Newman Endo Repro Flashcards

(100 cards)

1
Q

List primary symtpoms primary adrenal insuffiency

A

Ok

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2
Q

What space are kidneys and adrenals

A

Retroperitoneum

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3
Q

Short term stress

A

Catecholamines, NE, E

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4
Q

Long term stree

A

Glucocorticoids mineralocorticoids

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5
Q

GFR

A

Glomerulosclerosis, fasciculata, reticularis

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6
Q

Salty sweet spiced

A

Mineralocorticoids, glucocorticoids, sex steroids

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7
Q

Describe HPA axis cortisol

A

Hypothalamus release CRH to anterior pituitary

Anterior pituitary send acth to adrenal cortex

Adrenal cortex makes cortisol which sends negative feedback to anterior pituitary and hypothalamus

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8
Q

RAAS

A

Renin angiotensin aldosterone system

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9
Q

Aldosterone effect

A

Na retention

K secretion

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10
Q

Baby in looked good in nursery and feeding ok. Comes in day 6 thrown up but looks dehydrated, very dry mucous membranes(signs in baby—dry mucous membranes, no tears, anterior fontanells) bp 60/30, sluggish weak cry

Find a female genetalia, looks like a penis? Masculine , clitoramegonly, maasculinzation of female genetalia
VIRILIZED

A

Virilized, low bp, dehydration

21 hydroxylase defiency
Congenital adrenal hyperplasia

Lack of aldosterone

Lack of cortisol and aldosterone…flow over to DHEA< androstenedione, and testerostone

Lose na lose water

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11
Q

Lab work CAH

A

Na low, K high, Cl low, bicarb low, BUN high, glucose low

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12
Q

Why girl better CAH

A

Girl genital show virilization

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13
Q

Why glucose low

A

Cortisol stimulate gluconeogenesis (something insulin)

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14
Q

What order

A

17 a OH

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15
Q

Treat CAH

A

Hydroxycortisone, fluids/glucose, get K level down

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16
Q

Primary adrenal insuffiency

A

Autoimmune (most common cause in US)

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17
Q

Most common infectious cause primary adrenal insuffiency

A

TB-SEE CALCIFICATIONS ON MRI

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18
Q

X linked recessive disorder of long chain FA metabolism

A

Mimic MS

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19
Q

Waterhouse friedreich Sean syndrome

A

Bilateral adrenal hemorrhage

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20
Q

ACTH darkening of the skin

A

MSH of PMC

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21
Q

Inherited adrenal unresponsiveness to ACTH

A

Familial glucocorticoid defiency (AR)

Increase acth causes skin pigmentation

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22
Q

Triple A

A

Rare AR adrenal gland no respond to ACTH so ACTH so high

Mutation int he AAAAS gene that codes for the protein ALADIN

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23
Q

Congenital adrenal hyperplasia

A

21 hydroxylase defiency most common

Salt wasting

Defiency cortisol and aldosterone

Excessive androgens

Sometimes called Addison in older children/adulthood

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24
Q

Adrenal insuffiency how do they present

A

Fatigue, reduced stamina, weakness, anorexia, weight loss, skin hyperpigmentation

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25
What do patients complain of with primary adrenal insuffiency
Abdominal pain, nausea, vomiting, msk pain (joint, muscles, chest, back), psychiatric (depression, anxiety, irritability), HA, vomiting, gait disturbance, salt craving and orthostatic light headedness, BP is usually low and orthostatic
26
Female infant with 21 hydroxylase defiency will have virilized genetalia
Ok
27
Labs primary adrenal insuffiency
Na low, K high, low blood sugar, maybe hypercalcemia, plasma cortisol ***
28
Low 8 am plasma cortisol (low morning high night) and do ACTH level and its high
Primary adrenal insuffiency
29
Confirmatory test
Cosyntropin (synthetic acth) stimulation test
30
If cortisol doesn’t respond to acth
Confirm primary corticoinsuffiency
31
Serum antibodies to 21 hydroxylase
Confirm autoimmmune Addison
32
Lab to see 21 hydroxylase defiency
Elevated serum levels of 17 oh progesterone are required
33
Plasma renin high primary adrenal insuffiency
Sense hypovolemia
34
Treat primary adrenal insuffiency
Glucocorticoids replacement therapy, mineralocorticoids therapy
35
Acute adrenal crisis symptoms
Ab pain Confusion Dehydration Dizziness Fatigue High fever (
36
Labs acute adrenal crisis
ACTH stimulation test, cortisol blood sugar, K , serum Na, serum pH (
37
Secondary adrenal insuffiency
Low serum ACTH in the setting of low serum cortisol is the give away
38
32 year old purple tiger stripes on abdomen , high BP, periods irregular, crappy feeling, hump back
Striae, Cushing
39
Cushing
Excessive corticosteroids Vague weird symptoms
40
ACTH dependent cushing
Cushing disease (ACTH secretory adenoma)
41
ACTH independent cushing syndrome
Exogenous corticosteroid use (most common cause) | Adrenocortical tumor
42
Mccune albright syndrome
Polycyotic fibrous dysplasia, cafe at lair, endocrine hyperfunction from multiple organ)
43
Extopic acth
AAC of lung
44
Cushing disease
Pituitary adenoma
45
Cushing syndrome
Ectopic acth | SSC of lung
46
Dexamasone test
Draw serum acth before
47
ACTH lelvated in hundred, cortisol not suppressed by high or low doses
Ectopic acth syndrome is likely
48
Undetectable or low acth, cortisol not suppressed by high or low doses
Primary hypercortisolism is likely (not driven by acth, possible adrenal tumor)
49
Normal to elevated acth, cortisol not suppressed by low but suppressed by IgG
Cushing disease (pituitary still retains some feedback control)
50
Incidentalomas
Accidentally find nodule in adrenal >4 cm have to do work up and makes sure not malignant with CT and MRI
51
All patients with adrenal incidentaloma require testing for _____. How
Pheochromocytoma Plasma metanephrines Urine collection
52
Primary aldosteronism
HTN that is resistant and descent respond to three drugs think of this
53
Conn syndrome
Primary aldosteronism secondary to a unilateral aldosterone-producing adrenal adenoma 40% have a mutation in a gene coding for the K channel
54
Signs symtpoms primary aldosteronism
HTN and hypokalemia
55
Lab finding primary aldosteronism
Metabolic alkalosis (proton lows, H with K)
56
Pheochromocytoma
VHL type 2 AD Get it Bp high, sweating,
57
Lab pheochromocytoma
Plasma fractioned free metanephrines is the single msot sensitive test for secretory pheochromocytoma and paragangliomas
58
Treating suspected pheochromocytoma
Alpha blocker before beta blocker otherwise bp can go up
59
MEN
AD Have tumours in 2 or more different endocrine glands
60
Growth curve PEDS : how measure length
Laying down
61
Measure height or stature
Standing up
62
Over 36 months
Standing up
63
Normal BMI kids
Percentile
64
Normal weight kids
Greater than or equal to 5th percentile and less than 85th percentile
65
Normal weight adult
BMI 18,5-24.9
66
6 here’s of age till puberty growth per year
2 inches a year
67
Mid parental height
Genetic potential ...estimate of genetic potential for girls and boys different
68
Weight falls off first
Think nutrition Not getting enough or processing enough Higher than average caloric requirements
69
Length fallow off first
Think endocrine GH defiency, hypothyroidism, cushing syndrome (iatrogenic)
70
Head circumference falls off first
Primary failure of the brain to grow | Severe craniosynstosis
71
Bone age
Provides a rough estimate of a kids skeletal maturation by assessing the ossification of the epiphyseal centers Bone age is 2 SD below the chronological age is considered delayed
72
What bone age is considered delayed
2 SD
73
Abnormal growth
A comprehensive history and physical is essential in developing the differential diagnosis and guiding the work up
74
What most important of work up of kid not growing well
History and physical
75
Evaluating growth. How many points on the growth curve
More better
76
Get as many points on growth curve as you can
Ok
77
Catch up or catch down
First 18 months of life plotting kids make 25% downward adjustment as go from Abby food to solid food. They’ll catch p but a lot make a downward adjustment.
78
Constitutional growth delay
Late bloomer, few months big growth Grow grow grow, slow down then catch back up
79
End height if have precocious puberty
Shorter than normal. Growth plates mature cant grow anymore Grow really fast when dont expect then off Familial -come form land of little people
80
Cushing curve
Gaining weight, but height not up up
81
Short stature
Height 2SD less than the 3rd percentile (mean height for age and sex) Height more than 2SD below the mid parental height
82
Intima work up for concerning short stature and not explained by constitutional, familial, and idiopathic
IGF and IGFBP-3 levels
83
IGF and IGFBP-3 levels
Insulin like growth factor
84
Turner
45 XO with delayed puberty/short stature in females always check a karyotyoe Web neck Wide nipples
85
Lab work turner0short stature
Check karyotype and see if 45 XO
86
Precocious puberty less than 8 years old girls and 9 in men
Full activation of HPG axis before 8 years of age in girls and before 9 years of age in boys
87
Consider precocious puberty in girls
Progressive breast development Crossing major percentile lines upward on the linear growth chart
88
Consider precocious puberty in boys if
Evidence of both testicular and penile enlargement Crossing major percentile lines upward not he linear growth chart
89
Bone age precocious puberty
Advanced bone age
90
Premature adrenarche
Increase in DHEA and its storage from DHEA-S Pubic hair, axillary hair ,odor, sometimes acne
91
Straight thin hair
No worry
92
Curly thick hair and other secondary sexual characteristics of growth sky rocketing
Work up look at growth look at other sexual characteristics
93
Only thin little pubic hair
Fine
94
Premature thelarche
Breast early Usually feel bud in kids just watch If related to acceleration work up as precocious puberty
95
Hypothyroidism
Increase BMI from growth arrest with continued weight gain Family history always watch
96
Tall stature
Beckwith-wiedemann syndrome(big head wen young, normal adult height) Insulin like growth factor 2
97
Homocystinuria
Infant of diabetic mother Big when little
98
Klinefelter syndrome
47 XXY
99
Marian
AD Connective tissue Fibrillin-1 gene mutation genetic consultation Dissecting aortic aneurysm
100
When refer growth
Less than 3rd percentile or greater 95th and continues to rise or drop Increase growth velocity between schoool age and puberty Growth potential: projected height varies from midparental height by more than 2 inches Multiple dystrophic features send to geneticist Bone age advanced of delayed by more the 2 SF