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Is eculizumab indicated in patients with atypical hemolytic uremic syndrome already on prolonged dialysis? A case report and review of the literature

  • Orly HaskinEmail author
  • Yafa Falush
  • Miriam Davidovits
Brief Report

Abstract

Background

Eculizumab has caused a revolution in the treatment and prognosis of atypical hemolytic uremic syndrome. Early initiation of treatment is recommended to increase chances of renal recovery.

Case-diagnosis/treatment

We describe a boy with atypical hemolytic uremic syndrome who started eculizumab therapy after being on dialysis for 4.5 months, with complete anuria. With treatment, he was weaned off dialysis.

Conclusion

We review the evidence in the literature and discuss the possible mechanism by which eculizumab induces renal recovery even in patients already on prolonged dialysis. This case report highlights the importance of a treatment trial with eculizumab, even in patients already on prolonged dialysis.

Keywords

Child aHUS Eculizumab Dialysis 

Notes

Compliance with ethical standards

Conflict of interest

The authors declare that they have no conflicts of interest.

Supplementary material

467_2019_4341_MOESM1_ESM.docx (236 kb)
Supplementary Figure 1 Clinical course of the patient. During a 15-week period, the patient received 9 doses of eculizumab; his hemoglobin (Hb) level increased and his creatinine (Cr) level decreased. (DOCX 235 kb)

References

  1. 1.
    Legendre CM, Licht C, Muus P, Greenbaum LA, Babu S, Bedrosian C, Bingham C, Cohen DJ, Delmas Y, Douglas K, Eitner F, Feldkamp T, Fouque D, Furman RR, Gaber O, Herthelius M, Hourmant M, Karpman D, Lebranchu Y, Mariat C, Menne J, Moulin B, Nürnberger J, Ogawa M, Remuzzi G, Richard T, Sberro-Soussan R, Severino B, Sheerin NS, Trivelli A, Zimmerhackl LB, Goodship T, Loirat C (2013) Terminal complement inhibitor eculizumab in atypical hemolytic-uremic syndrome. N Engl J Med 368:2169–2181CrossRefGoogle Scholar
  2. 2.
    Licht C, Greenbaum LA, Muus P, Babu S, Bedrosian CL, Cohen DJ, Delmas Y, Douglas K, Furman RR, Gaber OA, Goodship T, Herthelius M, Hourmant M, Legendre CM, Remuzzi G, Sheerin N, Trivelli A, Loirat C (2015) Efficacy and safety of eculizumab in atypical hemolytic uremic syndrome from 2-year extension of phase 2 studies. Kidney Int 87:1061–1073CrossRefGoogle Scholar
  3. 3.
    Greenbaum LA, Fila M, Ardissino G, Al-Akash SI, Evans J, Henning P, Lieberman KV, Maringhini S, Pape L, Rees L, van de Kar NCAJ, Vande Walle J, Ogawa M, Bedrosian CL, Licht C (2016) Eculizumab is a safe and effective treatment in pediatric patients with atypical hemolytic uremic syndrome. Kidney Int 89:701–711CrossRefGoogle Scholar
  4. 4.
    Loirat C, Fakhouri F, Ariceta G, Besbas N, Bitzan M, Bejerre A, Coppo R, Emma F, Johnson S, Karpman D, Landau D, Langman CB, Lapeyraque AL, Licht C, Nester C, Pecoraro C, Riedl M, van de Kar NCAJ, Van de Walle J, Vivarelli M, Frémeaux-Bacchi V, HUS International (2016) An international consensus approach to the management of atypical hemolytic uremic syndrome in children. Pediatr Nephrol 31:15–39CrossRefGoogle Scholar
  5. 5.
    Renaud C, Niaudet P, Gagnadoux MF, Broyer M, Habib R (1995) Haemolytic uremic syndrome: prognostic factors in children over 3 years of age. Pediatr Nephrol 9:24–29CrossRefGoogle Scholar
  6. 6.
    Taylor CM, Chua C, Howie AJ, Risdon RA (2004) Clinico-pathological findings in diarrhea-negative haemolytic uraemic syndrome. Pediatr Nephrol 19:419–425CrossRefGoogle Scholar
  7. 7.
    Schaefer F, Ardissino G, Ariceta G, Fakhouri F, Scully M, Isbel N, Lommele A, Kupelian V, Gasteyger C, Greenbaum LA, Johnson S, Ogawa M, Licht C, Walle JV, Fremeaux-Bacchi V, Global aHUS Registry (2018) Clinical and genetic predictors of atypical hemolytic uremic syndrome phenotype and outcome. Kidney Int 94:408–418CrossRefGoogle Scholar
  8. 8.
    Fremeaux-Bacchi V, Fakhouri F, Garnier A, Bienaime F, Dragon-Durey MA, Ngo S, Moulin B, Servais A, Provot F, Rostaing L, Burtey S, Niaudet P, Deschenes G, Lebranchu Y, Zuber J, Loirat C (2013) Genetics and outcome of atypical hemolytic uremic syndrome: a nationwide French series comparing children and adults. Clin J Am Soc Nephrol 8:554–562CrossRefGoogle Scholar
  9. 9.
    Kim JJ, Waller SC, Reid CJ (2012) Eculizumab in atypical haemolytic-uraemic syndrome allows cessation of plasma exchange and dialysis. Clin Kidney J 5:34–36CrossRefGoogle Scholar
  10. 10.
    Povey H, Vundru R, Junglee N, Jibani M (2014) Renal recovery with eculizumab in atypical hemolytic uremic syndrome following prolonged dialysis. Clin Nephrol 82:326–331CrossRefGoogle Scholar
  11. 11.
    Emirova K, Volokhina E, Tolstova E, Van den Heuvel B (2016) Recovery of renal function after long-term dialysis and resolution of cardiomyopathy in a patient with aHUS receiving eculizumab. BMJ Case Rep.  https://doi.org/10.1136/bcr-2015-213928
  12. 12.
    Valente RA, Rodriguez GEG, Marcote YG, Diaz MF, Mosquera VB, Garcia DN, Martinez EC, Rodriguez CD (2017) Discontinuation of peritoneal dialysis after late initiation of Eculizumab in a case of familial atypical hemolytic-uremic syndrome: a case report. Case Rep Nephrol Dial 7:18–25CrossRefGoogle Scholar
  13. 13.
    Bonthuis M, Harambat J, Berard E, Cransberg K, Duzova A, Garneata L, Herthelius M, Lungu AC, Jahnukainen T, Kaltenegger L, Ariceta G, Maurer E, Palsson R, Sinha MD, Testa S, Groothoff JW, Jager KJ, ESPN/ERA-EDTA Registry (2018) Recovery of kidney function in children treated with maintenance dialysis. Clin J Am Soc Nephrol 13:1510–1416Google Scholar
  14. 14.
    Ohta T, Urayama K, Tada Y, Furue T, Imai S, Matsubara K, Onon H, Sakano T, Jinno K, Yoshida Y, Miyata T, Fujimura Y (2015) Eculizumab in the treatment of atypical hemolytic uremic syndrome in an infant leads to cessation of peritoneal dialysis and improvement of severe hypertension. Pediatr Nephrol 30:603–608CrossRefGoogle Scholar

Copyright information

© IPNA 2019

Authors and Affiliations

  1. 1.Department of Pediatric NephrolgySchneider Children’s Medical Center of IsraelPetah TikvaIsrael
  2. 2.Sackler Faculty of MedicineTel Aviv UniversityTel AvivIsrael

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