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Pediatrics > Adrenal Disorders > Flashcards

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
Q

By the end of first trimester, how is scrotum and penis form?

A

By conversion of testosterone by 5-a-reductase in genital skin into DHT
(From labioscrotal\uretheral fold > scrotum & penis)

26
Q

What is the role of antimullarian hormone?

A

Stimulate fusion of labioscrotal fold.

27
Q

What is the role of DHT

A

Stimulate fusion of labioscrotal fold

28
Q

What is the innate tendency of the bipotential fetus?

A

To develop into female

29
Q

SRY contains something that will make the fetus a boy, that is?

A

Sex determining region of the Y

30
Q

What are the factors that will make a fetus a boy?

A 
1- SRY 
2- AMH and testosterone 
3- gonadotropin by HPA in 2nd\3rd trimester
4- testosterone > DHT 
5- organs response to androgens
31
Q

What is the scale used to assess ambigous genetalia

A

Prader scale

32
Q

A palpable gonad is probably

A

Undervirlized male

33
Q

Impalpable gonad is probably

A

Over-virilized female

Pediatrics (41 decks)

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