Adrenocortical Hyperplasia

Question 1

What is adrenocortical hyperplasia?

Answer 1

• When adrenal cortex is larger than it should be

- Can be congenital or acquired

Question 2

Pathogenesis of congenital adrenocortical hyperplasia?

Answer 2

• Group of autosomal recessive disorders
• Deficiency/lack of enzyme required for steroid biosynthesis
• Altered biosynthesis leads to increased androgen production
• Reduced cortisol stimulates ACTH release and cortisol hyperplasia (10-15x normal weight)

Question 3

What does increased androgen production cause?

Answer 3

Masculinisation

Precocious puberty

Question 4

Pathogenesis of acquired adrenocortical hyperplasia?

Answer 4

• Endogenous ACTH production
• Bilateral adrenal enlargement
• Diffuse or nodular

Question 5

What drives diffuse adrenocortical hyperplasia?

Answer 5

ACTH driven

Question 6

Which type is nodular acquired adrenocortical hyperplasia?

Answer 6

ACTH independent

Question 7

What does endogenous ACTH production cause?

Answer 7

Pituitary adenoma (Cushing's disease) 
-Ectopic ACTH: Paraneoplastic syndrome