Congenital Adrenal Hyperplasia (CAH) – Adrenal Insufficiency

by Carlo Raj, MD

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    Let’s do Congenital Adrenal Hyperplasia. Another cause of adrenal insufficiency, but let’s get away from the adrenals being damaged, let’s get away from the pituitary being damaged. Congenital adrenal hyperplasia, as the name implies, how old is your patient? A child. What’s going on in the child? Enzyme deficiency. Here, in the very beginning section of adrenal patho-physiology, I walked you through, in great detail, the physiology of your adrenal cortex with your various enzymes. Name me the enzyme that will take you from zona glomerulosa into the zona fasciculate. 17 alpha-hydroxylase. Name me the enzyme that will take you from the first step of your zona glomerulosa, fasciculata, reticularis from your pregnenolone into progesterone. 3 beta- hydroxysteroid dehydrogenase. We talked about that. Give me the next two enzymes that we will be focusing upon. 21 and 11, go in chronological order. Of all the enzymes that you want to know, which one do you want to know for sure? 90 percent of your congenital adrenal hyperplasia… 90 percent of congenital adrenal hyperplasia is due to 21 beta-hydroxylase insufficiency, that’s where you look first. Be smart about how you study. What about the other enzyme deficiencies, could they occur? Yes, of course. The other five percent is actually 11 beta, that’s already 95 percent of all congenital adrenal hyperplasia. You have a measly three percent for the other enzymes. Whatever, I’m not even going to discuss them. So, I’ll do the 95 percent obviously. What happens? Child and when this occurs, enzyme is not present. If the enzyme isn’t present, it doesn’t matter which one actually and if there isn’t, then you don’t have enough cortisol. If you don’t have enough cortisol, what’s the feedback? ACTH. All congenital adrenal hyperplasia will have patients… will not have sufficient cortisol as a...

    About the Lecture

    The lecture Congenital Adrenal Hyperplasia (CAH) – Adrenal Insufficiency by Carlo Raj, MD is from the course Adrenal Gland Disorders.

    Included Quiz Questions

    1. 21-beta-hydroxylase
    2. 17-alpha-hydroxylase
    3. 3-beta-hydroxysteroid dehydrogenase
    4. 11-beta-hydroxylase
    5. Desmolase
    1. Decreased androgen production
    2. Decreased cortisol levels
    3. Bilateral adrenal hyperplasia
    4. Increased ACTH
    5. Variable production of mineralocorticoids
    1. Increased androgen production
    2. Decreased androgen production
    3. Decreased cortisol production
    4. Increased ACTH
    5. Decreased aldosterone
    1. Low serum DHEA
    2. Hyponatremia
    3. Hyperkalemia
    4. Hypotension
    5. Increased levels of 17-OH progesterone
    1. Lack of virilization in female patients
    2. Hypertension
    3. Hypokalemia
    4. Increased levels of 11-deoxycorticosterone
    5. Increased serum DHEA

    Author of lecture Congenital Adrenal Hyperplasia (CAH) – Adrenal Insufficiency

     Carlo Raj, MD

    Carlo Raj, MD

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