Premature Puberty

PREMATURE PUBERTY (from Latin pubertas-sexual maturation and praecox-premature), a developmental anomaly; in the period of childhood, and in rare cases also in infancy, i.e., long before the normal term, signs of sexual maturation appear. The clinical forms, etiology, and pathogenesis of this anomaly are very diverse. It is necessary to distinguish at least three most important clinical forms. 1. Sometimes in a child, simultaneously with the premature appearance of signs of sexual maturation, there is also intensified physical and psychological development. Such a form with premature harmonious and integral development resembles the normal physiological process of development in the period of puberty the most. Therefore some authors (V. I. Molchanov, Lenz) propose to leave only for it the old name pubertas praecox. Such cases are described a little. 2. Macrogenitosomia praecox (see) and 3. Hirsutism (see). Cases of premature sexual maturation were known already in deep antiquity. Cratenis mentions a man who was a child, a youth, an adult man, and an old man, was married, had children, and all this during the first seven years of life. The first systematic description of this developmental anomaly was made by Haller (Galen, 1776), who collected 28 cases. Kussmaul (1862) on the basis of material in 56 cases subjected the whole question of P. p. to a detailed critical review. A sufficiently complete review indicating the role of endocrine glands in the pathogenesis of the disease was given by Neurath (1909). Among newer reviews one should point to the work of Reuben and Manning (M. Reuben, R. Manning; 1922). In Russian literature there are brief descriptions of individual cases. A sufficiently complete illumination of the whole question is found in the articles of V. I. Molchanov and E. V. Klumov (1925-26). Premature sexual maturation, including all its forms, occurs not often. The most complete material was collected by Reuben and Manning (71 boys and 327 girls). If one adds to it the material of the last 10 years, one can in general accept that up to the present time 400-500 cases of premature sexual development have been described. The time of appearance of the first signs of sexual maturation is very diverse. In individual cases it was noted immediately after birth, or during the first months of life, more often in the first years of life; according to the statistics of Reuben and Manning in girls usually before 3 years, in boys after 3 years. Etiology. As etiological factors a whole series of moments were indicated: a large number of pregnancies in the mother, early sexual life, infections, etc., but none of them can be considered justified. The hereditary factor undoubtedly plays a great role: in individual cases the simultaneous existence of this anomaly in parents and child was noted. Bood, for example, reports a girl who had menstruations from the age of 1, became pregnant in the 9th year of life, gave birth to a girl in whom hair on the genital organs and under the armpits was found. Pathogenesis. Our knowledge of the pathogenesis of P. p. is relatively more definite. To a certain extent on the basis of experiments with animals and the results of organotherapy, but mainly on the basis of pathological-anatomical studies of those cases which reached autopsy or operation, one can consider established the connection of P. p. with damage to the internal secretion glands and the central nervous system. In individual cases changes were found in the pituitary gland, in the pineal and thyroid glands, but these changes were obviously of a secondary character and cannot explain premature development. In particular, in relation to the thyroid gland some authors (Lenz, Klumov, etc.) noted in their patients symptoms of hyperthyroidism - enlargement of the thyroid gland, tachycardia, exophthalmus. In some cases symptoms of hyperfunction of the anterior lobe of the pituitary gland were noted, for example acromegaly in the cases of Zondek, Molchanov, etc. Undoubted changes were found in three glands: 1) sexual, 2) adrenal, and 3) pineal. Sexual glands. Malignant tumors - sarcomas, carcinomas (see Ovaries) were detected, which by their rapid growth caused intensified functioning of the glands. In some cases sexual maturation proceeded according to the type of macrogenitosomia praecox, in others - with premature development also of intellect, i.e., according to the type of P. p. in the strict sense of the word. In part of the cases an operation for removal of the tumor was performed, after which the symptoms of P. p. disappeared and the patients returned to the child type. Thus the connection between the tumor of the sexual glands and P. p. is not subject to doubt. In the adrenal glands adenomas were found; cancers, nodular hyperplasia of the cortical substance, i.e., in these cases premature development was connected with hyperfunction of the gland. About 40 cases are described, of which only 4 relate to boys. Simultaneously with P. p. in boys the intensified development of musculature and skeleton was striking (boy Herculeses). Intellectual development was sometimes above, sometimes below normal. In girls the aforementioned tumors of the adrenal glands as a rule lead to the development of the syndrome of hirsutism, and in these cases after removal of the tumor immediately a return to the normal type of development occurred. The connection of P. p. with damage to the pineal gland is less proven. In this gland tumors (teratomas, sarcomas) were often found, but only in part of the cases was pubertas praecox observed according to the type of macrogenitosomia praecox, moreover almost exclusively in boys. The majority of authors considers P. p. in tumors of the pineal gland as a result of the loss of its function (hypopinealism), but another explanation is also possible, namely damage to the surrounding part of the brain and the vegetative centers contained in it; such a mechanism of origin of P. p. has to be admitted for those cases in which there were no changes in the endocrine glands, but they were found in the central nervous system (hydrocephalus, tumors of the floor of the third ventricle). Finally, cases are described in which there were no organic changes either in the endocrine glands or in the central nervous system; such cases Lenz, Molchanov, etc. consider as "functional hypergenitalism". In relation to this very category of cases one can speak of a primarily developing hypersecretion of the sex hormone of the anterior lobe of the pituitary gland (prolan). From all the above it follows that P. p. is not one disease, but a whole group of them, diverse both in etiology and in pathogenesis and united only due to the commonality of the symptom of premature maturation. The course and prognosis are diverse. Patients who have a tumor or other organic lesions die soon if an operation is not performed. Persons with "functional hypergenitalism" can live to deep old age. Sexual life in them begins early, some had numerous offspring. In the case of Haller a woman who menstruated from two years gave birth in the 9th year of life, menstruations continued until the age of 53. The patient Deseuret, who began menstruating in the 3rd year of life, had 8 children, twice had abortions. The fate of children with premature sexual maturation in relation to their growth is interesting. Discovering intensified growth during the first years of life and being among their peers giants, such children, as soon as their sexual maturation ends, stop growing, even if they are far from yet reaching the size of an adult person. In Lenz's case a girl stopped growing at 11 years, having only 127 cm, in Molchanov's case a boy who grew during the first 3 years of life by 60 cm stopped growing at 9 years, having 134 cm. Thus premature sexual maturation ultimately leads to dwarf growth (genitalis nanism), while the psyche and intellect, despite the cessation of growth, continue to develop. Surgical treatment in tumors of the sexual glands and adrenal glands gives a brilliant effect, attempts at removal of tumors of the pineal gland ended in failure: death occurred very soon. In functional hypergenitalism organotherapy (preparations of thyroid, pineal, and other glands) was applied, but without result.