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Endo Repro 2 > Disorders of Puberty > Flashcards

Disorders of Puberty Flashcards

1
Q

Puberty is the period when ___ develop and capability of ___ is attained

onset at __yrs (__ yrs in AA)

Thelarche/pubarche has peak ___ and ____

occurs over ___yrs in girls

___ inc allows for growth of ___ tissues such as b__, v___ and u

A

sec sex char, sex repro

8-13, 7-13

ht velocity, menses

4.5

estrogen, estrogen sens, breasts/vulvovaginal tissue, uterus

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2
Q

1/2 of ___ is laid down in puberty, allowing for

mediated by

90% total body mineral content by ___

adolescents req __ of Ca daily, ___ of VD and __ exercise

Body fat inc after ___ in girls

deposition occurs on

ones who mature early at inc risk for

menarche prior to _ assc w inc bodyweight

A

total body Ca, bone growth

estrogen/GH

17

1200-1300mg, 400 IU, weight bearing

peak height velo

back/arms/thighs

obesity

12yo, bodyweight

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3
Q

Puberty has higher rates of __ in girls, __ at greatest risk

Inc risk of ___ in those maturing earlier

__ is not mature

Pubertal skin changes- inc ___ results in acne

could also indicate other conditions, such as

A

depression, Caucasian

behavioral disorders

prefrontal cortex

androgenic activity

PCOS, Non-classic CAH

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4
Q

Breast development

Stage 1
Stage 2- breast bud stage w ___, enlarged ___
Stage 3- enlarged __, no __
Stage 4 __ and ___ form a secondary mound above breast
Stage 5– _ stage
projection of __, related to recession of

A

prepubertal

elevation of breast/papilla, areola

breast/areola, separation

areola/papilla

mature
papilla, areola

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5
Q

Pubic hair

Stage 1 is ___ may have __ hair, similar to forearms

Stage 2- sparse growth of ___, can be curled at

Stage 3 becomes ___, spreads of jxn of

Stage 4- __ in type, covers smaller area, does not spread to

Stage 5- __ in type, with __ border

A

prepubertal, vellus

long, slight pigmented, base of penis/labia

darker, coarser, curled, pubes

hair adult, medial surface of thighs

adult, horizontal upper border

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6
Q

Precocious puberty is the manifestation of __ prior to age

Sub categories- __ dep PP (central)

____ indep PP (peripheral)

benign or ___ pubertal variants (incomplete PP)

A

sec sex charact, 8 in females

gonadotropin

Gonadotropin

non-progressive

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7
Q

GDPP (central)

Premature maturation/development of \

Seq is same as puberty but

Typically

Gonadotropin/E2 levels are
Bone age is ___, ___ growth

Other etiology: ___ of CNS, r____, h___, h___, c___, g___, previous

A

HPG axis

early

idiopathic

inc
advanced, linear

tumor/infect, radiation, hydrocephalus, head trauma, cerebral edema, gene mutations, high dose steroid exposure

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8
Q

GDPP labs- if ___ inc, it is consistent

GnRH agonist will make it

GIPP has excess

___ source

can be ___/__sexual

Gonadotropin levels are

With GnRH agonists, there is

A

gonadotropins

inc even more

estrogen, androgens

adrenal/ovarian/exogenous

iso/contra (virilization)

low (prepubertal)

no inc gonadotropins

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9
Q

GIPP etiology fxn ___, o___ such as granulosa cell tumors/gonadoblastomas, C__, a____, M___, Primary __ as TSH crossrx w ovarian ___ receptors

McCune Albright Syndrome

mutation in __ of G3 protein activating

manifests as

more common in

A

ovarian cyst, ovarian neoplasm, CAH, Mccune Albright syndrome, HypoT (FSH)

alpha subunit, AC

PP, cafe au lait, fibrous dysplasia

girls

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10
Q

Benign/NP IPP

can be broken into premature

Isolated __ dev <8 and no additional

must be monitored for progression to true __ every 3-6mnths

Despite breast develop in premature thelarche, estradiol level is

premature adrenarche, a total __, D___ and ___ should be eval along w

more common in pt w

inc risk of ___

if overweight, counsel on

measure bone age on

A

adrenarche/thelarche

breast/pubic hair, sec sex charact

PP

prepubertal

testo, DHEAS, 17OHP, bone age

darker skin

PCOS

exercise/diet changes

non dominant wrist

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11
Q

Premature thelarche is

Premature adrenarche is

Tx of GDPP

this prevents

also slows ___ and prevents

Right adnexal mass, look for ___ as a tumor marker for ___ tumors

if tumor level elevated, refer to Gyn/Onc and Peds Surg for

A

isolated breast devel wo other SSC

isolated pubic/axillary hair devel wo other SSC

GnRH agonist

premature closure of epiphyseal plates

skeletal maturation, progression of SSC

inhibin, granulosa cell

oophorectomy, tumor staging

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12
Q

If right adnexal mass is benign appearing cyst and simple, it is a

monitor for signs of

sx will resolve

Delayed puberty is the absence/incomplete devel of ___ bounded by age at which ___ of children of that sex/culture have ___ sex maturation

ie absence of breast devel in girls at an age > than ___ than pop mean, usually above 13

A

folllicular cyst

ovarian torsion

spontaneously

SSC, initiated

2-2.5yrs

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13
Q

Hypergonadotrophic Hypogonadism

Inc __ >30
Indicates ___
Gonadotropin inc secondary to dec hormones from

Ddx includes P___ T___, C___. S____

A

FSH
Ovarian failure
gonad

Premature Ovarian Failure, Turner Syndrome, Complete Gonadal Dysgenesis, Swyer Syndrome

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14
Q

Premature ovarian failure/insufficiency

Amenorrhea prior to
Elevated __ and low___
Secondary to accelerated atresia of ____
Assc w ___ and __

Pts may have G___, T___, s____

Further workup includes K____, t____, a___

Tx w

Baseline __ to eval bone health

A

40 yo
FSH, estradiol
ovarian follicles
Turner syndrome and Pure gonadal dysgenesis

Galactosemia, Trisomy 21, sarcoid

karyotype analysis, thyroid studies, adrenal gland

estrogen/progestin

DEXA

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15
Q

Turner Syndrome
45 ___ or 45 __, ___ or 45 ___, ___

B
S
W
S
L
H
M
G
M
P
L
L
A

45X or 45, X/46 or 45, X/46, XY

Broad chest
short stature
webbed neck
short 4th metacarpal
low hairline
High palate
Micrognathia
Genu valgum
Multiple pigmented nevi
Ptsosis
Low ears
Lymphedema
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16
Q 
Turner syndrome at risk for
C- bicuspid aortic valve, ARD, MVP
R- such as
H/D
O
S

Pts have ___ intell
Defects in ___ processing and ___ coordination
Can become preg w ___ but risk of ___ inc

A 
cardiac abnorm
renal abnorm- horshoe kidney
HTN, DM
Osteopenia
Streak gonads

normal
spatiotemporal, visual motor
Donor eggs, aortic rupture

17
Q

If TS is suspected, get
K

If Y chromo is present, gonads must be removed to dec risk of

Treat w

After several yrs, pt bw 12-15 needs

Goal of therapy is

Add ___ after first bleeding episode or after 1yr of

A

Karyotype

germ cell tumor

GH

exogenous estrogen therapy

breast/bone dev, menses, linear growth

progestin

18
Q

Pure Gonadal Dysgenesis
46__ or 46___ phenotypic females w

Inheritance
Elevated ___ bc streak gonads do not

___ to __ stature

46XY known as
W Y chromo, ___

No ___ so uterus is

A

46XX or 46XY streak gonads

Xlinked, AR

FSH, produce hormones

normal/tall

Swyer syndrome
remove gonads

AMH, present

19
Q

Previous radiation/Chemotherapy needs ___ prior to tx

Subsequent depot ___ to attempt ___, alternative is

Worse outcomes w alkylating agents and

A

FSH/E2

lupron, ovarian preservation, oophoropexy

older age

20
Q

Hypogonadotropic Hypogonadism

Dec
Gonads producing low hormone as response to

Reversible causes- c__, a__, primary ____, C___, P____, F____

irreversible causes: K____, H____, CNS lesion, c___

A

FSH
low stimulation

constit, anorexia, hypoT, CAH, prolactinoma, Female Athlete Triad

Kallman Syndrome, Hypopotuitarism, chronic dz

21
Q

Anorexia is more than ___ below ideal body wt
Can ___ or __ puberty

Can develop (3)

GI sx such as __, ___ changes, blue ___, hair ___

PE: oral ___, ___ appearance, ___ swelling

Image for ___ > 1yr

TX decide bw

get to ___ of IBW

consider

Complication

A

15%
delay/interrupt

DI, hypoT, inc cortisol

bloating, early satiety, const, hands/feet, loss

erosions, scaphoid, parotid gland

bone density

hospital/outpt psych

90-92%

prozac

inc mortality rate

22
Q

Anorexia DSM 5

(a)Restricted ___ relative req, leading to signif low ___ (less than minimally normal)

in context of

(b) Intense fear of ___. or becoming ___, or behavior that prevents

(C) disturbance in way of which ones BW/shape is ____, w self eval or lack of

Restrcting type: last 3m, individual has not engaged in recurrent episodes of _____/____

wl is accomplished by ___

A

energy intake, BW

age, sex, devel, physical health

gaining weight, fat, wg

experienced, recog

binge eating, purging

diet/exercise/fasting

23
Q

Binge eating/purging tye: indiv has engaged in

In partial remission- met criteria for ___, but ___ has not been met, but __ or __ is met

In full remission: after criteria for __ met, ___ others have been met

Mild BMI >
Mod bw
Severe BMI bw
Extreme BMI

A

these behaviors

AN, criteria A, Criteria B/C

AN, no

17
16-16.9
15-15.9
15

24
Q

Kallman Syndrome aka

Failure of GnRH neuron to migrate from ___ to

Assc w
No __ or
May have ___
Inheritance

Eugonadism- Normal __ and anatomic ____

assc w M____, T___, I ___, A____

A

Congenital GnRH defic

olfactory placode, hypothalamus

Anosmia/hypogonad
Breast/oubic hair
cleft lip/palate
Xlinked/AD

FSH, defect

Mullerian agenesis, Transverse vaginal septum, imperforate hymen, androgen insens

25
Q

Mayer Rokitansky Kuster Hauser

\_\_ dimple
Absent upper 2/3 \_\_, \_\_, \_\_\_
Ovaries
Image to rule out
genotype is
\_\_\_ testo level
A 
Vaginal
Vagina, cervix, uterus
present
renal anomalies
46xx
Female
26
Q

AIS- defect in
scant ___/___, blind pouch and breast from
Karyotype is
Elevated
Need ___ post puberty to avoid malignancy
No internal

Imperforate hymen is failure to complete ___ of the vagina

Blue bulge at level of

__ ab pain

Tx is cruciate ___ at the area

A 
androgen receptor
axillary/pubic hair, aromatization of androgen
46, XY
testo
gonadectomy
female strctur

canalization

introitus

cyclic

incision

27
Q

Transverse Vaginal Septum

Failure to complete ____ bw upper 2/3 (___) and lower 1/3 of vagina (__)

Cyclic ___

shortened ___, __ on imaging

Tx w

A

fusion, paramesonephric duct, UG sinus

pelvic pain

vagina, hematocolpos

surgery

28
Q

Delayed Puberty history

Look for initiation of __ and rate of

Neonatal history may indicate

Screen for

PMH for

FH may indicate

FSH determines if issue is from __ or __ origin
if elevated, due a

Repeat __ to conffirm

A

puberty, progression

Turner Syndrome, congenital abnormalities

eating do/physical/dz

chemo/rad, chronic illness

congenital do

gonad, central
karyotupe

elevated FSH

29
Q

TX Turner Syndrome w inc __ or ___ give __ to pubrerty and then

w inc FSH/nl chromos (POI) provide ___ to complete SSC, as this helps w

high FSH and 46XY (Swyer Syndrome)

low/nl FSH

A

FSH, 45X, GH, estrogen/progestin

HRT, vaginal atrophy, breast devel, bone mass

hormone replacement, excise streak gonads

rule out other causes

30
Q

Primary Amenorrhea is no SSC by __ or presence of characteristics but no ___ by 15

If pt has a uterus and no breasts, she is def in ___

think ___ or defect in ___

if she has breasts/no uterus there is ____

no breasts/uterus means

if she has everything, tx as if she has

A

13, menses

estrogen

gonadal failure, HPO axis

mullerian abnorm, AIS

rare defect, gonadal dysgenesis, agonadism, MIS

secondary amenorrhea

Endo Repro 2 (24 decks)

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