Endocrine Flashcards

1
Q

What percentage of kids experience pubic hair growth as first sign of puberty?

A

20%

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2
Q

When do GnRH neurons migrate to hypothalamus

A

6-9 wga

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3
Q

When does FSH/LH secretion start in the fetus

A

12 weeks

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4
Q

When do fetal levels of FSH/LH peak

A

20-24 weeks

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5
Q

What happens to FSH/LH after delivery

A

GnRH pulsatility starts with FSH/LH rising with FSH > in females (peaks 12-18 months)

LH > males (peaks 3-6 months)

**Then there is change in central inhibition of pulsatile GnRH and high sensitivity to low levels of gonadal steroid feedback –> diphasic pattern of secretion

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6
Q

How does adrenarche start and when?

A

Age 6
Increased in responsiveness of adrenal glands to ACTH causing shift in sex steroid production
Decreased 3B HSD activity + increased activity in P450 Cyp 17
Shunts towards DHEA/DHEAS

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7
Q

Genes associated with early puberty

A

LIN28B, FTO, NEGR1

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8
Q

Shift in body composition associated with puberty

A

% body fat
16-23%

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9
Q

Leptin and puberty

A

Made by adipocytes and acts on CNS to regulate eating/energy balance
Leptin knockout mice have normal sexual development but are anovulatory

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10
Q

What is precocious puberty

A

Pubertal development occurring more than 2.5 SD earlier than the norm

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11
Q

Types of precocious puberty

A

GnRH Dependent: early activity of HPG axis
-constitutional (RUNS IN FAMILY) /idiopathic (does NOT RUN in family & onset is usually younger)
-CNS pathology
-Hypothyroidism

GnRH Independent:
-Sex steroid production independent of HPG axis
-Ovarian tumors
-McCune Albright
-Adrenal disease
-Exposure to estrogens

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12
Q

Hypothalamic hamartoma

A

Heterotopic neuronal masses of GnRH neurons that function as ectopic GnRH pulse generator
PP in first few years of life

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13
Q

Craniopharyngioma

A

Neuroepithelial CNS tumor
Disrupts normal pre-pubertal HP axis inhibition

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14
Q

Hypothyroidism and GnRH dependent PP

A

Delayed bone age (Thyroid hormones have permissive activity directly on bone and GH)
High TSH stimulates FSH-R and causes high prolactin and galactorrhea

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15
Q

Gonadoblastoma

A

complex tumor with a mix of gonadal elements
90% associated with abnormal karyotype, gonadal dysgenesis, or Y chromosome

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16
Q

Premature adrenarche

A

pubic hair < 8 yo
consider checking bone age to make sure there isn’t precocious puberty too (~20% have both)

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17
Q

Most significant outcome of precocious puberty

A

short stature because of premature epiphyseal closure

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18
Q

GnRH stimulation test

A

Check basal LH
leuprolide 20 ug/kg subcu
if LH > 5 IU/L suggests HPG axis activation
if LH is normal/low eg < 5 then likely to be GnRH independent

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19
Q

When to treat precocious puberty

A

-Sexual maturity advances to next stage in 3-6 months
-growth velocity > 6 cm/year
-bone age is advanced by > 1 year
-predicted adult height is < target range or decreased on serial measurements

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20
Q

Treatment for GnRH dependent precocious puberty

A

lupron
monitor symptoms and E2 (<10 pg/mL) at 3-6 month intervals

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21
Q

Delayed puberty

A

Absent or incomplete sexual maturation by age at which 95% of kids of same sex started pubertal development

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22
Q

Short stature

A

height 2 or more standard deviations below mean height for children of same sex/age
-Dx: familial/constitutional, chromosomal (downs/turners), idiopathic

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23
Q

Treatment of short stature

A

consider GH if height > 2.25 SD below mean

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24
Q

Treatment of tall stature

A

start treatment before menarche
ethinyl estradiol 15-30 ug (low dose OCP)
continue until epiphysis close

25
Metformin
Biguanide antihyperglycemic approved for T2DM not adequately managed by diet and exercise. Does not cause hypoglycemia Decrease hepatic glucose production, thus reducing the need for insulin secretion Decreases intestinal absorption of glucose. Lowers free fatty acid concentrations, thus reducing gluconeogenesis
26
Where is DHEA made?
Adrenal 50% Ovary 20% DHEAS conversion 30%
27
Where is androstenedione made?
Adrenal 50% Ovary 505
28
Where is testosterone made?
Adrenal 25% Ovary 25% Androstenedione conversion 50%
29
Insulin and PCOS
decreases SHBG increases activity of LH on ovary increase in IGF-1 potentiates response of ovary to LH
30
Hair stages
Anagen- growth Catagen- involution phase Telegen- quiescent phase
31
Clitoral index
> 35 mm (LxW)
32
Signs of virilization
deepening of voice hirsutism clitoromegaly breast atrophy muscle mass distribution
33
Spironolactone
aldosterone antagonist 50-100 mg BID SE: dyspepsia, nausea, fatigue, HA Feminization of male fetus
34
Eflornithine
Ornithine decarboxylase inhibitor inhibition of cell division and synthetic function 13.9% application SE: acne, folliculitis, pain/rash
35
Finasteride
5a reductase inhibitor inhibits type II mostly 5mg QD
36
flutamide
androgen receptor blocker 500 mg daily SE: green urine, hepatotoxicity
37
SRY gene
Gene encoding DNA-binding protein Located next to pseudo-autosomal region on short arm of Y Presence causes gonadal differentiation to testes No SRY - ovaries
38
Normal fasting insulin
25 mIU/L
39
Normal triglyceride
< 150 mg/dL
40
normal total cholesterol
200 mg/dL
41
HAIR-AN
Hyperandrogenic insulin-resistant acanthosis nigricans
42
Differential diagnosis for low E2 level in pregnancy?
placental sulfatase deficiency placental aromatase deficiency impending fetal demise fetal demise adrenal hypofunction drugs
43
Infant presentation with placental sulfatase deficiency
ichthyosis hyperkeratosis develops because of alteration in cholesterol: cholesteryl ester ratio from accumulation of cholesterol sulfate corneal opacities pyloric stenosis cryptorchidism
44
Amniotic fluid with placental sulfatase deficiency
Amniotic fluid has high levels of DHEAS, normal DHEA, and androstenedione
45
normal free T4
0.8-1.8 ng/dL
46
Premature ovarian insufficiency
<40 Elevated FSH and amenorrhea 1% of women and 90% idiopathic 5-10% may experience spontaneous ovulation
47
Ovulatory dysfunction for PCOS in adolescence
1. Lack of menarche by 15 yo or 3 yr s/p thelarche 2. Menarchal intervals < 21 or > 45 days (1-3 years post menarchal) 3. Persistent menstrual intervals < 21 or > 35 days or < 8 cycles/year > 3 years post menarche to menopause
48
What hormones do ovaries lack?
21 hydroxylase 11 beta hydroxylase **Favors androgen production
49
StAR
carrier protein aiding in cholesterol transport across the membrane functions in adrenals and gonads but NOT the placenta Congenital lipoid adrenal hyperplasia is caused by AR mutation in STAR causing males to have female external genitalia and adrenal glads to be filled with lipoid globules
50
Sequence of FSH/LH on receptors
FSH/LH bind to GPCR which stimulates adenylate cyclase and leads to release of cAMP and cholesterol transfer via StAR and nuclear steroidogenesis
51
What increases SHBG?
Hyperthyroidism Pregnancy Exogenous estrogen
52
What decreases SHBG?
Insulin IGF-1 Corticoids Androgens Progestins GH Prolactin
53
What proportion of hormones are bound to carrier proteins?
SHBG: 69% Albumin: 30% Free: 1%
54
how many carbons does cholesterol have
27
55
Function of FSH at granulosa cells
induce more FSH R induce transcription of aromatase leading to E2 production As follicle grows FSH induces LH R appearance on granulosa cells --> progesterone production (LH mediated)
56
Prolactin levels caused by meds?
25-100
57
What med can increase prolactin to 300?
Risperidone
58
What meds can cause hyperprolactinemia?
risperidone, olanzapine, haloperidol, bupropion, TCA, SSRI, reglan, cimetidine, prochlorperizine, verapamil, methyldopa, opiates, OCPs, transgender meds, medicinal herbs
59
How do dopamine agonists suppress prolactin?
DA bind to D2 receptors on lactotrophs and decrease intracellular cAMP and lower prolactin secretion