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KMID : 0352819960110010127
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Kosin Medical Journal
1996 Volume.11 No. 1 p.127 ~ p.134
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Two cases of Wilson's disease developed in sisters
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Abstract
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Wilson's disease is due to excessive accumulation of copper in the liver, brain, kidneys, and cornea, caused by a defect in billiary excretion of copper.
We have experienced two case of Wilson's disease developed in sisters.
case 1: The jaundice. She had low serum ceruloplasmin level, high urinary copper excretion and jaundice. She had low serum ceruloplasmin level, high urinary copper excretion and increased transaminase level. She died of fulminant
hepatitis
on
2nd hospital day.
case 2: The patient is younger sister of case 1. She was a 7 year old girl. She showed anorexia and abdominal pain. Serum ceruloplasmin and copper level was reduced, and transaminase levels was elevated. We have treated the
patient
with
D-penicillamine, and the blood level of SGOT and SGPT have decreased down to normal level. We reviewed literatures briefly and presented these cases.
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KEYWORD
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