| Two Cases of Wilson's Disease. |
|
Myung Ho Kang, Byoung Tae Kim, Jae Ock Park, Sang Jhoo Lee |
|
Department of Pediatrics, College of Medicine, Soon Chun Hyang University |
| Wilson 씨병 2 례 |
|
강명호, 김병태, 박재옥, 이상주 |
|
순천향대학 의학부 소아과학교실 |
|
|
|
|
| Abstract |
|
The authors experienced two cases of hepatic from of Wilson* s disease, which diagnosis were confirmed by family history, their characteristic clinical manifestations with hepatic symptoms, Kayser-Fleischer ring and laboratory data with decreased serum ceruloplasmin and copper level and increased urine copper excretion.
Case 1： An 8 8/12 year-old boy with chief complaints of abdominal distension and jaundice showed Kayser-Fleischer ring on the corneal margin of both eyes. The laboratory findings showed low level of serum ceruloplasmin(3.0 mg%) and high level of urine copper excretion(945.5 ug/day). Of the three siblings,
a 4-year-old younger sister showed 3.5 mg% of serum ceruloplasmin, 149.7 ug/day of urine copper excretion
and a 2-year-old younger brother showed 103.0 unit of SGOT, 125.5 unit of SGPT, 6.8 mg% of serum ceruloplasmin, 22.8ug/day of urine copper excretion. The patient was given 500 mg of D-penicillamine
and low copper diet with improvement of clinical and laboratory findings. Case 2- A 13 11/12-year-old boy with chief complaints of abdominal distension and painful mass on left upper abdomen showed icteric sclerae, Kayser-Fleischer ring, splenomegaly and pitting edema on the lower extremities. The laboratory findings showed low level of serum ceruloplasmin(7.0 mg%) and serum copper(43 ug%) and high level of urine copper excretion
(327.7 ug/day). Splenoportography revealed portal hypertesion(438 mmH20), marked collateral circulation (paraumbilical vein, superior & inferior mesenteric vein, coronary and esophageal veins) and small size of liver and liver biopsy findings revealed active macro and micro-nodular cirrhosis. After splenectomy with splenorenal shunt operation, 250 mg of D-penicillamine 4 times daily and low copper diet were given, resulting in clinical
improvement. The authors presented two cases of Wilson’s disease and reviewed the referential literatures.
|
| Key Words:
Wilson’s disease, Kayser-Fleischer ring, ceruloplasmin, Copper, D-penicillamine |
|
PDF Links
Download Citation
