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青少年肾性骨营养不良的早期诊断

Early diagnosis of juvenile renal osteodystrophy.

作者信息

Norman M E, Mazur A T, Borden S, Gruskin A, Anast C, Baron R, Rasmussen H

出版信息

J Pediatr. 1980 Aug;97(2):226-32. doi: 10.1016/s0022-3476(80)80479-3.

Abstract

Renal osteodystrophy has assumed growing importance as a major and frequently disabling complication of chronic renal failure in children since the advent of successful hemodialysis and renal transplantation programs. The frequency and severity of renal osteodystrophy appears greatest in younger children with congenital diseases of the kidney and urinary tract, who experience long intervals of chronic renal failure prior to reaching end-stage. Twenty-nine children with varying degrees of chronic renal failure were studied to learn: (1) how early renal osteodystrophy can be diagnosed; and (2) how the various clinical, biochemical, and hormonal abnormalities correlate with abnormal bone histomorphometry as determined from percutaneous transilial bone biopsies. Results showed: (1) marked-to-moderate reductions in GFR (mean = 35 ml/minute/1.73 m2; range 11 to 65 ml/minute/1.73 m2); (2) elevations of serum PTH concentrations in all patients with a GFR < 45 ml/minute/1.73 m2; (3) abnormal bone histomorphometry in all patients with elevated PTH concentrations; (4) "early" renal osteodystrophy (elevated PTH concentrations and abnormal bone histomorphometry but normal serum chemistry values and radiographs) in one quarter of the patients; (5) poor correlations of serum chemistry values and radiographs with bone histomorphometry; and (6) a wide range of histologic abnormalities including predominant osteomalacia (n = 7), predominant hyperparathyroidism (n = 6), or a mixed picture (n = 11).

摘要

自从成功开展血液透析和肾移植项目以来,肾性骨营养不良作为儿童慢性肾衰竭的一种主要且常常导致残疾的并发症,其重要性日益凸显。肾性骨营养不良的发生率和严重程度在患有先天性肾脏和尿路疾病的年幼儿童中似乎最高,这些儿童在进入终末期之前会经历较长时间的慢性肾衰竭。对29名不同程度慢性肾衰竭的儿童进行了研究,以了解:(1)肾性骨营养不良能多早被诊断出来;(2)各种临床、生化和激素异常与经皮髂骨穿刺活检确定的骨组织形态计量学异常之间的相关性。结果显示:(1)肾小球滤过率显著至中度降低(平均 = 35毫升/分钟/1.73平方米;范围为11至65毫升/分钟/1.73平方米);(2)所有肾小球滤过率<45毫升/分钟/1.73平方米的患者血清甲状旁腺激素浓度升高;(3)所有甲状旁腺激素浓度升高的患者骨组织形态计量学异常;(4)四分之一的患者出现“早期”肾性骨营养不良(甲状旁腺激素浓度升高和骨组织形态计量学异常,但血清化学值和X线片正常);(5)血清化学值和X线片与骨组织形态计量学之间的相关性较差;(6)组织学异常范围广泛,包括主要为骨软化症(n = 7)、主要为甲状旁腺功能亢进(n = 6)或混合表现(n = 11)。

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