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      Epidemiology and Predictors of End-stage Renal Disease in Taiwanese Children With Idiopathic Nephrotic Syndrome

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          Abstract

          Background

          The 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).

          Methods

          Claims 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.

          Results

          We 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).

          Conclusions

          Pediatric 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.

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          Most cited references25

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          Prognostic significance of the early course of minimal change nephrotic syndrome: report of the International Study of Kidney Disease in Children.

          The ability to predict the course in children with newly diagnosed minimal change nephrotic syndrome (MCNS) may have significant therapeutic implications. Previous attempts based on data available at disease onset have not been successful. Therefore, it was investigated whether characterization of the initial response to adrenocortical steroids and the course during the early months of disease are predictive of the subsequent outcome. Three hundred-eighty-nine children with MCNS, diagnosed at onset, were treated with standard prednisone regimens and monitored for up to 17 yr (mean, 9.4 yr). They were classified, after 8 wk of therapy, as initial responders (complete remission) or initial nonresponders (continued proteinuria). Subsequent classifications included nonrelapsers, infrequent relapsers, and frequent relapsers. At 8 yr of follow-up, 80% of patients were in remission. Three-fourths of initial responders who remained in remission during the first 6-month period after initial therapy (nonrelapsers; 40% of the entire series) either continued in remission during their entire course or relapsed rarely. In contrast, initial relapsers, both frequent and infrequent, achieved a nonrelapsing course only after an average of 3 yr. Unremitting proteinuria during the initial 8 wk of treatment was followed by progression to ESRD in 21%. When proteinuria during the initial 8 wk continued through the subsequent 6 months, progression to renal failure occurred for 35%. Although 95% of children with MCNS do well, 4 to 5% die from complications or undergo progression to ESRD. Documentation of the early course aids in identifying those at increased risk for a poor outcome. More aggressive therapy may be indicated for these individuals.
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            The primary nephrotic syndrome in children. Identification of patients with minimal change nephrotic syndrome from initial response to prednisone. A report of the International Study of Kidney Disease in Children.

            The accuracy of using the initial response to prednisone to identify children with minimal glomerular changes has been assessed in a prospective study of children between 12 weeks and 16 years of age with the primary nephrotic syndrome. The results indicate that, as generally held, the likelihood is quite high, although not 100%, that a child with the primary nephrotic syndrome who responds during eight weeks of initial intensive steroid treatment has MCNS. Prognosis in these patients can be considered to be very favorable and renal biopsy need not be done unless indicated by the subsequent clinical course. However, the prediction that a patient who fails to respond has a glomerular lesion other than MCNS would be incorrect in about one-fourth of all patients with the primary nephrotic syndrome, and in as many as one-half of patients less than or equal to 6 years of age. For these patients, predictions concerning prognosis should be withheld until a renal biopsy provides a histopathologic diagnosis.
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              Long-term outcome of idiopathic steroid-resistant nephrotic syndrome: a multicenter study.

              Long-term outcome of idiopathic steroid-resistant nephrotic syndrome was retrospectively studied in 78 children in eight centers for the past 20 years. Median age at onset was 4.4 years (1.1-15.0 years) and the gender ratio was 1.4. Median follow-up period was 7.7 years (1.0-19.7 years). The disease in 45 patients (58%) was initially not steroid-responsive and in 33 (42%) it was later non-responsive. The main therapeutic strategies included administration of ciclosporine (CsA) alone (n = 29; 37%) and CsA + mycophenolate mofetil (n = 18; 23%). Actuarial patient survival rate after 15 years was 97%. Renal survival rate after 5 years, 10 years and 15 years was 75%, 58% and 53%, respectively. An age at onset of nephrotic syndrome (NS) > 10 years was the only independent predictor of end-stage renal disease (ESRD) in a multivariate analysis using a Cox regression model (P < 0.001). Twenty patients (26%) received transplants; ten showed recurrence of the NS: seven within 2 days, one within 2 weeks, and two within 3-5 months. Seven patients lost their grafts, four from recurrence. Owing to better management, kidney survival in idiopathic steroid-resistant nephrotic syndrome (SRNS) has improved during the past 20 years. Further prospective controlled trials will delineate the potential benefit of new immunosuppressive treatment.
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                Author and article information

                Journal
                J Epidemiol
                J Epidemiol
                JE
                Journal of Epidemiology
                Japan Epidemiological Association
                0917-5040
                1349-9092
                5 November 2012
                8 September 2012
                2012
                : 22
                : 6
                : 517-522
                Affiliations
                [1 ]Division of Pediatrics, Department of Pediatrics, Taipei Veterans General Hospital, Taipei, Taiwan
                [2 ]Division of Pediatric Surgery, Department of Surgery, Taipei Veterans General Hospital, Taipei, Taiwan
                [3 ]Department of Family Medicine, Taipei Veterans General Hospital, Taipei, Taiwan
                [4 ]Institute of Clinical Medicine, National Yang-Ming University, School of Medicine, Taipei, Taiwan
                Author notes
                Address for correspondence. Tzeng-Ji Chen, MD, PhD, Division of Pediatric Surgery, Department of Surgery, Taipei Veterans General Hospital, No 201, Section 2, Shih-Pai Rd, Taipei, Taiwan, 11217 (e-mail: tjchen@ 123456vghtpe.gov.tw ).
                Address for correspondence. Hsin-Lin Tsai, MD, Division of Pediatric Surgery, Department of Surgery, Taipei Veterans General Hospital, No 201, Section 2, Shih-Pai Rd, Taipei, Taiwan, 11217 (e-mail: hltsai@ 123456vghtpe.gov.tw ).
                [*]

                Contributed equally to this work.

                Article
                JE20120033
                10.2188/jea.JE20120033
                3798563
                22971550
                7b5184fb-3a59-4f63-b10a-4dc21ef8da61
                © 2012 Japan Epidemiological Association.

                This is an open access article distributed under the terms of Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

                History
                : 27 February 2012
                : 29 May 2012
                Categories
                Original Article

                idiopathic nephrotic syndrome,long-term outcome,children,end-stage renal disease

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