Pituitary and Adrenal Disease Flashcards

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
What are the clinical features of Cushing's syndrome?
``` 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 ```
26
Why is the administration of corticosteroids avoided in the evening?
Causes insomnia
27
What are the investigations in Cushing's syndrome?
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
What are the tests used to diagnose Cushing's syndrome?
``` Midnight salivary cortisol test 24 hour urine free cortisol Overnight dexamethasone suppression test - 1 mg of dexamethasone at 0000 - Serum cortisol at 0800 ```
29
How do you interpret the results of an overnight dexamethasone suppression test?
ACTH - Suppressed > likely adrenal cause - Normal/slightly elevated > likely pituitary dependent - Very high > likely ectopic ACTH
30
What is the treatment for pituitary-dependent Cushing's syndrome?
Transphenoid hypophysectomy to remove tumour
31
What is the treatment for an adrenal adenoma or carcinoma?
Adrenal surgery
32
What is the treatment for ectopic ACTH production or increased CRH production?
Treat tumour associated with hormone production
33
What can cause adrenocortical insufficiency?
Insufficient secretion of ACTH = hypopituitarism | Destruction of adrenal glands = Addison's disease
34
What are the clinical features of Addison's disease?
Hyperpigmentation | Non-specific symptoms; eg: lethargy
35
What can cause Addison's disease?
``` Autoimmune atrophy Infections Infiltration of metastases Haemorrhage/infarction Drugs Adrenoleukodystrophy ```
36
What are the investigation results in Addison's disease?
``` Electrolytes - Hyponatraemia - Hyperkalaemia - Moderate acidosis - Increased urea Hypoglycaemia ```
37
What are the diagnostic tests for Addison's disease?
``` Low serum cortisol High ACTH Synacthen test Adrenal Abs Adrenal imaging - Calcification - Enlargement ```
38
How can Addison's disease present in a medical emergency?
Nausea Vomiting Hypovolaemic shock
39
What is the acute management for an Addisonian crisis?
Hydrocortisone 100 mg IV 6 hrly IV saline Glucose if hypoglycaemic
40
What is the long-term management of Addison's disease?
Cortisone acetate/hydrocortisone If also deficient in mineralocorticoids: fludrocortisone Medic alert Advice on increasing steroid dose in times of stress and infection
41
What adrenal diseases can cause an excess of hormone production?
Conn's syndrome = aldosterone excess | Phaeochromocytoma = catecholamine excess
42
When may you suspect Conn's syndrome?
Refractive HTN | Hypokalaemia, even if low normal
43
What are the subtypes of primary aldosteronism?
Most common = unilateral aldosterone producing adenoma Bilateral idiopathic hyperaldosteronism Glucocorticoid suppressible hyperaldosteronism Aldosterone producing adrenal carcinoma
44
What is the clinical triad of primary aldosteronism?
``` HTN Hypokalaemia - Headaches - Palpitations - Polydipsia - Polyuria - Nocturia Metabolic alkalosis ```
45
What is the diagnostic approach to primary aldosteronism?
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
How is primary aldosteronism localised using imaging?
CT/MRI 131-I-iodocholesterol scanning Adrenal vein sampling for aldosterone
47
What is the treatment for primary aldosteronism?
Adrenal adenoma - adrenal surgery Spironolactone = competitive antagonist to aldosterone Amiloride = blocks Na channels in proximal tubule > decreases K excretion
48
What is phaeochromocytoma?
Tumour of chromaffin cells in adrenal medulla | Derived from neural crest cells
49
What are the clinical features of phaeochromocytoma?
``` 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
How is phaeochromocytoma diagnosed?
``` 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
What is the pre-operative treatment of phaeochromocytoma?
Alpha adrenergic blockade - phenoxybenamine Beta blockers if necessary, but never before alpha blockade Treatment, as required of - Arrhythmias - Cardiac failure - Diabetes
52
What is polyglandular autoimmune syndrome type 1?
Autosomal recessive disorder Very rare Mutation in AIRE gene on chromosome 21
53
What are the endocrine effects of polyglandular autoimmune syndrome type 1?
``` Hypoparathyroidism Chronic mucocutaneous candidiasis Adrenal insufficiency Primary hypogonadism Hypothyroidism T1D Hypothyroidism Hypopituitarism Diabetes insipidus ```
54
What are the non-endocrine effects of polyglandular autoimmune syndrome type 1?
``` Malabsorption syndromes Alopecia totalis/areata Pernicious anaemia Chronic active hepatitis Vitiligo ```
55
What is polyglandular autoimmune syndrome type 2?
``` 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
What are the endocrine effects of polyglandular autoimmune syndrome type 2?
``` Adrenal insufficiency Autoimmune thyroid disease T1D Primary hypogonadism Diabetes insipidus ```
57
What are the non-endocrine effects of polyglandular autoimmune syndrome type 2?
``` Vitiligo Alopecia Pernicious anaemia Myaesthenia gravis Immune thrombocytopenic purpura Sjogrens syndromes Rheumatoid arthritis ```
58
Where is the MEN1 gene located?
Chromosome 11
59
What is the inheritance pattern of MEN1 syndrome?
Autosomal dominant
60
What is the triad of tumours in MEN1 tumours?
Pituitary Pancreas Parathyroid
61
What is the inheritance pattern of MEN2 syndrome?
Autosomal dominant
62
What causes MEN2 syndrome?
Mutations of RET proto-oncogene on chromosome 10
63
What are the tumours in MEN2a syndrome?
Medullary carcinoma of thyroid Phaeochromocytoma Parathyroid hyperplasia
64
What are the tumours of MEN2b syndrome?
``` Medullary carcinoma of thyroid Phaeochromocytoma Parathyroid hyperplasia Mucosal neuromas Intestinal ganglioneuromas Marfanoid habitus ```