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Flashcards in Adrenal Deck (131)
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1

Deficiency of 21-hydroxylase: Laboratory finding.

Elevated 17-hydroxyprogesterone.

2

Congenital lipoid adrenal hyperplasia: Definition.

Severest form of congenital adrenal hyperplasia: All gonadal and adrenal cortical steroids are markedly underproduced.

3

Congenital lipoid adrenal hyperplasia: Genes (2).

StAR (steroidogenic acute regulatory) protein.

P-450scc.

4

Classic presentation of congenital adrenal hyperplasia in females:

A. Newborn.
B. Older (3).

A. Virilization.

B. Postpubertal: Oligomenorrhea, hirsutism, acne.

5

Classic presentation of congenital adrenal hyperplasia in males:

A. Newborn.
B. Older (3).

A. Salt-losing crisis.

B. Prepubertal: Precocious puberty.

6

Non-classic presentation of congenital adrenal hyperplasia (3).

Newborns: Normal.

Around puberty: Virilization.

Pregnancy: May present with adrenal insufficiency.

7

Congenital adrenal hyperplasia: Histopathology (3).

All three cortical layers are thickened, but especially the zona reticularis.

Loss of zonation.

Lipid-poor cells.

8

Congenital lipoid adrenal hyperplasia: Histopathology.

Cholesterol-overloaded cells with rupture and cholesterol esters.

9

Congenital adrenal hyperplasia: Complication.

Adrenal cortical adenoma or carcinoma.

10

Congenital adrenal hyperplasia: Effect on adrenal medulla.

Glucocorticoid deficiency can impair development of the medulla, resulting in epinephrine deficiency and hypoglycemia.

11

Autoimmune adrenal insufficiency:

A. Frequency.
B. Antibodies (3).

A. Accounts for 75-90% of cases of primary adrenal insufficiency.

B. 21-hydroxylase, 17-hydroxylase, P-450scc.

12

Primary vs. secondary adrenal insufficiency: Biochemical differences (3).

Primary: Low ACTH, low aldosterone, high renin.

Secondary: High ACTH, normal aldosterone, normal renin.

13

Primary vs. secondary adrenal insufficiency: Gross-pathology difference.

Primary: Small, pale adrenal gland.

Secondary: Enlarged gland.

14

Adrenal insufficiency: How much adrenal tissue is essential to normal function?

About 10%.

15

Autoimmune polyglandular syndromes.

APS-1: Includes candidiasis and alopecia; mutation in AIRE-1.

APS-2: Schmidt's syndrome.

15

Primary adrenal cortical hyperplasia: Types (3).

ACTH-independent macronodular hyperplasia.

Primary pigmented nodular adrenal cortical disease.

Other.

16

ACTH-independent macronodular hyperplasia: Syndrome that can cause it, and its gene.

McCune-Albright syndrome, GNAS1.

17

Primary pigmented nodular adrenal cortical disease: Syndromes (2).

Carney's complex.

Isolated primary pigmented nodular adrenal cortical disease.

18

Primary pigmented nodular adrenal cortical disease: Genes (2).

PRKAR1A.

PDE11A.

19

Other syndromes (2) that can cause bilateral adrenal hyperplasia.

MEN-1.

FAP.

20

Secondary adrenal cortical hyperplasia: Causes (2).

Pituitary adenoma or hyperplasia.

Ectopic ACTH.

21

Adrenal cortical hyperplasia: Presentation.

Primary: Various endocrine abnormalities.

Secondary due to pituitary: Severe, typical Cushing's syndrome.

Secondary due to ectopic ACTH: Severe, atypical Cushing's syndrome.



22

Adrenal cortical hyperplasia: Pharmacological therapy.

Ketoconazole and similar drugs.

23

Adrenal cortical hyperplasia: Degree of enlargement (3).

Severe: Ectopic ACTH, AIMAH.

Mild or moderate: Pituitary disease, PPNAD.

Grossly inapparent: Conn's syndrome due to hyperplasia of zona glomerulosa.

Enlargement may be nodular or diffuse.

24

Hyperplasia of zona glomerulosa: Histopathology (3).

Involvement limited to the periphery of the gland.

Abnormal continuity of the zona.

More than 5 nests thick.

25

ACTH-independent macronodular adrenal cortical hyperplasia: Histopathology (2).

Mixture of large clear cells and small compact cells.

Nodules of cells containing dark brown pigment.

26

Primary pigmented nodular adrenal cortical disease: Histopathology.

Cortical tissue between nodules is atrophic and disorganized.

27

Adrenal cortical hyperplasia: Immunohistochemistry.

AIMAH: 3β-hydroxysteroid dehydrogenase.

PPNAD: Synaptophysin, 17α-hydroxylase.

28

Clinical findings that favor adrenal adenoma over adrenal hyperplasia (2).

Solitary, unilateral nodule.

Evidence of autonomous growth.

29

Adrenal cortical adenoma: Familial syndromes (3).

MEN-1.

Familial hyperaldosteronism.

Congenital adrenal hyperplasia.