ENDOCRINOLOGY

BOARD REVIEW
Presented by Med/Peds PGY III Class
 ENDOCRINOLOGY

Disorders of the
Hypothalamic Pituitary
Axis

K. Dionne Posey, MD, MPH

 ENDOCRINOLOGY
Pituitary Disorders
Thyroid Disorders
Adrenal Disorders
Gonadal Disorders
Calcium Disorders
Lipid Disorders
 Endocrine diseases

Hormone excess Hormone deficiency Hormone resistance

HypothalamicPituitary
Axis
 Pituitary Gland
Located within the sella tursica
Contiguous to vascular and neurologic
structures
Cavernous sinuses
Cranial nerves
Optic chiasm
Hypothalamic neural cells synthesize specific
releasing and inhibiting hormones
Secreted directly into the portal vessels of the
pituitary stalk
Blood supply derived from the superior and
inferior hypophyseal arteries
 Pituitary Gland
Anterior pituitary gland
Secrete various trophic hormones
Disease in this region may result in syndromes of
hormone excess or deficiency

Posterior pituitary gland

More of a terminus of axons of neurons in the
supraoptic and paraventricular nuclei of the
hypothalamus
Storehouse for the hormones
The main consequence of disease in this area is
disordered water homeostasis
 Anterior Pituitary Gland
Anterior Pituitary Master gland
Major blood source: hypothalamic-pituitary portal
plexus
Allows transmission of hypothalamic peptide pulses
without significant systemic dilution
Consequently, pituitary cells are exposed to sharp spikes
of releasing factors and in turn release their hormones as
discrete pulses
Production of six major hormones:
Prolactin (PRL)
Growth hormone (GH)
Adrenocorticotropin hormone (ACTH)
Luteinizing hormone (LH)
Follicle-stimulating hormone (FSH)
Thyroid-stimulating hormone (TSH)
 Anterior Pituitary Gland
Anterior Pituitary Master gland
Secreted in a pulsatile manner
Elicits specific responses in peripheral target
tissues
Feedback control at the level of the
hypothalamus and pituitary to modulate pituitary
function exerted by the hormonal products of the
peripheral target glands
Tumors cause characteristic hormone excess
syndromes
Hormone deficiency
may be inherited or acquired
Hypopituitarism
 Gonadotropin Deficiency
Women Men
Oligomenorrhea or Loss of libido
amenorrhea Erectile dysfunction
Loss of libido Infertility
Vaginal dryness or Loss of secondary
dyspareunia sex characteristics
(testosterone
Loss of secondary deficiency)
sex characteristics Atrophy of the testes
(estrogen deficiency) Gynecomastia
(testosterone
deficiency)
 ACTH Deficiency
Results in hypocortisolism
Malaise
Anorexia
Weight-loss
Gastrointestinal disturbances
Hyponatremia
Pale complexion
Unable to tan or maintain a tan
No features of mineralocorticoid deficiency
Aldosterone secretion unaffected
 TSH Deficiency

Hypothyroidism
Atrophic thyroid gland
 Prolactin Deficiency

Inability to lactate postpartum

Often 1st manifestation of Sheehan
syndrome
 Growth Hormone
Deficiency
Adults
Often asymptomatic
May complain of
Fatigue
Degrees exercise tolerance
Abdominal obesity
Loss of muscle mass

Children
GH Deficiency
Constitutional growth delay
 Hypopituitarism
Etiology
Anterior pituitary diseases
Deficiency one or more or all anterior pituitary
hormones

Common causes:
Primary pituitary disease
Hypothalamic disease
Interruption of the pituitary stalk
Extrasellar disorders
 Hypopituitarism
Primary pituitary disease Interruption of the
Tumors pituitary stalk
Pituitary surgery
Radiation treatment
Extrasellar disorders
Craniopharyngioma
Hypothalamic disease
Functional suppression of
Rathke pouch
axis
Exogenous steroid use
Extreme weight loss
Exercise
Systemic Illness
Hypopituitarism
 Hypopituitarism
Developmental and Acquired causes:
genetic causes Infiltrative disorders
Dysplasia Cranial irradiation
Septo-Optic dysplasia Lymphocytic
Developmental hypophysitis
hypothalamic Pituitary Apoplexy
dysfunction Empty Sella syndrome
Kallman Syndrome
Laurence-Moon-
Bardet-Biedl
Syndrome
Frohlich Syndrome
(Adipose Genital
Dystrophy)
Hypopituitarism: Developmental and
Genetic causes

Septo-Optic dysplasia

Kallman Syndrome
Laurence-Moon-Bardet-Biedl Syndrome
Frohlich Syndrome (Adipose Genital
Dystrophy)
 Hypopituitarism: Genetic

Septo-Optic dysplasia
Hypothalamic dysfunction and hypopituitarism
may result from dysgenesis of the septum pellucidum
or corpus callosum
Affected children have mutations in the HESX1 gene
involved in early development of the ventral
prosencephalon
These children exhibit variable combinations of:
cleft palate
syndactyly
ear deformities
hypertelorism
optic atrophy
micropenis
anosmia
Pituitary dysfunction
Diabetes insipidus
GH deficiency and short stature
Occasionally TSH deficiency
 Hypopituitarism: Developmental

Kallman Syndrome
Defective hypothalamic gonadotropin-releasing hormone
(GnRH) synthesis

Associated with anosmia or hyposmia due to olfactory

bulb agenesis or hypoplasia

May also be associated with: color blindness, optic

atrophy, nerve deafness, cleft palate, renal abnormalities,
cryptorchidism, and neurologic abnormalities such as
mirror movements

GnRH deficiency prevents progression through

puberty

characterized by
low LH and FSH levels
low concentrations of sex steroids
 Hypopituitarism: Developmental

Kallman Syndrome
Males patients
Delayed puberty and hypogonadism, including micropenis
result of low testosterone levels during infancy
Long-term treatment:
human chorionic gonadotropin (hCG) or testosterone
Female patients
Primary amenorrhea and failure of secondary sexual
development
Long-term treatment:
cyclic estrogen and progestin
Diagnosis of exclusion
Repetitive GnRH administration restores normal pituitary
Fertility may also be restored by the administration of
gonadotropins or by using a portable infusion pump to
deliver subcutaneous, pulsatile GnRH
 Hypopituitarism: Developmental

Laurence-Moon-Bardet-Biedl Syndrome
Rare autosomal recessive disorder
Characterized by mental retardation; obesity;
and hexadactyly, brachydactyly, or syndactyly
Central diabetes insipidus may or may not be
associated
GnRH deficiency occurs in 75% of males and
half of affected females
Retinal degeneration begins in early childhood
most patients are blind by age 30
 Hypopituitarism: Developmental

Frohlich Syndrome (Adipose Genital

Dystrophy)
A broad spectrum of hypothalamic lesions
hyperphagia, obesity, and central hypogonadism

Decreased GnRH production in these patients

results in
attenuated pituitary FSH and LH synthesis and
release

Deficiencies of leptin, or its receptor, cause

these clinical features
 Hypopituitarism
Acquired causes:
Infiltrative disorders
Cranial irradiation
Lymphocytic hypophysitis
Pituitary Apoplexy
Empty Sella syndrome
 Hypopituitarism: Acquired

Lymphocytic Hypophysitis
Etiology
Presumed to be autoimmune
Clinical Presentation
Women, during postpartum period
Mass effect (sellar mass)
Deficiency of one or more anterior pituitary hormones
ACTH deficiency is the most common
Diagnosis
MRI - may be indistinguishable from pituitary adenoma
Treatment
Corticosteroids often not effective
Hormone replacement
 Hypopituitarism: Acquired

Pituitary Apoplexy
Hemorrhagic infarction of a pituitary
adenoma/tumor
Considered a neurosurgical emergency
Presentation:
Variable onset of severe headache
Nausea and vomiting
Meningismus
Vertigo
+/ - Visual defects
+/ - Altered consciousness
Symptoms may occur immediately or may develop
over 1-2 days
 Hypopituitarism: Acquired

Pituitary Apoplexy
Risk factors:
Diabetes
Radiation treatment
Warfarin use
Usually resolve completely
Transient or permanent hypopituitarism is possible
undiagnosed acute adrenal insufficiency
Diagnose with CT/MRI
Differentiate from leaking aneurysm
Treatment:
Surgical - Transsphenoid decompression
Visual defects and altered consciousness
Medical therapy if symptoms are mild
Corticosteroids
 Quick Quiz!!!
When should you suspect pituitary
apoplexy?

MedStudy 2005 - Endocrine

 Answer

Suspect in patient presenting with

Variable onset of severe headache
Nausea and vomiting
Meningismus
Vertigo
+/ - Visual defects
+/ - Altered consciousness
 Hypopituitarism: Acquired

Empty Sella Syndrome

Often an incidental MRI finding
Usually have normal pituitary function
Implying that the surrounding rim of pituitary tissue is fully
functional
Hypopituitarism may develop insidiously
Pituitary masses may undergo clinically silent
infarction with development of a partial or totally
empty sella by cerebrospinal fluid (CSF) filling the
dural herniation.
Rarely, functional pituitary adenomas may arise
within the rim of pituitary tissue, and these are not
always visible on MRI
 Hypopituitarism
Clinical Presentation
Can present with features of deficiency
of one or more anterior pituitary
hormones
Clinical presentation depends on:
Age at onset
Hormone effected, extent
Speed of onset
Duration of the deficiency
 Hypopituitarism
Diagnosis
Biochemical diagnosis of pituitary
insufficiency
Demonstrating low levels of trophic
hormones in the setting of low target
hormone levels
Provocative tests may be required to
assess pituitary reserve
 Hypopituitarism
Treatment
Hormone replacement therapy
usually free of complications
Treatment regimens that mimic
physiologic hormone production
allow for maintenance of satisfactory
clinical homeostasis
 Hormone Replacement
Trophic Hormone Deficit Hormone Replacement

ACTH Hydrocortisone (10-20 mg A.M.; 10 mg P.M.)

Cortisone acetate (25 mg A.M.; 12.5 mg P.M.)
Prednisone (5 mg A.M.; 2.5 mg P.M.)

TSH L-Thyroxine (0.075-0.15 mg daily)

FSH/LH Males
Testosterone enanthate (200 mg IM every 2 wks)
Testosterone skin patch (5 mg/d)
Females
Conjugated estrogen (0.65-1.25 mg qd for 25days)
Progesterone (5-10 mg qd) on days 16-25
Estradiol skin patch (0.5 mg, every other day)
For fertility: Menopausal gonadotropins, human
chorionic gonadotropins
GH Adults: Somatotropin (0.3-1.0 mg SC qd)
Children: Somatotropin [0.02-0.05 (mg/kg per day)]
Vasopressin Intranasal desmopressin (5-20 ug twice daily)
Oral 300-600 ug qd
 Take home points:
Remember that the cause may be functional
Treatment should be aimed at the underlying cause
Hypopituitarism may present
Acutely with cortisol deficiency
After withdrawal of prolonged glucocorticoid therapy
that has caused suppression of the HPA axis.
Post surgical procedure
Post trauma
Hemorrhage
Exacerbation of cortisol deficiency in a patient
with unrecognized ACTH deficiency
Medical/surgical illness
Thyroid hormone replacement therapy
Pituitary Tumors
 Pituitary Tumors
Microadenoma < 1 cm
Macroadenoma > 1 cm

Is the tumor causing local mass effect?

Is hypopituitarism present?
Is there evidence of hormone excess?

Clinical presentation:
Mass effect
Superior extension
May compromise optic pathways leading to impaired visual
acuity and visual field defects
May produce hypothalamic syndrome disturbed thirst, satiety,
sleep, and temperature regulation
Lateral extension
May compress cranial nerves III, IV, V, and VI leaning to diplopia
Inferior extension
May lead to cerebrospinal fluid rhinorrhea
 Pituitary Tumors
Diagnosis
Check levels of all hormones produced
Check levels of target organ products

Treatment
Surgical excision, radiation, or medical therapy
Generally, first-line treatment surgical excision
Drug therapy available for some functional tumors
Simple observation
Option if the tumor is small, does not have local mass
effect, and is nonfunctional
Not associated with clinical features that affect quality of
life
 Craniopharyngioma
Derived from Rathke's pouch.
Arise near the pituitary stalk
extension into the suprasellar cistern common
These tumors are often large, cystic, and locally
invasive
Many are partially calcified
characteristic appearance on skull x-ray and CT images
Majority of patients present before 20yr
usually with signs of increased intracranial pressure,
including headache, vomiting, papilledema, and
hydrocephalus
 Craniopharyngioma
Associated symptoms include:
visual field abnormalities, personality changes
and cognitive deterioration, cranial nerve damage,
sleep difficulties, and weight gain.
Children
growth failure associated with either
hypothyroidism or growth hormone deficiency is
the most common presentation
Adults
sexual dysfunction is the most common problem
erectile dysfunction
amenorrhea
 Craniopharyngioma
Anterior pituitary dysfunction and diabetes
insipidus are common
Treatment
Transcranial or transsphenoidal surgical resection
followed by postoperative radiation of residual tumor
This approach can result in long-term survival and
ultimate cure
most patients require lifelong pituitary hormone
replacement.
If the pituitary stalk is uninvolved and can be
preserved at the time of surgery
Incidence of subsequent anterior pituitary
dysfunction is significantly diminished.
 Quick Quiz!!!
How does prolactin differ from LH/FSH
in regard to hypothalamic control?
 Answer
Tonic hypothalamic inhibition by
Dopamine
 Prolactinoma
Most common functional pituitary tumor
Usually a microadenoma
Can be a space occupying macroadenoma
often with visual field defects
Although many women with
hyperprolactinemia will have galactorrhea
and/ or amenorrhea
The absence these the two signs do not excluded
the diagnosis
GnRH release is decreased in direct
response to elevated prolactin, leading to
decreased production of LH and FSH
 Prolactinoma
Women
Amenorrhea this symptom causes
women to present earlier
Hirsutism
Men
Impotence often ignored
Tend to present later
Larger tumors
Signs of mass effect
 Prolactinoma
Essential to rule out secondary causes!!
Drugs which decrease dopamine stores
Phenothiazines
Amitriptyline
Metoclopramide
Factors inhibiting dopamine outflow
Estrogen
Pregnancy
Exogenous sources
Hypothyroidism
If prolactin level > 200, almost always a
prolactinoma (even in a nursing mom)
Prolactin levels correlate with tumor size in the
macroadenomas
Suspect another tumor if prolactin low with a large tumor
 Prolactinoma
Diagnosis
Assess hypersecretion
Basal, fasting morning PRL levels (normally <20 ug/L)
Multiple measurements may be necessary
Pulsatile hormone secretion
levels vary widely in some individuals with
hyperprolactinemia
Both false-positive and false-negative results may
be encountered
May be falsely lowered with markedly elevated PRL levels
(>1000 ug/L)
assay artifacts; sample dilution is required to measure these
high values accurately
May be falsely elevated by aggregated forms of circulating
PRL, which are biologically inactive (macroprolactinemia)
Hypothyroidism should be excluded by measuring
TSH and T4 levels
 Prolactinoma
Treatment
Medical
Cabergoline dopamine receptor agonist
Bromocriptine - dopamine agonist
Safe in pregnancy
Will restore menses
Decreases both prolactin and tumor size (80%)
Surgical
Transsphenoidal surgery irridation (if pt
cannot tolerate rx)
 Quick Quiz!!!
What type of tumors are most
prolactinomas?
Prolactin levels >200 almost always
indicate what?
Do prolactin levels correlate with tumor
size?

MedStudy 2005 - Endocrine

 Answer
What type of tumors are most
prolactinomas? Microadenomas
Prolactin levels >200 almost always
indicate what? Almost always indicates
prolactinoma
Do prolactin levels correlate with tumor
size? Yes, in macroadenomas
 Growth Hormone
Tumors
Gigantism
GH excess before closure of epipheseal
growth plates of long bones
Acromegaly
GH excess after closure of epipheseal
growth plates of long bones
Insidious onset
Usually diagnosed late
 Growth Hormone
Tumors
May have DM or glucose intolerance
Hypogonadism
Large hands and feet
Large head with a lowering brow and
coarsening features
Hypertensive 25%
Colon polyps
3-6 more likely than general population
Multiple skin tags
 Growth Hormone
Tumors
Diagnosis
Screen:
Check for high IGF-I levels (>3 U/ml)
Remember, levels very high during puberty
Confirm:
100gm glucose load
Positive: GH levels do not increase to <5ng/ml
Treatment
Surgical
Radiation
Bromocriptine - temporizing measure
May decrease GH by 50%
Octreotide
For suboptimal response to other treatment
 Quick Quiz!!!
How do you screen for acromegaly?

MedStudy 2005 - Endocrine

 Answer
Check for high IGF-I levels (>3 U/ml)
 Pituitary Gland
Anterior pituitary gland
Secrete various trophic hormones
Disease in this region may result in syndromes of
hormone excess or deficiency

Posterior pituitary gland

More of a terminus of axons of neurons in the
supraoptic and paraventricular nuclei of the
hypothalamus
Storehouse for the hormones
The main consequence of disease in this area is
disordered water homeostasis
Posterior Pituitary Gland
The Neurohypophysis
Major blood source: the inferior
hypophyseal arteries
Directly innervated by hypothalamic
neurons
(supraopticohypophyseal and
tuberohypophyseal nerve tracts) via the
pituitary stalk
Sensitive to neuronal damage by lesions that
affect the pituitary stalk or hypothalamus
Posterior Pituitary Gland
Production of
Vasopressin (antidiuretic hormone; ADH; AVP)
Oxytocin
Vasopressin (antidiuretic hormone; ADH; AVP)
Acts on the renal tubules to reduce water loss by
concentrating the urine
Deficiency causes diabetes insipidus (DI),
characterized by the production of large amounts
of dilute urine
Excessive or inappropriate production predisposes
to hyponatremia if water intake is not reduced in
parallel with urine output
Oxytocin
Stimulates postpartum milk letdown in response to
suckling
Posterior Pituitary Gland
Vasopressin (Anti Diuretic Hormone)
Some control via anterior hypothalamus
Contains separate osmoreceptors which aid in ADH
release and thirst regulation
Osmotic stimulus
Sodium
Mannitol
Non osmotic factors
Blood pressure and volume at extremes
Nausea
Angiotensin II
Insulin induced hypoglycemia
Acute hypoxia
Acute hypercapnia
Posterior Pituitary Gland
Rapidly secreted in direct proportion to serum
osmolality
Increased with
Aging
Hypercalcemia
Hypoglycemia
Lithium treatment
Volume contraction
Decreased with
Hypokalemia

Threshold set point

Increased
Hypervolemia, Acute hypertension, Corticosteroids
Decreased
Pregnancy, Pre-menses, Volume contraction
 Diabetes Insipdus
Etiology
Deficient AVP can be primary or secondary
The primary form
Deficiency in secretion
Agenesis or irreversible destruction of the
neurohypophysis
Malformation or destruction of the neurohypophysis by
a variety of diseases or toxins
Neurohypophyseal DI, Pituitary DI, or Central DI
Deficiency in action
Can be genetic, acquired, or caused by exposure to
various drugs
Nephrogenic DI

It can be caused by a variety of congenital, acquired, or

genetic disorders
50% idiopathic
 Diabetes Insipdus
Gestational DI
Primary deficiency of plasma AVP
Result from increased metabolism by an N-terminal
aminopeptidase produced by the placenta
Signs and symptoms manifest during pregnancy and
usually remit several weeks after delivery
 Diabetes Insipdus
Secondary deficiencies of AVP
Results from inhibition of secretion by
excessive intake of fluids
Primary polydipsia
Dipsogenic DI
characterized by an inappropriate increase in thirst
caused by a reduction in the "set" of the
osmoregulatory mechanism.
association with multifocal diseases of the brain such
as neurosarcoid, tuberculous meningitis, or multiple
sclerosis but is often idiopathic.
Psychogenic polydipsia
is not associated with thirst
polydipsia seems to be a feature of psychosis
Iatrogenic polydipsia
results from recommendations of health professionals
or the popular media to increase fluid intake for its
presumed preventive or therapeutic benefits for other
disorders
 Diabetes Insipdus
Secondary deficiencies of AVP
Antidiuretic response to AVP
Results from polyuria
Caused by washout of the medullary
concentration gradient and/or suppression of
aquaporin function.
Usually resolves 24 to 48 h after the polyuria is
corrected
Often complicate interpretation of tests commonly
used for differential diagnosis
 Diabetes Insipdus
Pathophysiology
When secretion or action of AVP is reduced to <80
to 85% of normal
urine concentration ceases and the rate of output
increases to symptomatic levels
Primary defect (pituitary, gestational, or
nephrogenic DI)
Polyuria results in a small (1 to 2%) decrease in body
water and a commensurate increase in plasma
osmolarity and sodium concentration that stimulate thirst
and a compensatory increase in water intake
Overt signs of dehydration do not develop unless the
patient also has a defect in thirst or fails to drink for some
other reason
 Diabetes Insipdus
Pathophysiology
Primary polydipsia
Pathogenesis of the polydipsia and polyuria is the
reverse of that in pituitary, nephrogenic, and gestational
DI
Excessive intake of fluids slightly increases body water,
thereby reducing plasma osmolarity, AVP secretion, and
urinary concentration.
Results in a compensatory increase in urinary free-water
excretion that varies in direct proportion to intake
Clinically appreciable overhydration uncommon
unless the compensatory water diuresis is impaired by
a drug or disease that stimulates or mimics
endogenous AVP
 Diabetes Insipdus
Clinical Presentation
Production of abnormally large volumes of dilute
urine
The 24-h urine volume is >50 mL/kg body weight and the
osmolarity is <300 mosmol/L.
The polyuria produces symptoms of urinary
frequency, enuresis, and/or nocturia, which may
disturb sleep and cause mild daytime fatigue or
somnolence.
It is also associated with thirst and a
commensurate increase in fluid intake
(polydipsia).
Clinical signs of dehydration are uncommon
unless fluid intake is impaired.
 Diabetes Insipdus
Diagnosis
Verify polyuria
a 24-h urine output collection
> 50 mL/kg per day (>3500 mL in a 70-kg man).
Check osmolarity
>300 mosmol/L
due to a solute diuresis and the patient should be
evaluated for uncontrolled diabetes mellitus or other less
common causes of excessive solute excretion
<300 mosmol/L
Due to water diuresis and should be evaluated further to
determine which type of DI is present
 Diabetes Insipdus
Diagnosis
Water deprivation test
If does not result in urine concentration
before body weight decreases by 5% or
plasma osmolarity/sodium exceed the
upper limit of normal
(osmolarity >300 mosmol/L, specific gravity
>1.010)
Primary polydipsia or a partial defect in AVP
secretion or action are largely excluded
Severe pituitary or nephrogenic DI are the only
remaining possibilities
 Diabetes Insipdus
Diagnosis: Neurogenic vs Nephrogenic
Administer Desmopressin (DDAVP)
1 g
0.03 ug/kg
subcutaneously or intravenously
Measure urine osmolality
(30,60,120 min)
1 to 2 h later
An increase of >50% indicates severe
pituitary DI
Smaller or absent response is strongly
suggestive of nephrogenic DI
 Diabetes Insipdus
Treatment
Neurogenic DI
DDAVP
Chlorpropamide (Diabinese)
Antidiuretic effect can be enhanced by cotreatment with a
thiazide diuretic
SE: hypoglycemia, disulfiram like reaction to ethanol
Contraindicated in Gestional DI
Nephrogenic DI
Not affected by treatment with DDAVP or chlorpropamide
May be reduced by treatment with a thiazide diuretic
and/or amiloride in conjunction with a low-sodium diet
Inhibitors of prostaglandin synthesis (e.g., indomethacin)
are also effective in some patients
Psychogenic or dipsogenic DI
there is no effective treatment
 Syndrome of Inappropriate
ADH secretion
Etiology
CNS
Lesions, Inflammatory disease
Trauma, psychosis
Drugs
Stimulate AVP release
Nicotine, phenothiazines, TCAs, SSRIs
Chlorpropamide, clofibrate, carbamazepine,
cyclophosphamide, vincristine
Pulmonary
Infection
Mechanical/ventilatory issue
 Syndrome of Inappropriate
ADH secretion
Pathophysiology
Excessive AVP production resulting in
decreased volume of highly concentrated
urine
Water retention
Decreased plasma osmolarity
Decreased plasma Na
 Syndrome of Inappropriate
ADH secretion
Clinical Presentation
Acute
Water intoxication
Headache, confusion
Nausea, vomiting
Anorexia
Coma, convulsions
Chronic
May be asymptomatic
 Syndrome of Inappropriate
ADH secretion
Diagnosis
Diagnosis of exclusion
AVP level inappropriately elevated relative
to plasma osmolality
 Syndrome of Inappropriate
ADH secretion
Treatment
Acute
Fluid restriction
Hypertonic saline
Central myelinolysis
Chronic
Demeclocyline 150-300mg PO TID-QID
Reversible Nephrogenic DI
 Treatment Guidelines
See Handout
 References
Harrison's Principles of Internal
Medicine - 16th Ed. (2005)
Up to Date
Med Study Endocrine
Mayo Clinic Board Review
Questions
 True or False
The pituitary:
1. Pituitary tumors are usually
macroadenomas.
2. Lack of galactorrhea essentially rules out a
prolactinoma.
3. Prolactin levels correlate with the size of a
prolactinoma
4. Prolactin level of 230 in a nursing woman is
probably due to a prolactinoma
5. An enlarged sella tursica can be seen in a
hypothyroid patient.
 Answers
The pituitary:
1. Pituitary tumors are usually
macroadenomas. True
2. Lack of galactorrhea essentially rules out a
prolactinoma. False
3. Prolactin levels correlate with the size of a
prolactinoma True
4. Prolactin level of 230 in a nursing woman is
probably due to a prolactinoma True
5. An enlarged sella tursica can be seen in a
hypothyroid patient. True
MedStudy 2005 - Endocrine
 A 24 year old woman complains of fatigue
and malaise. She gave birth to a healthy
infant 4 months before presentation. She
did not breastfeed. Menses have
subsequently been irregular and
infrequent, representing a change from
before pregnancy. The family history is
notable for a sister who has Hashimoto
thyroiditis. The pregnancy test is negative,
and the serum level of prolactin is normal.
Of interest, TSH is 0.9mIU/L (normal, 0.3-
5.0) and free thyroxine is 0.8ng/dL
(normal, 0.8-1.4). The results of MRI of
the pituitary are reported as normal. The
next step would be to:
A) Start thyroxine replacement therapy
B) Request a neurosurgeon to perform a
biopsy of the pituitary
Perform a water deprivation test
Perform a 1 g corticotropin (ACTH)
stimulation test
Measure IGF-1
A) Start thyroxine replacement therapy
B) Request a neurosurgeon to perform a
biopsy of the pituitary
C) Perform a water deprivation test
D) Perform a 1 g corticotropin (ACTH)
stimulation test
E) Measure IGF-1
A 38-year-old woman is referred to you by her
gynecologist. She first presented to her gynecologist
4.5 years ago with amenorrhea of 3 years duration
and galactorrhea of 1 years duration. She had been
taking no medications, and her initial physical
examination was unremarkable except for
expressible galactorrhea bilaterally. A routine
chemistry screen was normal; her T4 level was 7.8
g/dL, serum TSH was 1.4 U/mL, and prolactin
level was 48.2 ng/mL.
After taking bromocriptine for 2 months, her prolactin
level was 19 ng/mL, at which point her galactorrhea
ceased and she had her first menstrual period in 3
years. She continued to take bromocriptine over the
next 4 years; her prolactin level remained less than
20 ng/mL, and she continued to have regular
periods. However, she stopped taking her
bromocriptine 6 months ago and is now having
progressively worse headaches.
Her prolactin level is now 60.5 ng/mL, and a
visual field examination shows a small
superotemporal field cut in the right eye. A
computed tomographic (CT) scan shows a
2.4-cm 1.6-cm sellar mass with
considerable suprasellar extension. She is
now referred to you for further management.

What is the most likely diagnosis?

(A) Prolactinoma
(B) Clinically nonfunctioning pituitary adenoma
(C) Metastatic cancer to the sella
(D) Craniopharyngioma
What is the most likely diagnosis?

(A) Prolactinoma
(B) Clinically nonfunctioning pituitary adenoma
(C) Metastatic cancer to the sella
(D) Craniopharyngioma