Pediatrics Repro (Dow) Flashcards
adrenarche
Adrenarche is the activation of the adrenal cortex for the production of adrenal androgens, and typically occurs before the onset of puberty.
Pubic and axillary hair may appear before, at about the same time, or well after the appearance of breast tissue
thelarche
usually the first and most obvious sign of early puberty
this is the appearance of breast tissue
what is the “mini puberty of infancy”
the hypothalamic-pituitary-gonadal axis is biologically active in infancy with peak activity between six and eight weeks of age. This state yields sex steroid levels comparable with those seen in early-to-mid puberty, but without peripheral effects.
Hormonal changes after birth: falling levels of maternal estrogens
65-90% of neonates have breast tissue
Vaginal discharge can be observed.
“Witches’ milk” from breasts and even menses can be seen
Infantile acne also common
which have earlier puberty:
caucasian or african american girls
African american girls
b/c of genetics and race
when is the growth spurt in girls as compared to boys
two years earlier in girls
increased BMI
- early due to lean body mass
- later due to fat
what are some puberty related issues
anemia
Gynecomastia - one half of teenage boys - goes away
Acne
Psychosocial correlates of puberty
myopia Scoliosis STI's Dysfunctional uterine bleeding asthma
in male genitalia, what are the early signs of puberty?
testicular length more than 2.5 cm
OR
testicular volume (with Prader orchidometer beads) is 4 mL or more
stage 2 breast development
breast bud stage with elevation of breast and papilla
enlargement of areola
stage 3 breast development
further enlargement of breast and areola
no separation of their contour
stage 4 breast development
areola and papilla form a secondary mound above level of breast
stage 5 breast development
mature stage
projection of papilla only
related to recession of areola
stage 2 pubic hair development
sparse growth of long slightly pigmented hair straight or curled along labia
stage 3 pubic hair development
darker coarser and more curled hair
spreading sparsely
stage 4 pubic hair development
hair adult in type
but covering smaller area than in adult
no spread to medial surface of thighs
stage 2 external genitalia males
enlargement of scrotum and testes
stage 3 external genitalia males
enlargment of penis (length and first)
further growth of testes
stage 4 external genitalia males
increased size of penis with growth in breadth and development of glans
testes and scrotum larger
scrotal skin darker
is precocious puberty more common in boys or girls
girls
age of precocious puberty for boys and girls
age 8 girls
age 9 boys
early growth spurt in precocious puberty causes tall stature but rapid bone maturation. why is this a problem?
can cause premature and rapid skeletal maturation and fusion of the epiphyses –> can cause linear growth to cease too early and can result in short adult stature
evaluate with BONE AGE
central percocious puberty
what is it?
causes?
early matureation of the entire hypothalamic pituitary gonadal axis. Gonadotropin dependent
with the full spectrum of physical and hormonal changes of puberty
Causes:
1) idiopathic most of the time > 80 percent (a diagnosis of exclusion)
- girls you want to just slow their puberty down a bit
- boys you want to explore for a tumor
2) CNS abnormalities:
- Hypothalamic hamartomas (secrete GnRH)
- Tumors
- Acquired CNS injury
- congenital anomalies
3) Previous excess sex steroid exposure (rare)
peripheral percocious puberty
causes?
gonadotropin independent
1/5 th as common as central precocious puberty
peripheral secretions of sex hormones from:
Gonads
-ovarian cysts or tumors - most common cause in girls
Male limited syndromes
(FMPP) - familial male precocious puberty
Adrenal glands
- tumor
- CAH
- ectopic HCG by germ cell tumor
Exogenous sources of sex steroids
-possibly soy
McCune Albright
incomplete percocious puberty
Premature pubarche and premature thelarche are two common, benign, normal variant conditions that can resemble precocious puberty but are nonprogressive or very slowly progressive.
premature thelarche
Premature thelarche refers to the isolated appearance of breast development, usually in girls younger than 3 years;
premature pubarche
premature pubarche refers to appearance of pubic hair without other signs of puberty in girls or boys younger than 7-8 years (usually progression is slow, so ultimate height is okay)
how do you tell the difference b/w peripheral and central percocious puberty
measure LH levels
In central precocious puberty (GDPP), basal LH levels are often elevated into the pubertal range and show a pubertal (heightened) response to GnRH stimulation.
In peripheral precocious puberty (GIPP), the LH level is low at baseline and fails to respond to GnRH stimulation
central precocious puberty in girls
what are the findings?
breast enlargement
- usually the first and most obvious sign of early puberty
- due to increased estrogen production
Pubic and axillary hair may appear before, at about the same time, or well after the appearance of breast tissue
axillary odor
menarche - 2-3 years after breast development
enlargement of the clitoris is androgen excess and must be investigated
investigate severe acne
central precocious puberty in males
what are the findings
The earliest sign of central precocious puberty (CPP) is enlargement of the testes, which depends on increased production of follicle-stimulating hormone (FSH); testicular length is more than 2.5 cm or testicular volume (with Prader orchidometer beads) is 4 mL or more
subtle finding that often goes unnoticed by patients and parents
Other signs of puberty (eg, penis growth, reddening and thinning of the scrotum, increased pubic hair) are a consequence of increased testosterone production and occur within 1-2 years after testicular enlargement.
Accelerated linear growth (the pubertal growth spurt) occurs later in the course of male puberty than in female puberty but often occurs by the time other physical changes are noted.
what is the meaning of pubic hair that grows without penis and testicular enlargement and other signs of increased androgen produciton
Pubic hair growth that occurs without penis and testicular enlargement and other signs of increased androgen production is less likely true puberty - eg, premature adrenarche or a mild, nonclassic form of congenital adrenal hyperplasia.
What is McCune Albright syndrome
has 2 of the following features:
Polyostotic fibrous dysplasia
- many fx’s early
- gait anomalies, bony deformities
Cafe au lait skin
autonomous endocrine hyperfunction
- peripheral percocious puberty (more in girls)
- *** they sometimes have menarche first
other endocrine syndromes
- hyperthyroid
- acromegaly
- Cushings
what on physical exam is important in evaluating for precocious puberty
growth velocity
androgenic effects:
-acne, hirsutism, increased muscle mass and clitoromegaly ***
estrogenic effects:
-breast development and changes in vaginal mucosa (more pink and thickened with puberty)
how do you measure bone age
radiograph of the nondominant hand and wrist
Vital in the evaluation of sexual precocity; always advanced for chronologic age in cases of pathologic precocious puberty
to distinguish between benign early adrenarche and precocious puberty
Helps determine whether treatment to stop additional development will be of value
how do you work up precocious puberty
bone age **
LH levels ***
- high in central with a heightened response to GnRH stimulation
- low in peripheral and fail to respond to GnRH stimulation
Testosterone, estradiol, LH, FSH, cortisol, DHEA, 17-hydroxyprogesterone
HCG in boys
in boys that you suspect have precocious puberty, what must you perform in the workup
MRI of the head
The younger the child with central precocious puberty, the greater the chance of finding CNS pathology (among children younger than 6 years).
For boys younger than 9 years, the incidence of CNS findings is much higher than in girls, and MRI should be part of the evaluation.
what is the primary goal of treatment for GDPP (central)
allow a child to grow to a normal adult height
GnRH analogs or agonists
- Leuprolide
- increase final adult height
resection of mass if possible
treatment of peripheral percocious puberty
Does NOT respond to GnRH agonist therapy
surgical managment (if tumor of testis, adrenal, ovary)
how do you treat McCUne Albright
aromatase inhibitors to inhibit estrogen synthesis or block estrogen action
what is the most frequent type of CNS tumor to cause precocious puberty in very young children
hypothalamic hamartoma
early morning vomiting and headaches?
tumor in head
what is the classic triad of hypothalamic hamartoma
precocious puberty
developmental delay
seizures- laugh uncontrollably
how do you treat idiopathic precocious puberty
treat with GnRH (leuprolide)- delay the puberty
more common in girls
benign thelarche
know this!
effects of leuprolide
stopping closure of the epiphyses so they get taller
