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      Long-term prognosis of 4 children with steroid-sensitive nephrotic syndrome and relapse after 30 years of age

      case-report

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          Abstract

          Some children with steroid-sensitive nephrotic syndrome (SSNS) have been reported to suffer relapses in adulthood, but the clinical course of such adults is unclear. Four children with SSNS suffered relapses after 30 years of age. Those 4 patients developed frequently relapsing nephrotic syndrome (NS) between 2 and 10 years of age. They were treated with prednisolone (PSL) combined with cyclophosphamide in 3 patients, mizoribine in 2, and cyclosporine in 1 during childhood, and with cyclosporine in 2 during adulthood. After 20 years of age, the frequency of relapses gradually decreased. The last relapse occurred between 33 and 39 years of age, and proteinuria disappeared within 1 month after the start of treatment with PSL. At the last follow-up, all 4 patients continued to receive PSL, had normal renal function, and were in complete remission of NS when they were between 33 and 41 years of age. Although the long-term outcome of SSNS is usually considered to be favorable, pediatricians should be aware that some children with SSNS may require long-term treatment during adulthood.

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          Evaluation and management of proteinuria and nephrotic syndrome in children: recommendations from a pediatric nephrology panel established at the National Kidney Foundation conference on proteinuria, albuminuria, risk, assessment, detection, and elimination (PARADE).

          R E Portman, R E Hogg, F Ingelfinger … (2000)
          The development of this review article evolved from a National Kidney Foundation consensus conference on recent advances in the importance of evaluating and treating proteinuria. From this conference, a series of recommendations for the evaluation of adults with proteinuria was published. Because specific pediatric aspects of the problem were outside the scope of the original National Kidney Foundation publication, an ad hoc committee of 6 pediatric nephrologists who were active participants in the National Kidney Foundation conference was established to provide primary care physicians with a concise, up-to-date reference on this subject. The recommendations that are given represent the consensus opinions of the authors. These are based on data from controlled studies in children when available, but many of the opinions are, by necessity, based on uncontrolled series in children or controlled trials performed in adults, because controlled trials in children have not been performed to evaluate many of the treatments described. These recommendations are intended to provide primary care physicians with a useful reference when they are faced with a young child or teenager who presents with proteinuria, whether this is mild and asymptomatic or more severe, leading to nephrotic syndrome.
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            Long-term outcome of biopsy-proven, frequently relapsing minimal-change nephrotic syndrome in children.

            Henriette Kyrieleis, Elena N. Levtchenko, I Pronk … (2009)
            Frequently relapsing and steroid-dependent minimal-change nephrotic syndrome (MCNS) that originates in childhood can persist after puberty in >20% of patients. These patients require immunosuppressive treatment during several decades of their life. We examined long-term adverse effects of persistent nephrotic syndrome and immunosuppressive medications, focusing on renal function, growth, obesity, osteoporosis, hypertension, ocular complications, and fertility in adult patients with biopsy-proven childhood-onset MCNS. Molecular analysis was performed to evaluate a possible association of a complicated course of MCNS with podocyte gene mutations. We performed a prospective clinical examination of 15 adult patients that included serum and urine analysis; dual-energy x-ray absorptiometry; ophthalmologic examination; semen examination; and molecular analysis of NPHS1, NPHS2, CD2AP, and ACTN4 genes. All patients had normal GFR. Most frequent long-term complications were hypertension (in seven of 15 patients) and osteoporosis in one third of patients. Oligozoospermia was found in one patient, reduced sperm motility in four of eight patients, and teratozoospermia in six of eight patients. Ophthalmologic examination revealed myopia in 10 of 15 patients and cataract in three of 15 patients. Children with MCNS that persists after puberty are at risk for complications such as osteoporosis, hypertension, cataract, and sperm abnormalities. Our study underscores a need for more effective and less toxic therapies for relapsing MCNS.
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              Steroid-sensitive nephrotic syndrome: from childhood to adulthood.

              Paul Landais, M F Gagnadoux, P Lesavre … (2003)
              The clinical presentation, treatment, and outcome of steroid-sensitive nephrotic syndrome (SSNS) during childhood have been extensively studied. Conversely, few data regarding the outcome in adulthood of childhood SSNS have been published previously. We undertook to conduct a retrospective study of the outcome in adulthood of a large cohort of patients diagnosed with an SSNS during childhood. We identified all children born between 1970 and 1975 who had been admitted to our institution for an SSNS. Data regarding the outcome in adulthood of these patients were obtained through mailed questionnaires or phone calls to patients and/or their parents or through attending physicians. One hundred seventeen patients were identified. Data regarding the outcome of SSNS in adulthood were available for 102 patients (87.2%). Forty-three patients (42.2%) experienced at least one relapse of nephrotic syndrome in adulthood. By univariate analysis, young age at onset (<6 years) and more severe disease in childhood, indicated by a greater number of relapses (12.9 for adulthood relapsers versus 5.4 for adulthood nonrelapsers; P < 0.0001) and more frequent use of immunosuppressors (74.4% versus 31.6%; P < 0.0001) or cyclosporine (42.9% versus 7.3%; P < 0.0001) were predictive of the occurrence of SSNS relapse in adulthood. Conversely, relapse rate in the first 6 months of disease was not predictive of further relapses in adulthood. By multivariate analysis, only number of relapses during childhood was predictive of adulthood relapses (P < 0.0058). Long-term side effects of steroids were found in 44.2% of adulthood relapsers; the most frequent were osteoporosis and excess weight. The incidence of childhood SSNS relapses in adulthood was relatively high in our study. Further studies are required to assess long-term complications in adults with relapses and a history of prolonged steroid and immunosuppressor use. Copyright 2003 by the National Kidney Foundation, Inc.
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                Author and article information

                Contributors
                Osamu Motoyama: +81-43-4628811 , +81-43-4630801 , motoyan@basil.ocn.ne.jp
                Journal
                Journal ID (nlm-ta): CEN Case Rep
                Journal ID (iso-abbrev): CEN Case Rep
                Title: CEN Case Reports
                Publisher: Springer Japan (Tokyo )
                ISSN (Electronic): 2192-4449
                Publication date (Electronic): 14 September 2013
                Publication date PMC-release: 14 September 2013
                Publication date Collection: May 2014
                Volume: 3
                Issue: 1
                Pages: 106-109
                Affiliations
                [1 ]GRID grid.265050.4, ISNI 0000000092909879, Department of Pediatrics, , Toho University Medical Center, Sakura Hospital, ; 564-1 Shimoshizu, Sakura, Chiba 285-8741 Japan
                [2 ]GRID grid.265050.4, ISNI 0000000092909879, Department of Nephrology, , Toho University Medical Center, Omori Hospital, ; Tokyo, Japan
                [3 ]Narse Renal Clinic, Tokyo, Japan
                Article
                Publisher ID: 96
                DOI: 10.1007/s13730-013-0096-8
                PMC ID: 5411542
                SO-VID: 0d46a226-8f4e-4360-a338-72d1eb6976fc
                Copyright © © The Author(s) 2013
                License:

                Open AccessThis article is distributed under the terms of the Creative Commons Attribution License which permits any use, distribution, and reproduction in any medium, provided the original author(s) and the source are credited.

                History
                Date received : 19 July 2013
                Date accepted : 19 August 2013
                Categories
                Subject: Case Report
                Custom metadata
                issue-copyright-statement © Japanese Society of Nephrology 2014

                Keywords: childhood-onset nephrotic syndrome,steroid-sensitive nephrotic syndrome,frequently relapsing nephrotic syndrome,prognosis
                Data availability:
                Keywords: childhood-onset nephrotic syndrome, steroid-sensitive nephrotic syndrome, frequently relapsing nephrotic syndrome, prognosis

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