B8-060 Delayed Puberty

Question 1

[hormone] stimulates the development of secondary sex characteristics in females

Answer 1

estrogen

Question 2

[hormone] is mildly anabolic and stimulates calcification

Answer 2

estrogen

Question 3

[hormone] regulates behavior and secretion of gonadotropins

Answer 3

estrogen

Question 4

[hormone] induce body hair

Answer 4

androgens

Question 5

[hormone] influences sexual behavior

Answer 5

androgens

Question 6

[hormone] promotes protein anabolism, somatic growth, and ossificaiton

Answer 6

androgens

Question 7

[…] can be a significant source of androgen production in females

Answer 7

adrenal cortex

Question 8

during puberty, […] provides negative feedback on the hypothalamus, leading to low levels of LH/FSH

Answer 8

estrogen

Question 9

adrenarche refers to the production of […] a couple years prior to puberty

Answer 9

DHEA

Question 10

one of the first changes occurring pre-puberty is the nocturnal rise in […]

Answer 10

GnRH

(leads to increased LH)

Question 11

in childhood, high amounts of [neurontransmitter] inhibit GnRH neurons

Answer 11

GABA

Question 12

during puberty, inhibitory GABA decreases and excitatory […] increases GnRH [3]

Answer 12

glutamate
NMDA
kisspeptin

Question 13

how does the negative feedback of estrogen change throughout life?

Answer 13

in childhood, low levels of estrogen are needed to suppress GnRH

however, with age those low levels are no longer enough. Requires higher levels of estrogen to maintain LH/FSH

(decreasing sensitivity)

Question 14

in hypogonadotropic hypogonadism, LH and FSH will be

Answer 14

low

(problem at level of pituitary)

Question 15

in hypergonadotropic hypogonadism, LH and FSH will be

Answer 15

high

(problem at level of gonads)

Question 16

Kallmans is […]gonadotropic hypogonadism

Answer 16

hypo

(neurons fail to migrate, anosmia)

Question 17

Klinefelter’s is […]gonadotropic hypogonadism

Answer 17

hyper

Question 18

Turner’s is […]gonadotropic hypogonadism

Answer 18

hyper

Question 19

[what Turner stage]

no sexual hair
flat-appearing chest with raised nipples in females

Answer 19

stage 1

(pre-pubertal)

Question 20

[what Turner stage]
pubic hair appears
testicular enlargement
breast buds form

Answer 20

Stage 2

(8-11.5 years)

Question 21

[what Turner stage]
coarsening of pubic hair
penis size increases
breast enlarges

Answer 21

Stage 3

(11.5-13 years)

Question 22

[what Turner stage]
coarse hair across pubis sparing thigh
penis width/glans increases in size
breast enlarges, raised areola

Answer 22

Stage 4

(13-15 years)

Question 23

[what Turner stage]
coarse hair across pubis and thigh
penis/testis reach adult size
adult breast contour, areola flattens

Answer 23

Stage 5

> 15 years

Question 24

earliest detectable secondary sexual characteristic is […] in females, and […] in males

Answer 24

breast bud development
testicular enlargement

Question 25

precocious puberty is the appearance of secondary sexual characteristics under age [...] in females and [...] in males

Answer 25

8 in females 9 in males

Question 26

central precocious puberty is caused by an increase in [...] secretion

Answer 26

GnRH (can be idiopathic or due to CNS tumors)

Question 27

peripheral precocious puberty is [...] independent

Answer 27

GnRH due to increased sex hormone production of exposure to exogenous sex steroids

Question 28

causes of peripheral precocious puberty

Answer 28

congenital adrenal hyperplasia granulosa/leydig cell tumors

Question 29

delayed puberty is defined as the absence of secondary sex characteristics by age [...] in females and [...] in males

Answer 29

13 in females 14 in males

Question 30

chemotherapy/radiation would cause [...]gonadotropic hypogonadism

Answer 30

hyper

Question 31

male 47 XXY

Answer 31

klinefelters

Question 32

small firm testis infertility tall stature gynecomastia female hair pattern

Answer 32

klinefelters

Question 33

female 45 XO

Answer 33

Turners

Question 34

short stature streak ovary broad chest, widely spaced nipples webbed neck

Answer 34

Turners

Question 35

defective migration of neurons and subsequent failure of olfactory bulbs to develop due to defective NCAM

Answer 35

Kallmanns

Question 36

inheritance pattern of Kallmanns

Answer 36

X linked (can be dominant or recessive)

Question 37

what causes peak height velocity near puberty?

Answer 37

increased production of gonadal steroids increased secretion of GH and IGF1

Question 38

what causes the sharp decline in peak height velocity near puberty?

Answer 38

increased production of gonadal steroids also promotes calcification and closure of epiphyseal plates

Question 39

homozygous mutation causing loss of ESR1 function causes

Answer 39

estrogen resistance symptoms: amenorrhea, lack of breast development, epiphyseal plates don't close

Question 40

adverse effects of androgen therapy in adolescents

Answer 40

premature closure of epiphyseal plates polycythemia decreased spermatogenesis increased LCL male pattern baldness

Question 41

growth spurt in girls typically corresponds to Tanner Stage [...]

Answer 41

3

Question 42

defect in androgen receptor resulting in female appearing genetic male

Answer 42

androgen insensitivity syndrome

Question 43

female external genitalia with scant axillary or pubic hair rudimentary vagina uterus/fallopian tubes absent

Answer 43

androgen insensitivity syndrome (patients develop normally functioning testis)

Question 44

female phenotype absent uterus presence of breast development

Answer 44

androgen insensitivity syndrome in genotypic male (T can be aromatized to estrogen allowing for breast development)

Question 45

female presenting with: primary amenorrhea reduced/absent body hair absent breast development developmental delay

Answer 45

Kallmann syndrome (vs Turners: normal intelligence and have some body hair/breast development)

Question 46

what factors contribute to the "juvenille hiatus" [4]

Answer 46

elevated sensitivity of the hypothalamus to estrogen high levels of inhibitory GABA in hypothalamus low levels of kisspeptin secreted secretion of estrogen from the ovary in females