Abstract

BackgroundThe incidence of idiopathic nephrotic syndrome (INS) varies among countries, with Asia reporting a higher incidence in comparison with Western countries. We investigated the epidemiologic features of INS and attempted to identify factors that predispose individuals to develop end-stage renal disease (ESRD).MethodsClaims data from the Taiwanese National Health Insurance program from 1996 to 2008 were used to investigate the epidemiologic features and clinical variables of INS (International Classification of Diseases, Ninth Revision, Clinical Modification code, 581) in children younger than 18 years.ResultsWe enrolled 4083 children (male-female ratio, 1.91:1). During the 13 years of observation, annual incidence decreased from 9.91 to 3.36 per 100 000 children. Annual number of hospital admissions progressively decreased during the first 3 years after diagnosis. At 3.14 ± 2.77 years after INS onset, ESRD had developed in 145 (3.6%) children. Independent predictors of ESRD included older age at onset, acute renal failure (ARF), hypertensive encephalopathy, and a histologic subtype with focal segmental glomerulosclerosis (FSGS).ConclusionsPediatric INS in Taiwan was more frequent in boys. Unlike India, the current incidence of pediatric INS in Taiwan is very similar to that reported in Western studies. Older age at disease onset, ARF, hypertensive encephalopathy, and FSGS on biopsy are important predictors of poor renal outcome.

Highlights

  • Idiopathic nephrotic syndrome (INS) is characterized by edema, massive proteinuria, hypoalbuminemia, and hyperlipidemia

  • Demography, characteristics of patients, and epidemiologic features A total of 4083 children and adolescents aged from 6 months to 18 years were hospitalized for idiopathic nephrotic syndrome (INS) during the 13-year study period

  • INS incidence varied among age groups, and the highest incidence was among children aged 2 to 3 years

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Summary

Introduction

Idiopathic nephrotic syndrome (INS) is characterized by edema, massive proteinuria, hypoalbuminemia, and hyperlipidemia. INS most commonly appears in children aged from 4 to 8 years.[1] As many as 80% to 90% of children with INS respond to the steroid protocol developed by the International Study of Kidney Disease in Children. Long-term outcomes are generally favorable,[1] children with steroid-resistant INS may progress to end-stage renal disease (ESRD). We investigated the epidemiologic features of INS and attempted to identify factors that predispose individuals to develop end-stage renal disease (ESRD). During the 13 years of observation, annual incidence decreased from 9.91 to 3.36 per 100 000 children. Independent predictors of ESRD included older age at onset, acute renal failure (ARF), hypertensive encephalopathy, and a histologic subtype with focal segmental glomerulosclerosis (FSGS). Older age at disease onset, ARF, hypertensive encephalopathy, and FSGS on biopsy are important predictors of poor renal outcome

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