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Multicenter Study
. 2015 Apr;166(4):1079-82.
doi: 10.1016/j.jpeds.2014.12.015. Epub 2015 Jan 29.

Progression of renal damage in glycogen storage disease type I is associated to hyperlipidemia: a multicenter prospective Italian study

Daniela Melis  1 , Mariarosaria Cozzolino  2 , Giorgia Minopoli  2 , Francesca Balivo  2 , Rossella Parini  3 , Miriam Rigoldi  3 , Sabrina Paci  4 , Carlo Dionisi-Vici  5 , Alberto Burlina  6 , Generoso Andria  2 , Giancarlo Parenti  2
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Multicenter Study

Progression of renal damage in glycogen storage disease type I is associated to hyperlipidemia: a multicenter prospective Italian study

Daniela Melis et al. J Pediatr. 2015 Apr.

Abstract

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.

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