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AY Endocrine > Adrenal disorders > Flashcards

Adrenal disorders Flashcards

1
Q

Causes of primary adrenal insuficiency

A

Addisn’s disease
Cogenital adrenal hyperplasia
Adrenal TB
Adrenal malignancy

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2
Q

Causes of secondary arenal insufficiency

A

Lack of ATCH stimulation
Iatrogenic (excess exogenous steroid)
Pituitary/hypothalamic disorders

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3
Q

What is the commonest cause of primary adrenal insufficiency?

A

Addison’s disease

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4
Q

What is Addison’s disease?

A

Autoimmune destruction of adrenal cortes

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5
Q

What percentage of the adrenal cortex is usually destroyed before the patient becomes symptomatic?

A

> 90%

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6
Q

What is Addison’s disease associated with?

A

Type 1 diabetes
Autoimmune thyroid problems
Pernicious anaemia

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7
Q

Clinical features of Addison’s disease

A 
Anorexia/weight loss
Dizziness
Low BP 
Abdo pain 
Vomiting 
Diarrhoea 
Skin pigmentation
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8
Q

What causes skin pigmentation in Addison’s disease?

A

As when Addison’s destroy the adrenal cortex the brain responds by making more ACTH
ACTH is derived from POMC which is also a precursor for melanocyte stimulating hormone

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9
Q

What can precipitate Addisonian crisis?

A

Stress
Infection
Trauma
Surgery

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10
Q

Symptoms/signs of Addisonian crisis

A

Vomiting
Abdo pain
Hypotension
Shock & death

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11
Q

What biochemical findings would make you suspicious of Addison’s disease?

A

Low sodium
High potassium
Hypoglycaemia

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12
Q

How is Addison’s disease diagnosed?

A

Short synacthen test

(Addison’s excluded if after half an hour plasma cortisol >550mmol/L

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13
Q

When should treatment of Addison’s be commenced?

A

Do not delay treatment to confirm diagnosis

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14
Q

Treatment of Addison’s disease

A

Hydrocortisone (cortisol replacement

Flurocortisone (aldosterone replacement)

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15
Q

What it is important to monitor in fludrocortisone therapy?

A

K & BP

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16
Q

What is the commonest cause of secondary adrenal insufficiency?

A

Exogenous steroids

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17
Q

How does exogenous steroid use differ biochemically from Addisons disease?

A

Normal ACTH

Aldosterone production intact

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18
Q

Adrenocotical carcinoma

A

Rare
Usually functional
50% dead within 2 years

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19
Q

Adenoma appearance

A 
Well cricumscribed 
Usually small 
Yellow/brown cut surface 
Well differentiated 
Small nucleus
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20
Q

What is Conn’s syndrome?

A

Primary hyperaldosteronism

21
Q

ACTH-dependent causes of hypercortisolism

A 
ATCH secreting pituitary tumour (Cushing's disease) 
Ectopic ACTH (small cell lung cancer)
22
Q

ACTH-independent causes of hypercortisolism

A

Adrenal adenoma or carcinpoma

Non-lesional adrenal gland atrophies

23
Q

What is primary hyperaldosteronism usually associated with?

A

Diffuse or nodular hyperplasia of both adrenal glands

24
Q

Cuaes of secondary hyepraldosternism

A

Increased renin
Decreased renal perfusion
Hypovolaemia
Pregnancy

25
Q

When is an adrenal neuroblastoma usually diagnosed?

A

18 months

26
Q

What percentage of adrenal neuroblastomas arise in teh medulla?

A

40%

rest are mainly along sympathetic chain

27
Q

What are adrenal neuroblastomas composed of?

A

Primitive appearing cells but can show maturation and differentiation toward ganglion cells

28
Q

What 2 factors indicate a poor outcome in adrenal neuroblastoma?

A

Amplification of N-myc

Expression of telomerase

29
Q

What is phaechromocytoma a feature of?

A

MEN 1 gene
MEN 2A (Sipple syndrome)
MEN 2B

30
Q

What are the features of MEN 2A (Sipple syndrome)?

A

Phaechromocytoma (may be bilateral or occur at extra adrenal sites)
Medullary tyroid carcinoma
Parathyroid hyperplasia

31
Q

What are the features of MEN 2B?

A

Phaechromocytoma
mEDULLARY THYROID CARCINMOA
nEUROMAS

32
Q

MEN 2B & MEN 2A are both linked to which mutation?

A

Germline mutation in RET oncogene

33
Q

What is Von Hippel Lindau syndrome?

A

Mutation in VHL gene causing an accumulation of HIF proteins & stimulation of cellular proliferation

34
Q

What screening is vital in Von Hippel Lindau syndrome?

A

Vascular screening

35
Q

What is a phaeochromocytoma?

A

A neoplasm derived from chromaffin cells of the adrenal medulla
Secretes catecholamines
Rare cause of secondary hypertension

36
Q

What are the 10% rules of phaechromocytoma?

A

10% are extra-adrenal (paragangliomas, carotid body)
10% are bilateral
10% are malignant (defined by mets, more common in extra-adrenal)
10% are NOT associated with hypertension
25% are familial

37
Q

How do familial phaeo’s tend to present?

A

Younger
More often bilateral
More often malignant

38
Q

Treatment of phaeochromocytoma

A

Alpha blocker then beta blocker

39
Q

Where is the most common metastatic spread for phaechromocytoma?

A

Skeleton

40
Q

Complications of phaechromocytoma

A

Cardiac failure
Infarction
Arrythmias
CVA

41
Q

How is phaeochrmocytoma diagnosed?

A

Detection of urinary excretion of catecholamines + metabolites
MRI

42
Q

Symptoms/signs of Cushing’s disease

A 
Osteoporosis
Buffalo hump 
Thin skin 
AVN of femoral head
Proximal myopathy 
Frontal balding in women 
Moon face
Excess androgen (virilism, hirsutism, acne, ammenorrhoea) 
Conjunctival oedema 
Hypertension
43
Q

How is Cushing’s syndrome diagnosed? (second line)

A

2 day Dexamethasone Supression Test
- 2mg/day
Cortisol

44
Q

How is Cushing’s syndrome diagnosed? (first line)

A

Overnight 1mg dexamethasone supression test

cortisol

45
Q

Causes of cushing’s syndrome?

A

Pituitary
Adrenal adenoma
Ectopic (thymus, lung, pancreatic)
Steroid medication

46
Q

Treatment of adrenal caused Cushing’s

A

Adrenolectomy

47
Q

Treatmnet of ectopic cause of cushings

A

Remove ectopic tissue or bilateral adrenalectomy

48
Q

What would be the level of ACTH in a ectopic cause of cushing’s?

A

> 300

49
Q

What is the role of metyrapone in the treatment of Cushing’s?

A

While waiting for radiotherapy to work

S/E common

AY Endocrine (18 decks)

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