Episode two takes the other metal-handling disease, Wilson, where the toxin is copper and the ATP7B pump fails at two coupled jobs: pushing copper into bile and loading it onto ceruloplasmin. The diagnostic logic is harder because no single test stands alone, so the Leipzig score integrates weighted features and the chelator choice turns on the speed and site of copper movement. Low ceruloplasmin, high urinary copper, the fulminant fingerprint, and trientine-plus-zinc therapy run through the episode. The board traps are the young steatohepatitis patient without metabolic syndrome and the misread fulminant case that costs the transplant window. Topics covered ATP7B and the failed copper exit Low ceruloplasmin from accelerated degradation Kayser-Fleischer rings and sunflower cataracts Hepatic and neuropsychiatric phenotypes Leipzig diagnostic score and copper tests The fulminant Wilson fingerprint Trientine, penicillamine, and zinc Monitoring with urinary and free copper Pregnancy and contraception traps Key decisions Diagnose Wilson with at least two of five features (Kayser-Fleischer rings, ceruloplasmin under twenty, typical neurologic symptoms, twenty-four-hour urinary copper over forty, hepatic copper over two hundred fifty) using the Leipzig score, where four or higher establishes it. A ceruloplasmin over thirty essentially excludes Wilson because synthetic capacity is intact, and a hepatic copper under thirty-five excludes it; the intermediate band triggers molecular testing. Consider Wilson in any patient from about three to forty-five with unexplained liver disease, and specifically in a young steatohepatitis patient with no metabolic syndrome features. First-line chelation is trientine over penicillamine for neurologic Wilson because penicillamine causes paradoxical worsening in up to half; zinc induces enterocyte metallothionein and is for maintenance or pre-symptomatic patients only. Trientine chelates oral iron into a toxic complex and chelates zinc, so separate them in time, and overtreatment causes a reversible sideroblastic anemia from copper depletion. On long-term monitoring, high free copper with low urinary copper signals non-adherence, while low free copper signals overtreatment. In pregnancy continue chelation at a quarter-to-half reduced dose by the third trimester, avoid estrogen-containing contraceptives and copper IUDs, and replace prenatal vitamins with copper-free formulations. For the full chapter with MCQs, tables, and primary-guideline references, visit www.boardpearls.com. Questions or feedback: [email protected]. (00:00) - Introduction: the failed copper exit (00:25) - ATP7B mechanism and low ceruloplasmin (01:50) - Hepatic and neuropsychiatric phenotypes (03:01) - The Leipzig score and diagnostic tests (04:36) - The fulminant Wilson fingerprint (05:35) - Chelation: trientine, penicillamine, zinc (07:37) - Monitoring: urinary and free copper (08:15) - Pregnancy and contraception traps (09:11) - One-line summary

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“What did the council decide on item 42?” — answered with the speaker and the moment.

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