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. (J Pediatr 2015;-:---).