Two Cases of Wilson's Disease. |
Dae Young Kim, In Kwyn Park, jong Sik Kim, Kang Ho Kim, Sang Kee Park, Chang Soo Ra |
Department of Peidatrics, College of Medicine, Chosun University, Kwangju, Korea |
Wilson병 2례 |
김대영, 박인규, 김종식, 김강호, 박상기, 나창수 |
조선대학교 부속병원 소아과학교실 |
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Abstract |
Two cases of Wilson’s disease were experienced, which showed symptoms of hepatic dysfunction,
Kayser-Fleischer ring and decreased serum ceruloplasmin and increased urine copper excretion.
Case 1: An 9 years old boy was admitted with abdominal distension and general edema, he had low
serum ceruloplasmin levels (7.0 mg%) and high levels of urinary copper (2020 必g/24 hrs) and increased
transaminase levels (292 U/L of SGOT, 92 U/L of SGPT). Kayser-Fleischer ring was visible, but
neurologic abnormalities did not appear. The patient was treated with D-penicillamine (1.0 gm perday)
and copper rich diet was strictly restricted, however he was not improved and expired on 55th hospital day.
Case 2: This patient was a 10 years old boy, he showed abdominal distention, jaundice, facial edema
and Kayser-Fleischer ring. Serum ceruloplasmin level was reduced (14.2 mg%) and urinary copper
excretion was markedly increased (1470 /zg/24 hrs) and SGOT and SGPT were elevated (258 U/L of
SGOT, 108 U/L of SGPT). Neurologic abnormalities were not observed. D-penicillamine was given
orally (750 mg per day), but there was no evidence of improvement and the patient was discharged
by his parents on 10th hospital day.
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Key Words:
Wilson’s disease, Kayser-Fleischer ring.
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