Blockade of Alternative Complement Pathway in Dense Deposit Disease
A patient aged 17 with dense deposit disease associated with complement activation, circulating C3 Nef, and Factor H mutation presented with nephrotic syndrome and hypertension. Steroid therapy, plasma exchange, and rituximab failed to improve proteinuria and hypertension despite a normalization of...
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| Main Authors: | , , , , , , |
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| Format: | Article |
| Language: | English |
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Wiley
2014-01-01
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| Series: | Case Reports in Nephrology |
| Online Access: | http://dx.doi.org/10.1155/2014/201568 |
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| _version_ | 1849467858483937280 |
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| author | Aurore Berthe-Aucejo Mathieu Sacquépée Marc Fila Michel Peuchmaur Emilia Perrier-Cornet Véronique Frémeaux-Bacchi Georges Deschênes |
| author_facet | Aurore Berthe-Aucejo Mathieu Sacquépée Marc Fila Michel Peuchmaur Emilia Perrier-Cornet Véronique Frémeaux-Bacchi Georges Deschênes |
| author_sort | Aurore Berthe-Aucejo |
| collection | DOAJ |
| description | A patient aged 17 with dense deposit disease associated with complement activation, circulating C3 Nef, and Factor H mutation presented with nephrotic syndrome and hypertension. Steroid therapy, plasma exchange, and rituximab failed to improve proteinuria and hypertension despite a normalization of the circulating sC5b9 complex. Eculizumab, a monoclonal antibody directed against C5, was used to block the terminal product of the complement cascade. The dose was adapted to achieve a CH50 below 10%, but proteinuria and blood pressure were not improved after 3 months of treatment. |
| format | Article |
| id | doaj-art-e0bd678c202445d2bb60f003f2c337f7 |
| institution | Kabale University |
| issn | 2090-6641 2090-665X |
| language | English |
| publishDate | 2014-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Case Reports in Nephrology |
| spelling | doaj-art-e0bd678c202445d2bb60f003f2c337f72025-08-20T03:26:00ZengWileyCase Reports in Nephrology2090-66412090-665X2014-01-01201410.1155/2014/201568201568Blockade of Alternative Complement Pathway in Dense Deposit DiseaseAurore Berthe-Aucejo0Mathieu Sacquépée1Marc Fila2Michel Peuchmaur3Emilia Perrier-Cornet4Véronique Frémeaux-Bacchi5Georges Deschênes6Service de Pharmacie, Hôpital Robert Debré, 48 boulevard Sérurier, 75019 Paris, FranceService de Néphrologie, Centre Hospitalier Territorial de Nouvelle Calédonie, Gaston Bourret, BP J5, 98849 Nouméa, New CaledoniaService de Néphrologie Pédiatrique, Hôpital Robert Debré, 48 boulevard Sérurier, 75019 Paris, FranceLaboratoire d’Anatomopathologie, Hôpital Robert Debré, 48 boulevard Sérurier, 75019 Paris, FranceService de Pharmacie, Hôpital Robert Debré, 48 boulevard Sérurier, 75019 Paris, FranceLaboratoire d’Immunologie, Hôpital Européen Georges-Pompidou, 20-40 rue Leblanc, 75015 Paris, FranceService de Néphrologie Pédiatrique, Hôpital Robert Debré, 48 boulevard Sérurier, 75019 Paris, FranceA patient aged 17 with dense deposit disease associated with complement activation, circulating C3 Nef, and Factor H mutation presented with nephrotic syndrome and hypertension. Steroid therapy, plasma exchange, and rituximab failed to improve proteinuria and hypertension despite a normalization of the circulating sC5b9 complex. Eculizumab, a monoclonal antibody directed against C5, was used to block the terminal product of the complement cascade. The dose was adapted to achieve a CH50 below 10%, but proteinuria and blood pressure were not improved after 3 months of treatment.http://dx.doi.org/10.1155/2014/201568 |
| spellingShingle | Aurore Berthe-Aucejo Mathieu Sacquépée Marc Fila Michel Peuchmaur Emilia Perrier-Cornet Véronique Frémeaux-Bacchi Georges Deschênes Blockade of Alternative Complement Pathway in Dense Deposit Disease Case Reports in Nephrology |
| title | Blockade of Alternative Complement Pathway in Dense Deposit Disease |
| title_full | Blockade of Alternative Complement Pathway in Dense Deposit Disease |
| title_fullStr | Blockade of Alternative Complement Pathway in Dense Deposit Disease |
| title_full_unstemmed | Blockade of Alternative Complement Pathway in Dense Deposit Disease |
| title_short | Blockade of Alternative Complement Pathway in Dense Deposit Disease |
| title_sort | blockade of alternative complement pathway in dense deposit disease |
| url | http://dx.doi.org/10.1155/2014/201568 |
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