Background: Wilson disease is a genetic disorder in which copper accumulates in the liver and brain in excess of normal metabolic needs. The accumulation is based on an inher ited defect in the hepatic biliary excretion of copper. Left untreated, patients with Wilson disease have an unfavourable outcome. Case report: Describe a case of decompensated cirrhosis due to Wilson disease at Cho Ray hospital. A 17 year-old female has symptoms of hemolytic anemia, arthritis and hepatitis more than one year. On physical examination, the patient had jaundice and ascites. Kayser-Fleischer rings were positive. The laboratory tests showed: RBC count, platelet count and albumin level much decreased (2.2 T/L, 73G/L, 2.1g/dL respectively). Ceruloplasmin level was very low (2.4 mg/dL). Abdominal ultrasound and fibroscan revealed various pathological patterns such as chronic hepatitis, decompensated cirrhosis. The patient received trolovol (D-penicillamine) 900mg/day and returned to the hospital for laboratory assessment of biochemical and hematologic values. At the end of 12 months, the disease was stabilized, every biochemical and hematologic value was good. The cirrhosis has regressed. Conclusion: The patient with decompensated cirrhosis due to Wison disease was successfully treated in the hospital.
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