SURGICAL TREATMENT IN PATIENTS WITH WILSON’S DISEASE

Abstract

Background: Wilson’s disease is an autosomal recessive disorder due to mutation in ATB 7B gene, which is a membrane-bound copper-transporting ATPase. The impaired excretion of copper results in an increase in serum levels and accumulation of copper in the body. Various signs can be observed in liver, nervous system, kidneys, eyes, heart. There are also changes in blood chemistry. Objective: A forty-six-year-old female patient presents with Wilson’s disease in the treatment rooms at the Faculty of Dentistry in Plovdiv. She needs complete dental care. She has also had an ischaemic stroke. Methods and results: As the treatment plan started with extraction, of teeth under local anesthesia, a complete blood count and INR test were performed. Oral mucosa and bone were obtained by biopsy for evidence of pigments in these tissues. The biopsy results showed accumulation of copper in them as well. Conclusion: It’s a rare disorder, approximately 1 in 40 000, severely affecting the liver and nervous system. It requires in-depth analysis and discussion over the clinical and paraclinical approach with a view to avoid possible complications and achieve the desired results.

Keywords

• morbus Wilson
• INR
• hemorrhagic stroke
• Kayser-Fleischer ring