Pubertas praecox und Pubertas tarda

Summary

The commonest cause of retarded puberty is the constitutional form of delayed adolescence, — a functional and temporary disorder which occurs frequently in several members of the family. Growth, sceletal and sexual development are equally retarded by several years. At the end the children attain normal sexual maturity and (almost) normal height. Treatment appears indicated only when the patients suffer from psychological disturbances.

Although all types of true precocious puberty most probably take rise from the diencephalon they are divided into cerebral forms sensu strictori (I. hamartomas of the tuber cinereum, II. other anatomical lesions of the brain) and other forms (III. idiopathic type, IV. Weil-Albright-syndrome). I: The hamartomas consist of excessive hypothalamic tissue which histologically corresponds to the nerve cells of the tuber cinereum. They produce large amounts of LRF (Bierich et al, 1967). II: Such lesions appear to interfere with the suppression which the release-regulating-system during infancy and childhood normally exerts upon the hypophysiotropic area. III: This most frequent type of sexual precocity (more than 600 published cases) is encountered mainly in girls. IV: The same type of precocity is found in the Weil-Albright-Syndrome. In addition one observes large cafe-au-lait-colo\ired pigmentations of the skin and polyostotic fibrous dysplasia of the sceleton. - In both of the latter forms there is evidence not only for a premature gonadarche but also premature adrenarche, manifested by increased excretion of 17-oxosteroids, esp. 11-deoxy, 17-oxosteroids which exert androgenic activity. We have found a positive correlation between urinary 17-oxosteroids and sceletal development (bone age), pointing to a causal interrelationship between adrenal androgens, sceletal maturation and growth in height.

Today children with idiopathic sexual precocity are usually treated with medroxyprogesteron acetate (Depo-provera) which leads to the suppression of menses and breast development. Bone maturation and growth continue, however, to proceed in accelerated velocity. According to own investigations it is eventually possible to suppress this acceleration by use of chlormadinone acetate or cyproterone acetate, both steroids acting not only progestationally but in addition antiandrogenically.