Steely-Hair Syndrome and Copper

Abstract

To the Editor: The genetic defect underlying Menkes's disease, or steely-hair syndrome, though only partly understood, is assumed to result in impaired gastrointestinal absorption and transport of copper.¹ On the basis of results obtained in the study of an affected 11-month-old infant, Lott et al. (issue of January 23) have advanced the hypothesis that transport of copper into the liver is impaired because of the formation of an inorganic complex of copper. Unfortunately, their experimental measurements of the concentrations of ceruloplasmin and copper in serum appear to be so internally inconsistent as to raise doubt whether the data are accurate . . .