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原发性远端型肾小管酸中毒患儿生长发育迟缓

Failure to thrive in children with primary distal type renal tubular acidosis.

作者信息

Chang Chia-Yau, Lin Ching-Yuang

机构信息

Department of Pediatrics, Taipei Veterans General Hospital, Taipei, Taiwan.

出版信息

Acta Paediatr Taiwan. 2002 Nov-Dec;43(6):334-9.

Abstract

Primary distal type renal tubular acidosis (RTA-type1) results from defects of distal renal tubules in urinary acidification. In attempt to evaluate the clinical features, growth and outcome of primary distal type RTA in Taiwan, we retrospectively studied 28 patients (16 males and 12 females) of primary distal type RTA in our hospital in the past 13 years. The mean age at diagnosis was 2 years and 6.8 months old. Hematuria was found in 5 out of 25 cases (20%). Nephrocalcinosis was found in 5 out of 21 cases (23.8%). The mean value of Uca/Ucr was 0.313 +/- 0.067 in those older than 2 years and 0.262 +/- 0.152 in those younger than 2 years. Rickets was suspected in only one child by radiologic study. At initial diagnosis, the mean bone age was delayed for 16 months. The older the patient was, the more delayed was the bone age. The mean value of height was -2.81 standard deviation score (SDS); the mean value of body weight was -2.44 SDS; and the mean value of growth velocity in height was -2.62 SDS. After alkali therapy given from 8.5 months to 5 years and 7 months, improvement was found in height up to -1.69SDS, in body weight up to -1.10SDS, and in height velocity up to 1.06SDS.

摘要

原发性远端型肾小管酸中毒(1型肾小管酸中毒)是由远端肾小管在尿液酸化方面的缺陷引起的。为了评估台湾原发性远端型肾小管酸中毒的临床特征、生长情况及预后,我们回顾性研究了我院过去13年中28例原发性远端型肾小管酸中毒患者(16例男性和12例女性)。诊断时的平均年龄为2岁6.8个月。25例中有5例(20%)发现血尿。21例中有5例(23.8%)发现肾钙质沉着症。2岁以上患者的尿钙/尿肌酐平均值为0.313±0.067,2岁以下患者为0.262±0.152。仅1名儿童经影像学检查怀疑有佝偻病。初诊时,平均骨龄延迟16个月。患者年龄越大,骨龄延迟越明显。身高平均值为-2.81标准差评分(SDS);体重平均值为-2.44 SDS;身高生长速度平均值为-2.62 SDS。在给予8.5个月至5年7个月的碱治疗后,身高改善至-1.69 SDS,体重改善至-1.10 SDS,身高速度改善至1.06 SDS。

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