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      Post-vaccine glomerulonephritis in an infant with hereditary C2 complement deficiency: case study

      case-report
      Croatian Medical Journal
      Croatian Medical Schools

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          Abstract

          We describe a case of a post vaccine immune complex-mediated glomerulonephritis in an infant with compound heterozygous mutations of C2 complement component gene, which is the first such case in the literature. The three and a half months old boy presented with clinical and laboratory signs of nephritic syndrome and was successfully treated with methylprednisolone. An explanation of such a clinical picture may lie in the interaction between C2 deficiency and vaccination.

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          Most cited references17

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          Systemic lupus erythematosus, complement deficiency, and apoptosis.

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            Prevalence, incidence, and demographics of systemic lupus erythematosus and lupus nephritis from 2000 to 2004 among children in the US Medicaid beneficiary population.

            To investigate the nationwide prevalence, incidence, and sociodemographics of systemic lupus erythematosus (SLE) and lupus nephritis among children in the US Medicaid beneficiary population. Children ages 3 years to 30 days apart) were identified from the US Medicaid Analytic eXtract database from 2000 to 2004. This database contains all inpatient and outpatient Medicaid claims for 47 US states and the District of Columbia. Lupus nephritis was identified from ≥2 ICD-9 billing codes for glomerulonephritis, proteinuria, or renal failure, each recorded >30 days apart. The prevalence and incidence of SLE and lupus nephritis were calculated among Medicaid-enrolled children overall and within sociodemographic groups. Of the 30,420,597 Medicaid-enrolled children during these years, 2,959 were identified as having SLE. The prevalence of SLE was 9.73 (95% confidence interval [95% CI] 9.38-10.08) per 100,000 Medicaid-enrolled children. Among the children with SLE, 84% were female, 40% were African American, 25% were Hispanic, 21% were White, and 42% resided in the South region of the US. Moreover, of the children with SLE, 1,106 (37%) had lupus nephritis, representing a prevalence of 3.64 (95% CI 3.43-3.86) per 100,000 children. The average annual incidence of SLE was 2.22 cases (95% CI 2.05-2.40) and that of lupus nephritis was 0.72 cases (95% CI 0.63-0.83) per 100,000 Medicaid enrollees per year. The prevalence and incidence rates of SLE and lupus nephritis increased with age, were higher in girls than in boys, and were higher in all non-White racial/ethnic groups. In the current study, the prevalence and incidence rates of SLE among Medicaid-enrolled children in the US are high compared to studies in other populations. In addition, these data represent the first population-based estimates of the prevalence and incidence of lupus nephritis in the US to date. Copyright © 2012 by the American College of Rheumatology.
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              Clinical and laboratory evaluation of complement deficiency.

              The complement system provides innate defense against microbial pathogens and is a "complement" to humoral (antibody-mediated) immunity. Consisting of plasma and membrane proteins, this proinflammatory system works in part by a cascade involving limited proteolysis whereby one component activates the next, resulting in a dramatic amplification. The overall goal is deposition of complement fragments on pathologic targets for the purposes of opsonization, lysis, and liberation of peptides that promote the inflammatory response. Deficiencies of complement components predispose to infections and autoimmune syndromes. Even though total deficiency of a complement component is rare, patients presenting with certain bacterial infections and autoimmune syndromes, especially SLE, have a much greater incidence of deficiency. This review will summarize the clinical manifestations and pathophysiology of congenital and acquired complement deficiency diseases. We will also present an algorithm for laboratory diagnosis of complement deficiency and discuss current and future therapeutic options.
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                Author and article information

                Journal
                Croat Med J
                Croat. Med. J
                CMJ
                Croatian Medical Journal
                Croatian Medical Schools
                0353-9504
                1332-8166
                December 2013
                : 54
                : 6
                : 569-573
                Affiliations
                [1]Department of Pediatric Nephrology, University Medical Centre, Ljubljana, Slovenia
                Author notes
                Correspondence to: 
Tanja Kersnik Levart
Department of Pediatric Nephrology
University Medical Centre
Bohoričeva 20
1000 Ljubljana, Slovenia
 tanja.kersnik@ 123456guest.arnes.si
                Article
                CroatMedJ_54_0569
                10.3325/cmj.2013.54.569
                3893984
                24382852
                23d0f843-be92-4f6c-aa8d-efb1b5452ff1
                Copyright © 2013 by the Croatian Medical Journal. All rights reserved.

                This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                : 06 May 2013
                : 20 November 2013
                Categories
                Case Report

                Medicine
                Medicine

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