[Evaluation of body composition, resting metabolic rate and frequency of metabolic disorders in adolescents with Klinefelter syndrome].

BACKGROUND

Kleinfelter syndrome (KS) is a common genetic disease characterized by hypergonadotropic hypogonadism. The high risk of developing of metabolic disorders in patients with KS is be explained by the presence of androgen deficiency, which leads to a decrease in the amount of lean soft mass and an increase of the adipose tissue content. The basal metabolic rate (BMR) is determined by the amount of lean soft mass, and its reduction can contribute to weight gain and the progression of metabolic disorders in KS.

AIM

Body composition, assessment of basal metabolism and metabolic profile in adolescents with KS.

MATERIALS AND METHODS

The study included 28 adolescents with KS, comparable in age and stage of sexual maturation. All patients were divided into two groups depending on the presence of clinical laboratory signs of hypogonadism. Patients passed through the evaluation of metabolic profile, bioelectrical impedance analysis for the body composition and chamber-based indirect calorimetry for the evaluation of BMR.

RESULTS

Normal indicants of body composition were determined in 20 (71.4%) adolescents, excess of adipose mass – in 6 (21.4%) patients, and deficit of adipose mass – in 7.2% of cases. Among 6 patients with excess of adipose mass, three were obese or overweight, while the rest had normal SDS body mass index (BMI). 64.3% of adolescents showed normal indicants of energy exchange at rest, 6 (21.4%) – a decrease of BMR, 4 (14.3%) - an increase of BMR. A comparative analysis of patient groups did not reveal statistically significant differences in BMR adjusted to a lean soft mass (OO/TM) (p=0.36). Insulin resistance was detected in 11 patients (39.3%), and the incidence of dyslipidemia was 3.6%. The groups did not significantly differ in terms of glycemia levels in the fasted state and lipid profile indicants.

CONCLUSION

Most adolescents with KS have normal indicants of body composition and basal metabolism, as well as a low frequency of metabolic disorders, regardless of the level of total testosterone in blood. In some patients with normal SDS BMI, excess of adipose mass is detected. The lack of correlation between the level of testosterone and the intensity of BMR may indicate a slight effect of androgen deficiency on energy exchange at rest in adolescents with KS.