Eculizumab in secondary atypical haemolytic uraemic syndrome

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dc.contributor.author Cavero, Teresa
dc.contributor.author Rabasco, Cristina
dc.contributor.author López, Antía
dc.contributor.author Román, Elena
dc.contributor.author Ávila, Ana
dc.contributor.author Sevillano, Ángel
dc.contributor.author Huerta, Ana
dc.contributor.author Rojas-Rivera, Jorge
dc.contributor.author Fuentes, Carolina
dc.contributor.author Blasco, Miquel
dc.contributor.author Jarque, Ana
dc.contributor.author García, Alba
dc.contributor.author Mendizabal, Santiago
dc.contributor.author Gavela, Eva
dc.contributor.author Macía, Manuel
dc.contributor.author Quintana, Luis F.
dc.contributor.author Romera, Ana María
dc.contributor.author Borrego, Josefa
dc.contributor.author Arjona, Emi
dc.contributor.author Espinosa, Mario
dc.contributor.author Portolés, José
dc.contributor.author Gracia-Iguacel, Carolina
dc.contributor.author González-Parra, Emilio
dc.contributor.author Aljama, Pedro
dc.contributor.author Morales, Enrique
dc.contributor.author Cao, Mercedes
dc.contributor.author Rodríguez de Córdoba, Santiago
dc.contributor.author Praga, Manuel
dc.contributor.other UAM. Departamento de Medicina es_ES
dc.contributor.other Instituto de Investigación Sanitaria Fundación Jiménez Díaz (ISS-FJD) es_ES
dc.date.accessioned 2017-11-29T17:43:38Z
dc.date.available 2017-11-29T17:43:38Z
dc.date.issued 2017-02-20
dc.identifier.citation Nephrology Dialysis Transplantation 32.3 (2017): 466-474 en_:US
dc.identifier.issn 0931-050 (print) es_ES
dc.identifier.issn 1460-2385 (online) es_ES
dc.identifier.uri http://hdl.handle.net/10486/680548
dc.description.abstract Background. Complement dysregulation occurs in thrombotic microangiopathies (TMAs) other than primary atypical haemolytic uraemic syndrome (aHUS). A few of these patients have been reported previously to be successfully treated with eculizumab. Methods. We identified 29 patients with so-called secondary aHUS who had received eculizumab at 11 Spanish nephrology centres. Primary outcome was TMA resolution, defined by a normalization of platelet count (>150 × 109/L) and haemoglobin, disappearance of all the markers of microangiopathic haemolytic anaemia (MAHA), and improvement of renal function, with a ≥25% reduction of serum creatinine from the onset of eculizumab administration. Results. Twenty-nine patients with secondary aHUS (15 drug-induced, 8 associated with systemic diseases, 2 with postpartum, 2 with cancer-related, 1 associated with acute humoral rejection and 1 with intestinal lymphangiectasia) were included in this study. The reason to initiate eculizumab treatment was worsening of renal function and persistence of TMA despite treatment of the TMA cause and plasmapheresis. All patients showed severe MAHA and renal function impairment (14 requiring dialysis) prior to eculizumab treatment and 11 presented severe extrarenal manifestations. A rapid resolution of the TMA was observed in 20 patients (68%), 15 of them showing a ≥50% serum creatinine reduction at the last follow-up. Comprehensive genetic and molecular studies in 22 patients identified complement pathogenic variants in only 2 patients. With these two exceptions, eculizumab was discontinued, after a median of 8 weeks of treatment, without the occurrence of aHUS relapses. Conclusion. Short treatment with eculizumab can result in a rapid improvement of patients with secondary aHUS in whom TMA has persisted and renal function worsened despite treatment of the TMA-inducing condition en_US
dc.description.sponsorship Work in this report was funded by the Instituto de Salud Carlos III: REDinREN (RD 016/009 Feder Funds), the Fondo de Investigaciones Sanitarias (13/02502 and ICI14/00350), the Ministerio de Economia y Competitividad (SAF2015-66287R) and the Autonomous Region of Madrid (S2010/BMD-2316; Grupo de Investigación Complemento-CM). SRdeC is funded by the Seventh Framework Programme European Union Project EURenOmics (305608) en_US
dc.format.extent 9 pag. es_ES
dc.format.mimetype application/pdf en_US
dc.language.iso eng en
dc.publisher Oxford University Press on behalf of ERA-EDTA. en_US
dc.relation.ispartof Nephrology Dialysis Transplantation en_US
dc.rights © The Author 2017 en_US
dc.subject.other Atypical haemolytic uraemic syndrome en_US
dc.subject.other Complement activation en_US
dc.subject.other Eculizumab en_US
dc.subject.other Thrombotic microangiopathies en_US
dc.title Eculizumab in secondary atypical haemolytic uraemic syndrome en_US
dc.type article en
dc.subject.eciencia Medicina es_ES
dc.relation.publisherversion https://doi.org/10.1093/ndt/gfw453 es_ES
dc.identifier.doi 10.1093/ndt/gfw453 es_ES
dc.identifier.publicationfirstpage 466 es_ES
dc.identifier.publicationissue 3 es_ES
dc.identifier.publicationlastpage 474 es_ES
dc.identifier.publicationvolume 32 es_ES
dc.relation.projectID Gobierno de España. RD 016/009 es_ES
dc.relation.projectID Gobierno de España. ICI14/00350 es_ES
dc.relation.projectID Gobierno de España. SAF2015-66287R es_ES
dc.relation.projectID Comunidad de Madrid. S2010/BMD-2316/COMPLEMENTO es_ES
dc.relation.projectID info:eu-repo/grant/Agreement/EC/FP7/305608 es_ES
dc.relation.projectID Gobierno de España.13/02502 es_ES
dc.type.version info:eu-repo/semantics/publishedVersion en
dc.rights.cc Reconocimiento – NoComercial es_ES
dc.rights.accessRights openAccess en


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