Precocious Puberty (sexual precocity) Appearance of secondary sexual development before the age of 9 in boys (not really sure about girls). Central (complete or true): premature activation of the hypothalamic-pituitary axis and is gonadotropin dependent. Incomplete: Ectopic gonadotropin release in boys, autonomous sex steroid secretion in either sex, or if condition is not gonadotropin-dependent. Isosexual precocity: feminization occurs in girls and virilization occurs in boys Contrasexual precocity: feminization occurs in boys and virilization occurs in girls. Central (Complete or True) Precocious Puberty o Constitutional or familial central (complete or true) precocious puberty Normal children who demonstrate isosexual precocity at an age more than 2.5SD below the mean. Familial tendency toward early puberty. o Idiopathic Central isosexual precocious puberty No familial tendency and no organic disease.
Epilepsy and developmental delay in absence of central nervous system anatomic abnormality indicative of central nervous system process responsible for the condition. Serum gonadotropin and sex steroid concentrations and response to GnRH or GnRH agonists are similar to those in normal pubertal children. For boys, testicular enlargement should be first sign. For girls, breast development should be the first sign, and rarely, pubic hair appearance. o Central Nervous System Disorders Tumors Often include optic and hypothalamic gliomas, astrocytomas, ependymoma , germinomas. Interfere with neural pathways that inhibit GnRH secretion. Craniopharyngiomas can also trigger percocious pubertal development. Harmatomas of the tuber cinereum contain GnRH and neurosecretory cells similar to those found in the median eminience cause precocious puberty by secreting GnRH. Tumors may cause GH deficiency in relation with central precocious puberty. Other causes of true precocious puberty Infectious granulomatous conditions such as encephalitis, brain abscess, postinfectious suprasellar cysts, sarcoidosis, and tuberculous granulomas of the hypothalamus. o Virilizing Syndromes Patients with long-untreated virilizing adrenal hyperplasia who have advanced bone ages may manifest precocious puberty after treatment with glucocorticoud suppression. Incomple Isosexual Precocious Puberty in Boys o Premature sexual development in the absence of hypothalamic-pituitary maturation caused by: 1) ectopic or autonomous endogenous secretion of hCG or LH or iatrogenic administrtion of hCG; 2) autonomous endogenous secretion of androgens from the testes or adrenal glands or from iatrogenic exogenous administration of androgens. Gonadotropin-secreting tumors Hepatomas of hepatoblastomas of the liver, as well as teratomas of choriocarcinoma of the mediastinum, gonads, retroperitoneum, or pineal gland, or geminomas of the central nervous system.
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- Spring '14
- Precocious puberty, central precocious puberty