Becker K, Leichsenring M, Gana L, Bremer H J, Schirmer R H
University Children's Hospital, Division of Tropical Pediatrics, Heidelberg, Germany.
Free Radic Biol Med. 1995 Feb;18(2):257-63. doi: 10.1016/0891-5849(94)e0131-2.
Marasmus and kwashiorkor are manifestations of protein energy malnutrition. The pathophysiology of these disorders is poorly understood. We studied a number of blood antioxidants [glucose-6-phosphate dehydrogenase (G6PDH), glutathione reductase (GR) and its cofactor flavin adenine dinucleotide (FAD), the tripeptide glutathione as the major nonprotein thiol], serum albumin, and retinol-binding protein in 12 children suffering from kwashiorkor with all classical symptoms, in 13 patients with clinically severe marasmus, in 19 marasmic but active children, and in 23 controls. Significant changes were observed for erythrocyte glutathione and correspondingly for nonprotein thiols in whole blood (0.72 +/- 0.29 mM thiols in controls, 0.50 +/- 0.22 mM in marasmus, 0.35 +/- 0.23 mM in severe marasmus, and 0.22 +/- 0.13 mM in kwashiorkor). These differences were paralleled by a decrease in serum albumin concentration so that the molar ratio of nonprotein thiols/albumin had an average value of approximately 1.5 in all groups. The erythrocyte glutathione-reducing system, represented by G6PDH and glutathione reductase, showed only slight differences among the four groups of children; the supposition that kwashiorkor occurs predominantly in children with aberrant G6PDH could not be substantiated. Unexpectedly, erythrocyte FAD, an index of riboflavin status, was normal in most malnourished patients. Discussed is the prospect of administering glutathione in kwashiorkor patients.
消瘦症和夸休可尔症是蛋白质能量营养不良的表现形式。人们对这些疾病的病理生理学了解甚少。我们研究了12名患有夸休可尔症且有所有典型症状的儿童、13名临床症状严重的消瘦症患者、19名消瘦但活跃的儿童以及23名对照者的多种血液抗氧化剂（葡萄糖-6-磷酸脱氢酶（G6PDH）、谷胱甘肽还原酶（GR）及其辅因子黄素腺嘌呤二核苷酸（FAD）、作为主要非蛋白硫醇的三肽谷胱甘肽）、血清白蛋白和视黄醇结合蛋白。观察到红细胞谷胱甘肽以及全血中非蛋白硫醇有显著变化（对照组中硫醇为0.72±0.29 mM，消瘦症患者中为0.50±0.22 mM，严重消瘦症患者中为0.35±0.23 mM，夸休可尔症患者中为0.22±0.13 mM）。这些差异与血清白蛋白浓度的降低平行，因此所有组中非蛋白硫醇/白蛋白的摩尔比平均值约为1.5。以G6PDH和谷胱甘肽还原酶为代表的红细胞谷胱甘肽还原系统在四组儿童中仅显示出轻微差异；夸休可尔症主要发生在G6PDH异常的儿童中的假设无法得到证实。出乎意料的是，作为核黄素状态指标的红细胞FAD在大多数营养不良患者中是正常的。文中讨论了给夸休可尔症患者施用谷胱甘肽的前景。
