title: Evolutive study of children with diffuse mesangial sclerosis.
abstract: Diffuse mesangial sclerosis (DMS) is a renal disease that usually presents as a nephrotic syndrome. It is characterized by early onset and rapid progression to end-stage renal disease, and can occur as an isolated finding or as part of the Denys-Drash syndrome. The aim of this study was to characterize clinical features and outcomes of DMS in a cohort of children. We retrospectively analyzed all cases of DMS diagnosed in our hospital between 1973 and 2008 and evaluated the progression of the disease in relation to different variables. We studied 14 patients, four with incomplete Denys-Drash syndrome and one with Frasier syndrome. All patients developed renal failure. Eight patients received a renal transplant with no relapse of the disease. Bilateral nephrectomy was performed in nine patients with end-stage renal disease. Seven patients died, with sepsis being the main cause of death. Diffuse mesangial sclerosis must be suspected in a child that presents with early onset proteinuria and/or rapidly progressive renal failure. Karyotype and WT1 gene analysis should be performed because of the predisposition of patients to develop different types of tumors. This nephropathy has a poor prognosis, but the survival rate has improved in the last decade.
authors.affiliation: Department of Pediatric Nephrology, Hospital Infantil La Paz, Madrid, Spain.
authors.forename: Ana Pilar
authors.initials: AP
authors.lastname: Nso Roca
authors.forename: Antonia
authors.initials: A
authors.lastname: Peña Carrión
authors.forename: Marta
authors.initials: M
authors.lastname: Benito Gutiérrez
authors.forename: Carmen
authors.initials: C
authors.lastname: García Meseguer
authors.forename: Araceli
authors.initials: A
authors.lastname: García Pose
authors.forename: Mercedes
authors.initials: M
authors.lastname: Navarro
mesh_terms: D002675:Child, Preschool; D015331:Cohort Studies; D030321:Denys-Drash Syndrome / Q000473:pathology; D003937:Diagnosis, Differential; D018450:Disease Progression; D005260:Female; D052159:Frasier Syndrome / Q000175:diagnosis; D005920:Glomerular Mesangium / Q000473:pathology*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D016030:Kidney Transplantation; D008297:Male; D009400:Nephrosclerosis / Q000401:mortality / Q000473:pathology* / Q000601:surgery; D009404:Nephrotic Syndrome / Q000401:mortality / Q000473:pathology* / Q000601:surgery; D051437:Renal Insufficiency / Q000401:mortality / Q000473:pathology* / Q000601:surgery; D012189:Retrospective Studies; D015996:Survival Rate