disorders of the hypothalamus and pituitary axis Flashcards

1
Q

CRH

A

corticotrophin- releasing hormone

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

GHIH

A

growth hormone inhibiting hormone

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

PIH

A

prolactin inhibiting hormone

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

MSHIH

A

melanocyte-stimulating homrone inhibiting hromone

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

GnRH

A

gonadotropin releasing homrone

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

TRH

A

thyrotophin releasing hormone

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

ACTH

A

adrenocorticostopic hormone

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

TSH

A

thyroid stimulating hormone

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

GH

A

growth hormone

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

PRL

A

prolactin

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

TSH

A

follicule-stimulating hormone

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

LH

A

leuteinizing hormone

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

MSH

A

melanocyte stimulating hormone

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

ADH

A

antidiuretic hormone

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

what are products of the anterior pituitary

A

ACTH
TSH
GH
PRL
FSH
LH
MSH

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

What are the products of the posterior pituitary

A

ADH
oxytocin

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

what is hypopituitarism

A

low functioning pituitary - either problem with the hypothalaus upstream or pituitary gland itself

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

what are casues of hypopituitarism

A

primarily due to hypothalamus or pituitary lesions - pituitary adenoma
can be inherited
damage from trauma, infection, vascular disorder
infiltrative disorders (hemochromatosis, sarcoidosis, amyloidosis)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

what is pituitary adenoma

A

average age 30-40; mostly benign; can be functioning or non-functioning
prolactin-secreting adenomas (prolactinomas) m/c**

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

what is the presentation of hypopituitarism

A

headaches or visual changes (mass effect)
non-specific: fatigue, dizziness, hypotension, confusion, cognitive dysfunction, sexual dysfunction, polydipsia, cold intolerance

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

What is the typical order of hormone disruption with hypopituitarism

A

GH -> FSH/LH -> TSH -> ACTH

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

what is seen with GH deficiency

A

short stature, micropenis, central obesity, hyperlipidemia, reduced muscle/bone mass, CV dysfunction

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

What is seen with Gonatotropin deficiency

A

FSH and LH deficiency
hypogonadism and infertility, impaired/absent puberty, sexual dysfunction, loss of male secondary sex characteristics

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

what is seen with TSH deficiency

A

hypothyroidism

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

what is seen iwth ACTH deficiency

A

adrenal insufficiency

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

what is seen with prolactin deficiency

A

impaired or absent lactation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q

what is the workup of hypopituitarism

A

eval of hormone levels or downstream effects:
eval of GH levels - stimulation testing
adrenal deficiency testing (low serum cortison, ACTH, DHEA, ACTH with cosynotropin stimulation)
MRI of hypothalamus/pituitary to assess for mass

28
Q

what is the treatment for hypopituitarism

A

resection of tumor/mass
dopamine agonist first line for prolactinomas (bromocriptine or cabergoline)
radiation last line
lifetime hormone replacement therapy

29
Q

What is the prognosis for hypopituitarism

A

increased mortality risk overall - especially women who are younger
mortality risk due to risk for adrenal crisis
risk for local invasion of some pituitary tumors
if due to radiation, risk for other intracranial neoplasms or stroke

30
Q

what is the first line treatment for prolactinomas

A

dopamine agonists
bromocriptine or cabergoline

31
Q

what is the glucocorticoid replacement

A

oral hydrocortisone 10-20mg in AM and 5-15mg in afternoon
titrate to patient
watch for neutropenia/lymphopenia
Stress dosing PRN during physical illness

32
Q

who is not to taken hGH

A

during pregnancy or while using oral estrogen therapy

33
Q

What are pituitary adenomas

A

benign tumors arising from any one of the pituitary cell types
makeup about 15% of intracranial neoplasms
most common cause of hypo- and hyperpituitarism in adults

34
Q

what are the classifications of pituitary adenomas

A

micro-(<1cm diameter) verus macroadenomas (>1cm diameter - mass effect - hypopiutitarism)
functioning (secretion of hormone - hyperpituitatarism) vs non-functioning

35
Q

what are the different types of presentation of pituitary adenomas

A

symptoms related to mass effect (HA, visual changes)
symptoms related to pituitary hormone imbalance
- hyperfunctioning from tumor secretion
-hypofunctioning due to mass effect/destruction

36
Q

what different origin of tumors are there

A

Acidophils - growth hormone and prolactin
Basophile - gonadotrophs, ACTH and TSH

37
Q

what is the test of choice for pituitary adenomas

A

MRI with gadolinium
adjunctive CT if suspected bony invasion or to look for calcifications

38
Q

what is the workup for pituitary adenomas

A

MRI
secondary hormone dysfunction workup

39
Q

what is the treatment of incidental pituitary adenoma tumors

A

no homrone secretion and small size - monitoring with annual MRI

40
Q

what is the treatment for macroadenomas (pituitary adenomas)

A

resection due to risk of infiltration, mass effect
transsphenoidal approach preferred
+/- adjunctive radiation

41
Q

what is the treatment of functional tumors (pituitary adenomas)

A

medical therapys available - somatostatin and dopamine inhibit
prolactinomas - dopamine agonists
acromegaly - somatostatin analogues, GH receptor antagonists
TSH secretion - somatostatin analogues +/- dopamine agonist
ACTH secretion - +/- somatostatin anaolgues

42
Q

What is hyperprolacinemia

A

most common pituitary hormone hypersecretion syndrome
many different causes: physiologic, pharmacologic, pathologic
prolactin secreting pituitary tumor (prolactinomas): W>M, 30-40 yo

43
Q

what is the presentation of hyperprolactinemias in males

A

infertility
decreased libido
erectile dysfunction
gynecomastia
decreased muscle/bone mass
decreased facial/body hair
depression
hot flashes

44
Q

what is the presentation of hyperprolactinemias in females

A

amenorrhea
oligomenorrhea
infertility
vaginal dryness
irritability
anxiety/depression

45
Q

what are miscellaneous presentations of hyperprolactinemias

A

acromegaly
HA
visual disturbances
other pituitary deficiencies

46
Q

what is the workup for hyperprolactinemia

A

prolactin level
eval underling cause: pregnancy, hypothyroidism, CKD, cirrhosis, hyperparathyroidism
evaluate hypogonadism
Pituitary MRI

47
Q

what is the treatment for hyperprolactinemia

A

stop medications known to increase PRL levels
correct hypothyroidism with thyroxine
oral contraceptive or estrogen replacement to treat infertility
increased surveillance during pregnancy
dopamine therapy
resection
radiation
chemotherapy

48
Q

what is the treatment for large prolactinomas or symptomatic prolactinomas

A

dopamine agonists (carbergoline best tolerated)

49
Q

What is gigantism

A

excessive growth in childhood, prior to physeal closure (impacts long bones)

50
Q

what is acromegaly

A

excessive growth AFTER physeal closure (no long bone involvement)

51
Q

What are physical symptoms of gigantism and acromegaly

A

tall stature
delayed puberty
enlargement of hands, feet and digits
soft, doughy handshake
moist handshake
prominent underbite
enlargement of skull
excessive sweating
tooth space widens
enlarged forehead
enlarged tongue, nose and lips

52
Q

what are other associated symptoms with gigantism and acromegaly

A

CTS
arthritis
HA
DM
HTN
HD
deepening of voice
spinal stenosis
skin changes
colon polyps
hypogonadism
secondary hypothyroidism
OSA

53
Q

how do you work up acromegaly/gigantism

A

random serum IGF-1 (elevated)
100g glucose drink, measure GH 60 min later - normal r/o acro
prolactin level
glucose
LFTs, BUN, creatinine
serum calcium
Free T4 and TSH
imaging: MRI - skull radiographs/hand and foot xr

54
Q

what is the treatment of choice for acromegaly/gigantism

A

transsphenoidal resection
if remission not achieved with surgery - radiation or add adjunctive medcations

55
Q

What is achondroplasia

A

dwarfism
autosomal dominant: fibroblast growth factor receptor 3 (FGFR3) gene

56
Q

what are features of achondroplasia

A

disproportionate short statues, long bone shortening, large head, delayed motor development, normal cognition, short finger/toes, exaggerated lumbar lordosis, kyphocoliosis, prominent forehead, underdeveloped maxilla, genu varum, normal torso size

57
Q

what is the workup of achrondoplasia

A

diagnosis mainly clinical
support with imaging
confirm with genetic testing (+ FGFR3 mutation)

58
Q

how do you manage achondroplasia

A

manage complications: recurrent otitis media, OSA, obesity, spinal stenosis, narrowing foramen magnum)
PT for motor delays/genu varus
adaptation for ADLs
plot growth
weight management
? limb lengthening
? GH administration

59
Q

What is diabetes insipidus

A

deficiency in ADH (vasopressin) - posterior piutitary
loss of renal H20 reabsoprtion
results in compensatory polyuria
central D1 1/3 of cases
nephrogenic types also exist

60
Q

what is the presentation of diabetes insipidus

A

intense thirst(2-20L daily)
craving for icewater
polyuria
enuresis
symptoms worsened by corticosteroids

61
Q

what is the workup of diabetes insipidus

A

primarily clinical
associated findings: 24h urine collection with less than 2L / 24 hours r/o DI
+/- hyperuricemia (redcued renal clearance)
Vasopression (ADH) challenge test
MRI of pituitary and hypothalamus to r/o mass

62
Q

what is the treatment of DI

A

mild - no specific treatment, maintain fluid intake
avoid aggravating factors (corticosteroids)
pharmacologic treatment of choice: DDAVP (Desmopressin)

63
Q

What is SIADH

A

syndrome of inappropriate antidiuretic hormone
opposite of DI
inappropriate release of ADH from posterior pituitary

64
Q

what are the causes of SIADH

A

central - structural (CNS), psychiatric, pharmacologic (iatrogenic), metabolic issues
lungs - infection, mechanical, tumors (ectopic/paraneoplastic)

65
Q

what is the presentation of SIADH

A

decreased fluid output, even with adequate or increased intake
fluid overload -> edema, elevated BP, hyponatremia

66
Q

what should the workup for SIADH show

A

serum hyponatremia, hypoosmolality and urine osmolality about 100mosmol/kg
urine sodium concentration above 20 mEq/L
absence of heart, kidney or liver disease
normal thyroid and adrenal functon
low BUN and hypouricemia