Adrenal Causes of Hypertension Flashcards

1
Q

What is endocrinology about?

  • Too much hormone - single nodule, multiple nodules, generalised increase in tissue (…)
  • Not enough hormone
  • Gland too big - which … … …
  • Exclusion of … in a single nodule
A
  • Too much hormone - single nodule, multiple nodules, generalised increase in tissue (hyperplasia)
  • Not enough hormone
  • Gland too big - i.e. squashes surrounding tissue
  • Exclusion of cancer in a single nodule
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2
Q

What is shown in this image?

A

Adrenal gland section - note the shape

below is CT image of it

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3
Q

Layers of adrenal gland (GFR) and medulla

Cortex is divided into:

  • Zona Glomerulosa - makes …
  • Zona Fasciculata - makes …
  • Zona Reticularis - makes …

Medulla makes … (adrenaline, noradrenaline, dopamine)

A
  • Zona Glomerulosa - makes aldosterone
  • Zona Fasciculata - makes cortisol
  • Zona Reticularis - makes adrenal androgens
  • Medulla makes catecholamines (adrenaline, noradrenaline, dopamine)
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4
Q

Causes of Hypertension?

  • … hypertension
  • renal hypertension - primary renal …, renal artery …
  • … causes
A
  • essential hypertension
  • renal hypertension - primary renal disease, renal artery stenosis
  • endocrine causes
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5
Q

Adrenal causes of Hypertension

  • Primary …
    • zona glomerulosa - produces too much … due to either an …, hyper… or rare … causes
  • …chromocytoma
    • tumour of the adrenal …
  • some forms of … adrenal hyperplasia - … defect - uncommon
A
  • Primary Hyperaldosteronism
    • zona glomerulosa - produces too much aldosterone due to an adenoma, hyperplasia or rare genetic causes
  • Phaeochromocytoma
    • tumour of the adrenal medulla
  • some forms of congenital adrenal hyperplasia - enzyme defect - uncommon
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6
Q

What pathway is this? (what does it secrete)

A

Pathway for aldosterone secretion

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7
Q

Pharmacology - hypertension

  • Renin …
  • ACE …
  • A2 receptor …
  • Aldosterone …
A
  • Renin inhibitors
  • ACE inhibitors
  • A2 receptor blockers
  • Aldosterone antagonists
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8
Q

Primary Hyperaldosteronism - why screen?

  • Individuals with PA have more … and … pathology than people with essential hypertension and similar blood pressure
A

Individuals with PA have more vascular and renal pathology than people with essential hypertension and similar blood pressure

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9
Q

Hypertension - is it Primary Hyperaldosteronism? Who do we screen?

  • younger or older people?
  • people with hypo….
  • people with … hypertension - control on … drugs or more
A
  • younger or older people?
  • people with hypokalaemia
  • people with resistant hypertension - control on 4 drugs or more (inadequate control on 3 drugs)
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10
Q
  • What is resistant hypertension?
  • Inadequate control on … drugs, control on … drugs or more
A
  • Resistant hypertension is high blood pressure that does not respond well to aggressive medical treatment.
  • Inadequate control on 3 drugs, control on 4 drugs or more
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11
Q

What is shown here? (hypertension)

A

High aldosterone exists across the spectrum of hypertension

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12
Q

How to find out if someone has Primary Hyperaldosteronism

  • Initial screening tests:
  1. suppressed …
  2. normal/high …
  • Confirmatory tests
    1. Oral or IV … suppression test
A
  • Initial screening tests:
  1. Suppressed Renin
  2. normal/high aldosterone
  • Confirmatory tests
    1. Oral or IV Na+ suppression test - aldosterone should go down, but if it doesn’t, you have diagnosis
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13
Q

Primary Hyperaldosteronism - Tests for specific Aetiology:

  • Adrenal … scan
  • Adrenal … sampling - is the aldo secretion uni….?
  • Metomidate … CT
A
  • Adrenal CT scan
  • Adrenal venous sampling - is the aldo secretion unilateral?
  • Metomidate PET CT
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14
Q
  • What is adrenal venous sampling?
  • When may it be done?
A
  • In AVS, adrenal veins are cannulated and from each a blood sample is drawn
  • Used to determine cortisol and aldosterone
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15
Q

CT scan - what diagnosis? (A)

A

Conn’s syndrome (primary hyperaldosteronism)

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16
Q

What is this showing? (adrenal ….)

A

Adrenal venous sampling

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17
Q

What kind of scan is this? (for Primary Hyperaldosteronism)

A

11C Metomidate PET scan

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18
Q

Primary Hyperaldosteronism - Treatment

  • Unilateral Adenoma
    • Laparoscopic …
    • Medical Treatment (sometimes)
  • Bilateral Hyperplasia
    • medical treatment - aldosterone … such as
      • …lactone
      • eplerinone
A
  • Unilateral Adenoma
    • Laparoscopic Adrenalectomy
    • Medical Treatment (sometimes)
  • Bilateral Hyperplasia
    • medical treatment - aldosterone antagonists (such as….)
      • spironolactone
      • eplerinone
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19
Q

Primary hyperaldosteronism can be caused by either hyperactivity in one adrenal gland (… disease) or both (… disease).

A

Primary hyperaldosteronism can be caused by either hyperactivity in one adrenal gland (unilateral disease) or both (bilateral disease).

20
Q

What 2 aldosterone antagonists can be used to treat primary hyperaldosteronism? (usually bilateral?)

A
  • spironolactone
    • cheap but may lead to gynecomastia - men may not take
  • eplerinone
21
Q

Medical treatment for men with primary hyperaldosteronism - which drug is better?

  1. Spironolactone
  2. Eplerinone
A
  • Spironolactone is known to cause more gynecomastia or breast pain (up to 10%) in male patients than eplerenone (up to 0.5%)
  • Therefore, eplerenone is better suited
22
Q

What is Phaechromocytoma?

A

Tumour of the adrenal medulla

23
Q

The adrenal medulla is a modified group of … - …. nerve cells innervated by … nerves

A

The adrenal medulla is a modified group of post-ganglionic nerve cells innervated by preganglionic nerves

24
Q

Adrenal medulla - secreting catecholamines

  • …. neurons in spinal cord
  • Adrenal medulla -> Tyrosine
  • Tyrosine converted to L-….
  • This is converted to - Dopamine
  • Dopamine - excreted or converted to
  • this is converted to -> ….
A
  • Sympathetic neurons in spinal cord
  • Adrenal medulla - Tyrosine
  • Tyrosine - L-DOPA
  • L-DOPA - Dopamine
  • Dopamine - excreted or converted to noradrenalin
  • noradrenalin -> adrenalin
25
Q

Products of the Adrenal Medulla

  • …. such as 1) dopamine, 2) … and 3) …
  • How is 2 converted to 3? (high levels of …)
A
  • Catecholamines such as 1) dopamine, 2) Norepinephrine (Noradrenalin) and 3) Epinephrine (Noradrenalin)
  • How is 2 converted to 3? - Cortisol (requires high levels)
26
Q

Are catecholamines essential for life?

A

No - useful for stress/fight/flight

27
Q

Catecholamines - biological effects

  • Adrenalin stimulates what receptors?
  • Vasoconstriction or Vasodilation?
  • What does this lead to in terms of blood pressure and skin?
  • What happens to glucose levels?
A
  • Noradrenalin stimulates alpha 1 and 2 receptors
  • vasoconstriction
  • increased BP and pallor
  • glucose increases due to glycogenolysis
28
Q

Catecholamines - biological effects

  • Noradrenalin stimulates what receptors?
  • Vasoconstriction or Vasodilation? (BV and muscle)
  • What does this lead to in terms of HR and skin?
A
  • Adrenalin stimulates alpha 1, beta 1 and beta 2 receptors
  • Vasoconstriction, vasodilation in muscle
  • Increased HR, sweating
29
Q

Pathology of Phaeochromocytoma

  • …% of phaeos are diagnosed in the mortuary
A

20% of phaeos are diagnosed in the mortuary

30
Q

Presentation of Phaeochromocytoma

  • “spells” such as … (5)
  • Hypertension, which may be … or ….
  • … Hx present
A
  • “spells”
    • headache, sweat
    • pallor, palpitation
    • anxiety
  • Hypertension
    • permanent
    • intermittent
  • family Hx
31
Q

Several A-D Genetic conditions are associated with Phaechromocytoma

  • … Type 1 (NF1)
  • Multiple Endocrine … Type 2 (MEN 2)
  • Von hippel - … Syndrome
A
  • Neurofibromatosis Type 1 (NF1)
  • Multiple Endocrine Neoplasia Type 2 (MEN 2)
  • Von hippel - Lindau Syndrome
32
Q
  • What genetic condition is this? (Associated with phaeochromocytoma)
A
  • Neurofibromatosis Type 1 (NF1)
33
Q

The picture below shows Axillary freckling - what condition is this present in? (associated with phaeochromocytoma)

A

Neurofibromatosis Type 1 (NF1)

34
Q

What do people in a MEN2a family develop? (3 things)

A
  • Medullary carcinoma of the thyroid
  • Develop bilateral pheochromocytoma
  • Also may develop primary hyperparathyrodism
35
Q

To prevent children from developing a medullary carcinoma in a MEN2a family, what would you do?

A
  • Total thyroidectomy at age 5/6/7 to prevent development
  • Screen for other complications
36
Q
  • What is this image showing? (can get it in Von Hippel-Lindau syndrome)
  • What is it often associated with?
A
  • Retinal Hemangioglioblastoma
  • often associated with a cerebellar haemangioglioblastoma (shown below)
37
Q
A
38
Q

Phaeochromocytoma - Biochemical Diagnosis

  • 24 hour … test
    • measure the … and …
    • measure … to see if too much dopamine being made
  • Plasma (if issue with other method)
    • measure … and …
    • also measure the … as above
A
  • 24 hour urine test
    • measure the normetanephrines and metanephrines
    • measure 3 methoxytyromine to see if too much dopamine being made
  • Plasma (if issue with other method)
    • noradrenalin and adrenalin
    • metanephrines as above
39
Q

What else elevates the measured catecholamines?

  • Obstructive …. ….
  • … like drugs
  • L-…
  • Labetalol (hypertensive drug with some beta blocker activity)
A
  • Obstructive sleep apnoea
  • Amphetamine like drugs
  • L-DOPA
  • Labetalol
40
Q

Why do we not measure dopamine in 24 hour urine?

  • Urine dopamine does not come from the adrenal medulla, it comes from … and …. so we measure what instead?
A

Urine dopamine does not come from the adrenal medulla, it comes from kidney and nervous system so we measure methoxytyramine instead

41
Q

Pathology of adrenal medulla - what is shown?

A

Phaeochromocytoma - larger than adenomas in Conn’s syndrome (average 4cm)

42
Q

To be sure of a phaeochromocytoma. we do a … scan

A

To be sure of a phaeochromocytoma. we do a MIBG scan

43
Q

Other imaging for phaeochromocytoma

  • …. … scan to show extra-adrenal “phaeos” or metastases
A
  • PET CT scan
44
Q

Management of phaeochromocytoma

before surgical procedure (laparoscopic … ) give…

  • … blockers - prevent hypertension
    • phenocybenzamine
    • doxazocin
  • … blockers afterwards
    • propranolol
  • then surgery once blockade is satisfactory
A
  • surgery = laparoscopic adrenalectomy
  • alpha blockers
    • phenocybenzamine
    • doxazocin
  • beta blockers
    • propranolol
  • laparoscopic adrenalectomy
45
Q

Post-adrenalectomy care

  • Consider … testing as …% are …
  • annual … tests (via 24 hour urine and plasma)
  • additional treatment if it is … (10% are)
A
  • Consider genetic testing as 30% are genetic (13 mutations so far)
  • annual metanephrines (via 24 hour urine and plasma)
  • additional treatment if it is malignant (10% are)