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. 2019 Apr;7(Suppl 2):S65. doi: 10.21037/atm.2018.12.53

Table S1. Summary of guideline recommendations in WD.

Society guidelines Diagnosis Treatment Special circumstances
Diagnostic testing Diagnosis of ALF due to WD Family screening Medical therapy   Treatment monitoring   Transplant   ALF Pregnancy Surgery
AASLD 2008 Clinical and biochemical algorithmic approach Laboratory and clinical assessment Siblings: genetic testing*. Children, other first-degree relatives: clinical assessment Initial: chelator +/− zinc.
Maintenance and asymptomatic: chelator or zinc
  24-hour urine copper   Indication: decompensated cirrhosis, not primarily for neuropsychiatric disease   Bridging therapies, transplant Dose-reduction of chelator, no adjustment for zinc Dose-reduction of chelator, no adjustment for zinc
EASL 2012 Leipzig score Laboratory and clinical assessment First-degree relatives: genetic testing Initial: chelator.
Maintenance and asymptomatic: chelator or zinc
  24-hour urine copper   Indication: decompensated cirrhosis, not primarily for neuropsychiatric disease. Prognosis: revised King’s Wilson score   Transplant Dose-reduction of chelator, no adjustment for zinc
ESPGHAN 2018 Clinical and biochemical algorithm and Leipzig score Laboratory and clinical assessment First-degree relatives: clinical assessment and genetic testing* Initial: chelator +/− zinc.
Maintenance and asymptomatic: zinc
  24-hour urine copper. If zinc therapy: serum zinc, 24-hour urine zinc   Indication: decompensated cirrhosis, exclude severe neuropsychiatric disease. Prognosis: revised King’s Wilson score   Bridging therapies, transplant

*, if genetic testing unavailable, standard biochemical and clinical testing. WD, Wilson disease.