: Eur J Pediatr. 1990 Jun;149(9):630-3
references: 2373114
references: Diabetes Metab. 2013 Oct;39(5):377-87
references: 23643353
references: N Engl J Med. 1984 Jan 19;310(3):171-5
references: 6581385
references: Eur J Pediatr. 2002 Oct;161 Suppl 1:S2-9
references: 12373565
references: J Biol Chem. 2001 Jul 13;276(28):25727-35
references: 11346646