چكيده لاتين :
Objective
Wilson disease (WD) is an inherited copper metabolism dysfunction disease
characterized by cirrhosis and eNS findings. Wilson diseaseis important because
it is fatal if not recognized and treated. Our Goal of study is to investigate the
clinical signsand symptoms, lab results and other relevant matters in our patients
in order to obtain a better understanding of this potentially lethal disease in our
country.
Materials & Methods
We have evaluated 21 cases of children with Wilson disease who were referred
to Loghman and Imam Hussein Hospital between years 1998-2005. The mean
age of our patients was 9 years.
Results
The presenting symptom was ascites and extremity edema in 6(28.5%) patients,
behavioral changes or neurologi cal signs in 5 (24%) simultaneous Ascites and
icter in 9(43%) patients and in one patient the presenting manifestation was
hemolytic anemia(4.8%).One of our patients died becauseof fulminant hepatitis
in the course of admission(4.8%).
Conclusion
we showed in this study that Wilson disease can be presented by a manifold
symptoms in children and adolescence .Having a good concept of these
symptoms and high clinical suspicious are required to diagnose this potentially
lethal disease at the proper time in order to decrease the potential adverse
effects of the disease especially the neuropsychiatric damages significantly.
