Belay Brook, Esteban-Cruciani Nora, Walsh Christine A, Kaskel Frederick J
Department of Pediatrics, Temple University Children's Medical Center, Philadelphia, PA 19140, USA.
Pediatr Nephrol. 2006 Mar;21(3):308-17. doi: 10.1007/s00467-005-2085-4. Epub 2005 Dec 23.
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.
肉碱是一种氨基酸衍生物,在脂肪酸代谢和三磷酸腺苷(ATP)形成的调节中起关键作用。在包括慢性肾脏病(CKD)和终末期肾病(ESRD)在内的多种情况下,均已发现肉碱缺乏。据推测,这种微量营养素的缺乏会对多个器官系统产生不良影响。关于肉碱缺乏及其在儿童CKD和ESRD人群中的影响,目前信息匮乏。目前,尚无证据支持对ESRD患儿常规补充肉碱。在本文中,我们回顾了左旋肉碱补充剂的病理生理学、药代动力学及潜在影响,重点关注儿童CKD和ESRD人群。最后,讨论了未来潜在的研究方向。