Eculizumab in a child with atypical haemolytic uraemic syndrome and haemophagocytic lymphohistiocytosis triggered by cytomegalovirus infection

We present the case of a 21-month-old girl with two rare and life-threatening conditions, atypical haemolytic uraemic syndrome (aHUS) and haemophagocytic lymphohistiocytosis (HLH), triggered by a cytomegalovirus (CMV) infection. Soon after admission, the girl became anuric and required continuous ve...

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Autores: Fraga Rodriguez, Gloria Maria|||0000-0002-7682-1396, Brió Sanagustín, Sonia|||0000-0002-1520-4618, Turon Viñas, Eulalia|||0000-0001-6171-0282, Dixon, Bradley P.., Carreras, Eduardo
Formato: artículo
Fecha de publicación:2017
País:España
Recursos:Universitat Autònoma de Barcelona
Repositorio:Dipòsit Digital de Documents de la UAB
Idioma:inglés
OAI Identifier:oai:ddd.uab.cat:186218
Acesso em linha:https://ddd.uab.cat/record/186218
https://dx.doi.org/urn:doi:10.1136/bcr-2016-219065
Access Level:acceso abierto
Palavra-chave:Haematology (drugs and medicines)
Paediatrics (drugs and medicines)
Renal system
Haematology (incl blood transfusion)
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spelling Eculizumab in a child with atypical haemolytic uraemic syndrome and haemophagocytic lymphohistiocytosis triggered by cytomegalovirus infectionFraga Rodriguez, Gloria Maria|||0000-0002-7682-1396Brió Sanagustín, Sonia|||0000-0002-1520-4618Turon Viñas, Eulalia|||0000-0001-6171-0282Dixon, Bradley P..Carreras, EduardoHaematology (drugs and medicines)Paediatrics (drugs and medicines)Renal systemHaematology (incl blood transfusion)We present the case of a 21-month-old girl with two rare and life-threatening conditions, atypical haemolytic uraemic syndrome (aHUS) and haemophagocytic lymphohistiocytosis (HLH), triggered by a cytomegalovirus (CMV) infection. Soon after admission, the girl became anuric and required continuous venovenous haemodiafiltration. Initial treatments included methylprednisolone, fibrinogen and plasma infusion (for HLH), plasmapheresis (for thrombotic microangiopathy), immunoglobulins (for inflammation), ganciclovir (for CMV infection) and the antibiotic cefotaxime. On day 5, eculizumab (600 mg) was given for aHUS, with rapid improvement in haematological and nephrological parameters. Despite a subsequent isolated episode of right heart thrombosis that resolved with heparin treatment, the patient showed a favourable response to eculizumab (300 mg/15 days), with improved renal function, normal haematological values, and no treatment complications. In conclusion, eculizumab effectively treated aHUS in this case despite a comorbid immunological disease.Universitat Autònoma de Barcelona 22017-01-0120172017-01-01Articlehttp://purl.org/coar/resource_type/c_6501VoRhttp://purl.org/coar/version/c_970fb48d4fbd8a85info:eu-repo/semantics/articleapplication/pdfhttps://ddd.uab.cat/record/186218https://dx.doi.org/urn:doi:10.1136/bcr-2016-219065reponame:Dipòsit Digital de Documents de la UABinstname:Universitat Autònoma de BarcelonaInglésengopen accesshttp://purl.org/coar/access_right/c_abf2Aquest document està subjecte a una llicència d'ús Creative Commons. Es permet la reproducció total o parcial, la distribució, la comunicació pública de l'obra i la creació d'obres derivades, sempre que no sigui amb finalitats comercials, i sempre que es reconegui l'autoria de l'obra original.https://creativecommons.org/licenses/by-nc/4.0/info:eu-repo/semantics/openAccessoai:ddd.uab.cat:1862182026-06-06T12:50:31Z
dc.title.none.fl_str_mv Eculizumab in a child with atypical haemolytic uraemic syndrome and haemophagocytic lymphohistiocytosis triggered by cytomegalovirus infection
title Eculizumab in a child with atypical haemolytic uraemic syndrome and haemophagocytic lymphohistiocytosis triggered by cytomegalovirus infection
spellingShingle Eculizumab in a child with atypical haemolytic uraemic syndrome and haemophagocytic lymphohistiocytosis triggered by cytomegalovirus infection
Fraga Rodriguez, Gloria Maria|||0000-0002-7682-1396
Haematology (drugs and medicines)
Paediatrics (drugs and medicines)
Renal system
Haematology (incl blood transfusion)
title_short Eculizumab in a child with atypical haemolytic uraemic syndrome and haemophagocytic lymphohistiocytosis triggered by cytomegalovirus infection
title_full Eculizumab in a child with atypical haemolytic uraemic syndrome and haemophagocytic lymphohistiocytosis triggered by cytomegalovirus infection
title_fullStr Eculizumab in a child with atypical haemolytic uraemic syndrome and haemophagocytic lymphohistiocytosis triggered by cytomegalovirus infection
title_full_unstemmed Eculizumab in a child with atypical haemolytic uraemic syndrome and haemophagocytic lymphohistiocytosis triggered by cytomegalovirus infection
title_sort Eculizumab in a child with atypical haemolytic uraemic syndrome and haemophagocytic lymphohistiocytosis triggered by cytomegalovirus infection
dc.creator.none.fl_str_mv Fraga Rodriguez, Gloria Maria|||0000-0002-7682-1396
Brió Sanagustín, Sonia|||0000-0002-1520-4618
Turon Viñas, Eulalia|||0000-0001-6171-0282
Dixon, Bradley P..
Carreras, Eduardo
author Fraga Rodriguez, Gloria Maria|||0000-0002-7682-1396
author_facet Fraga Rodriguez, Gloria Maria|||0000-0002-7682-1396
Brió Sanagustín, Sonia|||0000-0002-1520-4618
Turon Viñas, Eulalia|||0000-0001-6171-0282
Dixon, Bradley P..
Carreras, Eduardo
author_role author
author2 Brió Sanagustín, Sonia|||0000-0002-1520-4618
Turon Viñas, Eulalia|||0000-0001-6171-0282
Dixon, Bradley P..
Carreras, Eduardo
author2_role author
author
author
author
dc.contributor.none.fl_str_mv Universitat Autònoma de Barcelona
dc.subject.none.fl_str_mv Haematology (drugs and medicines)
Paediatrics (drugs and medicines)
Renal system
Haematology (incl blood transfusion)
topic Haematology (drugs and medicines)
Paediatrics (drugs and medicines)
Renal system
Haematology (incl blood transfusion)
description We present the case of a 21-month-old girl with two rare and life-threatening conditions, atypical haemolytic uraemic syndrome (aHUS) and haemophagocytic lymphohistiocytosis (HLH), triggered by a cytomegalovirus (CMV) infection. Soon after admission, the girl became anuric and required continuous venovenous haemodiafiltration. Initial treatments included methylprednisolone, fibrinogen and plasma infusion (for HLH), plasmapheresis (for thrombotic microangiopathy), immunoglobulins (for inflammation), ganciclovir (for CMV infection) and the antibiotic cefotaxime. On day 5, eculizumab (600 mg) was given for aHUS, with rapid improvement in haematological and nephrological parameters. Despite a subsequent isolated episode of right heart thrombosis that resolved with heparin treatment, the patient showed a favourable response to eculizumab (300 mg/15 days), with improved renal function, normal haematological values, and no treatment complications. In conclusion, eculizumab effectively treated aHUS in this case despite a comorbid immunological disease.
publishDate 2017
dc.date.none.fl_str_mv 2
2017-01-01
2017
2017-01-01
dc.type.none.fl_str_mv Article
http://purl.org/coar/resource_type/c_6501
VoR
http://purl.org/coar/version/c_970fb48d4fbd8a85
dc.type.openaire.fl_str_mv info:eu-repo/semantics/article
format article
dc.identifier.none.fl_str_mv https://ddd.uab.cat/record/186218
https://dx.doi.org/urn:doi:10.1136/bcr-2016-219065
url https://ddd.uab.cat/record/186218
https://dx.doi.org/urn:doi:10.1136/bcr-2016-219065
dc.language.none.fl_str_mv Inglés
eng
language_invalid_str_mv Inglés
language eng
dc.rights.none.fl_str_mv open access
http://purl.org/coar/access_right/c_abf2
https://creativecommons.org/licenses/by-nc/4.0/
dc.rights.openaire.fl_str_mv info:eu-repo/semantics/openAccess
rights_invalid_str_mv open access
http://purl.org/coar/access_right/c_abf2
https://creativecommons.org/licenses/by-nc/4.0/
eu_rights_str_mv openAccess
dc.format.none.fl_str_mv application/pdf
dc.source.none.fl_str_mv reponame:Dipòsit Digital de Documents de la UAB
instname:Universitat Autònoma de Barcelona
instname_str Universitat Autònoma de Barcelona
reponame_str Dipòsit Digital de Documents de la UAB
collection Dipòsit Digital de Documents de la UAB
repository.name.fl_str_mv
repository.mail.fl_str_mv
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score 15,198674

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