Please take care when eating leftovers and when preparing food with copper utensils🍴
Medicine (full playlist) ►    • Medicine  
Reviewer: Dr. Joe Akin
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This is a case about Wilson's Disease. Wilson's Disease is a genetic disorder with an autosomal recessive inheritance pattern affecting the ATP7b gene on chromosome 13. The inherited genetic defect prevents the correct production of the ATP7b protein, which is a copper transporter, meaning the body's ability to transport copper is impaired. Causing copper to build up. Causing damage to the liver cells by creating reactive oxidative species and forming a sludge in the gallbladder as the acute liver failure in CG causes permanent damage. Autosomal recessive means that in a family pedigree, it can often be seen as "skipping" a generation, as more carriers for Wilson's are introduced into the population.

As the copper spills into the bloodstream, it enters the brain. In normal function, the same protein as found in the liver also transports copper out. But because of the genetic defect, that doesn’t happen. Copper accumulates in the brain, and this metal toxicity begins to damage nerve cells, showing up as motor dysfunction in CG.

Treatment for Wilson's disease when caught early is zinc, which will prevent the body from absorbing copper from daily intake. In acute hypercupremia and liver failure, the only treatment is liver transplant. The qualification of acute hepatic failure is important in placing the patient on the wait list for a liver.

These cases are patients who I, or my colleagues have seen. They are de-identified and many instances have been presented in more depth in an academic setting. These videos are not individual medical advice and are for general educational purposes only. I do not give medical advice over the internet, see your own physician in person for that.

References:

Dr. Akin prepared a Refigure for you to understand more about the disease pathophysiology here: http://bit.ly/wilsondisease

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-Overview of dietary trace minerals - UpToDate
-National Research Council (US) Committee on Copper in Drinking Water. Copper in Drinking Water. Washington (DC): National Academies Press (US); 2000. 5, Health Effects of Excess Copper.
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-Wilson disease Epidemiology and pathogenesis - UpToDate
-Wilson disease Treatment and prognosis - UpToDate
-Wilson disease Diagnostic tests - UpToDate
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-Hepatology. 2008 Jun;47(6):2089-111. PMID 18506894
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-Revised King's College score for liver transplantation in adult patients with Wilson's disease. Liver Transpl. 2007 Jan;13(1):55-61. PMID 17154398
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