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Lecture Notes - Endocrinology > Disorders of puberty > Flashcards

Disorders of puberty Flashcards

1
Q

What is puberty

A

process of acquisition of secondary sexual characteristics and attainment of reproductive function

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2
Q

What are some secondary sexual characteristics

A

development of genitalia
pubic and axillary hair in boys and girls
development of breasts
increase in testicular volume

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3
Q

When does normal puberty occur

A

between the ages of 8 and 13 in girls
9-14 in boys
Usually lasts 3-4 years

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4
Q

What is the first step in initiation of puberty

A

activation of hypothalamic GnRH pulse generator and the pulsatile secretion of GnRH

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5
Q

What does the release of GnRH result in

A

an increase in plasma concentrations of LH, FSH and sex steroids (testosterone or estradiol)

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6
Q

What is the initial change in gonadotrophin secretion during the early stages of puberty

A

nocturnal increase in serum LH during sleep

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7
Q

What happens to LH levels later in puberty

A

Daytime LH levels also increase, gradually changing to the adult pattern

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8
Q

What is the function of leptin

A

it is a hormone produced largely in adipocytes and may act as a signal of the availability of the metabolic reserve necessary for pubertal development

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9
Q

What are the 5 Tanner stages in boys

A

1: prepubertal
2: scrotum and testes enlarge and scrotum skin reddens and changes in texture
3. Penis enlarges and the testes grow further
4: size of penis increases with a growth in breadth and development of the glans
5: adult genitalia

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10
Q

What are the 5 Tanner stages in girls

A

1: prepubertal
2: breast bud stage with elevation of breast and papilla, enlargement of areola
3. further enlargement of the breast and areola, no separation of their contour
4: Areola and papilla form a secondary mound above the level of the breast
5: Mature stage with projection of papilla only related to recession of the areola

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11
Q

What is thelarche

A

The first sign of puberty - the onset of breast development

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12
Q

What is pubarche

A

The development of pubic hair

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13
Q

When does menarche occur

A

2-3 years after the onset of puberty

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14
Q

When does the peak growth velocity (growth spurt) occur

A

Within the first year after the onset of puberty in girls

Usually 2 years after the onset of puberty

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15
Q

What is the first sign of puberty in boys

A

an increase in testicular volume

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16
Q

At what Tanner stage does spermatogenesis begin

A

stage 3

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17
Q

What is delayed puberty

A

the absence or incomplete development of secondary sexual characteristics by an age at which 95% of children of that sex and culture have initiated sexual maturation

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18
Q

What is constitutional delay of growth and puberty characterised by

A

a delayed onset of puberty, pubertal growth spurt and skeletal maturation

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19
Q

How is the predicted height of a child calculated

A

an appropriate range for the parental heights

20
Q

What is highly suggestive of congenital GnRH deficiency

A

Persistent hypogonadism beyond age 18

21
Q

What is hypogonadootrophic hypogonadism due to

A

an impaired secretion of hypothalamic GnRH and or impaired FSH and LH level

22
Q

What is congenital hypogonadotrophic hypogonadism sometimes associated with

A

Anosmia: in Kallmann’s syndrome
Mental retardation and obesity: in Prader-Willi syndrome
Congenital adrenal hypoplasia

23
Q

What is Hypergonadotrophic hypogonadism associated with

A

high FSH and LH levels due to a lack of negative feedback of the sex steroids

24
Q

What should physical examination include

A

Assessment of secondary sex characteristics and staging according to the Tanner criteria
Measurement and plotting of height on a growth chart
Measurement of weight and calculation of BMI
General examination to look for any features of an underlying cause - e.g. visual field defects due to a pituitary tumour

25
Q

what investigations should be included for patients with delayed puberty

A 
FBC
U&E
LFT
erythrocyte sedimentation rate 
serum LH and FSH 
oestradiol (girls) 
Testosterone (boys) 
prolactin 
free T4 and TSH
26
Q

How is bone age determined

A

by the comparison of a radiograph of the patients bones in the left hand and wrist with the bones in a standard atlas,
This allows an assessment of skeletal maturation and the potential for future skeletal growth

27
Q

What is the treatment for girls

A

2ug ethinylestradiol orally daily

28
Q

What is the treatment for boys

A

50mg testosterone enanthate or cypionate IM monthly

29
Q

What are side effects of testosterone therapy

A

acne and rarely, priapism

30
Q

What is precocious puberty

A

the onset of secondary sexual characteristics before he age of 8 in girls or 9 in boys

31
Q

What are some causes of GnRH dependent precocious puberty

A 
Hydrocephalus 
hamartomas 
umours 
trauma 
radiotherapy 
inflammatory disease 
idiopathic
32
Q

What are some causes of GnRH independent precocious puberty

A

Increased sex steroids from the ovaries

Increased testosterone from testes

33
Q

What causes GnRH dependent precocious puberty

A

earlier activation of the hypothalamic pituitary-gonadal axis

34
Q

What is GnRH independent precocious puberty

A

Autonomous endogenous secretion of sex steroids from the gonads or adrenal glands or excess exogenous sex steroids

35
Q

What is testotoxicosis

A

an autosomal dominant disorder caused by an activating mutation of the LH receptor gene, resulting in premature Leydig cell maturation and increased testosterone secretion in boys

36
Q

What is McCune-Albright syndrome

A

a arar disorder characterised by precocious puberty, cafe au last skin pigmentation and fibrous dysplasia of bone

37
Q

What are variants of normal puberty

A

premature thelache and premature adrenarche

38
Q

What are important parts of the medical history

A

Details of pubertal development: when initial changed were first noticed
Previous history of CNS disease, radiotherapy, trauma or presence of any near symptoms such as headaches or seizures
History of exposure to exogenous androgens or oestrogens
presence of abdominal pain ( ovarian disease )

39
Q

What investigations are required in a patient with precocious puberty

A

bone age
sex steroid
LH and DSH levels
Thyroid function tests
GnRH dependent precocious puberty: brain MRI for CNS cause
GnRH independent: measure testosterone, oestradiol, dehydroepiandrosterone suplhate (elevated in adrenal tumours)
Pelvic US - ovarian cyst or tumour

40
Q

What is the treatment of central precocious puberty if a cause has been found

A

treat it appropriately

Decision to treat depends on the predicted final adult height and physchosocial effects of the precocity

41
Q

If deciding to treat, what should be given

A

GnRH analogue therapy
it induces the down regulation of pituitary GnRH receptors and results in the suppression of pulsatile gonadotrophin release
It slows accelerated puberty and improves final height

42
Q

What should be monitored during therapy

A

bone density

43
Q

What should children with congenital adrenal hyperplasia be treated with

A

glucocorticoids

44
Q

What are patients with McCune-Albright syndrome treated with

A

drugs that inhibit gonadal steroid synthesis or action rather than surgery

45
Q

What might delayed puberty be caused by

A

constitutional delay of growth and puberty, hypogonadotrophic hypogonadism or hypergonadotrophic hypogonadism

Lecture Notes - Endocrinology (40 decks)

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