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

Melis, Daniela; Cozzolino, Mariarosaria; Minopoli, Giorgia; Balivo, Francesca; Parini, Rossella; Rigoldi, Miriam; Paci, Sabrina; Dionisi-Vici, Carlo et al. · J Pediatr · 2015

prospective_cohort · Level II

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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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