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      Atypical Hemolytic Uremic Syndrome in Children Aged <2 Years

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          Background: 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 mutations 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<sup></sup> of the aHUS patients had disease onset during infancy. The prognosis of this life-threatening disease seems to get better with improved treatment modalities.

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          Author and article information

          S. Karger AG
          June 2018
          13 March 2018
          : 139
          : 3
          : 211-218
          aDepartment of Pediatric Nephrology, Ankara University Faculty of Medicine, Ankara, Turkey
          bDepartment of Pediatric Nephrology, Hacettepe University Faculty of Medicine, Ankara, Turkey
          cDepartment of Pediatric Nephrology, Akdeniz University Faculty of Medicine, Ankara, Turkey
          dDepartment of Pediatric Nephrology, Kirikkale University Faculty of Medicine, Kirikkale, Turkey
          eDepartment of Pediatric Nephrology, Karadeniz Technical University Faculty of Medicine, Trabzon, Turkey
          fDepartment of Pediatric Nephrology, Nephrogenetics Laboratory, Hacettepe University Faculty of Medicine, Ankara, Turkey
          gDepartment of Pediatric Nephrology, Istanbul University, Cerrahpasa Faculty of Medicine, Istanbul, Turkey
          hDepartment of Pediatric Nephrology, Gazi University Faculty of Medicine, Ankara, Turkey
          iHacettepe University Center for Biobanking and Genomics, Ankara, Turkey
          Author notes
          *Dr. Nilgun Çakar, Department of Pediatric Nephrology, Ankara University Faculty of Medicine, TR–06100 Cebeci, Ankara (Turkey), E-Mail
          487609 Nephron 2018;139:211–218
          © 2018 S. Karger AG, Basel

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          Page count
          Tables: 4, Pages: 8
          Clinical Practice: Original Paper


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