Syndrome de Turner: croissance staturopondérale et maturation osseuse spontanées

Abstract

Background. — Since growth hormone is effective in increasing the height of girls with Turner's syndrome, it is important to dispose of growth and bone maturation curves in a large number of untreated patients. Population and methods. — Data on growth and bone maturation were collected from 160 patients with Turner's syndrome (50 have reached final height), born 1965–1991, untreated with growth hormone or anabolic steroids. X monosomy was found in half of the patients, mosaicism or X abnormality was present in the other half. Spontaneous puberty occurred in 25% (n = 25) of patients older than 13 years, 38 patients received estrogen after 13 years. Final heights were compared to predicted height according to Lyon's method. Results. — Forty-five percent of patients were small for date. Height velocity decreased from 2 years of age and decreased faster during adolescence, when gonadal dysgenesis occurred. Bone maturation velocity decreased also during adolescence. Excessive weight appeared after the age of 5 years. Patients with partial deletion of the long arm of X ( n = 6) were taller than the other girls (r = 44) (mean ± DS) 152.5 ± 3.1 cm, range 150–158 cm versus 142.5 ± 4.9 cm, 130–150 cm ( P < 0.0001). Final height was not modified by spontaneous puberty. Final height was correlated with birth weight (r = 0.7), maternal height (r = 0.5) and mid parental height (r = 0.5). Finally, the Lyon's method for predicted final height seemed to be suitable for this population, (r = 0.8, P < 0.001). Conclusion. — Appropriate growth curve is an essential clinical tool in evaluating treatment aimed at increasing final stature in patients with Turner's syndrome.