Waterhouse-Friderichsen Syndrome & Addison´s Disease – Adrenal Insufficiency

00:01 Take a look at the picture here and we're seeing Waterhouse-Friderichsen.

00:05 That’s massive hemorrhage that’s taking place in the adrenals, what happened? It’s a fact that you had maybe an infection – meningococcemia, as they're a species or maybe perhaps DIC. In DIC what triggered this? All kind of triggers – maybe it was acute myalgia ischemia type III.

00:23 Maybe this was a patient that had amniotic fluid emboli. Maybe this was a patient that had severe sepsis.

00:29 Whatever the case maybe, once DIC kicks in, oh, my goodness, your patient looks like death.

00:35 There's bleeding from every single orifice, up and down his or her body and also internally.

00:42 You have an increase in bleeding time, PT and PTT, you have a decrease in platelet count and there might be massive hemorrhage into both of the adrenals resulting in primary adrenal insufficiency.

00:54 Let’s take out the symptoms of both primary and secondary adrenal insufficiency.

00:59 If you have complete destruction that’s taking place of your adrenal gland, then you can expect the following – you do not have cortisol, you do not have aldosterone, and you might not be producing your adrenal androgens.

01:14 If you don’t have aldosterone you could expect your blood pressure to be extremely weak.

01:18 If you don’t have aldosterone you could expect the patient to not have much sodium.

01:23 If you don’t have aldosterone you can expect hyperkalemia, if you don’t have aldosterone then you can expect your pH to be decreased, right? If you don’t have cortisol, adrenal insufficiency, wow, you're worried about death.

01:38 So, with all that said – weakness, weight loss, anorexia, nausea, vomiting.

01:44 There’ll be hypotension because there is no aldosterone but that would be much more pronounced in primary.

01:51 What's the difference between primary and secondary? Primary is the adrenal gland itself being destroyed by many, many causes including autoimmune disease, maybe infection, maybe Waterhouse-Friderichsen, maybe iron overload and such.

02:04 Secondary would mean that the anterior pituitary is not working properly and therefore not releasing ACTH.

02:13 Hyponatremia, less common in secondary more pronounced in primary because you don’t have the aldosterone, are we clear? The entire adrenal gland is being destroyed.

02:25 There’s something called acute adrenal insufficiency in other words it’s a crisis.

02:32 What is the most important stress hormone again? Cortisol.

02:37 So it can be precipitated by severe physiologic stress such as surgery, sepsis – that’s pretty severe significant stress.

02:46 In patients with chronic adrenal insufficiency or by rapid development of AI is what referred to being our crisis.

02:58 Adrenal hemorrhage, pituitary apoplexy may result in complete lack of cortisol being supplied into the patient because the adrenal or maybe perhaps the pituitary, has lost complete function during such physiologic severe stresses.

03:17 This is crisis – sudden death, no cortisol, that is how important cortisol is to you.

03:28 Here, we have POMC. What this picture is going to show you is up in the anterior pituitary.

03:34 Proopiomelanocortin , opio referring to your beta-endorphin.

03:39 Melano, then referring to your melanocytes stimulating hormone and they have ACTH.

03:46 If its primary adrenal insufficiency such as Addison’s, what is the ACTH level? Elevated.

03:53 What about POMC? Elevated.

03:55 If there is such melano – then what your patient look like especially on the mouth region or mucosa might be hyperpigmented, how come? Because of melano, melano, melano – melanocyte stimulating hormone.

04:09 If you had secondary adrenal insufficiency our problem is in the pituitary gland, there’s no ACTH, obviously there won’t be hyperpigmentation.

04:18 Hyperpigmentation is my topic of the skin and what you’re noticing here is hyper pigmentation of your oral mucosa.

04:29 Hyperkalemia due to loss of aldosterone.

04:36 Secondary adrenal insufficiency where is my problem? Grouped together, hypothalamus and pituitary, you focus upon the pituitary.

04:45 Hypothalamus technically will be called tertiary adrenal insufficiency, at least now two causes of adrenal insufficiency – primary or secondary.

04:57 Causes. Metastatic cancer. What is the cancer invading? The pituitary – infection, infarction, Sheehan, irradiation -- all of these may be then causing damage to whom? The pituitary – topic secondary.

05:16 There is no hyperpigmentation because there isn’t enough ACTH.

05:21 There’s no hyperkalemia as aldosterone is unaffected, that is incredibly important.

05:29 See, if secondary adrenal insufficiency and you're focusing upon the ACTH, what in the world is hyperkalemia not pronounced in secondary adrenal insufficiency? Because ACTH as I’ve mentioned numerous times, plays only a minor role in aldosterone feedback.

05:51 Your major role for aldosterone feedback would be your RAS system.

05:56 So therefore, even though ACTH in it not being there might affect your aldosterone negatively, please understand that you still would have proper feedback because renin is not being affected.

06:10 Clinically, that is absolutely critical for you to understand.

06:15 Pathology here, if you don’t have ACTH, gone are the adrenal.

06:21 So now, come back to my point.

06:23 Yet once again, exogenous use of cortisol – what's your patient look like? Prototypical Cushing, moon faces, so on and so forth. Inside the body what is happening? Secondary adrenal insufficiency.

06:38 Secondary because both of the adrenal are now undergoing atrophy – fascinating.

06:44 So what do you wanna do? Next step in management, once the use of prednisone clinically is done, you as a doctor have now understood that prednisone has completed the job for the patient.

06:58 Never stop the prednisone abruptly and you make sure you emphasize that to the patient.

07:04 Don’t just say passively.

07:06 You need to make sure that you actively and convincingly tell the patient you wean the patient off the prednisone, you have to because adrenals are atrophied or your patient’s dead. Do you wanna be in practice? You see what I’m saying. Obviously I’m being over dramatic but it’s important that you understand that.