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X连锁低磷性佝偻病中的肾钙质沉着症:其与治疗、肾功能及生长的关系

Nephrocalcinosis in X-linked hypophosphataemic rickets: its relationship to treatment, kidney function, and growth.

作者信息

Kooh S W, Binet A, Daneman A

机构信息

Department of Paediatrics, Hospital for Sick Children, Toronto, Ontario.

出版信息

Clin Invest Med. 1994 Apr;17(2):123-30.

PMID:8004848
Abstract

We studied 25 patients treated with oral phosphate and 1,25(OH)2 vitamin D for 0.5-15 y (mean, 7 y) to determine the incidence of nephrocalcinosis and its relationship to treatment, renal function, and growth. During the follow-up period, 3 hypercalcaemic and 13 random hypercalciuric episodes were documented. Creatinine clearances and urine concentration tests were normal in all patients. One patient's 24-h urine specimen indicated hypercalciuria. Kidney ultrasonography revealed nephrocalcinosis in 80% of the patients, but its severity was not significantly related to the dose of calcitriol or of phosphate, the duration of treatment, the age at which treatment was started, or growth. None of the 9 untreated affected family members had nephrocalcinosis. Longitudinal studies revealed that the greatest loss in height velocity occurred during the first 2 y of life. Our study shows that nephrocalcinosis is a common complication of phosphate and 1,25(OH)2D3 treatment, but it is not necessarily associated with impaired renal function. Although the treatment failed to prevent a decrease in height velocity during infancy, it effectively maintained height velocity after 2 y of age.

摘要

我们研究了25例接受口服磷酸盐和1,25(OH)₂维生素D治疗0.5 - 15年(平均7年)的患者,以确定肾钙质沉着症的发生率及其与治疗、肾功能和生长的关系。在随访期间,记录到3次高钙血症发作和13次随机高钙尿症发作。所有患者的肌酐清除率和尿液浓缩试验均正常。1例患者的24小时尿标本显示高钙尿症。肾脏超声检查显示80%的患者有肾钙质沉着症,但其严重程度与骨化三醇或磷酸盐的剂量、治疗持续时间、开始治疗的年龄或生长情况均无显著相关性。9名未经治疗的患病家庭成员均无肾钙质沉着症。纵向研究表明,身高增长速度下降最大的时期发生在生命的头2年。我们的研究表明,肾钙质沉着症是磷酸盐和1,25(OH)₂D₃治疗的常见并发症,但不一定与肾功能受损有关。尽管该治疗未能防止婴儿期身高增长速度下降,但在2岁以后能有效维持身高增长速度。

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Nephrocalcinosis in X-linked hypophosphataemic rickets: its relationship to treatment, kidney function, and growth.X连锁低磷性佝偻病中的肾钙质沉着症:其与治疗、肾功能及生长的关系
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