Management of Cushing’s Syndrome – Adrenal Gland

by Carlo Raj, MD

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    00:01 Management of Cushing’s syndrome, what do you want to do? Why do want to treat the underlying cause? If it’s iatrogenic, stop giving the Cushing… I mean, stop giving the prednisone.

    00:10 If it’s the adrenal source, adrenalectomy is performed.

    00:13 If it’s Cushing’s disease, where is this? In the anterior pituitary, perhaps you have an adenoma.

    00:21 Therefore, transsphenoidal resection is usually the first line of treatment.

    00:26 Next, there is something called pituitary radiation therapy, XRT.

    00:32 Sometimes used when tumour is not found or not surgically cured.

    00:37 It may decrease risk of what’s known as Nelson’s syndrome, the rapid tumour enlargement causing mass effect and hyperpigmentation after loss of negative feedback in patients for whom adrenalectomy becomes necessary.

    00:52 Okay, so, here, you’re thinking about what’s known as pituitary radiation.

    00:58 I need you to be familiar with what’s known as Nelson’s syndrome, a rapidly tumour enlarging and compression, meaning to say that now, you’re worried about maybe visual defects and the hyperpigmentation more so because of increased POMC… radiation.

    01:18 Bilateral adrenalectomy, Adx it stands for, removal of adrenalectomy with lifelong glucocorticoid and mineralocorticoid replacement is definitive treatment for Cushing’s disease.

    01:29 Why? Interesting enough, if it’s Cushing’s disease, what does that mean? It means that your excess cortisol is being caused by ACTH from the anterior pituitary, right? So, therefore, both adrenals are affected, so bilateral adrenalectomy with lifelong glucocorticoid and mineralocorticoid replacement; without the glucocorticoid, you’re dead.

    01:56 Without the mineralocorticoid, your blood pressure is depressed severely.

    02:01 Adrenalectomy, usually performed after pituitary radiation to decrease the risk of, once again, Nelson’s, Nelson’s, Nelson’s… Clinically, that is a very important point.

    02:12 If you missed that discussion, go back to the previous section where we just finished Nelson’s significance.

    02:22 For ectopic Cushing’s, where are you looking? Surgery or other treatment.

    02:27 Here, you might be looking at chemo or radiotherapy of the inciting tumour, maybe perhaps coming from a small cell lung cancer of the lung.

    02:39 Medical therapy for when surgery is contraindicated or delayed.

    02:42 For example, post Cushing’s disease and radiation.

    02:47 Adrenal enzyme inhibitors, 17-20 desmolase inhibitors; in the US-ketoconazole; in UK-metyrapone which inhibits 11-beta-hydroxylase.

    03:02 You want to know about ketoconazole and desmolase… remember desmolase? We talked about the patho-physiology.

    03:07 Desmolase is responsible for the first step of converting cholesterol into pregnenolone as you move down through your adrenocortical hormone synthesis.

    03:18 Adrenolytics, these are mitochondrial inhibitors of adrenal cortical cells mitotane.

    03:25 Neuro agents, things that you want to keep in mind, understand the concept first.

    03:31 What are we trying to take care of? Cushing’s… too much cortisol.

    About the Lecture

    The lecture Management of Cushing’s Syndrome – Adrenal Gland by Carlo Raj, MD is from the course Adrenal Gland Disorders.

    Included Quiz Questions

    1. Transsphenoidal resection
    2. Pituitary radiation
    3. Adrenalectomy
    4. Bilateral adrenalectomy
    5. Chemotherapy
    1. Pituitary radiation and adrenalectomy
    2. Transsphenoidal resection
    3. Pituitary radiation alone
    4. Bilateral adrenalectomy alone
    5. Chemotherapy
    1. 17, 20-desmolase
    2. 11-beta-hydroxylase
    3. DHEA
    4. 21-alpha-hydroxylase
    5. 20, 22-desmolase

    Author of lecture Management of Cushing’s Syndrome – Adrenal Gland

     Carlo Raj, MD

    Carlo Raj, MD

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