Anti-factor H autoantibody-associated hemolytic uremic syndrome in an Argentine pediatric cohort

Autores
Dos Santos, Célia; Trinidad, Jesica Aurora; Castera, Santiago; Alconcher, Laura; Coccia, Paula Alejandra; Manni, Federico Javier; Alberto, Maria Fabiana; Sánchez Luceros, Analía Gabriela
Año de publicación
2023
Idioma
inglés
Tipo de recurso
artículo
Estado
versión publicada
Descripción
Aim: To describe the clinical characteristics and frequency of anti-factor H (FH) autoantibody-associated atypical hemolytic uremic syndrome (aHUS) in the first cohort of Argentine patients. Methods: The presence of anti-FH autoantibodies in 70 pediatric patients with suspected aHUS was investigated between 2013 and 2022. Clinical and laboratory parameters were collected and compared between patients who were positive and negative for anti-FH antibodies. Results: The 70 patients screened for anti-FH autoantibodies presented clinical features of non-immune microangiopathic hemolytic anemia, thrombocytopenia and renal injury. Positive titers were found in 14 children [mean: 1,938 arbitrary units per mL (AU/mL), range 179–8,500]. Due to missing clinical data, two patients who tested positive for anti-FH and 20 patients who tested negative for anti-FH were excluded from the data analysis. The laboratory features and clinical manifestations of anti-FH-positive aHUS cases (n = 12) were very similar to those of subjects with no autoantibodies detected (n = 36). Treatment administration was heterogeneous among the 12 patients analyzed. Dialysis was performed in six patients in total. Five children received plasmapheresis, while three patients were treated with plasma exchange followed by administration of eculizumab. Two patients received eculizumab only and one showed significant improvement solely through supportive care. Eight patients in total received immunosuppressive therapy. Follow-up of three patients showed a significant decrease of anti-FH autoantibody titers in 2/3 after treatment and during clinical remission. Conclusions: The cohort of 70 pediatric patients in this study demonstrated that the frequency of anti-FH autoantibody-associated aHUS in Argentina is 20%. The implementation of anti-FH testing in the country can potentially contribute to improved treatment and follow-up for patients with autoimmune aHUS.
Fil: Dos Santos, Célia. Consejo Nacional de Investigaciones Científicas y Técnicas. Instituto de Medicina Experimental. Academia Nacional de Medicina de Buenos Aires. Instituto de Medicina Experimental; Argentina
Fil: Trinidad, Jesica Aurora. Consejo Nacional de Investigaciones Científicas y Técnicas. Instituto de Medicina Experimental. Academia Nacional de Medicina de Buenos Aires. Instituto de Medicina Experimental; Argentina
Fil: Castera, Santiago. Academia Nacional de Medicina de Buenos Aires. Instituto de Investigaciones Hematológicas "Mariano R. Castex"; Argentina
Fil: Alconcher, Laura. Hospital Municipal General de Agudos Doctor José Penna; Argentina
Fil: Coccia, Paula Alejandra. Hospital Italiano; Argentina
Fil: Manni, Federico Javier. Gobierno de la Provincia de Santa Fe. Hospital de Niños Doctor Orlando Alassia.; Argentina
Fil: Alberto, Maria Fabiana. Academia Nacional de Medicina de Buenos Aires. Instituto de Investigaciones Hematológicas "Mariano R. Castex"; Argentina
Fil: Sánchez Luceros, Analía Gabriela. Consejo Nacional de Investigaciones Científicas y Técnicas. Instituto de Medicina Experimental. Academia Nacional de Medicina de Buenos Aires. Instituto de Medicina Experimental; Argentina. Academia Nacional de Medicina de Buenos Aires. Instituto de Investigaciones Hematológicas "Mariano R. Castex"; Argentina
Materia
ATYPICAL HEMOLYTIC UREMIC SYNDROME
AUTOANTIBODY
CHILDREN
COMPLEMENT
FACTOR H
Nivel de accesibilidad
acceso abierto
Condiciones de uso
https://creativecommons.org/licenses/by/2.5/ar/
Repositorio
CONICET Digital (CONICET)
Institución
Consejo Nacional de Investigaciones Científicas y Técnicas
OAI Identificador
oai:ri.conicet.gov.ar:11336/227864
Acceder
id CONICETDig_be0f5e7c8a8d308e909ad1c5a2a737eb
oai_identifier_str oai:ri.conicet.gov.ar:11336/227864
network_acronym_str CONICETDig
repository_id_str 3498
network_name_str CONICET Digital (CONICET)
spelling Anti-factor H autoantibody-associated hemolytic uremic syndrome in an Argentine pediatric cohortDos Santos, CéliaTrinidad, Jesica AuroraCastera, SantiagoAlconcher, LauraCoccia, Paula AlejandraManni, Federico JavierAlberto, Maria FabianaSánchez Luceros, Analía GabrielaATYPICAL HEMOLYTIC UREMIC SYNDROMEAUTOANTIBODYCHILDRENCOMPLEMENTFACTOR Hhttps://purl.org/becyt/ford/3.5https://purl.org/becyt/ford/3Aim: To describe the clinical characteristics and frequency of anti-factor H (FH) autoantibody-associated atypical hemolytic uremic syndrome (aHUS) in the first cohort of Argentine patients. Methods: The presence of anti-FH autoantibodies in 70 pediatric patients with suspected aHUS was investigated between 2013 and 2022. Clinical and laboratory parameters were collected and compared between patients who were positive and negative for anti-FH antibodies. Results: The 70 patients screened for anti-FH autoantibodies presented clinical features of non-immune microangiopathic hemolytic anemia, thrombocytopenia and renal injury. Positive titers were found in 14 children [mean: 1,938 arbitrary units per mL (AU/mL), range 179–8,500]. Due to missing clinical data, two patients who tested positive for anti-FH and 20 patients who tested negative for anti-FH were excluded from the data analysis. The laboratory features and clinical manifestations of anti-FH-positive aHUS cases (n = 12) were very similar to those of subjects with no autoantibodies detected (n = 36). Treatment administration was heterogeneous among the 12 patients analyzed. Dialysis was performed in six patients in total. Five children received plasmapheresis, while three patients were treated with plasma exchange followed by administration of eculizumab. Two patients received eculizumab only and one showed significant improvement solely through supportive care. Eight patients in total received immunosuppressive therapy. Follow-up of three patients showed a significant decrease of anti-FH autoantibody titers in 2/3 after treatment and during clinical remission. Conclusions: The cohort of 70 pediatric patients in this study demonstrated that the frequency of anti-FH autoantibody-associated aHUS in Argentina is 20%. The implementation of anti-FH testing in the country can potentially contribute to improved treatment and follow-up for patients with autoimmune aHUS.Fil: Dos Santos, Célia. Consejo Nacional de Investigaciones Científicas y Técnicas. Instituto de Medicina Experimental. Academia Nacional de Medicina de Buenos Aires. Instituto de Medicina Experimental; ArgentinaFil: Trinidad, Jesica Aurora. Consejo Nacional de Investigaciones Científicas y Técnicas. Instituto de Medicina Experimental. Academia Nacional de Medicina de Buenos Aires. Instituto de Medicina Experimental; ArgentinaFil: Castera, Santiago. Academia Nacional de Medicina de Buenos Aires. Instituto de Investigaciones Hematológicas "Mariano R. Castex"; ArgentinaFil: Alconcher, Laura. Hospital Municipal General de Agudos Doctor José Penna; ArgentinaFil: Coccia, Paula Alejandra. Hospital Italiano; ArgentinaFil: Manni, Federico Javier. Gobierno de la Provincia de Santa Fe. Hospital de Niños Doctor Orlando Alassia.; ArgentinaFil: Alberto, Maria Fabiana. Academia Nacional de Medicina de Buenos Aires. Instituto de Investigaciones Hematológicas "Mariano R. Castex"; ArgentinaFil: Sánchez Luceros, Analía Gabriela. Consejo Nacional de Investigaciones Científicas y Técnicas. Instituto de Medicina Experimental. Academia Nacional de Medicina de Buenos Aires. Instituto de Medicina Experimental; Argentina. Academia Nacional de Medicina de Buenos Aires. Instituto de Investigaciones Hematológicas "Mariano R. Castex"; ArgentinaOpen Exploration Publishing2023-11info:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersionhttp://purl.org/coar/resource_type/c_6501info:ar-repo/semantics/articuloapplication/pdfapplication/pdfhttp://hdl.handle.net/11336/227864Dos Santos, Célia; Trinidad, Jesica Aurora; Castera, Santiago; Alconcher, Laura; Coccia, Paula Alejandra; et al.; Anti-factor H autoantibody-associated hemolytic uremic syndrome in an Argentine pediatric cohort; Open Exploration Publishing; Exploration of Immunology; 3; 6; 11-2023; 513-5242768-6655CONICET DigitalCONICETenginfo:eu-repo/semantics/altIdentifier/url/https://www.explorationpub.com/Journals/ei/Article/1003118info:eu-repo/semantics/altIdentifier/doi/10.37349/ei.2023.00118info:eu-repo/semantics/openAccesshttps://creativecommons.org/licenses/by/2.5/ar/reponame:CONICET Digital (CONICET)instname:Consejo Nacional de Investigaciones Científicas y Técnicas2025-09-10T13:21:59Zoai:ri.conicet.gov.ar:11336/227864instacron:CONICETInstitucionalhttp://ri.conicet.gov.ar/Organismo científico-tecnológicoNo correspondehttp://ri.conicet.gov.ar/oai/requestdasensio@conicet.gov.ar; lcarlino@conicet.gov.arArgentinaNo correspondeNo correspondeNo correspondeopendoar:34982025-09-10 13:21:59.869CONICET Digital (CONICET) - Consejo Nacional de Investigaciones Científicas y Técnicasfalse
dc.title.none.fl_str_mv Anti-factor H autoantibody-associated hemolytic uremic syndrome in an Argentine pediatric cohort
title Anti-factor H autoantibody-associated hemolytic uremic syndrome in an Argentine pediatric cohort
spellingShingle Anti-factor H autoantibody-associated hemolytic uremic syndrome in an Argentine pediatric cohort
Dos Santos, Célia
ATYPICAL HEMOLYTIC UREMIC SYNDROME
AUTOANTIBODY
CHILDREN
COMPLEMENT
FACTOR H
title_short Anti-factor H autoantibody-associated hemolytic uremic syndrome in an Argentine pediatric cohort
title_full Anti-factor H autoantibody-associated hemolytic uremic syndrome in an Argentine pediatric cohort
title_fullStr Anti-factor H autoantibody-associated hemolytic uremic syndrome in an Argentine pediatric cohort
title_full_unstemmed Anti-factor H autoantibody-associated hemolytic uremic syndrome in an Argentine pediatric cohort
title_sort Anti-factor H autoantibody-associated hemolytic uremic syndrome in an Argentine pediatric cohort
dc.creator.none.fl_str_mv Dos Santos, Célia
Trinidad, Jesica Aurora
Castera, Santiago
Alconcher, Laura
Coccia, Paula Alejandra
Manni, Federico Javier
Alberto, Maria Fabiana
Sánchez Luceros, Analía Gabriela
author Dos Santos, Célia
author_facet Dos Santos, Célia
Trinidad, Jesica Aurora
Castera, Santiago
Alconcher, Laura
Coccia, Paula Alejandra
Manni, Federico Javier
Alberto, Maria Fabiana
Sánchez Luceros, Analía Gabriela
author_role author
author2 Trinidad, Jesica Aurora
Castera, Santiago
Alconcher, Laura
Coccia, Paula Alejandra
Manni, Federico Javier
Alberto, Maria Fabiana
Sánchez Luceros, Analía Gabriela
author2_role author
author
author
author
author
author
author
dc.subject.none.fl_str_mv ATYPICAL HEMOLYTIC UREMIC SYNDROME
AUTOANTIBODY
CHILDREN
COMPLEMENT
FACTOR H
topic ATYPICAL HEMOLYTIC UREMIC SYNDROME
AUTOANTIBODY
CHILDREN
COMPLEMENT
FACTOR H
purl_subject.fl_str_mv https://purl.org/becyt/ford/3.5
https://purl.org/becyt/ford/3
dc.description.none.fl_txt_mv Aim: To describe the clinical characteristics and frequency of anti-factor H (FH) autoantibody-associated atypical hemolytic uremic syndrome (aHUS) in the first cohort of Argentine patients. Methods: The presence of anti-FH autoantibodies in 70 pediatric patients with suspected aHUS was investigated between 2013 and 2022. Clinical and laboratory parameters were collected and compared between patients who were positive and negative for anti-FH antibodies. Results: The 70 patients screened for anti-FH autoantibodies presented clinical features of non-immune microangiopathic hemolytic anemia, thrombocytopenia and renal injury. Positive titers were found in 14 children [mean: 1,938 arbitrary units per mL (AU/mL), range 179–8,500]. Due to missing clinical data, two patients who tested positive for anti-FH and 20 patients who tested negative for anti-FH were excluded from the data analysis. The laboratory features and clinical manifestations of anti-FH-positive aHUS cases (n = 12) were very similar to those of subjects with no autoantibodies detected (n = 36). Treatment administration was heterogeneous among the 12 patients analyzed. Dialysis was performed in six patients in total. Five children received plasmapheresis, while three patients were treated with plasma exchange followed by administration of eculizumab. Two patients received eculizumab only and one showed significant improvement solely through supportive care. Eight patients in total received immunosuppressive therapy. Follow-up of three patients showed a significant decrease of anti-FH autoantibody titers in 2/3 after treatment and during clinical remission. Conclusions: The cohort of 70 pediatric patients in this study demonstrated that the frequency of anti-FH autoantibody-associated aHUS in Argentina is 20%. The implementation of anti-FH testing in the country can potentially contribute to improved treatment and follow-up for patients with autoimmune aHUS.
Fil: Dos Santos, Célia. Consejo Nacional de Investigaciones Científicas y Técnicas. Instituto de Medicina Experimental. Academia Nacional de Medicina de Buenos Aires. Instituto de Medicina Experimental; Argentina
Fil: Trinidad, Jesica Aurora. Consejo Nacional de Investigaciones Científicas y Técnicas. Instituto de Medicina Experimental. Academia Nacional de Medicina de Buenos Aires. Instituto de Medicina Experimental; Argentina
Fil: Castera, Santiago. Academia Nacional de Medicina de Buenos Aires. Instituto de Investigaciones Hematológicas "Mariano R. Castex"; Argentina
Fil: Alconcher, Laura. Hospital Municipal General de Agudos Doctor José Penna; Argentina
Fil: Coccia, Paula Alejandra. Hospital Italiano; Argentina
Fil: Manni, Federico Javier. Gobierno de la Provincia de Santa Fe. Hospital de Niños Doctor Orlando Alassia.; Argentina
Fil: Alberto, Maria Fabiana. Academia Nacional de Medicina de Buenos Aires. Instituto de Investigaciones Hematológicas "Mariano R. Castex"; Argentina
Fil: Sánchez Luceros, Analía Gabriela. Consejo Nacional de Investigaciones Científicas y Técnicas. Instituto de Medicina Experimental. Academia Nacional de Medicina de Buenos Aires. Instituto de Medicina Experimental; Argentina. Academia Nacional de Medicina de Buenos Aires. Instituto de Investigaciones Hematológicas "Mariano R. Castex"; Argentina
description Aim: To describe the clinical characteristics and frequency of anti-factor H (FH) autoantibody-associated atypical hemolytic uremic syndrome (aHUS) in the first cohort of Argentine patients. Methods: The presence of anti-FH autoantibodies in 70 pediatric patients with suspected aHUS was investigated between 2013 and 2022. Clinical and laboratory parameters were collected and compared between patients who were positive and negative for anti-FH antibodies. Results: The 70 patients screened for anti-FH autoantibodies presented clinical features of non-immune microangiopathic hemolytic anemia, thrombocytopenia and renal injury. Positive titers were found in 14 children [mean: 1,938 arbitrary units per mL (AU/mL), range 179–8,500]. Due to missing clinical data, two patients who tested positive for anti-FH and 20 patients who tested negative for anti-FH were excluded from the data analysis. The laboratory features and clinical manifestations of anti-FH-positive aHUS cases (n = 12) were very similar to those of subjects with no autoantibodies detected (n = 36). Treatment administration was heterogeneous among the 12 patients analyzed. Dialysis was performed in six patients in total. Five children received plasmapheresis, while three patients were treated with plasma exchange followed by administration of eculizumab. Two patients received eculizumab only and one showed significant improvement solely through supportive care. Eight patients in total received immunosuppressive therapy. Follow-up of three patients showed a significant decrease of anti-FH autoantibody titers in 2/3 after treatment and during clinical remission. Conclusions: The cohort of 70 pediatric patients in this study demonstrated that the frequency of anti-FH autoantibody-associated aHUS in Argentina is 20%. The implementation of anti-FH testing in the country can potentially contribute to improved treatment and follow-up for patients with autoimmune aHUS.
publishDate 2023
dc.date.none.fl_str_mv 2023-11
dc.type.none.fl_str_mv info:eu-repo/semantics/article
info:eu-repo/semantics/publishedVersion
http://purl.org/coar/resource_type/c_6501
info:ar-repo/semantics/articulo
format article
status_str publishedVersion
dc.identifier.none.fl_str_mv http://hdl.handle.net/11336/227864
Dos Santos, Célia; Trinidad, Jesica Aurora; Castera, Santiago; Alconcher, Laura; Coccia, Paula Alejandra; et al.; Anti-factor H autoantibody-associated hemolytic uremic syndrome in an Argentine pediatric cohort; Open Exploration Publishing; Exploration of Immunology; 3; 6; 11-2023; 513-524
2768-6655
CONICET Digital
CONICET
url http://hdl.handle.net/11336/227864
identifier_str_mv Dos Santos, Célia; Trinidad, Jesica Aurora; Castera, Santiago; Alconcher, Laura; Coccia, Paula Alejandra; et al.; Anti-factor H autoantibody-associated hemolytic uremic syndrome in an Argentine pediatric cohort; Open Exploration Publishing; Exploration of Immunology; 3; 6; 11-2023; 513-524
2768-6655
CONICET Digital
CONICET
dc.language.none.fl_str_mv eng
language eng
dc.relation.none.fl_str_mv info:eu-repo/semantics/altIdentifier/url/https://www.explorationpub.com/Journals/ei/Article/1003118
info:eu-repo/semantics/altIdentifier/doi/10.37349/ei.2023.00118
dc.rights.none.fl_str_mv info:eu-repo/semantics/openAccess
https://creativecommons.org/licenses/by/2.5/ar/
eu_rights_str_mv openAccess
rights_invalid_str_mv https://creativecommons.org/licenses/by/2.5/ar/
dc.format.none.fl_str_mv application/pdf
application/pdf
dc.publisher.none.fl_str_mv Open Exploration Publishing
publisher.none.fl_str_mv Open Exploration Publishing
dc.source.none.fl_str_mv reponame:CONICET Digital (CONICET)
instname:Consejo Nacional de Investigaciones Científicas y Técnicas
reponame_str CONICET Digital (CONICET)
collection CONICET Digital (CONICET)
instname_str Consejo Nacional de Investigaciones Científicas y Técnicas
repository.name.fl_str_mv CONICET Digital (CONICET) - Consejo Nacional de Investigaciones Científicas y Técnicas
repository.mail.fl_str_mv dasensio@conicet.gov.ar; lcarlino@conicet.gov.ar
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score 12.48226

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