Urinary hydroxylysine and hydroxylysyl glycosides excretion in patients with Turner's syndrome.

Urinary excretion of 5-hydroxylysine (Hyl) and its two glycosides, a monoglycoside (Gal-Hyl) and a diglycoside (Glc-Gal-Hyl), and the ratio of the diglycoside to monoglycoside have been studied in 30 patients with Turner's syndrome and in 38 healthy controls. In patients, the urinary excretion of Hyl and its diglycoside was similar to that obtained in the controls, while the excretion of the monoglycoside was significantly lower before the age of 17 years. As a consequence, between 6 and 17 years of age the Glc-Gal-Hyl/Gal-Hyl ratio is significantly higher in patients with Turner's syndrome than in normal subjects. The results of our study seem to indicate a disturbance in the turnover of collagen in Turner's syndrome.