puberty and its disorders Flashcards
what is puberty ?
it is the period of transition from sexual immaturity to sexual maturity
what are the stages of puberty in order ?
- breast development ( Thelarche)
- Accelerated growth
- appearance of pubic hair (pubarche)
- Menarche
- Appearance of axillary hair (Axillarche)
- regular ovulatory cycles
what is the hormone responsible for breast development ?
estrogen effect
what hormones are responsible for the accelerated growth phase of puberty ?
growth hormone and oestrogen
what is the hormone responsible for the appearance of pubic hair (Pubarche) ?
adrenal androgens effect
what is another term used for pubarche ?
adrenarche
what is tanner staging used for ?
used to assess adolescents’ physical development during puberty in five stages
what does tanner stage 1 indicate ?
prepubertal
what does tanner stage 5 indicate ?
adult or mature age
what is the definition of delayed puberty ?
no secondary sexual characteristics by the age of 13-14, or no menses by the age of 15-16
what are the two main causes of delayed puberty ?
primary hypergonadotrophic hypogonadism
secondary hypogonadotrophic hypogonadism
what are the causes of primary hypogonadism ?
Turner syndrome
gonadal dysgenesis or agenesis
premature ovarian failure
autoimmune ovarian failure
what are the causes of secondary hypogonadism ?
- constitutional delay of growth
- genetic e.g kallman’s syndrome
- functional: due to stress or malnutrition
what is the difference between hypergonadotrophic hypogonadism and hypogonadotrophic hypogonadism ?
- hypergonadotrophic hypogonadism: sufficient amounts of hormones are being produced but the gonads are unresponsive
- hypogonadotrophic hypogonadism: not enough sex hormones are being produced due to a problem in the pituitary or the hypothalamus
what aspects do we look for when examining a patient with delayed puberty ?
- secondary sexual characteristics
- turner syndrome features
- serial weight and height measured against a growth curve
what investigations would you ask for ?
Xray for bone age FSH, LH and E2 genetic analysis ( turner) olfactory testing ( kallman's syndrome ) pelvic imaging
what is the management of delayed puberty ?
treatment of the cause
estrogen therapy to promote the appearance of 2ry sexual characteristics and to induce menses
what is precocious puberty ?
appearance of secondary sexual characteristics before 8 years of age with or without onset of menstruation
what are the types of precocious puberty ?
central PP
peripheral PP
benign and non progressive pubertal events
what is the aetiology behind central PP ?
it is GnRH dependent , due to the premature activation of hypothalamic-pituitary- gonadal axis
what is the most common cause of central PP ?
constitutional
what is the aetiology behind peripheral PP ?
not GnRH dependent but due to excessive sex hormones
what are the types of peripheral PP ?
isosexual precocious puberty
heterosexual precocious puberty
what is isosexual precocious puberty ?
secondary sexual characteristics that match with genetic and phenotypic sex
what are the causes of isosexual precocious puberty ?
estrogen producing ovarian or adrenal tumors
exogenous intake of oestrogen through foods or drugs
what is heterosexual precocious puberty ?
secondary sexual characteristics that do not match up with genetic and phenotypic sex
what are the causes of heterosexual precocious puberty ?
androgen producing adrenal and ovarian tumors
occult exogenous androgen intake
congenital adrenal hyperplasia
what is benign and non progressive pubertal events ( or false precocious puberty ) ?
isolated premature :
adrenarche or
menarche or a combination of them which is usually a benign condition
what to look for during the examination of an individual with precocious puberty ?
serial height and weight measured against a growth chart
secondary sexual characteristics
what investigations would you carry out for a case of precocious puberty ?
X ray for bone age
FSH LH and E2
what is the treatment of precocious puberty ?
aim is to slow down the accelerated growth especially before the closure of epiphyseal plate :
long acting GnRH agonist ( histrelin )
otherwise treat the cause ( excise tumor etc ..)
what is the presentation of kallman’s syndrome
small penis
undescended tester
loss of smelling sense
lack of secondary sexual characteristics
what are the features of Turner’s syndrome ?
Wide or weblike neck.
Low-set ears.
Broad chest with widely spaced nipples.
High, narrow roof of the mouth (palate)
