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Endocrinology

Premature sexual development

Table of contents
Premature sexual development
Diagnosis
Treatment of premature sexual development

преждевременное половое развитие у мальчика преждевременное половое развитие у девочек

Premature sexual development (maturing) call the complex of symptoms which is characterized by emergence of secondary sexual characteristics till 9 flyings at boys and till 8 flyings at girls.
The pathogenetic principle considering primary localization of pathogenetic process in system a hypothalamus - a hypophysis - gonads - adrenal glands is the basis for classification of a syndrome of premature sexual development. Allocate true, or central, disease forms which pathogeny is caused by premature activity of the central part of a gonadostat: gipotalamo-pituitary system. Increase of secretion of sexual steroids gonads in this case is a consequence of stimulation of gonads gonadotrophins. False, or peripheral, forms of premature sexual development are caused by premature secretion of sex hormones tumors of gonads or adrenal glands, independent of secretion of gonadotrophins. In independent group allocate so-called gonadotropinnezavisimy forms of premature sexual development at which autonomous activation of activity of gonads is caused by genetic disorders. At all listed disease forms sexual development possesses all main characteristics of the progressing puberty: in addition to emergence of secondary sexual characteristics the volume of gonads increases, growth rate and bone maturing, reflecting system influence of sexual steroids on the child's organism accelerates. The clinical options of premature sexual development possessing this complex of signs are defined as a full form of premature sexual development. The so-called partial (incomplete) forms of premature sexual development which are characterized by the isolated development of secondary pilosis (premature to a pubarkha) and the isolated increase in mammary glands are in addition allocated (premature to a telarkha). There are also options of premature sexual development which are not keeping within unambiguously one of the listed disease forms: premature sexual development against dekompensirovanny primary hypothyroidism.

Classification of premature sexual development

True premature sexual development
Idiopathic
Cerebral
TsNS tumors
(gamartoma of a hypothalamus, glioma of a visual tract and bottom of the 3rd ventricle, pinealoma)

Defeat of TsNS of not tumoral genesis
(arachnoidal cysts of the 3rd ventricle, hydrocephaly, birth trauma, encephalitis, meningitis, toxoplasmosis, radiation of TsNS, surgical intervention)

Inborn syndromes
Neurofibromatosis of the 1st type
Tuberous sclerosis
Russell-Silvera's syndrome
Syndrome Wang-Vika-Grombakh (at noncompensated primary hypothyroidism)

True premature sexual development at long influence of sexual steroids
(late treatment of VDKN, after removal of steroidsekretiruyushchy tumors)

False premature sexual development
 
At boys:

HGCh - the cosecreting tumors of cranial and ekstrakranialny localization

Tumors of testicles (leydigoma)

Tumors of adrenal glands (androsteroma)

Inborn dysfunction of bark of adrenal glands (deficit 21-and 11 (V-hydroxylases)

At girls:

Tumors of ovaries (granulocellular, ovarian carcinomas)

Tumors of adrenal glands
(kortikoestroma)

Ovarian follicular cysts

Gonadotropinnezavisimy forms

Mac-Kyyuna's syndrome - Albright - Braytseva

Testotoksikoz

Incomplete forms of premature sexual development

Accelerated to a pubarkha

Accelerated to a telarkha



 
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