Disease Profiles: Pituitary Disorders Flashcards

1
Q

Define a pituitary macro-adenoma

A

Adenomas >1cm

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2
Q

Describe the use of GH antagonists in the management of acromegaly

A

SC injection, blocks GH activity with an 85% response rate (but tumour size does not decrease)

Last line as very expensive

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3
Q

Describe the clinical presentation of hypopituitarism

A

Depend on the specific hormone deficiency, age of onset, the rate at which hypopituitarism develops, and the underlying cause of hypopituitarism

If caused by intra/parasellar masses (e.g. pituitary macroadenomas, craniopharyngiomas) can present with headaches and visual field defects (bitemporal hemianopia)

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4
Q

Is pituitary adenoma which produces ACTH more likely to be a micro or macroadenoma?

A

Microadenoma (Cushing’s, bilateral adrenocortical hyperplasia)

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5
Q

Craniopharyngiomas are derived from the remnants of _______ pouch

A

Rathke’s

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6
Q

Define a pituitary micro-adenoma

A

Adenomas <1cm

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7
Q

Describe the findings in a GTT suppression test which would indicate acromegaly

A

GH unchanged/no suppression or paradoxical rise following oral glucose challenge (normally GH suppresses after glucose)

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8
Q

Name the hormone replacement for TSH deficiency

A

Levothyroxine

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9
Q

A subset of pituitary adenomas behave aggressively and enlarge more rapidly. Name two features which indicate an aggressive lesion.

A

Lots of mitotic figures and p53 mutations

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10
Q

Name the Ur/serum osmol ratio which would indicate diabetes insipidus

A

< 2

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11
Q

Describe the management of diabetes insipidus

A

Desmospray (nasally) or desmopressin oral tablets

Desmopressin IM injection - generally reserved for emergency or post pituitary surgery

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12
Q

Describe the clinical presentation of GH excess

A

Giant (before epiphyseal fusion)

Thickened soft tissues - skin, large jaw, sweaty, large hands

Snoring/sleep apnoea (thickened nasopharynx)

Hypertension, cardiac failure

Headaches (vascular)

Diabetes mellitus

Local pituitary effects - visual fields, hypopituitarism

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13
Q

Define hypopituitarism

A

Inadequate production of one or more pituitary hormone as a result of damage to the pituitary gland and/or hypothalamus

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14
Q

Name 4 pathological causes of hyperprolactinaemia

A

Hypothyroidism, stalk compression e.g. due to pituitary adenoma, damage to stalk, prolactinoma

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15
Q

How is a water deprivation test to investigate diabetes insipidus performed?

A

Patients stop drinking water for 2–3 hours before the first measurement

Check serum and urine osmolarities hourly for 8hr, then 4hr after giving DDAVP

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16
Q

What would be the consequence of ↓ TSH production (hypopituitarism)?

A

Secondary hypothyroidism

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17
Q

How would you investigate a prolactinoma (pituitary adenoma producing prolactin)?

A

Serum prolactin (raised)

MRI pituitary

Visual fields - bitemporal hemianopia

Other pituitary hormone tests to assess whether other hormones are being affected

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18
Q

Name the dynamic test used to assess the adrenal axis

A

Synacthen test

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19
Q

What are the two classes of diabetes insipidus?

A

Cranial (central) and nephrogeneic

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20
Q

Describe the management of a craniopharyngioma

A

Usually resection + radiotherapy

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21
Q

Describe the principle of management for hypopituitarism

A

Hormone replacement and treatment of underlying cause

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22
Q

What does growth hormone (GH) stimulate?

A

Skeletal and soft tissue growth

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23
Q

What would be the consequence of ↓ LH/FSH production (hypopituitarism)?

A

Hypogonadism

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24
Q

Hyper__________ may cause galactorrhoea and hypogonadism, including amenorrhoea

A

Hyperprolactinaemia

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25
Q

Name the hormone replacement for diabetes insipidus (ADH deficiency)

A

Desmospray (nasal) or desmopressin tablets

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26
Q

Pituitary macroadenomas can cause pressure _______ of normal surrounding cell tissue

A

Atrophy

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27
Q

Name 4 physiological causes of hyperprolactinaemia

A

Breastfeeding, pregnancy, stress, sleep

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28
Q

Name the most common causes of panhypopituitarism

A

Most commonly caused by pituitary tumours, surgery or radiotherapy

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29
Q

What would be the consequence of ↓ ADH production (hypopituitarism)?

A

Diabetes insipidus

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30
Q

Define hyperprolactinaemia

A

Abnormally high levels of prolactin in the blood

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31
Q

GH deficiency in children leads to ______ _______

A

Growth retardation

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32
Q

How can a pituitary macroadenoma cause visual field defects?

A

Compression of the optic chiasma

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33
Q

Name a long term side effect of somatostatin analogues in the management of acromegaly

A

Gallstones (inhibit gallbladder contraction)

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34
Q

What would be the consequence of ↓ ACTH production (hypopituitarism)?

A

Hypoadrenal

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35
Q

Define nephrogenic diabetes insipidus

A

Characterised by renal resistance to ADH

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36
Q

Describe the aquired causes of cranial (central) diabetes insipidus

A

Idiopathic (often autoimmune), tumours, trauma, infiltrations (sarcoidosis), infections

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37
Q

What is the consequence of a functioning pituitary adenoma which produces GH in an adult?

A

Acromegaly

38
Q

Define panhypopituitarism

A

Refers to deficiency of all anterior pituitary hormones

39
Q

Hypo_______ causes weight gain, slowness of thought and action, dry skin, cold intolerance, constipation and potentially bradycardia and hyperthermia

A

Hypothyroidism

40
Q

How can a pituitary macroadenoma cause panhypopituitarism?

A

Can cause infarction

41
Q

Define cranial (central) diabetes insipidus

A

Occurs when there is insufficient levels of circulating ADH

42
Q

Describe the use of somatostatin analogues in the management of acromegaly

A

Monthly injections which selectively act on somatostatin receptor subtypes (highly expressed on GH-secreting tumours)

Shrink tumour (reexpands after stopping treatment)

Can be used before surgery to relieve symptoms or after surgery if GH levels fail to be normalised

43
Q

What is a craniopharyngioma?

A

Benign tumour which typically arises in the sellar/suprasella region

44
Q

In hypopituitarism, what percentage of pituitary cells must be damaged before the patient becomes symptomatic?

A

>80%

45
Q

How could you identify cranial diabetes insipidus from a water deprivation test?

A

If Ur/serum osmol ratio is low and improves after DDAVP then it is due to cranial DI (not nephrogenic)

46
Q

Name the condition that results from GH excess in children (before epiphyseal fusion)

A

Gigantism

47
Q

Name 2 consequences caused by secondary hypothyroidism, hypoadrenalism, hypogonadism and GH deficiency

A

Tiredness/general malaise, reduced quality of life

48
Q

Why might you consider GH replacement for adults with hypopituitarism?

A

Improve work capacity and psychological well-being

49
Q

How would you investigate a craniopharyngioma?

A

CT/MRI

50
Q

Describe the use of dopamine agonists in the management of acromegaly

A

PO, act on D2 receptors, work on 10-15% of patients, increased efficacy if co-secreting prolactin

51
Q

How would you investigate diabetes insipidus?

A

Obtain baseline values then perform a water deprivation test

52
Q

What would be the consequence of ↓ GH production (hypopituitarism)?

A

Growth failure

53
Q

Describe the typical presentation of a female with hyperprolactinaemia

A

Generally present early with features of hyperprolactinamia:

  • Galactorrhoea (30-80%)
  • Menstrual irregulatity (25%)
  • Decreased libido
  • Ammenorrhoea
  • Infertility
54
Q

Describe the clinical presentation of posterior hypopituitarism

A

Diabetes insipidus - polyuria, polydipsia

55
Q

Define diabetes insipidus

A

Kidneys are unable to concentrate urine

56
Q

How does a functioning pituitary adenoma present?

A

Depends on hormone produced, prolactin is most common but can also produce FSH/LH, GH and ACTH

57
Q

Name the visual field defect caused by a pituitary adenoma compressing on the optic chiasma

A

Bitemporal hemianopia

58
Q

Name the dynamic test used to assess the adrenal and GH axes

A

Insulin Tolerance Test (glucagon stimulation test if ITT contraindicated)

59
Q

Describe the growth of a craniopharyngioma

A

Slow growing, often cystic, may calcify

60
Q

Name the hormone replacement for ACTH deficiency

A

Hydrocortisone

61
Q

How would you investigate hypopituitarism?

A

Basal tests for relevant axis of the hypothalamo-pituitary system - LH/FSH, TSH, T4, prolactin, IGF-1 (GH axis), cortisol, Na+

Dynamic tests - synacthen test (adrenal), ITT (adrenal, GH), arginine and GH-releasing hormone (GH)

MRI if a hormone deficiency is identified to identify pituitary adenomas

62
Q

Describe the clinical presentation of a craniopharyngioma

A

Headaches and visual disturbances

Can cause pituitary hypofunction → hormonal imbalances

Children may have growth retardation

63
Q

What is a pituitary adenoma?

A

Benign tumour derived from cells of anterior pituitary

64
Q

Name 3 short term side effects of somatostatin analogues in the management of acromegaly

A

Flatuence, diarrhoea, abdominal pains

65
Q

Hypo__________ causes mild hypotension, hyponatreamia and CV collapse

A

Hypoadrenalism

66
Q

Name the two peaks of incidence for the development of a craniopharyngiomas

A

5-15 years, 6th-7th decades

67
Q

Name the most common cause of hypopituitarism in adults

A

Nonfunctioning pituitary macroadenomas

68
Q

List 3 side effects of dopamine agonists

A

Have the least side effects of alternatives

Can cause N+V, low mood/depression, and may cause fibrosis of heart valves/retroperitoneal fibrosis

69
Q

How would you investigate acromegaly/gigantism?

A

GF1 - age and sex matched, nearly always raised

Gold standard - GTT suppression test

Others - visual field, imaging to check adenoma size, pituitary function tests for other hormones

70
Q

Describe the prognosis of a craniopharyngioma

A

Excellent prognosis (especially if <5cm)

Following radiation, SSC may develop (but rare)

71
Q

How would you investigate hyperprolactinaemia?

A

Serum prolactin raised

Excluse physiological and drug causes then investigate pathological ones:

  • Visual fields - bitemporal temianopia
  • Exclude primary hypothyroidism
  • Assess anterior pituitary function
  • MRI pituitary
72
Q

How does a non-functioning pituitary adenoma present?

A

Mass effects e.g. headache, visual field defects

73
Q

Describe the management of a pituitary adenoma

A

Transphenoidal surgery

Replace hormones

74
Q

Which type of drugs can cause hyperprolactinaemia?

A

Any drugs which reduce dopamine will reduce inhibition of prolactin so increase prolactin levels - dopamine antagonists, antipsychotics, antidepressants etc.

75
Q

Pituitary micro-adenomas are much less ________ than macroadenomas

A

Aggressive

76
Q

What is the consequence of a functioning pituitary adenoma which produces GH in a child?

A

Gigantism

77
Q

Name the condition that results from GH excess in adults

A

Acromegaly

78
Q

Name the hormone replacement for sex steroid deficiency

A

HRT/oest/prog pill for female

Testosterone for males

79
Q

List 3 causes of nephrogenic diabetes insipidus

A

Idiopathic, familial and renal disease

80
Q

Which type of Multiple Endocrine Neoplasia is associated with the formation of pituitary adenomas?

A

MEN1

81
Q

What most commonly causes GH excess?

A

Nearly always due to a GH-secreting pituitary adenoma

82
Q

Hypo________ leads to loss of libido, loss of secondary sexual hair, amenorrhoea and erectile disfunction, and eventually osteoporosis

A

Hypogonadism

83
Q

Describe the management of acromegaly

A

Excision - transphenoidal approach

Radiotherapy +/- pharmacological treatment indicated when pituitary surgery fails to normalise

84
Q

Describe the pathophysiology of pituitary carcinoma

A

Rare, account for <1% of pituitary tumours

Often functional (prolactin or ACTH usually)

Metastasise late after multiple recurrences

85
Q

Describe the clinical presentation of diabetes insipidus

A

Polydipsia

Polyuria with dilute urine

86
Q

Describe the familial causes of cranial (central) diabetes insipidus

A

Isolated in most cases, can occur as part of DIDMOAD

87
Q

Describe the clinical presentation of longstanding panhypopituitarism

A

Gives the classic picture of pallor with hairlessness (‘alabaster skin’)

88
Q

Name the dynamic test used to assess GH insufficiency

A

Arginine and growth hormone releasing hormone test

89
Q

Describe the management of prolactimonas

A

Dopamine agonists normalise prolactin and shrink tumour in majority of cases - surgery rarely considered because of this

90
Q

Name the two groups of causes of cranial (central) diabetes insipidus

A

Familial (rare)

Acquired

91
Q

Describe the typical presentation of a male with hyperprolactinaemia

A

Impotence, generally present late with stuctural symptoms from a pituitary tumour - visual loss, headaches, anterior pituitary malfunction

92
Q

Which form of malignancy is associated with GH excess?

A

GH excess can result in the formation of colon polyps and colon cancer - may be presenting feature