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2014 ; 7
(ä): 231-9
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Management of hemolytic-uremic syndrome in children
#MMPMID24966691
Grisaru S
Int J Nephrol Renovasc Dis
2014[]; 7
(ä): 231-9
PMID24966691
show ga
Acute renal failure associated with a fulminant, life-threatening systemic
disease is rare in previously healthy young children; however, when it occurs,
the most common cause is hemolytic-uremic syndrome (HUS). In most cases (90%),
this abrupt and devastating illness is a result of ingestion of food or drink
contaminated with pathogens that produce very potent toxins. Currently, there are
no proven treatment options that can directly inactivate the toxin or effectively
interfere with the cascade of destructive events triggered by the toxin once it
gains access to the bloodstream and binds its receptor. However, HUS is
self-limited, and effective supportive management during the acute phase is
proven to be a life saver for children affected by HUS. A minority of childhood
HUS cases, approximately 5%, are caused by various genetic mutations causing
uncontrolled activation of the complement system. These children, who used to
have a poor prognosis leading to end-stage renal disease, now have access to
exciting new treatment options that can preserve kidney function and avoid
disease recurrences. This review provides a summary of the current knowledge on
the epidemiology, pathophysiology, and clinical presentation of childhood HUS,
focusing on a practical approach to best management measures.