Epidemiologia

Question 1

the most common cause of primary adrenal insufficiency

Answer 1

Tuberculosis was the most common cause of primary adrenal insuffi ciency during the fi rst half of the 20th century,17 but lately autoimmune adrenal insufficiency has become the most common form. autoimmune form was diagnosed in 82% of cases, the tuberculosis-related form in 9%, and other causes in about 8% of cases

Question 2

Primary adrenal insufficiency occurs more frequently in

Answer 2

women than in men, and can present at any age, although most often appears between the ages of 30 and 50 years

Question 3

Secondary adrenal insufficiency

Answer 3

is more common than primary adrenal insufficiency.1 It has an estimated prevalence of 150–280 per million

Question 4

Secondary adrenal insufficiency affects

Answer 4

women more frequently than men.The age at diagnosis peaks in the sixth decade of life

Question 5

The most common cause of tertiary adrenal insufficiency is

Answer 5

long-term administration of exogenous glucocorticoids, which leads to prolonged suppression of hypothalamic secretion of corticotropin-releasing hormone

Question 6

In developed countries, 80–90% of cases of primary adrenal insuffi ciency are caused by autoimmune adrenalitis, which can be

Answer 6

isolated (40%) or part of an autoimmune polyendocrinopathy syndrome (60%)

Question 7

chronic primary adrenal

Answer 7

93–144 cases per million

Question 8

Causes of primary SAI

Answer 8

Autoimmune adrenalitis
Infectious adrenalitis
Bilateral adrenal haemorrhage
Bilateral adrenal metastases
Bilateral adrenal infiltration
Bilateral adrenalectomy
Drug-induced adrenal insufficiency
Genetic disorders

Question 9

Autoimmune adrenalitis

Answer 9

Isolated
APS type 1 (APECED)
APS type 2
APS type 4

Question 10

genetic causes primary AI

Answer 10

Of the genetic causes, adrenoleukodystrophy, is an X-linked recessive disorder that aff ects one in 20 000 men and boys and is caused by mutations in the ATP-binding cassette, subfamily D, member 1 (ABCD1) gene. These mutations prevent normal transport of very-long-chain fatty acids into peroxisomes, thereby preventing their β-oxidation and breakdown. Accumulation of abnormal amounts of these fatty acids in aff ected organs (CNS, Leydig cells of the testes, adrenal cortex) is thought to be the underlying pathological process

Question 11

Primary adrenal insufficiency occasionally presents acutely as a consequence of bilateral adrenal haemorrhage in patients with

Answer 11

antiphospholipid syndrome.

Question 12

In children, the most common cause of primary adrenal insufficiency is

Answer 12

congenital adrenal hyperplasia, a group of autosomal recessive disorders resulting from defi ciency of one of the enzymes needed for synthesis of cortisol in the adrenal cortex. The most common form is classic 21-hydroxylase deficiency, a condition characterised by low synthesis of glucocorticoids and, in many cases, mineralocorticoids, adrenal hyper-androgenism, and impaired development and function of the adrenal medulla.50–52 More rare forms are caused by defi ciency of 11β-hydroxylase, 17α-hydroxylase, 17,20-lyase, 3β-hydroxysteroid dehydrogenase, or P450 oxidoreductase

Question 13

Secondary adrenal insufficiency results from

Answer 13

any process that involves the pituitary gland and interferes with corticotropin secretion

• Space-occupying lesions or trauma
• Autoinmune
• Genetic disorders

Question 14

Tertiary adrenal insufficiency results from

Answer 14

• Suppression of the hypothalamic-pituitary-adrenal (HPA) axis by long-term administration of high doses of glucocorticoids is the most common cause.
• processes that involve the hypothalamus and interfere with secretion of corticotropin-releasing hormone, arginine vasopressin, or both
• patients cured of Cushing’s syndrome, since the persistently high serum cortisol concentrations before treatment suppress the HPA axis in the same way as high exogenous doses of glucocorticoids
• drugs