Atypical Hemolytic Uremic Syndrome in Children Aged < 2 Years

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dc.contributor.authorCakar, Nilgun
dc.contributor.authorOzcakar, Z. Birsin
dc.contributor.authorOzaltin, Fatih
dc.contributor.authorKoyun, Mustafa
dc.contributor.authorAcar, Banu Celikel
dc.contributor.authorBahat, Elif
dc.contributor.authorYalcinkaya, Fatos
dc.date.accessioned2020-06-25T18:30:01Z
dc.date.available2020-06-25T18:30:01Z
dc.date.issued2018
dc.departmentKırıkkale Üniversitesi
dc.descriptionAgbas, Ayse/0000-0002-3658-8622; Ozcakar, Zeynep/0000-0002-6376-9189; Cakar, Nilgun/0000-0002-1853-0101
dc.description.abstractBackground: There are limited data on infants with atypical hemolytic uremic syndrome (aHUS). The aim of this study was to determine the clinical and laboratory features, and to evaluate treatment modalities and outcomes in infants with aHUS. Materials and Methods: Relevant data on patients with onset of aHUS at age <2 years were obtained from the Turkish Pediatric aHUS Registry. Results: Among the 146 patients included in the Registry, 53 (36%) (23 male and 30 female) were enrolled for the study. Age at disease onset was <= 1 year in 29 of the patients. In all, 21 (40%) of the patients developed neurological symptoms. Disease-causing muta tions were noted in 14 (36%) of the 39 patients in which genetic analysis was performed. Plasma therapy was performed in 42 (79%) patients; eculizumab therapy was administered to treat the first episode of aHUS in 33 (62%) patients and in 5 patients as the first- line therapy. In total, 38 (72%) patients received renal replacement therapy (RRT), 3 (6%) died due to acute illness, and 4 (8%) were discharged from hospital with RRT. Follow-up visit data were available for 46 patients and the median duration was 23 months (range 3-129 months). End-stage renal disease developed only in 1 patient. Proteinuria and hypertension persisted in 17 (37%) and 20 patients (44%) respectively. Eculizumab treatment was continued in 25 of the 39 patients during the follow-up period. Conclusion:One-third of the aHUS patients had disease onset during infancy. The prognosis of this life-threatening disease seems to get better with improved treatment modalities. (C) 2018 S. Karger AG, Baselen_US
dc.description.sponsorshipHacettepe University Scientific Research and Development OfficeHacettepe University [010A101009]; Alexion Pharmaceuticals, Inc.Fukuda Foundation for Medical TechnologyKayamori Foundationen_US
dc.description.sponsorshipThis study was supported by Hacettepe University Scientific Research and Development Office (grant No.: 010A101009). The article processing charge of this manuscript has been granted by the Alexion Pharmaceuticals, Inc.en_US
dc.identifier.citationclosedAccessen_US
dc.identifier.doi10.1159/000487609
dc.identifier.endpage218en_US
dc.identifier.issn1660-8151
dc.identifier.issn2235-3186
dc.identifier.issue3en_US
dc.identifier.pmid29533929
dc.identifier.scopus2-s2.0-85044167048
dc.identifier.scopusqualityQ1
dc.identifier.startpage211en_US
dc.identifier.urihttps://doi.org/10.1159/000487609
dc.identifier.urihttps://hdl.handle.net/20.500.12587/7535
dc.identifier.volume139en_US
dc.identifier.wosWOS:000437355600002
dc.identifier.wosqualityQ3
dc.indekslendigikaynakWeb of Science
dc.indekslendigikaynakScopus
dc.indekslendigikaynakPubMed
dc.language.isoen
dc.publisherKargeren_US
dc.relation.ispartofNephron
dc.relation.publicationcategoryMakale - Uluslararası Hakemli Dergi - Kurum Öğretim Elemanıen_US
dc.rightsinfo:eu-repo/semantics/closedAccessen_US
dc.subjectAtypical hemolytic uremic syndromeen_US
dc.subjectChildrenen_US
dc.subjectPlasma therapyen_US
dc.subjectEculizumaben_US
dc.subjectTreatmenten_US
dc.subjectOutcomeen_US
dc.titleAtypical Hemolytic Uremic Syndrome in Children Aged < 2 Yearsen_US
dc.typeArticle

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