endocrinology of puberty Flashcards

1
Q

define puberty in a nutshell

A

transition from sexual immaturity to sexual maturity.

achieving fertility, growth in stature and secondary sex characteristics

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2
Q

what hormones controlling puberty are released in the brain

A

FSH, LH, GnRH, neuroendocrine

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3
Q

what hormones contribute to female puberty

A

oestradiol, progesterone, adrenal androgen precursors

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4
Q

what hormones contribute to male puberty

A
Testosterone
Dihydrotestosterone
(DHT)
Adrenal androgens
Oestradiol( don't overlook this for growth)
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5
Q

define gonadarche

A

activation of gonads by fsh and lh

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6
Q

define adrenarche

A

increase in androgen production by adrenal cortex

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7
Q

define thelarche

A

appearance of breast tissue

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8
Q

what causes thelarche

A

estrogen from ovaries

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9
Q

define menarche

A

first menstrual bleed, non ovulatory

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10
Q

define spermarche

A

first sperm production, nocturnal sperm emissions

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11
Q

define pubarche

A

appearance of pubic hair, first appearance of axillary hair apocrine body odour and acne

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12
Q

what does (estradiol/oestradiol/estrogen) all the same thing contribute to in puberty

A

breast devlopment, growth accelarator, skeletal maturation

menstruation along with progesterone

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13
Q

At what point can hormones contributing to puberty be tested if mother is concerned about her child

A

best time is after birth where mini puberty of infancy occurs if this period is missed it is very hard to ascertain whether the hpa axis and gonads are communicating and functioning until expected start of puberty

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14
Q

when do girls start puberty on average

A

8yrs

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15
Q

what age do boys start puberty at

A

9 years

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16
Q

what are tanner stage in regards to boys and girls

A

tanners stages are stages of development, for genitial changes in boys and breast growth in girls. 5 stages
considers pubic hair growth testicles descent and size, and breast growth

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17
Q

what five factors contribute to timing of puberty

A

race, genetics, nutritional status, health, enviroment

18
Q

what other medical changes can be associated with

A

acne, gynecomastia(breast development) , anaemia

19
Q

define precocious puberty

A

when puberty starts early 2-2.5 standard deviations earlier than the norm. could be normal development or pathological cause

20
Q

define true central precocious puberty and it’s key characteristics

A

Gonadotropin dependent
• Early maturation of the HPG axis
• Sequential maturation
sexual characteristics have to be appropriate for the child gender

21
Q

define key characteristics of of peripheral precocity

A
Gonadotropin independent
• Excess of secretion of sex
hormones – gonads, adrenal
glands, exogenous sources of
sex steroids, ectopic production
of gonadotropin from a germ
cell tumour
• Non sequential maturation
• Isosexual or contrasexual
22
Q

state 2 BENIGN

PUBERTAL VARIANTS

A

premature adrenarche and thelarche, they dont generally cause long term impact to puberty, isolated incident.

23
Q

who is premature adrenarche (an example of a benign pubertal variant) more common in and what are potential risks and implications

A

afro carib and hispanic girls, can cause priming therefore risk of TCPP, also risk of PCOS in girls.

24
Q

describe characteristics of premature thelarche(benign pubertal variant)

A
diopathic
• Around two years of age
• Waxes and wanes – Does not progress
• Isolated breast development / Not beyond Tanner stage 3
• Absence of other secondary sexual characteristics
• Normal height velocity for age
• Normal or near normal bone age
• LH and FSH between normal range
25
Q

central precocious puberty what do you expect to find

A

advanced bone age, acclerated linear growth, pubertal levels of FSH and LH

26
Q

What causes TCPP

A

can be idiopathic(no clue what did )
CNS lesion, trauma affecting homromnal axis as a result,
genetic loss of function in MKRN3 or gain in KISS1 and KISS1r.
Pituitary gonadotrophin secreting tumours

27
Q

what causes periphial precocity in girls

A

ovarian cyst, ovarian tumours

28
Q

CAUSES OF PERIPHERAL PRECOCITY - BOYS

A

leydig cell tumours,

Mutation in Lh receptor gene causing lh cell to mature to quick, Familial male-limited precocious puberty (testotoxicosis)

germ cell tumours (• Germ Cell tumours secrete hCG
• hCG secretion activates LH receptors on Leydig cells Testosterone production
• Located in gonads, brain, liver, retroperitoneum, anterior mediastinum
• Testicular size smaller than expected for the testosterone concentration in plasma + pubertal staging)

29
Q

what can cause peripheral precocity in both males and females

A

Primary hypothyroidism

Exogenous sex steroids(creams or foods soya)

adrenal pathology

30
Q

what is mcune albright syndrome and it’s symptoms

A

McCune Albright syndrome
• Triad of peripheral precocious puberty, irregular café au lait spots, fibrous
dysplasia of bone
• Sequence of pubertal progression may be abnormal, often presenting with
vaginal bleeding

31
Q

how do you evaluate premature puberty clinically

A

first history and examination,
then basal fsh and lh, oestradiol,testosterone

thirdly gnrh stimulations and look at adrenal steroids in urine serum

other studies beta hcg afp

finally images of the brain, bone age and pelvic ultrasound

32
Q

what are the potential treatments for premature puberty gonadtrophin dependent

A

depends on cause, gonadtrophin dependent block with Gnrh analogues,

33
Q

what are the potential treatments for premature puberty gonadtrophin independent

A

treat with anti androgens if you can’t treat or find the cause

34
Q

what are the potential treatments for premature puberty is benign variance

A

just reassure family

35
Q

what are the treatments and things that have to be considered when a child is expieriencing delayed puberty

A

watchful waiting (they may start growingand end up even taller than predicted), counselling if they are being bullied, anabolic steroids to increase growth, short term sex therapy to kickstart puberty

36
Q

what gender is more affected by delayed puberty

A

boys

37
Q

what can cause primary hypogonadism congenital and acquired causes

A

chromosomal abnormalities turner syndrome or kinefelter

testicular regression, synthesis and action of sexual steroids

acquired causes can be surgery, trauma, post infection, metabolic or chemo or radiation

38
Q

what is the difference between primary and secondary hypogonadism

A

primary is issue with the gonad and secondary is issue with the hypothalamus

39
Q

what can cause congential and acquired secondary hypogonadism

A

congential, gnrh deficit (expierienced in PWS, issues witrh receptors, Kallmans), idiopathic, midline defect or cranial anomaly

acquired can be functional loss or physical

40
Q

what are the conc of fsh and lh like in primary hypogonadism

A

elevated

41
Q

how do you clinically recognise delayed puberty in girls 4 key points

A

13 no breast, 5 years between thelarche and menarche, no pubic haior by 14, no menarche by 16 no secondary sexual characteristics

42
Q

how do you clinically recognise delayed puberty in boys 3 key points

A

Testicular volume
less than 4 ml by
age 14, no pubic hair by 15, more than 5 years to finish penile and testes growth