Lloyd-Still J D, Powers C
Cystic Fibrosis Center, Children's Memorial Hospital, Northwestern University, Chicago, Illinois 60614.
Acta Univ Carol Med (Praha). 1990;36(1-4):78-80.
Decreased acylcarnitine levels have been found in cord blood of CF infants compared to siblings and controls. We therefore measured carnitine metabolites in blood and urine in 15 newly diagnosed (average age 4 mos) CF infants and followed the levels for one year. No consistent abnormality in carnitine status was detected in newly diagnosed infants; levels normalized at one year after therapy with predigested formula containing carnitine supplements. This data does not provide support for a primary abnormality of carnitine metabolism in CF.
与同胞和对照组相比,在囊性纤维化(CF)婴儿的脐带血中发现酰基肉碱水平降低。因此,我们测量了15名新诊断的(平均年龄4个月)CF婴儿血液和尿液中的肉碱代谢物,并跟踪了一年的水平。在新诊断的婴儿中未检测到肉碱状态的一致异常;在用含有肉碱补充剂的预消化配方奶治疗一年后,水平恢复正常。该数据不支持CF中肉碱代谢的原发性异常。