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endocrine > Adrenal Disorders > Flashcards

Adrenal Disorders Flashcards

1
Q

What is an adrenal adenoma

A

benign cancer emerging from the cells of the adrenal cortex

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2
Q

Associations with adrenal adenoma

A

Increase in frequency with age

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3
Q

Histological features of an adrenal adenoma

A

Well differentiated small, solitary lesions
Bright yellow and buried within the gland

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4
Q

How do patients usually get a diagnosis of adrenal adenoma

A

Often an incidental finding during abdominal imaging

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5
Q

How do patients with hyperfunctioning adrenal adenoma present

A

Excess hormone secretion e.g. cushings, conn syndrome

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6
Q

Investigations for adrenal adenoma

A

CT, MRI
hormonal testing

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7
Q

Management of adrenal adenoma without biochemical abnormality

A

Can be left in situ

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8
Q

When may a surgical excision of an adrenal adenoma be required

A

Lesion is functioning
Lesion is large - can be considered potentially malignant

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9
Q

What is an adrenocortical carcinoma

A

Very rare malignancy of the adrenal cortex

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10
Q

What is adrenocortical carcinoma associated with in young patients

A

Li-fraumeni syndrome

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11
Q

Histological features of an adrenocortical carcinoma

A

Large
Haemorrhage and necrosis
Capsular or vascular invasion

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12
Q

Local invasion of adrenocortical carcinoma

A

Retroperitoneum
Kidney

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13
Q

Metastasis of adrenocortical carcinoma

A

Usually haematogenous to the liver, lung and bone

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14
Q

Clinical presentation of adrenocortical carcinoma

A

Hormonal effects, abdominal mass effects
Carcinomas with necrosis can cause fever

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15
Q

Imaging of adrenocortical carcinoma

A

CT or MRI

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16
Q

Management of adrenocortical carcinoma

A

Resection with adjuvant therapy

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17
Q

What is neuroblastoma

A

Malignant neuroendocrine tumour of the sympathetic nervous system

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18
Q

Where does a neuroblastoma originate from

A

Neural crest cells

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19
Q

Mean age of diagnosis of a neuroblastoma

A

23 months

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20
Q

Symptoms of neuroblastoma

A

Loss of appetite
Vomiting
Weight loss
Fatigue
Bone pain

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21
Q

Bloods in neuroblastoma

A

Anaemia, raised inflammatory markers, abnormal coag

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22
Q

Investigations for neuroblastoma

A

Bloods, biopsy, CT

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23
Q

Management of neuroblastoma in low risk patients

A

Observed for spontaneous resolution or local resection

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24
Q

Management of neuroblastoma in high risk patients

A

Multi-agent chemotherapy, surgery and radiotherapy

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25
Q

What is a pheochromocytoma

A

Catecholamine secreting tumour

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26
Q

What is a pheochromocytoma typically derived from

A

Chromaffin cells of the adrenal medulla

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27
Q

What is pheochromocytoma known as

A

The 10% cancer

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28
Q

10% rule of pheochromocytoma

A

10% bilateral, malignant, outside of the adrenal gland

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29
Q

Name some genetic disorders associated with pheochromocytoma

A

MEN2
neurofibromatosis type 1
Von hippel-Lindau disease

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30
Q

Classic presentation of pheochromocytoma

A

Triad of hypertension, headache and sweating

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31
Q

Name some signs of complications of pheochromocytoma

A

LV failure, stroke, paralytic ileus of bowel, myocardial necrosis

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32
Q

2 initial tests of pheochromocytoma

A

Plasma free metanephrines
24 hour urine catecholamines

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33
Q

Imaging used in pheochromocytoma

A

CT or MRI

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34
Q

Management of pheochromocytoma

A

Alpha blockers
THEN beta blockers
THEN surgical removal

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35
Q

What is congenital adrenal hyperplasia

A

Inherited group of disorders with a deficiency in one of the enzymes necessary for cortisol synthesis

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36
Q

Inheritance in congenital adrenal hyperplasia

A

Autosomal recessive 21a-hydroxylase deficiency

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37
Q

Pathophysiology of congenital adrenal hyperplasia

A

21a-hydroxylase deficiency prevents the production of aldosterone and cortisol
Increased testosterone
Reduced cortisol stimulates ACTH release and cortical hyperplasia

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38
Q

What causes non-classical congenital adrenal hyperplasia

A

A partial 21a-dehydroxylase deficiency

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39
Q

When does non-classic congenital adrenal hyperplasia present

A

Adolescence/adulthood

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40
Q

How does congenital adrenal hyperplasia present

A

Precocious puberty
Hirtuism
Acne
Oligomenorrhoea, infertility or sub-fertility

41
Q

When does classic congenital adrenal hyperplasia present

A

At birth or just after

42
Q

How does classic congenital adrenal hyperplasia present

A

Genital ambiguity in females
Adrenal failure - biochemical pattern of Addisons disease

43
Q

Investigations of congenital adrenal hyperplasia

A

Basal 17-OH progesterone
Genetic analysis

44
Q

Paediatric management of congenital adrenal hyperplasia

A

Glucocorticoid replacement
Surgical correction
Mineralocorticoid replacement in some patients

45
Q

Adult management of congenital adrenal hyperplasia

A

Control androgen excess
Glucocorticoid replacement
Restore fertility

46
Q

What is adrenal crisis

A

Acute, severe glucocorticoid deficiency

47
Q

What causes adrenal crisis

A

Stress in a patient with underlying adrenal insufficiency
OR
sudden discontinuation of glucocorticoids after prolonged therapy

48
Q

Clinical presentation of adrenal crisis

A

Stress
Vomiting
Abdominal pain
Hypotension
Shock

49
Q

Management of adrenal crisis

A

Rehydration: rapid bolus then 0.9% sodium chloride
Steroids: 100mg hydrocortisone IV injection then 24 hour infusion OR IM injection every 6 hours

50
Q

What is Cushing’s syndrome

A

Increased free circulating glucocorticoid (cortisol)

51
Q

What is cushings disease

A

A pituitary adenoma secreting excess ACTH which causes excess cortisol release from the adrenal glands

52
Q

Who commonly presents with Cushing’s syndrome

A

Women aged 20-40

53
Q

What is the most common cause of cortisol excess

A

Administration of synthetic steroids

54
Q

Name the main causes of ACTH dependent Cushing’s syndrome

A

Cushing’s disease
Carcinoma of the lungs or pancreas

55
Q

Name the main causes of ACTH independent Cushing’s syndrome

A

Exogenous steroids
Adrenal tumour
Adrenal cortical nodular hyperplasia
False positive: severe depression, severe alcoholism

56
Q

Adrenal enlargement in ACTH-independent Cushing’s syndrome

A

Usually nodular

57
Q

Adrenal enlargement in ACTH-dependent Cushing’s syndrome

A

Usually diffuse

58
Q

Name some consequences of increased cortisol levels

A

Protein loss
Altered carb and lipid metabolism
Excess mineralocorticoid
Excess androgen

59
Q

Clinical features of Cushing’s syndrome

A

Moon face
Central obesity, stretch marks
Buffalo hump
Muscle wasting
Hirsutism
Hyperpigmentation in patients with Cushing’s disease

60
Q

3 tests to establish cortisol excess

A

Overnight dexamethasone suppression test
24 hr urine free cortisol
Diurnal cortisol variation

61
Q

Abnormal result for an overnight dexamethasone suppression test

A

> 130 nmol/l cortisol

62
Q

Diagnostic test for Cushing’s syndrome

A

Low dose dexamethasone suppression test

63
Q

How to determine the cause of Cushing’s syndrome

A

Serum ACTH
If low, adrenal imaging
If high, pituitary MRI

64
Q

Drug management of Cushing’s syndrome

A

Metyrapone when other treatments fail or waiting for radiotherapy to work

65
Q

Surgical management of Cushing’s syndrome

A

Trans-sphenoidal removal of pituitary tumour
Surgical removal of adrenal tumour OR ACTH-producing tumour elsewhere

66
Q

What is hyperaldosteronism

A

High levels of aldosterone

67
Q

What is conns syndrome

A

Adrenal adenoma producing too much aldosterone

68
Q

What is primary hyperaldosteronism

A

Where the adrenal glands are directly responsible for producing too much aldosterone

69
Q

Causes of primary hyperaldosteronism

A

Bilateral adrenal hyperplasia
Conns syndrome
Familial hyperaldosteronism

70
Q

What is the most common cause of secondary hypertension

A

Primary hyperaldosteronism

71
Q

Clinical presentation of primary hyperaldosteronism

A

Significant hypertension
Alkalosis
Hypokalaemia

72
Q

Management of conns syndrome

A

Unilateral laparoscopic adrenalectomy

73
Q

Management of bilateral adrenal hyperplasia

A

Spironolactone

74
Q

What causes secondary hyperaldosteronism

A

Excessive renin stimulating the release of excessive aldosterone

75
Q

Causes of secondary hyperaldosteronism

A

Renal artery stenosis
Heart failure
Liver cirrhosis and ascites

76
Q

What is renal artery stenosis

A

Narrowing of the artery supplying the kidney

77
Q

What causes renal artery stenosis usually

A

Atheroma

78
Q

What can be the cause of renal artery stenosis in younger females

A

Fibromuscular dysplasia

79
Q

Clinical presentation of secondary hyperaldosteronism

A

Hypertension

80
Q

Investigation in hyperaldosteronism

A

Aldosterone : renin ratio

81
Q

High aldosterone low renin indicates…

A

Primary aldosteronism

82
Q

High aldosterone high renin indicates…

A

Secondary hyperaldosteronism

83
Q

What can be used to investigate renal artery stenosis

A

Doppler US
CT angiogram
MRA - magnetic resonance angiography

84
Q

Management of renal artery stenosis

A

Percutaneous renal artery angioplasty via the femoral vein

85
Q

Management of secondary hyperaldosteronism

A

Spironolactone

86
Q

What causes secondary adrenal insufficiency

A

Lack of production of ACTH by the pituitary gland

87
Q

What causes tertiary adrenal insufficiency

A

Lack of CRH secretion by the hypothalamus

88
Q

Clinical presentation of secondary and tertiary adrenal insufficiency

A

SIMILAR TO ADDISONS
no hypertension
Pale skin

89
Q

Management of secondary and tertiary adrenal insufficiency

A

Hydrocortisone replacement

90
Q

What’s another name for primary adrenal insufficiency

A

Addisons disease

91
Q

Features of adrenal insufficiency in babies

A

Lethargy, vomiting, poor feeding, hypoglycaemia, jaundice, failure to thrive

92
Q

Clinical presentation of Addisons disease

A

Skin pigmentation
Weakness, fatigue, N+V, weight-loss, dizziness and low BP, abdo pain

93
Q

Biochemistry in Addisons disease

A

Low cortisol
Low ACTH
Low aldosterone
High renin

94
Q

Biochemistry in secondary adrenal insufficiency

A

Low cortisol
Low ACTH
normal aldosterone
Normal renin

95
Q

Test for adrenal insufficiency

A

Short synacthen test

96
Q

Pharmacological management of Addisons disease

A

Hydrocortisone as cortisol replacement
Fludrocortisone as aldosterone replacement

97
Q

Sick day rules in adrenal insufficiency

A

Increase steroid replacement when unwell or undergoing other stress

98
Q

what is the most common cause of addisons

A

autoimmune adrenalitis

endocrine (15 decks)

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