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

Autor: Celegen K; Department of Pediatric Nephrology, Kayseri Education and Research Hospital, Kayseri, Türkiye., Gulhan B; Department of Pediatric Nephrology, Faculty of Medicine, Hacettepe University, 06100, Sihhiye, Ankara, Turkey. bora.gulhan@hacettepe.edu.tr., Fidan K; Department of Pediatric Nephrology, Faculty of Medicine, Gazi University, Ankara, Türkiye., Yuksel S; Department of Pediatric Nephrology, Faculty of Medicine, Çanakkale Onsekiz Mart University, Çanakkale, Türkiye., Yilmaz N; Department of Pediatric Nephrology, Necip Fazil City Hospital, Kahramanmaras, Türkiye., Yılmaz AC; Department of Pediatric Nephrology, Faculty of Medicine, Baskent University, Ankara, Türkiye., Demircioğlu Kılıç B; Department of Pediatric Nephrology, Faculty of Medicine, Gaziantep University, Gaziantep, Türkiye., Gokce I; Department of Pediatric Nephrology, Faculty of Medicine, Marmara University, Istanbul, Türkiye., Kavaz Tufan A; Department of Pediatric Nephrology, Faculty of Medicine, Osmangazi University, Eskisehir, Türkiye., Kalyoncu M; Department of Pediatric Nephrology, Faculty of Medicine, Karadeniz Technical University, Trabzon, Türkiye., Nalcacıoglu H; Department of Pediatric Nephrology, Faculty of Medicine, Ondokuz Mayıs University, Samsun, Türkiye., Ozlu SG; Department of Pediatric Nephrology, Ankara City Training and Research Hospital, Ankara, Türkiye., Kurt Sukur ED; Department of Pediatric Nephrology, Faculty of Medicine, Hacettepe University, 06100, Sihhiye, Ankara, Turkey., Canpolat N; Department of Pediatric Nephrology, Cerrahpasa Faculty of Medicine, Istanbul University-Cerrahpasa, İstanbul, Türkiye., K Bayazit A; Department of Pediatric Nephrology, Faculty of Medicine, Cukurova University, Adana, Türkiye., Çomak E; Department of Pediatric Nephrology, Faculty of Medicine, Akdeniz University, Antalya, Türkiye., Tabel Y; Department of Pediatric Nephrology, Faculty of Medicine, Inonu University, Malatya, Türkiye., Tulpar S; Department of Pediatric Nephrology, Istanbul Bakirkoy Dr. Sadi Konuk Training and Research Hospital, University of Health Sciences, İstanbul, Türkiye., Celakil M; Department of Pediatric Nephrology, Sakarya University Training and Research Hospital, Sakarya, Türkiye., Bek K; Department of Pediatric Nephrology, Faculty of Medicine, Kocaeli University, Kocaeli, Türkiye., Zeybek C; Department of Pediatric Nephrology, Gulhane Training and Research Hospital, Ankara, Türkiye., Duzova A; Department of Pediatric Nephrology, Faculty of Medicine, Hacettepe University, 06100, Sihhiye, Ankara, Turkey., Özçakar ZB; Department of Pediatric Nephrology, Faculty of Medicine, Ankara University, Ankara, Türkiye., Topaloglu R; Department of Pediatric Nephrology, Faculty of Medicine, Hacettepe University, 06100, Sihhiye, Ankara, Turkey., Soylemezoglu O; Department of Pediatric Nephrology, Faculty of Medicine, Gazi University, Ankara, Türkiye., Ozaltin F; Department of Pediatric Nephrology, Faculty of Medicine, Hacettepe University, 06100, Sihhiye, Ankara, Turkey.; Nephrogenetics Laboratory, Faculty of Medicine, Hacettepe University, Ankara, Türkiye.; Center for Genomics and Rare Diseases, Hacettepe University, Ankara, Türkiye.; Department of Bioinformatics, Hacettepe University Institute of Health Sciences, Ankara, Türkiye.
Jazyk: angličtina
Zdroj: Clinical and experimental nephrology [Clin Exp Nephrol] 2024 Oct; Vol. 28 (10), pp. 1027-1037. Date of Electronic Publication: 2024 May 05.
DOI: 10.1007/s10157-024-02505-7
Abstrakt: 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.
(© 2024. The Author(s), under exclusive licence to Japanese Society of Nephrology.)
Databáze: MEDLINE
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