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慢性肾衰竭患儿的肾性骨营养不良:一种出人意料地常见且致残的并发症。

Renal osteodystrophy in children with chronic renal failure: an unexpectedly common and incapactating complication.

作者信息

Hsu A C, Kooh S W, Fraser D, Cumming W A, Fornasier V L

出版信息

Pediatrics. 1982 Nov;70(5):742-50.

PMID:6982452
Abstract

The incidence, age at onset, and progression of the biochemical, radiographic, and histologic characteristics of renal osteodystrophy were studied in 50 children in whom chronic renal failure had been recently diagnosed. During a ten-year observation period, 19 patients progressed to end-stage renal failure and radiographic signs of renal osteodystrophy developed in 15 of these (79%). Renal osteodystrophy developed in all nine patients whose chronic renal failure was diagnosed before 3 years of age and in six of the ten children with later onset of failure. The mean interval from diagnosis of renal failure to development of osteodystrophy was 1.4 years. Radiographically, growth zone lesions predominated in the younger children, whereas cortical erosions were more prevalent in the older children. Histologic examination, performed in 38 patients, showed both defective mineralization and excessive resorption and was a more sensitive diagnostic index than radiography. Noticeable deformities developed in one third of the patients with osteodystrophy, despite medical treatment including vitamin D2 therapy. Deformities were particularly frequent and severe in patients whose renal failure developed in infancy. In all 13 patients whose growth patterns were studied before and after osteodystrophy developed, the onset of bone lesions was associated with a deterioration of growth, indicating that osteodystrophy plays a major role in causing the growth retardation commonly observed in children with chronic renal failure.

摘要

对50例近期诊断为慢性肾衰竭的儿童,研究了肾性骨营养不良的生化、影像学和组织学特征的发病率、发病年龄及进展情况。在为期10年的观察期内,19例患者进展至终末期肾衰竭,其中15例(79%)出现了肾性骨营养不良的影像学表现。在9例3岁前诊断为慢性肾衰竭的患儿以及10例发病较晚的患儿中的6例中,均出现了肾性骨营养不良。从肾衰竭诊断到骨营养不良出现的平均间隔时间为1.4年。影像学上,年幼患儿以生长带病变为主,而年长患儿皮质侵蚀更为常见。对38例患者进行的组织学检查显示,既有矿化缺陷又有过度吸收,且是比放射学更敏感的诊断指标。尽管进行了包括维生素D2治疗在内的医学治疗,仍有三分之一的骨营养不良患者出现了明显畸形。畸形在婴儿期发生肾衰竭的患者中尤为常见和严重。在对13例骨营养不良发生前后生长模式进行研究的所有患者中,骨病变的出现与生长恶化相关,这表明骨营养不良在导致慢性肾衰竭儿童中常见的生长迟缓方面起主要作用。

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