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Treatment of the childhood haemolytic uraemic syndrome with plasma

A multicentre randomized controlled trial

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Abstract

Seventy-nine children with haemolytic uraemic syndrome (mean age 28 months) were randomly assigned either to a group receiving plasma infusions (plasma group,n=39) or to a group treated conservatively (control group,n=40). The duration of haemolysis, thrombocytopenia and anuria was similar in the two groups. Serum creatinine levels were similar in the two groups at the 1-month follow-up but were higher in the control group at 3 months (plasma group 49±14, control group 66±28 μmol/l;P<0.02) and at 6 months (plasma group 48±13, control group 63±21 μmol/l;P<0.005). The prevalence of proteinuria was also higher in the control group at the 6-month follow-up (plasma group 17%, control group 46%;P<0.02). However differences were no longer significant after 1 year. Renal tissue was examined in 54 cases (plasma group,n=27; control group,n=27). Diffuse cortical necrosis was present in 7 cases in the control group but was absent in the plasma group (P<0.02). Taking into consideration the higher serum creatinine levels, the higher prevalence of proteinuria during the first 6 months of follow-up in the control group and the presence of diffuse cortical necrosis in this group compared with the plasma group, we conclude that plasma infusions should be regarded as beneficial. Further study is needed to determine which plasma fraction is involved.

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References

  1. Remuzzi G, Misiani R, Marchesi D, Livion M., Mecca G, De Gaetano G, Donati NB (1978) Haemolytic-uraemic syndrome: deficiency of plasma factor(s) regulating prostacyclin activity. Lancet II: 871–872

    Google Scholar 

  2. Remuzzi G, Misiani R, Mecca G, De Gaetano G., Donati MB (1978) Thrombocytopenic purpura. A deficiency of plasma factors regulating platelet-vessel wall interaction? N Engl J Med 299: 311

    Google Scholar 

  3. Jorgensen KA, Pedersen RS (1981) Familial deficiency of prostacyclin production stimulating factor in the hemolytic uremic syndrome of childhood. Thromb Res 21: 311–315

    Google Scholar 

  4. Chamone DAF, Proesmans WC, Monnens LA, Binda Ki Muaka P, Vermylen J (1982) Reversible deficient prostacyclin release in childhood hemolytic uremic syndrome. Int J Pediatr Nephrol 3: 13–16

    Google Scholar 

  5. Stuart MJ, Spitzer RE, Coppe D (1983) Abnormal platelet and vascular prostaglandin synthesis in an infant with hemolytic uremic syndrome. Pediatrics 71: 120–124

    Google Scholar 

  6. Turi S, Beattie TJ, Belch JJF, Murphy AV (1986) Disturbances of prostacycline metabolism in children with hemolytic-uremic syndrome and in first degree relatives. Clin Nephrol 25: 193–198

    Google Scholar 

  7. 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

    Google Scholar 

  8. Schlegel N, Maclouf J, Loirat C, Drouet L, Marotte R, Scarabin PY, Mathieu H (1987) Childhood hemolytic uremic syndrome. Absence of deficiency of plasma prostacyclin stimulating activity. J Pediatr 111: 71–77

    Google Scholar 

  9. Lian ECY, Harkness DR, Byrnes JJ, Wallach H, Nunez R (1979) Presence of platelet aggregating factor in the plasma of patients with thrombotic thrombocytopenic purpura and its inhibition by normal plasma. Blood 53: 333–338

    Google Scholar 

  10. Kelton JG, Moore J, Santos A, Sheridan D (1984) Detection of a platelet-agglutinating factor in thrombotic thrombocytopenic purpura. Ann Intern Med 101: 589–593

    Google Scholar 

  11. Monnens L, Van de Meer W, Langenhuysen C, Van Munster P, Van Oostrom C (1985) Platelet aggregating factor in the epidemic form of haemolytic-uremic syndrome in childhood. Clin Nephrol 24: 135–137

    Google Scholar 

  12. Lian ECY, Mui PTK, Siddiqui FM, Chiu AYY, Chiu LLS (1984) Inhibition of platelet aggregating activity in thrombotic thrombocytopenic purpura plasma by normal adult immunoglobulin G. J Clin Invest 73: 548–555

    Google Scholar 

  13. Bukowski RM, King JW, Hewlett JS (1977) Plasmapheresis in the treatment of thrombotic thrombocytopenic purpura. Blood 50: 413–417

    Google Scholar 

  14. Ansell JE, Slepchuk NI, Pechet L (1979) Letter to the editor, Blood 54: 959–960

    Google Scholar 

  15. Mc Leod BC, Wu KK, Knoppe WH (1980) Plasmapheresis in thrombotic thrombocytopenic purpura. Arch Intern Med 140: 1059–1060

    Google Scholar 

  16. Myers TJ, Waken CJ, Ball ED, Tremont SJ (1980) Thrombotic thrombocytopenic purpura: combined treatment with plasmapheresis and antiplatelet agents. Ann Intern Med 92: 149–155

    Google Scholar 

  17. Thysell H (1982) Successful treatment of hemolytic uremic syndrome and thrombotic thrombocytopenic purpura with fresh frozen plasma and plasma exchange. Acta Med Scand 212: 285

    Google Scholar 

  18. Harden LB, Gluck RS, Salcedo JR (1980) Simultaneous hemodialysis and exchange transfusion in hemolytic uremic syndrome. Clin Pediatr 19: 640–642

    Google Scholar 

  19. Beattie TJ, Murphy AV, Willoughby MLN, Machin SJ, Defreyn G (1981) Plasmapheresis in the haemolytic-uraemic syndrome in children. Br Med J 282: 1667–1668

    Google Scholar 

  20. Denneberg T, Friedberg M, Holmberg L, Mathiasen C, Nilsson KO, Takolander R, Walder M (1982) Combined plasmapheresis and hemodialysis treatment for severe hemolytic- uremic syndrome following campylobacter colitis. Acta Paediatr Scand 71: 243–245

    Google Scholar 

  21. Byrnes JJ, Khurana M (1977) Treatment of thrombotic thrombocytopenic purpura with plasma. N. Engl J Med 297: 1386–1389

    Google Scholar 

  22. Remuzzi G, Misiani R, Marchesi D, Livio M, Mecca G, De Gaetano G, Donati MB (1979) Treatment of the hemolytic uremic syndrome with plasma. Clin Nephrol 12: 279–284

    Google Scholar 

  23. 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. Br Med J 285: 1304–1306

    Google Scholar 

  24. Rizzoni G, Pavenello L, Claris-Appiani A, Edefonti A, Facchin P, Franchini F, Gusmano R, Imbasciati E, Perfumo F, Remuzzi G (1987) Treatment of children with hemolytic uremic syndrome with plasma: a multicenter controlled trial. Pediatr Nephrol 1: C14

    Google Scholar 

  25. Thoenes W, John HD (1980) Endotheliotropic (hemolytic) nephroangiopathy and it various manifestation forms (thrombotic microangiopathy, primary malignant nephrosclerosis, hemolytic uremic syndrome). Klin Wochenschr 58: 173–182

    Google Scholar 

  26. Habib R, Levy M, Gagnadoux MF, Broyer M (1982) Prognosis of the hemolytic uremic syndrome in children. In: Hamburger J., Crosnier J, Grunfeld JP, Maxwell MH (eds) Advances in nephrology, vol 11. Year Book Medical Publishers, Chicago, pp 99–128

    Google Scholar 

  27. Loirat C, Sonsino E, Varga Moreno A, Pillion G, Mercier JC, Beaufils F, Mathieu H (1986) Hemolytic-uremic syndrome: an analysis of the natural history and prognostic features. Acta Paediatr Scand 73: 505–514

    Google Scholar 

  28. Fong JGB, De Chadaverian JP, Kaplan BS (1982) Hemolytic uremic syndrome. Current concepts and management. Pediatr Clin North Am 29: 835–856

    Google Scholar 

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

    Google Scholar 

  30. Taylor CM, White RHR, Winterborn MH, Rowe B (1986) Haemolytic-uraemic syndrome: clinical experience of an outbreak in the West Midlands. Br Med J 292: 1513–1516

    Google Scholar 

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Authors and Affiliations

  1. Service de Nephrologie Pédiatrique, Hôpital Robert Debré, 48 Boulevard Serurier, F-75019, Paris, France

    C. Loirat

  2. Laboratoire d'Anatomopathologie, Hôpital Robert Debré, 48 Boulevard Serurier, F-75019, Paris, France

    E. Sonsino

  3. Unité Inserm 192, Hôpital Necker, Paris, France

    N. Hinglais

  4. Laboratoire de Biostatistique et d'Informatique Médicale, Hôpital Necker, Paris, France

    J. P. Jais, P. Landais & J. Fermanian

Authors
  1. C. Loirat
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  2. E. Sonsino
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  3. N. Hinglais
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  4. J. P. Jais
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  5. P. Landais
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  6. J. Fermanian
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French Society of Paediatric Nephrology

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Loirat, C., Sonsino, E., Hinglais, N. et al. Treatment of the childhood haemolytic uraemic syndrome with plasma. Pediatr Nephrol 2, 279–285 (1988). https://doi.org/10.1007/BF00858677

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  • DOI: https://doi.org/10.1007/BF00858677

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