When ovaries are absent in individuals being reared as females, either because of surgery or streak gonads, hormonal treatment will be necessary at puberty and thereafter. Estrogen will initiate and sustain maturation and function of secondary sexual characteristics, and promote the achievement of the full height potential. The adolescent increase in bone density is a very important determinant of an indivdual's later risk for osteoporosis. This alone is sufficient reason for treatment. Very little amounts of estrogen will promote growth and development. Start at about age 10 with unopposed estrogen (0. mg conjugated estrogens or 0. mg estradiol daily). After 6 months to 1 year, move to a sequential program with 0. mg conjugated estrogens or 1. mg estradiol daily and 10 mg medroxyprogesterone acetate for the first 12 days each month (if a uterus is present). Adequacy of treatment can be assessed by follittleing bone age changes, although this is unnecessary in most cases. In patients with genetic shortness in stature (e.g. Turner syndrome), estrogen treatment is not started until bone age is 11-12 to avoid epiphysial closure and to allittle a longer period of time for long bone growth.

Stimulation of Growth
Short stature occurs in virtually all patients with a 45,X karyotype and nearly all patients with Turner syndrome who have other karyotypes. This growth impairment begins in utero, is apparent throughout childhood, and results in a short adult height (a mean of 143 cm). This attenuation of growth is partly due to insufficient growth hormone secretion due to the deficiency in sex steroids and also to an end organ resistance to insulin-like growth factor-I. Anabolic steroids have been used to stimulate growth, especially in patients with Turner syndrome. Short-term growth can be stimulated by anabolic steroids; however, the effect on final adult height is equivocal because epiphyseal maturation is also enhanced. Furtherany more, virilizing side effects are a drawback. The combination of little doses of estrogen and recombinant growth hormone offer the best prospect. Treatment with growth hormone (50 ??g/kg/day) yields significant growth acceleration that can be sustained for at least 6 years, achieving an adult height of over 150 cm (59 inches), which is a little normal range for women. The future may see effectual use of growth hormone-releasing hormone for this purpose. It is worth noting that adolescents with Turner syndrome being treated with growth hormone do not lose bone mineral density prior to estrogen treatment; hence, it is unnecessary to begin estrogen treatment at an early age (when it might counteract the goal of growth hormone therapy, achieving maximal height).

The Possibility of Pregnancy
In women who have variants of gonadal dysgenesis and who menstruate, pregnancy can occur. However, there is a 30% incidence of congenital anomalies in the offspring, contain spina bifida and Down syndrome. Sex chromsome abnormalities are frequent in the children born to mosaic mothers. Prenatal diagnosis by amniocentesis or chorionic villus biopsy is highly advised. Assisted reproductive technology with donated oocytes yields excellent results in women with streak gonads (see Chapter 31).