Drug of choice in pregnant women with Wilson&;s disease?
Wilson's disease is an inherited disorder of copper homeostasis with failure to excrete excess amounts of copper, leading to an accumulation in the liver. Wilson's disease typically affects adolescents and young adults. Prompt diagnosis before end-stage manifestations become irreversible can lead to significant clinical improvement.Diagnosis requires determination of ceruloplasmin levels, which are low; 24-h urine copper levels, which are elevated; typical physical examination findings, including Kayser-Fleischer corneal rings; and characteristic liver biopsy findings.Treatment consists of copper-chelating medications such as D-penicillamine and trientine. A role for zinc acetate during pregnancy has also beenestablished. zinc is the first line of management in pregnancy, pediatric age group, presymptomatic patients, and in maintenance therapy.Medical treatment is lifelong, and severe relapses leading to liver failure and death can occur with cessation of therapy.Liver transplantation is curative with respect to the underlying metabolic defect and restores the normal phenotype with respect to copper homeostasis.Ref: Harrison&;s Principles of Internal Medicine; 19th edition; Chapter 367e; Genetic, Metabolic, and Infiltrative Diseases Affecting the Liver
Zinc
Pencillamine
Molybdenum
Trientine
Nutrition