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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
What is a pheochromocytoma
Catecholamine secreting tumour
26
What is a pheochromocytoma typically derived from
Chromaffin cells of the adrenal medulla
27
What is pheochromocytoma known as
The 10% cancer
28
10% rule of pheochromocytoma
10% bilateral, malignant, outside of the adrenal gland
29
Name some genetic disorders associated with pheochromocytoma
MEN2 neurofibromatosis type 1 Von hippel-Lindau disease
30
Classic presentation of pheochromocytoma
Triad of hypertension, headache and sweating
31
Name some signs of complications of pheochromocytoma
LV failure, stroke, paralytic ileus of bowel, myocardial necrosis
32
2 initial tests of pheochromocytoma
Plasma free metanephrines 24 hour urine catecholamines
33
Imaging used in pheochromocytoma
CT or MRI
34
Management of pheochromocytoma
Alpha blockers THEN beta blockers THEN surgical removal
35
What is congenital adrenal hyperplasia
Inherited group of disorders with a deficiency in one of the enzymes necessary for cortisol synthesis
36
Inheritance in congenital adrenal hyperplasia
Autosomal recessive 21a-hydroxylase deficiency
37
Pathophysiology of congenital adrenal hyperplasia
21a-hydroxylase deficiency prevents the production of aldosterone and cortisol Increased testosterone Reduced cortisol stimulates ACTH release and cortical hyperplasia
38
What causes non-classical congenital adrenal hyperplasia
A partial 21a-dehydroxylase deficiency
39
When does non-classic congenital adrenal hyperplasia present
Adolescence/adulthood
40
How does congenital adrenal hyperplasia present
Precocious puberty Hirtuism Acne Oligomenorrhoea, infertility or sub-fertility
41
When does classic congenital adrenal hyperplasia present
At birth or just after
42
How does classic congenital adrenal hyperplasia present
Genital ambiguity in females Adrenal failure - biochemical pattern of Addisons disease
43
Investigations of congenital adrenal hyperplasia
Basal 17-OH progesterone Genetic analysis
44
Paediatric management of congenital adrenal hyperplasia
Glucocorticoid replacement Surgical correction Mineralocorticoid replacement in some patients
45
Adult management of congenital adrenal hyperplasia
Control androgen excess Glucocorticoid replacement Restore fertility
46
What is adrenal crisis
Acute, severe glucocorticoid deficiency
47
What causes adrenal crisis
Stress in a patient with underlying adrenal insufficiency OR sudden discontinuation of glucocorticoids after prolonged therapy
48
Clinical presentation of adrenal crisis
Stress Vomiting Abdominal pain Hypotension Shock
49
Management of adrenal crisis
Rehydration: rapid bolus then 0.9% sodium chloride Steroids: 100mg hydrocortisone IV injection then 24 hour infusion OR IM injection every 6 hours
50
What is Cushing’s syndrome
Increased free circulating glucocorticoid (cortisol)
51
What is cushings disease
A pituitary adenoma secreting excess ACTH which causes excess cortisol release from the adrenal glands
52
Who commonly presents with Cushing’s syndrome
Women aged 20-40
53
What is the most common cause of cortisol excess
Administration of synthetic steroids
54
Name the main causes of ACTH dependent Cushing’s syndrome
Cushing’s disease Carcinoma of the lungs or pancreas
55
Name the main causes of ACTH independent Cushing’s syndrome
Exogenous steroids Adrenal tumour Adrenal cortical nodular hyperplasia False positive: severe depression, severe alcoholism
56
Adrenal enlargement in ACTH-independent Cushing’s syndrome
Usually nodular
57
Adrenal enlargement in ACTH-dependent Cushing’s syndrome
Usually diffuse
58
Name some consequences of increased cortisol levels
Protein loss Altered carb and lipid metabolism Excess mineralocorticoid Excess androgen
59
Clinical features of Cushing’s syndrome
Moon face Central obesity, stretch marks Buffalo hump Muscle wasting Hirsutism Hyperpigmentation in patients with Cushing’s disease
60
3 tests to establish cortisol excess
Overnight dexamethasone suppression test 24 hr urine free cortisol Diurnal cortisol variation
61
Abnormal result for an overnight dexamethasone suppression test
>130 nmol/l cortisol
62
Diagnostic test for Cushing’s syndrome
Low dose dexamethasone suppression test
63
How to determine the cause of Cushing’s syndrome
Serum ACTH If low, adrenal imaging If high, pituitary MRI
64
Drug management of Cushing’s syndrome
Metyrapone when other treatments fail or waiting for radiotherapy to work
65
Surgical management of Cushing’s syndrome
Trans-sphenoidal removal of pituitary tumour Surgical removal of adrenal tumour OR ACTH-producing tumour elsewhere
66
What is hyperaldosteronism
High levels of aldosterone
67
What is conns syndrome
Adrenal adenoma producing too much aldosterone
68
What is primary hyperaldosteronism
Where the adrenal glands are directly responsible for producing too much aldosterone
69
Causes of primary hyperaldosteronism
Bilateral adrenal hyperplasia Conns syndrome Familial hyperaldosteronism
70
What is the most common cause of secondary hypertension
Primary hyperaldosteronism
71
Clinical presentation of primary hyperaldosteronism
Significant hypertension Alkalosis Hypokalaemia
72
Management of conns syndrome
Unilateral laparoscopic adrenalectomy
73
Management of bilateral adrenal hyperplasia
Spironolactone
74
What causes secondary hyperaldosteronism
Excessive renin stimulating the release of excessive aldosterone
75
Causes of secondary hyperaldosteronism
Renal artery stenosis Heart failure Liver cirrhosis and ascites
76
What is renal artery stenosis
Narrowing of the artery supplying the kidney
77
What causes renal artery stenosis usually
Atheroma
78
What can be the cause of renal artery stenosis in younger females
Fibromuscular dysplasia
79
Clinical presentation of secondary hyperaldosteronism
Hypertension
80
Investigation in hyperaldosteronism
Aldosterone : renin ratio
81
High aldosterone low renin indicates…
Primary aldosteronism
82
High aldosterone high renin indicates…
Secondary hyperaldosteronism
83
What can be used to investigate renal artery stenosis
Doppler US CT angiogram MRA - magnetic resonance angiography
84
Management of renal artery stenosis
Percutaneous renal artery angioplasty via the femoral vein
85
Management of secondary hyperaldosteronism
Spironolactone
86
What causes secondary adrenal insufficiency
Lack of production of ACTH by the pituitary gland
87
What causes tertiary adrenal insufficiency
Lack of CRH secretion by the hypothalamus
88
Clinical presentation of secondary and tertiary adrenal insufficiency
SIMILAR TO ADDISONS no hypertension Pale skin
89
Management of secondary and tertiary adrenal insufficiency
Hydrocortisone replacement
90
What’s another name for primary adrenal insufficiency
Addisons disease
91
Features of adrenal insufficiency in babies
Lethargy, vomiting, poor feeding, hypoglycaemia, jaundice, failure to thrive
92
Clinical presentation of Addisons disease
Skin pigmentation Weakness, fatigue, N+V, weight-loss, dizziness and low BP, abdo pain
93
Biochemistry in Addisons disease
Low cortisol Low ACTH Low aldosterone High renin
94
Biochemistry in secondary adrenal insufficiency
Low cortisol Low ACTH normal aldosterone Normal renin
95
Test for adrenal insufficiency
Short synacthen test
96
Pharmacological management of Addisons disease
Hydrocortisone as cortisol replacement Fludrocortisone as aldosterone replacement
97
Sick day rules in adrenal insufficiency
Increase steroid replacement when unwell or undergoing other stress
98
what is the most common cause of addisons
autoimmune adrenalitis

endocrine (15 decks)

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