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Guidelines for the management and investigation of hemolytic uremic syndrome

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References

1 Diagnosis and treatment of Shiga toxin producing Escherichia coli infection

  1. Diagnosis of Shiga toxin producing Escherichia coli infection

  2. Saito T, et al. Reported cases of hemorrhagic uremic syndrome associated with EHEC infection in 2008-NESID. IASR 2009;30:122–123. [in Japanese] (level 5)

  3. Komiya N, et al. Reported cases of hemorrhagic uremic syndrome associated with EHEC infection in 2008-NESID. IASR. 2010;31:170–172. [in Japanese] (level 5)

  4. Saito T, et al. Reported cases of hemorrhagic uremic syndrome associated with EHEC infection in 2010-NESID. IASR 2011;32:141–143. [in Japanese] (level 5)

  5. Saito T, et al. Reported cases of hemorrhagic uremic syndrome associated with EHEC infection in 2011-NESID. IASR. 2012;33:128–130. [in Japanese] (level 5)

  6. Kamioka I, et al. Risk factors for developing severe clinical course in HUS patients: a national survey in Japan. Pediatr Int. 2008;50:441–446. (level 4)

  7. Treatment of STEC infection

  8. Safdar N, et al. Risk of hemolytic uremic syndrome after antibiotic treatment of Escherichia coli O157:H7 enteritis: a meta-analysis. JAMA. 2002;288:996–1001. (level 2)

  9. Proulx F, et al. Randomized, controlled trial of antibiotic therapy for Escherichia coli O157:H7 enteritis. J Pediatr. 1992;121:299–303. (level 2)

  10. Menne J, et al. EHEC-HUS consortium: Validation of treatment strategies for enterohaemorrhagic Escherichia coli O104:H4 induced haemolytic uraemic syndrome: case–control study. BMJ. 2012;345:e4565. (level 4)

  11. Smith KE, et al. Antibiotic treatment of Escherichia coli O157 infection and the risk of hemolytic uremic syndrome, Minnesota. Pediatr Infect Dis J. 2012;31:37–41. (level 4)

  12. Wong CS, et al. The risk of the hemolytic-uremic syndrome after antibiotic treatment of Escherichia coli O157:H7 infections. N Engl J Med. 2000;342:1930–1936. (level 4)

  13. Wong CS, et al. Risk factors for the hemolytic uremic syndrome in children infected with Escherichia coli O157:H7: a multivariable analysis. Clin Infect Dis. 2012;55:33–41. (level 4)

  14. Dundas S, et al. The central Scotland Escherichia coli O157:H7 outbreak: risk factors for the hemolytic uremic syndrome and death among hospitalized patients. Clin Infect Dis. 2001;33:923–931. (level 4)

  15. Shiomi M, et al. Effect of early oral fluoroquinolones in hemorrhagic colitis due to Escherichia coli O157:H7. Pediatr Int. 1999;41:228–232. (level 4)

  16. Ikeda K, et al. Effect of early fosfomycin treatment on prevention of hemolytic uremic syndrome accompanying Escherichia coli O157:H7 infection. Clin Nephrol. 1999;52:357–362. (level 4)

  17. Bell BP, et al. Predictors of hemolytic uremic syndrome in children during a large outbreak of Escherichia coli O157:H7 infections. Pediatrics. 1997;100:E12. (level 4)

  18. Cimolai N, et al. A continuing assessment of risk factors for the development of Escherichia coli O157:H7-associated hemolytic uremic syndrome. Clin Nephrol. 1994;42:85–89. (level 4)

  19. Cimolai N, et al. Risk factors for the central nervous system manifestations of gastroenteritis-associated hemolytic-uremic syndrome. Pediatrics. 1992;90:616–621. (level 4)

2 Diagnosis of HUS

  1. Diagnosis procedure

  2. Kawamura N, et al. Risk factors for the development of Escherichia coli O157: H7 associated with hemolytic uremic syndrome. Pediatr Int. 1999;41:218–222. (level 4)

  3. Kamioka I, et al. Japanese Society for Pediatric Nephrology: Risk factors for developing severe clinical course in HUS patients: a national survey in Japan. Pediatr Int. 2008;50:441–446. (level 4)

  4. Oakes RS, et al. Predictors of fatality in postdiarrheal hemolytic uremic syndrome. Pediatrics. 2006;117:1656–1662. (level 4)

  5. Assessment of acute kidney injury (AKI)

  6. Kamioka I, et al. Japanese Society for Pediatric Nephrology. Risk factors for developing severe clinical course in HUS patients: a national survey in Japan. Pediatr Int. 2008;50:441–446. (level 4)

  7. Gerber A, et al. Clinical course and the role of shiga toxin-producing Escherichia coli infection in the hemolytic-uremic syndrome in pediatric patients, 1997–2000, in Germany and Austria: a prospective study. J Infect Dis. 2002;15:493–500. (level 4)

  8. Balestracci A, et al. Dehydration at admission increased the need for dialysis in hemolytic uremic syndrome children. Pediatr Nephrol. 2012;27:1407–1410. (level 4)

  9. Hickey CA, et al. Early volume expansion during diarrhea and relative nephroprotection during subsequent hemolytic uremic syndrome. Arch Pediatr Adolesc Med. 2011;165:884–889. (level 4)

  10. Diagnosis of encephalopathy

  11. Gasser C, et al. Haemolytisch-uraemische syndrome: Bilaterale nierenrindennekrosen bei akuten erworbenen haemolytischen anaemien. Schweiz Med Wochenschr. 1955;85:905–909. (level 5)

  12. Akashi S, et al. An outbreak of Escherichia coli associated colitis in a kindergarten. Committee for epidemiological study of epidemic diarrhea due to pathogenic E. coli in a kindergarten, Saitama, Japan. J Jpn Pediatr Soc. 1991;95:2607–2615. [in Japanese] (level 5)

  13. Siegler RL. Spectrum of extrarenal involvement in postdiarrheal hemolytic-uremic syndrome. J Pediatr. 1994;125:511–518. (level 5)

  14. Furuse A. Clinical analysis of the cases complicated with central nervous system involvement in diarrhea associated hemolytic uremic syndrome. J Jpn Pediatr Soc. 2006;110:919–925. [in Japanese] (level 5)

  15. Sheth KJ, et al. Neurologic involvement in hemolytic-uremic syndrome. Ann Neurol. 1986;19:90–93. (level 5)

  16. Bale CP, et al. CNS manifestations of the hemolytic-uremic syndrome. Am J Dis Child. 1980;134:869–872. (level 5)

  17. Theobald I, et al. Central nervous system involvement in hemolytic uremic syndrome (HUS)–a retrospective analysis of cerebral CT and MRI studies. Clin Nephrol. 2001;56:S3–8. (level 5)

  18. Steinborn M, et al. CT and MRI in haemolytic uraemic syndrome with central nervous system involvement: distribution of lesions and prognostic value of imaging findings. Pediatr Radiol. 2004;34:805–810. (level 5)

  19. Donnerstag F, et al. Patterns in early diffusion-weighted MRI in children with haemolytic uraemic syndrome and CNS involvement. Eur Radiol. 2012;22:506–513. (level 5).

  20. Dhuna A, et al. EEG and seizures in children with hemolytic-uremic syndrome. Epilepsia. 1992;33:482–486. (level 5)

  21. Shiraishi M, et al. Soluble tumor necrosis factor receptor 1 and tissue inhibitor of metalloproteinase-1 in hemolytic uremic syndrome with encephalopathy. J Neuroimmunol. 2008;196:147–152. (level 4)

  22. Shimizu M, et al. Cytokine profiles of patients with enterohemorrhagic Escherichia coli O111-induced hemolytic-uremic syndrome. Cytokine. 2012;60:694–700. (level 4)

  23. Crisp DE, et al. Hemorrhagic cerebral infarction in the hemolytic-uremic syndrome. J Pediatr. 1981;99:273–276. (level 5)

  24. DiMario FJ, et al. Lacunar infarction of the basal ganglia as a complication of hemolytic-uremic syndrome. Clin Pediatr. 1987;26:586–590. (level 5)

  25. Acute-phase extrarenal complication (excluding encephalopathy)

  26. Spinale JM, et al. Long-term outcomes of Shiga toxin hemolytic uremic syndrome. Pediatr Nephrol 2013 Jan 4. [Epub ahead of print]. (level 4)

  27. Habib R, et al. Hemolytic-uremic syndrome in children and arterial hypertension. Arch Mal Coeur Vaiss. 1981;74:37–43. (level 5)

  28. Siegler RL, et al. Hemolytic-uremic syndrome in adolescents. Arch Pediatr Adolesc Med. 1997;151:165–169. (level 4)

  29. Friedland JA, et al. Escherichia coli O157: H7-associated hemolytic-uremic syndrome: Value of colonic color Doppler sonography. Pediatr Radiol. 1995;25:S65–S67. (level 5)

  30. Bernard A, et al. Digestive manifestations in hemolytic uremic syndrome in children. Arch Pediatr. 1996;3:533–540. (level 4)

  31. Yoden A. Echographic significance in gastrointestinal disease. Jp J Pediatric Medicine. 1999;31:1700–1707. [in Japanese]. (level 5)

  32. Crabbe DCG, et al. Gastrointestinal complications of the haemolytic uraemic syndrome. J Roy Soc Med. 1990;83:773–775. (level 5)

  33. de Buys Roessingh AS, et al. Gastrointestinal complications of post-diarrheal hemolytic uremic syndrome. Eur J Pediatr Surg. 2007;17:328–334. (level 4)

  34. Brandt JR, et al. Cholelithiasis following Escherichia coli O157: H7-assosiated hemolytic uremic syndrome. Pediatr Nephrol. 1998;12:222–225. (level 4)

  35. Nagita A, et al. Report on nine cases of gallbladder calculus disease. J Jpn Pediatric Society. 1993;97:2140–2144. [in Japanese] (level 5)

  36. Yamazaki T, et al. Case Report of Two-year-old Boy with Bile-duct Stones Associated with Hemolytic Uremic Syndrome. J Jpn Pediatric Society. 1996;103:865–868. [in Japanese] (level 5)

  37. Spizirri FD, et al. Childhood hemolytic uremic syndrome in Argentina: Long-term follow-up and prognostic features. Pediatr Nephrol. 1997;11:156–160. (level 4)

  38. Poulton J, et al. Dilated cardiomyopathy associated with haemolytic uraemic syndrome. Br Heart J. 1987;57:181–183. (level 5)

  39. Mohammed J, et al. Cardiac tamponade in diarrhea-positive haemolytic uraemic syndrome. Nephrol Dial Transplant. 2009;24:679–681. (level 5)

  40. Askiti V, et al. Troponin I levels in a hemolytic uremic syndrome patient with severe cardiac failure. Pediatr Nephrol. 2004;19:345–348. (level 5)

  41. Abu-Arafeh I, et al. Myocarditis and haemolytic uraemic syndrome. Arch Dis Child. 1995;72:46–47. (level 5)

3 Treatment of HUS

  1. Fluid therapy and blood transfusion

  2. Ake JA, et al. Relative nephroprotection during Escherichia coli O157:H7 infections: Association with intravenous volume expansion. Pediatrics. 2005;115:e673–80. (level 4)

  3. Hickey CA, et al. Early volume expansion during diarrhea and relative nephroprotection during subsequent hemolytic uremic syndrome. JAMA 2012;165:884–889. (level 4)

  4. Balestracci A, et al. Dehydration at admission increased the need for dialysis in hemolytic uremic syndrome children. Pediatr Nephrol. 2012;27:1407–1410. (level 4)

  5. Pape L, et al. Early erythropoietin reduced the need for red blood cell transfusion in childhood hemolytic uremic syndrome: a randomized prospective pilot trial. Pediatr Nephrol. 2009;24:1061–1064. (level 2)

  6. Weil BR, et al. Bleeding risk for surgical dialysis procedures in children with hemolytic uremic syndrome. Pediatr Nephrol. 2010;25:1693–1698. (level 4)

  7. Antihypertensive therapy

  8. Spinale JM, et al. Long-term outcomes of Shiga toxin hemolytic uremic syndrome. Pediatr Nephrol. 2013 Jan 4. [Epub ahead of print]. (level 4)

  9. Renal replacement therapy

  10. Bagshaw SM, et al. Dialysis Disequilibrium Syndrome: brain death following hemodialysis for metabolic acidosis and acute renal failure–a case report. BMC Nephrol. 2004;9:5–9. (level 5)

  11. Plasma exchange therapy

  12. Dundas S, et al. Effectiveness of therapeutic plasma exchange in the 1996 Lanarkshire Escherichia coli O157:H7 outbreak. Lancet. 1999;354:1327–1330. (level 5)

  13. Menne J, et al. Validation of treatment strategies for enterohaemorrhagic Escherichia coli O104:H4 induced haemolytic uraemic syndrome: case–control study. BMJ. 2012;345:e4565. (level 5)

  14. Yagi K et al. Clinical experience of E. coli O-157-related hemolytic uremic syndrome. J Jpn Soc Peiatr Nephrol. 1997;10:209–213. [in Japanese] (level 5)

  15. Antithrombotic therapy for HUS

  16. Diekmann L: Treatment of the hemolytic-uremic syndrome with streptokinase and heparin (author’s transl). Klin padiatr. 1980;192:430–435. (level 4)

  17. Loirat C, et al. Treatment of childhood hemolytic-uremic syndrome with urokinase. Cooperative controlled trial. Arch Fr Pediatr. 1984;41:15–19. (level 4)

  18. Van Damme-Lombaarts R, et al. Heparin plus dipyridamole in childhood hemolytic-uremic syndrome: a prospective, randomized study. J Pediatr. 1988;113:913–918. (level 2)

  19. O’Regan S, et al. Aspirin and dipyridamole therapy in the hemolytic-uremic syndrome. J Pediatr. 1980;97:473–476. (level 4)

  20. Asaga T, et al: A case of thrombotic thrombocytopenic purpura/hemolytic uremic syndrome coincident with disseminated intravascular coagulation caused by abdominal hysterectomy. J Jpn Soc Intensive Care Med. 2008;15:339–340. [in Japanese] (level 5)

  21. Kaneda M, et al. Treatment of hemolytic uremic syndrome with recombinant thrombomodulin. Thrombosis Medicine. 2012;2:198–202. [in Japanese] (level 5)

  22. Treatment of encephalopathy associated with STEC infection

  23. Robson WL, et al. Causes of death in hemolytic uremic syndrome. Child Nephrol Urol. 1991;11:228–233. (level 5)

  24. Kahn SI, et al. Spontaneous recovery of the hemolytic uremic syndrome with prolonged renal and neurological manifestations. Nephron. 1982;32:188–191. (level 5)

  25. Steel BT, et al. Recovery from prolonged coma in hemolytic uremic syndrome. J Pediatr. 1983;102:402–404. (level 5)

  26. Siegler RL. Spectrum of extrarenal involvement in postdiarrheal hemolytic-uremic syndrome. J Pediatr. 1994;125:511–518. (level 5)

  27. Perez N, et al. Steroids in the hemolytic uremic syndrome. Pediatr Nephrol. 1998;12:101–104. (level 4)

  28. Shimizu M, et al. Cytokine profiles of patients with enterohemorrhagic Escherichia coli O111-induced hemolytic-uremic syndrome. Cytokine. 2012;60:694–700. (level 4)

  29. Shiraishi M, et al. Soluble tumor necrosis factor receptor 1 and tissue inhibitor of metalloproteinase-1 in hemolytic uremic syndrome with encephalopathy. J Neuroimmunol. 2008;196:147–152. (level 4)

  30. Yanagisawa A, et al. [Hemolytic uremic syndrome complicated by acute childhood necrotizing encephalopathy]. J Jpn Soc Peiatr Nephrol. 2009;22:161–165., in Japanese. (level 5)

  31. Dundas S, et al. Effectiveness of therapeutic plasma exchange in the 1996 Lanarkshire Escherichia coli O157:H7 outbreak. Lancet. 1999;354:1327–1330. (level 5)

  32. Nathanson S, et al. Acute neurological involvement in diarrhea-associated hemolytic uremic syndrome. Clin J Am Soc Nephrol. 2010;5:1218–1228. (level 4)

  33. Colic E, et al. Management of an acute outbreak of diarrhoea-associated haemolytic uraemic syndrome with early plasma exchange in adults from southern Denmark: an observational study. Lancet. 2011;378:1089–1093. (level 4)

  34. Lapeyraque AL, et al. Eculizumab in severe Shiga-toxin-associated HUS. N Engl J Med. 2011;364:2561–2563. (level 5)

  35. Menne J, et al. STEC-HUS consortium. Validation of treatment strategies for enterohaemorrhagic Escherichia coli O104:H4 induced haemolytic uraemic syndrome: case–control study. Br Med J. 2012;345:e4598. (level 4)

  36. Honda T, et al. A novel strategy for hemolytic uremic syndrome: successful treatment with thrombomodulin α. Pediatrics. 2013;131:e928–33. (level 5)

  37. Renal sequelae of HUS

  38. Gerber A, et al. Clinical course and the role of Shiga toxin-producing Escherichia coli infection in the hemolytic-uremic syndrome in pediatric patients, 1997–2000, in Germany and Austria: a prospective study. J Infect Dis. 2002;186:493–500. (level 4)

  39. Kamioka I, et al. Japanese Society for Pediatric Nephrology: Risk factors for developing severe clinical course in HUS patients: a national survey in Japan. Pediatr Int. 2008;50:441–446 (level 4)

  40. Oakes RS, et al. Predictors of fatality in postdiarrheal hemolytic uremic syndrome. Pediatrics. 2006;117:1656–1662. (level 4)

  41. Spinale JM, et al. Long-term outcomes of Shiga toxin hemolytic uremic syndrome. Pediatr Nephrol. 2013 Jan 4. [Epub ahead of print]

  42. Garg AX, et al. Long-term renal prognosis of diarrhea-associated hemolytic uremic syndrome: A systematic review, meta-analysis, and meta-regression. JAMA. 2003;290:1360–1370. (level 4)

  43. Garg AX, et al. Microalbuminuria three years after recovery from Escherichia coli O157 hemolytic uremic syndrome due to municipal water contamination. Kidney Int. 2005;67:1476–1482. (level 4)

  44. Sharma AP, et al. Chronic renal disease is more prevalent in patients with hemolytic uremic syndrome who had a positive history of diarrhea. Kidney Int. 2010;78:598–604. (level 4)

  45. Siegler RL, et al. Long-term outcome and prognostic indicators in the hemolytic-uremic syndrome. J Pediatr. 1991;118:195–200. (level 4)

  46. Fitzpatrick MM, et al. Long term renal outcome of childhood haemolytic uraemic syndrome. BMJ. 1991;303:489–492. (level 4)

  47. Small G, et al. Hemolytic uremic syndrome: defining the need for long-term follow-up. Clin Nephrol. 1999;52:352–356. (level 4)

  48. Kelles A, et al. Childhood haemolytic uraemic syndrome: long-term outcome and prognostic features. Eur J Pediatr. 1994;153:38–42. (level 4)

  49. Spizzirri FD, et al. Childhood hemolytic uremic syndrome in Argentina: long-term follow-up and prognostic features. Pediatr Nephrol. 1997;11:156–160. (level 4)

  50. Gagnadoux MF, et al. Long-term (15–25 years) outcome of childhood hemolytic-uremic syndrome. Clin Nephrol. 1996;46:39–41. (level 4)

  51. Hüseman D, et al. Long-term prognosis of hemolytic uremic syndrome and effective renal plasma flow. Pediatr Nephrol. 1999;13:672–677. (level 4)

  52. Oakes RS, et al. Duration of oliguria and anuria as predictors of chronic renal-related sequelae in post-diarrheal hemolytic uremic syndrome. Pediatr Nephrol. 2008;23:1303–1308. (level 4)

  53. Cobeñas CJ, et al. Long-term follow-up of Argentinean patients with hemolytic uremic syndrome who had not undergone dialysis. Pediatr Nephrol. 2007;22:1343–1347. (level 4)

  54. Garg AX, et al. Absence of renal sequelae after childhood Escherichia coli O157:H7 gastroenteritis. Kidney Int. 2006;70:807–812. (level 4)

  55. Extra-renal sequelae in patients with HUS

  56. Crabbe DCG, et al. Gastrointestinal complications of the haemolytic uraemic syndrome. J Roy Soc Med. 1990;83:773–775. (level 5)

  57. Brandt JR, et al. Cholelithiasis following Escherichia coli O157:H7-associated hemolytic uremic syndrome. Pediatr Nephrol. 1988;12:222–225. (level 5)

  58. Suri RS, et al. Relationship between Escherichia coli O157:H7 and diabetes mellitus. Kidney Int Suppl. 2009;112:S44–S46. (level 4)

  59. Suri RS, et al. Diabetes during diarrhea-associated hemolytic uremic syndrome: a systematic review and meta-analysis. Diabetes Care. 2005;28:2556–2562. (level 4)

  60. Nathanson S, et al. Acute neurological involvement in diarrhea-associated hemolytic uremic syndrome. Clin J Am Soc Nephrol. 2010;5:1218–1228. (level 5)

  61. Brasher C, et al. The hemolytic-uremic syndrome. West J Med. 1981;134:193–197. (level 5)

  62. Sheth KJ, et al. Neurological involvement in hemolytic-uremic syndrome. Ann Neurol. 1986;19:90–93. (level 4)

  63. Schlieper A, et al. Sequelae of hemolytic uremic syndrome. Arch Dis Child. 1992;67:930–934. (level 4)

  64. Schlieper A, et al. Neuropsychological sequelae of haemolytic uraemic syndrome. Investigators of the HUS Cognitive Study. Arch Dis Child. 1999;80:214–220. (level 4)

  65. Poulton J, et al. Dilated cardiomyopathy associated with haemolytic uraemic syndrome. Br Heart J. 1987;57:181–183. (level 5)

  66. Mohammed J, et al. Cardiac tamponade in diarrhea-positive haemolytic uraemic syndrome. Nephrol Dial Transplant. 2009;24:679–681. (level 5)

  67. Askiti V, et al. Troponin I levels in a hemolytic uremic syndrome patient with severe cardiac failure. Pediatr Nephrol. 2004;19:345–348. (level 5)

4 Diagnosis and treatment of HUS in adults

  1. Diagnosis and treatment of HUS in adults

  2. Melnyk AMS, et al. Adult hemolytic-uremic syndrome: a review of 37 cases. Arch Intern Med. 1995;155:2077–2084. (level 4)

  3. Tostivint I, et al. Adult haemolytic and uraemic syndrome: causes and prognostic factors in the last decade. Nephrol Dial Transplant. 2002;17:1228–1234. (level 4)

  4. George JN: The thrombotic thromobytopenic purpura and hemolytic uremic syndromes: overview of pathogenesis (Experience of the Oklahoma TTP-HUS Registry, 1989–2007). Kidney Int. 2009;75:S8–S10. (level 4)

  5. Schieppati A, et al. Renal function at hospital admission as a prognosis factor in adult hemolytic uremic syndrome. J Am Soc Nephrol. 1992;2:1640–1644. (level 4)

  6. Bell WR, et al. Improved survival in thrombotic thrombocytopenic purpura-hemolytic uremic syndrome. Clinical experience in 108 patients. N Engl J Med. 1991;325:398–403. (level 4)

  7. von Baeyer H: Plasmapheresis in thrombotic microangiopathy-associated syndromes: review of outcome data derived from clinical trials and open studies. Ther Apher. 2002;6:320–328. (level 4)

  8. Brunskill SJ, et al. A systematic review of randomized controlled trials for plasma exchange in the treatment of thrombotic thrombocytopenic purpura. Transfus Med. 2007;17:17–35. (level 1)

  9. Forzley BR, et al. Treating TTP/HUS with plasma exchange: a single centre’s 25-year experience. Br J Haematol. 2008;143:100–106. (level 5)

  10. Clark WF: Thrombotic microangiopathy: current knowledge and outcomes with plasma exchange. Semin Dial. 2012;25:214–219. (level 4)

  11. Swisher KK, et al. Clinical outcomes after platelet transfusions in patients with thrombotic thrombocytopenic purpura. Transfusion. 2009;49:873–887. (level 4)

  12. Diagnosis and treatment of STEC-associated HUS in adults

  13. Karpac CA, et al. Sporadic bloody diarrhoea-associated thrombotic thrombocytopenic purpura-haemolytic uraemic syndrome: an adult and paediatric comparison. Br J Haematol. 2008;141:696–707. (level 4)

  14. Frank C, et al. HUS Investigation Team. Epidemic profile of Shiga-toxin-producing Escherichia Coli O104:H4 outbreak in Germany. N Eng J Med. 2011;365:1771–1780. (level 4)

  15. Dundas S, et al. The Central Scotland Escherichia coli O157:H7 outbreak: risk factors for the hemolytic uremic syndrome and death among hospitalized patients. Clin Infect Dis. 2001;33:923–931. (level 4)

  16. Karmali MA, et al. Age-specific frequencies of antibodies to Escherichia coli verocytotoxins (Shiga toxins) 1 and 2 among urban and rural populations in southern Ontario. J Infect Dis. 2003;188:1724–1729. (level 4)

  17. Dundas S, et al. Effectiveness of therapeutic plasma exchange in the 1996 Lanarkshire Escherichia coli O157:H7 outbreak. Lancet. 1999;354:1327–1330. (level 4)

  18. Colic E, et al. Management of an acute outbreak of diarrhoea-associated haemolytic uraemic syndrome with early plasma exchange in adults from southern Denmark: an observational study. Lancet. 2011;378:1089–1093. (level 5)

  19. Menne J, et al. EHEC–HUS consortium. Validation of treatment strategies for enterohaemorrhagic Escherichia coli O104:H4 induced haemolytic uraemic syndrome: case–control study. Br Med J. 2012;345:e4598. (level 4)

  20. Greinacher A, et al. Treatment of severe neurological deficits with IgG depletion through immunoadsorption in patients with Escherichia coli O104:H4-associated haemolytic uraemic syndrome: a prospective trial. Lancet. 2011;378:1166–1173. (level 4)

  21. Nitschke M, et al. Association between azithromycin therapy and duration of bacterial shedding among patients with Shiga toxin-producing enteroaggregative Escherichia coli O104:H4. JAMA. 2012;307:1046–1052. (level 4)

  22. LapeyraqueAL, et al. Eculizumab in severe Shiga-toxin-associated HUS. N Engl J Med. 2011;364:2561–2563. (level 5)

5 Diagnosis and treatment of atypical hemolytic uremic syndrome (aHUS)

  1. Treatment of aHUS

  2. Krysan DJ, et al. Renal transplantation after streptococcus pneumonia-associated hemolytic uremic syndrome. Am J Kidney Dis. 2001;37:e15. (level 5)

  3. McGraw ME, et al. Haemolytic uremic syndrome and the Thomsen-Friedenreich antigen. Pediatr Nephrol. 1989;3:135–139. (level 5)

  4. Gilbert RD, et al. Streptococcus pneumonia-associated hemolytic uremic syndrome. Pediatr Infect Dis J. 1998;17:530–532. (level 5)

  5. Noris M, et al. Relative role of genetic complement abnormalities in sporadic and familiar aHUS and their impact on clinical phenotype. Clin J Am Soc Nephrol. 2010;5:1844–1859. (level 4)

  6. Remuzzi G, et al. Combined kidney and liver transplantation for familial haemolytic uremic syndrome. Lancet. 2002;359:1671–1672. (level 5)

  7. Noris M, et al. Relative role of genetic complement abnormalities in sporadic and familiar aHUS and their impact on clinical phenotype. Clin J Am Soc Nephrol. 2010;5:1844–1859. (level 4)

  8. Remuzzi G, et al. Hemolytic uremic syndrome: a fatal outcome after kidney and liver transplantation performed to correct factor H gene mutation. Am J Transplant. 2005;5:1146–1150. (level 5)

  9. Remuzzi G, et al. Hemolytic uremic syndrome: a fatal outcome after kidney and liver transplantation performed to correct factor H gene mutation. Am J Transplant. 2005;5:1146–1150. (level 5)

  10. Cheong HI, et al. Attempted treatment of factor H deficiency by liver transplantation. Pediatr Nephrol. 2004;19:454–458. (level 5)

  11. Saland JM, et al. Favorable long-term outcome after liver-kidney transplant for recurrent hemolytic uremic syndrome associated with a factor H mutation. Am J Transplant. 2006;6:1948–1952. (level 5)

  12. Jalanko H, et al. Successful liver-kidney transplantation in two children with aHUS caused by a mutation in complement factor H. Am J Transplant. 2008;8:8216–221. (level 5)

  13. Saland JM, et al. Successful split liver-kidney transplant for factor H associated hemolytic uremic syndrome. Clin J Am Soc Nephrol. 2009;4:201–206. (level 5)

  14. Gruppo RA, et al. Eculizumab for congenital atypical hemolytic-uremic syndrome. N Eng J Med. 2009;360:544–546. (level 5)

  15. Nürnberger J, et al. Eculizumab for atypical hemolytic-uremic syndrome. N Eng J Med. 2009;360:542–544 (level 5)

  16. Ohanian M, et al. Eculizumab safety reverses neurologic impairment and eliminates need for dialysis in severe atypical hemolytic uremic syndrome. Clin Pharmacol. 2011;3:5–12. (level 5)

  17. Dorresteijn EM, et al. Eculizumab as rescue therapy for atypical hemolytic uremic syndrome with normal platelet count. Pediatr Nephrol. 2012;27:1193–1195. (level 5)

  18. Zimmerhackl LBHofer J, et al. Prophylactic eculizumab after renal transplantation in atypical hemolytic uremic syndrome. N Eng J Med. 2010;362:1746–1748. (level 5)

  19. Weitz M, et al. Prophyractic eculizumab prior to kidney transplantation for atypical hemolytic uremic syndrome. Pediatr Nephrol. 2011;26:1325–1329. (level 5)

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Acknowledgments

These guidelines were supported by Grant-in-Aid for Scientific Research from the Ministry of Health, Labor and Welfare of Japan (Research fund for emerging and re-emerging infections including new types of influenza infection; Study group for pathological factors in severe form of enterohemorrhagic Escherichia coli infections and the generalization of therapy. # H24-Shinkou-Ippan-012, head: Makoto Ohnishi).

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Correspondence to Takashi Igarashi.

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All authors are committee members for Guidelines for the Management and Investigation of Hemolytic Uremic Syndrome, English edition.

Representative: Takashi Igarashi.

Members: Akihiko Saitoh, Shuichi Ito, Hiroshi Hataya, Masashi Mizuguchi, Tsuneo Morishima, Makoto Ohnishi.

Assistant members: Kenji Ohnishi, Naohisa Kawamura, Hirotsugu Kitayama, Akira Ashida, Shinya Kaname, Hiromichi Taneichi, Mayumi Sako, Julian Tang.

This is the English version of Guidelines for the Management and Investigation of Hemolytic Uremic Syndrome, which was published in 2013 (in Japanese). (http://www.jspn.jp/kaiin/guideline.shtml)

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Igarashi, T., Ito, S., Sako, M. et al. Guidelines for the management and investigation of hemolytic uremic syndrome. Clin Exp Nephrol 18, 525–557 (2014). https://doi.org/10.1007/s10157-014-0995-9

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