Adolescence-onset atypical hemolytic uremic syndrome: is it different from infant-onset?

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dc.authoridZeybek, Cengiz/0000-0002-4820-0373
dc.authoridCaltik Yilmaz, Aysun/0000-0003-0774-4419
dc.authoridcelakil, mehtap/0000-0002-5354-1455
dc.authoridNalcacioglu, Hulya/0000-0002-0686-9714
dc.contributor.authorCelegen, Kubra
dc.contributor.authorGulhan, Bora
dc.contributor.authorFidan, Kibriya
dc.contributor.authorYuksel, Selcuk
dc.contributor.authorYilmaz, Neslihan
dc.contributor.authorYilmaz, Aysun Caltik
dc.contributor.authorKilic, Beltinge Demircioglu
dc.date.accessioned2025-01-27T20:43:53Z
dc.date.available2025-01-27T20:43:53Z
dc.date.issued2024
dc.departmentÇanakkale Onsekiz Mart Üniversitesi
dc.description.abstractBackground: Atypical hemolytic uremic syndrome (aHUS) is a rare, mostly complement-mediated thrombotic microangiopathy. The majority of patients are infants. In contrast to infantile-onset aHUS, the clinical and genetic characteristics of adolescence-onset aHUS have not been sufficiently addressed to date. Methods: A total of 28 patients (21 girls, 7 boys) who were diagnosed as aHUS between the ages of >= 10 years and <18 years were included in this study. All available data in the Turkish Pediatric aHUS registry were collected and analyzed. Results: The mean age at diagnosis was 12.8 +/- 2.3 years. Extra-renal involvement was noted in 13 patients (46.4%); neurological involvement was the most common (32%). A total of 21 patients (75%) required kidney replacement therapy. Five patients (17.8%) received only plasma therapy and 23 (82%) of the patients received eculizumab. Hematologic remission and renal remission were achieved in 25 (89.3%) and 17 (60.7%) of the patients, respectively. Compared with the infantile-onset aHUS patients, adolescent patients had a lower complete remission rate during the first episode (p = 0.002). Genetic analyses were performed in all and a genetic variant was detected in 39.3% of the patients. The mean follow-up duration was 4.9 +/- 2.6 years. At the last visit, adolescent patients had lower eGFR levels (p = 0.03) and higher rates of chronic kidney disease stage 5 when compared to infantile-onset aHUS patients (p = 0.04). Conclusions: Adolescence-onset aHUS is a rare disease but tends to cause more permanent renal dysfunction than infantile-onset aHUS. These results may modify the management approaches in these patients.
dc.description.sponsorshipScientific Research Projects Coordination Unit of Hacettepe University (Project No. TSA-2019-18022)
dc.description.sponsorshipNo Statement Available
dc.identifier.doi10.1007/s10157-024-02505-7
dc.identifier.endpage1037
dc.identifier.issn1342-1751
dc.identifier.issn1437-7799
dc.identifier.issue10
dc.identifier.pmid38704765
dc.identifier.scopus2-s2.0-85192103978
dc.identifier.scopusqualityQ2
dc.identifier.startpage1027
dc.identifier.urihttps://doi.org/10.1007/s10157-024-02505-7
dc.identifier.urihttps://hdl.handle.net/20.500.12428/24407
dc.identifier.volume28
dc.identifier.wosWOS:001214205200001
dc.identifier.wosqualityN/A
dc.indekslendigikaynakWeb of Science
dc.indekslendigikaynakScopus
dc.indekslendigikaynakPubMed
dc.language.isoen
dc.publisherSpringer
dc.relation.ispartofClinical and Experimental Nephrology
dc.relation.publicationcategoryinfo:eu-repo/semantics/openAccess
dc.rightsinfo:eu-repo/semantics/closedAccess
dc.snmzKA_WoS_20250125
dc.subjectAtypical hemolytic uremic syndrome
dc.subjectAdolescence
dc.subjectGenetics
dc.subjectComplement
dc.subjectTurkish aHUS registry
dc.titleAdolescence-onset atypical hemolytic uremic syndrome: is it different from infant-onset?
dc.typeArticle

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