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Wilson's disease in Albania
1University Hospital Center of Tirana, Albania.
Panminerva Medica
|March 1, 1995
Summary
This study characterizes Wilson's disease (WD) in Albania, finding a high incidence of liver damage and hypersplenism, which occurs at younger ages in WD patients compared to controls. Platelet counts were significantly lower in WD patients.
Area of Science:
- Hepatology
- Genetics
- Internal Medicine
Background:
- Wilson's disease (WD) is a rare genetic disorder of copper metabolism.
- Albania has a documented population of WD patients requiring clinical characterization.
- Understanding the clinical spectrum of WD is crucial for early diagnosis and management.
Purpose of the Study:
- To describe the epidemiological and clinical characteristics of Wilson's disease in Albania.
- To compare specific clinical and laboratory findings in WD patients with a control group.
Main Methods:
- Retrospective analysis of 40 diagnosed Wilson's disease patients in Albania.
- Clinical examination for Kayser-Fleischer rings, neurological, and mental disturbances.
- Assessment of liver damage, hypersplenism, portal hypertension, and hematological parameters.
- Comparison with a control group of 60 patients with alcoholic or viral cirrhosis.
Main Results:
- The study identified 40 WD patients (24 males, 16 females) with an average age of 18.8 years.
- Kayser-Fleischer rings (75%), neurological (60%), and mental disturbances (27.5%) were common. All patients had liver damage.
- Hypersplenism was observed in 35% of WD patients, occurring at significantly younger ages than in controls (p < 0.0001). WD patients had lower platelets (p < 0.05) and higher leucocytes compared to controls.
- Portal hypertension affected 42.5% of cases, with 35.3% experiencing gastrointestinal hemorrhage. Congenital anomalies and parotid gland hypertrophy were also noted.
Conclusions:
- Wilson's disease in Albania presents with significant hepatic and extrahepatic manifestations, including a high rate of hypersplenism in younger individuals.
- The clinical and hematological findings highlight the systemic impact of WD and the importance of comparative analysis with other liver diseases.
- Early detection and management strategies are essential, considering the observed complications like portal hypertension and gastrointestinal bleeding.