Adrenal hyperactivity Flashcards

1
Q

What are some causes or examples of Adrenal hyperactivity?

A

Primary hyperaldosteronism
Secondary hyperaldosteronism
Bilateral adrenal hyperplasia
Phaeochromocytoma
Neuroblastoma

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

What is meant by primary hyperaldosteronism?

A

This is a condition in which there is autonomous production of aldosterone, independant of its regulators (Angiotensin II and potassium)

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

What are some of the cardiovascular actions of aldosterone?

A
  • Increased sympathetic outflow
  • Increased cardiac collagen levels
  • Cytokine and ROS synthesis
  • Sodium retention
  • Increased blood pressure
  • Vasoconstriction
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

What is the most common cause of secondary hypertension?

A

Primary hyperaldosteronism

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

What are the main causes of primary hyperaldosteronism?

A

Adrenal adenoma (Conn’s syndrome)
Bilateral adrenal hyperplasia
Genetic
Unilateral hyperplasia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

What are some clinical features of primary hyperaldosteronism?

A
  • Significant hypertension
  • Hypokalaemia
  • Alkalosis
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

How is an aldosterone excess confirmed?

A
  • Measure plasma aldosterone and renin and express as a ratio (ARR)
  • If ratio raised, then investigate further with saline suppression test
  • Failure of plasma aldosterone to suppress by >50% with 2L of normal saline confirms primary aldosteronism
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

How is the sub-type of primary aldosteronism confirmed?

A
  • Adrenal CT to demonstrate adenoma (Conn’s disease)
  • Sometimes adrenal vein sampling to confirm adenoma is a true source of aldosterone excess
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

What is the main surgical management option for primary hyperaldosteronism?

A

Unilateral laparoscopic adrenalectomy

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

What is the medical management of primary hyperaldosteronism?

A

Spironolactone or eplerenone

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

What is an adrenal adenoma (Conn’s syndrome)?

A

Benign neoplasm emerging from the cells of the adrenal cortex

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Describe the genetics of adrenal adenoma formation

A
  • KCNJ5 channel is a rectifying selective channel which maintains membrane hyperpolarisation
  • Mutations lead to loss of ion selectivity; Na+ entry and depolarisation and therefore increased aldosterone production
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Describe the histology of adrenal adenoma

A
  • Composed of cells resembling adrenocortical cells
  • Well-differentiated, small nuclei
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

How will adrenal adenoma present?

A
  • Often asymptomatic
  • Excess hormone secretion (E.g. Conn’s syndrome, Cushing’s)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

How is adrenal adenoma investigated?

A
  • Imaging (CT, MRI)
  • Hormonal testing
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

How would a small adrenal lesion without biochemical abnormality be managed?

A

It can be safely left in situ

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

When would surgical excision of an adrenal adenoma be required?

A

Functioning lesion
Large lesion >3-5cm as this could be malignant

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

What is secondary hyperaldosteronism?

A

Increased adrenal production of aldosterone in response to nonpituitary, extra-adrenal stimuli such as renal hypoperfusion

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

What causes secondary hyperaldosteronism?

A

Reduced renal blood flow causes production of excess renin (Physiological response to low BP), causing production of angiotensin II and then aldosterone

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

What are some causes of reduced renal blood flow?

A
  • Obstructive renal artery disease (eg, atheroma, stenosis)
  • Renal vasoconstriction (as occurs in accelerated hypertension)
  • Oedematous disorders (e.g. heart failure, cirrhosis with ascites)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

What are some causes of renal artery stenosis?

A

Atherone
Fibromuscular dysplasia (In young females)

22
Q

How will secondary hyperaldosteronism usually present?

A

Hypertension

23
Q

What investigations are required in secondary hyperaldosteronism?

A
  • Renin/aldosterone ratio - high aldosterone and high renin indicates secondary hyperaldosteronism
  • Doppler ultrasound, CT angiogram or magnetic resonance angiography (MRA) to look for for renal artery stenosis or obstruction
24
Q

How is secondary hyperaldosteronism managed?

A
  • Aldosterone antagonists e.g. spironolactone
  • Treat underlying cause e.g. percutaneous renal artery angioplasty via the femoral artery to treat in renal artery stenosis
25
Q

What is a phaeochromocytoma?

A

These are rare tumours affecting the adrenal medulla

26
Q

What are some possible complications of phaeochromocytoma?

A

Arrhythmia
Ischaemia
Cerebral haemorrhage
Death

27
Q

How can phaeochromocytoma cause complications?

A

They can produce high levels of adrenaline and noradrenaline

28
Q

How may phaeochromocytoma affect the sympathetic chain?

A

They can form extra-adrenal tumours (Paragangliomas)

29
Q

What are some factors that increase the risk of phaeochromocytoma?

A

Surgery
Pregnancy

30
Q

What are some causative syndromes of phaeochromocytoma?

A
  • MEN2
  • Von Hippel Lindau (VHL)
  • Succinate dehydrogenase mutations
  • Neurofibromatosis
  • Tuberose sclerosis
31
Q

What is the main triad of symptoms in phaeochromocytoma?

A
  • Labile hypertension (On and off)
  • Headache
  • Paroxysmal sweating
32
Q

What are some other symptoms of phaeochromocytoma?

A
  • Pallor
  • Palpitations
  • Constipation
  • Anxiety
  • Weight loss
33
Q

What are some clinical signs of phaeochromocytoma?

A
  • Postural hypotension
  • Bradycardia and tachycardia
  • Pyrexia
34
Q

What are some investigations required in diagnosis of phaeochromocytoma?

A
  • Urine or plasma catecholamine excess
  • MRI scanning
  • MIBG scanning (Meta-iodobenzylguanidine)
  • Biochemistry
  • PET scanning
  • Gallium-Dotate scan
35
Q

What will biochemistry show in phaeochromocytoma?

A
  • Hyperglycaemia - Adrenaline secreting tumours
  • Possible hypokalaemia
  • High haematocrit
  • Mild hypercalcaemia
  • Lactic acidosis in absence of shock
36
Q

What are some conditions that can elevate urinary metanephrines?

A

Phaeochromocytoma
Heart failure
Hypertension
Drugs

37
Q

On which body region would MRI scanning be performed in phaeochromocytoma?

A

MRI scanning is usually performed on the abdomen or pelvis, unless a paraganglioma is suspected, in which a whole body scan is performed

38
Q

Who is screened for phaeochromocytoma?

A

Those will family history
Those with resistant hypertension
Those under 50 with hypertension
Those with classical symptoms of phaeochromocytoma

39
Q

Why is phaeochromocytoma known as the 10% tumour?

A

10% Malignant

10% Extra-adrenal (probably 20-30%)

10% bilateral

10% associated with hyperglycaemia

10% in children

10% familial (Probably 25-40%)

40
Q

What is the management plan in phaeochromocytoma?

A
  1. Full alpha and beta blockade (Alpha before Beta)
  2. Fluid and/or blood replacement (High salt diet as well)
  3. Careful anaesthetic assessment
  4. Stop blockade before surgery
  5. Surgical recision (If possible)
  6. Chemotherapy if malignant
  7. Radio-labelled MIBG
  8. Long term follow-up (10 years - lifelong)
  9. Genetic testing and family tracing
41
Q

What alpha blocker is used in phaeochromocytoma?

A

Phenoxybenzamine

42
Q

What ß-blockers can be used in phaeochromocytoma?

A

Propranolol
Atenolol
Metoprolol

43
Q

What is a neuroblastoma?

A

Malignant neuroendocrine tumour of the sympathetic nervous system that originates from neural crest cells

44
Q

Where can neuroblastoma occur?

A

40% arise in the adrenal medulla, remainder mostly occur along the sympathetic chain

45
Q

Describe the histology of neuroblastoma

A

Composed of primitive appearing cells but can show maturation and differentiation towards ganglioid cells

46
Q

What are some features of the tumour that predict a poor outcome?

A

Amplification fo N-myc and expression of telomerase

47
Q

What are some symptoms of neuroblastoma?

A

Late onset:
- Loss of appetite
- Vomiting
- Weight loss
- Fatigue
- Bone pain

48
Q

What are some investigations required in neuroblastoma?

A
  • Bloods - FBC may detect anaemia, raised inflammatory markers, abnormal coagulation tests
  • CT
  • Biopsy
49
Q

How is a low risk patient with neuroblastoma managed?

A

Low risk patients are simply observed for spontaneous resolution or are treated with local resection

50
Q

How is a high risk patient with neuroblastoma managed?

A

High risk patients are given multi-agent chemotherapy, surgery and radiotherapy