Wilson’s Disease –Pediatric Guideline

P Socha et al. JPGN 2018; 334-4. This ESPGHAN position paper makes recommendations for Wilson’s disease. This is a helpful paper, though the AASLD Wilson’s guideline is more comprehensive. A couple of pointers from the JPGN publication:

  • The authors recommend molecular testing if available and using liver copper measurement “if molecular testing is inconclusive”
  • Screen siblings of any new patients
  • Urinary copper excretion in the 200-500 mcg per 24 hours is consistent with adequacy of treatment
  • With treatment, liver function tests improve over 2-6 months. “If increased transaminases remain or relapse despite treatment, poor compliance should be suspected.”

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