Glomerular Pathology in Dent Disease and Its Association with Kidney Function.

ABSTRACT:

Background and objectives

Dent disease is a rare X-linked disorder characterized by low molecular weight proteinuria and often considered a renal tubular disease. However, glomerulosclerosis was recently reported in several patients. Thus, Dent disease renal histopathologic features were characterized and assessed, and their association with kidney function was assessed.

Design, setting, participants, & measurements

Clinical renal pathology reports and slides (where available) were collected from 30 boys and men in eight countries who had undergone clinical renal biopsy between 1995 and 2014.

Results

Median (25th, 75th percentiles) age at biopsy was 7.5 (5, 19) years with an eGFR of 69 (44, 94) ml/min per 1.73 m² and a 24-hour urine protein of 2000 (1325, 2

SUBMITTER: Wang X 

PROVIDER: S-EPMC5142066 | biostudies-literature | 2016 Dec

REPOSITORIES: biostudies-literature