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trientine hydrochloride capsules (generic)Medica

Wilson’s disease (hepatolenticular degeneration)

Initial criteria

  • Diagnosis of Wilson’s disease is confirmed by ONE of the following (i or ii):
  • i. Genetic testing results confirming biallelic pathogenic ATP7B mutations (in either symptomatic or asymptomatic individuals); OR
  • ii. Confirmation of at least TWO of the following (TWO of a, b, c, or d):
  • a. Presence of Kayser-Fleischer rings; OR
  • b. Serum ceruloplasmin level < 20 mg/dL; OR
  • c. Liver biopsy findings consistent with Wilson’s disease; OR
  • d. 24-hour urinary copper > 40 mcg/24 hours; AND
  • Patient meets ONE of the following (i, ii, iii, iv, v, or vi):
  • i. According to the prescriber, patient has tried one penicillamine product and is intolerant to penicillamine therapy; OR
  • ii. According to the prescriber, patient has clinical features indicating the potential for intolerance to penicillamine therapy (e.g., renal disease, congestive splenomegaly causing severe thrombocytopenia, autoimmune tendency); OR
  • iii. According to the prescriber, patient has a contraindication to penicillamine therapy; OR
  • iv. Patient has neurologic manifestations of Wilson’s disease; OR
  • v. Patient is pregnant; OR
  • vi. Patient has been started on therapy with trientine (Cuvrior or Syprine, generic); AND
  • The medication is prescribed by or in consultation with a gastroenterologist, hepatologist, or liver transplant physician.

Approval duration

1 year