ICD-10 Codes
E23.0 Hypopituitarism.
E25.0 Congenital adrenogenital disorders associated with enzyme deficiency. E28.3 Primary ovarian failure. Е30.0 Delayed puberty. Е30.1 Precocious puberty.
E45 Retarded development following protein-energy malnutrition. Q78.1 Polyostotic fibrous dysplasia (McCune-Albright syndrome). Q96 Turner's syndrome.
General Information
The course of puberty is genetically predetermined; it proceeds in accordance with clear-cut physiological criteria for what is "normal"; these are the time and sequence of emergence of secondary sex characters (the tempo of sexual development).
The period of puberty begins at the age 7-9 and is complete by the age 17-19; thus its normal duration is ten years.
Numerous quantitative and qualitative changes in the girl's body prepare her for childbearing; they are described in Section 3.2.
The term disorder of sexual development means deviations from normal tempo and/or sequence in the processes of sexual maturation due to disturbed synthesis and/or secretion of steroid hormones in the girl's body.
NB! All processes associated with puberty take place in the second decade of a girl?s life.
Etiology
Disorders of sexual development are in a great part due to abnormalities arising during intrauterine development, during labor and in the postnatal period (pre-eclampsia, threatened abortion, abnormalities, fetal asphyxia, injuries of the central nervous system, preterm birth, fetal hypotrophy, maternal infections and alcohol abuse, and other). A certain role is played by infections sustained by the girl in early childhood and at puberty.
In rare cases disorders of sexual development are attributable to neoplasms and cysts in the hypothalamic-pituitary system, ovaries and adrenal glands. These disorders can be lesions of organs or dysfunction of organs. Chromosomal and genetic abnormalities are accountable for 30% of sexual development disorder.
Classification
Three main types of disorder are distinguished.
• Precocious puberty:
- isosexual (female) precocious puberty;
- heterosexual (male) precocious puberty.
• Late onset of puberty, delayed puberty:
- of central origin;
- of ovarian origin;
- with underlying somatic abnormalities.
• Gonadal dysgenesis, or absence of sexual development.