All issues > Volume 30(12); 1987

Original Article

J Korean Pediatr Soc. 1987;30(12):1475-1479. Published online December 31, 1987.

Two Cases of Wilson's Disease.

Dae Young Kim¹, In Kwyn Park¹, jong Sik Kim¹, Kang Ho Kim¹, Sang Kee Park¹, Chang Soo Ra¹

¹Department of Peidatrics, College of Medicine, Chosun University, Kwangju, Korea

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.

Keywords :Wilson’s disease; Kayser-Fleischer ring.