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经典型肾小管酸中毒婴幼儿及儿童碱疗法对正常身高的获得与维持

Attainment and maintenance of normal stature with alkali therapy in infants and children with classic renal tubular acidosis.

作者信息

McSherry E, Morris R C

出版信息

J Clin Invest. 1978 Feb;61(2):509-27. doi: 10.1172/JCI108962.

Abstract

Growth was evaluated in a group of 10 infants and children with familial or idiopathic classic renal tubular acidosis in whom alkali therapy was initiated at ages ranging from 8 days to 9.5 yr and administered at dosage schedules documented to sustain correction of acidosis in at least four prolonged observation periods on the Pediatric Clinical Research Ward. When alkali therapy was begun, six patients (four infants and two children) were stunted (height <2.5 SD below mean). Of the four who were not, two infants were too young (<2 wk of age) to have become stunted, and two children had been documented earlier to be nonacidotic. At the start of alkali therapy, the heights of the patients correlated inversely with the maximal possible duration of prior acidosis. WITH SUSTAINED ALKALI THERAPY: (a) each patient attained and maintained normal stature; (b) the mean height of the 10 patients increased from the 1.4+/-4 to the 37.0+/-33 percentile (of a normal age- and sex-matched population); (c) the mean height reached the 69th percentile in the eight patients whose heights could be analyzed according to parental prediction (Tanner technique); (d) the rate of growth increased two- to threefold, and normal heights were attained within 6 mo of initiating alkali therapy in the stunted infants and within 3 yr in the stunted children; (e) the height attained correlated inversely with the maximal possible duration of acidosis (before alkali therapy) only in those patients in whom alkali therapy was started after 6 mo of age, and not in those treated earlier. The amount of alkali required to sustain correction of acidosis increased substantially during the course of treatment in each patient. The maximal alkali requirement ranged from 4.8 to 14.1 meq/kg per day, and in each patient its amount was determined principally by the magnitude of renal bicarbonate wasting.

摘要

对一组10名患有家族性或特发性经典肾小管酸中毒的婴幼儿及儿童进行生长情况评估。这些患儿开始接受碱疗法的年龄在8天至9.5岁之间,碱疗法的给药方案是根据儿科临床研究病房至少四个延长观察期内维持酸中毒纠正的记录制定的。开始碱疗法时,6名患者(4名婴儿和2名儿童)发育迟缓(身高低于平均水平2.5个标准差)。另外4名未发育迟缓的患者中,2名婴儿因年龄太小(<2周龄)尚未出现发育迟缓,2名儿童此前已被记录为非酸中毒状态。开始碱疗法时,患者的身高与先前酸中毒的最长可能持续时间呈负相关。持续进行碱疗法后:(a) 每名患者均达到并维持了正常身高;(b) 这10名患者的平均身高从第1.4±4百分位升至第37.0±33百分位(与正常年龄和性别匹配人群相比);(c) 根据父母预测(坦纳技术)可分析身高的8名患者,其平均身高达到了第69百分位;(d) 生长速率提高了两到三倍,发育迟缓的婴儿在开始碱疗法后6个月内、发育迟缓的儿童在3年内达到了正常身高;(e) 只有在6个月龄后开始碱疗法的患者中,达到的身高才与酸中毒(碱疗法前)的最长可能持续时间呈负相关,而早期接受治疗的患者则不然。在每位患者的治疗过程中,维持酸中毒纠正所需的碱量大幅增加。最大碱需求量在每天4.8至14.1 mEq/kg之间,且每位患者的碱需求量主要由肾碳酸氢盐流失量决定。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7dc0/372562/debb635a870a/jcinvest00662-0292-a.jpg

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