title: Dent's disease complicated by nephrotic syndrome: A case report.
abstract: Dent's disease is an X-linked recessive proximal tubular disorder that mostly affects male patients in childhood or early adult life. The condition is caused by mutations in theCLCN5(Dent disease 1) orOCRL(Dent disease 2) genes located on chromosome Xp11.22 and Xq25, respectively. In most male patients, proteinuria is subnephrotic but may reach nephrotic levels. Here, we report the first case of Dent's disease complicated by nephrotic syndrome. Dent's disease should be considered in the differential diagnosis of nephrotic syndrome, and especially in male patients with early onset of nephrotic syndrome. A urinary α1-microglobulin/albumin ratio > 1 may provide the first clue to a tubulopathy.
authors.affiliation: Pediatrics, Peking University First Hospital, Beijing, China; Pediatrics, Foshan Maternal and Children's Hospital, Foshan, China.
authors.forename: Guohua
authors.initials: G
authors.lastname: He
authors.affiliation: Pediatrics, Peking University First Hospital, Beijing, China.
authors.forename: Hongwen
authors.initials: H
authors.lastname: Zhang
authors.affiliation: Pediatrics, Peking University First Hospital, Beijing, China; Health Center, Beijing Entry-Exit Inspection and Quarantine Bureau, Beijing, China.
authors.forename: Shanshan
authors.initials: S
authors.lastname: Cao
authors.affiliation: Pediatrics, Peking University First Hospital, Beijing, China.
authors.forename: Huijie
authors.initials: H
authors.lastname: Xiao
authors.affiliation: Pediatrics, Peking University First Hospital, Beijing, China.
authors.forename: Yong
authors.initials: Y
authors.lastname: Yao
keywords: Dent's disease; OCRL gene; nephrotic syndrome; proteinuria