Progression of Renal Damage in Glycogen Storage Disease Type I Is Associated to Hyperlipidemia: A Multicenter Prospective Italian Study

• Published in: The Journal of pediatrics Vol. 166; no. 4; pp. 1079 - 1082
• Main Authors: Melis, Daniela, MD, PhD, Cozzolino, Mariarosaria, MD, Minopoli, Giorgia, MD, Balivo, Francesca, MD, Parini, Rossella, MD, Rigoldi, Miriam, MD, Paci, Sabrina, MD, Dionisi-Vici, Carlo, MD, Burlina, Alberto, MD, Andria, Generoso, MD, PhD, Parenti, Giancarlo, MD, PhD
• Format: Journal Article
• Language: English
• Published: United States Elsevier Inc 01.04.2015
• Subjects: Adolescent; Adult; Angiotensin-Converting Enzyme Inhibitors - therapeutic use; Child; Disease Progression; Female; Follow-Up Studies; Forecasting; Glomerular Filtration Rate - drug effects; Glycogen Storage Disease Type I - blood; Glycogen Storage Disease Type I - complications; Glycogen Storage Disease Type I - drug therapy; Humans; Hyperlipidemias - blood; Hyperlipidemias - complications; Hyperlipidemias - drug therapy; Hypolipidemic Agents - therapeutic use; Incidence; Italy - epidemiology; Kidney Diseases - diagnosis; Kidney Diseases - epidemiology; Kidney Diseases - etiology; Lipids - blood; Male; Pediatrics; Prospective Studies; Retrospective Studies; Young Adult; Italy; GSDI; TGF; ACE; Glycogen storage disease type I subtype b; Transforming growth factor; Angiotensin converting enzyme; Diabetic nephropathy; DN; Glycogen storage disease type I subtype a; Glycogen storage disease type I; GSDIb; GSDIa
• ISSN: 0022-3476; 1097-6833
• DOI: 10.1016/j.jpeds.2014.12.015

Angiotensin converting enzyme (ACE)-inhibitors decrease glomerular hyperfiltration but not microalbuminuria and proteinuria in glycogen storage disease type I. In the current study, we demonstrated that severe hyperlipidemia is associated with ACE-inhibitor ineffectiveness. We underline the importance of adequate metabolic control in glycogen storage disease type I. A combination therapy with ACE-inhibitors and lipid lowering drugs might be considered.