Flashcards in Adrenal Deck (131)
Deficiency of 21-hydroxylase: Laboratory finding.
Congenital lipoid adrenal hyperplasia: Definition.
Severest form of congenital adrenal hyperplasia: All gonadal and adrenal cortical steroids are markedly underproduced.
Congenital lipoid adrenal hyperplasia: Genes (2).
StAR (steroidogenic acute regulatory) protein.
Classic presentation of congenital adrenal hyperplasia in females:
B. Older (3).
B. Postpubertal: Oligomenorrhea, hirsutism, acne.
Classic presentation of congenital adrenal hyperplasia in males:
B. Older (3).
A. Salt-losing crisis.
B. Prepubertal: Precocious puberty.
Non-classic presentation of congenital adrenal hyperplasia (3).
Around puberty: Virilization.
Pregnancy: May present with adrenal insufficiency.
Congenital adrenal hyperplasia: Histopathology (3).
All three cortical layers are thickened, but especially the zona reticularis.
Loss of zonation.
Congenital lipoid adrenal hyperplasia: Histopathology.
Cholesterol-overloaded cells with rupture and cholesterol esters.
Congenital adrenal hyperplasia: Complication.
Adrenal cortical adenoma or carcinoma.
Congenital adrenal hyperplasia: Effect on adrenal medulla.
Glucocorticoid deficiency can impair development of the medulla, resulting in epinephrine deficiency and hypoglycemia.
Autoimmune adrenal insufficiency:
B. Antibodies (3).
A. Accounts for 75-90% of cases of primary adrenal insufficiency.
B. 21-hydroxylase, 17-hydroxylase, P-450scc.
Primary vs. secondary adrenal insufficiency: Biochemical differences (3).
Primary: Low ACTH, low aldosterone, high renin.
Secondary: High ACTH, normal aldosterone, normal renin.
Primary vs. secondary adrenal insufficiency: Gross-pathology difference.
Primary: Small, pale adrenal gland.
Secondary: Enlarged gland.
Adrenal insufficiency: How much adrenal tissue is essential to normal function?
Autoimmune polyglandular syndromes.
APS-1: Includes candidiasis and alopecia; mutation in AIRE-1.
APS-2: Schmidt's syndrome.
Primary adrenal cortical hyperplasia: Types (3).
ACTH-independent macronodular hyperplasia.
Primary pigmented nodular adrenal cortical disease.
ACTH-independent macronodular hyperplasia: Syndrome that can cause it, and its gene.
McCune-Albright syndrome, GNAS1.
Primary pigmented nodular adrenal cortical disease: Syndromes (2).
Isolated primary pigmented nodular adrenal cortical disease.
Primary pigmented nodular adrenal cortical disease: Genes (2).
Other syndromes (2) that can cause bilateral adrenal hyperplasia.
Secondary adrenal cortical hyperplasia: Causes (2).
Pituitary adenoma or hyperplasia.
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.
Adrenal cortical hyperplasia: Pharmacological therapy.
Ketoconazole and similar drugs.
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.
Hyperplasia of zona glomerulosa: Histopathology (3).
Involvement limited to the periphery of the gland.
Abnormal continuity of the zona.
More than 5 nests thick.
ACTH-independent macronodular adrenal cortical hyperplasia: Histopathology (2).
Mixture of large clear cells and small compact cells.
Nodules of cells containing dark brown pigment.
Primary pigmented nodular adrenal cortical disease: Histopathology.
Cortical tissue between nodules is atrophic and disorganized.
Adrenal cortical hyperplasia: Immunohistochemistry.
AIMAH: 3β-hydroxysteroid dehydrogenase.
PPNAD: Synaptophysin, 17α-hydroxylase.
Clinical findings that favor adrenal adenoma over adrenal hyperplasia (2).
Solitary, unilateral nodule.
Evidence of autonomous growth.