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Haemolytic uraemic syndrome: prognostic factors in children over 3 years of age

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Abstract

Previous studies have shown that age at onset of primary haemolytic uraemic syndrome (HUS) is a feature of prognostic significance, the disease being of much better outcome in paediatric patients younger than 3 years than in older children. In an attempt to find an explanation for such a difference, we analysed the clinical and pathological features of 42 children over 3 years of age who presented with HUS between 1955 and 1990 in our department. On the basis of the presence of a prodromal diarrhoea, we divided our patients into two groups: 21 children presented with the diarrhoea-associated (typical or D+) form of HUS, whereas 21 had the non-diarrhoea-associated (atypical or D-) form. Of the 42 children, 20 (47.5%) progressed to end-stage renal failure. However, our study shows that age at onset of HUS is not a prognostic feature per se. The difference in outcome between children and infants is most likely related to the high incidence of the atypical subset of HUS in children over 3 years, a subset that is very uncommon in infants. The ominous features which characterise this form of the disease are: (1) the absence of a diarrhoeal prodrome, (2) normal urine output, (3) marked proteinuria, (4) hypertension, (5) the occurrence of relapses or recurrences and (6) the presence of widespread and severe arteriolar changes on renal biopsy. The poor prognosis of the atypical form of HUS warrants the use of fresh-frozen plasma infusions and/or plasma exchange as early as possible in the course of the disease.

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References

  1. Gasser C, Gautier C, Steck A, Siebenmann RE, Oechslin R (1955) Hämolytisch-urämische Syndrome. Bilaterale Nierenrindennekrosen bei akuten erworbenen hämolytischen Anämien. Schweiz Med Wochenschr 85:905–909

    PubMed  Google Scholar 

  2. Levin M, Barratt TM (1984) Haemolytic uremic syndrome. Arch Dis Child 59:397–400

    PubMed  Google Scholar 

  3. Kaplan BS, Thomson PD, Chadaverian JP de (1976) The haemolytic uraemic syndrome. Pediatr Clin North Am 23:761–777

    PubMed  Google Scholar 

  4. Drummond KN (1985) Haemolytic uraemic syndrome — then and now. N Engl J Med 312:116–118

    PubMed  Google Scholar 

  5. Kaplan BS, Proesmans W (1987) The haemolytic uraemic syndrome of childhood and its variants. Semin Hematol 24:148–160

    PubMed  Google Scholar 

  6. Taylor CM, Milford DV, White RHR (1991) A plea for standardized terminology within the haemolytic uraemic syndromes. Pediatr Nephrol 5:97

    PubMed  Google Scholar 

  7. Gervais M, Richardson JB, Chiu J, Drummond KN (1971) Immunoflurescent and histologic findings in the haemolytic uraemic syndrome. Pediatrics 47:352–359

    PubMed  Google Scholar 

  8. Gianantonio C, Vitacco M, Mendilharzu F, Gallo G, Sojo E (1973) The haemolytic uraemic syndrome. Nephron 14:692–698

    Google Scholar 

  9. Donckerwolke RA, Tiddens H, Kuijten R (1973) Prospectief onderzoek naar het haemolytisch-uraemisch syndrom. Acta Paediatr Belg 27:284–298

    PubMed  Google Scholar 

  10. Tune BM, Leavitt TJ, Gribble TJ (1973) The haemolytic-uraemic syndrome in California: a review of 28 non heparinized cases with long-term follow-up. J Pediatr 82:304–310

    PubMed  Google Scholar 

  11. Janssen F, Potvliege PR, Vainsel M, Vis HL (1974) Le pronostic à court et à long terme du syndrome hémolytique et urémique. Arch Fr Pediatr: 59–69

  12. Sorrenti LY, Lewy PR (1978) The haemolytic uraemic syndrome. Experience at a center in the Midwest. Am J Dis Child 132:59–62

    PubMed  Google Scholar 

  13. Dolislager D, Tune B (1978) The haemolytic uraemic syndrome. Spectrum of severity and significance of prodrome. Am J Dis Child 132:55–58

    PubMed  Google Scholar 

  14. Binda ki Muaka P, Proesmans W, Eeckels R (1981) The haemolytic uraemic syndrome in childhood: a study of the long-term prognosis. Eur J Pediatr 136:237–243

    PubMed  Google Scholar 

  15. Habib R, Levy M, Gagnadoux MF, Broyer M (1982) Prognosis of haemolytic-uraemic syndrome in children. Adv Nephrol 11: 99–128

    Google Scholar 

  16. Trompeter RS, Schwartz R, Chantler C, Dillon MJ, Haycock GB (1983) Haemolytic uraemic syndrome: an analysis of prognostic features. Arch Dis Child 58:101–105

    PubMed  Google Scholar 

  17. Loirat C, Sonsino E, Varga Moreno A, Pillion G, Mercier JC, Beaufils F, Mathieu H (1984) Haemolytic uraemic syndrome: an analysis of the natural history and prognostic features. Acta Paediatr Scand 73:505–514

    PubMed  Google Scholar 

  18. De Jong M, Monnens L (1988) Haemolytic-uraemic syndrome: a 10-year follow-up study of 73 patients. Nephrol Dial Transplant 3:379–382

    PubMed  Google Scholar 

  19. Van Dyck M, Proesmans W, Depraetere M (1988) Haemolytic uraemic syndrome in childhood: renal function 10 years later. Clin Nephrol 3:109–112

    Google Scholar 

  20. O'Regan S, Blais N, Russo P, Pison CF, Rousseau E (1989) Haemolytic uraemic syndrome: glomerular filtration rate, 6–11 years later measured by 99 m Tc DTPA plasma slope clearance. Clin Nephrol 32:217–220

    PubMed  Google Scholar 

  21. Sieniawska M, Korniszewska J, Gura C, Welc-Dobies J, Lewicki Z (1990) Prognostic significance of certain factors in the haemolytic uraemic syndrome. Pediatr Nephrol 4:213–218

    PubMed  Google Scholar 

  22. Coad NAG, Marshall T, Rowe B, Taylor CM (1991) Changes in the postenteropathic form of the haemolytic uraemic syndrome in children. Clin Nephrol 35:10–16

    PubMed  Google Scholar 

  23. Fitzpatrick MM, Shah V, Trompeter RS, Dillon MJ, Barratt TM (1991) Long term renal outcome of childhood haemolytic uraemic syndrome. BMJ 303:489–492

    PubMed  Google Scholar 

  24. Siegler RL, Milligan MK, Burningham TH, Christofferson RD, Chang SY, Jorde LB (1991) Long term outcome and prognostic indicators in the haemolytic-uraemic syndrome. J Pediatr 118:195–200

    PubMed  Google Scholar 

  25. Loirat C, Baudouin V, Sonsino E, Mariani-Kurdjian P, Elion J (1993) Haemolytic uraemic syndrome. Adv Nephrol 22: 141–168

    Google Scholar 

  26. Fitzpatrick M, Walters MDS, Trompeter RS, Dillon MJ, Barratt TM (1993) Atypical (non-diarrhoea-associated) haemolytic-uraemic syndrome in childhood. J Pediatr 122:532–537

    PubMed  Google Scholar 

  27. Caprioli A, Luzzi I, Rosmini F, Pasquini P, Cirrincione R, Gianviti A, Mateucci MC, Rizzoni G and the HUS Italian Study Group (1992) Haemolytic uraemic syndrome and verocytotoxin-producingEscherichia coli infection in Italy. J Infect Dis 166:154–158

    PubMed  Google Scholar 

  28. Argyle JC, Hogg RJ, Pyscher TJ, Silva FG, Siegler RL (1990) A clinico-pathological study of 24 children with haemolytic uraemic syndrome. A report of the Southwest Pediatric Nephrology Study Group. Pediatr Nephrol 4:52–58

    PubMed  Google Scholar 

  29. Siegler RL (1988) Management of haemolytic uraemic syndrome. J Pediatr 112:1014–1020

    PubMed  Google Scholar 

  30. Loirat C (1992) Treatment of HUS with fresh frozen plasma or plasmapheresis. In: Kaplan BS, Trompeter RS, Moake JL (eds) Haemolytic uraemic syndrome and thrombotic thrombocytopenic purpura. Dekker, New York, pp 431–440

    Google Scholar 

  31. Remuzzi G, Misiani R, Marchesi D, Livio M, Mecca G, Gaetano G de, Donati MB (1978) Haemolytic uraemic syndrome: deficiency of plasma factor(s) regulating prostacyclin activity. Lancet II:871–872

    Google Scholar 

  32. Remuzzi G, Mecca G, Livio M, Gaetano G de, Donati MB, Pearson JK, Gordon JL (1980) Prostacyclin generation by cultured endothelial cells in haemolytic uraemic syndrome. Lancet I:656–657

    Google Scholar 

  33. Levin M, Elkon KB, Nokes TJC, Buckle AM, Dillon MJ, Hardisty RM, Barratt TM (1983) Inhibitor of prostacyclin production in sporadic haemolytic uraemic syndrome. Arch Dis Child 58: 703–708

    PubMed  Google Scholar 

  34. Walters MDS, Levin M, Smith C, Nokes TJC, Hardisty RM, Dillon MJ, Barratt TM (1988) Intravascular platelet activation in the haemolytic uremic syndrome. Kidney Int 33:107–115

    PubMed  Google Scholar 

  35. Barratt JM, Dillon MJ, Hardisty RM, Levin M, Nokes TJC, Smith C, Stroobant P, Walters MDS (1987) The role of platelet-derived growth factors in the pathogenesis of the haemolytic-uraemic syndrome. In: Murakami K, Kitagawa T, Yabuta K, Sakai T (eds) Recent advances in nephrology. Elsevier, Amsterdam, pp 577–580

    Google Scholar 

  36. Misiani R, Appiani AC, Edefonti A, Gotti E, Bettinelli A, Giani M, Rossi E, Remuzzi G, Mecca G (1982) Haemolytic uraemic syndrome: therapeutic effect of plasma infusion. BMJ 285: 1304–1306

    PubMed  Google Scholar 

  37. Cole BR (1988) Plasma infusion therapy in haemolytic uraemic syndrome: is it warranted? Pediatr Nephrol 2:286–287

    PubMed  Google Scholar 

  38. Rizzoni G, Claris-Appiani, Edefonti A, Facchin P, Franchini F, Gusmano R, Imbasciati E, Pavanello L, Perfumo F, Remuzzi G (1988) Plasma infusion for haemolytic uraemic syndrome in children: results of a multicenter controlled trial. J Pediatr 112:284–290

    PubMed  Google Scholar 

  39. Loirat C, Sonsino E, Hinglais N, Jais JP, Landais P, Fermanian J (1988) Treatment of the childhood haemolytic uraemic syndrome with plasma. A multicenter randomized controlled trial. Pediatr Nephrol 2:279–285

    PubMed  Google Scholar 

  40. Eddy AA, Geary DF, Balfe JW, Clark WF, Baumal R (1989) Prolongation of acute renal failure in two patients with haemolytic uraemic syndrome due to excessive plasma infusion therapy. Pediatr Nephrol 3:420–423

    PubMed  Google Scholar 

  41. Shepard KV, Bukowski RM (1987) The treatment of thrombotic thrombocytopenic purpura with exchange transfusions, plasma infusions and plasma exchange. Semin Hematol 24:178–193

    PubMed  Google Scholar 

  42. Remuzzi G, Misiani R, Marchesi D, Livio M, Mecca G, Gaetano G de, Donati MB (1979) Treatment of the haemolytic uraemic syndrome with plasma. Clin Nephrol 12:279–284

    PubMed  Google Scholar 

  43. Beattie TJ, Murphy AV, Willoughby MLN, Machin SJ, Defreyn G (1981) Plasmapheresis in the haemolytic uraemic syndrome in children. BMJ 282:1667–1668

    PubMed  Google Scholar 

  44. Dillon MJ, Barratt TM, Sabbagh PA, Urquhart IA, Ryall MA, Deal JE, Walters MDS (1987) Plasma exchange in children: results in vasculitis and haemolytic uraemic syndrome. In: Murakami K, Kitagawa T, Yabuta K, Sakai T (eds) Recent advances in paediatric nephrology. Elsevier, Amsterdam, pp 447–452

    Google Scholar 

  45. Müller-Wiefel DE, Bulla M, Klare B, Balzar E, Bambauer R, Tönschoff B (1989) Therapeutic plasma exchange in haemolytic uraemic syndrome (abstract). Pediatr Nephrol 3:C183

    Google Scholar 

  46. Ogborn MR, Crocker JFS, Barnard DR (1990) Plasma therapy for severe haemolytic uraemic syndrome in children in Atlantic Canada. Can Med Assoc J 143:1323–1326

    Google Scholar 

  47. Bell WR, Braine HG, Ness PM, Klicker TS (1991) Improved survival in thrombotic thrombocytopenic purpura — haemolytic uraemic syndrome. N Engl J Med 325:398–403

    PubMed  Google Scholar 

  48. Robson WLM, Leung AKC (1991) The successful treatment of atypical haemolytic uraemic syndrome with plasmapheresis. Clin Nephrol 35:119–122

    PubMed  Google Scholar 

  49. Rock GA, Shumak KH, Buskard NA, Blanchette VS, Kelton JG, Nair RC, Spasoff RA (1991) Comparison of plasma exchange with plasma infusion in the treatment of thrombotic thrombocytopenic purpura. N Engl J Med 325:393–397

    PubMed  Google Scholar 

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Authors and Affiliations

  1. Department of Paediatric Nephrology, Necker-Enfants Malades Hospital, 149 rue de Sèvres, F-75743, Paris Cedex 15, France

    C. Renaud, P. Niaudet, M. F. Gagnadoux, M. Broyer & R. Habib

  2. INSERM U. 192 Tour Lavoisier-6ème étage, Necker-Enfants Malades Hospital, 149 rue de Sèvres, F-75743, Paris Cedex 15, France

    C. Renaud, P. Niaudet, M. F. Gagnadoux, M. Broyer & R. Habib

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  1. C. Renaud
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  2. P. Niaudet
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  3. M. F. Gagnadoux
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  4. M. Broyer
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  5. R. Habib
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Additional information

Since the submission of this manuscript, similar findings have been published by Bhuyan et al: Bhuyan UN, Bagga A, Srivastava RN (1994) Acute renal failure and severe hypertension in children with renal thrombotic microangiopathy. Nephron 66: 302–306

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Renaud, C., Niaudet, P., Gagnadoux, M.F. et al. Haemolytic uraemic syndrome: prognostic factors in children over 3 years of age. Pediatr Nephrol 9, 24–29 (1995). https://doi.org/10.1007/BF00858960

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  • DOI: https://doi.org/10.1007/BF00858960

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