A retrospective study of pregnancy-associated atypical hemolytic uremic syndrome

34 p.-2 fig.-3 tab.

Detalles Bibliográficos
Autores: Huerta, Ana, Arjona, Emilia, Portoles, Jose, Lopez-Sanchez, Paula, Rabasco, Cristina, Espinosa, Mario, Cavero, Teresa, Blasco, Miquel, Cao, Mercedes, Manrique, Joaquin, Cabello-Chaves, Virginia, Suñer, Marta, Heras, Manuel, Fulladosa, Xavier, Belmar-Vega, Lara, Sempere, Amparo, Peralta, Carmen, Castillo, Lorena, Arnau, Alvaro, Praga, Manuel, Rodríguez de Córdoba, Santiago
Tipo de recurso: artículo
Estado:Versión aceptada para publicación
Fecha de publicación:2018
País:España
Institución:Consejo Superior de Investigaciones Científicas (CSIC)
Repositorio:DIGITAL.CSIC. Repositorio Institucional del CSIC
OAI Identifier:oai:digital.csic.es:10261/187586
Acceso en línea:http://hdl.handle.net/10261/187586
Access Level:acceso abierto
Palabra clave:Cesarean section
Complement
Eculizumab
Hemolytic uremic syndrome
Postpartum
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spelling A retrospective study of pregnancy-associated atypical hemolytic uremic syndromePregnancy-associated atypical hemolytic uremic syndrome; a retrospective study from the Spanish Registry.Huerta, AnaArjona, EmiliaPortoles, JoseLopez-Sanchez, PaulaRabasco, CristinaEspinosa, MarioCavero, TeresaBlasco, MiquelCao, MercedesManrique, JoaquinCabello-Chaves, VirginiaSuñer, MartaHeras, ManuelFulladosa, XavierBelmar-Vega, LaraSempere, AmparoPeralta, CarmenCastillo, LorenaArnau, AlvaroPraga, ManuelRodríguez de Córdoba, SantiagoCesarean sectionComplementEculizumabHemolytic uremic syndromePostpartum34 p.-2 fig.-3 tab.Pregnancy-associated atypical hemolytic uremic syndrome (aHUS) refers to the thrombotic microangiopathy resulting from uncontrolled complement activation during pregnancy or the postpartum period. Pregnancy-associated aHUS is a devastating disease for which there is a limited clinical understanding and treatment experience. Here we report a retrospective study to analyze the clinical and prognostic data of 22 cases of pregnancy-associated aHUS from the Spanish aHUS Registry under different treatments. Sixteen patients presented during the first pregnancy and as many as nine patients required hemodialysis at diagnosis. Identification of inherited complement abnormalities explained nine of the 22 cases, with CFH mutations and CFH to CFHR1 gene conversion events being the most prevalent genetic alterations associated with this disorder (66%). In thirteen of the cases, pregnancy complications were sufficient to trigger a thrombotic microangiopathy in the absence of genetic or acquired complement alterations. The postpartum period was the time with highest risk to develop the disease and the group shows an association of cesarean section with pregnancy-associated aHUS. Seventeen patients underwent plasma treatments with a positive renal response in only three cases. In contrast, ten patients received eculizumab with an excellent renal response in all, independent of carrying or not inherited complement abnormalities. Although the cohort is relatively small, the data suggest that pregnancy-associated aHUS is not different from other types of aHUS and suggest the efficacy of eculizumab treatment over plasma therapies. This study may be useful to improve prognosis in this group of aHUS patients.Work in this report was funded by the Instituto de Salud Carlos III: REDinREN (RD016/009 and RD012/0021) and Fondo de Investigaciones Sanitarias (ISCIII/FEDER) ref 13/02502 and ref 16/01685. SRdeC is supported by the Spanish “Ministerio de Economía y Competitividad-FEDER” (SAF2015-66287R), the Seventh Framework Programme European Union Project EURenOmics (305608) and the Autonomous Region of Madrid (S2010/BMD-2316).Peer reviewedElsevierInstituto de Salud Carlos IIIMinisterio de Economía y Competitividad (España)European CommissionComunidad de MadridHuerta, Ana [0000-0003-3342-7628]Arjona, Emilia [0000-0002-0753-3657]Portoles, Jose [0000-0002-2114-1385]Lopez-Sanchez, Paula [0000-0002-4332-5759]Cavero, Teresa [0000-0001-5187-9906]Cao, Mercedes [0000-0002-8389-1800]Fulladosa, Xavier [0000-0003-1974-9874]Sempere, Amparo [0000-0001-6727-3343]Praga, Manuel [0000-0001-9270-1071]Rodríguez de Córdoba, Santiago [0000-0001-6401-1874]Consejo Superior de Investigaciones Científicas [https://ror.org/02gfc7t72]201920192018info:eu-repo/semantics/articlehttp://purl.org/coar/resource_type/c_6501Postprintinfo:eu-repo/semantics/acceptedVersionhttp://hdl.handle.net/10261/187586reponame:DIGITAL.CSIC. Repositorio Institucional del CSICinstname:Consejo Superior de Investigaciones Científicas (CSIC)Inglés#PLACEHOLDER_PARENT_METADATA_VALUE##PLACEHOLDER_PARENT_METADATA_VALUE#info:eu-repo/grantAgreement/MINECO/Plan Estatal de Investigación Científica y Técnica y de Innovación 2013-2016/SAF2015-66287Rinfo:eu-repo/grantAgreement/EC/FP7/305608https://doi.org/10.1016/j.kint.2017.06.022Síinfo:eu-repo/semantics/openAccessoai:digital.csic.es:10261/1875862026-05-22T06:33:51Z
dc.title.none.fl_str_mv A retrospective study of pregnancy-associated atypical hemolytic uremic syndrome
Pregnancy-associated atypical hemolytic uremic syndrome; a retrospective study from the Spanish Registry.
title A retrospective study of pregnancy-associated atypical hemolytic uremic syndrome
spellingShingle A retrospective study of pregnancy-associated atypical hemolytic uremic syndrome
Huerta, Ana
Cesarean section
Complement
Eculizumab
Hemolytic uremic syndrome
Postpartum
title_short A retrospective study of pregnancy-associated atypical hemolytic uremic syndrome
title_full A retrospective study of pregnancy-associated atypical hemolytic uremic syndrome
title_fullStr A retrospective study of pregnancy-associated atypical hemolytic uremic syndrome
title_full_unstemmed A retrospective study of pregnancy-associated atypical hemolytic uremic syndrome
title_sort A retrospective study of pregnancy-associated atypical hemolytic uremic syndrome
dc.creator.none.fl_str_mv Huerta, Ana
Arjona, Emilia
Portoles, Jose
Lopez-Sanchez, Paula
Rabasco, Cristina
Espinosa, Mario
Cavero, Teresa
Blasco, Miquel
Cao, Mercedes
Manrique, Joaquin
Cabello-Chaves, Virginia
Suñer, Marta
Heras, Manuel
Fulladosa, Xavier
Belmar-Vega, Lara
Sempere, Amparo
Peralta, Carmen
Castillo, Lorena
Arnau, Alvaro
Praga, Manuel
Rodríguez de Córdoba, Santiago
author Huerta, Ana
author_facet Huerta, Ana
Arjona, Emilia
Portoles, Jose
Lopez-Sanchez, Paula
Rabasco, Cristina
Espinosa, Mario
Cavero, Teresa
Blasco, Miquel
Cao, Mercedes
Manrique, Joaquin
Cabello-Chaves, Virginia
Suñer, Marta
Heras, Manuel
Fulladosa, Xavier
Belmar-Vega, Lara
Sempere, Amparo
Peralta, Carmen
Castillo, Lorena
Arnau, Alvaro
Praga, Manuel
Rodríguez de Córdoba, Santiago
author_role author
author2 Arjona, Emilia
Portoles, Jose
Lopez-Sanchez, Paula
Rabasco, Cristina
Espinosa, Mario
Cavero, Teresa
Blasco, Miquel
Cao, Mercedes
Manrique, Joaquin
Cabello-Chaves, Virginia
Suñer, Marta
Heras, Manuel
Fulladosa, Xavier
Belmar-Vega, Lara
Sempere, Amparo
Peralta, Carmen
Castillo, Lorena
Arnau, Alvaro
Praga, Manuel
Rodríguez de Córdoba, Santiago
author2_role author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
dc.contributor.none.fl_str_mv Instituto de Salud Carlos III
Ministerio de Economía y Competitividad (España)
European Commission
Comunidad de Madrid
Huerta, Ana [0000-0003-3342-7628]
Arjona, Emilia [0000-0002-0753-3657]
Portoles, Jose [0000-0002-2114-1385]
Lopez-Sanchez, Paula [0000-0002-4332-5759]
Cavero, Teresa [0000-0001-5187-9906]
Cao, Mercedes [0000-0002-8389-1800]
Fulladosa, Xavier [0000-0003-1974-9874]
Sempere, Amparo [0000-0001-6727-3343]
Praga, Manuel [0000-0001-9270-1071]
Rodríguez de Córdoba, Santiago [0000-0001-6401-1874]
Consejo Superior de Investigaciones Científicas [https://ror.org/02gfc7t72]
dc.subject.none.fl_str_mv Cesarean section
Complement
Eculizumab
Hemolytic uremic syndrome
Postpartum
topic Cesarean section
Complement
Eculizumab
Hemolytic uremic syndrome
Postpartum
description 34 p.-2 fig.-3 tab.
publishDate 2018
dc.date.none.fl_str_mv 2018
2019
2019
dc.type.none.fl_str_mv info:eu-repo/semantics/article
http://purl.org/coar/resource_type/c_6501
Postprint
info:eu-repo/semantics/acceptedVersion
format article
status_str acceptedVersion
dc.identifier.none.fl_str_mv http://hdl.handle.net/10261/187586
url http://hdl.handle.net/10261/187586
dc.language.none.fl_str_mv Inglés
language_invalid_str_mv Inglés
dc.relation.none.fl_str_mv #PLACEHOLDER_PARENT_METADATA_VALUE#
#PLACEHOLDER_PARENT_METADATA_VALUE#
info:eu-repo/grantAgreement/MINECO/Plan Estatal de Investigación Científica y Técnica y de Innovación 2013-2016/SAF2015-66287R
info:eu-repo/grantAgreement/EC/FP7/305608
https://doi.org/10.1016/j.kint.2017.06.022
Sí
dc.rights.none.fl_str_mv info:eu-repo/semantics/openAccess
eu_rights_str_mv openAccess
dc.publisher.none.fl_str_mv Elsevier
publisher.none.fl_str_mv Elsevier
dc.source.none.fl_str_mv reponame:DIGITAL.CSIC. Repositorio Institucional del CSIC
instname:Consejo Superior de Investigaciones Científicas (CSIC)
instname_str Consejo Superior de Investigaciones Científicas (CSIC)
reponame_str DIGITAL.CSIC. Repositorio Institucional del CSIC
collection DIGITAL.CSIC. Repositorio Institucional del CSIC
repository.name.fl_str_mv
repository.mail.fl_str_mv
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