Abstract
Copper deficiency has been demonstrated in seven babies with Menkes' kinky-hair syndrome. Gross changes in free sulphydryl groups in hair keratin, consistent with copper deficiency, probably explain the kinky hair. Copper deficiency also explains the important changes in the elastic fibres in the arterial walls, the scorbutic bone changes, and the hypothermia observed in these babies. Evidence for a defect in the intestinal absorption of copper is given. Treatment of Menkes' syndrome may become possible as a result of these findings.
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