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Wilson’s Disease in Children

A genetic illness called Wilson’s disease (WD) occurs
when the ATP7B protein fails, causing copper to build
up in the liver, brain, and other organs. Patients typically
present with liver disease, neuropsychiatric symptoms,
ophthalmologic abnormalities, Kayser-Fleischer corneal
rings.1-3 Examples of hepatic disease include clinically
asymptomatic cases with mild liver enzyme elevation
and steatosis, as well as clinically overt advanced
chronic liver disease (ACLD) with varices, splanchnic
collaterals, splenomegaly, or hepatic decompensation.
Fulminant hepatic failure (usually caused by ACLD) with
Coombs’ negative hemolytic anemia is rare.4

 

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