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Adrenal Insufficiency (Addison's Disease) in Children

by Brian Alverson, MD
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    00:02 Let?s shift gears here and talk a little bit now about adrenal insufficiency.

    00:07 This is when the adrenal glands are not creating enough steroid.

    00:11 Addison?s disease is a primary disease of adrenal insufficiency which is a flaw in the adrenal production of cortisol.

    00:20 This is because the adrenal gland is affected broadly, mineralocorticoids are usually affected as well.

    00:28 So in Addison?s disease, which is the autoimmune attack on the adrenal gland, these patients have a normal HPA access at first and then after disease they're going to have an attack on both the cortisol and the mineralocorticoid elements of their adrenal function.

    00:48 Flip side in secondary disease, it's the pituitary or the hypothalamus that?s the problem.

    00:55 This decreases stimulation of cortisol.

    00:59 But remember, mineralocorticoid is stimulated by ACTH from another part of the pituitary.

    01:05 So it is still produced by the functioning adrenal gland.

    01:09 So in these patients, you'll see something more like these where they have low ability for their hypothalamus, the pituitary, or the adrenals to function correctly, you'll see less cortisol but you will see a normal amount of mineralocorticoid effect.

    01:24 So this distinction between secondary and primary disease is really whether or not there is any mineralocorticoid affect going on.

    01:32 So what are some causes of primary adrenal insufficiency? Well, rarely patients can have underdeveloped or impaired function of their adrenal glands.

    01:42 This can be from transcription factor deficiencies or enzyme production problems.

    01:48 One example of this would be Smith-Lemli-Opitz disease, this is a genetic syndrome where patients have a hard time making cortisol and these patients can absolutely have adrenal insufficiency.

    02:00 They also have a host of other problems with kidneys, lungs, bones, you name it, but adrenal insufficiency is certainly part of the problem.

    02:09 Also, patients more commonly can have a destruction of the adrenal gland.

    02:15 That?s an example of say, Addison?s disease.

    02:18 So there are lots of conditions that can cause destruction of the adrenal gland and I wanna go through them here because it's important to understand the differences between them.

    02:28 Addison?s disease is absolutely the most common cause of acquired adrenal insufficiency in children.

    02:34 It's an autoimmune attack on the adrenal glands.

    02:36 It may be a part of PAS (Polyglandular Autoimmune Syndrome) and so when we see patients with autoimmune Addison?s disease we also wanna check for things, their other glandular functions, such as thyroid and other hormones to make sure that they are intact.

    02:55 Infection can absolutely cause adrenal insufficiency and the classic infection we think of is meningococcal sepsis.

    03:03 This syndrome where there is a watershed infarct in the adrenal gland during the setting of meningococcal sepsis is called Waterhouse?Friderichson syndrome That might be on an exam that you'll see sometime soon, so remember that Waterhouse?Friderichson is meningococcal sepsis causing adrenal insufficiency.

    03:24 Before the recent history, when we had a lot more tuberculosis in the United States that was also a cause of adrenal insufficiency and could rarely be a cause now.

    03:35 There can be metabolic diseases that cause adrenal insufficiency and the one you might wanna remember is adrenoleukodystrophy.

    03:44 Remember, adrenoleukodystrophy is the disease that was seen in the movie, Lorenzo?s oil.

    03:49 This is an X-linked disease that almost universally affects males.

    03:54 These patients have a hard time breaking medium-chain fatty acids down and they can have buildup of toxic metabolites in the adrenal glands resulting in adrenal dysfunction.

    04:06 They also have problems in their brain and can get ataxia and motor problems as well and the treatment is bone marrow transplant.

    04:13 Infiltrative problems can also occur in the adrenal glands.

    04:18 Diseases such as hemochromatosis, sarcoid, or histiocytosis can all affect the adrenal gland.

    04:26 Rarely, and very rarely, drugs can cause these problems in children.

    04:31 We don?t use etomidate very often in children but certainly that can cause it, also ketoconazole.

    04:37 Also, hemorrhage can cause it.

    04:40 We do rarely see this in the neonatal population where a birth trauma, sepsis, or a coagulopathic problem causes an infarct in the adrenal gland which can cause problems.

    04:52 This is a horrible and dramatic picture of an adult with a meningococcus.

    04:56 Meningococcus is a highly fatal condition.

    05:00 Patients need to be aggressively treated.

    05:02 They can go rapidly into shock.

    05:04 You will not miss Waterhouse- Friderichson but it's much more common in children than it is in adults.

    05:11 So children with meningococcus are more likely to get the adrenal gland dysfunction than adults do.

    05:18 Adrenoleukodystrophy, as a reminder, very rare on girls.

    05:23 Its inability to break down long-chain fatty acids, there?s a buildup of these fats.

    05:28 It damages the myelin, results in a progressive weakness, and ataxia.

    05:33 The adrenals are destroyed as well and the treatment is a bone marrow transplant.

    05:38 Damage done is done so we need to transplant as early as we can make the diagnosis and that may well be before neurologic symptoms occur.

    05:49 So secondary adrenal insufficiency is perhaps less common and this is the result of an underdeveloped HPA axis from taking glucocorticoids which are inhibiting the HPA axis, or from hypothalamic or pituitary destruction such as a tumor or an infarct say in a patient with a sickle cell disease.

    06:14 So how does adrenal insufficiency present? Well, usually before Addisonian crisis, when a patient has an autoimmune attack on their adrenal glands there is a preceding viral illness.

    06:28 Then patients will develop complaints of drowsiness, fatigue, anorexia, weight loss, abdominal pain, and nausea.

    06:35 These are fairly nonspecific so there may be a delay in diagnosis.

    06:40 What?s clear is that if there is a history of steroid use, this implies this may be HPA axis suppression rather than actually Addison?s disease.

    06:53 An intercurrent illness in a patient already on steroids, that patient may just need stress dosing.

    07:01 Remember, stress dosing is important.

    07:03 Patients chronically on steroids when sick are effectively adrenal insufficient.

    07:10 What?s key and perhaps the best diagnostic thing you can see is a tan without tanlines.

    07:17 What?s happening is a patient who has adrenal insufficiency that?s because of an assualt to the adrenal gland, that patient will have very high ACTH levels.

    07:26 There is no steroid inhibiting the ACTH.

    07:29 ACTH excites melanin in the skin and causes a tanning.

    07:35 So these patients will be very dark skinned.

    07:38 A child who is tan in the winter without tanning lines who comes in tired and sick probably has Addison?s disease.

    07:48 When you get a lab in a patient with Addison?s disease, you may see abnormalities in every single part of the Chem-7.

    07:56 Because the child has mineralocorticoid deficiency, they are gonna be wasting sodium and retaining potassium so they'll have a low sodium and a high K.

    08:05 Because they are profoundly dehydrated, likely because of that sodium loss, they're gonna be acidotic and have a low CO2.

    08:14 They'll have a low chloride because you have to be electrically neutral here if your sodium is low your chlorides are likely a lot going to fall as well.

    08:23 Because they're so dehydrated, they may have an elevated BUN and creatinine.

    08:29 Additionally, they'll probably have a low glucose.

    08:32 The reason they'll have a low glucose is that children use steroids as a counter regulatory hormone to insulin.

    08:40 So now they'll have unopposed insulin without the steroids and as a result they'll drive down their sugar.

    08:48 In fact, that low glucose can sometimes get them into trouble.

    08:52 In adrenal insufficiency, we're gonna make a diagnosis on primary versus secondary disease essentially by making sure we measure an ACTH level.

    09:03 It makes perfect sense, if you have primary adrenal insufficiency you're gonna have no inhibition of your pituitary gland and you have a high ACTH as well as a low cortisol.

    09:15 In secondary disease, you will have a low cortisol and a low ACTH.

    09:20 Other labs you might get and see patients will have an elevated plasma renin activity.

    09:27 This is because there?s sodium loss and so volume loss so the kidneys kick up their renin production as a result of decreased availability of blood pressure.

    09:38 Also, aldosterone levels will obviously be low.

    09:41 In imaging, we will see adrenal MRI if we suspect an adrenal hemorrhage.

    09:47 We'll see it right there and certainly if we suspect a tumor, we'll see it right there.

    09:52 But generally, there's no imaging needed for Addison?s disease.

    09:56 We figured out this is an autoimmune attack and we know where to go from there.

    10:00 Brain MRI is of course indicated for secondary adrenal insufficiency so we can see what?s going on in that pituitary or the hypothalamus.

    10:10 Management of adrenal insufficiency is to rapidly fix their hypovolemia.

    10:16 And that?s actually the most critical thing.

    10:18 These patients are profoundly dehydrated and required large amounts of volume repletion We have to fix their hypoglycemia so they won?t seize so we give them boluses of sugar as well.

    10:28 And if they have very high potassium, we have to fix that too before they have a fatal arrhythmia.

    10:35 Slowly, we will fix the hyponatremia over 1 to 2 days to prevent brain problems as a result of rapid shifts in osmos.

    10:44 We'll give oral glucocorticoid, usually hydrocortisone because it has some also mineralocorticoid effect.

    10:50 And we'll give oral mineralocorticoid, usually fludrocortisone, if the low aldosterone is present.

    10:59 In patients with Addison?s they will need steroids for life.

    11:03 There's no bringing these adrenal glands back.

    11:06 Don?t forget, and this is incredibly important, if a patient is on steroids all the time for their adrenal insufficiency and they come in with an illness, pneumonia, gastroenteritis, we have to give them stress dose steroids, much higher doses to accommodate their sick state, so they aren't effectively adrenally insufficient.

    11:29 That?s all I have for you today about cortisone and adrenal insufficiency.

    11:35 Thanks for your attention.


    About the Lecture

    The lecture Adrenal Insufficiency (Addison's Disease) in Children by Brian Alverson, MD is from the course Pediatric Endocrinology. It contains the following chapters:

    • Pathology
    • Clinical Presentation
    • Diagnosis
    • Management

    Included Quiz Questions

    1. Hyperkalemia
    2. Hyperglycemia
    3. Hypernatremia
    4. Alkalosis
    5. Hyperchloremia
    1. Mineralocorticoids are not usually affected by Addison’s disease.
    2. Addison’s disease is a primary adrenal insufficiency resulting from decreased production of cortisol.
    3. The primary site of the defect in secondary adrenal insufficiency is pituitary or hypothalamus.
    4. The most common cause of primary adrenal insufficiency is an underdeveloped gland.
    5. Along with adrenal insufficiency, Smith-Lemli-Opitz disease also presents with defects in the kidneys, lungs and bones.
    1. Meningococcal sepsis
    2. Actinomycosis
    3. Histoplasmosis
    4. Neisserial bacteremia
    5. Staphylococcal infection
    1. Autosomal dominant Adrenoleukodystrophy
    2. Autoimmune destruction
    3. Ketoconazole
    4. Histiocytosis
    5. Birth trauma
    1. …is the buildup of fat damaging myelin.
    2. …is very common in girls than boys.
    3. …is the inability to break down fibrin.
    4. …is treated with plasmapheresis.
    5. …involves destruction of the thyroid gland as well.
    1. Low ACTH
    2. Low cortisol
    3. Increased plasma renin
    4. Low aldosterone
    5. Increased creatinine
    1. Give steroids for life.
    2. Give high dose aldosterone.
    3. Rapidly fix hyponatremia.
    4. Slowly fix hypovolemia.
    5. Avoid fludrocortisone.

    Author of lecture Adrenal Insufficiency (Addison's Disease) in Children

     Brian Alverson, MD

    Brian Alverson, MD


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