A Detail Insight of Wilson’s Disease and its Treatment
Abstract
Wilson disease is a rare inherited autosomal recessive genetic disorder caused by abnormal copper accumulation in the body, particularly involving the brain, liver, eyes, and other vital organs. This is also known as hepatolenticular degeneration. It primarily affects the liver and basal ganglia of the brain, but it can affect other vital organs too. It results from a mutation in the gene encoding the Wilson disease protein (ATP7B). It results from a mutation in the gene encoding the Wilson disease protein (ATP7B). A healthy body excretes extra copper through urine after the liver filters it out. Wilson's disease prevents the liver from effectively removing the excess copper. The extra copper then builds up in organs such as the brain, liver, and eyes; which is the reason why symptoms are usually related to the brain and liver. One of the 23 chromosomes in humans, chromosome 13, has a gene that causes Wilson's disease. For six months, a 12-year-old kid with Wilson's disease complained of speech problems, swallowing issues, and general stiffness. About 97 percent of those with Wilson's illness have K-F rings. Vomiting, weakness, ascites, yellowing of the skin, and brain or neurological symptoms like tremors, muscle stiffness, difficulty speaking, personality changes, or visual hallucinations are only a few of the symptoms associated with the liver. Sunflower cataracts are visible in 1 in 5 patients with Wilson's illness. One in 90 people in the general population are carriers of Wilson's illness. A liver biopsy, clinical evaluation, blood testing, urine tests, and a challenging diagnosis are all required. The family members of persons with the condition may be screened via genetic testing. For Wilson's disease to be stopped in its tracks, early detection is essential. Treatment can be done by both ayurvedic remedies using herbs and allopathy using drugs with specific target sites important for managing Wilson disease. WD can be treated with Copper chelating agents and Herbs act as a supporting treatment. Some drugs are still in the clinical trial phase.
Keywords: Kayser-Fleischer rings, Wilson disease (WD), ALXN1840, d-Penicillinase, Vatavyadhi.
Keywords:
Kayser-Fleischer rings, Wilson disease(WD), ALXN1840, d-Penicillinase, VatavyadhiDOI
https://doi.org/10.22270/jddt.v13i4.5982References
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