ABSTRACT

Werner’s syndrome is an autosomal recessive condition, a “model” of accelerated aging, characterized by growth arrest at puberty, cataracts by age 30 or 40 years, premature graying and balding, scleroderma-like skin and chronic ulcers over the extremities, decreased muscle and subcutaneous tissue, beak-shaped nose and premature atherosclerosis and death in middle life. Although coronary artery disease is a recognized occurrence in this syndrome, the extent and consequence of the coronary atherosclerotic process in it has received little attention despite descriptions of at least 25 previously reported necropsy patients. A 24-year-old white man who died in June 1979, had been well until age 13 years, when he noted painful swellings of his knees and wrists and tightness of the skin over his hands. In July 1979 he was hospitalized for anorexia, vomiting, orthopnea (4 pillow) and paroxysmal nocturnal dyspnea, peripheral cyanosis and seizure.