title: The long-term use of enalapril and hydrochlorothiazide in two novel mutations patients with Dent's disease type 1.
abstract: Dent's disease type 1 is an X-linked tubular disease caused by mutations in the renal chloride channel CLCN-5, and it is characterized by low molecular weight proteinuria, hypercalciuria, nephrocalcinosis, and renal failure. Several cases have been described in which the only presenting symptoms were asymptomatic proteinuria, and focal segmental or global glomerulosclerosis. The renal failure in these patients may be caused by hypercalciuria and persistent proteinuria. Therefore, angiotensin converse enzyme inhibitor and thiazides could be useful. Our aim is to report the effects of these drugs in two novel mutations patients with Dent's disease type 1. In this report, no significant correlations between dosage of hydrochlorothiazide and calciuria and no significant correlations between proteinuria and dosage of enalapril were detected. This is important since these are polyuric patients and these drugs could be dangerous to their renal function.
authors.affiliation: Unidade de Nefrologia, Instituto da Criança do Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo – HC-FM/USP. vaisbich@terra. com.br
authors.forename: Maria Helena
authors.initials: MH
authors.lastname: Vaisbich
authors.forename: Luciana Dos Santos
authors.initials: Ldos S
authors.lastname: Henriques
authors.forename: Takashi
authors.initials: T
authors.lastname: Igarashi
authors.forename: Takashi
authors.initials: T
authors.lastname: Sekine
authors.forename: George
authors.initials: G
authors.lastname: Seki
authors.forename: Vera H
authors.initials: VH
authors.lastname: Koch
mesh_terms: D000806:Angiotensin-Converting Enzyme Inhibitors / Q000008:administration & dosage*; D002648:Child; D002675:Child, Preschool; D004656:Enalapril / Q000008:administration & dosage*; D040181:Genetic Diseases, X-Linked / Q000188:drug therapy* / Q000235:genetics*; D006801:Humans; D006852:Hydrochlorothiazide / Q000008:administration & dosage*; D008297:Male; D009154:Mutation; D053040:Nephrolithiasis / Q000188:drug therapy* / Q000235:genetics*; D013997:Time Factors
chemical_list: D000806:Angiotensin-Converting Enzyme Inhibitors; D006852:Hydrochlorothiazide; D004656:Enalapril