Long-term follow-up in distal renal tubular acidosis with sensorineural deafness

A 20-year-old man presented with failure to thrive and bilateral genu valgu m. On the basis of growth failure, skeletal deformity, hyperchloremic metab olic acidosis with alkaline urine and hypokalemia, nephrocalcinosis, and he aring loss, a diagnosis of distal renal tubular acidosis (DRTA) with sensor ineural deafness was made. The genu valgum was treated by corrective osteot omy. Skeletal deformity was corrected and impaired growth improved after su stained therapy of metabolic acidosis with alkali supplementation. During a n 8-year follow-up period the patient's glomerular filtration rate remained stable, the nephrocalcinosis did not progress, and his height increased 10 cm. Although nephrolithiasis led to atrophy of the right kidney, at last f ollow-up, when the patient was 44 years old, his creatinine clearance was 5 0 ml/min per 1.73 m(2) body surface.