Alexander Robert Todd, Bitzan Martin
Department of Pediatrics and Physiology, Stollery Children's Hospital, 11405-87 Avenue, Edmonton, Alberta T6G 1C9, Canada.
Division of Nephrology, Department of Pediatrics, The Montreal Children's Hospital, McGill University Health Centre, Room B RC.6651, Montreal, Quebec H4A 3J1, Canada; Al Jalila Children's Hospital, Al Jadaf PO Box 7662, Dubai, UAE.
Pediatr Clin North Am. 2019 Feb;66(1):135-157. doi: 10.1016/j.pcl.2018.08.011.
Renal tubular acidosis should be suspected in poorly thriving young children with hyperchloremic and hypokalemic normal anion gap metabolic acidosis, with/without syndromic features. Further workup is needed to determine the type of renal tubular acidosis and the presumed etiopathogenesis, which informs treatment choices and prognosis. The risk of nephrolithiasis and calcinosis is linked to the presence (proximal renal tubular acidosis, negligible stone risk) or absence (distal renal tubular acidosis, high stone risk) of urine citrate excretion. New formulations of slow-release alkali and potassium combination supplements are being tested that are expected to simplify treatment and lead to sustained acidosis correction.
对于患有高氯性、低钾性正常阴离子间隙代谢性酸中毒且有/无综合征特征、生长发育不良的幼儿,应怀疑存在肾小管酸中毒。需要进一步检查以确定肾小管酸中毒的类型及推测的病因发病机制,这对治疗选择和预后判断具有指导意义。肾结石和钙质沉着的风险与尿枸橼酸盐排泄的存在(近端肾小管酸中毒,结石风险可忽略不计)或缺失(远端肾小管酸中毒,结石风险高)有关。正在测试缓释碱和钾联合补充剂的新剂型,有望简化治疗并实现酸中毒的持续纠正。