3 - Puberty and Pubertal Disorders Flashcards
What is pubarche?
appearance of pubic hair
What is the first line treatment for Mullerian Agenesis?
create neo-vagina with dilators
In boys, puberty is considered precocious if it occurs before this age.
< 9 years old
In this condition the upper vagina and uterus fails to form or fuse as a result of a failure of the lateral fusion of mullerian ducts.
Mullerian Agenesis
What is the diagnostic criteria for POI?
amenorrhea plus elevated FSH levels on 2 separate occasions
remember hormones secreted in pulsatile fashion
What 2 factors result in males generally being taller than females?
- have 2-3 years of extra “pre-pubertal growth”
- have larger max growth spurt
Growth spurt in boys usually occur in between these Tanner stages
Tanner stage 3-4
How long after the growth spurt do FEMALES begin gaining weight?
6-9 months later
How is peripheral PP treated?
treat underlying cause
Cafe au lait spots and polycistic lesions in the setting of precocious puberty is consistent with this diagnoses.
- McCrune Albright Syndrome
What cells are responsible for secreting AMH in males?
Sertoli cells
In relation to the growth spurt, when do females on average have menarche?
menarche occurs 6 months after growth spurt
This needs to happen for puberty to be complete.
full achievement of reproductive capabilities (ovulatory cycles needs to be present)
What is the first line treatment for POI?
Transdermal 18 B-estradiol [estrogen] - start low and increase every 6 months
add progesterone later (if added too early will mess up thelarche)
What is the most common cause of central PP?
idiopathic (85%)
What is the most common anatomical cause of delayed puberty?
imperforate hymen
Menarche should coincide with what bone age?
age 13
What is the most common cause of delayed puberty in females?
primary ovarian insufficiency
A short stature, streak gonads, and a webbed neck is consistent with this phenotype.
Turner’s Phenotype
An increase in this hormone leads to an older bone age.
Estrogen
At what breast Tanner stage do females most often have menses?
Tanner Stage 4 (62%)
What is the first sexual sign of puberty in 85% of girls?
thelarche
Amenorrhea with blood behind the hymen (e.g. hematocolpos) is a hallmark sign of this diagnosis.
imperforate hymen
What is the most common type of incomplete precocious puberty?
precocious thelarche
This condition is characterized by an absence of some or all of an X chromosome.
Turner’s Syndrome
What diagnostic study is used to assess bone age?
xray of non-dominant hand and wrist
What is thelarche?
appearance of breast tissue
How does obesity affect the onset of puberty?
obesity leads to an earlier onset of puberty
What is the most common cause of Karyotype causing delayed puberty?
Turner’s Syndrome
What is menarche?
when first menses occurs
How is central PP medically treated?
GnRH agonists - Synarel or Lupron
How is an imperforate hymen treated?
hymenectomy
What ducts form the uterine?
mullerian (paramesonephric) ducts
What test do you use to differentiate between central and peripheral PP?
GnRH stimulation test
How is delayed puberty defined in females?
- no menses by age 15
- no secondary sexual characteristics by age 13
What findings on the GnRH stimulation test are consistent with central vs. peripheral PP?
- central = LH > FSH
- peripheral = FSH > LH
What is the most common neoplasm associated with delayed puberty?
craniopharyngioma
Breast development should coincide with what bone age?
age 11
What is the necessary treatment for a patient with Swyer’s Syndrome?
remove gonads d/t greater risk of malignancy
What is the most common cause of delayed puberty in males?
constitutional
On average this sexual sign follows thelarche in 85% of girls.
pubarche
How is precocious puberty defined in girls?
Pubertal development 2.5 SD below mean
prior to age 6 in AA, prior to age 7 in caucasian
Adolescent menstruation has a cycle range of how many days?
21-45 days
What is the most common type of PP in girls?
central or GnRH dependent
What are the 2 main consequences of precocious puberty in girls?
- shorter adult height
- increase risk of sexual abuse
T/F: Hormonal changes occur before physical signs of puberty.
True
In males what is the relationship between height and weight gain during puberty?
males gain height and weight at the same time
Absence of this hormone results in hypogonadal hypogonadism which results in the patient being unable to enter puberty.
kisspeptin
This hormone produced by adipocytes is required for the onset of normal puberty.
leptin
How is premature (precocious) thelarche defined?
onset of thelarche prior to age 7 in whites, 6 in blacks
In this condition there is a mutation of the SRY gene resulting in non-functioning ovaries. The patient will not have a growth spurt or breast development. They will have a vagina, uterus. The gonads will be testicles and streak.
Swyer’s Syndrome
What is the first somatic sign of puberty in girls?
pubertal growth spurt
Patients with Turner’s syndrome are at an increased risk of this life threatening vascular complication.
thoracic aortic rupture
what is the most common cause of oligomenorrhea?
PCOS
