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Cuprimine for Wilson's disease: How does it work? How is it consumed? Evidence? Scientific Studies? Who shouldn't have it? Dosages?

Wilson's disease is an autosomal recessive genetic disorder, which is caused by mutations in a gene called ATP7B. This gene is the gene responsible for regulating the transport of copper and excretion of it outside the body. When this gene has mutations, it leads to copper accumulation inside the body, beginning in places like the liver, and eventually spreading to the brain and kidneys, causing symptoms such as: Hepatitis, cirrhosis, liver failure, tremors, difficulties in speaking, depression, psychosis, personality changes and even copper deposits in the cornea in the shape of rings, and some renal problems. 

To treat Wilson's disease, cuprimine is administered in patients. It is a chelating agent, meaning that it is a substance which easily bonds with metal ions to form complexes. It bonds to copper ions, turning into a stable complex; this lets it be excreted through urine much more easily. By binding to the ions, cuprimine consumption results in a lower copper concentration in the body, preventing further damage to the organs caused by the metal. 

Cuprimine is consumed orally, usually with an hour's worth of a gap between consumption of food, since some foods interrupt the absorption of the medicine into the bloodstream. It is usually administered in dosages of 0.25-0.5g a day, and is then increased up to 1g-1.5g based on the patient's condition. 

Individuals with a known allergy to medicines like penicillamine or any of the components within it are strongly advised not to take this medication. Along with those, people who are pregnant are not recommended to consume cuprimine unless clearly needed, since it may harm the fetus. Most importantly, people who do not consult a medical professional are strongly advised not to take this medication since it has some serious effects on blood osmolarity, and if it is not maintained appropriately, it can cause strong repercussions. 

Common side effects of the medicine include, nausea, vomiting, rash, loss of appetite, taste alteration, etc. In worst case scenarios, it also manifests as proteinuria, nephrotic syndrome or suppression of the brain marrow. 

However, it is important to note the positive effects of Cuprimine on those with Wilson's disease, as supported by the study titled 'Penicillamine in the treatment of Wilson's Disease: A review of long-term outcomes" which evaluted the efficacy of administering cuprimine in patients with the disease, and found that the medication effectively reduces copper levels, thus supporting liver function, neurological development and so much more. 

However

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