Clearance of amino acids by hemodialysis in argininosuccinate synthetase deficiency.
Clearance of amino acids by hemodialysis in argininosuccinate synthetase deficiency.
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精氨酸琥珀酸合成酶缺乏症通过血液透析清除氨基酸。
DOI：
10.1016/j.jpeds.2004.01.037
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发表时间：
2004
期刊：
影响因子：
--
通讯作者：
Smoyer,WilliamE
中科院分区：
文献类型：
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作者：
McBryde,KevinD;Kudelka,TimothyL;Kershaw,DavidB;Brophy,PatrickD;Gardner,JohnJ;Smoyer,WilliamE
我们测定了一名精氨酸琥珀酸合成酶缺乏症新生儿在接受急性血液透析后，透析中与氨化和氮排泄有关的氨基酸的清除。在基线、血液透析期间30分钟间隔和血液透析完成后30分钟测量血氨、血浆和透析液氨基酸浓度。在90分钟的血液透析治疗中，血氨浓度下降了56%，而精氨酸、瓜氨酸、谷氨酰胺和甘氨酸的浓度分别下降了65%、55%、40%和34%。精氨酸、瓜氨酸、谷氨酰胺和甘氨酸的平均透析量分别为24、282、263和189毫升/分钟/1.73平方米。瓜氨酸的高透析率提示了一种新的血液透析脱氮机制。透析清除谷氨酰胺和甘氨酸可防止高氨血症患者的进一步氨化。然而，我们的数据表明，血液透析影响替代途径清除氨的前体。需要进一步的研究来优化基质的透析内和透析间的剂量。
We determined the dialytic clearance of amino acids involved in ammoniagenesis and nitrogen excretion in a neonate with argininosuccinate synthetase deficiency who underwent acute hemodialysis. Plasma ammonia and plasma and dialysate amino acid concentrations were obtained at baseline, 30-minute intervals during hemodialysis, and 30 minutes after the completion of hemodialysis. Plasma ammonia concentrations declined by 56% during the 90-minute hemodialysis treatment, whereas arginine, citrulline, glutamine, and glycine concentrations decreased by 65%, 55%, 40%, and 34%, respectively. Mean dialytic clearances for arginine, citrulline, glutamine, and glycine were 24, 282, 263, and 189 mL/min per 1.73 m2, respectively. The high dialytic clearance of citrulline suggests a novel mechanism of hemodialysis removal of nitrogen. Dialytic clearances of glutamine and glycine may prevent further ammoniagenesis in hyperammonemic patients. However, our data suggest that hemodialysis affects the precursors of alternative pathway removal of ammonia. Further study is needed to optimize the intradialytic and interdialytic dosing of substrates.