A Multicenter Study Evaluating the Discontinuation of Eculizumab Therapy in Children with Atypical Hemolytic Uremic Syndrome.

Saeed AlZabali,Sawsan AlBatati,Khawla Rahim,Hassan Faqeehi, Abubaker Osman,Abdulaziz Bamhraz,Mohammed A Saleh,Jameela A Kari, Majed Aloufi,Loai Eid,Haydar Nasser,Abubakr Imam,Entesar AlHammadi,Omar Alkandari,Mohammed Al Riyami,Sidharth Sethi,Christoph Licht,Khalid A Alhasan,Abdulkarim AlAnazi

Children (Basel, Switzerland)（2022）

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摘要

The discontinuation of eculizumab in patients with aHUS is not without risk; it can result in HUS recurrence. Eculizumab discontinuation can be performed with close monitoring of the patients. It is essential to assess risk the factors for relapse before eculizumab discontinuation, in particular in children with a positive complement variant and any degree of residual CKD, as HUS relapse may lead to additional loss of kidney function. Resuming eculizumab promptly after relapse is effective in most patients.

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关键词

HUS relapse,atypical hemolytic uremic syndrome,discontinuation of eculizumab,microangiopathy,thrombotic

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