Atypical hemolytic uremic syndrome and eculizumab therapy in children / 소아과
Korean Journal of Pediatrics
; : 37-42, 2018.
Article
ي En
| WPRIM
| ID: wpr-741362
المكتبة المسؤولة:
WPRO
ABSTRACT
Hemolytic uremic syndrome (HUS) is often encountered in children with acute kidney injury. Besides the well-known shiga toxin-producing Escherichia coli-associated HUS, atypical HUS (aHUS) caused by genetic complement dysregulation has been studied recently. aHUS is a rare, chronic, and devastating disorder that progressively damages systemic organs, resulting in stroke, end-stage renal disease, and death. The traditional treatment for aHUS is mainly plasmapheresis or plasma infusion; however, many children with aHUS will progress to chronic kidney disease despite plasma therapy. Eculizumab is a newly developed biologic that blocks the terminal complement pathway and has been successfully used in the treatment of aHUS. Currently, several guidelines for aHUS, including the Korean guideline, recommend eculizumab as the first-line therapy in children with aHUS. Moreover, life-long eculizumab therapy is generally recommended. Further studies on discontinuation of eculizumab are needed.
Key words
النص الكامل:
1
الفهرس:
WPRIM
الموضوع الرئيسي:
Plasma
/
Complement System Proteins
/
Plasmapheresis
/
Stroke
/
Escherichia
/
Renal Insufficiency, Chronic
/
Acute Kidney Injury
/
Atypical Hemolytic Uremic Syndrome
/
Hemolytic-Uremic Syndrome
/
Kidney Failure, Chronic
نوع الدراسة:
Guideline
المحددات:
Child
/
Humans
اللغة:
En
مجلة:
Korean Journal of Pediatrics
السنة:
2018
نوع:
Article