ENDOCRINE DISORDERS-THYROID DISORDERS 1.3 (AB)

Question 1

What is pheochromocytoma?

Answer 1

A chromaffin tumor that secretes catecholamines, usually located in the adrenal medulla.

Question 2

What is the ‘Rule of 10’ in pheochromocytoma?

Answer 2

10% are bilateral, 10% are extra-adrenal, 10% are malignant, 10% occur in children, 10% are familial.

Question 3

What is the incidence of pheochromocytoma in pregnancy?

Answer 3

Approximately 1 per 50,000 pregnancies.

Question 4

What percentage of hypertensive patients have pheochromocytoma?

Answer 4

0.2 to 0.6%.

Question 5

What are the common symptoms of pheochromocytoma?

Answer 5

Paroxysmal hypertensive crisis, seizures, anxiety attacks, headache, sweating, palpitations, chest pain, nausea, vomiting, pallor.

Question 6

What is the standard screening test for pheochromocytoma?

Answer 6

Quantification of metanephrines and catecholamine metabolites in a 24-hour urine specimen.

Question 7

What is the most sensitive diagnostic test for pheochromocytoma?

Answer 7

Determination of plasma catecholamine metabolite levels.

Question 8

What are the normal plasma and urine metanephrine levels?

Answer 8

Plasma free metanephrine: <0.3 - 0.5 nmol/L; Urine total metanephrine: <1.3 - 2.4 mg/24 hours.

Question 9

What imaging modalities are used for pheochromocytoma diagnosis?

Answer 9

CT or MRI for adrenal localization; MRI is preferred in pregnancy.

Question 10

Why is it important to differentiate pheochromocytoma from preeclampsia?

Answer 10

Both can cause hypertension in pregnancy, but pheochromocytoma can lead to life-threatening complications if not diagnosed.

Question 11

What is the first-line treatment for pheochromocytoma?

Answer 11

Immediate control of hypertension with an ⍺-adrenergic blocking agent (e.g., phenoxybenzamine).

Question 12

What medication is added after α-blockade in pheochromocytoma?

Answer 12

β-blocking drugs such as propranolol for tachycardia.

Question 13

When is surgical removal of pheochromocytoma performed in pregnancy?

Answer 13

Preferably in the second trimester.

Question 14

What are the management options for pheochromocytoma diagnosed later in pregnancy?

Answer 14

Planned cesarean delivery with tumor excision or postpartum resection.

Question 15

What is Cushing syndrome?

Answer 15

A condition caused by excessive glucocorticoids due to a pituitary adenoma, adrenal tumor, or bilateral adrenal hyperplasia.

Question 16

What is the female-to-male ratio in Cushing syndrome?

Answer 16

3:1.

Question 17

What is the most common cause of iatrogenic Cushing syndrome?

Answer 17

Long-term corticosteroid treatment.

Question 18

What are the characteristic physical features of Cushing syndrome?

Answer 18

Moon facies, buffalo hump, truncal obesity due to adipose tissue deposition.

Question 19

How does Cushing syndrome affect fertility?

Answer 19

CRH can interfere with GnRH in the HPO axis, leading to anovulation.

Question 20

What is Cushing disease?

Answer 20

Bilateral adrenal hyperplasia caused by corticotropin-producing pituitary adenomas.

Question 21

What tumors can cause ectopic Cushing syndrome?

Answer 21

Non-endocrine tumors producing corticotropin-releasing factor or corticotropin.

Question 22

What percentage of Cushing syndrome cases are corticotropin-independent?

Answer 22

Less than 25%, mostly caused by adrenal adenomas.

Question 23

What is the diagnostic test for Cushing syndrome?

Answer 23

Elevated plasma cortisol levels that cannot be suppressed by dexamethasone or elevated 24-hour urinary free cortisol excretion.

Question 24

What imaging modalities are used to localize tumors in Cushing syndrome?

Answer 24

Serum corticotropin levels, CT, and MRI.

Question 25

Why is Cushing syndrome diagnosis challenging in pregnancy?

Answer 25

Normal cortisol levels are elevated in pregnancy, making interpretation difficult.

Question 26

What are the causes of Cushing syndrome in pregnancy?

Answer 26

>50% corticotropin-dependent adrenal adenomas, ~30% pituitary adenomas, 10% adrenal carcinomas.

Question 27

What are the major complications of hypercortisolism in pregnancy?

Answer 27

Heart failure, poor wound healing, osteoporotic fractures, psychiatric complications.

Question 28

What is the most common pharmacologic treatment for Cushing syndrome before surgery?

Answer 28

Metyrapone (adrenal steroid synthesis inhibitor).

Question 29

Why is ketoconazole concerning for male fetuses?

Answer 29

It blocks testicular steroidogenesis.

Question 30

Why is mifepristone contraindicated in pregnancy?

Answer 30

It blocks cortisol action and is also used for abortion and labor induction.

Question 31

What is the first-line surgical treatment for Cushing syndrome?

Answer 31

Transsphenoidal resection of pituitary tumors or laparoscopic adrenalectomy in the second trimester.

Question 32

When can unilateral adrenalectomy be performed in pregnancy?

Answer 32

Early third trimester.

Question 33

What is Addison disease?

Answer 33

Primary adrenal insufficiency due to adrenal gland destruction.

Question 34

What is the most common cause of Addison disease in developed countries?

Answer 34

Autoimmune adrenalitis.

Question 35

What is the most common cause of Addison disease in resource-poor countries?

Answer 35

Tuberculosis.

Question 36

What effect does untreated adrenal insufficiency have on fertility?

Answer 36

It frequently causes infertility, but ovulation is restored with replacement therapy.

Question 37

What are the classic symptoms of Addison disease?

Answer 37

Weakness, nausea, vomiting, weight loss.

Question 38

What are the key diagnostic findings in Addison disease?

Answer 38

Low plasma cortisol, elevated ACTH levels.

Question 39

What pregnancy complications are associated with adrenal insufficiency?

Answer 39

Preterm delivery, low birth weight, cesarean delivery.

Question 40

What is the treatment for Addison disease?

Answer 40

Glucocorticoid and mineralocorticoid replacement.

Question 41

How is glucocorticoid dosing adjusted in pregnancy for Addison disease?

Answer 41

A 20-40% dose increase after midpregnancy to mimic physiological cortisol elevation.

Question 42

What is the stress dose of corticosteroids for labor or surgery in Addison disease?

Answer 42

Hydrocortisone 100 mg IV every 8 hours for 48 hours.

Question 43

What is the most common cause of primary aldosteronism (Conn syndrome)?

Answer 43

Adrenal adenoma (75% of cases)

Question 44

What are the three main causes of primary aldosteronism?

Answer 44

Adrenal adenoma, idiopathic bilateral adrenal hyperplasia, rare cases of adrenal carcinoma

Question 45

What are the hallmark symptoms of primary aldosteronism?

Answer 45

Hypertension, hypokalemia, muscle weakness

Question 46

How is primary aldosteronism diagnosed?

Answer 46

Confirmed by high serum or urine aldosterone levels

Question 47

Why is diagnosing hyperaldosteronism in pregnancy difficult?

Answer 47

Progesterone blocks aldosterone action, leading to very high aldosterone levels

Question 48

What laboratory test is useful for diagnosing hyperaldosteronism?

Answer 48

Plasma aldosterone-to-renin activity ratio

Question 49

What happens to hypertension in hyperaldosteronism as pregnancy progresses?

Answer 49

It worsens

Question 50

What are the medical management options for hyperaldosteronism?

Answer 50

Potassium supplementation, antihypertensive therapy (spironolactone, beta-blockers, calcium channel blockers), amiloride, eplerenone

Question 51

What is the definitive treatment for primary aldosteronism?

Answer 51

Laparoscopic tumor resection

Question 52

What pregnancy complications are associated with hyperaldosteronism?

Answer 52

Higher prevalence of chronic hypertension, preeclampsia, preterm birth, intrauterine growth restriction (IUGR)

Question 53

What is the most common type of pituitary adenoma in women?

Answer 53

Prolactinoma (75%)

Question 54

What are the key symptoms of prolactinoma?

Answer 54

Amenorrhea, hyperprolactinemia, galactorrhea

Question 55

How are prolactinomas classified based on size?

Answer 55

Microadenomas (<10mm) and macroadenomas (>10mm)

Question 56

What imaging modality is recommended for prolactinoma diagnosis?

Answer 56

CT or MRI, but only if symptoms develop

Question 57

What is the first-line treatment for microadenomas?

Answer 57

Bromocriptine (dopamine agonist)

Question 58

What is the recommended treatment for suprasellar macroadenomas before pregnancy?

Answer 58

Surgical resection

Question 59

How are macroprolactinomas monitored during pregnancy?

Answer 59

Frequent monitoring with visual field testing each trimester

Question 60

What are the two main dopamine agonists used to treat prolactinomas?

Answer 60

Bromocriptine and cabergoline

Question 61

What is the first-line treatment for acromegaly outside pregnancy?

Answer 61

Transsphenoidal resection of the pituitary adenoma

Question 62

What laboratory finding confirms acromegaly?

Answer 62

Elevated insulin-like growth factor 1 (IGF-1) serum levels

Question 63

Why is pregnancy rare in women with acromegaly?

Answer 63

50% have hyperprolactinemia and anovulation

Question 64

What pregnancy complications are associated with acromegaly?

Answer 64

Gestational diabetes, hypertension

Question 65

What is the first-line treatment for acromegaly during pregnancy?

Answer 65

Monitoring for tumor enlargement; dopamine agonists are less effective

Question 66

What is the cause of diabetes insipidus (DI)?

Answer 66

Vasopressin (ADH) deficiency due to neurohypophysis agenesis or destruction

Question 67

What are the types of DI encountered during pregnancy?

Answer 67

Primary polydipsia, gestational DI, nephrogenic DI, transient secondary DI

Question 68

What is the treatment for DI in pregnancy?

Answer 68

Desmopressin (DDAVP)

Question 69

Why do pregnant women with DI require higher doses of desmopressin?

Answer 69

Increased metabolic clearance rate due to placental vasopressinase

Question 70

What causes Sheehan syndrome?

Answer 70

Pituitary ischemia and necrosis due to obstetrical blood loss

Question 71

What are the key symptoms of Sheehan syndrome?

Answer 71

Persistent hypotension, tachycardia, hypoglycemia, lactation failure

Question 72

What is the treatment for Sheehan syndrome?

Answer 72

Glucocorticoid replacement therapy, thyroid, gonadal, and growth hormone replacement

Question 73

What is lymphocytic hypophysitis?

Answer 73

A rare autoimmune disorder causing pituitary inflammation and destruction

Question 74

What are the common symptoms of lymphocytic hypophysitis?

Answer 74

Headaches, visual field defects, sellar mass, elevated serum prolactin

Question 75

What autoimmune diseases are associated with lymphocytic hypophysitis?

Answer 75

Hashimoto thyroiditis, Addison disease, type 1 diabetes, pernicious anemia

Question 76

What is the treatment for lymphocytic hypophysitis?

Answer 76

Glucocorticoids, pituitary hormone replacement therapy