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ENDOCRINE > Adrenal > Flashcards

Adrenal Flashcards

1
Q

Cigarette paper appearance on dorsum of hand

A

Liddle sign

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2
Q

MC presentation cushings bone

A

Poor linear growth and weight gain

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3
Q

Most common form of hirautism in cushings

A

Vellus hypertrichosis on the face

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4
Q

Cut off for salivary cortisol value

A

> 2 ng/mL or

>5.5 nmol/L

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5
Q

Sensi and speci of salivary cortisol

A

100% sensi

97 % specific

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6
Q

Measurement of acth suggestive of cushings

A

> 90 pg/mL

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7
Q

% of patients with ectipicnsyndrome witb hypokalemix alkalosis

A

95%

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8
Q

How to do high dose dexa and what is the positive response

A

2 mg dexa q6 hrs x 2 days

(+) response= 50% fall cortisol from basal value

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9
Q

Ratio of petrosal sinus to peripheral vein

A

Ectopic- less than 1.4:1

Cishings dse- greater than 2

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10
Q

Dose of ketoconazole to block steroidogenesis

A

400-1600

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11
Q

Mode of action mitotane in cushings

A

Adrenolyto. Deug that is taken by both normal ans malignant adrenal tissue causing ateophy and necrosis

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12
Q

Improves diabetes in patients with cushings

A

Mifepristone

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13
Q

The multireceptor somatostatin analogue which is effective in px with cushings

A

Pasireotide

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14
Q

Triple A syndrome

Inherited primary adrenal insuffieicncy

A

Allgrove syndrome
aCTH resistance
Achalasia
Alacrima

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15
Q

AI eith demyelination in CNs

A

Adrenoleukodystrophy

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16
Q

Adequate response after acth

A

> 550

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17
Q

Triad of Triple A syndrome/ Allgeove syndrome

A

ACtH resistance
Achalasia
Alacrima

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18
Q

Defect in Allgrove syndrome

A

Mutationj AAAS gene which encodes ALADIN

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19
Q

Aldosterone is secretes from the ZG from 3 principal secretagogues

A
  • angiotensin II
  • potassium
  • acth
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20
Q

ACTH binds to what receptor

A

MC2R

Melanocortin 2 receptor

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21
Q

Why is aldosterone limited to ZG?

A

Zone specific expression of aldosterone synthase CYP11B2

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22
Q

% of cortisol bound by the cortisol binding globulin

A

90%

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23
Q

Conditions with jn teased levels of cortisol binding globulin

A

Estrogen

Chronic active hepatitis

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24
Q

Conditions with

Reduced cortisol binding globulin

A

Cirrhosis
Nephrotic syn
Hyperthyroidism

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25
Q

The urinary free cortisol is ___% of the total cortisol

A

1%

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26
Q

Half life of circulating cortisol

A

70-120 mins

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27
Q

What are the 2 isoenzymes of 11B HSD

A

Type 1 NADPH dependent

Type 2 Nad dependent

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28
Q

Conversion of cortisol to cortisone in kidney

A

HSD 11B 2

11 B hydroxydehydrogenase type 2

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29
Q

Hsd that inactivates cortisol to cortisone and permits aldosterone to bind to the MR jn vivo

A

Type 2

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30
Q

Type of 11 B HSD

That converts cortisone to cortisol (all oral steroid)

A

Type 1

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31
Q

Effect of GC on bone

A

Inhibit osteoblast function

Inhibits intestinal calcium absorption

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32
Q

How do Glucocorticoids cause hypertension

A

Increase sensitivity to pressor agents such as cathexolamines and angiotensin II and
Reduces NO mediated endithelial dilation

Cortisol can act on distal nephron to cause sodium retention and potassium loss

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33
Q

Mineralocorticoid activity of dexamethasone

A

0

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34
Q

Affinity of synthetic steroids to COrtisol binding globulin

A

Low. Most circulate as free steroid or bound to albumin

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35
Q

% of patients with cushings syndrome with hypertension

A

75%

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36
Q

Effect if steroids to eye

A

Raised intraocular pressure and exophthalmos

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37
Q

Diagnosis of Cushings Syndrome

Rationale for midnight cortisol

A

Nadir of cortisol midnight lost in patients with Cushings syndrome
Level greater than 200 nmol/L
>7.5 ug/dL

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38
Q

Diagnosis of cushings daluvary cortisol

A

> 2 ng/mL

5.5 nmol/L

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39
Q

Inferior petrosal sinus sampling rationale

A

Normal= ratio of ACTH concentration in the inferior petrosal sinus to that in simultaneously drawn peripheral blood is less than 1.4:1

In cushings disease, ratio is greater than 2

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40
Q

Diagnostic of cushings: IPSS with CRH administration

A

Acth in petrosal sinus/peripheral ratio greater than 3

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41
Q

Used in some centers in lieu od CRH (during IPSS)

A

Desmopressin

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42
Q

How to check if hydrocortisone can be discontinued

Omitting HCT dose in am and check for cortisol

A

<200 nmol /L (7 ug/dL) continue
>500 nmol/ L (18.3 ug/dL) replacement can be stopped
Values bet 200-500 ACTH testing can be used

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43
Q

% pf normal aubjects with adrenal incodentalomas

A

5%

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44
Q

Drugs medical treatment of cushings syndrome

A

Metyrapone
Ketoconazole
Mitotane
Somatostatin analogues octreotide and lanreotide

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45
Q

Discriminatory test Glucocorticoid resistance versus cushings

A

Bone mineral densitometry= preserved in patients with glucocorticoid resistance

46
Q

Most common cause of primary adrenal insufficiency in the western world

A

Autoimmune adrenalitis

47
Q

Most common cause of primary adrenal insufficiency in the world

A

Infection

48
Q

Most common sources of adrenal metastases

A

Lung/ breast tumors

49
Q

Anticipare adrenal atrophy in what steroid dose

A

30 mg HcT more than 3 weeks
>7.5 mg pred
>0.75 mg dexa

50
Q

A basal cortisol value more than ____ denotes an intact HPA axis

A

400 nmol/L

14.5 ug/dL

51
Q

Tests to asses adequacy of HPA axis aside from SST

A 
- overnight metyrapone test 
Metyrapone at midnight
Blocks cortisol synthesis 
(Ms cortisol ans 11 deoxycortisol in am) 
Causes elevation of ACTH 
- CRH stimulation test 
Diff primary from secondary causes of AI: 
1) primary- high ACTH after CRH
2) secondary- low ACTH after CRH
52
Q

Why does pregnancy necessitate jncreasing doses of fludrocortisone in parients with primary AI

A

Because progesterone is a mineralocorticoid antagonist

53
Q

Congenital lipoid adrwnal hyperplasia

A

STAR DEFICIENCY
Failure of transport of cholesterol from outer to inner mitochondrial membrane
Pregnenolone synthesis is abolished

54
Q

Same manifestation as STAR deficiency but do not have enlared adrenals

A

p450 side xhain cleavage deficiency

55
Q

Mechanism of pseudohypoaldosteronism

A

Defective renal tubular response to mineralocorticoid in infancy

56
Q

Treatment of Pseudohypoaldosteronism type I

A

Supplementation with salt
2-8 g/day
(Resistant to mineralocorticoid therapy)

57
Q

Treatment for hgpercalciuria and hypercalcemia in pseudohypoaldosteronism

A

Indomethacin

Hydrochlorothoazide

58
Q

Converts cortisol to cortisone

A

Hsd B 2

59
Q

Converts cortisone to cortisol

A

11 HSD B 1

Cortisone reductase

60
Q

Predominant cathecolamine in pheo MEN2

A

Epinephrine

61
Q

Syndromic forma of pheochromocytoma

A 
MEN2A
MEN 2B 
Von hippel lindau disease
NF1 
Carney triad/ syndrome
62
Q

VHL type at risk of developing pheo

A

VH2

63
Q

What is the carney triad

A

GIST
Pulmonary chondromS
Cathecolamine secreting paraganglioma

64
Q

In SDH missense mutations occured frequently except in

A

SDHD

65
Q

Cortisol to creatinine ratio cut off for cushings

A

25

66
Q

Inhibits both uptake and storAge f MIBG

A

Labetalol

67
Q

Inhibits both uptake and taansport of MIBG

A

Reserpint

68
Q

Aloha blockade prior to OR of pheo

A

Phenoxybenzamines 10-20 mg

7-10 days pre op

69
Q

Target for alpha blockade

A

SBP > 90

BP 120/80

70
Q

Beta blockade

A

Trget 60-80 bpm

71
Q

Intraop HTH

A

Phentolamjbe
Nitroprusside
Nicardipine

72
Q

Avoid anesthetic

A

-ine and -ane

Fentanyl …

73
Q

Recurrent pheo

A

Runs in family
Right sided
paRaganglioma

74
Q

Highest concentration of MC receptors are found in:

A

Distal nephron
Colon
Hippocampus

75
Q

(+) results for primary hyperaldosteronism screening

A

pac >15 ng/dL
Pra <1.0
Pac/pra >20 ng/dL

76
Q

(+) result of oral sodium loading

5000 mg Na for 3 days

A

Urine aldosterone > 12 ug/24h

77
Q

(+) result saline infusion test

A

Post infusion PAC > 10 ng/dL

78
Q

(+) result fludrocortisone suppression test

A

(+) result PAC > 6 ng/dL

79
Q

Test for confirmation of hyperaldosterinisn

A

F
I
O

80
Q

Which of the ff xgaracteristic prjnary aldosteronism vfrom iha conpared to Apa

A

Less HTB
Less hyookalemia
Older age

81
Q

When to Do repeat pac after apa surgey

A

1-2 days

82
Q

Recommended post op to avoid hyperK due to chronic suppression tRAAS

A

High sodium

83
Q

Result of impaired activity of the microsomal enzyme HSD 11B2

A

Apparent mineralocorticoid excess

84
Q

Adrenal mets mostly grom

A

Lung

Breast

85
Q

Infection with intra aftenal bleeding in children

A

Psudomonas

86
Q

APS Type 2

A

Addisons
Autoimmune thyroid disease
DM
Hypogonadism

(+) abx to 21 hydroxylase

87
Q

APS type 1

A

Apeced

Addison
Hypoparathyroidis.
Chronic mucocutaneous candidiasis

88
Q

Treatment of glucocorticoid resistance

A

Dexa > 3 mg/day

89
Q

Discriminatory test cushing syn cs gc resistance

A

BMD

90
Q

Improve DM in px with cushings

A

GR antagonist mifepristone

91
Q

Common side effect of pasireotide

A

Hypereglycemia

92
Q

X linked disorder
Combined primary adrenal and
Secondary hypogonadotropic hypogonadism

A

Adrenal hypoplasia congenita

93
Q

Most common form of AI in px younger than 7 yrs

A

Adrenoleukodystrophy

94
Q

Inherited AI that manifesrs eith spastic paresus and peripheral neuropathy

A

Adrenomyeloneuropathy

95
Q

Rx adrenoleukodystrophy

A
  1. MUFA- blocj synthesis of saturated VLCFA

2 erucic acid+ oleic acid (Lorenzo’s oil)

96
Q

Presents neonatal hypoglycemia or increasing pigmentTjon

A

Familial glucocorticoid deficiency

97
Q

Type 1 variant of familial glucocorticoid deficiency

A

MCR 2

FGD variaant 2= mutations in mrap gene

98
Q

Secretion of all classes of adrenal and ovarian steroids is impaired

A

3 B hsd

99
Q

Apparent combinded
17 OH def
21 OF def

A

P 450 oxidoreductase deficiency

100
Q

Rx paeudohypoaldosteronism type I

A

Salt
Sodium bicarbonate
INDOMETHACIN or hydrochlorothiazide
(Reisstant to Gc)

101
Q

Hall mark of type III PHA

A

Reduxed GFR

102
Q

Rx primary renin deficiency

A

Fludrocortisone

K testriction

103
Q

Causes of mineralocorticoid deficiency (table)

A 
Addison diseSe 
Adrenal hypoplasia 
Congenital adrenal hyperplasia (21- Oh ams 3B hydroxysteroid dehydrogenase)
Pseudohypoaldosteronism I and II
Hyporeninemic hypoaldosteronism
104
Q

Autoimmune disease in px with autoimmune adrenal insufficiency

A

None 53%
Ovarian failure 20%
Insulin dep DM 11%

105
Q

Drugs implicated in AI primary and why

A

Ketoconazole inhibits cortisol synthesis

Rifampicin increases cortisol metab

106
Q

Pseudohypoaldosterpnism types

A

Type I- inactivating mutations in the MR; Rc is salt indomethacin HCTz

Type II- Gordon syndrome; opposite of Gitelman syndrome inc activation of thiazide sensitive sodium chloride transporter

Type III- acquired; underlying renal disease

107
Q

Tests to assess adequacy of the HPA axis

A
  • insulin tolerance
  • synacthen stimulation (tetracoside)
  • overnight metyrapone test
  • CRH stimulation test
108
Q

Give the rationale Overnight metyrapone test for AI

A

Give metyrapone ar midnight
It blocks cortisol synthesis (increasing ACTH)
Measure plasma cortisol and 11-deoxycortisol at 8 am
Normal: greater than 7 ug/L

109
Q

Distribution of causes of adrenal incidentalomas

A 
In percent:
52- adenoma
12- adrenal carcinoma
11- pheochromocytoma
5- cyst 
2- metastases
110
Q

Weight of the adrenal glands

A

4 g

111
Q

Why is pregnancy rare in cushing syndrome

A

Because of the associated amenorrhea due to androgen excess

112
Q

Rx for cushings syndrome in pregnancy, not teratogenic

A

Metyrapone

ENDOCRINE (12 decks)

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