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      A case of regression of atypical dense deposit disease without C3 deposition in a child

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          Abstract

          Dense deposit disease (DDD) is a rare disorder characterized by the deposition of abnormal electron-dense material within the glomerular basement membrane of the kidneys. The diagnosis is made in most patients between 5 and 15 years of age, and within 10 years, approximately half of the affected patients progress to end-stage renal disease. We report a rare case of regressive DDD without C3 deposition after steroid therapy in an 11-year-old boy. The patient presented with edema, gross hematuria, and nephrotic-range proteinuria. Laboratory testing revealed a serum creatinine level of 1.17 mg/dL, albumin level of 2.3 g/dL, and serum C3 level of 125 mg/dL (range 90-180 mg/dL). The results of the renal biopsy were consistent with DDD without C3 deposition. After 6 weeks of steroid therapy, the nephrotic syndrome completely resolved. The follow-up renal biopsy showed a significant reduction in mesangial proliferation and disappearance of electron-dense deposits in the GBM.

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          Membranoproliferative glomerulonephritis type II (dense deposit disease): an update.

          H. Cook, Charles Jennette, Gregory Hageman … (2005)
          Membranoproliferative glomerulonephritis type II (MPGN II) is a rare disease characterized by the deposition of abnormal electron-dense material within the glomerular basement membrane of the kidney and often within Bruch's membrane in the eye. The diagnosis is made in most patients between the ages of 5 and 15 yr, and within 10 yr, approximately half progress to end-stage renal disease, occasionally with the late comorbidity of visual impairment. The pathophysiologic basis of MPGN II is associated with the uncontrolled systemic activation of the alternative pathway (AP) of the complement cascade. In most patients, loss of complement regulation is caused by C3 nephritic factor, an autoantibody directed against the C3 convertase of the AP, but in some patients, mutations in the factor H gene have been identified. For the latter patients, plasma replacement therapy prevents renal failure, but for the majority of patients, there is no proven effective treatment. The disease recurs in virtually all renal allografts, and a high percentage of these ultimately fail. The development of molecular diagnostic tools and new therapies directed at controlling the AP of the complement cascade either locally in the kidney or at the systemic level may lead to effective treatments for MPGN II.
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            Dense deposit disease: clinicopathologic study of 32 pediatric and adult patients.

            D A Valeri, Gerald B Appel, Vivette D. D'Agati … (2008)
            Dense deposit disease (DDD) is a rare disorder that most commonly affects children. This study reports the largest North American series addressing clinicopathologic and outcome differences in children and adults. Thirty-two patients with DDD were analyzed from the archives of Columbia University between 1977 and 2007. Characteristic intramembranous electron-dense deposits defined all diagnoses. The cohort included 14 children ( 60 yr. The female/male ratio was 1.9. At presentation, the mean 24-h urine protein was 4.6 g, nephrotic syndrome was present in 33%, renal insufficiency in 59%, and hematuria in 87% of patients. Compared with adults, children had lower incidence of renal insufficiency and were more likely to have reduced C3. Histologic pattern included membranoproliferative, mesangial, endocapillary, and crescentic glomerulonephritis. Treatment included immunosuppression (IS) alone in seven, renin angiotensin system (RAS) blockade alone in six, and combined IS/RAS blockade in 11. On follow-up (mean 63 mo) available in 27 patients, 26% had complete response, 48% had persistent renal dysfunction, and 26% had ESRD. Correlates of ESRD were older age and higher creatinine at biopsy, the absence of combined IS/RAS blockade therapy and the presence of subepithelial humps, but not histologic pattern. On multivariate analysis, age and creatinine emerged as the only independent predictors of ESRD. DDD is clinically and pathologically heterogeneous. Adults have worse outcome than children, despite similar treatment. Combined IS/RAS blockade appears superior to either agent alone.
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              Complement analysis in children with idiopathic membranoproliferative glomerulonephritis: a long-term follow-up.

              N Gretz, R Schwertz, M Kirschfink … (2001)
              Fifty children with idiopathic membranoproliferative glomerulonephritis (MPGN), aged 2-14 years at apparent onset, were monitored for the presence of C3 nephritic factor (C3 NeF) and signs of complement activation in serum. In addition, C3 allotyping was performed in 32 patients. Observation time ranged from 2 to 20 (median 11) years. C3 NeF activity was detected at least once in 60% of the patients (in 11 of 26 with type I, in 15 of 17 with type II, and in four of seven with type III). C3 NeF-positive patients had significantly reduced levels of CH50 and C3 and elevated levels of C3dg/C3d. During follow-up, C3 levels were persistently normal in 62% of the patients with MPGN type I and in 43% with type III but in only 18% with type II. C3 allotype frequencies differed from those found in healthy controls with a significant shift to the C3F/C3FS variants in C3 NeF-positive patients. C3b(Bb)P as a marker for alternative pathway activation was not increased in C3 NeF-positive patients. Despite the presence of C3 NeF activity, C3 levels remained normal in six patients throughout the observation period. C3 NeF became undetectable in six patients, whereas seven developed C3 NeF activity during follow-up. There was no significant difference in renal survival probability in patients with or without C3 NeF activity. Neither C3 variants nor continuous low C3 or low CH50 levels had any prognostic value for the clinical outcome. No factor H deficiency was detected.
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                Author and article information

                Journal
                Journal ID (nlm-ta): Korean J Pediatr
                Journal ID (publisher-id): KJP
                Title: Korean Journal of Pediatrics
                Publisher: The Korean Pediatric Society
                ISSN (Print): 1738-1061
                ISSN (Electronic): 2092-7258
                Publication date (Print): July 2010
                Publication date (Electronic): 31 July 2010
                Volume: 53
                Issue: 7
                Pages: 766-769
                Affiliations
                [1 ]Department of Pediatrics and Research Institute of Clinical Medicine, Chonbuk National University Medical School, Jeonju, Korea.
                [2 ]Department of Pathology and Research Institute of Clinical Medicine, Chonbuk National University Medical School, Jeonju, Korea.
                Author notes
                Corresponding author: Dae-Yeol Lee, M.D, Ph.D. Department of Pediatrics, Chonbuk National University Hospital, 634-18, Keumam-dong, Jeonju 561-712, Korea. Tel: +82.63-250-1469, Fax: +82.63-250-1464, leedy@ 123456chonbuk.ac.kr
                Article
                DOI: 10.3345/kjp.2010.53.7.766
                PMC ID: 3004489
                PubMed ID: 21189953
                SO-VID: 1d4ff1af-3471-47cc-86ba-f5b310b58baa
                Copyright © Copyright © 2010 by The Korean Pediatric Society
                License:

                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
                Date received : 11 March 2010
                Date revision received : 13 April 2010
                Date accepted : 18 May 2010
                Categories
                Subject: Case Report

                ScienceOpen disciplines: Pediatrics
                Keywords: glomerulonephritis,child,remission induction,membranoproliferative
                Data availability:
                ScienceOpen disciplines: Pediatrics
                Keywords: glomerulonephritis, child, remission induction, membranoproliferative

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