Abstract
Spontaneous pubertal growth was studied in 8 patients with classic Tfm, and the effect of exogenous estrogens in 1(gonadectomized (GE) before puberty). Mean chronologic age (CA) at peak height velocity(PHV) was 12.7yrs,which is closer to CA at PHV in normal girls(NG,12.4) than in normal boys (NB,13.9). Mean PHV(7.4 cm/yr) was as in NG(7.3 cm/yr)),but significantly lower than in NB(9.3).Conjugated estrogens in the GE-patient started at CA 10.2yrs caused a PHV of 12cm/yr. Bone age(BA) corresponded better to NB than to NG standards. Adult height is 172.3∓4.1cm(n=6,2 not yet adult).It is significantly lower than in NB(-0.6∓0.7SD),but higher than in NG (+1.4∓0.8 SD). Adult height in the GE-patient is only 160.5cm. Body proportions(n=3) are as in NG (sitting height (SH)+0.8, subischial leg height(SILH)+ 0.6SD).Compared to NB, the legs are relatively short (SH-0.4,SILH-0.9SD).Tfm-patients provide a model to study estrogen effects without interference of androgens.It is concluded that in NG the pubertal growth spurt also is mostly due to the action of estrogens rather than of adrenal androgens, and that physiological estrogen replacement in hypogonadism should not be started late,but at the appropriate BA in order to ensure normal pubertal growth.
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Zachmann, M., Prader, A., Sobel, E. et al. Pubertal growth in patients with testicular feminisation (Tfm). Pediatr Res 18, 1210 (1984). https://doi.org/10.1203/00006450-198411000-00055
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DOI: https://doi.org/10.1203/00006450-198411000-00055