Adrenal Disorders Flashcards

1
Q

Differentiate between
(11) - VS (3) - VS (21) hydroxylase.

In BP

A

All No cortisol - high androgen

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2
Q

Differentiate between
(11) - VS (3) - VS (21) hydroxylase.

In veralization

A

All no cortisol - high androgen

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3
Q

Which type of CAH has high aldosteroene?

A

Defect in 17 alfa hydroxylase enzyme

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4
Q
Name the defect in each 
[21]
[17]
[11]
[3]
A
  • No aldosterone, cortisol - yes androgen
  • No cortisol, androgren - yes aldosterone
  • No cortisol - yes androgen, deoxycortison
  • No aldosterone, cortisol - yes DHEA
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5
Q

Which type of CAH has high deoxyxortison?

A

11

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6
Q

Which type of CAH has high DHEA?

A

3

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7
Q

What is the most common cause of primary adrenal insufficency?

A

Conginital adrenal hyperplasia

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8
Q

What is the CRH, ACTH, and cortisol level of primary adrenal insufficency?

A

High CRH, ACTH and low cortisol

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9
Q

What is the clinical presentation of primary AI?

A
  • non-specific: abdominal pain, slow growth, Fatigue, nausea, weakness, headache
  • salt wasting symptoms: dehydration, hypotension, weight loss, salt craving
  • low cortisol symptoms: hypoglycemia
  • high ACTH symptoms: hyperpigmentation
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10
Q

What are the symptoms present in primary AI with salt wasting?

A
  • dehydration, weight loss, lethargy

- hyponatermia\hyperkalemia, hypoglycemia

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11
Q

What is adrenal crisis?

A

Gradual hypotension or shock

  • high renin activity
  • low aldosterone secretion
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12
Q

Where’s the lesion in

  • primary AI
  • secondary AI
  • tertiary AI
A
  • adrenal cortex
  • pituitary
  • hypothalamus
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13
Q

What is the CRH, ACTH, and cortisol level of secondary adrenal insufficency?

A

Low ACTH, CRH, cortisol

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14
Q

Differentiate between primary and secondary AI?

A

Secondary don’t have:

  • High acth: no pigmentation
  • high aldosterone: no dehydration etc.

Both have: hypoglycemia.

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15
Q

When is AI has more pronounced hypoglycemia?

A

In secondary which may be combined with growth hormone deficiency

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16
Q

What is the most definitive test for AI?

A

Serum cortisol levels

Morning cortisol <3

17
Q

Positive adrenal autoantibodies can help you identify which adrenal disorder?

A

Addison’s

18
Q

What test to orders when suspecting CAH in newborn?

“Either due to ambigous genetalia or salt wasting”

A
  • random cortisol studies
  • random androgen studies
  • dynamic testing of HPA axis by ACTH stimulation test
19
Q

How to treat

  • primary AI:
  • secondary AI:
A
  • glucocorticoid & mineralocorticoid

- cortisol

20
Q

When to give supaphysiological dose of hydrocortisone?

A

In CAH to supress adrenal androgens

21
Q

Define the following:

  • clitoromegaly
  • hypospadia
  • labioscrotal fusion
  • micophallus:
  • posterior fusion
A
  • enlargement of clitoris
  • opening of uretheral other than the tip of the penis
  • fusion of labia
  • phallus less than 2.5
  • fusion of labia at posterior aspect near anus
22
Q

Criteria for suspecting DSD

In newborn\infant

A
  • discordance between genital appearance and prentalal karyotype
  • overt ambiguity can’t be determined (cloacal exstrophy)
  • F genetalia: + enalrged clitoris\posterior labial fusion
  • M genetalia + bilateral undecendant tstes\ hypospadia\ micropenia
23
Q

What’s the criteria for suspecting DSD in older childre

A
  • previously unrecognized genital ambiguity
  • inguinal hernia in a girl
  • delayed or incomplete puberty
  • primary amenorrhea or virilization
  • breast development in boy
  • hematuria in boy with suprapubic mass (hemaatocolpos) with breast enlargment after puberty in boy with hypospadia\undescended gonads
24
Q

How is the fetal genital development in the first and second trimester?

A

First: testosterone under HCG control

Second\3rd: pituitary gonadotropins

25
By the end of first trimester, how is scrotum and penis form?
By conversion of testosterone by 5-a-reductase in genital skin into DHT (From labioscrotal\uretheral fold > scrotum & penis)
26
What is the role of antimullarian hormone?
Stimulate fusion of labioscrotal fold.
27
What is the role of DHT
Stimulate fusion of labioscrotal fold
28
What is the innate tendency of the bipotential fetus?
To develop into female
29
SRY contains something that will make the fetus a boy, that is?
Sex determining region of the Y
30
What are the factors that will make a fetus a boy?
``` 1- SRY 2- AMH and testosterone 3- gonadotropin by HPA in 2nd\3rd trimester 4- testosterone > DHT 5- organs response to androgens ```
31
What is the scale used to assess ambigous genetalia
Prader scale
32
A palpable gonad is probably
Undervirlized male
33
Impalpable gonad is probably
Over-virilized female