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  Vol. 137 No. 1, January 2001 TABLE OF CONTENTS
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Is Kinky-Hair Disease a Misnomer for Menkes Syndrome?

Since this article does not have an abstract, we have provided the first 150 words of the full text and any section headings.

Although we had the satisfaction of correctly diagnosing "Fragile Hair and Seizures in a Child" in your March issue,1 and were interested to read about it, we would like to add a little further information about the hair microscopy findings in Menkes syndrome. In his original article in 1962, John Menkes2 described a sex-linked recessive disorder with retardation of growth, peculiar hair, and focal cerebral and cerebellar degeneration in 5 children. In only 2 of the children was the hair kinky, although in all of them it was sparse and wiry.

It was Danks in Australia from 1972 onward who carried out most of the work on Menkes syndrome. With the help of research into the wool of sheep deficient in copper, he and his colleagues3 established the presence of decreased levels of copper and ceruloplasmin (the copper transport protein) in the serum of children with Menkes syndrome, and low . . . [Full Text of this Article]







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