Normal serum alanine concentration differentiates transient neonatal lactic acidemia from an inborn error of energy metabolism.

Biology of the neonate Pub Date : 2006-01-01 Epub Date: 2006-05-29 DOI:10.1159/000093590
Eva Morava, Marije Hogeveen, Maaike De Vries, Wim Ruitenbeek, Willem-Pieter de Boode, Jan Smeitink
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引用次数: 9

Abstract

Background: Elevated blood lactate levels are common in the critically ill neonate; however, sometimes they are difficult to interpret. Persistent or recurrent lactic acidemia might point to an inborn error of metabolism, like disturbances of the oxidative phosphorylation. Chronic lactic acidemia results in increased serum alanine levels. Serum alanine levels in newborns with transient lactic acidemia have not yet been studied.

Objective: We designed a pilot study to evaluate the use of serum alanine levels as an additional metabolic marker to differentiate the transient effect of circulatory failure from a possible mitochondrial dysfunction.

Methods: We prospectively evaluated 10 newborns with transient lactic acidemia after mild dysoxia, and 10 newborns with recurrent lactic acidemia consecutively diagnosed with a disorder in oxidative phosphorylation.

Results: No significant serum alanine level elevation was found in transient lactic acidemia. Increased serum alanine was a sensitive marker in mitochondrial dysfunction.

Conclusions: We propose to measure the serum alanine level in hypotonic newborns with lactic acidemia to facilitate the decision making in further diagnostics and management.

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正常血清丙氨酸浓度可区分短暂性新生儿乳酸血症和先天性能量代谢错误。
背景:血乳酸水平升高在危重新生儿中很常见;然而,有时它们很难解释。持续性或复发性乳酸血症可能指向先天性代谢错误,如氧化磷酸化紊乱。慢性乳酸血症导致血清丙氨酸水平升高。新生儿短暂性乳酸血症的血清丙氨酸水平尚未研究。目的:我们设计了一项初步研究,以评估血清丙氨酸水平作为一种额外的代谢标志物,以区分循环衰竭的短暂影响和可能的线粒体功能障碍。方法:对10例轻度缺氧后出现短暂性乳酸血症的新生儿和10例连续诊断为氧化磷酸化障碍的复发性乳酸血症新生儿进行前瞻性评估。结果:短暂性乳酸血症患者血清丙氨酸水平未见明显升高。血清丙氨酸升高是线粒体功能障碍的敏感标志物。结论:我们建议在低渗新生儿乳酸血症中检测血清丙氨酸水平,以便于进一步诊断和治疗决策。
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