Endo - Adrenals Flashcards

1
Q

Hormone produced by Adrenal Medulla?

A

Adrenaline

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2
Q

Hormone produced by zona glomerulosa and mediated by?

A

Aldosterone

Mediated by K+ level and angiotensin II

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3
Q

Hormone produced by zona fasciculata and mediated by?

A

Cortisol

ACTH

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4
Q

Hormone produced by zona reticularis and mediated by?

A

DHEAS (androgens)

ACTH

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5
Q

Cortisol peak and trough?

A
Highest on waking
Lowest asleep (midnight)
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6
Q

Functions of cortisol?

A
Hyperglycaemia
Increased gluconeogenesis and resistance to insulin
Decreased lymph/mono/eos and B & T cells
Decreased growth
Decreased Ca+
Increased bone resorption
Increased androgens
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7
Q

Aldosterone stimulated by?

A

Angiotensin II
Hyperkalameia
ACTH

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8
Q

Aldosterone inhibited by?

A

Atrial Naturietic Peptide

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9
Q

Function of aldosterone?

A

Maintain intravascular volume
Increases Na channel expression in renal collecting ducts which increases Na+ resorption (bringing H20) and increases K+ excretion

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10
Q

Function of adrenaline and noradrenaline?

A

Raise MAP

Adrenaline increases cardiac output

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11
Q

1mg pred =?hydrocort
1mg Methylpred = ?hydrocort
1mg dexamethasone = ?hydrocort

A

1mg pred =4mg hydrocort
1mg Methylpred = 5mg hydrocort
1mg dexamethasone = 25mg hydrocort

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12
Q

Physiological requirement of hydrocortisone?

A

10mg/m2/day

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13
Q

Primary adrenal insufficiency due to what?

A

Inadequate adrenal function

Deficit in glucocorticoids and mineralocorticoids

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14
Q

Central adrenal insufficiency due to what?

A

Secondary - pituitary defect (ACTH)
Tertiary - hypothalamic defect (corticotrophin releasing hormone)
Deficit in glucocorticoids only

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15
Q

Signs of primary adrenal insufficiency?

A
Low cortisol
High ACTH
Hyponatremia
Hyperkalaemia
HTN
Hyperpigmentation of skin (bronze)
Fasting hypoglycaemia
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16
Q

Signs of secondary adrenal insufficiency?

A
Low cortisol
Low ACTH
Fasting hypoglycaemia
Increased insulin sensitivity
FTT, GIT upset, fatigue, weakness
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17
Q

Most common cause of primary adrenal insufficiency?

A

Congenital Adrenal Hyperplasia

18
Q

Signs of over replacement of glucocorticoids?

A

Excess hydrocortisone dose = weight gain, cushingoid features

19
Q

Sign of under replacement of glucocorticoids?

A

Insufficient hydrocortisone dose = hyperpigmentation, hypoglycaemia, FTT, high ACTH

20
Q

Signs of over replacement of mineralocorticoids?

A

Excess fludrocortisone = HTN and low renin

21
Q

Signs of under replacement of mineralocorticoids?

A

Insufficient fludrocortisone = salt craving, hyponatremia, hyperkalaemia, hypotension, high renin

22
Q

Most common form of CAH?

A

21 hydroxylase deficiency

23
Q

Second most common cause of CAH?

A

11-beta-hydroxylase deficiency

24
Q

Signs of 21 hydroxylase deficiency (classic) CAH?

A

Glucocorticoid deficiency
Mineralocorticoid deficiency
(typically adrenal crisis by D10-14 of life)
Ambiguous genitalia in females
Early virilisation - pubic/axillary hair, early skeletal maturation

25
Q

How is height affected in classic CAH?

A

Final height 8-10cm shorter

26
Q

Signs non-classic CAH?

A

Normal glucocorticoid and mineralocorticoid function
Isolated hyperandrogenism without adrenal insufficiency
Premature pubarche, BO, hisutism, pubic hair
Advanced bone age, reduced adult height

27
Q

Why does 11 beta hydroxylase deficiency have some mineralocorticoid function?

A

Blocks conversion of DOC to corticosterone and 11-DOC to cortisol however there is an adjacent pathway that is unaffected allowing aldosterone synthase production
DOC has mineralocorticoid function

28
Q

Clinical manifestation of 11 beta hydroxylase deficiency?

A

Excess androgens (but milder than CAH)
Excess mineralocorticoids (via DOC)
Cortisol insufficiency
-> HTN, hyperkalaemia, ambiguous female genitalia/increased penis size

29
Q

Treatment of 11 beta hydroxylase deficiency?

A

Hydrocortisone

Don’t give fludrocortisone usually - sometimes even need Spiro as mineralocorticoid antagonist

30
Q

Cushing syndrome vs Cushing disease?

A
Syndrome = prolonged glucocorticoid excess
Disease = specifically ACTH secreting pit adenoma
31
Q

Cushing syndrome + precocious puberty, fibrous dysplasia and cafe au lait?

A

McCune Albright

32
Q

Causes of ectopic ACTH production?

A
Islet cell ca 
neuroblastoma
Ganglioneuroblastoma
Wilms
Thymic carcinoid
33
Q

How does 2 step Dex suppression test differentiate ACTH dependant and independent cushing syndrome?

A

ACTH-independant (secondary) causes not suppressed with smaller or larger dose of cortisol
ACTH-dependant (central) causes will be suppressed with larger dose

34
Q

What is Conn’s Syndrome?

A

Excess aldosterone secretion (independent of RAAS)

35
Q

Child with hypernatremia, hypokalaemia, hypertension and high aldosterone and decreased renin activity?

A

Conn’s Syndrome

36
Q

Causes of Conn’s?

A

Aldosterone secreting adenoma
Bilateral nodular adrenocortical hyperplasia
Unilateral hyperplasia

37
Q

Cancer syndromes assoc w adrenocortical tumours?

A
Li Fraumeni (p53)
MEN1
Familial adenomatous polyposis 
PRKAR1A
Beckwith-Wiedemann
38
Q

Child presents with HTN, tachycardia, sweating, headache. Progresses to hypertensive encephalopathy. Good appetite but failing to thrive. Dx?

A

Phaechromocytoma

39
Q

What is a phaechromocytoma?

A

Catecholamine secreting tumour arising from chromatin cells -> produced adrenaline, noradrenaline and dopamine
In adrenal medulla 90%
10% elsewhere in sympathetic chain

40
Q

80% of phaeos related to familial disorders - what are these?

A
Von Hippel-Lindau 
MEN2A and 2B (bilat adrenalectomy req due to risk)
NF1
TS
Sturge Weber
Ataxia-Telangiectasia
41
Q

How to differentiate catecholamine secreting phaeo vs neuroblastoma?

A

Urine catechols higher in phaeo

Dopamine and homovanillic acid higher in neuroblastoma

42
Q

Retinal and CNA haemangioblastomas, phaechromocytoma what tumour suppressor defect?

A

Von Hippel-Lindau (VHL tumour suppressor mutation)