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Phase 3a > Puberty > Flashcards

Puberty Flashcards

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1
Q

what is the definition of premature puberty

A

development of secondary sexual characteristics before 8y (female) and 9y (male)

aka precoucious puberty

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2
Q

what are the two types of precoucius puberty

A

central

periferal

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3
Q

what is central precoucius puberty

A

dependant of gonadotrophins through premature activation of HPG axis

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4
Q

what is periferal precoucius puberty

A

actually pseudo precoucious pubert as there are

normal gonadotrophins

but XS sex steriods

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5
Q

what is the mechanism of central precoucius puberty

A

HPG axis is activated causing an increase in GnRH

Release of FSH and LH causing increase in levels

Activation of gonads causing +in sex hormones

(oestrogen-ovaries or testosterone)

development of secondary sexual characteristics

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6
Q

what is the mechanism of peripheral precoucius puberty

A

HPG axis not activated, no pituitary hormones produced (GnRH, FSH, LH)

so hormones come from gonads or are exogenous

theres increased oestrogen or testosterone causing development of secondary sexual characteristics

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7
Q

what are causes of central precoucius puberty

A

idiopathic

hydrocephalus

hypothyroidism

neurofibromatosis

acquired: -infections, tumours, irradiation

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8
Q

what are causes of periferal precoucius puberty

A

adrenal tumours/ CAH

ovarian tumours

testicular tumours

exogenous sex steriods

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9
Q

how would you manage precoucius puberty

A

treat underlying cause

GnRH analogues = central

androgen/oestrogen blockers = periferal

  • medroxyprogesterone
  • testolactone
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10
Q

what is teh definition of Thelarche

A

development of breast tissue (breast budding)

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11
Q

at what age does thelarche usually occur

A

6m-2y

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12
Q

what is the presentation of thelarche

A

palpable breast tissue with some enlargement of the areola

but no separation between the contours of the two breasts

no pubic hair or growth spurt

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13
Q

what is the definition of Adenarche

A

early pubic hair growth w/o other sexual characteristics

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14
Q

what causes adenarche

A

early maturation of adrenal glands

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15
Q

why is it important to Ix adenarche

A

exclude causes of central precaucious puberty such as adrenal tumours

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16
Q

what Ix would you perform for adenarche

A

US

Urinary steriod profile

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17
Q

what is the treatement of adenarche

A

if theres no central cause then nothing

is benign and self limiting

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18
Q

what is congenital adrenal hyperplasia

A

a number of autosomal recessive disorders resulting in adrenal hyperplasia

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19
Q

what is the pathophysiology of congenital adrenal hyperplasia

A

due to mutations theres a decrease in cortisol en utero

this means the fetal pituitary releases +++ ACTH to try and increase cortisol levels

this causes an overgrowth in adrenal cells

this causes an increasein androgens

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20
Q

what are the two types of patient types in congenital adrenal hyperplasia

A

salt loosers

non salt loosers

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21
Q

what is the infant presentation of salt loosing congenital adrenal hyperplasia

A

vomiting

weight loss

floppy baby due to circulatory collapse

@1-3 weeks

this is an adrenal crisis!!

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22
Q

when do non salt loosing congenital adrenal hyperplasiapatients present

Study These Flashcards
A

at puberty due to precocious puberty

23
Q

what is the classic phenotype of a male in congenital adrenal hyperplasia

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A

enlarged and overpigmented penis

precoucous puberty

tall and muscular

24
Q

what is the phenotype of females with congenital adrenal hyperplasia

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A

clitoral hypertrophy + vaginal fusion of the labia

muscular build

precoucious puberty

25
how would you diagnose congenital adrenal hyperplasia
bloods * 21-hydroxylase (-) * 17-hydroxyprogesterone (+) karyotype testing if genitals are ambiguous
26
what Ix would yoou perform in a ?adrenal crisis
plasma Na (-) Plasma K (+) hypoglycaemia metabolic acidosis
27
what is the management of a non salt loosing congenital adrenal hyperplasia
administer glucocorticoids +/- reconstructive surgery
28
how do glucocorticoids work in the management of congenital adrenal hyperplasia
decrease ACTH so less andorgens allows for normal growth and maturation
29
what is the management of salt loosing congenital adrenal hyperplasia
glucocorticoids = (cortisol replacement) minaralocorticoids = (aldosterone replacement) salt suppliments +/- reconstructive surgery
30
what is the Mx of an adrenal crisis
IV saline + dextrose IV hydrocortisone
31
what can cause disorders of sexual differentiation
XS androgens in females (CAH) inadequate androgen action in males gonadotrophen insufficiency (prader - willi ie hypopituitarism) Ovotesticular disorder
32
what are the three ways there can be infficient androgen action
androgen insensitivity inability to convert testosterone to dihydrotestosterone inability to converst cholesterol to androgens
33
what enzyme converts testosterone \> dihydrotestosterone
5 alpha reductase
34
what is ovotesticular disorder of sexual differentiation
when the karyotype = XXY both ovarian and testicular tissue is present often have a complex external phenotype
35
how would you Ix problems of disorder of sexual differentiation
karyotype chromosomal screening bloods: * adrenal hormones * sex hormones US - gonads + otherinternal structures
36
how would you manage disorder of sexual differentiation
gender reassignment surgery gonadectomy HRT councelling * family * individual
37
what are the most common types of hypothalamic tumours
hypothalamic glioma
38
what is the Px of a hypothalamic glioma
precoucious puberty failure to thrive cachexia euphoric hyperactive headaches IC pressure Sx visual loss
39
how would you Ix a hypothalamic tumour
CT/MRI - head
40
how would you manage hypothalamic umours
surgery +/- adjuvants
41
what is the differentiation of delayed puberty
absence of pubertal development by 14 (female) and 15 (male)
42
what are causes of delayed puberty
familial low gonadotrophins * systemic: chrons, CF, anorexia * Hypothalamo-pituitary disorders: IC tumours, panhypopituitarism Gonadal problems * + in GH, FSH, LH, GnRH * chromosomal ie turners andkleinfelters * aquired gonadal damage
43
what is the Px of delayed puberty
short duing childhood delayed sexual maturation Delayed skeletal maturation andbne age longer legs than trunk
44
how would you diagnose delayed puberty in males
check testicular volue for pubertal staging screen for systemic disorders chech testosterone chromosomal screen GnRG stimulation test
45
how would you Ix delayed puberty in females
LH, FSH testing chromosomal screen TFT GnRH stimulation test
46
how would you manage delayed puberty
often normal = reassurance BUT Oxanarolone in boys as doesnt deveop 2ry sex characteristics then testosterone in men oestrodiol in females
47
what is prader-willi syndrome
a generic disorder from deletion of chromosome 15 from father causing growth, pubertal and neurodevelopmental issues
48
on US you notice a fetus has: decreased movements polyhydramnious is Breech and you beleve the mum will need c section what is the cause of this
prader-willi syndrome
49
what are features of childhood prader-willi syndrome
striabasmus scoliosis dev delay
50
what are teenage features of prader-willi syndrome
+ apetite and weight short stature narrowed upper forehead small hands OCD like Hypogonadism infertility Hypotonia
51
what is Kallman syndrome
a genetic disorder causedby multiple genes causing hypogonadotrophic hypogonadism thus preventing a person from reaching full puberty
52
what is the Px of Kallman syndrome
lack of testicular development or amenorrhoea - gonadotrophins cleft pallete/lip cerebra ataxia total lack of sense of smell!! 2ry osteoporosis + osteopenia
53
how do ou diagnose Kallman syndrome
tanner stage testicular checks + penis chechs/ breast/menarch checks bloods * FSH,LH, testosterone, oestrogen, prolactin GnRh+hCG stimulation test smell test
54
how would you manage Kallman syndrome
oestrogen + progesterone testosterone GnRH Pulsatile therapy

Phase 3a (22 decks)

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