aHUS

1. Zuber J, Fakhouri F, Roumenina LT, Loirat C, Frémeaux-Bacchi V. Use of eculizumab for atypical haemolytic uraemic syndrome and C3 glomerulopathies. Nat Rev Nephrol. 2012;8:643–57.

2. Masias C, Vasu S, Cataland SR. None of the above: Thrombotic microangiopathy beyond TTP and HUS. Blood. 2017;129:2857–63.

3. Amirlak I, Amirlak B. Haemolytic uraemic syndrome: An overview. Nephrology. 2006;11:213–8.

4. Meri S. Complement activation in diseases presenting with thrombotic microangiopathy. Eur J Intern Med [Internet]. European Federation of Internal Medicine.; 2013;24:496–502. Available from: http://dx.doi.org/10.1016/j.ejim.2013.05.009

5. Wong EKS, Kavanagh D. Diseases of complement dysregulation — an overview . Seminars in Immunopathology 2018 DOI : https://doi.org/10.1007/s00281-017-0663-8 Date deposited : Seminars in Immunopathology; 2018;49–64.

6. Spasiano A, Palazzetti D, Dimartino L, Bruno F, Baccaro R, Pesce F, et al. Underlying Genetics of aHUS: Which Connection with Outcome and Treatment Discontinuation? Int J Mol Sci. 2023;24.

7. Juliette Leon, Marie-Bénédicte LeStang RS-S, Aude Servais, Dany Anglicheau VF-B, Zuber J. Complement‐driven hemolytic uremic syndrome. Am Jounral oh Hematol. 2023;98:S44–56.

8. Registry B, Uremic AH. B razilian S ociety of N ephrology Atypical Hemolytic Uremic Syndrome ( aHUS ): an expert consensus statement from the Rare Diseases Committee. 2025;1–22.

9. Bogdan RG, Anderco P, Ichim C, Cimpean AM, Todor SB, Glaja-Iliescu M, et al. Atypical Hemolytic Uremic Syndrome: A Review of Complement Dysregulation, Genetic Susceptibility and Multiorgan Involvement. J Clin Med. 2025;14:1–20.

10. Halimi JM, Al-Dakkak I, Anokhina K, Ardissino G, Licht C, Lim WH, et al. Clinical characteristics and outcomes of a patient population with atypical hemolytic uremic syndrome and malignant hypertension: analysis from the Global aHUS registry. J Nephrol [Internet]. Springer International Publishing; 2023;36:817–28. Available from: https://doi.org/10.1007/s40620-022-01465-z

11. Zuber, Julien, Moglie Le Quintrec, Heather Morris, Véronique Frémeaux-Bacchi, Chantal Loirat CL. Targeted strategies in the prevention and management of atypical HUS recurrence after kidney transplantation. Transplant Rev. 2013;27:117–25.

12. Goodship THJ, Cook HT, Fakhouri F, Fervenza FC, Frémeaux-Bacchi V, Kavanagh D, et al. Atypical hemolytic uremic syndrome and C3 glomerulopathy: conclusions from a “Kidney Disease: Improving Global Outcomes” (KDIGO) Controversies Conference. Kidney Int. 2017;91:539–51.

13. Vivarelli M, Barratt J, Beck LH, Fakhouri F, Gale DP, Goicoechea de Jorge E, et al. The role of complement in kidney disease: conclusions from a Kidney Disease: Improving Global Outcomes (KDIGO) Controversies Conference. Kidney Int. 2024;106:369–91.

14. Laurence J. Atypical hemolytic uremic syndrome (aHUS): making the diagnosis. Clin Adv Hematol Oncol. 2012;10:1–12.

15. Noris M, Remuzzi G. Atypical Hemolytic–Uremic Syndrome. N Engl J Med. 2009;361:1676–87.

16. Legendre CM, Licht C, Muus P, Greenbaum LA, Babu S, Bedrosian C, et al. Terminal Complement Inhibitor Eculizumab in Atypical Hemolytic–Uremic Syndrome. N Engl J Med. 2013;368:2169–81.

17. Laurence J. Atypical hemolytic uremic syndrome (aHUS): treating the patient. Clin Adv Hematol Oncol. 2013;11:4–15.

18. Frémeaux-Bacchi CL and V. Atypical hemolytic uremic syndrome. Orphanet J Rare Dis Rare Dis. 2011;6:1–30.

19. Zini G, d’Onofrio G, Briggs C, Erber W, Jou JM, Lee SH, et al. ICSH recommendations for identification, diagnostic value, and quantitation of schistocytes. Int J Lab Hematol. 2012;34:107–16.

20. Schapkaitz E, Mezgebe MH. The Clinical Significance of Schistocytes: A Prospective Evaluation of the International Council for Standardization in Hematology Schistocyte Guidelines. Turkish J Hematol. 2017;34:59–63.

21. Noutsos T, Laidman AY, Survela L, Arvanitis D, Segalla R, Brown SG, et al. An evaluation of existing manual blood film schistocyte quantitation guidelines and a new proposed method. Pathology. 2021;53:746–52.

22. McFarlane PA, Bitzan M, Broome C, Baran D, Garland J, Girard LP, et al. Making the Correct Diagnosis in Thrombotic Microangiopathy: A Narrative Review. Can J Kidney Heal Dis. 2021;8.

23. Arif Asif , Tushar Vachharajani LS and AN. A Simplified Approach to the Diagnosis of Atypical HUS: Clinical Considerations and Practical Implications. Open Urol Nephrol J. 2014;7:91–100.

24. Kato H, Nangaku M, Hataya H, Sawai T, Ashida A, Fujimaru R, et al. Clinical guides for atypical hemolytic uremic syndrome in Japan. Clin Exp Nephrol. Springer Japan; 2016;20:536–43.

25. Sawai T, Nangaku M, Ashida A, Fujimaru R, Hataya H, Hidaka Y, et al. Diagnostic criteria for atypical hemolytic uremic syndrome proposed by the joint committee of the Japanese society of nephrology and the Japan pediatric society. Clin Exp Nephrol. 2014;18:4–9.

26. Cheong H Il, Jo SK, Yoon SS, Cho H, Kim JS, Kim YO, et al. Clinical practice guidelines for the management of atypical hemolytic uremic syndrome in Korea. J Korean Med Sci. 2016;31:1516–28.

27. Marie-Agnès Dragon-Durey, Sidharth Kumar Sethi, Arvind Bagga, Caroline Blanc, Jacques Blouin, Bruno Ranchin, Jean-Luc André, Nobuaki Takagi, Hae Il Cheong, Pankaj Hari, Moglie Le Quintrec, Patrick Niaudet, Chantal Loirat, Wolf Herman Fridman VF-B. Clinical features of anti-factor H autoantibody-associated hemolytic uremic syndrome. J Am Soc Nephrol. 2010;21:2180–7.

28. Józsi M, Licht C, Strobel S, Zipfel SLH, Richter H, Heinen S, et al. Factor H autoantibodies in atypical hemolytic uremic syndrome correlate with CFHR1/CFHR3 deficiency. Blood. 2008;111:1512–4.

29. Shereen Shawky, Hesham Safouh, Mona Gamal, Mohammed M. Abbas, Azza Aboul-Enein, Toshihiro Sawai, Yosra Fahmy  and HS. Anti‐Factor H Antibodies in Egyptian Children with Hemolytic Uremic. Int J Nephrol. 2021;6904858.

30. Johannes Hofer , Andreas R Janecke, L B Zimmerhackl, Magdalena Riedl, Alejandra Rosales, Thomas Giner, Gerard Cortina, Carola J Haindl, Barbara Petzelberger, Miriam Pawlik, VerenaJohannes Hofer 1, Andreas R Janecke, L B Zimmerhackl, Magdalena Riedl, Aleja RW. Complement factor H-related protein 1 deficiency and factor H antibodies in pediatric patients with atypical hemolytic uremic syndrome. Clin J Am Soc Nephrol. 2013;8:407–15.

31. Schaefer F, Ardissino G, Ariceta G, Fakhouri F, Scully M, Isbel N, et al. Clinical and genetic predictors of atypical hemolytic uremic syndrome phenotype and outcome. Kidney Int. 2018;94:408–18.

32. Khandelwal P, Bagga A. Managing anti-factor H antibody-associated hemolytic uremic syndrome: time for consensus. Pediatr Nephrol [Internet]. Springer Berlin Heidelberg; 2024;39:3137–41. Available from: https://doi.org/10.1007/s00467-024-06374-w

33. Fengxiao Bu , Tara Maga, Nicole C Meyer, Kai Wang, Christie P Thomas, Carla M Nester RJHS. Comprehensive genetic analysis of complement and coagulation genes in atypical hemolytic uremic syndrome. J Am Soc Nephrol. 2014;25:55–64.

34. Marina Noris , Jessica Caprioli , Elena Bresin , Chiara Mossali , Gaia Pianetti , Sara Gamba , Erica Daina , Chiara Fenili , Federica Castelletti , Annalisa Sorosina , Rossella Piras , Roberta Donadelli , Ramona Maranta, Irene van der Meer, Edward M Conwa GR. Relative Role of Genetic Complement Abnormalities in Sporadic and Familial aHUS and Their Impact on Clinical Phenotype. Clin J Am Soc Nephrol. 2010;5:1844–59.

35. Cataland SR, Wu HM. How I treat: The clinical differentiation and initial treatment of adult patients with atypical hemolytic uremic syndrome. Blood. 2014;123:2478–84.

36. Murphy D, Jha V, Banerjee D. Diabetes and CKD. Manag Kidney Dis. 2023;105:147–66.

37. Kurihara S, Yamaguchi A, Sonoda K, Yamada Y, Harada M, Hashimoto K, et al. Anti-C5 monoclonal antibody treatment showing pathological resolution of complement-mediated atypical hemolytic uremic syndrome: a case report. BMC Nephrol [Internet]. BioMed Central; 2024;25:1–10. Available from: https://doi.org/10.1186/s12882-024-03662-3

38. Sinha A, Gulati A, Saini S, Blanc C, Gupta A, Gurjar BS, et al. Prompt plasma exchanges and immunosuppressive treatment improves the outcomes of anti-factor H autoantibody-associated hemolytic uremic syndrome in children. Kidney Int. 2014;85:1151–60.

39. Boyer O, Balzamo E, Charbit M, Biebuyck-Gougé N, Salomon R, Dragon-Durey M-A, et al. Pulse Cyclophosphamide Therapy and Clinical Remission in Atypical Hemolytic Uremic Syndrome With Anti–Complement Factor H Autoantibodies. Am J Kidney Dis. 2010;55:923–7.

40. Kwon T, Dragon-Durey MA, Macher MA, Baudouin V, Maisin A, Peuchmaur M, et al. Successful pre-transplant management of a patient with anti-factor H autoantibodies-associated haemolytic uraemic syndrome. Nephrol Dial Transplant. 2008;23:2088–90.

41. Le Quintrec M, Zuber J, Noel LH, Thervet E, Frémeaux-Bacchi V, Fridman WH, et al. Anti-factor H autoantibodies in a fifth renal transplant recipient with atypical hemolytic and uremic syndrome. Am J Transplant. 2009;9:1223–9.

42. Nester CM, Thomas CP. Atypical hemolytic uremic syndrome: what is it, how is it diagnosed, and how is it treated? Hematology Am Soc Hematol Educ Program. 2012;2012:617–25.

43. 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.

44. Fadi Fakhouri, Marc Fila, François Provôt, Yahsou Delmas, Christelle Barbet, Valérie Châtelet, Cédric Rafat, Mathilde Cailliez, Julien Hogan, Aude Servais, Alexandre Karras, Raifah Makdassi, Fadi Fakhouri 1, Marc Fila, François Provôt, Yahsou Delmas, Chri VF-B. Pathogenic Variants in Complement Genes and Risk of Atypical Hemolytic Uremic Syndrome Relapse after Eculizumab Discontinuation. Clin J Am Soc Nephrol. 2017;12:50–9.

45. Maritati F, Corradetti V, Bini C, Provenzano M, Cuna V, Busutti M, et al. “Eculizumab First” in the Management of Posttransplant Thrombotic Microangiopathy. Kidney Int Reports [Internet]. Elsevier Inc; 2024;9:982–93. Available from: https://doi.org/10.1016/j.ekir.2024.01.013

46. Andrew M. Siedlecki, Nicole Isbel, Johan Vande Walle JJE, Cohen DJ. Eculizumab Use for Kidney Transplantation in Patients With a Diagnosis of Atypical Hemolytic Uremic Syndrome. Kidney Int Reports. 2019;4:434–46.

47. Aydan Mütiş Alan, Mevlüt Tamer Dinçer, Ata Kaykioğlu, Bahar Türk, Eda Nuhoğlu Kantarci, Nurhan Seyahi, Sinan Trabulus AEE. Perioperative Management of Atypical Hemolytic Uremic Syndrome in a Patient on Maintenance Eculizumab Therapy: A Case Report and Review of the Literature. Nephron. 2025;40414204.

48. Anuja Java. Peri- and Post-operative Evaluation and Management of Atypical Hemolytic Uremic Syndrome (aHUS) in Kidney Transplantation. Adv Chronic Kidney Dis. 2020;27:128–37.

49. Ha Tran, Abanti Chaudhuri WC& PCG. Use of eculizumab and plasma exchange in successful combined liver–kidney transplantation in a case of atypical HUS associated with complement factor H mutation. Pediatr Nephrol. 2014;29:477–80.