Reference values for body proportions and body composition in adult women with Ullrich-Turner syndrome.

This cross sectional study was undertaken to establish reference values for adult women with Ullrich-Turner syndrome (UTS) verified cytogenetically by blood karyotyping and not treated with growth hormone during childhood and adolescence, with respect to anthropometric and body composition measurements, for future evaluations of growth promoting therapy. All members of the Danish Turner Association were invited, and 79 women with UTS participated. Forty-two had the 45,X karyotype and the other 37 had different karyotypes. Outcome measures were height, sitting height, arm span, length of hand and foot, biacromial and biiliac diameter, and hip, waist, and head circumference. Bioelectrical impedance was performed, and total body water, lean body mass, and fat mass were calculated. Results give a very distinct anthropometric picture of adult women with the UTS, with a mean height of 146.8+/-6.7 cm (mean+/-SD), sitting height of 78.6+/-3.6 cm, arm span measurements of 147.9+/-7.1 cm, being between 3 and 4 standard deviation scores (SDS) below average; with a mean hand length of 17.0+/-1.1 cm and foot length of 22.4+/-1.2 cm, being around 1.5 SDS below average; a mean weight of 56.3+/-12.8 kg, head circumference of 55.3+/-2.0 cm and biacromial diameter of 36.5+/-2.0 cm, being around 0 SDS; and finally, biiliacal diameter of 29.5+/-2.2 cm, being 1.4 SDS above average. The average body mass index (BMI) in the study was 26.3+/-5.3 kg/m2. As a group, females with UTS are overweight when compared with a group of "normal" women, with a higher fat mass, a lower lean body mass, but with a comparable amount of total body water (in %). This study presents the first comprehensive reference data on body proportions in the adult UTS. It shows that adult women with the Ullrich-Turner syndrome has a characteristic anthropometric shape. The data should be of use for future evaluations of growth hormone treatment or other growth promoting therapy in the UTS on anthropometric and body composition variables.