Hemolytic-Uremic Syndrome Questions & Answers

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2. Conway EM. HUS and the case for complement. Blood. 2015 Oct 29. 126 (18):2085-90. [Medline].

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5. Gasser C, Gautier E, Steck A, Siebenmann RE, Oechslin R. Hamolytisch uramische syndrome: Bilaterale Nierenridennekrosen bei akuten erworbenen hamolytischen Anamien. Schweiz Med Wochenschr. 1955. 85:905-909. [Full Text].

6. Joseph A, Cointe A, Mariani Kurkdjian P, Rafat C, Hertig A. Shiga Toxin-Associated Hemolytic Uremic Syndrome: A Narrative Review. Toxins (Basel). 2020 Jan 21. 12 (2):[Medline]. [Full Text].

7. Kaper JB, O'Brien AD. Overview and Historical Perspectives. Microbiol Spectr. 2014 Dec. 2 (6):[Medline]. [Full Text].

8. Furlan M, Robles R, Galbusera M, et al. von Willebrand factor-cleaving protease in thrombotic thrombocytopenic purpura and the hemolytic-uremic syndrome. N Engl J Med. 1998 Nov 26. 339(22):1578-84. [Medline]. [Full Text].

9. Tsai HM, Lian EC. Antibodies to von Willebrand factor-cleaving protease in acute thrombotic thrombocytopenic purpura. N Engl J Med. 1998 Nov 26. 339(22):1585-94. [Medline]. [Full Text].

10. George JN. ADAMTS13, thrombotic thrombocytopenic purpura, and hemolytic uremic syndrome. Curr Hematol Rep. 2005 May. 4(3):167-9. [Medline].

11. Haspel RL, Jarolím P. The "cutting" edge: von Willebrand factor-cleaving protease activity in thrombotic microangiopathies. Transfus Apher Sci. 2005 Apr. 32(2):177-84. [Medline].

12. Blackall DP, Marques MB. Hemolytic uremic syndrome revisited: Shiga toxin, factor H, and fibrin generation. Am J Clin Pathol. 2004 Jun. 121 suppl:S81-8. [Medline].

13. Tarr PI, Gordon CA, Chandler WL. Shiga-toxin-producing Escherichia coli and haemolytic uraemic syndrome. Lancet. 2005 Mar 19-25. 365(9464):1073-86. [Medline].

14. Frank C, Werber D, Cramer JP, et al. Epidemic profile of Shiga-toxin-producing Escherichia coli O104:H4 outbreak in Germany. N Engl J Med. 2011 Nov 10. 365(19):1771-80. [Medline].

15. Saad AF, Roman J, Wyble A, Pacheco LD. Pregnancy-Associated Atypical Hemolytic-Uremic Syndrome. AJP Rep. 2016 Mar. 6 (1):e125-8. [Medline]. [Full Text].

16. Meinel C, Spartà G, Dahse HM, Hörhold F, König R, Westermann M, et al. Streptococcus pneumoniae From Patients With Hemolytic Uremic Syndrome Binds Human Plasminogen via the Surface Protein PspC and Uses Plasmin to Damage Human Endothelial Cells. J Infect Dis. 2018 Jan 17. 217 (3):358-370. [Medline]. [Full Text].

17. Riesbeck K. Hemolytic Uremic Syndrome Associated With Pneumococci in Children-An Elusive Mystery Now Explained?. J Infect Dis. 2018 Jan 17. 217 (3):341-343. [Medline]. [Full Text].

18. Caprioli J, Bettinaglio P, Zipfel PF, et al. The molecular basis of familial hemolytic uremic syndrome: mutation analysis of factor H gene reveals a hot spot in short consensus repeat 20. J Am Soc Nephrol. 2001 Feb. 12(2):297-307. [Medline]. [Full Text].

19. Fremeaux-Bacchi V, Kemp EJ, Goodship JA, Dragon-Durey MA, Strain L, Loirat C, et al. The development of atypical haemolytic-uraemic syndrome is influenced by susceptibility factors in factor H and membrane cofactor protein: evidence from two independent cohorts. J Med Genet. 2005 Nov. 42(11):852-6. [Medline]. [Full Text].

20. Edey MM, Mead PA, Saunders RE, et al. Association of a factor H mutation with hemolytic uremic syndrome following a diarrheal illness. Am J Kidney Dis. 2008 Mar. 51(3):487-90. [Medline].

21. Lapeyraque AL, Wagner E, Phan V, et al. Efficacy of plasma therapy in atypical hemolytic uremic syndrome with complement factor H mutations. Pediatr Nephrol. 2008 Aug. 23(8):1363-6. [Medline].

22. Kojouri K, Vesely SK, George JN. Quinine-associated thrombotic thrombocytopenic purpura-hemolytic uremic syndrome: frequency, clinical features, and long-term outcomes. Ann Intern Med. 2001 Dec 18. 135(12):1047-51. [Medline].

23. Adams DA, Thomas KR, Jajosky RA, Foster L, Baroi G, Sharp P, et al. Summary of Notifiable Infectious Diseases and Conditions - United States, 2015. MMWR Morb Mortal Wkly Rep. 2017 Aug 11. 64 (53):1-143. [Medline]. [Full Text].

24. Schuppner R, Maehlmann J, Dirks M, Worthmann H, Tryc AB, Sandorski K, et al. Neurological Sequelae in Adults After E coli O104: H4 Infection-Induced Hemolytic-Uremic Syndrome. Medicine (Baltimore). 2016 Feb. 95 (6):e2337. [Medline].

25. Ardissino G, Tel F, Testa S, Paglialonga F, Longhi S, Martelli L, et al. A simple prognostic index for Shigatoxin-related hemolytic uremic syndrome at onset: data from the ItalKid-HUS network. Eur J Pediatr. 2018 Nov. 177 (11):1667-1674. [Medline].

26. Alconcher LF, Coccia PA, Suarez ADC, et al. Hyponatremia: a new predictor of mortality in patients with Shiga toxin-producing Escherichia coli hemolytic uremic syndrome. Pediatr Nephrol. 2018 Oct. 33 (10):1791-1798. [Medline].

27. Santos AH Jr, Casey MJ, Wen X, Zendejas I, Faldu C, Rehman S, et al. Outcome of kidney transplants for adults with hemolytic uremic syndrome in the U.S.: a ten-year database analysis. Ann Transplant. 2014 Jul 21. 19:353-61. [Medline].

28. Walsh PR, Johnson S, Brocklebank V, Salvatore J, Christian M, Kavanagh D. Glucose-6-Phosphate Dehydrogenase Deficiency Mimicking Atypical Hemolytic Uremic Syndrome. Am J Kidney Dis. 2018 Feb. 71 (2):287-290. [Medline].

29. Burns ER, Lou Y, Pathak A. Morphologic diagnosis of thrombotic thrombocytopenic purpura. Am J Hematol. 2004 Jan. 75(1):18-21. [Medline].

30. Karnisova L, Hradsky O, Blahova K, Fencl F, Dolezel Z, Zaoral T, et al. Complement activation is associated with more severe course of diarrhea-associated hemolytic uremic syndrome, a preliminary study. Eur J Pediatr. 2018 Dec. 177 (12):1837-1844. [Medline].

31. McGannon CM, Fuller CA, Weiss AA. Different classes of antibiotics differentially influence Shiga toxin production. Antimicrob Agents Chemother. 2010 Sept. 54(9):3790-3798.

32. Michael M, Elliott EJ, Ridley GF, Hodson EM, Craig JC. Interventions for haemolytic uraemic syndrome and thrombotic thrombocytopenic purpura. Cochrane Database Syst Rev. 2009 Jan 21. CD003595. [Medline].

33. FDA approves Soliris for rare pediatric blood disorder. U.S. Food and Drug Administration. Available at https://wayback.archive-it.org/7993/20170114063405/http://www.fda.gov/NewsEvents/Newsroom/PressAnnouncements/ucm272990.htm. September 23, 2011; Accessed: December 13, 2018.

34. 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 Jun 6. 368(23):2169-81. [Medline]. [Full Text].

35. Fakhouri F, Hourmant M, Campistol JM, Cataland SR, Espinosa M, Gaber AO, et al. Terminal Complement Inhibitor Eculizumab in Adult Patients With Atypical Hemolytic Uremic Syndrome: A Single-Arm, Open-Label Trial. Am J Kidney Dis. 2016 Mar 16. [Medline].

36. Greenbaum LA, Fila M, Ardissino G, Al-Akash SI, Evans J, Henning P, et al. Eculizumab is a safe and effective treatment in pediatric patients with atypical hemolytic uremic syndrome. Kidney Int. 2016 Mar. 89 (3):701-11. [Medline]. [Full Text].

37. Fakhouri F, Fila M, Hummel A, et al. Eculizumab discontinuation in children and adults with atypical hemolytic-uremic syndrome: a prospective multicenter study. Blood. 2021 May 6. 137 (18):2438-2449. [Medline].

38. Ultomiris (ravulizumab) [package insert]. Boston, MA: Alexion Pharmaceuticals, Inc. October 2019. Available at [Full Text].

39. Wong CS, Jelacic S, Habeeb RL, Watkins SL, Tarr PI. The risk of the hemolytic-uremic syndrome after antibiotic treatment of Escherichia coli O157:H7 infections. N Engl J Med. 2000 Jun 29. 342(26):1930-6. [Medline]. [Full Text].

40. Zhang K, Lu Y, Harley KT, Tran MH. Atypical Hemolytic Uremic Syndrome: A Brief Review. Hematol Rep. 2017 Jun 1. 9 (2):7053. [Medline]. [Full Text].

41. Sridharan M, Go RS, Abraham RS, Fervenza FC, Sethi S, Bryant SC, et al. Diagnostic Utility of Complement Serology for Atypical Hemolytic Uremic Syndrome. Mayo Clin Proc. 2018 Oct. 93 (10):1351-1362. [Medline].

Malvinder S Parmar, MBBS, MS, FRCPC, FACP, FASN Professor of Medicine, Northern Ontario School of Medicine; Assistant Professor, Department of Medicine, University of Ottawa Faculty of Medicine; Consulting Physician, Timmins and District Hospital, Ontario, Canada

Malvinder S Parmar, MBBS, MS, FRCPC, FACP, FASN is a member of the following medical societies: American College of Physicians, American Society of Nephrology, Ontario Medical Association, Royal College of Physicians and Surgeons of Canada

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Ronald A Sacher, MBBCh, FRCPC, DTM&H Professor of Internal Medicine and Pathology, Director, Hoxworth Blood Center, University of Cincinnati Academic Health Center

Ronald A Sacher, MBBCh, FRCPC, DTM&H is a member of the following medical societies: American Association for the Advancement of Science, American Association of Blood Banks, American Clinical and Climatological Association, American Society for Clinical Pathology, American Society of Hematology, College of American Pathologists, International Society of Blood Transfusion, International Society on Thrombosis and Haemostasis, Royal College of Physicians and Surgeons of Canada

Disclosure: Nothing to disclose.

Srikanth Nagalla, MD, MS, FACP Chief of Benign Hematology, Miami Cancer Institute, Baptist Health South Florida; Clinical Professor of Medicine, Florida International University, Herbert Wertheim College of Medicine

Srikanth Nagalla, MD, MS, FACP is a member of the following medical societies: American Society of Hematology, Association of Specialty Professors

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: Alexion; Alnylam; Kedrion; Sanofi; Dova<br/>Serve(d) as a speaker or a member of a speakers bureau for: Dova; Sanofi.

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