Pituitary and Adrenal Disease Flashcards

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1
Q

What are some benign tumours of the pituitary?

A

Most common = pituitary adenoma
Rare
- Craniopharyngioma
- Meningioma

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2
Q

What are some malignant tumours of the pituitary?

A

Primary
Metastastic
- Lung
- Breast

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3
Q

Are pituitary cysts common?

A

No, very rare

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4
Q

What are some cysts of the pituitary?

A

Rathke’s cleft
Arachnoid
Dermoid

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5
Q

What is lymphocytic hypophysitis?

A

Inflammation of pituitary gland
Becoming more common
Commonly cases thyroid dysfunction but can affect whole gland

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6
Q

What is a vascular cause of a sellar mass?

A

Carotid aneurysm

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7
Q

What are the local effects of pituitary tumours?

A

Tumour stretches dura > headaches + sometimes nausea and vomiting
Rare - hydrocephalus
Tumour compresses nasal retinal fibres > visual field defects
Tumour extends laterally > cranial nerve palsies and temporal lobe epilepsies
Tumour extends downwards > CSF rhinorrhoea

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8
Q

What do you do if you find a non-functioning pituitary adenoma?

A

Nothing, unless size big enough to cause concern

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9
Q

What is prolactin secretion sensitive to?

A

Stress
Certain medications; eg:
- Antidepressants
- Atypical antipsychotics

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10
Q

What is the treatment for a prolactinoma?

A

Dopamine agonists > reduce tumour size and prolactin secretion

  • Bromocriptine
  • Cabergoline
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11
Q

What pituitary hormones does prolactin suppress?

A

Gonadotrophins

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12
Q

What are the symptoms of hyperprolactinaemia in females?

A

Galactorrhoea
Oligomenorrhoea
Headaches

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13
Q

What are the symptoms of hyperprolactinaemia in males?

A

Gynaecomastia
Infertility
Rarely, galactorrhoea
Testosterone deficiency

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14
Q

If prolactin is raised, what other pituitary hormones are screened?

A

Full pituitary hormone screen

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15
Q

What are the common causes of acromegaly?

A

Growth hormone secreting pituitary adenoma

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16
Q

What are the rare causes of acromegaly?

A

Growth hormone secreting extra pituitary tumour

Growth hormone release hormone (GHRH) secreting tumour

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17
Q

What are the clinical features of acromegaly?

A

Growth of many tissues

  • Skin
  • Connective tissue
  • Cartilage
  • Bone
  • Viscera
  • Epithelial tissues
  • Heart
    • Cardiomyopathy
    • Arrhythmias
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18
Q

What sort of questions can you ask to determine if it’s acromegaly?

A
Change in 
- Shoe size
- Glove size
- Hat size
Compare with previous photos
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19
Q

What affect growth hormone levels?

A

Diurnal variation

Decreases with food

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20
Q

What are the biochemical results in acromegaly?

A

Elevated growth hormone
- Failure to suppress with oral glucose tolerance test
Increased IGF1
Diabetes/impaired glucose tolerance

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21
Q

What is the treatment for acromegaly?

A

1st line: transphenoid hypophysectomy to remove tumour (usually macroadenomas)
If not cured surgically, possible treatments
- Radiotherapy
- Octreotide
- Bromocriptine

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22
Q

What are the ACTH-dependent causes of Cushing’s syndrome?

A

Cushing’s disease
Ectopic ACTH
Ectopic CRH

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23
Q

What is Cushing’s disease?

A

ACTH producing tumour - ectopic tumours included

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24
Q

What are the ACTH-independent causes of Cushing’s syndrome?

A

Adrenal adenoma
Adrenal carcinoma
Micronodular hyperplasia
Macronodular hyperplasia

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25
Q

What are the clinical features of Cushing’s syndrome?

A
Redistribution of weight centrally
Moon face
Buffalo hump
Wasting of buttocks
Atrophy of epidermis > thin skin
Plethoric face
Easy bruising
Striae
Depressed mood and crying
Decreased concentration and memory
Insomnia
Decreased libido
Proximal myopathy
Osteopaenia > crush fractures of vertebrae
Glucose intolerance (75%)/diabetes (25%)
Hirsutism
HTN
Menstrual disorders
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26
Q

Why is the administration of corticosteroids avoided in the evening?

A

Causes insomnia

27
Q

What are the investigations in Cushing’s syndrome?

A

FBE
- Hb = high normal range
- WCC = slightly elevated; decreased neutrophils
Electrolytes
- Hypokalaemia and metabolic alkalosis in ectopic ACTH
Insulin resistance > hyperglycaemia
Increased Ca absorption and hypercalciuria

28
Q

What are the tests used to diagnose Cushing’s syndrome?

A
Midnight salivary cortisol test
24 hour urine free cortisol
Overnight dexamethasone suppression test
- 1 mg of dexamethasone at 0000
- Serum cortisol at 0800
29
Q

How do you interpret the results of an overnight dexamethasone suppression test?

A

ACTH

  • Suppressed > likely adrenal cause
  • Normal/slightly elevated > likely pituitary dependent
  • Very high > likely ectopic ACTH
30
Q

What is the treatment for pituitary-dependent Cushing’s syndrome?

A

Transphenoid hypophysectomy to remove tumour

31
Q

What is the treatment for an adrenal adenoma or carcinoma?

A

Adrenal surgery

32
Q

What is the treatment for ectopic ACTH production or increased CRH production?

A

Treat tumour associated with hormone production

33
Q

What can cause adrenocortical insufficiency?

A

Insufficient secretion of ACTH = hypopituitarism

Destruction of adrenal glands = Addison’s disease

34
Q

What are the clinical features of Addison’s disease?

A

Hyperpigmentation

Non-specific symptoms; eg: lethargy

35
Q

What can cause Addison’s disease?

A
Autoimmune atrophy
Infections
Infiltration of metastases
Haemorrhage/infarction
Drugs
Adrenoleukodystrophy
36
Q

What are the investigation results in Addison’s disease?

A
Electrolytes
- Hyponatraemia
- Hyperkalaemia
- Moderate acidosis
- Increased urea
Hypoglycaemia
37
Q

What are the diagnostic tests for Addison’s disease?

A
Low serum cortisol
High ACTH
Synacthen test
Adrenal Abs
Adrenal imaging
- Calcification
- Enlargement
38
Q

How can Addison’s disease present in a medical emergency?

A

Nausea
Vomiting
Hypovolaemic shock

39
Q

What is the acute management for an Addisonian crisis?

A

Hydrocortisone 100 mg IV 6 hrly
IV saline
Glucose if hypoglycaemic

40
Q

What is the long-term management of Addison’s disease?

A

Cortisone acetate/hydrocortisone
If also deficient in mineralocorticoids: fludrocortisone
Medic alert
Advice on increasing steroid dose in times of stress and infection

41
Q

What adrenal diseases can cause an excess of hormone production?

A

Conn’s syndrome = aldosterone excess

Phaeochromocytoma = catecholamine excess

42
Q

When may you suspect Conn’s syndrome?

A

Refractive HTN

Hypokalaemia, even if low normal

43
Q

What are the subtypes of primary aldosteronism?

A

Most common = unilateral aldosterone producing adenoma
Bilateral idiopathic hyperaldosteronism
Glucocorticoid suppressible hyperaldosteronism
Aldosterone producing adrenal carcinoma

44
Q

What is the clinical triad of primary aldosteronism?

A
HTN
Hypokalaemia
- Headaches
- Palpitations
- Polydipsia
- Polyuria
- Nocturia
Metabolic alkalosis
45
Q

What is the diagnostic approach to primary aldosteronism?

A

24 hr urine collection to document inappropriate K wasting in presence of hypokalaemia
Plasma renin activity - suppressed
Plasma/urine aldosterone - increased
Increased aldosterone-to-renin ratio
Aldosterone fails to suppress after infusion of 2 L of saline over 4 hrs (=saline suppression test)/high Na intake for 3 days

46
Q

How is primary aldosteronism localised using imaging?

A

CT/MRI
131-I-iodocholesterol scanning
Adrenal vein sampling for aldosterone

47
Q

What is the treatment for primary aldosteronism?

A

Adrenal adenoma - adrenal surgery
Spironolactone = competitive antagonist to aldosterone
Amiloride = blocks Na channels in proximal tubule > decreases K excretion

48
Q

What is phaeochromocytoma?

A

Tumour of chromaffin cells in adrenal medulla

Derived from neural crest cells

49
Q

What are the clinical features of phaeochromocytoma?

A
Episodic symptoms
- Sudden and severe (<15 min)
- Spontaneous/due to 
   - Exercise
   - Bending over
   - Defecation
   - Abdominal pressure
   - Drugs
Paroxysms
- Pallor
- Perspiration
- Palpitations
- (Labile blood) pressure > headache
Accelerated HTN
Supra-renal masses
Rarely hypotension
Tachyarrhythmias
50
Q

How is phaeochromocytoma diagnosed?

A
24 hour urine chatecholamines
Plasma catecholamines - marked variability
Plasma metanephrines = breakdown products of catecholamines
- More sensitive and specific
Localise tumour
- CT of chest and pelvis
- MIBG scanning
- Octreotide scanning
- Venous sampling for lateralisation
51
Q

What is the pre-operative treatment of phaeochromocytoma?

A

Alpha adrenergic blockade - phenoxybenamine
Beta blockers if necessary, but never before alpha blockade
Treatment, as required of
- Arrhythmias
- Cardiac failure
- Diabetes

52
Q

What is polyglandular autoimmune syndrome type 1?

A

Autosomal recessive disorder
Very rare
Mutation in AIRE gene on chromosome 21

53
Q

What are the endocrine effects of polyglandular autoimmune syndrome type 1?

A
Hypoparathyroidism
Chronic mucocutaneous candidiasis
Adrenal insufficiency
Primary hypogonadism
Hypothyroidism
T1D
Hypothyroidism
Hypopituitarism
Diabetes insipidus
54
Q

What are the non-endocrine effects of polyglandular autoimmune syndrome type 1?

A
Malabsorption syndromes
Alopecia totalis/areata
Pernicious anaemia
Chronic active hepatitis
Vitiligo
55
Q

What is polyglandular autoimmune syndrome type 2?

A
More common than type 1
More than 50% familial
Inheritance
- Autosomal recessive
- Autosomal dominant
- Polygenic
Associated with high Ab titres to steroid producing cells
- Adrenals
- Thyroid
- Testis/ovary
56
Q

What are the endocrine effects of polyglandular autoimmune syndrome type 2?

A
Adrenal insufficiency
Autoimmune thyroid disease
T1D
Primary hypogonadism
Diabetes insipidus
57
Q

What are the non-endocrine effects of polyglandular autoimmune syndrome type 2?

A
Vitiligo
Alopecia
Pernicious anaemia
Myaesthenia gravis
Immune thrombocytopenic purpura
Sjogrens syndromes
Rheumatoid arthritis
58
Q

Where is the MEN1 gene located?

A

Chromosome 11

59
Q

What is the inheritance pattern of MEN1 syndrome?

A

Autosomal dominant

60
Q

What is the triad of tumours in MEN1 tumours?

A

Pituitary
Pancreas
Parathyroid

61
Q

What is the inheritance pattern of MEN2 syndrome?

A

Autosomal dominant

62
Q

What causes MEN2 syndrome?

A

Mutations of RET proto-oncogene on chromosome 10

63
Q

What are the tumours in MEN2a syndrome?

A

Medullary carcinoma of thyroid
Phaeochromocytoma
Parathyroid hyperplasia

64
Q

What are the tumours of MEN2b syndrome?

A
Medullary carcinoma of thyroid
Phaeochromocytoma
Parathyroid hyperplasia
Mucosal neuromas
Intestinal ganglioneuromas
Marfanoid habitus