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Werner's SyndromeBiochemical and Cytogenetic Studies
David J. Gawkrodger, MB;
Graham C. Priestley, PhD;
Vijayalaxmi, PhD;
James A. Ross, PhD;
Paolo Narcisi, MSc;
John A. A. Hunter, MD
Arch Dermatol. 1985;121(5):636-641.
Abstract
Werner's syndrome is a rare condition of autosomalrecessive inheritance, showing some features of accelerated aging. We describe the clinical findings and laboratory studies in a 29-year-old man with this disorder, who presented because of a leg ulcer. Skin fibroblasts from our patient were difficult to culture and proliferated more slowly than those of controls. They produced less glycosaminoglycans than those of controls but synthesized more collagen, which was normal in type. The patient's urinary glycosaminoglycan level was slightly elevated, with hyaluronic acid as a major component. His peripheral blood lymphocytes showed no chromosomal instability and responded normally to mutagens.
(Arch Dermatol 1985;121:636-641)
Author Affiliations
From the Department of Dermatology, University of Edinburgh, Royal Infirmary (Drs Gawkrodger, Priestley, Ross, and Hunter); the MRC Clinical and Population Cytogenetics Unit, Western General Hospital, Edinburgh (Dr Vijayalaxmi); and the Dermatology Research Group, Clinical Research Centre, Northwick Park Hospital, Harrow, England (Mr Narcisi).
Footnotes
Accepted for publication Sept 19, 1984.
Reprints not available.
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