Skip to main content
SpringerLink
Log in

Encore d’actualité ! Escherichia coli et syndrome hémolytique et urémique chez l’enfant et l’adulte

A question of burning importance! Hemolytic and uremic syndrome in children and adults

  • Mise au Point / Update
  • Published:
Réanimation

Résumé

Le syndrome hémolytique et urémique (SHU) à Escherichia coli est dû à une microangiopathie thrombotique touchant principalement le rein, entraînant une hypertension artérielle (HTA) et une insuffisance rénale aiguë (IRA). Sa pathogénie comprend une lésion/activation de l’endothélium, essentiellement au niveau rénal, induite par des entérotoxines ou shigatoxines bactériennes. Le SHU typique est la première cause d’IRA du nourrisson et de l’enfant, mais peut aussi être observé chez l’adulte, à tout âge. L’anémie hémolytique mécanique avec présence de schizocytes, la thrombopénie sans coagulation intravasculaire disséminée et l’insuffisance rénale sont caractéristiques. Dans les SHU typiques, une diarrhée prodromique, volontiers sanglante, est observée, en rapport avec une infection par une entérobactérie pathogène, souvent le E. coli O157:H7. D’autres sérotypes peuvent émerger comme l’a montré récemment l’épidémie de diarrhée aiguë et de SHU en Allemagne et en France due à E. coli O104:H4. Le SHU typique postinfectieux doit être distingué du SHU atypique lié à des anomalies génétiques des facteurs de régulation de la voie alterne du complément ou des SHU médicamenteux. Le pronostic s’est amélioré, avec une survie des malades de plus de 85 % à un an. L’insuffisance rénale chronique séquellaire est observée dans 10 à 20 % des cas. Le traitement est essentiellement symptomatique: réhydratation, épuration extrarénale, contrôle de l’HTA. Il n’y a pas d’indication aux ralentisseurs du transit ni à une antibiothérapie spécifique. Les perfusions de plasma frais ou les échanges plasmatiques sont discutés chez l’enfant, réservés aux formes graves avec signes neurologiques, et presque systématiques chez l’adulte. L’eculizumab, anticorps anti-C5, pourrait être utile dans les formes sévères.

Abstract

The hemolytic uremic syndrome (HUS) is related to a thrombotic microangiopathy in the kidney, leading to hypertension and acute renal failure. In the post-infectious, typical form, its pathogenesis includes a lesion/activation of endothelial cells, mainly in the kidney vasculature, induced by bacterial enterotoxins known as the shiga-toxins. Haemolytic anemia with schizocytes, thrombocytopenia without intravascular coagulation and renal failure are present. In typical HUS, a prodromic hemorragic diarrhea is observed due to an infection by an enteropathogenic bacteria, mainly the O157:H7 Escherichia coli. Recently a very large epidemic of diarrhea complicated by HUS in about 25% of the cases has been observed in Germany. Prognosis has improved greatly, and the patient survival rate is higher than 85% in a year. Chronic renal failure may result in 10 to 20% of the cases. Fresh plasma infusion or plasma exchanges are recommended in most of the cases of typical HUS, especially in severe forms in both children and adults. However, eculizumab, an anti-C5 antibody which is now recommended in complement deficiency-associated HUS, has been shown recently to be effective in post-infectious HUS as well. It may become the firstline treatment of infectious HUS in the near future.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Références

  1. Frank C, Werber D, Cramer JP, et al (2011) Epidemic profile of shiga-toxin-producing Escherichia coli O104:H4 Outbreak in Germany — Preliminary Report. N Engl J Med 365:1771–1780

    Article  PubMed  CAS  Google Scholar 

  2. Benz K, Amann K (2010) Thrombotic microangiopathy: new insights. Curr Opin Nephrol Hypertens 19:242–247

    Article  PubMed  Google Scholar 

  3. Konowalchuk J, Speirs JI, Stavric S (1977) Vero response to a cytotoxin of Escherichia coli. Infect Immun 18:775–779

    PubMed  CAS  Google Scholar 

  4. Tarr PI, Gordon CA, Chandler WL (2005) Shiga-toxin-producing Escherichia coli and haemolytic uraemic syndrome. Lancet 365:1073–1078

    PubMed  CAS  Google Scholar 

  5. Moake JL (2002) Thrombotic microangiopathies. N Engl J Med 347:589–600

    Article  PubMed  CAS  Google Scholar 

  6. Tsai HM (2006) The molecular biology of thrombotic microangiopathy. Kidney International 70:16–23

    Article  PubMed  CAS  Google Scholar 

  7. Morigi M, Galbusera M, Gastoldi S, et al (2011) Alternative pathway activation of complement by shiga-toxin promotes exuberant C3a formation that triggers microvascular thrombosis. J Immunol 187:172–180

    Article  PubMed  CAS  Google Scholar 

  8. Ståhl AL, Sartz L, Karpman D (2011) Complement activation on platelet-leukocyte complexes and microparticles in enterohemorrhagic Escherichia coli-induced hemolytic uremic syndrome. Blood 117:5503–5513

    Article  PubMed  Google Scholar 

  9. Orth D, Khan AB, Naim A, et al (2009) Shiga-toxin activates complement and binds factor H: evidence for an active role of complement in hemolytic uremic syndrome. J Immunol 182:6394–6400

    Article  PubMed  CAS  Google Scholar 

  10. Lapeyraque AL, Malina M, Fremeaux-Bacchi V, et al (2011) Eculizumab in severe shiga-toxin-associated HUS. N Engl J Med 364:2561–2563

    Article  PubMed  CAS  Google Scholar 

  11. Te Loo DM, Monnens LA, Van der Velden TJ, et al (2000) Binding and transfer of verototoxin by polynuclear leukocytes in hemolytic uremic syndrom. Blood 95:3396–3402

    Google Scholar 

  12. Dundas S, Todd WT, Stewart AI, et al (2001) The central scotland Escherichia coli O157:H7 outbreak: risk factors for the hemolytic uremic syndrome and death among hospitalized patients. Clin Infect Dis 33:923–931

    Article  PubMed  CAS  Google Scholar 

  13. Decludt B, Bouvet E, Mariani-Kurkdjian E, et al (2000) Haemolytic uraemic syndrome and shiga-toxin producing Escherichia coli infection in children in France. Epidemiol Infect 124:215–220

    Article  PubMed  CAS  Google Scholar 

  14. Karpac CA, Li X, Terrell DR, et al (2008) Sporadic bloody diarrhoea-associated thrombotic thrombocytopenic purpurahaemolytic uraemic syndrome: an adult and paediatric comparison. Br J Haematol 141:696–707

    Article  PubMed  Google Scholar 

  15. Noris M, Remuzzi G (2005) Hemolytic uremic syndrome. J Am Soc Nephrol 16:1035–1050

    Article  PubMed  CAS  Google Scholar 

  16. Rasko DA, Webster DR, Sahl JW, et al (2011) Origins of the Escherichia coli strain causing an outbreak of hemolytic-uremic syndrome in Germany. N Engl J Med 365:709–717

    Article  PubMed  CAS  Google Scholar 

  17. Buchholz U, Bernard H, Werber D, et al (2011) German outbreak of Escherichia coli O104:H4 associated with sprouts. N Engl J Med 365:1763–1770

    Article  PubMed  CAS  Google Scholar 

  18. Pennington H (2010) Escherichia coli O157 Lancet 376:1428–1435

    CAS  Google Scholar 

  19. Coppo P, Schwarzinger M, Buffet M, et al (2010) Predictive features of severe acquired ADAMTS13 deficiency in idiopathic thrombotic microangiopathies: the French TMA reference center experience. PLoS One 5:e10208

    Article  PubMed  Google Scholar 

  20. Hertig A, Ridel C, Rondeau E (2010) Hemolytic uremic syndrome in adults. Nephrol Ther 6:258–271

    Article  PubMed  Google Scholar 

  21. Tostivint I, Mougenot B, Flahault A, et al (2002) Adult haemolytic and uraemic syndrome: causes and prognostic factors in the last decade. Nephrol Dial Transplant 17:1228–1234

    Article  PubMed  Google Scholar 

  22. Noris M, Remuzzi G (2009) Atypical hemolytic-uremic syndrome. N Engl J Med 361:1676–1687

    Article  PubMed  CAS  Google Scholar 

  23. Medina PJ, Sipols JM, George JN (2001) Drug-associated thrombotic thrombocytopenic purpura-hemolytic uremic syndrome. Curr Opin Hematol 8:286–293

    Article  PubMed  CAS  Google Scholar 

  24. Frangie C, Lefaucheur C, Medioni J, et al (2007) Renal thrombotic microangiopathy caused by anti-VEGF-antibody treatment for metastatic renal-cell carcinoma. Lancet Oncol 8:177–178

    Article  PubMed  Google Scholar 

  25. Roncone D, Satoskar A, Nadasdy T, et al (2007) Proteinuria in a patient receiving anti-VEGF therapy for metastatic renal cell carcinoma. Nat Clin Pract Nephrol 3:287–293

    Article  PubMed  Google Scholar 

  26. Hahn T, Alam AR, Lawrence D, et al (2004) Thrombotic microangiopathy after allogeneic blood and marrow transplantation is associated with dose-intensive myeloablative conditioning regimens, unrelated donor, and methylprednisolone T-cell depletion. Transplantation 78:1515–1522

    Article  PubMed  Google Scholar 

  27. Wong CS, Jelacic S, Habeeb RL, et al (2000) The risk of the hemolytic-uremic syndrome after antibiotic treatment of Escherichia coli O157: H7 infections. N Engl J Med 342:1930–1936

    Article  PubMed  CAS  Google Scholar 

  28. Garg AX, Suri RS, Barrowman N, et al (2003) Long-term renal prognosis of diarrhea-associated hemolytic uremic syndrome: a systematic review, meta-analysis, and meta-regression. JAMA 290:1360–1370

    Article  PubMed  CAS  Google Scholar 

  29. Rock GA, Shumak KH, Buskard NA, et al (1991) Comparison of plasma exchange with plasma infusion in the treatment of thrombotic thrombocytopenic purpura. N Engl J Med 325:393–397

    Article  PubMed  CAS  Google Scholar 

  30. Bitzan M, Schaefer F, Reymond D (2010) Treatment of typical (enteropathic) hemolytic uremic syndrome. Semin Thromb Hemost 36:594–610

    Article  PubMed  CAS  Google Scholar 

  31. Loirat C, Sonsino E, Hinglais N, et al (1988) Treatment of the childhood haemolytic uraemic syndrome with plasma: a multicentre randomized controlled trial. Pediatr Nephrol 2:279–285

    Article  PubMed  CAS  Google Scholar 

  32. Dundas S, Murphy J, Soutar RL, et al (1999) Effectiveness of therapeutic exchange in the1996 Lanarkshire Escherichia coli O157: H7 outbreak. Lancet 354:1327–1330

    Article  PubMed  CAS  Google Scholar 

  33. Colic E, Dieperink H, Titlestad K, Tepel M (2011) Management of an acute outbreak of diarrhoea-associated haemolytic uraemic syndrome with early plasma exchange in adults from southern Denmark: an observational study. Lancet 378:1089–1093

    Article  PubMed  Google Scholar 

  34. Greinacher A, Friesecke S, Abel P, et al (2011) 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 378:1166–1173

    Article  PubMed  Google Scholar 

  35. Köse O, Zimmerhackl LB, Jungraithmayr T, et al (2010) New treatment options for atypical hemolytic uremic syndrome with the complement inhibitor eculizumab. Semin Thromb Hemost 36:669–672

    Article  PubMed  Google Scholar 

  36. Prescott HC, Wu HM, Cataland SR, Baiocchi RA (2010) Eculizumab therapy in an adult with plasma exchange-refractory atypical hemolytic uremic syndrome. Am J Hematol 5:976–977

    Article  Google Scholar 

  37. Kemper MJ (2011) Outbreak of hemolytic uremic syndrome caused by Escherichia coli O104:H4 in Germany: a pediatric perspective. Pediatr Nephrol 27:161–164

    Article  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Service de néphrologie, hôpital Bichat-Claude-Bernard, 46, rue Henri-Huchard, F-75877, Paris cedex 18, France

    Q. Raimbourg

  2. Service des urgences néphrologiques et transplantation rénale, hôpital Tenon, 4, rue de la Chine, F-75020, Paris, France

    G. d’Ythurbide & E. Rondeau

Authors
  1. Q. Raimbourg
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. G. d’Ythurbide
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. E. Rondeau
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to E. Rondeau.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Raimbourg, Q., d’Ythurbide, G. & Rondeau, E. Encore d’actualité ! Escherichia coli et syndrome hémolytique et urémique chez l’enfant et l’adulte. Réanimation 21, 280–285 (2012). https://doi.org/10.1007/s13546-012-0467-8

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s13546-012-0467-8

Mots clés

Keywords

Search

Navigation