Abstract

BackgroundProteinuria is the most important risk factor for IgA nephropathy progression. The purpose of this study is to evaluate the long-term outcome and risk factors for poor prognosis in childhood IgA nephropathy.MethodsPatients who were diagnosed with IgA nephropathy between 1972 and 1992 at the Tokyo Metropolitan Kiyose Children’s Hospital were included. We analyzed risk factors for progression to end-stage kidney disease (ESKD) and chronic renal insufficiency (CRI) using Kaplan-Meier method and multivariate analyses of Cox proportional hazard model.ResultsOne hundred patients were included and the median observation period was 11.8 years. Twelve and 17 patients progressed to ESKD and CRI, respectively. The survival probabilities were 90.0% at 10 years and 79.8% at 20 years for ESKD, and 86.1% at 10 years and 72.3% at 20 years for CRI. Notably, patients with heavy proteinuria with hypoalbuminemia during follow-up period showed extremely poor prognosis. In this group, the survival rate at 10 years from ESKD and CRI was 40.6% and 20.8%, respectively. By multivariate analysis, proteinuria at diagnosis and proteinuria during follow-up period were risk factors for ESKD, whereas glomeruli showing mesangial proliferation ≥50% and proteinuria during follow-up period were risk factors for CRI. Patients without heavy proteinuria during follow-up period did not develop CRI and 63% of patients with mild proteinuria during follow-up period showed no proteinuria at the last observation.ConclusionsThe degree of proteinuria during follow-up period is the strongest risk factor for ESKD and CRI.

Highlights

  • IgA nephropathy is the leading cause of chronic glomerulonephritis worldwide today

  • Twelve and 17 patients progressed to end-stage kidney disease (ESKD) and chronic renal insufficiency (CRI), respectively

  • Patients without heavy proteinuria during follow-up period did not develop CRI and 63% of patients with mild proteinuria during follow-up period showed no proteinuria at the last observation

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Summary

Introduction

Longterm follow-up studies revealed that 20–50% of adult patients progressed to end-stage kidney disease (ESKD) [1]. Among pediatric patients with IgA nephropathy in Japan, 11% have been reported to reach ESKD within 15 years [8], there have been few reports regarding long-term prognosis of childhood IgA nephropathy. As the incidence of pediatric IgA nephropathy patients who show hypertension or decreased renal function at onset is relatively rare in comparison with adults, proteinuria is the most important risk factor for progression of the disease in childhood. Proteinuria is the most important risk factor for IgA nephropathy progression. The purpose of this study is to evaluate the long-term outcome and risk factors for poor prognosis in childhood IgA nephropathy

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