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Öğe Adolescence-onset atypical hemolytic uremic syndrome: is it different from infant-onset?(Springer, 2024) Celegen, Kubra; Gulhan, Bora; Fidan, Kibriya; Yuksel, Selcuk; Yilmaz, Neslihan; Yilmaz, Aysun Caltik; Kilic, Beltinge DemirciogluBackground: 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.Öğe Adolescence-onset atypical hemolytic uremic syndrome: is it different from infant-onset? (MAY, 10.1007/s10157-024-02505-7, 2024)(Springer, 2024) Celegen, Kubra; Gulhan, Bora; Fidan, Kibriya; Yuksel, Selcuk; Yilmaz, Neslihan; Yilmaz, Aysun Caltik; Kilic, Beltinge Demircioglu[Abstract Not Available]Öğe CLINICAL COURSE OF ADOLESCENT ONSET ATYPICAL HEMOLYTIC UREMIC SYNDROME: A STUDY OF TURKISH AHUS REGISTRY(Springer, 2022) Celegen, Kubra; Gulhan, Bora; Fidan, Kibriya; Yuksel, Selcuk; Yilmaz, Neslihan; Yilmaz, Aysun Caltik; Kilic, Beltinge Demircioglu[Abstract Not Available]
