Medullary Sponge Kidneyに合併せる遠位型Renal Tubular Acidosis(RTA)の1例

Abstract

Studies of a 15-year-old boy with urinary acidification defect, distal type of RTA and medullary sponge kidney are described. His illness began at the age of 5 years, when he developed polyuria, generalized acidosis and impaired urinary concentrating ability. Over a 10-year followup period, sodium and potassium cit rates being administered, he shows normal physical development, has nogeneralized acidosis and has normal renal function except urinary acidification defect and slightly impaired concentrating capacity. A number of small calculi within the pyramids on IVP indicate medullary sponge kidney and sodium bicarbonate and ammonium chloride loading tests disclosed Type I (distal) RTA in this patient. The literatures on the combination of RTA and medullary sponge kidney are briefly reviewed.