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Renal transplantation in HUS patients with disorders of complement regulation

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Abstract

Haemolytic uraemic syndrome (HUS) is the primary diagnosis of 4.5% of children on chronic renal replacement therapy. Approximately 5% of all HUS cases have an “atypical” or recurrent course. Atypical HUS is an inadequate term that applies to a heterogeneous group of conditions. We describe this group as non-diarrhoeal (D-), non-EHEC (EHEC-) HUS. Patients in the non-diarrhoeal, non-EHEC, relapsing group are much more likely to exhibit severe hypertension, histological findings of arterial as well as arteriolar disease, chronic and end-stage renal failure. In general, these patients have an alarmingly high risk of graft loss from disease recurrence or thrombosis ranging from 60–100%. Family history is crucial, and where family members have relapsing disease, transplantation is a very high risk procedure (recurrence 100%). Patients with D–HUS need very careful consideration before transplantation, including molecular investigation of complement regulators (and von Willebrandt protease (ADAMTS13) activity, although this goes beyond the scope of this review). Guidelines are accessible under https://doi.org/www.espn.ucwm.ac.uk. On no account should live related donation take place unless the risks of graft loss are understood. International collaboration to identify safer ways of transplanting these challenging patients is urgently needed.

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Acknowledgments

Members of the ESPN study group [16]: N. Besbas (Turkey), H. Karch (Germany), D. Karpman (Sweden), D. Landau (Israel) , C. Loirat (France), W. Proesmans (Belgium), G. Rizzoni (Italy), C.M. Taylor (United Kingdom), N. van de Kar (Netherlands), L.B. Zimmerhackl (Austria). Contributing physicians and hospitals for the rec HUS study [17]: A. Gerber, Ch. Jacobi (UKK Freiburg), W. Rascher, K. Nüsken (UKK Erlangen), R. Beetz (UKK Mainz), M. Konrad (Inselspital Bern), G. Klaus (UKK Marburg), S. Fründ, M. Bulla (UKK Münster), G. Offner (Kinderklinik MHH, Hannover), H. Fehrenbach, D. Kleinert (KK Memmingen), B. Klare, M. Griebel, S. Martini (Krankenhaus München-Schwabing), B. Tönshoff, S. Weber (UKK Heidelberg), J. Misselwitz, U. John (HKI Jena), B. Hoppe, C. Licht (UKK Köln), M. Bald, M. Holder (Olgahospital Stuttgart), W. Radauer (KK Salzburg), R. Urbanek, E. Balzer, T. Müller (AKH Wien), J. Janda, T. Seemann (UKK Prag), M. Kemper, T. Neuhaus (UKK Zürich), E. Sulyok (Pécs), G. Reusz (UKK Budapest), G. Miltenberger (Innsbruck), P. Zipfel, S. Heinen (Jena), M. Kirschfink (Heidelberg), F. Allerberger (Wien, Innsbruck), H. Karch (Münster). Parts of these data were presented at IPTA meeting in Innsbruck 2005 and appeared in abstract form (Scheiring et al.: Pediatric Transplantation 9: Supplement 6:48, 2005). This analysis was the basis of the thesis of Johanna Scheiring (Medical University Innsbruck). This study was supported by a “Förderstipendium” of the Medical University Innsbruck to Johanna Scheiring. This study was supported by ESPN and APN.

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Authors and Affiliations

  1. Univ. Klinik für Kinder-und Jugendheikunde, Innsbruck, Austria

    Lothar Bernd Zimmerhackl, Johanna Scheiring & Friederike Prüfer

  2. Birmingham Children’s Hospital Birmingham, Birmingham, UK

    C. Mark Taylor

  3. Hôpital Robert Debré, Paris, France

    Chantal Loirat

  4. Department of Paediatrics, Medical University, Anichstr. 35, Innsbruck, 6020, Austria

    Lothar Bernd Zimmerhackl

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  1. Lothar Bernd Zimmerhackl
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  2. Johanna Scheiring
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  3. Friederike Prüfer
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  4. C. Mark Taylor
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  5. Chantal Loirat
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Correspondence to Lothar Bernd Zimmerhackl.

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Zimmerhackl, L.B., Scheiring, J., Prüfer, F. et al. Renal transplantation in HUS patients with disorders of complement regulation. Pediatr Nephrol 22, 10–16 (2007). https://doi.org/10.1007/s00467-006-0210-7

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  • DOI: https://doi.org/10.1007/s00467-006-0210-7

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