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原发性草酸尿症:大剂量维生素B6治疗的临床及生化反应

Primary oxalosis: clinical and biochemical response to high-dose pyridoxine therapy.

作者信息

Will E J, Bijvoet O L

出版信息

Metabolism. 1979 May;28(5):542-8. doi: 10.1016/0026-0495(79)90195-1.

Abstract

Although pyridoxine hydrochloride (vitamin B6) is known to reduce the endogenous production of oxalate in some individuals with primary oxalosis, the dose for a satisfactory trial of treatment is not established. We report two cases of primary oxalosis on a daily regimen of 1 g pyridoxine hydrochloride, in which 24-hr urinary oxalate excretion decreased by 60% and 70%, respectively, with corresponding clinical benefit. The responses have been sustained up to 2.5 yr in one case, and 20 mo in the other. In the patient with renal failure, serum creatinine decreased from 243 to 146 mumole/liter after 15 mo of treatment. The decrease in glycollic acid excretion in both patients was consistent with an increase of glyoxalate transaminase activity by the vitamin. Supranormal levels of erythrocyte glutamic oxaloacetate transaminase (egot) activity were observed during therapy, and these may be useful as a measure of the effective dose of pyridoxine.

摘要

尽管已知盐酸吡哆醇(维生素B6)可减少某些原发性草酸血症患者草酸盐的内源性生成,但尚未确定进行满意治疗试验的剂量。我们报告了两例原发性草酸血症患者,他们每日服用1g盐酸吡哆醇,其中24小时尿草酸盐排泄量分别减少了60%和70%,并伴有相应的临床益处。其中一例患者的反应持续了2.5年,另一例持续了20个月。在肾衰竭患者中,治疗15个月后血清肌酐从243微摩尔/升降至146微摩尔/升。两名患者乙醇酸排泄量的减少与维生素使乙醛酸转氨酶活性增加一致。治疗期间观察到红细胞谷草转氨酶(EGOT)活性超正常水平,这些水平可用作衡量吡哆醇有效剂量的指标。

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