Puberty Dr. Tara Husain
Tanner staging BREAST B1;prepubertal B2;Breast Buding B3; actual breast mound B4; Areola projects at an angle to breast mound B5; Adult configuration Axillary hair; A1; No axillary hair A2; Hair present A3; Adult
Genital staging; G1;Prepubertal (P 2.5-6 cm, Ts < 3ml) G2; Ts >4 ml, scrotal laxity, no penile enlargement G3; penile enlargement, with further dev. Ts, and scrotum G4; penile enlargement, ts 10-12 G5; adult genitalia Pubic hair staging P1; No hair P2;fine hair over monus, scrotum or labia P3; Adult type hair, but distribution confined to pubis P4; extension to near adult distribution P5; Adult
Tanner staging;
Milestones of puberty; Males enter puberty 6 mn after females In girls B2-3 is accompanied with PHV, with marked deceleration after puberty BOYS; PHV coincides with ts vol of 6-10 ml In both sexes age and onset and duration of puberty is subjected to individual variation
Difinitions; Adrenarche is the activation of the adrenal cortex for the production of adrenal androgens. Gonadarche is the activation of the gonads by the pituitary (FSH) and (LH) Pubarche is the appearance of pubic hair Thelarche is the appearance of breast tissue Menarche is the age of onset of the first menstrual period Spermarche is the age at first ejaculation
Sexual precocity; Is the onset of secondary sexual development before the age of eight years in girls and nine years in boys
Classification; Gonadotropin-dependent precocious puberty Gonadotropin-independent precocious puberty Incomplete precocious puberty
Gonadotropin-dependent precocious puberty early maturation of the hypothalamic-pituitarygonadal axis characterized by sequential maturation of Isosexual 10-20 times more common in female
Causes; Idiopathic; 80% of cases almost all females CNS tumers ; e.g; Hamartomas CNS lesions; hydrocephalus, cysts, trauma, CNS inflammatory disease, and congenital mid-line defects, such as optic nerve hypoplasia Genetics Previous excess sex steroid exposure
Gonadotropin-independent caused by excess secretion of sex hormones derived from the gonads or adrenal glands exogenous sources of sex steroids ectopic production of gonadotropin from a germ cell tumor isosexual or contrasexual FSH and LH levels are suppressed and do not increase with GnRH stimulation
Incomplete precocious puberty premature thelarche premature adrenarche
premature thelarche; present around two years of age Isolated breast development, either unilateral or bilateral Absence of other secondary sexual characteristics Normal linear growth Normal bone age Progress in 14-20% of cases.
Premature adrenarche appearance of pubic and/or axillary hair prior to the age of eight years in girls and nine years in boys. more common; girls, Black females obesity and insulin resistance
Initial evaluation Medical history Physical examination Bone age Tanner staging
Workup; Testosterone Estradiol LH, FSH GnRH stimulation test DHEA-S Thyroid function test Imaging studies;
Further evaluation; Basal LH levels and LH levels following administration of GnRH; a markedly elevated LH > 5 miu/ml indicate GDPP. patients with low or intermediate basal levels of LH should have a GnRH stimulation test to clarify the diagnosis.
Decision to treat depends on; child s age the rate of pubertal progression height velocity estimated adult height as determined from the rate of bone age advancement.
TREATMENT FOR GDPP; gonadotropin-releasing hormone (GnRH) agonist in idiopathic cases Treatment of the primary cause when faceable Treatment is usually continued until about age 11 in girls, and age 12 in boys, but decision is individualized according to child, bone age and height age, predicted height, and social desire to join their peers in puberty
Treatment of GIPP; According to the primary cause Tumors of the gonads are treated surgically If due to exposure to exogenous sex steroids this must stopped classic congenital adrenal hyperplasia, should be treated with glucocorticoid therapy
Delay in puberty absence or incomplete development of initial secondary sexual characteristics beyond 14 in boys and 12 in girls.
PRIMARY VERSUS SECONDARY HYPOGONADISM High LH, FSH; primary hypogonadism and/or defects in their receptors on the membrane of the gonadal cells Low LH, FSH; hypothalamic dysfunction hypopituitarism, Hypothyroidism hyperprolactinemia
Evaluation; History; pubertal development is totally absent or had started but then "stalled Nutritional exercise intensity prior medical illness, or medication usage associated congenital abnormalities A positive family history of either constitutional delay of puberty or congenital GnRH deficiency presence of an abnormal sense of smell
Physical examination; height, weight, span, and secondary sex characteristics An arm span exceeding the height by more than 5 cm(suggests delayed epiphyseal closure secondary to hypogonadism Secondary sexual characteristics and Tannar staging Uterin size by ultrasound Testicular size by by a Prader orchidometer
Investigation Bone age ( x-ray of left hand & wrist) pelvic or testicular ultrasonography Head MRI; in patient suggesting a hypothalamic or pituitary cause CBC, ESR, BUN, LFT LH, FSH, testosterone, estradiol serum prolactin; in case of hyperprolactinemia we should do MRI TFT; hypothyroidism may cause delay puberty karyotype
Treatment; distinction between congenital GnRH deficiency and constitutional delay of puberty, can be resolved only with serial observations. therapy should be restricted to boys older than 14 years and girls older than 12 years who show no signs of puberty Administration of gonadal steroids; estrogen and testosterone