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Year 2 LCRS > Endocrinology 7 - Hypoadrenal Disorders > Flashcards

Endocrinology 7 - Hypoadrenal Disorders Flashcards

1
Q

List the causes of adrenocortical failure

A
  • Adrenal glands destroyed (tuberculosis Addisons or autoimmune Addisons)
  • Enzymes in the steroid synthetic pathway not working - congenital adrenal hyperplasia
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2
Q

List the symptoms of hypoadrenalism

A
  • Mucous membrane pigmentation
  • Skin pigmentation darkening (tan, creases of palms, scars) and vitiligo
  • Hair darkening
  • Muscular weakness
  • Loss of weight and nausea
  • Feeling fatigue
  • Postural hypotension
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3
Q

List the consequences of adrenocortical failure

A
  • Fall in blood pressure
  • Loss of salt in the urine
  • Increased plasma potassium
  • Fall in glucose (glutocorticoid deficiency)
  • High ACTH resulting in increased pigmentation (as POMC makes both ACTH and MSH)
  • Eventual death due to hypotension
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4
Q

How is addisons diseases tested for?

A
  • 9am cortisol will be low, ACTH will be high
  • Synacthen test - measure cortisol response following injection of synthetic ACTH
  • Low sodium and high potassium
  • Antibodies
  • Measure urea
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5
Q

What is the commonest cause or congenital adrenal hyperplasia?

A
  • 21-hydroxylase deficiency

- Can be complete or partial

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6
Q

Which hormones are absent in both complete and partial 21-hydroxylase deficiency? Why are they absent?

A
  • Aldosterone and cortisol
  • 21-hydroxylase converts progesterone to 11-deoxycosticosterone (interferes with aldosterone) and 17- OH prog to 11-deoxycortisol (interferes with cortisol)
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7
Q

How long can you survive with complete 21 hydroxylase deficiency?

A

Less than a few weeks

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8
Q

Which hormones are in excess in both complete and partial 21-hydroxylase deficiency?

A
  • Sex steroids (particularly testosterone)
  • This is because the sex steroid is the only pathway that can be undertaken (in complete this is more drastic than in partial)
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9
Q

How does 21-hydroxylase deficiency usually present?

A
  • As a neonate with a salt losing addisonian crisis, as before birth the foetus gets steroids across the placenta
  • Girls may have ambiguus genitalia
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10
Q

What is are the main reasons people with partial 21 hydroxylase deficiency present?

A
  • Hirsutism and virilisation (too many androgens in utero, so therefore born with ambiguous genitalia) in girls
  • Precocius puberty in boys
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11
Q

List the symptoms of partial 21-hydroxylase deficiency.

A
  • Acne
  • Recieding hair-line and baldness
  • Facial hirsutism
  • Androgenic flush
  • Variable pigmentation
  • Small breasts
  • Heavy arms and legs
  • Clitoral enlargement
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12
Q

What does excess 11 deoxycorticosterone cause?

A

Hypertension and hypokalaemia

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13
Q

Which hormones are deficient in 11-hydroxylase deficiency?

A

Cortisol and aldosterone, as these pathways are blocked (11-doxycorticosterone to corticosterone and 11-deoxycortisol to cortisol blocked)

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14
Q

Which hormones are in excess in 11-hydroxylase deficiency?

A

Sex steroids and testosterone, as well as 11-deoxycorticosterone

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15
Q

What are the problems with 11-hydroxylase deficiency?

A

Virilisation, hypertension (due to 11-deoxycorticosterone acting like aldosterone), low potassium

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16
Q

List the three pathways that happen in the adrenal cortex.

A
  • Cortisol first to pregnenolone
  • Mineralocorticoids (aldoserone)
  • Glucocorticoids (cortisol)
  • Sex steroids (testosterone)
  • Similar structures - due to dehydrogenase
17
Q

List the enzymes where things can go wrong in the adrenal cortex

A
  • 21-hydroxylase
  • 11-hydroxylase
  • 18-hydroxylase
  • 17-hydroxylase
18
Q

How do adrenal glands look in autoimmune distruction?

A

They are atrophied, very small and not very useful

19
Q

Which hormones are deficient in 17-hydroxylase deficiency?

A
  • Cortisol and sex steroids

- Progesterone to 17-OH progesterone is blocked

20
Q

Which hormones are in excess in 17-hydroxylase deficiency?

A

11-deoxycorticosterone and aldosterone

21
Q

List the problems seen in patients with 17-hydroxylase deficiency

A
  • Hypertension
  • Low potassium
  • Sex steroid deficiency
  • Glucocorticoid deficiency
22
Q

Describe the synthesis of adrenocortical steroids

A
  • Cholesterol to progesterone
  • Progesterone to 11-deoxycorticosterone (via 21-hydroxylase), to corticostrone via 11-hydroxylase to aldosterone via 18-hydroxylase
  • Progesterone to 17 OH progesterone via 17 hydroxylase, to 11-deoxycortisol via 21 hydroxylase, to cortisol via 11 hydroxylase
  • 17-OH progesterone to sex steroids
23
Q

Define the term addisonian crisis

A
  • A potentially fatal condition where the adrenal glands stop working and there is not enough cortisol
24
Q

List the signs of addisonian crisis

A
  • Extreme tiredness and weakness
  • Confusion/psychosis
  • Nausea, vomiting
  • Fever
  • Extremely low blood pressure
  • Loss of consciousness
  • Fast heart rate

Year 2 LCRS (128 decks)

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