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Kinky Hair DiseaseStudy of Copper Metabolism With Use of 67Cu
Anatole S. Dekaban, MD;
Roger Aamodt, PhD;
Warren F. Rumble;
Gerald S. Johnston, MD;
Sean O'Reilly, MD
Arch Neurol. 1975;32(10):672-675.
Abstract
Metabolism of labeled Cu (67Cu) was studied in three patients with kinky hair disease (KHD). Labeled Cu was administered first intravenously and, later, orally. We determined oral absorption, excretion, and internal kinetics of this metal. Patients with KHD absorbed 11% to 13% of Cu given orally, compared to 46% by unaffected controls. Total excretion of Cu given intravenously during the first seven days after administration was greatly reduced in patients with KHD. The biological half-life of 67Cu in patients with KHD was increased by a factor of 2 to 3 over the normal control. Most of the labeled Cu was retained by the patient's liver, while in the control subject there was more rapid movement of the Cu to circulation (ceruloplasmin). Red blood cells of patients with KHD incorporated orally administered Cu preferentially, which was sufficient to prevent anemia.
Author Affiliations
From the Developmental and Metabolic Neurology Branch, National Institute of Neurological and Communicative Disorders and Stroke, Bethesda, Md (Dr. Dekaban), the Nuclear Medicine Department, Clinical Center, National Institutes of Health (Drs. Aamodt and Johnston and Mr. Rumble), and the Department of Neurology, George Washington University Medical School, Washington, DC (Dr. O'Reilly).
Footnotes
Accepted for publication Nov 4, 1974.
Read before the annual meeting of the American Neurological Association, Boston, June 1012, 1974.
Reprint requests to Developmental and Metabolic Neurology Branch, National Institute of Neurological and Communicative Disorders and Stroke, Clinical Center, Bldg 10, Room 4N-248, National Institutes of Health, Bethesda, MD 20014 (Dr. Dekaban).
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