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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
The urinary free cortisol is ___% of the total cortisol
1%
26
Half life of circulating cortisol
70-120 mins
27
What are the 2 isoenzymes of 11B HSD
Type 1 NADPH dependent | Type 2 Nad dependent
28
Conversion of cortisol to cortisone in kidney
HSD 11B 2 11 B hydroxydehydrogenase type 2
29
Hsd that inactivates cortisol to cortisone and permits aldosterone to bind to the MR jn vivo
Type 2
30
Type of 11 B HSD | That converts cortisone to cortisol (all oral steroid)
Type 1
31
Effect of GC on bone
Inhibit osteoblast function | Inhibits intestinal calcium absorption
32
How do Glucocorticoids cause hypertension
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
33
Mineralocorticoid activity of dexamethasone
0
34
Affinity of synthetic steroids to COrtisol binding globulin
Low. Most circulate as free steroid or bound to albumin
35
% of patients with cushings syndrome with hypertension
75%
36
Effect if steroids to eye
Raised intraocular pressure and exophthalmos
37
Diagnosis of Cushings Syndrome | Rationale for midnight cortisol
Nadir of cortisol midnight lost in patients with Cushings syndrome Level greater than 200 nmol/L >7.5 ug/dL
38
Diagnosis of cushings daluvary cortisol
>2 ng/mL | 5.5 nmol/L
39
Inferior petrosal sinus sampling rationale
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
40
Diagnostic of cushings: IPSS with CRH administration
Acth in petrosal sinus/peripheral ratio greater than 3
41
Used in some centers in lieu od CRH (during IPSS)
Desmopressin
42
How to check if hydrocortisone can be discontinued | Omitting HCT dose in am and check for cortisol
<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
43
% pf normal aubjects with adrenal incodentalomas
5%
44
Drugs medical treatment of cushings syndrome
Metyrapone Ketoconazole Mitotane Somatostatin analogues octreotide and lanreotide
45
Discriminatory test Glucocorticoid resistance versus cushings
Bone mineral densitometry= preserved in patients with glucocorticoid resistance
46
Most common cause of primary adrenal insufficiency in the western world
Autoimmune adrenalitis
47
Most common cause of primary adrenal insufficiency in the world
Infection
48
Most common sources of adrenal metastases
Lung/ breast tumors
49
Anticipare adrenal atrophy in what steroid dose
30 mg HcT more than 3 weeks >7.5 mg pred >0.75 mg dexa
50
A basal cortisol value more than ____ denotes an intact HPA axis
400 nmol/L | 14.5 ug/dL
51
Tests to asses adequacy of HPA axis aside from SST
``` - 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
Why does pregnancy necessitate jncreasing doses of fludrocortisone in parients with primary AI
Because progesterone is a mineralocorticoid antagonist
53
Congenital lipoid adrwnal hyperplasia
STAR DEFICIENCY Failure of transport of cholesterol from outer to inner mitochondrial membrane Pregnenolone synthesis is abolished
54
Same manifestation as STAR deficiency but do not have enlared adrenals
p450 side xhain cleavage deficiency
55
Mechanism of pseudohypoaldosteronism
Defective renal tubular response to mineralocorticoid in infancy
56
Treatment of Pseudohypoaldosteronism type I
Supplementation with salt 2-8 g/day (Resistant to mineralocorticoid therapy)
57
Treatment for hgpercalciuria and hypercalcemia in pseudohypoaldosteronism
Indomethacin | Hydrochlorothoazide
58
Converts cortisol to cortisone
Hsd B 2
59
Converts cortisone to cortisol
11 HSD B 1 | Cortisone reductase
60
Predominant cathecolamine in pheo MEN2
Epinephrine
61
Syndromic forma of pheochromocytoma
``` MEN2A MEN 2B Von hippel lindau disease NF1 Carney triad/ syndrome ```
62
VHL type at risk of developing pheo
VH2
63
What is the carney triad
GIST Pulmonary chondromS Cathecolamine secreting paraganglioma
64
In SDH missense mutations occured frequently except in
SDHD
65
Cortisol to creatinine ratio cut off for cushings
25
66
Inhibits both uptake and storAge f MIBG
Labetalol
67
Inhibits both uptake and taansport of MIBG
Reserpint
68
Aloha blockade prior to OR of pheo
Phenoxybenzamines 10-20 mg | 7-10 days pre op
69
Target for alpha blockade
SBP > 90 | BP 120/80
70
Beta blockade
Trget 60-80 bpm
71
Intraop HTH
Phentolamjbe Nitroprusside Nicardipine
72
Avoid anesthetic
-ine and -ane Fentanyl ...
73
Recurrent pheo
Runs in family Right sided paRaganglioma
74
Highest concentration of MC receptors are found in:
Distal nephron Colon Hippocampus
75
(+) results for primary hyperaldosteronism screening
pac >15 ng/dL Pra <1.0 Pac/pra >20 ng/dL
76
(+) result of oral sodium loading | 5000 mg Na for 3 days
Urine aldosterone > 12 ug/24h
77
(+) result saline infusion test
Post infusion PAC > 10 ng/dL
78
(+) result fludrocortisone suppression test
(+) result PAC > 6 ng/dL
79
Test for confirmation of hyperaldosterinisn
F I O
80
Which of the ff xgaracteristic prjnary aldosteronism vfrom iha conpared to Apa
Less HTB Less hyookalemia Older age
81
When to Do repeat pac after apa surgey
1-2 days
82
Recommended post op to avoid hyperK due to chronic suppression tRAAS
High sodium
83
Result of impaired activity of the microsomal enzyme HSD 11B2
Apparent mineralocorticoid excess
84
Adrenal mets mostly grom
Lung | Breast
85
Infection with intra aftenal bleeding in children
Psudomonas
86
APS Type 2
Addisons Autoimmune thyroid disease DM Hypogonadism (+) abx to 21 hydroxylase
87
APS type 1
Apeced Addison Hypoparathyroidis. Chronic mucocutaneous candidiasis
88
Treatment of glucocorticoid resistance
Dexa > 3 mg/day
89
Discriminatory test cushing syn cs gc resistance
BMD
90
Improve DM in px with cushings
GR antagonist mifepristone
91
Common side effect of pasireotide
Hypereglycemia
92
X linked disorder Combined primary adrenal and Secondary hypogonadotropic hypogonadism
Adrenal hypoplasia congenita
93
Most common form of AI in px younger than 7 yrs
Adrenoleukodystrophy
94
Inherited AI that manifesrs eith spastic paresus and peripheral neuropathy
Adrenomyeloneuropathy
95
Rx adrenoleukodystrophy
1. MUFA- blocj synthesis of saturated VLCFA | 2 erucic acid+ oleic acid (Lorenzo’s oil)
96
Presents neonatal hypoglycemia or increasing pigmentTjon
Familial glucocorticoid deficiency
97
Type 1 variant of familial glucocorticoid deficiency
MCR 2 FGD variaant 2= mutations in mrap gene
98
Secretion of all classes of adrenal and ovarian steroids is impaired
3 B hsd
99
Apparent combinded 17 OH def 21 OF def
P 450 oxidoreductase deficiency
100
Rx paeudohypoaldosteronism type I
Salt Sodium bicarbonate INDOMETHACIN or hydrochlorothiazide (Reisstant to Gc)
101
Hall mark of type III PHA
Reduxed GFR
102
Rx primary renin deficiency
Fludrocortisone | K testriction
103
Causes of mineralocorticoid deficiency (table)
``` Addison diseSe Adrenal hypoplasia Congenital adrenal hyperplasia (21- Oh ams 3B hydroxysteroid dehydrogenase) Pseudohypoaldosteronism I and II Hyporeninemic hypoaldosteronism ```
104
Autoimmune disease in px with autoimmune adrenal insufficiency
None 53% Ovarian failure 20% Insulin dep DM 11%
105
Drugs implicated in AI primary and why
Ketoconazole inhibits cortisol synthesis | Rifampicin increases cortisol metab
106
Pseudohypoaldosterpnism types
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
Tests to assess adequacy of the HPA axis
- insulin tolerance - synacthen stimulation (tetracoside) - overnight metyrapone test - CRH stimulation test
108
Give the rationale Overnight metyrapone test for AI
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
Distribution of causes of adrenal incidentalomas
``` In percent: 52- adenoma 12- adrenal carcinoma 11- pheochromocytoma 5- cyst 2- metastases ```
110
Weight of the adrenal glands
4 g
111
Why is pregnancy rare in cushing syndrome
Because of the associated amenorrhea due to androgen excess
112
Rx for cushings syndrome in pregnancy, not teratogenic
Metyrapone

ENDOCRINE (12 decks)

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