Inherited Diseases: Devastating Defect

The technical name of the disorder is hepatolenticular degeneration, a mouthful that has moved physicians to call it Wilson's disease, after the doctor who first described it. But by any name it is still one of the most mysterious and devastating defects in the body's complex chemistry. It can be inherited from outwardly healthy parents and involves the metabolism of minute amounts of copper. As recently as 1960, medical textbooks stated: "The course of the disease is inexorably downhill if untreated." Most baffling is the fact that the inherited defect may either produce severe illness within the first year...

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