Clinical Aspects of Adrenal Disorders Flashcards

1
Q

What does each layer of the adrenal glands secrete?

A

Zone Glomerulosa- Aldosterone

Zone Faticulata- Cortisol

Zona Reticularis- Androgens

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2
Q

What can hypofunction of the adrenal glands be caused by?

A
  • Primary adrenal insufficiency
    • Addison’s disease
      • Immune destruction
      • Invasion
      • Infiltration
      • Infection
      • Infarction
      • Iatrogenic
    • Adrenal enzyme defect
      • Congenital adrenal hyperplasia (most commonly 21-hydroxylase deficiency)
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3
Q

What enzyme most commonly causes adrenal enzyme defect?

A

21-hydrolase deficiency

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4
Q

What does adrenal enzyme defect cause?

A

Congenital adrenal hyperplasia

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5
Q

State the common autoantibodies responsible for Addisions disease

A

Positive adrenal autoantibodies to 21-hydroxylase

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6
Q

How is the adrenal cortex involved in Addison’s disease?

A

Lymphatic infiltrate of adrenal cortex

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7
Q

Addison’s disease is often associated with other autoimmune disease, what are some examples?

A

Thyroid disease

Type 1 diabetes mellitus

Premature ovarian failure

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8
Q

What are some common symptoms of primary adrenal failure?

A
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9
Q

What are some clues to a diagnosis of adrenal failure?

A
  • Disproportion between severity of illness and circulatory collapse/hypotension/dehydration
  • Unexplained hypoglycaemia
  • Other endocrine features (hypothyroidism, body hair loss, amenorrhoea)
  • Previous depression or weight loss
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10
Q

Investigations for Adrenal Insufficiency

A

Synacthen test (and basal ACTH)- synthetic ACTH given- +ve if cortisol does not increase

Routine bloods (U and Es, glucose, FBC)

Random cortisol (<450nmol/L)

MRI/CT for Adrenal/Pituitary tumours

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11
Q

Treatment for Primary Adrenal Insuffiency

A

Glucocorticoid replacement (replace cortisol):

  • Hydrocortisone 20-30mg
  • Prednisolone 7.5mg
  • Dexamethasone 0.75mg

Minerocorticoid Replacement (aldosterone):

  • Fludocrtisone 50-300mg
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12
Q

What are 3 important self-care rules for patients on steroids?

A
  • Never miss steroid dose
  • Double the hydrocortisone dose in the event on intercurrent illness (such as flu or UTI)
  • If severe vomiting or diarrhoea call for help without delay
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13
Q

What are some causes of hypersecretion of adrenal glands?

A
  • Cortex
    • Cushing’s syndrome (cortisol, androgens)
      • Adenoma, carcinoma or bilateral hyperplasia
    • Conn’s syndrome (aldosterone)
      • Adenoma or bilateral hyperplasia
  • Medulla
    • Phaeochromocytoma
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14
Q

What does Cushing’s syndrome cause hypersecretion of?

A

Cortisol, androgens

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15
Q

What does Conn’s syndrome cause hypersecretion of?

A

Aldosterone

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16
Q

What are some causes of primary Cushing’s syndrome?

A

Pituitary Adenoma, carcinoma or bilateral hyperplasia

17
Q

What are some causes of primary Conn’s syndrome?

A

Adenoma or bilateral hyperplasia

18
Q

What is the clinical presentation of Cushing’s syndrome?

A
19
Q

What are some ACTH dependent causes of Cushing’s syndrome?

A
  • Pituitary tumour (Cushing’s disease)
    • 75% of cases
  • Ectopic ACTH secretion (such as lung carcinoid)
    • 5% of cases
20
Q

What are some ACTH indendent causes of Cushing’s syndrome?

A

Adrenal Tumour

21
Q

Investigations for Cushings

A

1) 24hr Urinary free cortisol (12-135nmol/day)
2) Low Dose Dex test
3) High Dose Dex test (if low dose comes back abnormal)

22
Q

Investigations for Conns Syndrome

A
  • 1st Line: Aldosterone to renin ratio (Plasma Aldosterone:Plasma Renin Activity Ratio PA:PRA)
  • 24Hr Urinary aldosterone (12 ug/day)
  • CT MRI
  • Serum Electrolytes (High Na, Low K)
  • Blood gas- alkalosis
23
Q

What does low PA low PRA potentially indicate

A

Congenital Adrenal Hyperplasia

24
Q

What does high PA low PRA potentially indicate

A

Conns Syndrome

25
Q

What does high PA high PRA potentially indicate

A

Seconday Hyperaldosteronism (renal problems)

26
Q

What is phaeochromocytoma?

A

Rare tumour of adrenal gland tissue that causes release of epinephrine and norepinephrine

27
Q

What is the clinical presentation of phaeochromocytoma?

A
  • Hypertension
  • Paroxysmal attacks
    • Headache
    • Sweating
    • Palpitations
    • Tremor
    • Pallor
    • Anxiety
28
Q

What percentage of phaemchromocytoma cases are due to inherited causes and state 2 common ones

A

30% of the time

MEN 2 & Von Hippel- Landau

29
Q

Investgations for Phaechomocytoma

A

1) Clinical suspicion
2) Endocrine workout

  • Free metanephrine in plasma or urine
  • ALD/PRA ratio

3) Imaging work up

  • CT
  • Chemical shift MRI
30
Q

Pathophysiology of congenital adrenal hyperplasia

A

21-hydroxylase deficiency (90%)

31
Q

Complications of congenital adrenal hyperplasia

A

Neonatal salt crises

Ambiguous genitalia (girls)

Pseudo-precocious puberty (boys)

Hirturism (women)

32
Q

Complications of Addisions Disease (Addisonian Crisis)

A

Hyperkalemia

Hyponatreamia

Hypoglycaemia

Hypotension

Reduced Consciousness

33
Q

Management of an Addisionian Crisis

A

Paraenteral Steriods

IV Fluid Resus

Correct Hypoglycaemia

Electrolyte Correction

34
Q

Complications of Cushings Syndrome

A

Tissue breakdown causes:

  • Muscle wasting
  • Bone weakness
  • Skin Weakness

Insulin is anatogonised by cortisol:

  • Diabetes

Immune supression

Hypertension & Cardiac Hypertrophy due to Na++++

35
Q

Management of Cushings

A

Reduce Steriods

Surgery

Meds to detroy adrenal cortico cells:

  • Mitotane

Radiotherapy

36
Q

Management of Conns Syndrome

A

Surgey

Aldosterone Antagonists:

  • Epilerenone
  • Spironolactone
37
Q

1 Complication of Conns

A

Secondary Hypertension due to Na+++++

38
Q

Phaechromocytoma is the 10% tumour

A

10%

  • Bilateral
  • Tumour
  • Malignant
  • Outside adrenal gland
  • Hyperglycaemia