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Urinary Podocyte Loss Is Increased in Patients with Fabry Disease and Correlates with Clinical Severity of Fabry Nephropathy.
Fall, Brent; Scott, C Ronald; Mauer, Michael; Shankland, Stuart; Pippin, Jeffrey; Jefferson, Jonathan A; Wallace, Eric; Warnock, David; Najafian, Behzad.
Afiliação
  • Fall B; Department of Pathology, University of Washington, Seattle, United States America.
  • Scott CR; Department of Pediatrics, University of Washington, Seattle, United States America.
  • Mauer M; Departments of Pediatrics, University of Minnesota, Minneapolis, United States America.
  • Shankland S; Departments of Medicine, University of Minnesota, Minneapolis, United States America.
  • Pippin J; Department of Medicine, University of Washington, Seattle, United States America.
  • Jefferson JA; Department of Medicine, University of Washington, Seattle, United States America.
  • Wallace E; Department of Medicine, University of Washington, Seattle, United States America.
  • Warnock D; Department of Medicine, University of Alabama, Birmingham, United States America.
  • Najafian B; Department of Medicine, University of Alabama, Birmingham, United States America.
PLoS One ; 11(12): e0168346, 2016.
Article em En | MEDLINE | ID: mdl-27992580
Chronic kidney disease is a major complication of Fabry disease. Podocytes accumulate globotriaosylceramide inclusions more than other kidney cell types in Fabry patients. Podocyte injury occurs early in age, and is progressive. Since injured podocytes detach into the urine (podocyturia), we hypothesized that podocyturia would increase in Fabry patients and correlate with clinical severity of Fabry nephropathy. Urine specimens from 39 Fabry patients and 24 healthy subjects were evaluated for podocyturia. Most of the Fabry patients and many healthy subjects had podocyturia. The number of podocytes per gram of urine creatinine (UPodo/g Cr) was 3.6 fold greater in Fabry patients (3,741 ± 2796; p = 0.001) than healthy subjects (1,040 ± 972). Fabry patients with normoalbuminuria and normoproteinuria had over 2-fold greater UPodo/g Cr than healthy subjects (p = 0.048). UPodo/gCr was inversely related to eGFR in male patients (r = -0.69, p = 0.003). UPodo/gCr was directly related to urine protein creatinine ratio (r = 0.33; p = 0.04) in all Fabry patients. These studies confirm increased podocyturia in Fabry disease, even when proteinuria and albuminuria are absent. Podocyturia correlates with clinical severity of Fabry nephropathy, and potentially may be of prognostic value.
Assuntos

Texto completo: 1 Base de dados: MEDLINE Assunto principal: Urina / Doença de Fabry / Podócitos / Insuficiência Renal Crônica Tipo de estudo: Diagnostic_studies / Etiology_studies / Observational_studies / Prognostic_studies / Risk_factors_studies Limite: Adolescent / Adult / Aged / Child / Child, preschool / Female / Humans / Male / Middle aged Idioma: En Ano de publicação: 2016 Tipo de documento: Article

Texto completo: 1 Base de dados: MEDLINE Assunto principal: Urina / Doença de Fabry / Podócitos / Insuficiência Renal Crônica Tipo de estudo: Diagnostic_studies / Etiology_studies / Observational_studies / Prognostic_studies / Risk_factors_studies Limite: Adolescent / Adult / Aged / Child / Child, preschool / Female / Humans / Male / Middle aged Idioma: En Ano de publicação: 2016 Tipo de documento: Article