Kasap Belde, Soylu Alper, Oren Oğuz, Türkmen Mehmet, Kavukçu Salih
Department of Pediatrics, Dokuz Eylül University, Inciralti, Izmir, Turkey.
Eur J Pediatr. 2006 Sep;165(9):648-51. doi: 10.1007/s00431-006-0125-0. Epub 2006 Apr 7.
Medullary sponge kidney (MSK) is characterized by cystic dilatation of the inner medullary collecting ducts, which causes the kidneys to resemble a sponge.
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).
Metabolic acidosis, hypercalciuria, hypocitraturia, tubular phosphate reabsorption and growth retardation in the patient improved with alkali therapy.
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。
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。
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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