Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

TAS Growth And Puberty ✅ > Precocious Puberty ✅ > Flashcards

Precocious Puberty ✅ Flashcards

1
Q

What can precocious puberty be defined as in girls?

A

Evidence of breast or pubic hair development in girls before the age of 8 years

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

What can precocious puberty be defined as in boys?

A

Signs of testicular volume increase to at least 4ml, or other evidence of vitalisation, in boys occurring before the age of 9 years

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

What can the causes of precocious puberty be divided into?

A
  • Gonadotrophin dependent

- Gonadotrophin independent

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

How can gonadotrophin dependent precocious puberty be differentiated from gonadotrophin independent?

A

LH and FSH are increased in gonadotrophin dependent, decreased in gonadotrophin independent

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Is FSH or LH increased more in gonadotrophin dependent precocious puberty?

A

LH

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Where does the increased LH and FSH come from in gonadotrophin dependent precocious puberty?

A

The pituitary

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

What is the result of increased FSH and LH in gonadotrophin dependent precocious puberty?

A

Enlargement of the gonads

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

What is the result of the enlargement of the gonads in gonadotrophin dependent precocious puberty?

A
  • Increased oestrogen from the ovary

- Increased testosterone from the testis and adrenals

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Is more testosterone produced by the testis or adrenals in gonadotrophin dependent precocious puberty?

A

Testis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

What does the increased oestrogen in gonadotrophin dependent precocious puberty lead to in girls?

A

Breast development

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

What does the increased testosterone in gonadotrophin dependent precocious puberty lead to in boys?

A
  • Pubic hair growth
  • Acne
  • Body odour
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

What are the causes of gonadotrophin dependent precocious puberty?

A
  • Idiopathic/familial
  • CNS abnormalities
  • Hypothyroidism
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

What can cause CNS anomalies leading to gonadotrophin dependent precocious puberty?

A
  • Congenital abnormalities
  • Acquired abnormalities
  • Tumours
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Give an example of a congenital anomaly that can lead to gonadotrophin dependent precocious puberty

A

Hydrocephalus

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Give 3 examples of acquired causes of CNS anomalies that can cause gonadotrophin dependent precocious puberty?

A
  • Post-irradiation
  • Infection
  • Surgery
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Give a tumour that can cause gonadotrophin dependent precocious puberty

A

Microscopic hamartomas

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

What happens in gonadotrophin independent precocious puberty?

A

A gonadal or extra-gonadal source leads to increased oestrogen or testosterone

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

Does the gonad shrink or enlarge in gonadotrophin independent precocious puberty?

A

Can be either

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

What happens to FSH and LH in gonadotrophin independent precocious puberty?

A

They are reduced

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

Why are FSH and LH reduced in gonadotropin independent precocious puberty?

A

The raised oestrogen/testosterone results in negative feedback on the pituitary, so there is reduced LH and FSH production

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

What are the causes of gonadotrophin independent precocious puberty?

A
  • Adrenal disorders
  • Ovarian causes
  • Testicular causes
  • McCune Albright syndrome
  • Genetic mutation of LH receptor
  • Exogenous sex steroids
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

Give 2 examples of adrenal disorders causing gonadotrophin independent precocious puberty

A
  • Tumours

- Congenital adrenal hyperplasia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

Give 2 examples of ovarian causes of gonadotrophin independent precocious puberty

A
  • Cysts

- Tumours

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

Give an example of an ovarian tumour that can cause gonadotrophin independent precocious puberty

A

Granulosa cell tumours

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

Give an example of a testicular cause of gonadotrophin independent precocious puberty

A

Leydig cell tumour

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

What should be included in the history of a child with precocious puberty?

A
  • Details of previous growth pattern and weight gain
  • Timing of onset of features of puberty
  • Presence of symptoms suggestive of intracranial pathology
  • Risk factors in perinatal history
  • Presence of disorders associated with sexual precocity
  • Drug history
  • Family history
  • Extent to which early onset of puberty is impacting on child’s psychosocial wellbeing
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q

What features of puberty should be asked about in the history for precocious puberty?

A
  • Acne
  • Body odour
  • Breast, pubic hair, and genital development
  • Increased vaginal secretions or periods
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
28
Q

What risk factors in the perinatal history should be asked about in the history for precocious puberty?

A
  • Prematurity
  • Intraventricular haemorrhage
  • Small for gestational age
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
29
Q

Give 2 disorders that are associated with sexual precocity?

A
  • McCune-Albright syndrome

- Neurofibromatosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
30
Q

Give a drug that can be associated with precocious puberty

A

Oxymethalone

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
31
Q

What should be asked about in the family history of precocious puberty?

A

Timing of maternal menarche

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
32
Q

What should be noted on examination in a child with precocious puberty?

A
  • Height
  • Weight
  • Tanner stage
  • Measurement of testicular volume
  • Features of other disorders, e.g. hyperthyroidism, McCune-Albright syndroome, neurofibromatosis
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
33
Q

How should testicular volumes be measured?

A

Using a Prayer orchidometer

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
34
Q

What features on examination may be suggestive of neurofibromatosis?

A
  • Cafe-au-lait patches

- Axillary freckling

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
35
Q

What examination should be performed to look for signs of raised ICP?

A
  • Fundoscopy

- Visual field examinations

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
36
Q

What investigations might be considered in precocious puberty?

A
  • XR of left wrist
  • Pelvic ultrasound
  • Basal blood sample
  • LHRH stimualtion test
  • MRI of hypothalamopituitary axis
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
37
Q

Why might a XR of the left wrist be done in precocious puberty?

A

To calculate bone age

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
38
Q

Why might bone age be useful in precocious puberty?

A

Is a guide to the extent of physiological advance

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
39
Q

What information may be obtained from a pelvic USS in precocious puberty?

A
  • Size of uterus
  • Extent of endometrial response
  • Size of ovaries
  • Presence of ovarian follicles
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
40
Q

What hormones may be measured in basal blood samples in precocious puberty?

A
  • Testosterone
  • Oestrogen
  • LH and FSH
  • Adrenal androgens
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
41
Q

What adrenal androgens may be measured in precocious puberty?

A
  • DHEAS
  • Androstenodione
  • 17-OH progesterone
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
42
Q

What is measured in a basal blood sample for precocious puberty in boys?

A

Testosterone

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
43
Q

What is measured in a basal blood sample for precocious puberty in girls?

A

Oestradiol

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
44
Q

What is the purpose of a basal blood sample for testosterone/oestradiol in precocious puberty?

A

Confirm biochemical evidence of puberty

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
45
Q

Why can measurement of LH and FSH be helpful in precocious puberty?

A

Can distinguish between gonadotrophin-dependent and independent causes

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
46
Q

Why might adrenal androgens be measured in precocious puberty?

A

To exclude a defect in adrenal hormone biosynthesis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
47
Q

What other investigation might be useful in excluding a defect in adrenal hormone biosynthesis?

A

A urinary steroid metabolite profile

48
Q

When is a LHRH stimulation test indicated in precocious puberty?

A

If basal gonadotrophins are low and sex steroids are high

49
Q

What does low basal gonadotrophins and high sex steroids indicate?

A

Gonadotrophin-independent precocious puberty

50
Q

Why is a LHRH stimulation test indicated when there is suspected gonadotrophin-independent precocious puberty?

A

To confirm the lack of gonadotrophin response in this scenario

51
Q

When is a MRI of the hypothalamopituitary axis required in precocious puberty>

A

In gonadotrophin-dependent precocious puberty (especially in boys)

52
Q

Why is an MRI of the hypothalamo-pituitary axis required in gonadotrophin-dependent precocious puberty?

A

To exclude intracranial tumour

53
Q

What is true central precocious puberty defined as?

A

Early onset puberty which has been stimulated by activation of the hypothalamo-pituitary axis

54
Q

Is true central precocious puberty more common in boys or girls?

A

Girls

55
Q

What is the most common cause of true central precocious puberty in girls?

A

Usually unknown

56
Q

What is the most common cause of true central precocious puberty in boys?

A

Consequence of intracranial pathology

57
Q

What intracranial pathologies can cause true central precocious puberty in boys?

A
  • Tumours in the region of the hypothalamus

- Following previous cerebral trauma that occurred in the perinatal period

58
Q

What tumours can occur in the region of the hypothalamus?

A
  • Gliomas
  • Astrocytomas
  • Benign hamartomas
59
Q

Give a cause of cerebral trauma occurring in the perinatal period that can lead to true central precocious puberty

A

Periventricular haemorrhage

60
Q

What does a diagnosis of true central precocious puberty require?

A
  • Measurement of serum oestradiol concentrations

- Usually a LHRH stimulation test

61
Q

Why is a LHRH stimulation test usually required to diagnose true central precocious puberty?

A

To demonstrate activation of gonadotrophin

62
Q

What other investigations may be done in true central precocious puberty?

A
  • Left wrist x-ray
  • Pelvic ultrasound in girls
  • MRI
63
Q

What will the left wrist x-ray show in true central precocious puberty?

A

Advanced bone development

64
Q

What may a pelvic USS show in true central precocious puberty in girls?

A
  • Ovarian enlargement with follicles

- Uterine enlargement

65
Q

Why is an MRI done in true central precocious puberty?

A

To exclude intracranial haemorrhage

66
Q

What does treatment of true central precocious puberty require?

A

GnRH analogue therapy

67
Q

How does GnRH analogue therapy work in true central precocious puberty?

A

It suppresses gonadotrophin and oestradiol secretion

68
Q

What is the purpose of treating true central precocious puberty?

A
  • Prevent psychosocial effects of advancing puberty

- Maximise potential adult height

69
Q

How do GnRH analogues work?

A

They bind to GnRH receptors

70
Q

What effect do GnRH analogues have when they bind to GnRH receptors?

A

Initially stimulate them, but thereafter achieve downregulation

71
Q

What side effect may be produced by down-regulation of GnRH receptors by GnRH analogues?

A

Temporary episode of vaginal blood loss

72
Q

What causes a temporary episode of vaginal blood loss with downregulation of GnRH receptors?

A

Faling oestradiol levels

73
Q

How long should GnRH treatment be continued for?

A

Until the child and family are comfortable to allow puberty to progress, or until the child has achieved the normal age-range for the stage of puberty that has been achieved

74
Q

What happens to FSH and LH in gonadotrophin independent precocious puberty?

A

They are reduced

75
Q

What causes androgen-mediated precocious puberty?

A

Excess androgen secretion

76
Q

What does excess androgen secretion lead to?

A

Virilization, including pubic and axillary hair development

77
Q

What can excess androgen secretion lead to in severe cases?

A
  • Genital maturation in boys

- Cliteromegaly in girls

78
Q

What is the most common form of androgen-mediated precocious puberty?

A

Exaggerated adrenarche

79
Q

What is exaggerated adrenarche associated with?

A

The physiological activation of the adrenal gland from the age of 6 years

80
Q

What does the physiological activation of the adrenal gland from the age of 6 years produce?

A

Relatively weak androgens such as DHEAS (dehydroepiandrosterone) and androstrenedione

81
Q

How does the activation of the adrenal gland at age 6 usually clinically manifest?

A

Usually unnoticed

82
Q

How does exaggerated adrenarche present?

A
  • Production of small amounts of pubic or axillary hair

- Sometimes increased skin secretions and body odour

83
Q

How is exaggerated adrenarche managed?

A
  • No specific therapy required

- Reassurance

84
Q

What is exaggerated adrenarche associated?

A
  • Reduced brith weight

- PCOS

85
Q

What is it important to distinguish exaggerated adrenarche from?

A

Congenital adrenal hyperplasia (CAH)

86
Q

How is exaggerated adrenarche distinguished from CAH?

A
  • Measurement of 17OH-progesterone

- Urinary steroid metabolite profile

87
Q

In what form of CAH will there be abnormalities in 18OH-progesterone levels?

A

21-hydroxylase deficient variant

88
Q

In what form of CAH will there be abnormalities in urinary steroid metabolite profiles?

A

Virilising forms

89
Q

What form of CAH is more common?

A

21-hydroxylase deficient variant

90
Q

What are virilising forms of CAH usually associated with?

A

Significant virilisation including cliteromegaly and evidence of growth spurt with marked advance in bone age

91
Q

What tumours can produce androgen-mediated precocious puberty?

A

Virilising tumours of the ovarian or adrenal glands

92
Q

What is required to treat virilising tumours producing precocious puberty?

A

Surgery

93
Q

Give 2 causes of gonadotrophin-independent precocious puberty caused by genetic mutations

A
  • Testotoxicosis

- McCune-Albright syndrome

94
Q

What causes testotoxicosis?

A

Autosomal dominant activating mutations of the LH receptor

95
Q

What gender is affected by testotoxicosis?

A

Males

96
Q

What causes precocious puberty in McCune-Albright syndrome?

A

Activating mutations of a gene involved in G-protein coupled signalling

97
Q

What gender can be affected by McCune-Albright syndrome?

A

Both

98
Q

Will GnRH therapy work in gonadotrophin independent precocious puberty?

A

No

99
Q

Why will GnRH therapy not work in gonadotrophin-independent precocious puberty?

A

Because the defect lies distal to the action of GnRH

100
Q

What does therapy for gonadotrophin-independent precocious puberty use in girls?

A

Anti-oestrogens

101
Q

What does therapy for gonadotrophin-independent precocious puberty use in girls?

A
  • Androgen synthesis blockers
  • Aromatase inhibitors
  • Androgen receptor blockers
102
Q

Give 2 androgen synthesis blockers

A
  • Cyproterone

- Ketoconazole

103
Q

Give an aromatase inhibitor

A

Testolactone

104
Q

Give 4 androgen receptor blockers

A
  • Spironolactone
  • Ketoconazole
  • Cyproterone
  • Flutamide
105
Q

Is premature thelarche common?

A

Relatively

106
Q

What is premature thelarche?

A

Isolated breast development in young girls 6-12 months in the absence of any wider evidence of puberty or rising oestradiol concentration

107
Q

What happens on LHRH stimulation testing in premature thelarche?

A

There is a FSH response

108
Q

What is the mechanism of breast development in premature thelarche?

A

Unclear

109
Q

What treatment is required for premature thelarche?

A

None - condition benign and self-limiting, no therapy required

110
Q

What is thelarche variant?

A

A similar phenomenon to premature thelarche that occurs at 5-8 years

111
Q

What might thelarche variant be associated with?

A
  • Slight increase in height velocity

- Brief period of vaginal blood loss

112
Q

What is premature menarche?

A

Cyclical vaginal bleeding occurring in the absence of wider signs of puberty

113
Q

At what age does premature thelarche occur?

A

5-8 years

114
Q

What is the mechanism of premature menarche?

A

Unknown

115
Q

What are the differential diagnoses for premature menarche?

A
  • Vulvovaginitis
  • Vaginal trauma
  • Foreign bodies
  • Tumours
  • Sexual abuse

TAS Growth And Puberty ✅ (12 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions