Management

In all patients with Wilson disease, whether symptomatic or not, consequently implemented treatment is obligatory for maintaining normal life expectancy. The aim of therapy is the elimination of excess body copper and prevention of copper reaccumulation. Treatment should be initiated as soon as the diagnosis is established, and the mainstay remains lifelong pharmacotherapy.1-11-1 Cessation of therapy leads to progression of disease and can even cause acute fulminant liver failure. Thus, the patient's compliance is essential and should be monitored as well as potential side effects of medication.

Chelating agents such as D-penicillamine promote urinary excretion of excess copper. In the initial phase of therapy, penicillamine is usually administered in doses of 1000-1500 mg/day in two divided dosages. Maintenance therapy can be started when 24-hr urinary copper excretion is less than 500 pg. Maintenance dose is usually 750-1000 mg/day administered in two divided dosages. In pediatric patients, 15-25 mg D-penicillamine per kilogram body weight should be administered in two divided dosages. Food inhibits absorption of penicillamine; thus the drug should be taken 1 hr before or 2 hr after meals. D-Penicillamine antagonizes the effect of pyridoxine and daily supplementation with 25 mg is recommended. D-Penicillamine therapy has to be discontinued in 20-30% because of severe side effects including fever, rash, lymphadenopathy, neutropenia, thrombocyto-penia, lupuslike symptoms, nephrotoxicity, Goodpasture syndrome, myasthenia gravis, and polymyositis. It is crucial to realize that these side effects can occur even years after starting initial therapy. Worsening of neurological symptoms has been reported in some patients treated with D-penicillamine.

Trientine (Methylene tetramine dihydrochloride) is an alternative, rather expensive chelator with fewer side effects. Trientine is given at an average daily dose of 750-1500 mg as two to three divided dosages (in children, 15-20 mg/kg body weight). Side effects include iron deficiency anemia because of its chelating action requiring iron supplementation.

Zinc salts have few side effects, and three daily dosages of 100 mg ingested before meals are effective in maintenance therapy (in children, 3-4 mg/kg body weight). Zinc interferes with copper absorption in the gastrointestinal tract and induces intestinal metallothio-neins, which act as endogenous chelators. Zinc salts can be combined with D-penicillamine or trientine. Because of interactions, chelating agents should be administered with some hours delay and not simultaneously with zinc.

Ancillary to pharmacological treatment dietary copper restriction is recommended, but its therapeutic value remains to be determined.

In patients with fulminant hepatic failure or decompensation of chronic liver disease liver transplantation is required. The molecular defect in Wilson disease is cured by a liver graft and no specific therapy in respect to Wilson disease is necessary after transplantation. The neurological course of disease after transplantation is currently under investigation.

Getting Started With Dumbbells

Getting Started With Dumbbells

The use of dumbbells gives you a much more comprehensive strengthening effect because the workout engages your stabilizer muscles, in addition to the muscle you may be pin-pointing. Without all of the belts and artificial stabilizers of a machine, you also engage your core muscles, which are your body's natural stabilizers.

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