Warning: fopen(/home/virtual/pediatrics/journal/upload/ip_log/ip_log_2024-03.txt) [function.fopen]: failed to open stream: Permission denied in /home/virtual/pediatrics/journal/ip_info/view_data.php on line 82

Warning: fwrite(): supplied argument is not a valid stream resource in /home/virtual/pediatrics/journal/ip_info/view_data.php on line 83
Two Cases of Wilson's Disease.

Journal of the Korean Pediatric Society 1986;29(3):336-341.
Published online March 31, 1986.
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


METRICS Graph View
  • 920 View
  • 14 Download