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      Idiopathic immunoglobulin A nephropathy in children and adolescents

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      Pediatric Nephrology (Berlin, Germany)
      Springer-Verlag
      Berger’s disease, Chronic kidney disease, Glycosylation, Glomerular disease

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          Abstract

          Immunoglobulin A nephropathy is now recognized as the glomerular disease most often associated with progressive renal failure in patients around the world. In many cases it is not known when the disease starts to inflict glomerular injury, but recent studies that have shown genetically determined abnormalities in glycosylation of the IgA molecule suggest that this may begin in early life. This review focuses on recent advances in our understanding of IgA nephropathy, with special emphasis on clinical aspects of the disease when it presents in children and adolescents. In addition, the sections dealing with therapeutic options for patients with IgA nephropathy concentrate on studies that have been carried out on children. Whenever possible, data from randomized controlled clinical trials have formed the basis for recommendations. Unfortunately, this is not always possible, because of the lack of such trials in patients with IgA nephropathy.

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          Most cited references34

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          National Kidney Foundation's Kidney Disease Outcomes Quality Initiative clinical practice guidelines for chronic kidney disease in children and adolescents: evaluation, classification, and stratification.

          A series of new guidelines has been developed by the National Kidney Foundation's Kidney Disease Outcomes Quality Initiative to improve the detection and management of chronic kidney disease (CKD). In most instances of CKD, the earliest manifestations of the disorder may be identified by relatively simple tests. Unfortunately, CKD is often "underdiagnosed," in part because of the absence of a common definition of CKD and a classification of the stages in its progression. The Kidney Disease Outcomes Quality Initiative clinical practice guidelines for CKD evaluation, classification, and stratification provide a basis to remedy these deficits. The specific goals of the guidelines described in this review are to provide: 1) an overview of the clinical practice guidelines as they pertain to children and adolescents, 2) a simple classification of the stages of CKD, and 3) a practical approach to the laboratory assessment of kidney disease in children and adolescents. The guidelines were developed as part of an evidence-based evaluation of CKD and its consequences in patients of all ages. The data that were used to generate the guidelines in this article were extracted from a structured analysis of articles that reported on children with CKD. This review presents the definition and 5-stage classification system of CKD developed by the work group assigned to develop the guidelines, and summarizes the major recommendations regarding the early detection of CKD. Major emphasis is placed on the identification of children and adolescents with CKD by measuring the protein-to-creatinine ratio in spot urine specimens and by estimating the glomerular filtration rate from serum creatinine using prediction equations.
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            [Intercapillary deposits of IgA-IgG].

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              IgACE: a placebo-controlled, randomized trial of angiotensin-converting enzyme inhibitors in children and young people with IgA nephropathy and moderate proteinuria.

              This European Community Biomedicine and Health Research-supported, multicenter, randomized, placebo-controlled, double-blind trial investigated the effect of an angiotensin-converting enzyme inhibitor (ACE-I) in children and young people with IgA nephropathy (IgAN), moderate proteinuria (>1 and 50 ml/min per 1.73 m(2). Sixty-six patients who were 20.5 yr of age (range 9 to 35 yr), were randomly assigned to Benazepril 0.2 mg/kg per d (ACE-I) or placebo and were followed for a median of 38 mo. The primary outcome was the progression of kidney disease, defined as >30% decrease of CrCl; secondary outcomes were (1) a composite end point of >30% decrease of CrCl or worsening of proteinuria until > or =3.5 g/d per 1.73 m(2) and (2) proteinuria partial remission ( 6 mo. Analysis was by intention to treat. A single patient (3.1%) in the ACE-I group and five (14.7%) in the placebo group showed a worsening of CrCl >30%. The composite end point of >30% decrease of CrCl or worsening of proteinuria until nephrotic range was reached by one (3.1%) of 32 patients in the ACE-I group, and nine (26.5%) of 34 in the placebo group; the difference was significant (log-rank P = 0.035). A stable, partial remission of proteinuria was observed in 13 (40.6%) of 32 patients in the ACE-I group versus three (8.8%) of 34 in the placebo group (log-rank P = 0.033), with total remission in 12.5% of ACE-I-treated patients and in none in the placebo group (log-rank P = 0.029). The multivariate Cox analysis showed that treatment with ACE-I was the independent predictor of prognosis; no influence on the composite end point was found for gender, age, baseline CrCl, systolic or diastolic BP, mean arterial pressure, or proteinuria.
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                Author and article information

                Contributors
                +1-254-7248913 , +1-254-7245675 , rhogg@swmail.sw.org
                Journal
                Pediatr Nephrol
                Pediatric Nephrology (Berlin, Germany)
                Springer-Verlag (Berlin/Heidelberg )
                0931-041X
                1432-198X
                5 February 2009
                5 February 2009
                May 2010
                : 25
                : 5
                : 823-829
                Affiliations
                Department of Pediatrics, The Children’s Hospital, Scott and White Memorial Hospital and Clinic, and the Scott, Sherwood and Brindley Foundation and Texas A&M College of Medicine, 2401 South 31st Street, Temple, Texas 76508 USA
                Article
                1096
                10.1007/s00467-008-1096-3
                2839527
                19194728
                cceb93b6-c755-49fa-b79f-ec1399ed4912
                © IPNA 2009
                History
                : 21 October 2008
                : 25 November 2008
                : 1 December 2008
                Categories
                Educational Review
                Custom metadata
                © IPNA 2010

                Nephrology
                berger’s disease,glomerular disease,glycosylation,chronic kidney disease
                Nephrology
                berger’s disease, glomerular disease, glycosylation, chronic kidney disease

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