دوره 4، شماره 4 - ( 9-1394 )                   جلد 4 شماره 4 صفحات 347-344 | برگشت به فهرست نسخه ها

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Aliasgharpour M. A review on copper, ceruloplasmin and wilson's disease. J Emerg Health Care 2015; 4 (4) :344-347
URL: http://intjmi.com/article-1-190-fa.html
A review on copper, ceruloplasmin and wilson's disease. . 1394; 4 (4) :344-347

URL: http://intjmi.com/article-1-190-fa.html


چکیده:   (10996 مشاهده)

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.

     
نوع مطالعه: مروری | موضوع مقاله: عمومى

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