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Pesquisa : D12.776.124.486.274.920.287 [Categoria DeCS]
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[PMID]:27263803
[Au] Autor:Egan M; Sullivan K; Frazer-Abel A; Cunningham-Rundles C
[Ad] Endereço:Division of Allergy and Immunology, Department of Medicine, The Icahn School of Medicine at Mount Sinai, One Gustave L Levy Place, Box 1089, 10029 New York, NY, USA. Electronic address: MaureenSEgan@gmail.com.
[Ti] Título:A healthy female with C3 hypocomplementemia and C3 Nephritic Factor.
[So] Source:Clin Immunol;169:14-15, 2016 08.
[Is] ISSN:1521-7035
[Cp] País de publicação:United States
[La] Idioma:eng
[Mh] Termos MeSH primário: Fator Nefrítico do Complemento 3/análise
Complemento C3/análise
[Mh] Termos MeSH secundário: Adolescente
Complemento C3/deficiência
Feminino
Seres Humanos
[Pt] Tipo de publicação:CASE REPORTS; LETTER
[Nm] Nome de substância:
0 (Complement C3); 0 (Complement C3 Nephritic Factor)
[Em] Mês de entrada:1703
[Cu] Atualização por classe:170813
[Lr] Data última revisão:
170813
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:160607
[St] Status:MEDLINE


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[PMID]:26895476
[Au] Autor:Iatropoulos P; Noris M; Mele C; Piras R; Valoti E; Bresin E; Curreri M; Mondo E; Zito A; Gamba S; Bettoni S; Murer L; Fremeaux-Bacchi V; Vivarelli M; Emma F; Daina E; Remuzzi G
[Ad] Endereço:IRCCS-Istituto di Ricerche Farmacologiche Mario Negri, Clinical Research Center for Rare Diseases Aldo e Cele Daccò, Ranica, Bergamo, Italy.
[Ti] Título:Complement gene variants determine the risk of immunoglobulin-associated MPGN and C3 glomerulopathy and predict long-term renal outcome.
[So] Source:Mol Immunol;71:131-42, 2016 Mar.
[Is] ISSN:1872-9142
[Cp] País de publicação:England
[La] Idioma:eng
[Ab] Resumo:BACKGROUND: Membranoproliferative glomerulonephritis (MPGN) is an uncommon cause of chronic nephropathy recently reclassified into immunoglobulin-associated MPGN (Ig-MPGN) and C3 glomerulopathy (C3G). In this study we aimed: (1) to evaluate the complement genetic and biochemical profile in patients with Ig-MPGN/C3G; (2) to investigate whether genetic variants and different patterns of complement activation (i.e., fluid versus solid phase) correlate with disease manifestations and outcomes. METHODS: In 140 patients with idiopathic Ig-MPGN or C3G we performed complement biochemical and genetic screening and correlated genetic, biochemical and histology data with clinical features. RESULTS: Mutations in genes encoding alternative pathway complement proteins were found in both Ig-MPGN and C3G, and mutations in the two components of the C3 convertase are the most prevalent. We also report a mutation in THBD encoding thrombomodulin in a C3G patient. The presence of mutations alone does not significantly increase the risk of Ig-MPGN or C3G, but it does so when combined with common susceptibility variants (CD46 c.-366A in Ig-MPGN; CFH V62 and THBD A473 in C3G). Finally, patients without complement gene mutations or C3NeFs--autoantibodies that stabilize the alternative pathway C3 convertase--have a higher risk of progressing to end-stage renal disease than patients with identified mutations and/or C3NeFs, suggesting the existence of different pathogenetic mechanisms that lead to renal disease. CONCLUSIONS: We provide new insights into the pathogenesis of Ig-MPGN/C3G that underscore the complex nature of these diseases and suggest that the current C3G classification may miss many cases associated with abnormalities of the complement alternative pathway.
[Mh] Termos MeSH primário: Via Alternativa do Complemento/genética
Variação Genética
Glomerulonefrite Membranoproliferativa/classificação
Glomerulonefrite Membranoproliferativa/genética
Trombomodulina/genética
[Mh] Termos MeSH secundário: Adolescente
Fator Nefrítico do Complemento 3/genética
Feminino
Imunofluorescência
Predisposição Genética para Doença
Glomerulonefrite Membranoproliferativa/patologia
Sequenciamento de Nucleotídeos em Larga Escala
Seres Humanos
Imunoglobulinas
Falência Renal Crônica/etiologia
Masculino
Reação em Cadeia da Polimerase Multiplex
Polimorfismo de Nucleotídeo Único
Fatores de Risco
Adulto Jovem
[Pt] Tipo de publicação:JOURNAL ARTICLE; RESEARCH SUPPORT, NON-U.S. GOV'T
[Nm] Nome de substância:
0 (Complement C3 Nephritic Factor); 0 (Immunoglobulins); 0 (THBD protein, human); 0 (Thrombomodulin)
[Em] Mês de entrada:1608
[Cu] Atualização por classe:170922
[Lr] Data última revisão:
170922
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:160220
[St] Status:MEDLINE


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[PMID]:26660535
[Au] Autor:Corvillo F; Bravo García-Morato M; Nozal P; Garrido S; Tortajada A; Rodríguez de Córdoba S; López-Trascasa M
[Ad] Endereço:Unidad de Inmunología, IdiPAZ, Hospital Universitario La Paz, Madrid, Spain.
[Ti] Título:Serum properdin consumption as a biomarker of C5 convertase dysregulation in C3 glomerulopathy.
[So] Source:Clin Exp Immunol;184(1):118-25, 2016 Apr.
[Is] ISSN:1365-2249
[Cp] País de publicação:England
[La] Idioma:eng
[Ab] Resumo:Properdin (P) stabilizes the alternative pathway (AP) convertases, being the only known positive regulator of the complement system. In addition, P is a pattern recognition molecule able to initiate directly the AP on non-self surfaces. Although P deficiencies have long been known to be associated with Neisseria infections and P is often found deposited at sites of AP activation and tissue injury, the potential role of P in the pathogenesis of complement dysregulation-associated disorders has not been studied extensively. Serum P levels were measured in 49 patients with histological and clinical evidence of C3 glomerulopathy (C3G). Patients were divided into two groups according to the presence or absence of C3 nephritic factor (C3NeF), an autoantibody that stabilizes the AP C3 convertase. The presence of this autoantibody results in a significant reduction in circulating C3 (P < 0·001) and C5 levels (P < 0·05), but does not alter factor B, P and sC5b-9 levels. Interestingly, in our cohort, serum P levels were low in 17 of the 32 C3NeF-negative patients. This group exhibited significant reduction of C3 (P < 0·001) and C5 (P < 0·001) and increase of sC5b-9 (P < 0·001) plasma levels compared to the control group. Also, P consumption was correlated significantly with C3 (r = 0·798, P = 0·0001), C5 (r = 0·806, P < 0·0001), sC5b-9 (r = -0·683, P = 0·043) and a higher degree of proteinuria (r = -0·862, P = 0·013). These results illustrate further the heterogeneity among C3G patients and suggest that P serum levels could be a reliable clinical biomarker to identify patients with underlying surface AP C5 convertase dysregulation.
[Mh] Termos MeSH primário: Convertases de Complemento C3-C5/imunologia
Via Alternativa do Complemento
Glomerulonefrite/imunologia
Properdina/imunologia
Proteinúria/imunologia
[Mh] Termos MeSH secundário: Adolescente
Adulto
Biomarcadores/sangue
Criança
Complemento C3/genética
Complemento C3/imunologia
Fator Nefrítico do Complemento 3/genética
Fator Nefrítico do Complemento 3/imunologia
Convertases de Complemento C3-C5/genética
Complemento C5/genética
Complemento C5/imunologia
Fator B do Complemento/genética
Fator B do Complemento/imunologia
Inativadores do Complemento/sangue
Complexo de Ataque à Membrana do Sistema Complemento/genética
Complexo de Ataque à Membrana do Sistema Complemento/imunologia
Feminino
Regulação da Expressão Gênica
Glomerulonefrite/sangue
Glomerulonefrite/genética
Glomerulonefrite/patologia
Seres Humanos
Masculino
Meia-Idade
Properdina/genética
Proteinúria/sangue
Proteinúria/genética
Proteinúria/patologia
Estudos Retrospectivos
Índice de Gravidade de Doença
Transdução de Sinais
[Pt] Tipo de publicação:JOURNAL ARTICLE; RESEARCH SUPPORT, NON-U.S. GOV'T
[Nm] Nome de substância:
0 (Biomarkers); 0 (Complement C3); 0 (Complement C3 Nephritic Factor); 0 (Complement C5); 0 (Complement Inactivating Agents); 0 (Complement Membrane Attack Complex); 0 (SC5b-9 protein complex); 11016-39-0 (Properdin); EC 3.4.21.- (Complement C3-C5 Convertases); EC 3.4.21.47 (Complement Factor B)
[Em] Mês de entrada:1607
[Cu] Atualização por classe:170403
[Lr] Data última revisão:
170403
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:151215
[St] Status:MEDLINE
[do] DOI:10.1111/cei.12754


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[PMID]:26187133
[Au] Autor:Alexander MP; Fervenza FC; De Vriese AS; Smith RJH; Nasr SH; Cornell LD; Herrera Hernandez LP; Zhang Y; Sethi S
[Ad] Endereço:Division of Anatomic Pathology, Department of Laboratory Medicine and Pathology, Mayo Clinic, 200 First Street, SW, Rochester, MN, 55905, USA.
[Ti] Título:C3 glomerulonephritis and autoimmune disease: more than a fortuitous association?
[So] Source:J Nephrol;29(2):203-209, 2016 Apr.
[Is] ISSN:1724-6059
[Cp] País de publicação:Italy
[La] Idioma:eng
[Ab] Resumo:C3 glomerulonephritis (C3GN) results from genetic or acquired dysregulation of the alternative complement pathway. A subset of patients may have clinical and biochemical characteristics compatible with an autoimmune disorder. We studied a cohort of 85 patients with confirmed C3GN (2007-2014), of which ten patients (3 male, 7 female; mean age 38.5 years) had an associated autoimmune disorder. All patients had abnormal ANA titers, 6 also had positive ds-DNA titers. At the time of presentation with C3GN, all 7 female patients had autoimmune-related presentations. Of the 3 male patients, only 1 patient had autoimmune-related presentations. Kidney biopsy showed predominantly mesangial proliferative or membranoproliferative glomerulonephritis. In 5 patients, the alternative pathway was evaluated. All had allele variants/polymorphisms associated with C3GN. One patient was also positive for C3Nefs. Treatment varied form conservative management to the use of prednisone alone or with cytotoxic therapy. Mean serum creatinine decreased from 2.0 to 1.4 mg/dL while proteinuria decreased from 2300 to 994 mg/24 h in 8 patients with follow-up. The study highlights the association between C3GN and autoimmune disorders, particularly in female patients. The study suggests that an autoimmune milieu may act as a trigger for the development of C3GN in genetically susceptible patients. Short-term prognosis of C3GN associated with autoimmune disorders appears excellent.
[Mh] Termos MeSH primário: Doenças Autoimunes/imunologia
Autoimunidade
Ativação do Complemento
Complemento C3/imunologia
Glomerulonefrite Membranoproliferativa/imunologia
Glomérulos Renais/imunologia
[Mh] Termos MeSH secundário: Adulto
Doenças Autoimunes/diagnóstico
Doenças Autoimunes/tratamento farmacológico
Doenças Autoimunes/genética
Autoimunidade/efeitos dos fármacos
Autoimunidade/genética
Biomarcadores/sangue
Ativação do Complemento/efeitos dos fármacos
Ativação do Complemento/genética
Complemento C3/genética
Fator Nefrítico do Complemento 3/análise
Quimioterapia Combinada
Feminino
Marcadores Genéticos
Predisposição Genética para Doença
Glomerulonefrite Membranoproliferativa/diagnóstico
Glomerulonefrite Membranoproliferativa/tratamento farmacológico
Glomerulonefrite Membranoproliferativa/genética
Glucocorticoides/uso terapêutico
Seres Humanos
Glomérulos Renais/efeitos dos fármacos
Glomérulos Renais/patologia
Masculino
Meia-Idade
Fenótipo
Prednisona/uso terapêutico
Fatores de Risco
Fatores Sexuais
Resultado do Tratamento
Adulto Jovem
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Biomarkers); 0 (C3 protein, human); 0 (Complement C3); 0 (Complement C3 Nephritic Factor); 0 (Genetic Markers); 0 (Glucocorticoids); VB0R961HZT (Prednisone)
[Em] Mês de entrada:1612
[Cu] Atualização por classe:171115
[Lr] Data última revisão:
171115
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:150719
[St] Status:MEDLINE
[do] DOI:10.1007/s40620-015-0218-9


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[PMID]:26374720
[Au] Autor:Fidder AR; van der Deure H; Westra DW
[Ad] Endereço:Deventer Ziekenhuis, afd. Kindergeneeskunde, Deventer.
[Ti] Título:[Partial lipodystrophy: a spot diagnosis].
[Ti] Título:Partiële lipodystrofie: een diagnose à vue..
[So] Source:Ned Tijdschr Geneeskd;159:A8872, 2015.
[Is] ISSN:1876-8784
[Cp] País de publicação:Netherlands
[La] Idioma:dut
[Ab] Resumo:BACKGROUND: Partial lipodystrophy is a rare acquired disorder characterised by gradual loss of subcutaneous adipose tissue in the upper half of the body. CASE DESCRIPTION: We saw a 9-year-old girl who had been referred on account of recurrent urinary tract infections. On physical examination, she was noticed to be very thin in the face. Her upper extremities were also skinny. Strikingly, the lower half of her body was normally proportioned, which immediately suggested a diagnosis of partial lipodystrophy. Additional examinations showed a low level of complement factor C3 and the presence of C3 nephritic factor. CONCLUSION: Partial lipodystrophy is rare but it is important to include it in the differential diagnosis of unwanted disproportional subcutaneous fat loss because of the somatic and psychological consequences.
[Mh] Termos MeSH primário: Fator Nefrítico do Complemento 3/análise
Complemento C3/deficiência
Lipodistrofia/diagnóstico
[Mh] Termos MeSH secundário: Criança
Diagnóstico Diferencial
Feminino
Seres Humanos
Exame Físico
[Pt] Tipo de publicação:CASE REPORTS; ENGLISH ABSTRACT; JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Complement C3); 0 (Complement C3 Nephritic Factor)
[Em] Mês de entrada:1605
[Cu] Atualização por classe:150916
[Lr] Data última revisão:
150916
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:150917
[St] Status:MEDLINE


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[PMID]:25986912
[Au] Autor:Häffner K; Michelfelder S; Pohl M
[Ad] Endereço:Center for Pediatrics and Adolescent Medicine, University Hospital Freiburg, Mathildenstr. 1, 79106, Freiburg, Germany. karsten.haeffner@uniklinik-freiburg.de.
[Ti] Título:Successful therapy of C3Nef-positive C3 glomerulopathy with plasma therapy and immunosuppression.
[So] Source:Pediatr Nephrol;30(11):1951-9, 2015 Nov.
[Is] ISSN:1432-198X
[Cp] País de publicação:Germany
[La] Idioma:eng
[Ab] Resumo:BACKGROUND: C3 glomerulopathies (C3G) are characterized by uncontrolled activation of the alternative pathway of complement. In most patients these diseases progress towards end-stage renal disease, and the risk of recurrence after renal transplantation is high. In the majority of patients, only antibodies against the C3 convertase, termed C3Nef, can be found as a potential pathogenic factor. Although a large variety of therapeutic approaches have been used, no generally accepted therapy exists. METHODS: In four consecutive patients with C3G in whom all known complement factor mutations were excluded and only C3Nef could be identified as a potential cause of disease, a multimodal therapeutic regimen with plasma therapy, corticosteroids and mycophenolate mofetil was used. RESULTS: The multimodal regimen achieved normalization of renal function in all four patients, with complete remission in two patients and a distinct reduction of proteinuria in the other two patients. The single patient with C3 glomerulonephritis (C3GN) and marked terminal complement complex elevation only showed partial remission; further improvement was achieved following the addition of eculizumab to the therapeutic regimen. Repeatedly measured C3Nef levels did not correlate with disease course or therapeutic response in any of the patients. CONCLUSIONS: As this multimodal therapeutic approach was effective in all four treated patients with suspected autoimmune etiology of C3G, it offers a treatment option for severely affected patients with this rare disease until more specific regimens are available.
[Mh] Termos MeSH primário: Fator Nefrítico do Complemento 3/metabolismo
Glomerulonefrite Membranoproliferativa/sangue
Glomerulonefrite Membranoproliferativa/terapia
Imunossupressores/uso terapêutico
Troca Plasmática
[Mh] Termos MeSH secundário: Adolescente
Corticosteroides/uso terapêutico
Criança
Terapia Combinada
Feminino
Seres Humanos
Ácido Micofenólico/análogos & derivados
Ácido Micofenólico/uso terapêutico
[Pt] Tipo de publicação:JOURNAL ARTICLE; RESEARCH SUPPORT, NON-U.S. GOV'T
[Nm] Nome de substância:
0 (Adrenal Cortex Hormones); 0 (Complement C3 Nephritic Factor); 0 (Immunosuppressive Agents); HU9DX48N0T (Mycophenolic Acid)
[Em] Mês de entrada:1608
[Cu] Atualização por classe:171010
[Lr] Data última revisão:
171010
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:150520
[St] Status:MEDLINE
[do] DOI:10.1007/s00467-015-3111-9


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[PMID]:25929733
[Au] Autor:Zipfel PF; Skerka C; Chen Q; Wiech T; Goodship T; Johnson S; Fremeaux-Bacchi V; Nester C; de Córdoba SR; Noris M; Pickering M; Smith R
[Ad] Endereço:Leibniz Institute for Natural Product Research and Infection Biology, Jena, Germany; Friedrich Schiller University Jena, Germany. Electronic address: peter.zipfel@hki-jena.de.
[Ti] Título:The role of complement in C3 glomerulopathy.
[So] Source:Mol Immunol;67(1):21-30, 2015 Sep.
[Is] ISSN:1872-9142
[Cp] País de publicação:England
[La] Idioma:eng
[Ab] Resumo:C3 glomerulopathy describes a spectrum of disorders with glomerular pathology associated with C3 cleavage product deposition and with defective complement action and regulation (Fakhouri et al., 2010; Sethi et al., 2012b). Kidney biopsies from these patients show glomerular accumulation or deposition of C3 cleavage fragments, but no or minor deposition of immunoglobulins (Appel et al., 2005; D'Agati and Bomback, 2012; Servais et al., 2007; Sethi and Fervenza, 2011). At present the current situation asks for a better definition of the underlining disease mechanisms, for precise biomarkers, and for a treatment for this disease. The complement system is a self activating and propelling enzymatic cascade type system in which inactive, soluble plasma components are activated spontaneously and lead into an amplification loop (Zipfel and Skerka, 2009). Activation of the alternative pathway is spontaneous, occurs by default, and cascade progression leads to amplification by complement activators. The system however is self-controlled by multiple regulators and inhibitors, like Factor H that control cascade progression in fluid phase and on surfaces. The activated complement system generates a series of potent effector components and activation products, which damage foreign-, as well as modified self cells, recruit innate immune cells to the site of action, coordinate inflammation and the response of the adaptive immune system in form of B cells and T lymphocytes (Kohl, 2006; Medzhitov and Janeway, 2002; Ogden and Elkon, 2006; Carroll, 2004; Kemper and Atkinson, 2007; Morgan, 1999; Muller-Eberhard, 1986; Ricklin et al., 2010). Complement controls homeostasis and multiple reactions in the vertebrate organism including defense against microbial infections (Diaz-Guillen et al., 1999; Mastellos and Lambris, 2002; Nordahl et al., 2004; Ricklin et al., 2010). In consequence defective control of the spontaneous self amplifying cascade or regulation is associated with numerous human disorders (Ricklin and Lambris, 2007; Skerka and Zipfel, 2008; Zipfel et al., 2006). Understanding the exact action and regulation of this sophisticated homeotic cascade system is relevant to understand disease pathology of various complement associated human disorders. Furthermore this knowledge is relevant for a better diagnosis and appropriate therapy. At present diagnosis of C3 glomerulopathy is primarily based on the kidney biopsy, and histological, immmunohistological and electron microscopical evaluation (D'Agati and Bomback, 2012; Fakhouri et al., 2010; Medjeral-Thomas et al., 2014a,b; Sethi et al., 2012b). The challenge is to define the actual cause of the diverse glomerular changes or damages, to define how C3 deposition results in the reported glomerular changes, the location of the cell damage and the formation of deposits.
[Mh] Termos MeSH primário: Fator Nefrítico do Complemento 3/química
Complemento C3/química
Proteínas Inativadoras do Complemento C3b/química
Glomerulonefrite Membranoproliferativa/patologia
Glomérulos Renais/patologia
Agregação Patológica de Proteínas/patologia
[Mh] Termos MeSH secundário: Imunidade Adaptativa
Linfócitos B/imunologia
Linfócitos B/patologia
Biomarcadores/sangue
Ativação do Complemento
Complemento C3/genética
Fator Nefrítico do Complemento 3/genética
Proteínas Inativadoras do Complemento C3b/genética
Expressão Gênica
Glomerulonefrite Membranoproliferativa/diagnóstico
Glomerulonefrite Membranoproliferativa/genética
Glomerulonefrite Membranoproliferativa/imunologia
Seres Humanos
Glomérulos Renais/química
Glomérulos Renais/imunologia
Agregação Patológica de Proteínas/diagnóstico
Agregação Patológica de Proteínas/genética
Agregação Patológica de Proteínas/imunologia
Linfócitos T/imunologia
Linfócitos T/patologia
[Pt] Tipo de publicação:JOURNAL ARTICLE; RESEARCH SUPPORT, NON-U.S. GOV'T; REVIEW
[Nm] Nome de substância:
0 (Biomarkers); 0 (Complement C3); 0 (Complement C3 Nephritic Factor); 0 (Complement C3b Inactivator Proteins)
[Em] Mês de entrada:1508
[Cu] Atualização por classe:151119
[Lr] Data última revisão:
151119
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:150502
[St] Status:MEDLINE


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[PMID]:25395361
[Au] Autor:Niel O; Dallocchio A; Thouret MC; Guigonis V; Cassuto É; Frémeaux-Bacchi V; Bérard É
[Ad] Endereço:Inserm U1091, Genetics of Renal Development and Diseases, UNS Université Nice Sophia Antipolis, 28, avenue de Valrose, 06000, Nice, France, o.r.p.niel@free.fr.
[Ti] Título:C3 nephritic factor can be associated with membranous glomerulonephritis.
[So] Source:Pediatr Nephrol;30(2):353-5, 2015 Feb.
[Is] ISSN:1432-198X
[Cp] País de publicação:Germany
[La] Idioma:eng
[Ab] Resumo:BACKGROUND: C3 nephritic factor (C3NeF) has been described in association with membranoproliferative glomerulonephritis and is involved in 80 % of cases of dense deposit disease. C3NeF is an immunoglobulin G (IgG) autoantibody which binds to the complement component 3 (C3) convertase C3bBb, thereby inhibiting its decay and leading to massive C3 cleavage. Commonly associated with C3NeF are low C3 levels, decreased total haemolytic complement (CH50) and normal C4 levels. C3NeF patients often present with proteinuria, haematuria and high blood pressure. Evolution to end-stage renal disease is common. Treatment consists of steroids and/or immunosuppressants, with variable efficiency. Renal transplantation is marked by histological recurrence, leading to higher rates of allograft loss. CASES: We report C3NeF in association with membranous glomerulonephritis type 3-4 in two unrelated children. We also demonstrate that, under adequate immunosuppressive therapy, proteinuria is significantly lowered, blood pressure is kept within normal range and long-term renal function remains normal. CONCLUSIONS: C3NeF can be associated with membranous glomerulonephritis in children. Clinical presentation is mild, and mid-term outcome is favourable under adequate therapy. However, complement anomalies persist for several years.
[Mh] Termos MeSH primário: Fator Nefrítico do Complemento 3/imunologia
Glomerulonefrite Membranosa/imunologia
[Mh] Termos MeSH secundário: Adolescente
Criança
Feminino
Seres Humanos
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Complement C3 Nephritic Factor)
[Em] Mês de entrada:1509
[Cu] Atualização por classe:171010
[Lr] Data última revisão:
171010
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:141115
[St] Status:MEDLINE
[do] DOI:10.1007/s00467-014-3004-3


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[PMID]:25341723
[Au] Autor:Fervenza FC; Sethi S
[Ad] Endereço:Divisions of Nephrology and Hypertension and fervenza.fernando@mayo.edu.
[Ti] Título:Circulating complement levels and C3 glomerulopathy.
[So] Source:Clin J Am Soc Nephrol;9(11):1829-31, 2014 Nov 07.
[Is] ISSN:1555-905X
[Cp] País de publicação:United States
[La] Idioma:eng
[Mh] Termos MeSH primário: Fator Nefrítico do Complemento 3/metabolismo
Complemento C3/metabolismo
Complemento C5/metabolismo
Complemento C7/metabolismo
Fator B do Complemento/metabolismo
Glomerulonefrite Membranoproliferativa/sangue
[Mh] Termos MeSH secundário: Feminino
Seres Humanos
Masculino
[Pt] Tipo de publicação:COMMENT; EDITORIAL
[Nm] Nome de substância:
0 (Complement C3); 0 (Complement C3 Nephritic Factor); 0 (Complement C5); 0 (Complement C7); EC 3.4.21.47 (Complement Factor B)
[Em] Mês de entrada:1507
[Cu] Atualização por classe:151107
[Lr] Data última revisão:
151107
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:141025
[St] Status:MEDLINE
[do] DOI:10.2215/CJN.09620914


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[PMID]:25341722
[Au] Autor:Zhang Y; Nester CM; Martin B; Skjoedt MO; Meyer NC; Shao D; Borsa N; Palarasah Y; Smith RJ
[Ad] Endereço:Molecular Otolaryngology and Renal Research Laboratories.
[Ti] Título:Defining the complement biomarker profile of C3 glomerulopathy.
[So] Source:Clin J Am Soc Nephrol;9(11):1876-82, 2014 Nov 07.
[Is] ISSN:1555-905X
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:BACKGROUND AND OBJECTIVES: C3 glomerulopathy (C3G) applies to a group of renal diseases defined by a specific renal biopsy finding: a dominant pattern of C3 fragment deposition on immunofluorescence. The primary pathogenic mechanism involves abnormal control of the alternative complement pathway, although a full description of the disease spectrum remains to be determined. This study sought to validate and define the association of complement dysregulation with C3G and to determine whether specific complement pathway abnormalities could inform disease definition. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: This study included 34 patients with C3G (17 with C3 glomerulonephritis [C3GN] and 17 with dense deposit disease [DDD]) diagnosed between 2008 and 2013 selected from the C3G Registry. Control samples (n=100) were recruited from regional blood drives. Nineteen complement biomarkers were assayed on all samples. Results were compared between C3G disease categories and with normal controls. RESULTS: Assessment of the alternative complement pathway showed that compared with controls, patients with C3G had lower levels of serum C3 (P<0.001 for both DDD and C3GN) and factor B (P<0.001 for both DDD and C3GN) as well as higher levels of complement breakdown products including C3d (P<0.001 for both DDD and C3GN) and Bb (P<0.001 for both DDD and C3GN). A comparison of terminal complement pathway proteins showed that although C5 levels were significantly suppressed (P<0.001 for both DDD and C3GN) its breakdown product C5a was significantly higher only in patients with C3GN (P<0.05). Of the other terminal pathway components (C6-C9), the only significant difference was in C7 levels between patients with C3GN and controls (P<0.01). Soluble C5b-9 was elevated in both diseases but only the difference between patients with C3GN and controls reached statistical significance (P<0.001). Levels of C3 nephritic factor activity were qualitatively higher in patients with DDD compared with patients with C3GN. CONCLUSIONS: Complement biomarkers are significantly abnormal in patients with C3G compared with controls. These data substantiate the link between complement dysregulation and C3G and identify C3G interdisease differences.
[Mh] Termos MeSH primário: Fator Nefrítico do Complemento 3/metabolismo
Complemento C3/metabolismo
Complemento C5/metabolismo
Complemento C7/metabolismo
Fator B do Complemento/metabolismo
Glomerulonefrite Membranoproliferativa/sangue
[Mh] Termos MeSH secundário: Adolescente
Adulto
Biomarcadores/sangue
Estudos de Casos e Controles
Criança
Complemento C3d/metabolismo
Complemento C5a/metabolismo
Feminino
Glomerulonefrite Membranoproliferativa/imunologia
Seres Humanos
Masculino
Meia-Idade
Adulto Jovem
[Pt] Tipo de publicação:JOURNAL ARTICLE; RESEARCH SUPPORT, NON-U.S. GOV'T
[Nm] Nome de substância:
0 (Biomarkers); 0 (Complement C3); 0 (Complement C3 Nephritic Factor); 0 (Complement C5); 0 (Complement C7); 80295-45-0 (Complement C3d); 80295-54-1 (Complement C5a); EC 3.4.21.47 (Complement Factor B)
[Em] Mês de entrada:1507
[Cu] Atualização por classe:151119
[Lr] Data última revisão:
151119
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:141025
[St] Status:MEDLINE
[do] DOI:10.2215/CJN.01820214



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