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对接受无肉碱和补充肉碱肠外营养的儿科患者血清肉碱水平的评估。

Evaluation of serum carnitine levels for pediatric patients receiving carnitine-free and carnitine-supplemented parenteral nutrition.

作者信息

Winther Brian, Jackson Daniel, Mulroy Cecilia, MacKay Mark

机构信息

Department of Pharmacy, Primary Children's Hospital , Salt Lake City, Utah.

Department of Gastroenterology, Primary Children's Hospital, and University of Utah School of Medicine , Salt Lake City, Utah.

出版信息

Hosp Pharm. 2014 Jun;49(6):549-53. doi: 10.1310/hpj4906-549.

Abstract

PURPOSE

Carnitine is a carrier molecule transporting long-chain fatty acids (LCFAs) into the mitochondria for fatty acid β-oxidation. The purpose of this study is to evaluate the role of carnitine supplementation in parenteral nutrition (PN) within the pediatric population. Our goal was to determine a weight range for which empiric carnitine supplementation is justified and to determine a weight range at which a carnitine level should first be drawn to confirm a deficiency prior to supplementation. Secondarily, we tried to determine a relationship among carnitine deficiency, hypoglycemia, and hypertriglyceridemia.

METHODS

This was a retrospective observational study to evaluate 2 groups of pediatric patients (weighing 0.68 kg to 60 kg) who were NPO and receiving PN. The first group of patients (n = 454) received carnitine supplementation (15 mg/kg/day) upon initiation of PN. The second group (n = 299) did not receive carnitine supplementation until they were determined to have a carnitine deficiency.

RESULTS

The data indicated that 82% of the patients weighing less than 5 kg were deficient. Patients weighing more than 5 kg had serum carnitine levels within the normal range. Therefore, patients receiving PN and weighing less than 5 kg should be supplemented with carnitine. Comparison of triglyceride, glucose, and carnitine showed no statistically significant difference (P = .1936).

CONCLUSION

Patients weighing more than 5 kg should have serum carnitine levels drawn within 7 days to determine whether supplementation is needed. There is no statistical correlation among carnitine deficiency, hypoglycemia, and hypertriglyceridemia.

摘要

目的

肉碱是一种载体分子,可将长链脂肪酸(LCFAs)转运至线粒体进行脂肪酸β氧化。本研究的目的是评估补充肉碱在儿科肠外营养(PN)中的作用。我们的目标是确定一个合理进行经验性肉碱补充的体重范围,并确定一个在补充前应首先检测肉碱水平以确认缺乏的体重范围。其次,我们试图确定肉碱缺乏、低血糖和高甘油三酯血症之间的关系。

方法

这是一项回顾性观察研究,评估两组禁食且接受PN的儿科患者(体重0.68千克至60千克)。第一组患者(n = 454)在开始PN时接受肉碱补充(15毫克/千克/天)。第二组(n = 299)直到被确定存在肉碱缺乏才接受肉碱补充。

结果

数据表明,体重小于5千克的患者中有82%存在缺乏。体重超过5千克的患者血清肉碱水平在正常范围内。因此,接受PN且体重小于5千克的患者应补充肉碱。甘油三酯、葡萄糖和肉碱的比较显示无统计学显著差异(P = 0.1936)。

结论

体重超过5千克的患者应在7天内检测血清肉碱水平,以确定是否需要补充。肉碱缺乏、低血糖和高甘油三酯血症之间无统计学相关性。

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