Pediatric Adrenal Flashcards

1
Q

What does the HPA axis control reactions to?

A

Stress

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2
Q

What is the HPA axis?

A

CRH
ACTH
Cortisol
– Cortisol provides (-) feedback to pituitary and hypothalamus

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3
Q

Why is (-) feedback important?

A

Regulates hormone concentration in the blood

– not too much or too little

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4
Q

With the Short-Term Stress Response, what is activated and how?

A

Adrenal medulla via preganglionic sympathetic fibers

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5
Q

With the Short-Term Stress Response, what is released?

A

Catecholamines

- Sympathetics (+) Adrenal Medulla

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6
Q

If a patient presents with an elevated HR, RR, glucose, metabolic rate and bronchiol dilation… what type of stress response is occurring?

A

Short-Term Stress Response

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7
Q

With the Long-Term Stress Response, describe the cascade

A
  • CRH (+) anterior pituitary

- ACTH (+) Adrenal cortex

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8
Q

With the Long-Term Stress Response, what is released?

A

Mineralcorticoids (aldosterone)

Glucocorticoids (cortisol)

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9
Q

With the Long-Term Stress Response, what does the Aldosterone and Cortisol do?

A
Aldosterone = Na+/H2O retention to increased BP
Cortisol = Increased blood glucose and immune suppression
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10
Q

If there is decreased renal perfusion or sympathetic activity, what does the kidney do?

A

Secretes Renin => Aldosterone release through RAAS system

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11
Q

What is the most common cause of Newborn Ambiguous Genitalia?

A

Congenital Adrenal Hyperplasia

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12
Q

What is the most common cause of Newborn Ambiguous Genitalia?

A

Congenital Adrenal Hyperplasia

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13
Q

What is usually deficient with Congenital Adrenal Hyperplasia?

A

21-Hydroxylase

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14
Q

What occurs with Congenital Adrenal Hyperplasia if there is deficient 21-Hydroxylase?

A
  • Decreased Aldosterone, Cortisol

- ELEVATED 17-hydroxyprogesterone and ANDROGENS

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15
Q

Besides ambiguous genitalia, what are some signs of Congenital Adrenal Hyperplasia?

A

Failure to thrive
Vomiting, Dehydration and Shock
Hyponatremia and Hyperkalemia = Hypotension

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16
Q

What is the treatment for Congenital Adrenal Hyperplasia?

A

Hydrocortisone + Fluids/glucose + Management of Hyperkalemia

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17
Q

With the treatment of Congenital Adrenal Hyperplasia, what is the most essential life saving step?

A

Hydrocortisone - IV/IM

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18
Q

What are 3 common causes of Primary Adrenal Insufficiency?

A
  • Adrenal dysfunction/destruction (autoimmune)
  • Adrenal dysgenesis (x-linked adrenal HYPOplasia)
  • Impaired Steroidogenesis (adrenal HYPERplasia)
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19
Q

What are the common symptoms of Primary Adrenal Insuffiiciency?

A

Fatigue
Weakness
Weight loss
Skin Hyperpigmentation

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20
Q

What are 4 lab changes with Primary Adrenal Insufficiency?

A
  • Neutropenia
  • LOW Na+
  • HIGH K+
  • Fasting hypoglycemia
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21
Q

With Primary Adrenal Insufficiency, the 8 am Cortisol is ____ and accompanied by a ____ ACTH

A

LOW morning Cortisol

HIGH morning ACTH

22
Q

Primary Adrenal Insufficiency levels of Cortisol and ACTH

A

LOW cortisol

HIGH ACTH

23
Q

Secondary Adrenal Insufficiency levels of Cortisol and ACTH?

A

LOW cortisol

LOW ACTH

24
Q

Adrenal Crisis presents similarly to sepsis. What are the levels of cortisol, blood sugar, sodium?

25
Adrenal Crisis presents similarly to sepsis. There will be metabolic _____
Acidosis
26
What is the treatment for Adrenal Crisis?
Hydrocortisone + Fluids/glucose + Fludrocortisone + management of hyperkalemia if necessary
27
What lab is indicitave of Cushing Syndrome?
Elevated midnight cortisol levels
28
Elevated midnight cortisol level
Cushing Syndrome
29
What test should normally suppress ACTH?
Dexamethasone
30
If the serum cortisol is suppressed with Dexamethasone, is it Cushing Syndrome?
NO
31
Primary Hypercortisolism, there will be low ACTH. Will there be cortisol suppression with the dexamethasone suppression test?
NO
32
When will there be Cortisol suppression when you perform the dexamethasone suppression test?
Cushing Disease only
33
Another name for Primary Aldosteronism?
Conn Syndrome
34
Can an Adrenal Adenoma producing primary aldosteronism be present with Conn Syndrome?
Can or cannot -- doesn't matter either way
35
What are some symptoms of Primary Aldosteronism?
HTN | Hypokalemia = muscle weakness, polyuria, polydipsia, paresthesias
36
Triad with Primary Aldosteronism?
LOW renin HTN Hypokalemia Metabolic Alkalosis
37
Triad with Primary Aldosteronism
LOW renin HTN Hypokalemia Metabolic Alkalosis
38
Where are Paragangliomas?
Outside the adrenal gland
39
What can Paragangliomas secrete? (although they do not have to)
Catecholamines
40
What tumor will present identically to a Pheochromocytoma?
Paragangliomas
41
How do you test for Pheochromocytomas/Paragangliomas?
Plasma fractionated free metanephrines
42
Paragangliomas will present like?
Pheochromocytomas | = paroxysmal HTN, headaches, palpitations, sweating
43
How will the patient present after the paroxysmal attack of a pheochromocytoma or paraganglioma?
Pallor
44
When treating a Pheochromocytoma, what should always be given first?
Alpha Blocker
45
What can be given after the Alpha Blocker when treating Pheochromocytomas?
Beta blocker
46
What happens if you give a beta blocker before an alpha blocker with a pheochromocytoma?
It can raise the BP even higher
47
How is Von Hippel Lindau Type 2 disease inherited and what 3 things can present?
AD | = Pheochromocytoma + hemangioblastoma of cns/eye + RCC
48
What is an Adrenal Gland Incidentaloma?
Mass found incidentally when patient is getting an abdominal CT/MRI for another reason
49
Most Adrenal Gland Incidentalomas are benign but if they are > 4 cm or if the patient has a history of malignancy they should be _____
Resected
50
What 3 things should you assess for if you find an Adrenal Gland Incidentaloma?
Cushing Syndrome Hyperaldosteronism Pheochromocytoma