Lecture 5 - Pituitary Disorders Flashcards

1
Q

What is the best imaging modality for looking at pituitary?

A

MRI

at T1

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2
Q

What are common disorders of the anterior pituitary?

A

pituitary tumors
hypofunction
hyperfunction

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3
Q

What are the common disorders of the posterior pituitary?

A

Diabetes insipidus

SIADH

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4
Q

What hormones are synthesized and stored in the anterior pituitary?

A
FSH 
LH
TSH
ACTH
PRL
GH
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5
Q

What hormones are stored in the posterior pituitary and where are they produced?

A

AVP
Oxytocin

produced in the hypothalamus

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6
Q

The 7 I’s

A

causes of hypopituitarism

Invasive (adenomas) 
Infarction 
Infiltrative
Injury
Immunologic
Iatrogenic
Isolated
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7
Q

Sheehan’s syndrome

A

infarction hypopiutitarism

seen in pregnancy MC

mechanism for ischemia uncertain - believed to be hypotension and vasospasm of the hypophysial arteries

extent of pituitary damage determines speed of onset of hypopituitarism: days to weeks to months

failure to lactate, post-partum menstrual irregularities

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8
Q

What infiltrating diseases can cause hypopituitarism?

A

sarcoidosis
hemochromatosis
Langerhan’s Histiocytosis

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9
Q

Lymphocytic hypophysitis

A

immunologic cause for hypopituitarism

seen during pregnancy and post-partum period

infiltration of pituitary by lymphocytes and plasma cells that destroys anterior pituitary

may result in isolated hormone deficiencies - ACTH most common

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10
Q

Gonadotropin deficiency

A

hypogonadotropic hypogonadism
Kallman’s syndrome:
defect in GnRH secretion and maldevelopment of olfactory center

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11
Q

List the hormones from early to late that become deficient in hypopiutitarism.

A
GH 
LH and FSH 
TSH
ACTH
PRL
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12
Q

What signs and sxs do you see in GH deficiency?

A

decreased muscle mass

increased fat mass

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13
Q

What signs and sxs do you see in LH and FSH deficiency?

A

amenorrhea
ED
decreased libido

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14
Q

Pituitary failure vs primary gland failure

A

pituitary failure = low levels of pituitary hormones

primary gland failure = high levels of pituitary hormones

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15
Q

Stimulation testing

A

not often used for thyroid or gonadotropin

used for GH or ACTH to determine if there is a true deficiency

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16
Q

ACTH stimulation test

A

draw baseline labs for ACTH and Cortsiol levels

inject cosyntropin (synthetic ACTH)

check cortisol levels 30 - 60 minutes later

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17
Q

If a person at baseline has low ACTH, after cosyntropin they have low cortisol and normal aldosterone, what does this mean?

A

this means central adrenal insufficiency

pituitary or hypothalamic failure

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18
Q

If a person at baseline has high ACTH, after cosyntropin they have low cortisol and low aldosterone, what does this mean?

A

primary adrenal insufficiency

adrenal gland failure

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19
Q

How do you dx isolated growth hormone deficiency in children?

A

typically noticed clinically vis length/height growth charts

then explore the possibility of systemic disease, hypothyroidism, Turner’s syndrome, or skeletal disorders

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20
Q

What percentile gets pts have evaluated for GH deficiency?

A

less than 25th percentile in height velocity

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21
Q

How do you treat ACTH deficiency?

A

glucocorticoids (PO)

hydrocortisone twice daily or prednisone daily

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22
Q

How do you treat ACTH deficiency if partial hypopituitarism?

A

may only need glucocorticoids during times of stress (like sepsis in the ICU)

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23
Q

Prolactinomas

A

prolactin - secreting pituitary adenomas

microprolactinoma <10mm (1cm) in size
- more common in women because the first sign they see is missing periods

macroprolactinoma >10mm (1cm)
- more common in men

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24
Q

What medications can you use to help shrink prolactinomas?

A

dopamine agonists (ideally D2)

  • bromocriptine (D1 and D2 receptors)
  • Cabergoline - more specific for D2 receptor
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25
Cabergoline
D2 agonist used to shrink prolactinomas long half life (can dose twice a wekk) contraindications: uncontrolled HTN or known sensitivity in the liver take at bed time, might have N/ HA, dizziness, or mental fogginess
26
Which prolactinomas are candidates for gamma knife radiotherapy?
tumors must be at least 5mm from the optic chiasm (any closer they can't have the surgery)
27
Hypopituitarism
diminished or absent secretion of >1 pituitary hormones development often slow and insidious clinical presentation depends upon etiology of hypopituitarism
28
Which is more likely to cause hypofunction, large pituitary adenomas or small pituitary adenomas?
large pituitary adenomas
29
What kinds of trauma can result in hypopituitarism?
severe head trauma physically abused children suffering closed head trauma subdural hematomas
30
What infections can cause hypopituitarism?
TB syphilis fungal infections - rare unless immunocompromised
31
Isolated anterior pituitary hormone deficiencies
GH deficiency - usually in childhood ACTH - RARE; lymphocytoic hypophysitis Gonadotropin: hypogonadotropic hypogonadism --Kallman's Syndrome TSH deficiency - rare PRL - sign of severe hypopiutitarism
32
What are the early and late hormone deficiencies seen with hypopituitarism?
early = GH late = PRL
33
What is the normal progression of hormone deficiencies in hypopituitarism?
``` GH then LH and FSH then TSH then ACTH then PRL ```
34
How can you tell the difference between pituitary failure and primary gland failure?
pituitary failure = LOW levels of TSH, ACTH, LH and FSH primary gland failure = HIGH TSH, ACTH, LH, FSH
35
When is stimulation testing used?
dx adrenal insufficiency and GH deficiency ACTH is monitored before cosyntropin is administered and then cortisol and aldosterone are measured after
36
What does it mean if ACTH is high during stimulation test, and cortisol and aldosterone are low?
primary AI (adrenal gland failure)
37
What does it mean if ACTH is low during stimulation test and cortisol is low and aldosterone is high?
Central AI (pituitary or hypothalamic failure)
38
When do you see isolated GH deficiency?
in children typically marked short stature more than 2.5 SD below the mean this dx would be excluded if a child was of normal height and bone age
39
How do you dx GHD?
growth hormone deficiency this would have to be in a child of short stature Low GH, IGF1, IGFBP3
40
ACTH deficiency
secondary d/t it being a problem with the pituitary gland itself
41
How do you treat ACTH deficiency?
glucocorticoids (PO) hydrocortisone twice daily or prednisone daily if partial hypopiutiatrism, may only need glucocorticoids during time of stress
42
How do you treat TSH deficiency?
levothyroixine PO --titrated based on FT4 and T3
43
How does the treatment of hypopituitarism change when there is concomitant adrenal insufficiency?
treat with glucocorticoid s prior to treatment with levothyroxine to avoid adrenal crisis
44
What is the treatment for gonadotropin deficiency?
women: estorgen/progesterone men: testosterone
45
What is the treatment for GHD?
hGH (sc only) | titrate based on IGF-1
46
Sheehan's Syndrome
Hypopituitarism secondary to infarction often see postpartum d/t increased aldosterone or metabolic need of pituitary during pregnancy? theories.
47
Why is the cortisol low in ACTH stimulation test fo secondary or central AI?
even though ACTH is low d/t a pituitary problem the cortisol is still low because the zona fasciculata has atrophied, so even in the presence of ACTH it won't produce cortisol (at least not at first)
48
What are 4 things you need to rule out in children with GHD?
systemic diseases hypothyroidism Turner's syndrome (girls -45X) Skeletal disorders if these are negative then assess IGF-1 and bone age (via hand xray)
49
What are early and late signs of pituitary adenomas?
early: - hypogonadism and oligomenorrhea/amenorrhea Late: -mass effect: HA, peripheral visual deficit
50
What kind of visual deficits might you see with pituitary adenoma?
bitempotal hemianopsia
51
Prolactinoma
prolactin secreting pituitary adenoma
52
Microprolactinoma
<10 mm in size MC in women PRL level correlates with size or prolactinoma
53
Macroprolactinoma
>10mm in size MC in men PRL level correlates with size or prolactinoma
54
How does a pt with prolactinoma present?
``` menstrual irregularities galactorrhea visual field defects HA libido potency failure ```
55
What is the main medical treatment for prolactinomas?
Dopamine dopamine inhibits PRL secretion Bromocriptine - D1 and D2 receptor agonists Cabergoline - D2 receptor agonist --LONG half life, taken twice a week, take at bedtime
56
What are the non medicine treatments for prolactinomas?
typically if the tumor has become resistant to dopamine transsphenoidal surgery Gamma knife radiotherpay (only if tumor in >5mm from optic chiasm)
57
If you want to do gamma knife for a pituitary adenoma, what must you make sure?
that the tumor is at least 5mm from the adenoma
58
Post removal of a pituitary adenoma, what must be checked?
pituitary function must be assessed annually
59
Acromegaly
excess GH in adults overgrowth of bones: skill, mandible mean age 40 years sxs usually 5-10 years prior to dx
60
Gigantism
excess GH in children long bones affected
61
How do you dx acromegaly?
IGF1 - since you cant measure GH d/t it changing so much throughout the day OGTT - oral glucose tolerance test
62
OGTT
oral glucose tolerance test done for acromegaly dx Glucose and GH are indirectly related given glucose and then measure GH in 1 and 2 hours for a normal person GH would be low for acromegaly or excess GH in general it does not suppress in the presence of glucose
63
When do you get imaging for acromegaly?
AFTER biochemical confirmation to determine etiology of GH excess majority of cases pituitary adenoma >1cm
64
What is the treatment for acromegaly?
transsphenoidal surgery -80-90% success in those with tumors <1cm large tumors may require medical therapy Somatostatin --inhibit GH secretion Pegvisomant - GH receptor antagonist Gamma knife radiotherapy
65
What kind of long term follow up is required for acromegaly pts?
colonoscopy every 3-4 years d/t increased risk of colon polyps CV eval regularly IGF1, OGTT follow tumor size via MRI yearly
66
What is the criteria for cure with acromegaly?
Fasting GH very low Glucose suppressed GH very low Normal IGF1 (idk why this would be normal if it is stimulated by GH in the liver?)
67
Cushings Disease
pituitary adenoma secreting ACTH typically <10mm
68
What is the dx for Cushings Disease?
``` 24 hour urine free cortisol or late night salivary cortisol or low dose dexamethasone suppression test ```
69
What is the treatment for Cushings disease?
transsphenoidal surgery
70
If you have a gonadotropin secreting adenoma, which hormone is it producing more of?
FSH > LH
71
What is associated with MEN 1 but not MEN 2?
pituitary adenomas are only seen in MEN 1 ``` 3 Ps Pituitary adenomas (prolactinoma, GH) Parathyroid adenomas (or hyperplasia) Pancreatic tumors (gastrinoma, insulinoma) ```
72
Pituitary apoplexy
hypopituitarism d/t hemorrhagic necrosis of a tumor of pituitary gland infarction endocrine emergency
73
What are the clinical sxs of pituitary apoplexy?
``` sudden onset of frontal HA visual acuity and field loss ophthalmoplegia AMS N/V fever hypotension ```
74
What is the treatment for pituitary apoplexy?
IV dexamethason Transphenoidal surgery
75
What are causes of central DI?
AVP deficiency Spontaneous DI - infiltrative disease - lymphocytic hypophysitis - head trauma - neurosurgery
76
How do pts with central DI present?
cannot go long without water cant leave home without water frequent urination throught day and night
77
What is the treatment for central DI?
replace fluids and DDVAP (desmopressin)
78
SIADH
elevated urine sodium excretion while on normal salt and water intake hypoosmolality of ECF
79
What are the causes of SIADH?
``` tumors CNS disorders drug-induced pulmonary diseases prolonged exercise (marathon running) ```
80
What is the treatment for SIADH?
fluid restriction
81
How do you dx prolactinoma?
MRI
82
Which prolactinomas get surgery?
macro
83
ADH and aldosterone
ADH --H2O retention Aldosterone - Na+ reabsorption