Urinary Sediment Dolichol Excretion in Patients with Batten Disease and Other Neurodegenerative and Storage Disorders
Urinary Sediment Dolichol Excretion in Patients with Batten Disease and Other Neurodegenerative and Storage Disorders
复制标题
巴顿病和其他神经退行性和储存障碍患者的尿沉渣多甘醇排泄
DOI：
10.1203/00006450-198502000-00014
复制
发表时间：
1985
影响因子：
3.6
通讯作者：
G. Hosking
中科院分区：
文献类型：
--
作者：
M. Bennett;N. Mathers;F. Hemming;I. Zweije;G. Hosking
摘要：在20例晚期婴儿和青少年形式的神经元蜡样脂褐质沉积症（Batten病），15例其他储存和神经退行性疾病和10例对照组患者的尿沉渣中测定了非酯化的多萜醇。Dolichol通过高效液相色谱法测定，并与尿肌酐浓度相关。与正常受试者或患有其他神经退行性疾病的患者相比，Batten病中的dolichols水平没有显著升高。最高水平分别见于2例粘多糖样沉积症II型和IV型患者。尿沉渣中长胆固醇的测定对诊断Batten病或进一步了解潜在的原发性缺陷价值不大。
ABSTRACT: Nonesterified dolichols have been measured in the urinary sediment of 20 patients with the late infantile and juvenile forms of neuronal ceroid lipofuscinosis (Batten disease), in 15 patients with other storage and neurodegenerative disorders and in 10 control subjects. Dolichols were measured by a high performance liquid chromatographic method and were related to urinary creatinine concentration. The levels of dolichols in Batten disease were not significantly elevated when compared to the normal subjects or to patients with other neurodegenerative disorders. The highest levels seen were in two patients with mucopolysaccharidosis types II and IV, respectively. Measurement of dolichols in urinary sediment is of little value in the diagnosis of Batten disease or in furthering our understanding of the underlying primary defect.