Affiliation:
1. MANISA CELAL BAYAR UNIVERSITY, SCHOOL OF MEDICINE
2. EGE UNIVERSITY, EGE FACULTY OF MEDICINE
Abstract
Aim: Hemolytic uremic syndrome (HUS) is the clinical triad of anemia, thrombocytopenia and acute renal injury. Atypical hemolytic uremic syndrome (aHUS) is a rare disease of alternative complement pathways. It is aimed to evaluate long-term follow-up of patients with aHUS in the present study. Materials and methods: Eleven children diagnosed with aHUS were retrospectively evaluated. Demographic, clinical, and laboratory data and treatment details were reported. Results: A total of 11 patients were enrolled in the study. The mean age of patients at aHUS onset was 2.9±6 years. The mean follow-up time was 72 ± 4 months All patients had renal involvement. Extrarenal manifestations of aHUS were present in four patients. All patients had eculizumab treatment. Conclusion: Our study insight into diagnosing and managing aHUS, a very rare disease, in our pediatric patients. Genetic testing is used to improve the diagnosis of aHUS. We demonstrated the long-term efficacy and safety of eculizumab in our aHUS patients. Further studies are needed to determine the optimal time for discontinuation of eculizumab treatment.
Publisher
Saglik Bilimleri Universitesi
Reference24 articles.
1. Referans1. Raina R, Krishnappa V, Blaha T, Kann T, Hein W, Burke L et al. Atypical hemolytic-uremic syndrome: An update on pathophysiology, diagnosis, and treatment. Ther Apher Dial. 2019;23:4-21.
2. Referans2. Gasser C, Gautier E, Steck A, Siebenmann RE, Oechslin R. Hemolytic-uremic syndrome: bilateral necrosis of the renal cortex in acute acquired hemolytic anemia. Schweiz Med Wochenschr. 1955;85:905–9.
3. Referans3. Loirat C, Fakhouri F, Ariceta G, Besbas N, Bitzan M, Bjerre A et al. An international consensus approach to the management of atypical hemolytic uremic syndrome in children. Pediatr Nephrol. 2016;31:15–39.
4. Referans4. Zimmerhackl LB, Besbas N, Jungraithmayr T, Van De Kar N, Karch H, Karpman D et al. Epidemiology, clinical presentation, and pathophysiology of atypical and recurrent hemolytic uremic syndrome. Semin Thromb Hemost. 2006;32:113-20.
5. Referans5. Valoti E, Alberti M, Iatropoulos P, Piras R, Mele C, Breno M et al. Rare functional variants in complement genes and anti-FH autoantibodies-associated aHUS. Front Immunol. 2019;10:853.
Referans6. Campistol JM, Arias M, Ariceta G, Blasco M, Espinosa L, Espinosa M et al. An update for atypical haemolytic uraemic syndrome: diagnosis and treatment. A consensus document. Nefrologia. 2015;35:421-47.
Referans7. Dixon BP, Gruppo RA. Atypical hemolytic uremic syndrome. Pediatr Clin North Am. 2018;65:509-25.