Schröder C H, Monnens L A, van Lith-Zanders H M, Trijbels J M, Veerkamp J H, Langeveld J P
Nephron. 1986;44(2):103-7. doi: 10.1159/000183975.
Alport's syndrome probably is a molecular disorder of basement membrane composition. Investigation of urine on basement membrane components such as hydroxylysine and its glycosides, glucosylgalactosylhydroxylysine and galactosylhydroxylysine, may be helpful for diagnosis of the disease. Urinary specimens of 33 patients and 12 siblings were investigated, and the results were compared with those of 14 healthy adults and of 29 healthy children. The urine of patients with glomerulopathies, occurring during childhood (IgA nephropathy, benign recurrent hematuria, poststreptococcal glomerulonephritis, Henoch-Schönlein nephropathy, membranoproliferative glomerulonephritis, and nephrotic syndrome due to minimal lesions), was also investigated. No marked differences between normal and diseased subjects could be demonstrated, with respect to excretion of hydroxylysine and its glycosides, in contrast to data reported in the literature.
阿尔波特综合征可能是一种基底膜成分的分子紊乱疾病。对尿液中基底膜成分如羟赖氨酸及其糖苷、葡萄糖基半乳糖基羟赖氨酸和半乳糖基羟赖氨酸进行检测,可能有助于该疾病的诊断。研究了33例患者和12名亲属的尿液样本,并将结果与14名健康成年人和29名健康儿童的结果进行了比较。还研究了儿童期发生的肾小球疾病(IgA肾病、良性复发性血尿、链球菌感染后肾小球肾炎、过敏性紫癜性肾炎、膜增生性肾小球肾炎和微小病变所致肾病综合征)患者的尿液。与文献报道的数据相反,在羟赖氨酸及其糖苷的排泄方面,正常受试者和患病受试者之间未显示出明显差异。