title: Diagnosis and treatment of Dent disease in 10 Chinese boys.
abstract: Dent disease is a rare X-linked recessive proximal tubular disorder that affects mostly male patients in childhood or early adult life. Dent disease is clinically characterized by the presence of low molecular weight proteinuria (LMWP), hypercalciuria, medullary nephrocalcinosis, nephrolithiasis, and progressive renal failure. The clinical features, diagnosis, and treatment of Dent disease were examined in 10 Chinese boys. All 10 childhood cases of Dent disease in China presented with tubular proteinuria in the nephrotic range and hypercalciuria. The ratio of α1-microglobulinuria to microalbuminuria, if close to or above 1, can be used as a diagnostic criterion for tubuloproteinuria. Lotensin was ineffective at treating proteinuria while dihydrochlorothiazide reduced urine calcium excretion.
authors.affiliation: Department of Pediatric, Peking University First Hospital, Beijing, China; Department of Pediatric, Foshan Maternal and Children Hospital, Foshan, China.
authors.forename: Guohua
authors.initials: G
authors.lastname: He
authors.affiliation: Department of Pediatric, Peking University First Hospital, Beijing, China.
authors.forename: Hongwen
authors.initials: H
authors.lastname: Zhang
authors.affiliation: Department of Pediatric, Peking University First Hospital, Beijing, China.
authors.forename: Fang
authors.initials: F
authors.lastname: Wang
authors.affiliation: Department of Pediatric, Peking University First Hospital, Beijing, China.
authors.forename: Xiaoyu
authors.initials: X
authors.lastname: Liu
authors.affiliation: Department of Pediatric, Peking University First Hospital, Beijing, China.
authors.forename: Huijie
authors.initials: H
authors.lastname: Xiao
authors.affiliation: Department of Pediatric, Peking University First Hospital, Beijing, China.
authors.forename: Yong
authors.initials: Y
authors.lastname: Yao
keywords: China; Dent disease; childhood; diagnosis; treatment