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a review on copper, ceruloplasmin and wilson's disease
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نویسنده
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aliasgharpour mehri
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منبع
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international journal of medical investigation - 2015 - دوره : 4 - شماره : 4 - صفحه:344 -347
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چکیده
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Objective: copper as an essential trace element plays a vital function in biochemical systems. its reduction or raise under/above a certain limit results consistently in disturbed physiologically functions. wilson's disease is an auto-somal recessive copper transport disorder and clinical manifestations in adults are presentation of hepatic or neuropsychiatric symptoms. however, in children clinical symptoms may be absent that makes the diagnosis more difficult than in adults. methods: many different literatures on the subject matter from different database sources were reviewed and used . results: clinical diagnosis for wilson's disease includes screening tests such as determination of serum ceruloplasmin level in addition to the slit lamp examination for kayser fieischer rings to decide on or decline further testing such as 24 hours urinary copper excretion and liver biopsy tests. because starting an early treatment is the most effective plan to control the condition, screening is also recommended for people who have relatives with this disorder . furthermore, lifelong treatment by copper chelators or zinc is mandatory for patients regardless of symptoms. conclusion: wilson's disease results from defective function of a copper transporting protein called p-type atpase (atp7b). factors influencing its function may be genetic, nutritional or environmental. early diagnosis of wilson's disease is important not only to reduce or even prevent organ damage but also to ensure that patients may start on proper treatment plan.
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کلیدواژه
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trace elements ,copper ,ceruloplasmin ,diagnosis ,prognosis
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آدرس
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ministry of health & medical educations, reference health laboratory research center, dept of clinical biochemistry laboratory, iran
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پست الکترونیکی
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m.asgharpour@ymail.com
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Authors
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