Клинические рекомендации по ведению взрослых пациентов с атипичным гемолитико-уремическим синдромом

Введение. Атипичный гемолитико-уремический синдром (аГУС) - системное заболевание из группы тромботических микроангиопатий с прогрессирующим течением и неблагоприятным прогнозом, в основе которого лежит неконтролируемая активация альтернативного пути комплемента наследственной или приобретённой природы, приводящая к генерализованному тромбообразованию в сосудах микроциркуляторного русла. Цель - предоставить актуальные клинические рекомендации по оказанию медицинской помощи взрослым пациентам с аГУС. Основные сведения. Экспертами Национальной ассоциации нефрологов разработаны актуальные клинические рекомендации по оказанию медицинской помощи взрослым пациентам с аГУС. В рекомендациях подробно рассмотрены вопросы этиологии, патогенеза, эпидемиологии и клинических проявлений заболевания. Особое внимание уделено диагностике, дифференциальной диагностике и лечению аГУС, основанным на принципах доказательной медицины.

атипичный гемолитико-уремический синдром, система комплемента, тромботическая микроангиопатия, молекулярно-генетическое исследование, хроническая болезнь почек, экулизумаб, atypical hemolytic-uremic syndrome, complement system, thrombotic microangiopathy, molecular genetic study, chronic kidney disease, eculizumab

1. Noris M., Remuzzi G. Atypical hemolytic-uremic syndrome. N Engl J Med. 2009; 361(17):1676-1687. doi: 10.1056/NEJMra0902814.

2. Campistol J.M., Arias M., Ariceta G. et al. An update for atypical haemolytic uraemic syndrome: diagnosis and treatment. A consensus document. Nephrologia. 2013; 33(1):27-45. doi: 10.1016/j.nefro.2015.07.005.

3. Gavriilaki E., Anagnostopoulos A., Mastellos D.C. Complement in thrombotic microangoipathies: unreveling Ariadne's thread into labyrinth of complement therapeutics. Front Immunol. 2019; 10:337. doi: 10.3389/fimmu.2019.00337.

4. Greenbaum L.A. Atypical hemolytic uremic syndrome. Adv Pediatr. 2014; 61(1):335-356. doi: 10.1016/j.yapd.2014.04.001.

5. Dragon-Durey M.A., Sethi S.K., Bagga A. et al. Clinical features of anti-factor H autoantibody-associated hemolytic uremic syndrome. J Am Soc Nephrol. 2010; 21(12):2180-2187. doi: 10.1681/ASN.2010030315.

6. Fremeaux-Bacchi V., Fakhouri F., Garnier A. et al. Genetics and outcome of atypical hemolytic uremic syndrome: a nationwide French series comparing children and adults. Clin J Am Soc Nephrol. 2013; 8(4):554-562. doi: 10.2215/CJN.04760512.

7. Yoshida Y., Kato H., IkedaY., Nangaku M. Pathogenesis of atypical haemolytic uraemic syndrome. J Atheroscler Thromb. 2019; 26(2):99-110. doi: 10.5551/jat.RV17026.

8. Rodriguez de Cordoba S., Hidalgo M.S., Pinto S., Tortajada A. Genetics of atypical hemolytic uremic syndrome (aHUS). Semin Thromb Hemost. 2014; 40:422-430. doi: 10.1055/s-0034-1375296.

9. Jozsi M., Licht C., Strobel S. et al. Factor F autoantibodies in atypical hemolytic uremic syndrome correlate with CFHR1/CFHR3 deficiency. Blood. 2008; 111(3):1512-1514. doi: 10.1182/blood-2007-09-109876.

10. Noris M., Mescia F., Remuzzi G. STEC-HUS, atypical HUS and TTP are all diseases of complement activation. Nat Rev Nephrol. 2012; 8(11):622-633. doi: 10.1038/nrneph.2012.195.

11. Meri S. Complement activation in diseases presenting with thrombotic microangiopathy. Eur J Intern Med 2013;24(6):496-502. doi: 10.1016/j.ejim.2013.05.009.

12. Riedl M., Fakhouri F., Le Quintrec M. et al. Spectrum of complement-mediated thrombotic microangiopathies: pathogenetic insights identifying novel treatment approaches. Semin Thromb Hemost. 2014; 40(4):444-464. doi: 10.1055/s-0034-1376153.

13. Fakhouri F., Zuber J., Fremeaux-Bacchi V., Loirat C. Hemolytic uremic syndrome. Lancet. 2017; 390(10095):681-696. doi: 10.1016/S0140-6736(17)30062-4.

14. Cataland S.R., Wu H.M. Diagnosis and management of complement mediated thrombotic microangiopathies. Blood Rev. 2014;28(2):67-74. doi: 10.1016/j.blre.2014.01.003.

15. Loirat C., Fremeaux-Bacchi V. Atypical hemolytic uremic syndrome. Orphanet J Rare Dis. 2011; 6:60. doi: 10.1186/1750-1172-6-60.

16. Yan K., Desai K., Gullapalli L. et al. Epidemiology of atypical hemolytic uremic syndrome: a systematic literature review. Clinical Epidemiology 2020; 12:295-305. doi: 10.2147/CLEP.S245642.

17. Besbas N., Karpman D., Landau D. et al. A classification of hemolytic uremic syndrome and thrombotic thrombocytopenic purpura, and related disorders. Europian Paediatric Research Group for HUS. Kidney International. 2006;70(3):423-431. doi: 10.1038/sj.ki.5001581.

18. Tsai H.M., Lian E.C. Antibodies to von Willebrand factor-cleaving protease in acute thrombotic thrombocytopenic purpura. N Engl J Med. 1998; 339(22):1585-1594. doi: 10.1056/NEJM199811263392203.

19. Moake J. Thrombotic thrombocytopenic purpura and other thrombotic microangiopathies. Best Practice&Research Clinical Haematology. 2009; 22(4):567-76. doi: 10.1016/j.beha.2009.07.004.

20. Goodship T.H., Cook H.T., Fakhouri F. et al. Atypical hemolytic uremic syndrome and C3 glomerulopathy: conclusion from a “Kidney Disease: improving Global Outcomes” (KDIGO) controversies conference. Kidney Int. 2017;91(3):539-551. doi: 10.1016/j.kint.2016.10.005.

21. Kato H., Nangaku M., Okada H., Kagami S. Controversies of the classification of TMA and the terminology of aHUS. Clinical and Experimental Nephrology. 2017;22(1):979-980. doi: 10.1007/ s10157-017-1524-4.

22. Le Clech A., Simon-Tillaux N., Provot F. et al. Atypical and secondary hemolytic uremic syndrome have a distinct presentation and no common genetic risk factors. Kidney Int. 2019;95(6):1443-1452. doi: 10.1016/j.kint2019.01.023.

23. Praga M., Rodriguez de Cordoba S. Secondary atypical hemolytic uremic syndrome in the era of complement blockade. Kidney Int. 2019;95(6):1298-1300. doi: 10.1016/j/kint2019.01.043.

24. Brocklebank V., Wood K.M., Kavanagh D. Thrombotic microangiopathy and the кidney. Clin J Am Soc Nephrol 2018;13(2):300-317. doi: 10.2215/CJN.00620117.

25. Aigner C., Schmidt A., Gaggl M., Sunder-Plassmann G. An updated classification of thrombotic microangiopathies and treatment of complement gene variant-mediated thrombotic microangiopathy. Clinical Kidney Journal. 2019;12(3):333-337. doi: 10.1093/ckj/sfz040.

26. Sallée M., Ismail K., Fakhouri F. et al. Thrombocytopenia is not mandatory to diagnose haemolytic and uremic syndrome. BMC Nephrol. 2013; 14:3. doi: 10.1186/1471-2369-14-3.

27. Hofer J., Rosales A., Fisher C., Giner T. Extra-renal manifestations of complement-mediated thrombotic microangiopathies. Front Pediatr. 2014;2:97. doi: 10.3389/fped.2014.00097.

28. Swisher K.K., Doan J.T., Vesely S.K. et al. Pancreatitis preceding acute episodes of thrombotic thrombocytopenic purpura-hemolytic uremic syndrome: report of five patients with a systematic review of published reports. Haematologica. 2007;92(7):936-43. doi: 10.3324/haematol.10963.

29. Noris M., Remuzzi G. Cardiovascular complications in atypical haemolytic uraemic syndrome. Nat Rev Nephrol. 2014;10(3):174-80. doi: 10.1038/nrneph.2013.280.

30. Sallee M., Daniel L., Piercecchi M. et al. Myocardial infarction is a complication of factor H associated atypical HUS. Nephrol Dial Transplant. 2010; 25(6):2028-32. doi: 10.1093/ndt/gfq160.

31. Jokiranta S. HUS and atypical HUS. Blood. 2017; 129(21):2847-2856. doi: 10.1182/blood-2016-11-709865.

32. Gulleroglu K., Fidan K., Hançer V.S. et al. Neurologic involvement in atypical hemolytic uremic syndrome and successful treatment with eculizumab. Pediatr Nephrol. 2013;28(5):827-30. doi: 10.1007/s00467-013-2416-9.

33. Кучиева А.М., Козловская Н.Л., Демьянова К.А., Добросмыслов И.А. Рецидивирующий атипичный гемолитико-уремический синдром, манифестировавший поражением поджелудочной железы. Нефрология и диализ. 2013; 15(3):236-239

34. Коротчаева Ю.В, Козловская Н.Л., Бондаренко Т.В., Веселов Г.А. Особенности течения и лечения «акушерского» гемолитико-уремического синдрома. Нефрология. 2015; 19(2):76-81

35. Loirat C., Fakhouri F., Ariceta G. et al. An international consensus approach to the management of the atypical hemolytic uremic syndrome in children. Pediatr Nephrol. 2016;31(1):15-39. doi: 10.1007/s00467-015-3076-8

36. Scully M., Goodship T. How I Treat thrombotic thrombocytopenic purpura and atypical hemolytic uremic syndrome. Br J Haematol. 2014;164(6):759-766. doi: 10.1111/bjh.12718

37. Azoulay E., Knoeble P., Garnacho-Montero J. et al. Expert Statements on the Standard of Care in Critically Ill Adult Patients with Atypical Hemolytic Uremic Syndrome. Chest. 2017;152(2):424-434. doi: 10.1016/j.chest.2017.03.055

38. Shen Y.M. Clinical evaluation of thrombotic microangiopathy: identification of patients with suspected atypical hemolytic uremic syndrome. Thrombosis Journal. 2016;14(suppl 1):19. doi: 10.1186/s12959-016-0114-0

39. Cheong H.I., Jo S.K., Yoon S.S. et al. Clinical practice guidelines for the management of atypical hemolytic uremic syndrome in Korea. J Korean Med Sci. 2016;31(10):1516-1528. doi: 10.3346/jkms.2016.31.10.1516

40. Taylor C.M., Machin S., Wigmore S.J., Goodship T.H.J. Clinical practice guidelines for the management of atypical haemolytic uraemic syndrome in the United Kingdom. BJH. 2009;148(1):37-47. doi: 10.1111/j.1365-2141.2009.07916.x.

41. Kato H., Nangaku M., Hataya H. et al. Clinical guides for atypical haemolytic uraemic syndrome in Japan. Clin Exp Nephrol. 2016;20(4):536-543. doi: 10/1007/s10157-016-1276-6

42. Go R.S., Winters J.L., Leug N. et al. Thrombotic microangiopathy care pathway: a consensus statement for the Mayo Clinic complement alternative pathway - thrombotic microangiopathy (CAP-TMA) disease-oriented group. Mayo Clin Proc. 2016;91(9):1189-1211. doi: 10.1016/j.mayocp.2016.05.015

43. Al-Nouri Z.L., Reese J.A., Terrell D.R. et al. Drug-induced thrombotic microangiopathy: a systematic review of published reports. Blood. 2015;125(4):616-618. doi: 10.1182/blood-2014-11-611335

44. Eremina V., Jefferson J.A., Kowalewska J. et al. VEGF inhibition and renal thrombotic microangiopathy. N Engl J Med. 2008;358(11):1129-1136. doi: 10.1056/NEJMoa0707330

45. Asif A., Nayer A., Haas C.S. Atypical hemolytic uremic syndrome in the setting of complement-amplifying conditions: case reports and a review of the evidence for treatment with eculizumab. J Nephrol. 2017;30(3):347-362. doi: 10.1007/s40620-016-0357-7

46. El-Husseini A., Hannan S., Awad A. et al. Thrombotic microangiopathy in systemic lupus erythematosus: efficacy of eculizumab. Am J Kidney Dis. 2015;65(1):127-130. doi: 10.1053/j.ajkd.2014.07.031.

47. Fakhouri F., Roumenina L., Provot F. et al. Pregnancy-associated hemolytic uremic syndrome revisited in the era of complement gene mutations. J Am Soc Nephrol. 2010;21(5):859-867. doi: 10.1681/ASN.2009070706

48. Zhang B., Xing C., Yu X. et al. Renal thrombotic microangiopathies induced by severe hypertension. Hypertens Res. 2008;31(3):479-483. doi: 10.1291/hypres.31.479

49. Timmermans S.A.M.E.G., Abdul-Hamid M.A., Vanderlocht J. et al. Patients with hypertension-associated thrombotic microangiopathy may present with complement abnormalities. Kidney Int. 2017;91(6):1420-1425. doi: 10.1016/j.kint.2016.12.009

50. Abbas F., El Kossi M., Kim J.J. et al. Thrombotic microangiopathy after renal transplantation: current insights in de novo and recurrent disease. World J Transplant. 2018;8(5):122-141. doi: 10.5500/wjt.v8.i5.122

51. Menne J., Nitschke M., Stingele R. et al. Validation of treatment strategies for enterohaemorrhagic Escherichia coli O104:H4 induced haemolytic uraemic syndrome:case-control study. BMJ. 2012;19;345:e4565. doi: 10.1136/bmj.e4565

52. Cataland S.R., Wu H.M. Atypical haemolytic uraemic syndrome and thrombotic thrombocytopenic purpura: clinically differentiating the thrombotic microangiopathies. Eur J Intern Med. 2013;24(6):486-491. doi: 10.1016/j.ejim.2013.05.007

53. Scully M., Hunt B.J., Benjamin S. et al. Guidelines on the diagnosis and management of thrombotic thrombocytopenic purpura and other thrombotic microangiopathies. BJH. 2012;158(3):323-335. doi: 10.1111/j.1365-2141.2012.09167.x

54. Cataland S.R., Yang S., Wu H.M. The use of ADAMTS13 activity, platelet count, and serum creatinine to differentiate acquired thrombotic thrombocytopenic purpura from other thrombotic microangiopathies. Br J Haematol. 2012;157(4):501-503. doi: 10.1111/j.1365-2141.2012.09032.x.

55. Coppo P., Schwarzinger M., Buffet M. et al. Predictive features of severe acquired ADAMTS13 deficiency in idiopathic thrombotic microangiopathies: the French TMA reference center experience. PLoS One. 2010;5(4):e10208. doi: 10.1371/journal.pone.0010208.

56. Hassan S., Westwood J.P., Ellis D. et al. The utility of ADAMTS13 in differentiating TTP from other acute thrombotic microangiopathies: results from the UK TTP Registry. Br J Haematol. 2015;171(5):830-5. doi: 10.1111/bjh.13654

57. Phillips E.H., Westwood J.P., Brocklebank V. et al. The role of ADAMTS-13 activity and complement mutational analysis in differentiating acute thrombotic microangiopathies. J Thromb Haemost. 2016;14(1):175-85. doi: 10.1111/jth.13189

58. Wada H., Matsumoto T., Suzuki K. et al. Differences and similarities between disseminated intravascular coagulation and thrombotic microangiopathy. Thromb J. 2018;16:14. doi: 10.1186/s12959-018-0168-2

59. Lee H., Kang E., Kang H.G. et al. Consensus regarding diagnosis and management of atypical hemolytic uremic syndrome. Korean J Intern Med. 2020;35(1):25-40. doi: 10.3904/kjim.2019.388

60. Lupu F., Keshari R.S., Lambris J.D., Coggeshall K.M. Crosstalk between the coagulation and complement systems in sepsis. Thromb Res. 2014;133(suppl 1):S28-S31. doi: 10.1016/j.thromres.2014.03.014

61. Noris M., Caprioli J., Bresin E. et al. Relative role of genetic complement abnormalities in sporadic and familial aHUS and their impact on clinical phenotype. Clin J Am Soc Nephrol. 2010;5(10):1844-1859. doi: 10.2215/CJN.02210310

62. Fremeaux-Bacchi V., Fakhouri F., Garnier A. et al. Genetics and outcome of atypical hemolytic uremic syndrome: a nationwide French series comparing children and adults. Clin J Am Soc Nephrol. 2013;8(4):554-62. doi: 10.2215/CJN.04760512

63. Kavanagh D., Goodship T.H., Richards A. Atypical hemolytic uremic syndrome. Semin Nephrol. 2013;33(6):508-530. doi: 10.1016/j.semnephrol.2013.08.003

64. Cataland S.R., Holers V.M., Geyer S. et al. Biomarkers of the alternative pathway and terminal complement activity at presentation confirms the clinical diagnosis of aHUS and differentiates aHUS from TTP. Blood. 2014;123(24):3733-3738. doi: 10.1182/blood-2013-12-547067

65. Noris M., Galbusera M., Gastoldi S. et al. Dynamics of complement activation in aHUS and how to monitor eculizumab therapy. Blood. 2014;124(11):1715-1726. doi: 10.1182/blood-2014-02-558296

66. Schaefer F., Ardissino G., Ariceta G. et al. Clinical and genetic predictors of atypical hemolytic uremic syndrome phenotype and outcome. Kidney Int. 2018;94(2):408-418. doi: 10.1016/j.kint.2018.02.029

67. Jiang H., Fan M.N., Yang M. et al. Association among Complement Factor H Autoantibodies, Deletions of CFHR, and the Risk of Atypical Hemolytic Uremic Syndrome. Int J Environ Res Public Health. 2016;13(12):1209. doi: 10.3390/ijerph13121209

68. Kellum J.A., Lameire N., Aspelin P. et al. KDIGO Clinical practice guideline for acute kidney injury. Kidney Int Suppl. 2012;2(1):1-138. doi:10.1038/kisup.2012.1

69. Campistol J.M., Arias M., Ariceta G. et al. An update for atypical haemolytic uraemic syndrome: diagnosis and treatment. A consensus document. Nephrologia. 2015;35(5):421-447. doi: 10.1016/j.nefro.2015.07.005

70. De Serres S.A., Isenring P. Athrombocytopenic thrombotic microangiopathy, a condition that could be overlooked based on current diagnostic criteria. Nephrol Dial Transplant. 2009;24(3):1048-1050. doi: 10.1093/ndt/gfn687

71. Zuber J., Le Quintrec M., Sberro-Soussan R. et al. New insights into postrenal transplant hemolytic uremic syndrome. Nat Rev Nephrol. 2011;7(1):23-35. doi: 10.1038/nrneph.2010.155

72. Caprioli J., Noris M., Brioschi S. et al. International Registry of Recurrent and Familial HUS/TTP. Genetics of HUS: the impact of MCP, CFH, and IF mutations on clinical presentation, response to treatment, and outcome. Blood. 2006;108(4):1267-1279. doi: 10.1182/blood-2005-10-007252

73. Clark W.F. Thrombotic microangiopathy: current knowledge and outcomes with plasma exchange. Semin Dial. 2012;25(2):214-219. doi: 10.1111/j.1525-139X.2011.01035.x

74. Fox L.C., Cohney S.J., Kausman J.Y. et al. Consensus opinion on diagnosis and management of thrombotic microangiopathy in Australia and New Zealand. Nephrology (Carlton). 2018;23(6):507-517. doi: 10.1111/nep.13234

75. Michael M., Elliott E.J., Craig J.C. et al. Interventions for hemolytic uremic syndrome and thrombotic thrombocytopenic purpura: a systematic review of randomized controlled trials. Am J Kidney Dis. 2009;53(2):259-72. doi: 10.1053/j.ajkd.2008.07.038

76. von Baeyer H. Plasmapheresis in thrombotic microangiopathy-associated syndromes: review of outcome data derived from clinical trials and open studies. Ther Apher. 2002;6(4):320-8. doi: 10.1046/j.1526-0968.2002.00390.x

77. Coppo P., Bussel A., Charrier S. et al. High-dose plasma infusion versus plasma exchange as early treatment of thrombotic thrombocytopenic purpura/hemolytic-uremic syndrome. Medicine (Baltimore). 2003;82(1):27-38. doi: 10.1097/00005792-200301000-00003

78. Loirat C., Grnier A., Sellier-Leclerc A.L., Kwon T. Plasmatherapy in atypical hemolytic uremic syndrome. Semin Thromb Hemost. 2010;36(6):673-681. doi: 10.1055/s-0030-1262890

79. Clark W.F., Huang S.S., Walsh M.W. et al. Plasmapheresis for the treatment of kidney diseases. Kidney Int. 2016;90(5):974-984. doi: 10.1016/j.kint.2016.06.009

80. Schwartz J., Padmanabhan A., Aqui N. et al. Guidelines on the use of therapeutic apheresis in clinical practice-evidence-based approach from the Writing Committee of the American Society for Apheresis: the seventh special issue. J Clin Apher. 2016;31(3):149-162. doi: 10.1002/jca.21470

81. Claes K.J., Massart A., Collard L. et al. Belgian consensus statement on the diagnosis and management of patients with atypical hemolytic uremic syndrome. Acta Clin Belg. 2018;73(1):80-89. doi: 10.1080/17843286.2017.1345185

82. Cohen J.A., Brecher M.E., Bandarenko N. Cellular source of serum lactate dehydrogenase elevation in patients with thrombotic thrombocytopenic purpura. J Clin Apher. 1998;13(1):16-19. doi: 10.1002/(sici)1098-1101(1998)13:1<16::aid-jca3>3.0.co;2-c

83. Zuber J., Fakhouri F., Roumenina L.T. et al.; French Study Group for aHUS/C3G. Use of eculizumab for atypical haemolytic uraemic syndrome and C3 glomerulopathies. Nat Rev Nephrol. 2012;8(11):643-657. doi: 10.1038/nrneph.2012.214

84. Krishnappa V., Gupta M., Elrifai M. et al. Atypical Hemolytic Uremic Syndrome: A Meta-Analysis of Case Reports Confirms the Prevalence of Genetic Mutations and the Shift of Treatment Regimens. Ther Apher Dial. 2018;22(2):178-188. doi: 10.1111/1744-9987.12641

85. Kise, T., Fukuyama S., Uehara M. Successful Treatment of Anti-Factor H Antibody-Associated Atypical Hemolytic Uremic Syndrome. Indian J Nephrol. 2020;30(1):35-38. doi: 10.4103/ijn.IJN_336_18

86. Sana G., Dragon-Durey M.A., Charbit M. et al. Long-term remission of atypical HUS with anti-factor H antibodies after cyclophosphamide pulses. Pediatr Nephrol. 2014;29(1):75-83. doi: 10.1007/s00467-013-2558-9

87. Cofiell R., Kukreja A., Bedard K. et al. Eculizumab reduces complement activation, inflammation, endothelial damage, thrombosis, and renal injury markers in aHUS. Вlood. 2015;125(21):3253-62. doi: 10.1182/blood-2014-09-600411

88. Legendre C.M., Licht C., Muus.PL. et al. Terminal complement inhibitor Eculizumab in atypical hemolytic-uremic syndrome. N Engl J Med. 2013;368(23):2169-2181. doi: 10.1056/NEJMoa1208981

89. Licht C., Greenbaum L.A., Muus P. et al. Efficacy and safety of Eculizumab in atypical hemolytic uremic syndrome from 2-year extensions of phase 2 studies. Kidney Int. 2015;87(5):1061-1073. doi: 10.1038/ki.2014.423

90. Pugh D., O'Sullivan E.D., Duthie F.A. et al. Interventions for atypical haemolytic uraemic syndrome. Cochrane Database Syst Rev. 2021;3(3):CD012862. doi: 10.1002/14651858.CD012862.pub2

91. Bernuy-Guevara C., Chehade H., Muller Y.D. et al. The Inhibition of Complement System in Formal and Emerging Indications: Results from Parallel One-Stage Pairwise and Network Meta-Analyses of Clinical Trials and Real-Life Data Studies. Biomedicines. 2020;8(9):355. doi: 10.3390/biomedicines8090355

92. Menne J., Delmas Y., Fakhouri F. et al. Eculizumab prevents thrombotic microangiopathy in patients with atypical hemolytic uremic syndrome in a long-term observational study. Clin Kidney J. 2018;12(2):196-205. doi: 10.1093/ckj/sfy035

93. Menne J., Delmas Y., Fakhouri F. et al. Outcome in patients with atypical hemolytic uremic syndrome treated with Eculizumab in a long-term observational study. BMC Nephrology. 2019;20(1):125-137. doi: 10.1186/s12882-019-1314-1

94. Walle J.V., Delmas Y., Ardissino G. et al. Improved renal recovery in patients with atypical hemolytic uremic syndrome following rapid initiation of eculizumab treatment. J Nephrol. 2017;30(1):127-134. doi: 10.1007/s40620-016-0288-3

95. Ardissino G., Possenti I., Tel F. et al. Discontinuation of Eculizumab treatment in atypical hemolytic uremic syndrome: an update. Am J Kidney Dis. 2015;66(1):172-173. doi: 10.1053/j.ajkd.2015.04.010

96. Olson S.R., Lu E., Sulpizio E. et al. When to stop Eculizumab in complement-mediated microangiopathies. Am J Nephrol. 2018;48(2):96-107. doi: 10.1159/000492033

97. Хроническая болезнь почек (ХБП). Клинические рекомендации. https://rusnephrology.org/wp-content/uploads/2020/12/CKD_final.pdf

98. Каабак М.М., Молчанова Е.А., Нестеренко И.В. и др. Резолюция Междисциплинарного совета экспертов. Трансплантация почки у пациентов с атипичным гемолитико-уремическим синдромом: клинические и организационно-методические аспекты ведения пациентов. Нефрология. 2018;3:8-14. doi: 10.18565/nephrology.2018.3.8-14

99. Gonzalez Suarez M.L., Thongprayoon C., Mao M.A. et al. Outcomes of Kidney Transplant Patients with Atypical Hemolytic Uremic Syndrome Treated with Eculizumab: A Systematic Review and Meta-Analysis. J Clin Med. 2019;8(7):pii:E919. doi: 10.3390/jcm8070919

100. Siedlecki A.M., Isbel N., Vande Walle J. et al.; Global aHUS Registry. Eculizumab use for kidney transplantation patients with a diagnosis of atypical hemolytic uremic syndrome. Kidney Int Rep. 2018;4(3):434-446. doi: 10.1016/j.ekir.2018.11.010

101. Keating G.M. Eculizumab: a review of its use in atypical haemolytic uraemic syndrome. Drugs. 2013;73(18):2053-66. doi: 10.1007/s40265-013-0147-7

102. Avery R.K., Ljungman P. Prophylactic measures in the solid-organ recipient before transplantation. Clin Infect Dis. 2001;33(1):15-21. doi: 10.1086/320899

103. Danzinger-Isakov L., Kumar D. AST Infectious Diseases Community of Practice Guidelines for vaccination of solid organ transplant candidates and recipients. Am J Transplant. 2009;9(4):258-262. doi: 10.1111/j.1600-6143.2009.02917.x

104. Резолюция Междисциплинарного совета экспертов по профилактике тяжелых инфекций у пациентов с генетическими нарушениями регуляции системы комплемента, получающих терапию экулизумабом. Эпидемиология и Вакцинопрофилактика. 2017;16(1):51-54.

105. Bresin E., Daina E., Noris M. et al. Outcome of renal transplantation in patients with non-Shiga toxin-associated hemolytic uremic syndrome: prognostic significance of genetic background. Clin J Am Soc Nephrol. 2006;1(1):88-99. doi: 10.2215/CJN.00050505

106. Keller K., Daniel C., Schöcklmann H. et al. Everolimus inhibits glomerular endothelial cell proliferation and VEGF, but not long-term recovery in experimental thrombotic microangiopathy. Nephrol Dial Transplant. 2006;21(10):2724-2735. doi: 10.1093/ndt/gfl340

107. Nava F., Cappelli G., Mori G. et al. Everolimus, cyclosporine, and thrombotic microangiopathy: clinical role and preventive tools in renal transplantation. Transplant Proc. 2014;46(7):2263-2268. doi: 10.1016/j.transproceed.2014.07.062

108. Schwimmer J., Nadasdy T.A., Spitalnik P.F. et al. De novo thrombotic microangiopathy in renal transplant recipients: a comparison of hemolytic uremic syndrome with localized renal thrombotic microangiopathy. Am J Kidney Dis. 2003;41(2):471-479. doi: 10.1053/ajkd.2003.50058

109. Rolla D., Fontana I., Ravetti J.L. et al. De novo post-transplant thrombotic microangiopathy localized only to the graft in autosomal dominant polycystic kidney disease with thrombophilia. J Renal Inj Prev. 2015;4(4):135-138. doi: 10.12861/jrip.2015.28

110. Zuber J., Le Quintrec M., Krid S. et al.; French Study Group for Atypical HUS. Eculizumab for atypical hemolytic uremic syndrome recurrence in renal transplantation. Am J Transplant. 2012;12(12):3337-3354. doi: 10.1111/j.1600-6143.2012.04252.x

111. Matar D., Naqvi F., Racusen L.C. et al. Atypical hemolytic uremic syndrome recurrence after kidney transplantation. Transplantation. 2014;98(11):1205-1212. doi: 10.1097/TP.0000000000000200

112. Cugno M., Gualtierotti R., Possenti I. et al. Complement functional tests for monitoring eculizumab treatment in patients with atypical hemolytic uremic syndrome. J Thromb Haemost. 2014;12(9):1440-1448. doi: 10.1111/jth.12615

113. Nishimura J., Yamamoto M., Hayashi S. et al. Genetic variants in C5 and poor response to eculizumab. N Engl J Med. 2014;370(7):632-639. doi: 10.1056/NEJMoa1311084

114. Bruel A., Kavanagh D., Noris M. et al. Hemolytic uremic syndrome in pregnancy and postpartum. Clin J Am Soc Nephrol. 2017;12(8):1237-1247. doi: 10.2215/CJN.00280117

115. Gupta M., Burwick R.M. Pregnancy-associated atypical hemolytic uremic syndrome. A systematic review. Obstetrics&Gynecology. 2020;135(1):46-58. doi: 10.1097/AOG0000000000003554

116. Grand'Maison S., Lapinsky S. Insights into pregnancy associated and atypical hemolytic uremic syndrome [published correction appears in Obstet Med. 2018;11(4):198]. Obstet Med. 2018;11(3):137-140. doi: 10.1177/1753495X18780099

117. Коротчаева Ю.В., Козловская Н.Л., Демьянова К.А. и др. Генетические аспекты акушерского гемолитико-уремического синдрома. Клиническая нефрология. 2017;1:12-17

118. Huerta A., Arjona E., Portoles J. et al. A retrospective study of pregnancy-associated atypical hemolytic uremic syndrome. Kidney Int. 2018;93(2):450-459. doi: 10.1016/j.kint.2017.06.022

119. Козловская Н.Л., Коротчаева Ю.В., Шифман Е.М., Кудлай Д.А. Акушерский атипичный гемолитико-уремический синдром: виновата беременность или ее осложнения? Вопросы гинекологии, акушерства и перинатологии. 2020;19(4):81-91. doi: 10.20953/1726-1678-2020-4-81-91

120. Кирсанова Т.В., Виноградова М.А., Федорова Т.А. Имитаторы тяжелой преэклампсии и HELLP-синдрома: различные виды тромботической микроангиопатии, ассоциированной с беременностью. Акушерство и гинекология. 2016;12:5-14. doi: 10.18565/aig.2016.12.5-14

121. Padmanabhan A., Connelly-Smith.L, Aqui N. et al. Guidelines on the Use of Therapeutic Apheresis in Clinical Practice - Evidence-Based Approach from the Writing Committee of the American Society for Apheresis: The Eighth Special Issue. J Clin Apher. 2019;34(3):171-354. doi: 10.1002/jca.21705

122. Stefanovic V. The Extended Use of Eculizumab in pregnancy and complement activation-associated diseases affecting maternal, fetal and neonatal kidneys - the future is now? J Clin Med. 2019;8(3):407. doi: 10.3390/jcm8030407

123. Burlinson C.E.G., Sirounis D., Wally K.R., Chau A. Sepsis in pregnancy and the puerperium. Int J Obstet Anesth. 2018;36:96-107. doi: 10.1016/j.ijoa.2018.04.010

124. Nabhan A.F., Allam N.E., Hamed Abdel-Aziz Salama M. Routes of administration of antibiotic prophylaxis for preventing infection after caesarean section. Cochrane Database Syst Rev. 2016;2016(6):CD011876. doi: 10.1002/14651858.CD011876.pub2