A case of Japanese Dent's disease (J-Dent) with follow-up renal biopsy

Abstract

 nbsp;We reported a case of J-Dent. The patient was 17-year-old boy who was detected at a mild proteinuria on urinary screening at the age of 3 years. He was diagnosed with J-Dent based on a marked increase in excretion of urinary β2-microglobulin and CLCN5 gene anomaly. First percutaneous renal biopsy specimens showed minor alterations in glomeruli and tubulointerstitium in 9-year old. During follow up duration, significant increases in urinary protein and albumin were observed. Second renal biopsy performed after 8 years revealed several global sclerosis with tubulointerstitial changes. These results suggest that the prognosis of the patient with J-Dent might be poor.