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Clin Med I - Endocrine > Pituitary Disorders > Flashcards

Pituitary Disorders Flashcards

1
Q

What is released to the posterior pituitary from the hypothalamus?

A

ADH and Oxytocin

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2
Q

What is produced in anterior pituitary?

A

The 6 anterior pituitary hormones:

  1. ACTH
  2. TSH
  3. LH
  4. FSH
  5. PRL
  6. GH
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3
Q

What dz is indicated by increase GH?

A

Acromegaly/gigantism

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4
Q

What dz is indicated by decreased GH?

A

Dwarfism

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5
Q

What dz is indicated by increased Prolactin?

A

Galactorrhea

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6
Q

Endocrine glands secrete ___________.

A

Hormones directly into the blood stream (Diffuse effects)

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7
Q

Examples of endocrine glands include?

A

Pituitary, pancreas, ovaries, testes, thyroid

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8
Q

Exocrine glands secrete__________.

A

Substances into the ducts (more direct effects)

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9
Q

Examples of exocrine glands include?

A

Sweat glands, mammary glands, salivary glands

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10
Q

What causes Gigantism?

A

High linear growth during childhood due to excess of IGF-1

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11
Q

What causes Acromegaly?

A

Excess of IGF-1 in adulthood.

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12
Q

Most common cause of gigantism or acromegaly?

A

Growth Hormone secreting pituitary adenomas or hyperplasia

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13
Q

Gigantism is typically isolated but can be a feature of other conditions. What are the other conditions?

A
  1. Multiple endocrine neoplasia (MEN) type I
  2. McCune-Albright syndrome
  3. Neurofibromatosis
  4. Tuberous Sclerosis
  5. Carney Complex
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14
Q

What are you more likely to see as a PA in the United States, Gigantism or Acromegaly?

A

Gigantism is EXTREMELY RARE.

ACROMEGALY is more common than gigantism.

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15
Q

What is the hallmark of Acromegaly?

A

Insidious onset, delayed detection for about 10 years.

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16
Q

Signs and symptoms of Gigantism due to intracranial mass effect or excess GH/IGF-1?

A 
Tall stature
macrocephaly
visual changes
hypopituitarism
HA
Frontal bossing
Hyperhidrosis
Osteoarthritis
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17
Q

Signs and symptoms of acromegaly due to intracranial mass effect or excess GH/IGF-1?

A

Symptoms due to Intracranial tumor: Visual field defect (bitemporal hemianopsia), hyperprolactinemia.

Symptoms due to excess of GH/IGF-1
Increase ring/shoe size, Hyperhidrosis, Coarsening of facial features, Prognathism, arthritis, doughy-feeling skin over face and extremities. Enlargement of lower lip and nose. Hypertrichosifs, oily skin, Hyperpigmentation (acanthosis nigricans).

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18
Q

What is the DDX for gigantism?

A 
Familial tall stature
Exogenous obesity
Cerebral gigantis
Weaver syndrome
Carney complex
McCune-Albright syndrome
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19
Q

Define McCune-Albright syndrome

A

fibrous dysplasia of bone
hyper pigmented skin macules,
precocious sexual development in children.
Goiter, hyperthyroidism, acromegaly, Cushing Syndrome

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20
Q

Diagnosis and W/u for Gigantism?

A

Dramatic linear growth, macrocephaly, weight gain
MRI-pituitary adenoma (producing GH)
Screening test increased GH and IGF-I

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21
Q

Tx for Gigantism?

A

Transsphenoidal surgery-1st line
Bromocriptine, octretide
Radiation therapy

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22
Q

Diagnosis and w/u for acromegaly?

A

Clinical suspicion: enlarged hat/shoe size.
GH levels are UNRELIABLE.
OGTT: GH levels obtained at 0, 30, 60, 90, 120 min. Lack of suppression of GH is diagnostic.
IGF-1: If elevated diagnostic (unless pregnant/puberty)
MRI: Wit hand without GAD confirms pituitary adenoma

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23
Q

First line TX for Acromegaly?

A

Surgical removal of pituitary gland tumor-Primary tx.
f/u imaging study 12wks post surg-determines residual tumor.
Eval post sure for damage secondary to tumor
For pt’s with postoperative disease only meds are necessary
-somatastatin
-Dopamine-receptor agonist
-GH receptor antagonist

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24
Q

What follow up is necessary for pt’s with Acromegaly?

A

IGF-I levels monitored
Eval for severe GH deficiency.
Follow up cardiac eval for regurgitant valvular heart disease.

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25
Q

What is the major characteristic of Diabetes Insipidus (DI)

A

Large volumes of dilute urine

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26
Q

What are the two types of Diabetes Insipidus?

A

Central

Nephrogenic

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27
Q

Define Central Diabetes Insipidus

A

Neurogenic, pituitary, or neurohypophyseal: Decreased secretin of ADH.

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28
Q

Define Nephrogenic DI

A

Decreased ability to concentrate urine because of resistance to ADH action in the kidney

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29
Q

Etiology of Central DI

A 
Most commonly: Idiopathic (inflammatory/autoimmune)
Malignant or benign tumors of brain or pituitary (25%) 
Cranial surgery (20%)
Head trauma (16%)
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30
Q

Etiology of Nephrogenic DI

A 
Most common-lithium toxicity (20% of pt's on lithium) or hypercalcemia
hypokalemia
renal disease
pregnancy (transient)
Hyperglycemia
other drugs - amphotericin B. Cidofovir
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31
Q

What hormone is the primary driver of free water excretion in the body?

A

ADH

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32
Q

What does ADH function to do?

A

Alters the water permeability of the cortical and medullary collecting tubes in the kidney.

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33
Q

What response does a decrease in extracellular fluid volume cause in the body?

A

ADH secretion - to increase water retention
Thirst - to raise water intake
Aldosterone - to preserve sodium

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34
Q

Is DI common in the US?

A

No. It is relatively uncommon in the US.

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35
Q

What are the symptoms of DI?

A

Polyuria, Polydipsia, and notcuria

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36
Q

What type of DI is most common?

A

Central DI following trauma or surgery to the region of the pituitary and hypothalamus

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37
Q

What are signs of DI?

A

PE is usually normal.
There may be pelvic fullness, flank pain, tenderness if hydronephrosis,
Bladder enlargement

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38
Q

DDX for DI

A 
DMtype1
Histiocytosis
Hypercalcemia
Hypokalemia
Medullary Cystic Disease
Sickle Cell Anemia
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39
Q

Testing for DI

A

24 hr Urine-volume, specific gravity
Serum lytes and glucose
simultaneous plasma and urinary osmolarity
Plasma ADH level
Water deprivation test - aka Vasopressin challenge test

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40
Q

Define Water Deprivation test

A
  • Water deprivation followed by administration of vasopressin differentiates between central and nephrogenic DI.
  • caution with this because partial nephrogenic DI or primary polydipsia may have similar results to nephrogenic DI.

Central, and nephrogenic urinary osmolality will be <300mOsm/kg after water deprivation. After admin of ADH osmolality will raise to more than 750mOsm/kg in CENTRAL DI but will not rise at all in nephrogenic DI

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41
Q

Treatment for DI

A

Fluid replacement-most patients can just drink enough fluid to replace losses.

  • in presence of hypernatremia IV fluid with dextrose that is hyperosmolar with respect to serum will rehydrate pt.
  • instruct pts when traveling to avoid dehydration, vomitting, and diarrhea must be addressed quickly
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42
Q

What medications can be prescribed for pt with central DI

A
  • Desmopressin; available subQ, IV, Intranasal, and oral.

- Vasopressin: if response to desmopressin to not complete or pt can’t afford desmopressin

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43
Q

What is the difference between desmopressin and Pitressin (vasopressin)

A

Desmopressin has NO vasopressor activity. Pitressin (vasopressin) HAS vasopressor activity. Desmopressin has more potent antidiuretic activity.

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44
Q

t/f Desmopressin’s primary role is in nephrogenic DI

A

F: Desmopressin has NO ROLE in tx of nephrogenic DI. Non hormonal drugs are usually more effective in tx of nephrogenic.

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45
Q

What are the second line meds that can be used in tx of DI?

A

Antidiabetics-sulfonylureas, Anticonvulsants-Carbamazepine, Diuretics-thiazide, Diretics Potassium-sparing, NSAIDs(usually used in nephrogenic)

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46
Q

What is the definition of SIADH

A

Syndrome of inappropriate antidiuretic hormone secretion (EXCESS ADH). Excess ADH interferes with water intake. Increase in ADH results in increased water reabsorption by kidneys, decreased aldosterone secretion.

47
Q

Etiology of SIADH?

A

Neoplasia
Nervous system disorder
Pulmonary disease
Drug induced

48
Q

What is the cardinal feature of SIADH?

A

Too much ADH.=water load enhanced

ADH increases the water permeabiltiy of the renal collection ducts, thereby increasing renal water reabsorption.

49
Q

What is the most common electrolyte derangement occurring in hospitalized patients?

A

Hyponatremia

50
Q

Signs and symptoms of SIADH?

A

anorexia
nausea
vomiting
Neuro symptoms: HA, dulled sensorium, confusion, convulsions, Death
Progressivly more severe psych, neuro, resp as time goes on.

51
Q

What is diagnostically significant to SIADH?

A 
Thyroid and adrenal function will be normal
Serum hypo-osmolarity 
hyponatremia
Urine hyperosmolarity
Normal renal function
absence of CHF, hypovolemia
52
Q

What labs should be drawn if SIADH is suspect?

A 
Serum Na+, potassium, chloride, bicarb
Plasma osmolality
Blood urea nitrogen
Serum creatinine
Blood glucose
Urine osmolality
Serum uric acid
serum cortisol
TSH
53
Q

Tx of SIADH

A
  • important to remember that if you are correcting hyponatremia this needs to be done very carefully and slowly.
  • Rapidity of tx depends on
  • degree of hyponatremia
  • symptoms
  • acuity (<48h)
54
Q

Review of cause of SIADH

A

too much ADH-serum dilute-urine concentrated

55
Q

Review of cause of DI central

A

not enough ADH-serum concentrated-urine dilute

56
Q

Review of cause of DI Nephrogenic

A

Kidney does not respond to ADH-Serum concentrated-urine dilute with normal/high serum ADH level

57
Q

Review of cause of psychogenic water intoxication

A

Serum dilute urine dilute

58
Q

Definition of dwarfism

A

Person of short stature - under 4’10” in adulthood
Achondroplasia is MC cause (70%)
other causes - genetic, kidney dz, and problems with metabolism or hormones

59
Q

What are the 2 categories of dwarfism?

A

Disproportionate dwarfism

Proportionate dwarfism

60
Q

Define disproportionate dwarfism

A

some parts of body are small, others are average size - disorders that cause this inhibit the development of bones -

61
Q

Define proportionate dwarfism

A

All parts of the body are small. These conditions present at birth and in early childhood and limit overall growth and development-

62
Q

MC cause of disproportionate dwarfism

A

Achondroplasia (avg size trunk, short extremities)

63
Q

MC cause of proportionate dwarfism

A

growth hormone deficiency (pituitary fails to produce enough GH)

64
Q

Signs and symptoms of disproportionate dwarfism

A 
Achondroplasia-recessive genetic d/o.  
Delay in motor skills
Frequent ear infections
Bowing of legs
sleep apnea
hydrocephalus
crowded teeth
Spinal stenosis*
Arthritis*
65
Q

Signs and symptoms of proportionate dwarfism

A

complications with poorly developed organs
Heart problems - Turner syndrome
Absence of sexual maturation - physical and social function development

66
Q

Testing for Dwarfism

A

Measurements (current percentile) ht, wt, head circumference
Appearance- physical exam reveals distinct facial and skeletal features
Imaging: X-rays (delayed mat of bones), MRI (abnormalities of pituitary/hypothalamus)
Genetic tests
Family hx
Hormone (GH)

67
Q

Surgical Tx for dwarfism

A

limb lengthening used to be popular but is more controversial now.
For disproportionate dwarfism:
-can correct bone growth direction
-stabilize and correct the spine
-increase size and shape of spine alleviate pressure
-shunt for hydrocephalus

68
Q

Hormone tx for dwarfism

A

If d/t GH deficiency: tx w/ injections of synth version of hormone
if Turner syndrome-estrogen, and related hormone tx for sexual development
***-GH supplementation will not improve final adult height for Achondroplasia!!

69
Q

What are the 5 classifications for pituitary gland d/o

A
  1. Neoplastic (Pit adenoma, tumors, mets)
  2. Inflammatory (sarcoid)
  3. Infectious (TB)
  4. Vascular (aneurysm, carotid/sheehan’s=apoplexy)
  5. Congenital
70
Q

Pituitary adenoma epidemiology/pathyphys.

A 
benign/common (10%+)
MC cause of pituitary hormone hyper/hyposecretion in adults
15% of all intracranial neoplasms!!
1/3 are clinically nonfunctioning
Of the hormonally functioning
10-15%=GH And ACTH
50% secrete PROLACTIN (prolactinoma)
71
Q

Symptoms/signs of pituitary adenomas

A

50-60% present with visual symptoms due to compression of optic nerve
Nonspecific HA
Lateral extension ophthalmoplegia, diploipa &/or Ptosis
Extension into sphenoid can cause spontaneous CSF Rhinorrhea
endocrine dysfunction-Prolaticn, GH, ACTH
Visual acuity decreased in one or both eyes
pupillary light reaction can be abnormal

72
Q

What might a bitemporal hemianopsia indicate?

A

Large pituitary lesion

73
Q

What hormonal physical findings might be present with prolactinoma?

A

Females-galactorrhea

Males-galactorhea is uncommon, decreased size of testicles

74
Q

What hormonal findings would be present with acromegaly?

A

large hands and feet, coarse facial features with frontal bossing, women may appear masculinized, Prognothism, carpal tunnel syndrome, voice quality changes

75
Q

hormonal findings for cushings dz.

A

Obesity, centripetal fat deposition, proximal myopathy, moon face, buffalo hump, post subspace cataracts, atrial htn, bruises, striae

76
Q

Hormonal findings for hypopituitarism

A

Chronic=hypotension, generalized weakness, hypothermia, malaise, depression
Acute=sudden hypopituitarism, shock, coma, death

77
Q

DDX for pituitary adenomas

A 
Basilar artery thrombosis
Brainstem gliomas
Cavernous sinus syndrome
cerebral venous thrombosis
craniopharyngioma
Dizziness, vertigo, imbalance
Intracranial hemorrhage
asytrocytoma
meningioma
Primary CNS Lymphoma
TB
78
Q

Testing for prolactinoma

A

Elevated prolactin level

Serum prolactin level should be measured if pt is suspected to have a sellar mass (order imaging if high)

79
Q

What is the best endocrinologic test for acromegaly?

A

Serum insulin like growth factor 1 (IGF-1) best endocrinology test for acromegaly. IGF-1 reflects concentration in the last 24 hours.

80
Q

What is the most definitive test for acromegaly?

A

OGTT (oral glucose tolerance test). + result is the failure of GH to decrease to <1mcg/L after ingesting 50-100 g of glucose

81
Q

What is the imaging study suggested for pituitary adenomas?

A

MRI

82
Q

Tx of pituitary adenomas

A 
Goal is to improve:
mass effect
hormonal hypo- or hyper- secretion
Prevent recurrence
Transsphenoidal surgery tx of choice for all pituitary tumors except prolactinomas.  Radiation and med tx are adjunct to surgical approach
83
Q

Tx for Prolactinomas (medical)

A

Most respond to dopamine receptor agonists.

84
Q

Tx for acromegaly (medical)

A

Somatastatin analogues - tx increased post op levels of GH.
Dopamine agonists are sometimes used
Replacement tx for absent hormones

85
Q

What type of Pituitary adenomas are candidates for Transsphenoidal surgery?

A

Acromegaly (decreases GH to less than 5mcg/L in 60%)

Cushing disease: First line of tx. Curative in 80%

86
Q

What additional consultations should be made for a pt with a pituitary adenoma?

A 
Ophtho.,
neuro-ophtho
neuroradiologist
endocrinologist
Neurosurgeon
gynecologist
neuropathologist
radiation medicine
87
Q

What d/o is considered an endocrine emergency and is caused by either a hemorrhage into a pre-existing adenoma, or Sheehan’s syndrome post-partum?

A

Pituitary apoplexy

88
Q

What are signs and symptoms of pituitary apoplexy?

A

Severe H/A, Bilateral vision changes, ophthalmoplegia.

If severe: CV collapse, LOC, hypoglycemia, CNS hemorrhage-death

89
Q

What is the tx for pituitary apoplexy?

A

If no visual loss or impaired conciousness- glucocorticoids

If visual/neuro sx-surgical decompression

90
Q

Define hyperprolactinemia

A

hyper secretion of the pituitary leading to elevated serum prolactin.

91
Q

What hormone inhibits the production of prolactin?

A

Dopamine (from the pituitary)

92
Q

What hormone mildly increases prolactin?

A

Mildly increased by TRH/estrogens

93
Q

What does high prolactin suppress?

A

GNrH to decrease FSH/LH

94
Q

What percent of people with amenorrhea and galactorrhea have hyperprolactinemia?

A

75%

95
Q

Signs and symptoms of hyperprolactinemia in females

A

Menstrual changes (amenorrhea),
Galactorrhea
Infertility
MC presentation at a young age always consider in females with nipple d/c

96
Q

Signs and symptoms of hyperprolactinemia in males

A 
Hypogonadism
decreased libido
erectile dysfunction
infertility
sometimes gynecomastia
sometimes galactorrhea
97
Q

testing for hyperprolactinemia

A

Fasting, morning prolactin level
if elevated exclude non-neoplastic reasons
pregnancy, hypothyroidism, medications
If cause is unknown obtain MRI of pituitary

98
Q

tx of hyperprolactinemia (medical)

A

Dopamine agonist (bromocriptine, cabergoline)

  • dopamines inhibit PRL
  • decreases tumor size
  • lower PRL level
  • may cause nausea
99
Q

Tx of hyperprolactinemia (surgical)

A

Trans-sphenoid resection

for pt’s with drug intolerance, tumors resistant to med tx., persistent visual field defect, and large tumors

100
Q

Follow up for hyperprolactinemia

A

Fasting PRL monitored monthly

Visual field testing and MRI

101
Q

Complications of hyperprolactinemia

A

Blindness
hemorrhage
osteoporosis
infertility

102
Q

What is it important to educate your patients about when treating them for hyperprolactinemia?

A

A decrease in PRL levels may restore ovulation-when returning to normal pregnancy is possible (educate re birth control)

103
Q

Galactorrhea Definition

A

Not a disease - a sign
nipple discharge unrelated to normal milk production of breast feeding
MC cause is hyperprolactinemia

104
Q

Etiology for galactorrhea

A 
Hyperprolactinemia, prolactinoma
medications: sedatives, antidepressives, antipsychotics, antihtn, BCP's
cocaine/opiods
Herbal supp. (fennel, annise, fenugreek)
Hypothyroid
Chronic renal dz
Excessive breast tim
Spinal cord surgery
Idiopathic
105
Q

signs and sx of glactorrhea

A

Pathologic-persistant d/c, unilateral, limited to 1 duct, serous, sanguinous, serosanguinous

106
Q

What must you do if the d/c dt galactorrhea is bloody?

A

cancer workup

107
Q

Testing for galactorrhea

A

Labs: PRL level, Beta HCG (preg), TSH, LFT, BUN, Creat, FSH, LH
RAD: MRI, US, Mammo,

108
Q

definition of hypopituitarism

A

Clinical syndrome of deficiency of pituitary hormone.
Can be Panhypopituitarism: effecting all of pituitary hormones
of
Partial hypopituitarism: effecting only 1 or several pituitary hormones

109
Q

etiology of hypopituitarism

A

Neoplastic (MC), genetic, congenital, traumatic, vascular, infectious.

110
Q

Is hypopituitarism rare or common?

A

Rare-affects less than 20,000 individuals in the US

111
Q

Hormonal signs of hypopituitarism

A 
ACTH deficiency
TSH deficiency - hypothyroid
Gonadotropin deficieny - hypogonadism
GH deficiency - Failure to thrive, short stature
ADH deficiency - polyuria and polydipsia
112
Q

Structural signs of hypopituitarism

A 
HA
Diplopia
Visual field deficit
Polydipsia/polyuria 
-rarely SIADH
113
Q

tests for hypopituitarism

A 
8am cortisol
TSH
FT4
IGF-1
TTEST-men
PRL level
mentrual status - females
Provocative tests for GH and ACTH deficiency
114
Q

TX of hypopituitarism

A

Directed at etiology

Individualized hormone replacement

Clin Med I - Endocrine (6 decks)

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