Werner's syndrome with a report of two cases

Abstract

Two cases of Werner's syndrome are presented, with a discussion of the important clinical features of this entity which include short stature, premature senility, juvenile cataracts, scleroderma-like changes, skin ulcers over the feet and ankles, and certain endocrine abnormalities. The diagnosis depends upon the physician's awareness of this entity and can usually be made by general inspection of the patient. The etiology of Werner's syndrome is unknown. Hereditary factors seem to be important. Although endocrine abnormalities are usually present, they are variable in their occurrence and probably are of no etiologic significance. An endocrine evaluation of our patients revealed that both had a decrease in glucose tolerance. One patient had clinical hypogonadism and both had osteoporosis. There was no evidence of primary thyroid, parathyroid or pituitary abnormality. In both cases there was evidence of arteriosclerosis. The S f 12–20 and S f 20–100 values of the sera were normal in one patient and elevated in another. Treatment is not specific and is directed toward manifestations such as cataracts, diabetes mellitus, skin ulcers and hypogonadism.