SKELETAL DEVELOPMENT IN GONADAL DYSGENESIS, FEMALE IN PHENOTYPE

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Abstract
1. The skeletal development has been investigated roentgenographically in 50 patients affected by gonadal dysgenesis, of whom 37 had Turner's syndrome and 13 had pure gonadal dysgenesis. In 15 patients the skeletal development has been controlled after substitutive hormonal therapy. 2. Both in patients with Turner's syndrome and those with pure gonadal dysgenesis there is a delay in skeletal maturation affecting subjects of any age; the skeletal ages comprised those between 10 and 13.6 years. This behavior is referred to as a lack of gonadal steroids. 3. All the patients who underwent substitutive hormonal treatment showed maturation of the examined bones. 4. Discrepancy in maturation delay between different anatomic areas has been found. 5. The appearance of the epiphysis of the iliac crest seems to be a sufficiently reliable index of sexual maturity. In 26 out of 30 patients, whose iliac crest has been examined, this epiphysis was absent and it appeared in most of the cases in whom menstruation was induced therapeutically. 6. The skeletal growth was altered differently in patients with pure gonadal dysgenesis and those with Turner's syndrome; only the latter had a short stature which is regarded as malformative in origin.