Velázquez A, Martín-del-Campo C, Báez A, Zamudio S, Quiterio M, Aguilar J L, Pérez-Ortiz B, Sánchez-Ardines M, Guzmán-Hernández J, Casanueva E
Unidad de Genética de la Nutrición, Universidad Nacional Autónoma de México.
Eur J Clin Nutr. 1989 Mar;43(3):169-73.
Several of the clinical and biochemical manifestations of biotin deficiency also occur in severe protein-energy malnutrition (PEM). Average plasma biotin concentrations were lower in 16 malnourished children (10 with marasmus, 3 with kwashiorkor and 3 with marasmic kwashiorkor) than in 31 controls. Lymphocyte mitochondrial carboxylase activities were studied in 11 controls and in 10 patients with PEM; on the average, they were lower in the patients. Their activation indices (the ratio of enzyme activity in cells incubated with biotin to activity in cells incubated without the vitamin) were higher in PEM. All these differences were statistically significant. None of these parameters were age-dependent in a range between 3 and 72 months. Carboxylase activities and plasma biotin levels increased to normal during nutritional recovery in two malnourished patients who were further studied. These results suggest that there is biotin deficiency in severe PEM. Urinary biotin concentrations, expressed per g of creatinine, were higher in the patients than in the controls; this may have been caused by increased renal clearance or by the reduced creatinine excretion which occurs in malnourished individuals. It will be important in future studies to determine the relative contribution of biotin deficiency to the malnourished phenotype.
生物素缺乏的一些临床和生化表现也出现在严重的蛋白质 - 能量营养不良(PEM)中。16名营养不良儿童(10名消瘦型、3名夸希奥科病型和3名消瘦 - 夸希奥科病混合型)的平均血浆生物素浓度低于31名对照组儿童。对11名对照组和10名PEM患者的淋巴细胞线粒体羧化酶活性进行了研究;平均而言,患者的活性较低。PEM患者的激活指数(与添加生物素孵育的细胞中酶活性与未添加该维生素孵育的细胞中酶活性的比值)较高。所有这些差异均具有统计学意义。在3至72个月的范围内,这些参数均与年龄无关。对两名营养不良患者进行进一步研究发现,在营养恢复过程中,羧化酶活性和血浆生物素水平恢复正常。这些结果表明,严重PEM患者存在生物素缺乏。以每克肌酐表示的尿生物素浓度,患者高于对照组;这可能是由于肾脏清除率增加或营养不良个体中肌酐排泄减少所致。在未来的研究中,确定生物素缺乏对营养不良表型的相对影响将很重要。