nach oben

Pediatric Nephrology

Erschienen in:

13.09.2019 | Brief Report

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

verfasst von: Orly Haskin, Yafa Falush, Miriam Davidovits

Erschienen in: Pediatric Nephrology | Ausgabe 12/2019

Einloggen, um Zugang zu erhalten

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.

Nur mit Berechtigung zugänglich

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 368:2169–2181CrossRef

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 87:1061–1073CrossRef

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 89:701–711CrossRef

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 31:15–39CrossRef

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 9:24–29CrossRef

Taylor CM, Chua C, Howie AJ, Risdon RA (2004) Clinico-pathological findings in diarrhea-negative haemolytic uraemic syndrome. Pediatr Nephrol 19:419–425CrossRef

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 94:408–418CrossRef

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 8:554–562CrossRef

Kim JJ, Waller SC, Reid CJ (2012) Eculizumab in atypical haemolytic-uraemic syndrome allows cessation of plasma exchange and dialysis. Clin Kidney J 5:34–36CrossRef

10.

Povey H, Vundru R, Junglee N, Jibani M (2014) Renal recovery with eculizumab in atypical hemolytic uremic syndrome following prolonged dialysis. Clin Nephrol 82:326–331CrossRef

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.

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 7:18–25CrossRef

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 13:1510–1416PubMedPubMedCentral

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 30:603–608CrossRef

Titel: Is eculizumab indicated in patients with atypical hemolytic uremic syndrome already on prolonged dialysis? A case report and review of the literature
verfasst von: Orly Haskin
Yafa Falush
Miriam Davidovits
Publikationsdatum: 13.09.2019
Verlag: Springer Berlin Heidelberg
Erschienen in: Pediatric Nephrology / Ausgabe 12/2019
Print ISSN: 0931-041X
Elektronische ISSN: 1432-198X
DOI: https://doi.org/10.1007/s00467-019-04341-4

Neu im Fachgebiet Pädiatrie

23.04.2024 | Typ-1-Diabetes | Nachrichten

Update Pädiatrie

Bestellen Sie unseren Fach-Newsletter und bleiben Sie gut informiert.

Newsletter bestellen

Live-Webinar: Aktuelle Leitlinien bei Herz-Kreislauf-Erkrankungen

Springer Medizin

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

Abstract

Background

Case-diagnosis/treatment

Conclusion

Neu im Fachgebiet Pädiatrie

Neuer Typ-1-Diabetes bei Kindern am Wochenende eher übersehen

Neue Studienergebnisse zur Myopiekontrolle mit Atropin

Spinale Muskelatrophie: Neugeborenen-Screening lohnt sich

Fünf Dinge, die im Kindernotfall besser zu unterlassen sind

Update Pädiatrie

Live-Webinar: Aktuelle Leitlinien bei Herz-Kreislauf-Erkrankungen

Springer Medizin

Abstract

Background

Case-diagnosis/treatment

Conclusion

Bitte loggen Sie sich ein, um Zugang zu diesem Inhalt zu erhalten

Weitere Artikel der Ausgabe 12/2019

Defining a threshold for tacrolimus intra-patient variability associated with late acute cellular rejection in paediatric kidney transplant recipients

Can childhood obesity influence later chronic kidney disease?

An adolescent with lupus nephritis presenting with fever, lymphadenomegaly, and arthralgia: Questions

Infrequent tacrolimus-induced nephrotoxicity in French patients with steroid-dependent nephrotic syndrome

Hypokalemia in a pediatric patient on continuous renal replacement therapy: Answers

An adolescent with lupus nephritis presenting with fever, lymphadenomegaly, and arthralgia: Answers

Neu im Fachgebiet Pädiatrie

Neuer Typ-1-Diabetes bei Kindern am Wochenende eher übersehen

Neue Studienergebnisse zur Myopiekontrolle mit Atropin

Spinale Muskelatrophie: Neugeborenen-Screening lohnt sich

Fünf Dinge, die im Kindernotfall besser zu unterlassen sind

Update Pädiatrie