Failure to Thrive in Children with Primary Distal Type Renal Tubular Acidosis

原發性遠端型腎小管酸血症兒童之生長遲滯

張家堯(Chia-Yau Chang) ； 林清淵(Ching-Yuang Lin)

《Acta Paediatrica Taiwanica》 43卷6期 (2002/12) Pp. 334-339

https://doi.org/10.7097/APT.200212.0334

摘要

原發性遠端型腎小管酸血症用起因於遠端腎小管之缺陷造成酸化尿液困難所致，爲了了解國人原發性腎小管性酸中毒第一型之臨床病程，我們將過去13年間28位有連續追蹤之病人的資料做回顧性的探討及研究，28位中有16位男性，12位女性，平均診斷年齡爲2歲6.8個月大；血尿的比例25位中有5例(20%)；腎髓質鈣化或腎結石的比例21位中有5例(23.8%)；尿中鈣及肌酸肝之比值(Uca/Ucr)在2歲以上平均爲0.313±0.067，在2歲以下平均爲0.262±0.152。僅1位病童由影像學懷疑罹患痀僂症；初診斷時，骨齡檢查平均延遲16個月，診斷時的年齡越大則骨齡的延遲越嚴重；身高百分位平均落後2.81SDS，體重百分位平均落後2.44SDS，生長速度百分位平均落後2.6SDS；病人接受8.5個月至5年7個月一段時間的鹸治療後，身高百分位平均進步至只1.69SDS，體重百分位平均進步至只落後1.10SDS，生長速度百分位平均進步至1.06SDS

關鍵字

遠端型腎小管性酸中毒 ； 生長遲滯 ； 腎髓質鈣化 ； 骨齡 ； 鹼治療

並列摘要

Primary distal type renal tubular acidosis (RTA-typel) 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 -2.44SDS; and the mean value of growth velocity in height was -2.62SDS. 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.

並列關鍵字

renal tubular acidosis ； failure to thrive ； nephrocalcinosis ； bone age ； alkali therapy