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1. Internal Medicine I > 15. Secondary endocrine HT (primary aldosteronism, pheocromocytoma) > Flashcards

15. Secondary endocrine HT (primary aldosteronism, pheocromocytoma) Flashcards

1
Q

What are some causes of secondary hypertension?

A
  • Non-endocrine origin (renal, cardiac)
  • endocrine origin such as acromegaly, Cushing syndrome, thyroid, congenital adrenal hyperplasia, primary hyperaldosteronism, and phaeochromocytoma.
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2
Q

When should we suspect secondary hypertension and who should be examined?

A

We should suspect secondary hypertension in patients with
- hypertension in youth
- malignant hypertension (180/120 Hgmm),
- sudden onset with high blood pressure value hypertension,
- patients with hypertension and adrenal tumor,
- patients with hypertension and OSAS,
- and therapy-refractory hypertension (insufficient blood pressure with >3 antihypertensive medications).

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3
Q

What are some screening examinations for secondary hypertension?

A
  • Family history, inherited diseases
  • physical examination (blood pressure in 4 limbs, “typical” signs),
  • lab results (routine lab: creatinine, BUN, GFR, Sodium).
  • radiology (tumors, arteries)
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4
Q

What is primary hyperaldosteronism?

A

Independant adrenal overexpression of aldosterone with suppressed renin value, hypertension and hypokalemia

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5
Q

What is the classification of primary hyperaldosteronism?

A
  • > 50% aldosterone-producing adenoma,
  • 35-50% bilateral adrenal hyperplasia,
  • < 1 % familial hyperaldosteronism,
  • < 1% aldosterone-producing adrenal cancer.
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6
Q

What is the epidemiology of primary hyperaldosteronism?

A
  • most frequent secondary hypertension cause: 5-10%,
  • highest incidence between 30-50 Y.
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7
Q

What is the most frequent endocrine cause of secondary hypertension?

A

Primary hyperaldosteronism (Conn-syndrome).

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8
Q

What are the clinical signs of primary hyperaldosteronism?

A
  • Hypertension - moderate/severe, hard to adjust, headache
  • hypokalemic (50%) : spontaneous, diuretics induced, fatigue, weakness, muscle cramp, arrhythmia.
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9
Q

What are the complications of hypertension in primary hyperaldosteronism?

A

Stroke, cardiac failure, MI, atrial fibrillation, kidney failure (proteinuria), metabolic syndrome/(Pre)diabetes mellitus.

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10
Q

What is the primary goal in the diagnosis of primary hyperaldosteronism?

A

To differentiate between essential hypertension and primary hyperaldosteronism.

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11
Q

What is the next step in finding the cause of primary hyperaldosteronism?

A

Adrenal adenoma vs. bilateral hyperplasia.

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12
Q

What is the recommendation for a morning sample used in screening for primary hyperaldosteronism?

A

At least 20-30 minutes in rest (sitting, lying), fasting

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13
Q

What is the essential condition for accurate screening of primary hyperaldosteronism?

A

Normokalemia.

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14
Q

Which medications should be temporarily left out during screening for primary hyperaldosteronism?

A

ACE-blockers, ARB, other diuretics, dihydropyridine calcium-channel blockers, and estrogens.

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15
Q

What is the mechanism of action of spironolactone and eplerenone?

A

They block the action of aldosterone on its receptors.

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16
Q

Which medication can cause a false-positive result in the calculation of the ARR ratio?

A

Beta-blockers, NSAIDS

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17
Q

Which medication can cause a false-negative result in the calculation of the ARR ratio?

A

ACE-blockers and ARB.

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18
Q

What is the recommended duration of medication withdrawal before calculating the ARR ratio?

A

At least 6 weeks.

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19
Q

What is the recommended ratio used in screening for primary hyperaldosteronism?

A

Ratio of plasma aldosterone concentration and plasma renin activity (ARR).

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20
Q

What is the recommended antihypertensive medication for primary hyperaldosteronism (Conn-syndrome)?

A

Doxazosin, Prazosin, Verapamil, Hidralazin.

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21
Q

What is the screening criteria for primary hyperaldosteronism (Conn-syndrome)?

A

Screening is positive if ARR > 30 and the plasma aldosterone concentration > 15 ng/dl (416 pmol/L) and the PRA is suppressed (< 0.2 ng/ml/H) in most cases.

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22
Q

What is the confirmatory testing for primary hyperaldosteronism (Conn-syndrome)?

A
  • Intravenous sodium loading test,
  • Oral sodium loading test
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23
Q

What is the purpose of the Intravenous sodium loading test?

A

To evaluate primary hyperaldosteronism (Conn-syndrome) by measuring plasma aldosterone levels after intravenous infusion of sodium chloride.

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24
Q

What is the cutoff value for plasma aldosterone concentration after the Intravenous sodium loading test to confirm the diagnosis of primary hyperaldosteronism?

A

Plasma aldosterone concentration > 5 ng/dl.

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25
Q

What is the significance of plasma aldosterone concentration between 5-10 ng/dl after the Intravenous sodium loading test?

A

It might refer to bilateral hyperplasia.

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26
Q

What is the significance of plasma aldosterone concentration > 10 ng/dl after the Intravenous sodium loading test?

A

It might refer to aldosterone-producing adrenal adenoma.

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27
Q

What is the first choice for localizing the subtype of primary hyperaldosteronism?

A

Adrenal CT (MR).

28
Q

What is the gold-standard for confirming the subtype of primary hyperaldosteronism?

A

Adrenal vein sampling.

29
Q

What is the sensitivity and specificity of adrenal vein sampling?

A

Sensitivity: 95%, specificity: 100%.

30
Q

How is the correct catheter localization confirmed during adrenal vein sampling?

A

Adrenal vein cortisol concentration is 2-3x higher vs. cortisol concentration in vena cava.

31
Q

What does a 3-4x higher aldosterone/cortisol ratio on one side during adrenal vein sampling suggest?

A

Aldosterone-producing adenoma on that side.

32
Q

What does less or no difference in ratio between the two sides during adrenal vein sampling suggest?

A

Bilateral adrenal hyperplasia.

33
Q

What is the difference between one-sided aldosterone-producing adenoma and bilateral hyperplasia in primary hyperaldosteronism?

A

One-sided adenoma has a higher aldosterone/cortisol ratio on one side compared to the other, while bilateral hyperplasia has less or no difference in ratio between the sides.

34
Q

What is the treatment for one-sided aldosterone-producing adenoma in primary hyperaldosteronism?

A

Surgical resection, specifically adrenalectomy.

35
Q

What is the postoperative effect of adrenalectomy in one-sided aldosterone-producing adenoma?

A

Temporarily “hypoaldosteronism” and hyperkalemia.

36
Q

What is the treatment for bilateral hyperplasia or aldosterone-producing adenoma in primary hyperaldosteronism if no operation is done?

A

Mineralocorticoid-receptor antagonist therapy, specifically spironolactone or eplerenone.

37
Q

What are the side effects of spironolactone in primary hyperaldosteronism treatment?

A

Progesterone/androgen-receptor effect, leading to dysmenorrhea, breast tension, gynecomastia, and impotence.

38
Q

What is phaeochromocytoma?

A

It is a catecholamine-producing neuroendocrine adrenal tumor.

39
Q

What are the forms of phaeochromocytoma?

A

Extraadrenal-paraganglioma, pediatric cases, one-sided/bilateral adrenal, and benign/malignant.

40
Q

What is the genetic involvement in phaeochromocytoma?

A

It is involved in 50% of cases, with 35% being germline and 15% being somatic.

41
Q

What is the prevalence of phaeochromocytoma?

A

0.3-0.8/100 000.

42
Q

What is the mechanism of action of PNMT?

A

PNMT stands for phenyletanolamine-N-methyltransferase, which is an enzyme that converts norepinephrine to epinephrine.

43
Q

What are the three types of catecholamines produced by phaeochromocytoma?

A

Epinephrine, norepinephrine, and dopamine.

44
Q

What are the two pathways involved in phaeochromocytoma pathogenesis?

A

The VHL and SDH pathway, and the Ret and NF1 pathway.

45
Q

What is the prevalence of pheochromocytoma in the hypertension population?

A

0.1-0.6%.

46
Q

What are the signs of pheochromocytoma?

A
  • Headache,
  • palpitation,
  • perspiration, pallor,
  • hypertension / paroxysmal hypertension,
  • fatigue,
  • weight loss,
  • panic attack, anxiety,
  • orthostatic hypotension.
47
Q

What are the life-threatening situations associated with pheochromocytoma?

A
  • Angina pectoris, malignant arrhythmia, cardiac failure, aorta dissection,
  • high blood glucose,
  • catecholamine crisis,
  • mechanic irritation of the tumor,
  • and thyramine and beta-blockers.
48
Q

When should we rule out pheochromocytoma?

A

Unusual presence of symptoms such as headache, palpitations, sweating, and weight loss, hypertension that is difficult to control with medication, hypertension that develops at a young age or in a patient with a family history of pheochromocytoma, and hypertension that is associated with other endocrine disorders such as hyperthyroidism or hyperparathyroidism.

49
Q

What is the recommended approach for screening hypertension in all hypertensive patients?

A

Rare case of hypertension, screening is not recommended in all hypertensive patients.

50
Q

What are the unusual cases where hypertension screening is recommended?

A

Unusual presence of hypertension, hypertension in youth or in young adults, therapy-resistant hypertension, blood-pressure lability, paroxysmal hypertension, adrenal incidentalom + hypertension, unknown cause of dilatative cardiomyopathy, unknown cause of shock, paradox blood-pressure elevation after beta-blocker initiation.

51
Q

What are the confirmatory tests for catecholamine overproduction in phaeochromocytoma?

A

(nor)metanephrine excretion (plasma/24H urine) in low pH, in dark bowl, positive if 3x > ULN, vanilin mandelic acid, 3-metoxithyramine (malignant?), chromogranine A.

52
Q

What is the common neuroendocrine tumor marker for phaeochromocytoma?

A

Chromogranine A, serum level is proportional with tumor size.

53
Q

What are the imaging tests used for diagnosis and localization of phaeochromocytoma?

A

Adrenal CT/MR scans, 20% are extra-adrenal, full-body scan is needed, hereditary phaeochromocytoma can be multifocal, 131I-metaiodine-benzil-guanidine (131I-MIBG) scintigraphy, sensitivity.

54
Q

What is the recommended imaging technique for detecting hereditary phaeochromocytoma?

A

18F-fluorodopamine/18fluoroDOPA -PET/CT.

55
Q

What is the sensitivity of 131I-metaiodine-benzil-guanidine (131I-MIBG) scintigraphy in detecting phaeochromocytoma?

A

95-100%.

56
Q

What is the differential diagnosis for phaeochromocytoma?

A

Thyrotoxicosis, carcinoid syndrome, medullary thyroid cancer (high calcitonin), perimenopausal syndrome, migraine, epilepsy, panic attack, anxiety disorders, drugs, mastocytosis, porphyria.

57
Q

What is the recommended preoperative treatment for phaeochromocytoma?

A

Alpha-blocker, such as doxazosine, urapidil, or prazosine, for at least two weeks.

58
Q

What is the first step during the operation for phaeochromocytoma?

A

Adrenal vein ligature, to avoid mechanical irritation.

59
Q

What is the first step during an operation to remove a pheochromocytoma?

A

Adrenal vein ligature, avoiding mechanical irritation.

60
Q

What is the typical postoperative blood pressure change in patients who have had a pheochromocytoma removed?

A

Temporarily hypotension is usual, but in many cases, long-term hypertension can occur.

61
Q

What is the first treatment for pheochromocytoma?

A

Removal of the tumor.

62
Q

What is the incidence of malignant pheochromocytoma?

A

In 5-26% of cases.

63
Q

What is the recommended follow-up for all patients with pheochromocytoma?

A

Lifelong follow-up is recommended.

64
Q

What are the common sites of metastasis for malignant pheochromocytoma?

A

Liver, bone, lung, and skull.

65
Q

What is the role of SHDB gene mutations in the development of malignant pheochromocytoma?

A

They increase the risk of developing malignant pheochromocytoma.

66
Q

Can histology provide a diagnosis of malignant pheochromocytoma?

A

No, it can only confirm the presence of distant metastasis.

67
Q

What are the treatment options for malignant pheochromocytoma?

A

Operation, alpha-methyl-parathyrosine treatment, and 131I-MIBG treatment.

1. Internal Medicine I (20 decks)

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