Wilson_disease by pengxiuhui

VIEWS: 150 PAGES: 11

									                Treatment


Penicillamine
Trientine
Ammonium tetrathimolybdate
Zinc
Vitamin E
Diet
Liver Transplantation
                       Penicillamine
•   Mode of Action
    General chelator Induces cupruria

•   Dose
    Adult : 1-1.5 g/day (maximum 2 g) in 2-3 doses
             Maintenance dose: 0.75-1 g/day in 2 doses
    Child : 20 mg/kg/day (maximum 35 mg/kg/day) in 2-3 doses

•   Side effects
    Fever, rash, proteinuria, lupuslike reaction
    Aplastic anemia, Leukopenia, Thrombocytopenia
    Nephrotic syndrome
    Degenerative changes in skin
    Elastosis perforans serpingosa
    Serous retinitis
    Hepatotoxicity
                         Trientine
• Mode of Action
  General chelator Induces cupruria


• Dose
  Adult : 0.75-1.25 g/day (maximum 2 g) in 2-3 doses
  child : 20 mg/kg/day (maximum 1.5 g) in 2-3 doses
  Maintenance dose: same


• Side effects
  Gastritis
  Aplastic anemia rare
  Sideroblastic anemia
   Ammonium Tetrathiomolybdate

• Mode of Action
  Chelator, blocks copper absorption

• Side effects
  Anemia; neutropenia
  Hepatotoxicity


• Experimental in the United States and Canada
                          Zinc
• Mode of Action
 Metallothionein inducer, blocks intestinal absorption of
 copper
• Dose
  Zinc acetate
    Adult : 50 mg elemental zinc, 3 times/day
    Child (5-12yrs) : 25 mg elemental zinc, 3 times/day
  Zinc sulfate (elemental Zinc content; 23%):
    300-1200 mg of zinc sulfate/day in 3 doses
• Side effects
  Gastritis; biochemical pancreatitis
  Zinc accumulation Possible changes in immune function
               Vitamin E / Diet

• Vitamin E
  reducing toxic concentrations of copper in the liver
  -> prevent oxidant damage to mitochondria, which can be
 reduced


• Diet
 Avoidance of Some foods(eg, chocolate, liver, nuts,
 mushrooms, and shellfish)
 - contain high concentrations of copper
  Clinical Presentation and
Management of Wilson Disease




         서정기. 대한간학회지. 2006;12(3):333-363
    Recommended Anticopper Drugs
    for Wilson Disease by George J. Brewer
       Disease Status                   First Choice          Second Choice
Initial hepatic
 Hepatitis or cirrhosis          Zinc                  Trientine
without decompensation
 Hepatic decompensation
   Mild                          Trientine and zinc    Penicillamine and zinc

   Moderate                      Trientine and zinc    Hepatic transplantation

   Severe                        Hepatic               Trientine and zine
                                 transplantation
Initial neurologic/psychiatric   Tetrathiomolybdate    Zinc
                                 and zinc
Maintenance                      Zinc                  Trientine
Presymptomatic                   Zinc                  Trientine
Pediatric                        Zinc                  Trientine
Pregnant                         Zinc                  Trientine


    S. Fauci et al. Harrison/s Principle of Internal Medicine, 17th Edition
   Recommendation of Treatment
• Initial treatment for symptomatic patients should include a
  chelating agent (D-penicillamine or trientine). Trientine may
  be better tolerated

• Patients should avoid intake of foods and water with high
  concentrations of copper, especially during the first year of
  treatment

• Treatment of presymptomatic patients or those on
  maintenance therapy can be accomplished with a chelating
  agent or with zinc. Trientine may be better tolerated

                   Eve A. Roberts and Michael L. Schilsky. Hepatology
                   2008;47(6):2089-2111
   Recommendation of Treatment
• Patients with acute liver failure due to WD should be referred
  for and treated with liver transplantation immediately

• Patients with decompensated cirrhosis unresponsive to
  chelation treatment should be evaluated promptly for liver
  transplantation

• Treatment for WD should be continued during pregnancy,
  but dosage reduction is advisable for D-penicillamine and
  trientine

• Treatment is lifelong and should not be discontinued, unless
  a liver transplant has been performed

                   Eve A. Roberts and Michael L. Schilsky. Hepatology
                   2008;47(6):2089-2111
 Monitoring of Medical Treatment

• During chelation therapy
   Urine copper : 200-500 μg/24 h
   Nonceruloplasmin copper <20 μg/day


• During zinc therapy
   Urine copper <75 μg/24 h
   Urine Zinc >1,000 μg/24 h
   Nonceruloplasmin copper <20 μg/day

								
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