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      Effects of Cyclosporin A Therapy Combined with Steroids and Angiotensin Converting Enzyme Inhibitors on Childhood IgA Nephropathy

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          Abstract

          To evaluate the effects of cyclosporin A (CyA) on clinical outcome and pathologic changes in children with IgA nephropathy (IgAN), we retrospectively evaluated 14 children (mean age 8.9±2.9 yr; eight males, six females) who were treated with CyA and steroids. The starting dose of CyA was 5 mg/kg per day, and the drug level was maintained at 100-200 ng/mL. The mean CyA level was 183.8±48.3 ng/mL (range 120.7-276.0 ng/mL) and the mean duration of CyA therapy was 10.9±1.9 months (range 8-12 months). After CyA therapy the mean 24 hr urinary protein excretion declined from 107.1±35.1 mg/m 2/hr to 7.4±2.4 mg/m 2/hr ( P<0.001) and serum albumin increased from 3.3±0.6 g/dL to 4.3±0.3 g/dL ( P<0.001). At a follow-up biopsy the histological grade of IgAN was improved in seven (50%) of the 14 patients, remained the same in three (21%), and was aggravated in four (29%). Serum creatinine, creatinine clearance, and blood pressure did not differ before and after CyA therapy. Two patients (14%) showed CyA-induced nephrotoxicity at the second biopsy. Our findings indicate that CyA therapy may be effective in reducing proteinuria and regressing renal pathology in a subset of children with IgAN.

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          IgA nephropathy.

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            Natural history of idiopathic IgA nephropathy: role of clinical and histological prognostic factors.

            G D'Amico (2000)
            Idiopathic immunoglobulin A nephropathy is characterized by an extreme variability in clinical course and sometimes by the unpredictability of the ultimate outcome. Among the numerous studies published in the last 15 years that have calculated the actuarial renal survival and tried to individuate the prognostic role of the clinical and histological features present at the onset of the disease or the time of biopsy, we chose to analyze critically the results of the most valid (30 studies). Actuarial renal survival at 10 years in adults was between 80% and 85% in most of the European and Asian studies, but it was less in studies from the United States and exceeded 90% in the few studies of children. Concordance existed in this selected literature that impairment of renal function, severe proteinuria, and arterial hypertension are the strongest and more reliable clinical predictors of an unfavorable outcome. However, analysis of the prognostic value of morphological lesions was more difficult because they have been characterized in some studies using an overall score or histological classes of progressively more severe involvement and, in others, using a semiquantitative grading of individual glomerular, tubular, interstitial, and vascular changes. In adult patients, a high score of glomerular and tubulointerstitial lesions, corresponding to classes IV and V of the Lee or Haas classifications, predicted a more rapid progression. When single lesions were analyzed separately, glomerulosclerosis and interstitial fibrosis appeared to be the strongest, most reliable predictors of unfavorable prognosis. More controversial was the role of crescents and capsular adhesions. None of the immunohistological features was found to be a risk factor for progression in the more accurate statistical analyses. The same histological features predicted outcome in children, although the severity of lesions at the time of biopsy was usually less than that in adults. However, in the single patient, even the evaluation of these prognostic markers sometimes fails to correctly predict outcome, probably because of the heterogeneity of the disease and the discontinuous activity of some injuring mechanisms during its course.
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              [Intercapillary deposits of IgA-IgG].

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                Author and article information

                Journal
                J Korean Med Sci
                JKMS
                Journal of Korean Medical Science
                The Korean Academy of Medical Sciences
                1011-8934
                1598-6357
                May 2010
                22 April 2010
                : 25
                : 5
                : 723-727
                Affiliations
                [1 ]The Institute of Kidney Disease, Department of Pediatrics, Yonsei University College of Medicine, Severance Children's Hospital, Seoul, Korea.
                [2 ]Department of Pathology, Chungnam National University College of Medicine, Daejeon, Korea.
                [3 ]Department of Pediatrics, Kwangdong University College of Medicine, Goyang, Korea.
                [4 ]The Institute of Kidney Disease, Department of Pathology, Yonsei University College of Medicine, Seoul, Korea.
                Author notes
                Address for Correspondence: Ji Hong Kim, M.D. Department of Pediatrics, Gangnam Severance Hospital, Yonsei University College of Medicine, 712 Eonju-ro, Gangnam-gu, Seoul 135-720, Korea. Tel: +82.2-2019-3352, Fax: +82.2-3461-9473, kkkjhd@ 123456yuhs.ac

                *Jae Il Shin and Beom Jin Lim contributed equally to this work.

                Article
                10.3346/jkms.2010.25.5.723
                2858831
                20436708
                90314979-407e-4bdb-a9fb-edfc98e95597
                © 2010 The Korean Academy of Medical Sciences.

                This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License ( http://creativecommons.org/licenses/by-nc/3.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                : 24 March 2009
                : 28 September 2009
                Categories
                Original Article
                Nephrology

                Medicine
                heavy proteinuria,glomerulonephritis, iga,cyclosporine,child
                Medicine
                heavy proteinuria, glomerulonephritis, iga, cyclosporine, child

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