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Two Cases of Wilson's Disease.

Journal of the Korean Pediatric Society 1987;30(12):1475-1479.
Published online December 31, 1987.
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
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.
Key Words: Wilson’s disease, Kayser-Fleischer ring.

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