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Pediatr Nephrol (2006) 21:308317 DOI 10.1007/s00467-005-2085-4
REVIEW
Brook Belay Nora Esteban-Cruciani
Christine A. Walsh Frederick J. Kaskel
The use of levo-carnitine in children with renal disease: a review and a call for future studies
Received: 30 May 2005 / Revised: 3 August 2005 / Accepted: 3 August 2005 / Published online: 23 December 2005 IPNA 2005
Abstract Carnitine is an amino acid derivative that has a key role in the regulation of fatty acid metabolism and ATP formation. Carnitine deficiency has been described in various conditions, including chronic kidney disease (CKD) and end stage renal disease (ESRD). The deficiency of this micronutrient is postulated to lead to adverse effects across multiple organ systems. There is a paucity of information on carnitine deficiency and its effects in the pediatric CKD and ESRD populations. Currently, there is no evidence supporting the routine use of carnitine supplementation in children with ESRD. In this article, we review the pathophysiology, pharmacokinetics and the potential effects of levo-carnitine supplementation with a focus on the pediatric CKD and ESRD populations. Finally, potential future directions of research are discussed.
Keywords Carnitine deficiency Children Kidney disease Anemia Cardiac disease
Introduction
Carnitine plays a crucial role in fatty-acid metabolism and energy production in multiple organs. Carnitine is produced mainly in the liver and kidneys and is actively stored in cardiac and skeletal muscle, liver and red blood cells [1]. It transports activated long-chain fatty acids into the mitochondria for beta-oxidation and energy production.
Secondary carnitine deficiency has been described in chronic illnesses, including chronic kidney disease (CKD) and end-stage renal disease (ESRD). Because of carnitines physiological role, its deficiency can manifest in multiple ways. This article will briefly review the metabolism and pathophysiology of carnitine. Subsequently, the pharmacokinetics and effects of levo-carnitine supplementation in ESRD will be considered. The focus of this discussion will be on carnitine deficiency and its treatment in children with renal disorders. Since there is a paucity of information available on carnitine deficiency in children with CKD and ESRD, this article will also review current information in the adult CKD and ESRD populations. It is hoped that such a review will serve to focus and stimulate further research in children.
Metabolism and pathophysiology
Recently, the metabolism and physiology of carnitine were...