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Comparative Study
. 2024 Oct;28(10):1027-1037.
doi: 10.1007/s10157-024-02505-7. Epub 2024 May 5.

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

Kubra Celegen #  1 , Bora Gulhan #  2 , Kibriya Fidan  3 , Selcuk Yuksel  4 , Neslihan Yilmaz  5 , Aysun Caltik Yılmaz  6 , Beltinge Demircioğlu Kılıç  7 , Ibrahim Gokce  8 , Aslı Kavaz Tufan  9 , Mukaddes Kalyoncu  10 , Hulya Nalcacıoglu  11 , Sare Gulfem Ozlu  12 , Eda Didem Kurt Sukur  13 , Nur Canpolat  14 , Aysun K Bayazit  15 , Elif Çomak  16 , Yılmaz Tabel  17 , Sebahat Tulpar  18 , Mehtap Celakil  19 , Kenan Bek  20 , Cengiz Zeybek  21 , Ali Duzova  13 , Zeynep Birsin Özçakar  22 , Rezan Topaloglu  13 , Oguz Soylemezoglu  3 , Fatih Ozaltin  13   23   24   25
Affiliations
Comparative Study

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

Kubra Celegen et al. Clin Exp Nephrol. 2024 Oct.

Erratum in

  • Correction to: Adolescence‑onset atypical hemolytic uremic syndrome: is it different from infant‑onset?
    Celegen K, Gulhan B, Fidan K, Yuksel S, Yilmaz N, Yılmaz AC, Demircioğlu Kılıç B, Gokce I, Kavaz Tufan A, Kalyoncu M, Nalcacıoglu H, Ozlu SG, Kurt Sukur ED, Canpolat N, K Bayazit A, Çomak E, Tabel Y, Tulpar S, Celakil M, Bek K, Zeybek C, Duzova A, Özçakar ZB, Topaloglu R, Soylemezoglu O, Ozaltin F. Celegen K, et al. Clin Exp Nephrol. 2024 Oct;28(10):1038-1039. doi: 10.1007/s10157-024-02520-8. Clin Exp Nephrol. 2024. PMID: 38970651 No abstract available.

Abstract

Background: 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.

Keywords: Adolescence; Atypical hemolytic uremic syndrome; Complement; Genetics; Turkish aHUS registry.

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References

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