一名5岁女孩患髓质海绵肾并伴有远端肾小管酸中毒。
Medullary sponge kidney associated with distal renal tubular acidosis in a 5-year-old girl.
作者信息
Kasap Belde, Soylu Alper, Oren Oğuz, Türkmen Mehmet, Kavukçu Salih
机构信息
Department of Pediatrics, Dokuz Eylül University, Inciralti, Izmir, Turkey.
出版信息
Eur J Pediatr. 2006 Sep;165(9):648-51. doi: 10.1007/s00431-006-0125-0. Epub 2006 Apr 7.
INTRODUCTION
Medullary sponge kidney (MSK) is characterized by cystic dilatation of the inner medullary collecting ducts, which causes the kidneys to resemble a sponge.
CASE REPORT
Although distal renal tubular acidosis (dRTA) is commonly observed in patients with MSK, we report a 5-year-old girl with MSK who had features of both dRTA (nephrocalcinosis, hypercalciuria, hypocitraturia) and proximal tubular dysfunction (hyperuricosuria, impaired tubular phosphate reabsorption and proteinuria).
DISCUSSION
Metabolic acidosis, hypercalciuria, hypocitraturia, tubular phosphate reabsorption and growth retardation in the patient improved with alkali therapy.
引言
髓质海绵肾（MSK）的特征是髓质内集合管的囊性扩张，这使得肾脏类似海绵。
病例报告
虽然远端肾小管酸中毒（dRTA）在MSK患者中很常见，但我们报告了一名5岁患有MSK的女孩，她同时具有dRTA（肾钙质沉着、高钙尿症、低枸橼酸尿症）和近端肾小管功能障碍（高尿酸尿症、肾小管磷重吸收受损和蛋白尿）的特征。
讨论
该患者的代谢性酸中毒、高钙尿症、低枸橼酸尿症、肾小管磷重吸收及生长发育迟缓通过碱疗法得到改善。
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