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
Q

Cabergoline

A

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

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26
Q

Which prolactinomas are candidates for gamma knife radiotherapy?

A

tumors must be at least 5mm from the optic chiasm (any closer they can’t have the surgery)

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27
Q

Hypopituitarism

A

diminished or absent secretion of >1 pituitary hormones
development often slow and insidious
clinical presentation depends upon etiology of hypopituitarism

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28
Q

Which is more likely to cause hypofunction, large pituitary adenomas or small pituitary adenomas?

A

large pituitary adenomas

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29
Q

What kinds of trauma can result in hypopituitarism?

A

severe head trauma
physically abused children suffering closed head trauma
subdural hematomas

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30
Q

What infections can cause hypopituitarism?

A

TB
syphilis
fungal infections - rare unless immunocompromised

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31
Q

Isolated anterior pituitary hormone deficiencies

A

GH deficiency - usually in childhood

ACTH - RARE; lymphocytoic hypophysitis

Gonadotropin: hypogonadotropic hypogonadism –Kallman’s Syndrome

TSH deficiency - rare

PRL - sign of severe hypopiutitarism

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32
Q

What are the early and late hormone deficiencies seen with hypopituitarism?

A

early = GH

late = PRL

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33
Q

What is the normal progression of hormone deficiencies in hypopituitarism?

A
GH 
then 
LH and FSH 
then 
TSH 
then 
ACTH
then 
PRL
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34
Q

How can you tell the difference between pituitary failure and primary gland failure?

A

pituitary failure = LOW levels of TSH, ACTH, LH and FSH

primary gland failure = HIGH TSH, ACTH, LH, FSH

35
Q

When is stimulation testing used?

A

dx adrenal insufficiency and GH deficiency

ACTH is monitored before
cosyntropin is administered and then cortisol and aldosterone are measured after

36
Q

What does it mean if ACTH is high during stimulation test, and cortisol and aldosterone are low?

A

primary AI (adrenal gland failure)

37
Q

What does it mean if ACTH is low during stimulation test and cortisol is low and aldosterone is high?

A

Central AI (pituitary or hypothalamic failure)

38
Q

When do you see isolated GH deficiency?

A

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
Q

How do you dx GHD?

A

growth hormone deficiency

this would have to be in a child of short stature

Low GH, IGF1, IGFBP3

40
Q

ACTH deficiency

A

secondary d/t it being a problem with the pituitary gland itself

41
Q

How do you treat ACTH deficiency?

A

glucocorticoids (PO)
hydrocortisone twice daily or prednisone daily

if partial hypopiutiatrism, may only need glucocorticoids during time of stress

42
Q

How do you treat TSH deficiency?

A

levothyroixine PO –titrated based on FT4 and T3

43
Q

How does the treatment of hypopituitarism change when there is concomitant adrenal insufficiency?

A

treat with glucocorticoid s prior to treatment with levothyroxine to avoid adrenal crisis

44
Q

What is the treatment for gonadotropin deficiency?

A

women: estorgen/progesterone

men:
testosterone

45
Q

What is the treatment for GHD?

A

hGH (sc only)

titrate based on IGF-1

46
Q

Sheehan’s Syndrome

A

Hypopituitarism secondary to infarction

often see postpartum d/t increased aldosterone or metabolic need of pituitary during pregnancy? theories.

47
Q

Why is the cortisol low in ACTH stimulation test fo secondary or central AI?

A

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
Q

What are 4 things you need to rule out in children with GHD?

A

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
Q

What are early and late signs of pituitary adenomas?

A

early:
- hypogonadism and oligomenorrhea/amenorrhea

Late:
-mass effect: HA, peripheral visual deficit

50
Q

What kind of visual deficits might you see with pituitary adenoma?

A

bitempotal hemianopsia

51
Q

Prolactinoma

A

prolactin secreting pituitary adenoma

52
Q

Microprolactinoma

A

<10 mm in size
MC in women

PRL level correlates with size or prolactinoma

53
Q

Macroprolactinoma

A

> 10mm in size
MC in men

PRL level correlates with size or prolactinoma

54
Q

How does a pt with prolactinoma present?

A
menstrual irregularities 
galactorrhea 
visual field defects 
HA
libido potency failure
55
Q

What is the main medical treatment for prolactinomas?

A

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
Q

What are the non medicine treatments for prolactinomas?

A

typically if the tumor has become resistant to dopamine

transsphenoidal surgery

Gamma knife radiotherpay (only if tumor in >5mm from optic chiasm)

57
Q

If you want to do gamma knife for a pituitary adenoma, what must you make sure?

A

that the tumor is at least 5mm from the adenoma

58
Q

Post removal of a pituitary adenoma, what must be checked?

A

pituitary function must be assessed annually

59
Q

Acromegaly

A

excess GH in adults

overgrowth of bones: skill, mandible

mean age 40 years
sxs usually 5-10 years prior to dx

60
Q

Gigantism

A

excess GH in children

long bones affected

61
Q

How do you dx acromegaly?

A

IGF1 - since you cant measure GH d/t it changing so much throughout the day

OGTT - oral glucose tolerance test

62
Q

OGTT

A

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
Q

When do you get imaging for acromegaly?

A

AFTER biochemical confirmation to determine etiology of GH excess

majority of cases pituitary adenoma >1cm

64
Q

What is the treatment for acromegaly?

A

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
Q

What kind of long term follow up is required for acromegaly pts?

A

colonoscopy every 3-4 years d/t increased risk of colon polyps

CV eval regularly

IGF1, OGTT

follow tumor size via MRI yearly

66
Q

What is the criteria for cure with acromegaly?

A

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
Q

Cushings Disease

A

pituitary adenoma secreting ACTH

typically <10mm

68
Q

What is the dx for Cushings Disease?

A
24 hour urine free cortisol 
or 
late night salivary cortisol 
or 
low dose dexamethasone suppression test
69
Q

What is the treatment for Cushings disease?

A

transsphenoidal surgery

70
Q

If you have a gonadotropin secreting adenoma, which hormone is it producing more of?

A

FSH > LH

71
Q

What is associated with MEN 1 but not MEN 2?

A

pituitary adenomas are only seen in MEN 1

3 Ps 
Pituitary adenomas (prolactinoma, GH) 
Parathyroid adenomas (or hyperplasia) 
Pancreatic tumors (gastrinoma, insulinoma)
72
Q

Pituitary apoplexy

A

hypopituitarism d/t hemorrhagic necrosis of a tumor of pituitary gland infarction

endocrine emergency

73
Q

What are the clinical sxs of pituitary apoplexy?

A
sudden onset of frontal HA 
visual acuity and field loss 
ophthalmoplegia 
AMS
N/V
fever
hypotension
74
Q

What is the treatment for pituitary apoplexy?

A

IV dexamethason

Transphenoidal surgery

75
Q

What are causes of central DI?

A

AVP deficiency

Spontaneous DI

  • infiltrative disease
  • lymphocytic hypophysitis
  • head trauma
  • neurosurgery
76
Q

How do pts with central DI present?

A

cannot go long without water
cant leave home without water
frequent urination throught day and night

77
Q

What is the treatment for central DI?

A

replace fluids and DDVAP (desmopressin)

78
Q

SIADH

A

elevated urine sodium excretion while on normal salt and water intake

hypoosmolality of ECF

79
Q

What are the causes of SIADH?

A
tumors 
CNS disorders
drug-induced
pulmonary diseases 
prolonged exercise (marathon running)
80
Q

What is the treatment for SIADH?

A

fluid restriction

81
Q

How do you dx prolactinoma?

A

MRI

82
Q

Which prolactinomas get surgery?

A

macro

83
Q

ADH and aldosterone

A

ADH –H2O retention

Aldosterone - Na+ reabsorption