Adrenal Disorders Flashcards

1
Q

What are some features of Addison’s disease?

A
  • Lethargy
  • Weakness
  • Anorexia
  • Nausea & Vomiting
  • Weight loss, ‘salt-craving’
  • Hyperpigmentation (especially palmar creases), vitiligo, loss of pubic hair in women
  • Hypotension
  • Hypoglycaemia
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

What are some causes of Addison’s disease?

A

Primary causes

  • Autoimmune destruction of the adrenal glands is the commonest cause of primary hypoadrenalism in the UK, accounting for 80% of cases.
  • Tuberculosis
  • Metastases (e.g. bronchial carcinoma)
  • Meningococcal septicaemia (Waterhouse-Friderichsen syndrome)
  • HIV
  • Antiphospholipid syndrome

Secondary causes

  • Pituitary disorders (e.g. tumours, irradiation, infiltration)
  • Exogenous glucocorticoid therapy
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

How is Addison’s disease managed?

A

Both Glucocorticoid and Mineralocorticoid replacement therapy as combination of:

  • Hydrocortisone: usually given in 2 or 3 divided doses. Patients typically require 20-30 mg per day, with the majority given in the morning dose
  • Fludrocortisone
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

How are patients educated in regards to their Addison’s disease?

A
  • Emphasise the importance of not missing glucocorticoid doses o
  • Consider MedicAlert bracelets and steroid cards
  • Discuss how to adjust the glucocorticoid dose during an intercurrent illness
    • Glucocorticoid dose should be doubled in simple terms
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

What are some precipitating factors of an Addisonian Crisis?

A
  • Sepsis or Surgery causing an acute exacerbation of chronic insufficiency (Addison’s, Hypopituitarism)
  • Adrenal haemorrhage e.g. Waterhouse-Friderichsen syndrome (fulminant meningococcaemia)
  • Steroid withdrawal
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

What are symptoms of Addisonian Crisis?

A
  • Collapse
  • Shock
  • Pyrexia
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

What are some electrolyte imbalances experienced thin an Addisonian Crisis?

A
  • Hyponatraemia
  • Hyperkalaemia
  • Hypoglycaemia
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

How is Addisonian Crisis managed?

A
  • IV Hydrocortisone 100 mg
  • 1 litre normal saline infused over 30-60 mins or with dextrose if hypoglycaemia
  • Continue hydrocortisone 6 hourly until the patient is stable
  • Oral replacement may begin after 24 hours and be reduced to maintenance over 3-4 days
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

What is more common: endogenous or exogenous causes of Cushing’s syndrome?

A

Exogenous causes of Cushing’s more common than endogenous ones.

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

What are some causes of Cushing’s syndrome?

A

ACTH dependent causes

  • Cushing’s disease (80%): pituitary tumour secreting ACTH producing adrenal hyperplasia
  • Ectopic ACTH production (5-10%): e.g. small cell lung cancer

ACTH independent causes

  • Iatrogenic: steroids
  • Adrenal adenoma (5-10%)
  • Adrenal carcinoma (rare)
  • Carney complex: syndrome including cardiac myxoma
  • Micronodular adrenal dysplasia (very rare)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

What is Pseudo-Cushing’s syndrome?

A
  • Often due to alcohol excess or severe depression
  • False positive dexamethasone suppression test or 24 hr urinary free cortisol
  • Insulin stress test is used to differentiate between true Cushing’s and pseudo-Cushing’s
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

What are clinical features of Cushing’s syndrome?

A

Cushing’s syndrome

  • Moon face
  • Buffalo hump
  • Striae
  • Weak muscles
  • Acne
  • Fragile skin
  • High Blood pressure
  • Weight gain
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Who do blood results show in Cushing’s syndrome?

A
  • Hypokalaemic metabolic alkalosis
  • Impaired glucose tolerance
  • Ectopic ACTH secretion (e.g. secondary to small cell lung cancer) is characteristically associated with very low potassium levels
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

What are definitive investigations for Cushing’s syndrome?

A
  • 24 hr urinary free cortisol
    • The first-line localisation is 9am and midnight plasma ACTH (and cortisol) levels. If ACTH is suppressed then a non-ACTH dependent cause is likely such as an adrenal adenoma
  • Overnight dexamethasone suppression test (most sensitive)
    • Both low- and high-dose dexamethasone suppression tests may be used to localise the pathology resulting in Cushing’s syndrome.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

How is low- and high-dose ODST interpreted?

A

These tests may be interpreted as follows:

  • Not suppressed by low-dose dexamethasone: Cushing’s syndrome not due to primary causes, i.e. likely secondary to corticosteroid therapy
  • Not suppressed by low-dose, but suppressed by high-dose dexamethasone indicates Cushing’s disease
  • Not suppressed by low- or high-dose dexamethasone indicates ectopic ACTH syndrome likely
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

What is Phaeochromocytoma?

A
  • Phaeochromocytoma is a rare catecholamine secreting tumour.
  • It is a neuroendocrine tumour of chromaffin cells of the adrenal medulla.
17
Q

What are symptoms of Pheochromocytoma?

A
  • Hypertension (around 90% of cases, may be sustained)
  • Headaches
  • Palpitations
  • Sweating
  • Anxiety
18
Q

What are the investigations of Pheochromocytoma?

A
  • 1st line: 24 hr urinary collection of metanephrines (sensitivity 97%*)
  • 2nd line: 24 hr urinary collection of catecholamines (sensitivity 86%)

CT and MRI scanning are both used to localise the lesion.

19
Q

What is the medical treatment of Pheochromocytoma?

A
  • Phenoxybenzamine: irreversible alpha blockade
  • Labetolol: may be co-administered for cardiac chronotropic control.
20
Q

What is the surgical management of Pheochromocytoma?

A
  • Fluids Perioperatively
  • Surgery once medically optimised
    • Adrenals are highly vascular structures and removal can be complicated by catastrophic haemorrhage in the hands of the inexperienced
21
Q

What are factors of a Pheochromocytoma that suggest benign lesions?

A
  • Size less than 3cm
  • Homogeneous texture
  • Lipid rich tissue
  • Thin wall to lesion
22
Q

What are the causes of Primary Aldosteronism?

A
  • Adrenal Adenoma (Conn’s Syndrome)
  • Bilateral idiopathic adrenal hyperplasia
  • Adrenal Carcinoma
23
Q

What are features of Primary Aldosteronism?

A
  • Hypertension
  • Hypokalaemia (e.g. muscle weakness)
  • Alkalosis
24
Q

What are investigations of Primary Aldosteronism?

A

1st line: Plasma aldosterone/renin ratio in suspected primary hyperaldosteronism

  • Shows high aldosterone levels alongside low renin levels (negative feedback due to sodium retention from aldosterone) in primary hyperaldosteronism

High-resolution CT abdomen and adrenal vein sampling is used to differentiate between unilateral and bilateral sources of aldosterone excess

25
Q

What is the management of Primary Aldosteronism?

A
  • Adrenal adenoma: surgery
  • Bilateral adrenocortical hyperplasia: aldosterone antagonist e.g. spironolactone
26
Q

What is Congenital Adrenal Hyperplasia?

A

Group of autosomal recessive disorders which affect adrenal steroid biosynthesis

27
Q

What causes changes within Congenital Adrenal Hyperplasia?

A
  • In response to resultant low cortisol levels the anterior pituitary secretes high levels of ACTH
  • ACTH stimulates the production of adrenal androgens that may virilize a female infant
28
Q

What are the causes of Congenital Adrenal hyperplasia?

A
  • 21-hydroxylase deficiency (90%)
  • 11-beta hydroxylase deficiency (5%)
  • 17-hydroxylase deficiency (very rare)
29
Q

What are the biochemical abnormalities of Congenital Adrenal Hyperplasia?

A
  • Increased plasma 17-hydroxyprogesterone levels
  • Increased plasma 21-deoxycortisol levels
  • Increased urinary adrenocorticosteroid metabolites
30
Q

What is a Pituitary Adenoma?

A

A pituitary adenoma is a benign tumour of the pituitary gland. They are common (10% of all people1)

31
Q

How are Pituitary Adenomas classified?

A
  • Size (a microadenoma is <1cm and a macroadenoma is >1cm)
  • Hormonal status
    • Secretory/functioning adenoma produces and excess of a particular hormone and a non-secretory/functioning adenoma does not produce a hormone to excess
32
Q

What are the most common types of Pituitary Adenomas?

A
  • Prolactinomas are the most common type and they produce an excess of prolactin.
  • After prolactinomas, non-secreting adenomas are the next most common, then GH secreting and then ACTH secreting adenomas.
33
Q

How do Pituitary Adenomas causes problems?

A
  • Excess of a hormone (e.g. Cushing’s disease due to excess ACTH, acromegaly due to excess GH or amenorrhea and galactorrhoea due to excess prolactin)
  • Depletion of a hormone(s) (due to compression of the normal functioning pituitary gland)
  • Stretching of the Dura within/around pituitary fossa (causing headaches)
  • Compression of the Optic Chiasm (causing a bitemporal hemianopia due to crossing nasal fibres
34
Q

What are investigations undertaken for Suspected Pituitary Adenomas?

A
  • Pituitary blood profile (including: GH, prolactin, ACTH, FH, LSH and TFTs)
  • Formal visual field testing
  • MRI brain with contrast
35
Q

How is a Pituitary Adenoma Treated?

A
  • Hormonal therapy
  • Surgery (e.g. transsphenoidal transnasal hypophysectomy)
  • Radiotherapy
36
Q

What are differentials for Pituitary Adenomas?

A
  • Pituitary hyperplasia
  • Craniopharyngioma
  • Meningioma
  • Brain metastases
  • Lymphoma
  • Hypophysitis
  • Vascular malformation (e.g. aneurysm)