Belgian Journal of Paediatrics
Early recognition and access to terminal complement blockers in patients with atypical HUS significantly improves their outcome: a case report
Vol. 25 No. 2 (2023), Case reports
Vol. 25 No. 2 (2023)

Early recognition and access to terminal complement blockers in patients with atypical HUS significantly improves their outcome: a case report

Case reports
Published 2023-07-10
Amelien Vanacker
Department of Paediatrics, Ghent University Hospital, Ghent, Belgium
https://orcid.org/0000-0003-1027-9167
Nathalie Segers
Department of Paediatric Nephrology, Paola’s Children’s Hospital Antwerp, Belgium
An Bael
Department of Paediatric Nephrology, Paola’s Children’s Hospital Antwerp, Belgium
https://orcid.org/0000-0003-2819-708X
Muriel Koninckx
Department of Paediatric Intensive Care, Paola’s Children’s Hospital Antwerp, Belgium
Ann Raes
Department of Paediatric Nephrology, member of the European Reference Network for Rare Kidney Diseases (ERKNet), Ghent University Hospital, Ghent, Belgium
https://orcid.org/0000-0001-7809-2505
Johan Vande Walle
Department of Paediatric Nephrology, member of the European Reference Network for Rare Kidney Diseases (ERKNet), Ghent University Hospital, Ghent, Belgium
Joke Dehoorne
Department of Paediatric Nephrology, member of the European Reference Network for Rare Kidney Diseases (ERKNet), Ghent University Hospital, Ghent, Belgium
Agnieszka Prytula
Department of Paediatric Nephrology, member of the European Reference Network for Rare Kidney Diseases (ERKNet), Ghent University Hospital, Ghent, Belgium
https://orcid.org/0000-0001-9551-8758
Lien Dossche
Department of Paediatric Nephrology, member of the European Reference Network for Rare Kidney Diseases (ERKNet), Ghent University Hospital, Ghent, Belgium
https://orcid.org/0000-0003-1030-9848
Thomas Renson
Department of Paediatric Nephrology, member of the European Reference Network for Rare Kidney Diseases (ERKNet), Ghent University Hospital, Ghent, Belgium
https://orcid.org/0000-0002-5503-000X
Evelien Snauwaert
Department of Paediatric Nephrology, member of the European Reference Network for Rare Kidney Diseases (ERKNet), Ghent University Hospital, Ghent, Belgium
https://orcid.org/0000-0003-2660-7460
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Keywords

thrombotic microangiopathy
atypical haemolytic uremic syndrome
eculizumab
anti-factor H antibodies
case report

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How to Cite

Vanacker, A., Segers, N., Bael, A., Koninckx, M., Raes, A., Vande Walle, J., Dehoorne, J., Prytula, A., Dossche, L., Renson, T., & Snauwaert, E. (2023). Early recognition and access to terminal complement blockers in patients with atypical HUS significantly improves their outcome: a case report. Belgian Journal of Paediatrics, 25(2), 129–132. Retrieved from http://belgjpaediatrics.com/index.php/bjp/article/view/62
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Abstract

A 7-year-old boy presented with fever, pruritus and petechiae. Laboratory analysis revealed haemolytic anaemia with presence of schistocytes, thrombocytopenia and acute kidney injury, diagnostic for a thrombotic microangiopathy (TMA). Given the severe clinical presentation, the lack of evidence for verotoxin positive TMA and a normal ADAMTS13, treatment with eculizumab was started. This resulted in a complete resolution of the TMA course with recovery of the kidney injury. Workup revealed high titre of anti-factor H antibodies, diagnostic for an atypical haemolytic uremic syndrome (aHUS). Early recognition and treatment of TMA is essential to optimize the outcome of these patients.

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