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Anterior Hypopituitarism & Diabetes Insipidus
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108
Medical
Professional
08/30/2011

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Term
partial or complete deficiency of one or any combination of anterior pituitary hormones
Definition
anterior hypopituitarism
Term
ACTH deficiency leads to reduced secretion of _________, ________, and ________. However, ________ secretion remains intact.
Definition
cortisol, testosterone, epinephrine

aldosterone
Term
What does GH deficiency lead to in children?
Definition
short stature
Term
What does GH deficiency lead to in adults?
Definition
obesity and increased cardiac mortality
Term
What does prolactin deficiency result in?
Definition
inhibition of postpartum lactation
Term
What does TSH deficiency result in?
Definition
secondary hypothyroidism
Term
What does LH and FSH deficiency result in?
Definition
hypogonadism and infertility
Term
Anterior hypopituitarism can be caused by a problem in the ________ _________ or the _________.
Definition
anterior pituitary; hypothalamus
Term
What is the most likely mass lesion that can cause anterior hypopituitarism?
Definition
pituitary adenoma
Term
Pituitary adenomas are usually __________, but may be associated with _______.
Definition
sporadic; MEN-1
Term
What do pituitary adenomas with MEN-1 usually secrete?
Definition
prolactin (63%)
GH (9%)
or both
Term
Pituitary adenomas associated with MEN-1 are more ____________ than sporadic ones.
Definition
aggressive
Term
What other mass lesions, besides pituitary adenomas, can cause anterior hypopituitarism?
Definition
granulomas, brain tumors, Rathke cleft cysts, apoplexy, metastatic carcinoma, aneurysm
Term
What is a potential cause of anterior hypopituitarism that presents in youth with either hypopituitarism or diabetes insipidus?
Definition
Langerhan cell histiocytosis
Term
What is the treatment for Langerhan cell histiocytosis?
Definition
bisphosphonates
2-chlorodexoyadenosine (Cladribine)
Term
What are non-mass lesion causes of anterior hypopituitarism?
Definition
cranial radiation
surgery
encephalitis
hemochromatosis
autoimmunity
stroke
traumatic brain injury
s/p CABG
congenital anterior hypopituitarism
Term
What is a type of congenital anterior hypopituitarism?
Definition
septo-optic dysplasia (de Morsier syndrome)
Term
How are the types of congenital isolated hypogonadotropic hypogonadism classified?
Definition
sense of smell
Term
What are the different types of congenital isolated hypogonadotropic hypogonadism (IHH)?
Definition
congenital adrenal hypoplasia
Prader-Willi syndrome
Kallmann syndrome
Term
What types of congenital IHH are characterized by normosmia?
Definition
congenital adrenal hypoplasia
Prader-Willi syndrome
Term
What type of congenital IHH is characterized by anosmia?
Definition
Kallmann syndrome
Term
What is the inheritance pattern for congenital adrenal hypoplasia?
Definition
autosomal recessive
X-linked
Term
What gene is mutated in congenital adrenal hypoplasia?
Definition
DAX-1 gene
Term
When does congenital adrenal hypoplasia typically present?
Definition
infancy or early childhood in boys with DAX-1 gene mutation
Term
What happens with congenital adrenal hypoplasia?
Definition
permanent zone of adrenal cortex never develops

boys do not enter puberty
Term
Under what circumstance might congenital adrenal hypoplasia present in adulthood?
Definition
if only partial loss of function from mutation in DAX-1 gene
Term
What mutation is present in patients with Prader-Willi syndrome?
Definition
genes on paternal chromosome 15 are deleted or unexpressed
Term
What are the clinical manifestations of Prader-Willi syndrome?
Definition
hypogonadism has features of hypopituitarism and primary gonadal dysfunction

mental retardation
short stature
hyperphagia and obesity
autonomic dysregulation
Term
What causes Kallmann syndrome?
Definition
abnormal hypothalamic GnRH neuron development

hypoplasia of olfactory bulbs (causes anosmia)
Term
What are the clinical manifestations of Kallmann syndrome?
Definition
anosmia
cryptorchidism
sensorineural deafness
cerebellar dysfunction
bilateral sykinesis
nystagmus
cleft lip
high arched palate
Term
What is the inheritance pattern for Kal 1?
Definition
x-linked recessive
Term
What is the inheritance pattern of Kal 2?
Definition
autosomal dominant
Term
What is the inheritance pattern of Kal 3?
Definition
autosomal recessive
Term
What clinical findings are associated with congenital gonadotropin deficiency?
Definition
loss of LH and FSH
hypogonadism and infertility
(hypogonadotropic hypogonadism)

partial or complete lack of pubertal development
Term
What clinical findings are consistent with acquired gonadotropin deficiency?
Definition
-loss of axillary, pubic, and body hair gradually
-decreased libido
-amenorrhea
-decreased erections
Term
What does acquired gonadotropin deficiency predispose a patient to?
Definition
osteopenia and muscle atrophy
Term
What conditions can cause partial acquired male hypogonadism?
Definition
advancing age
obesity
poor health
Term
What does TSH deficiency cause?
Definition
hypothyroidism

fatigue, weakness, weight change, hyperlipidemia
Term
What is a retinoid chemotherapeutic agent that suppresses TSH secretion and circulating levels of T3 and T4, causing reversible secondary hypothyroidism?
Definition
bexarotene
Term
What clinical manifestations are associated with ACTH deficiency?
Definition
diminished cortisol

weakness, fatigue, weight loss, hypotension

adrenal mineralocorticoid (aldosterone) secretion continues

hyponatremia may occur, but potassium is generally unaffected
Term
What does congenital GH deficiency cause in infants?
Definition
hypoglycemia
Term
What does GH deficiency cause in childhood?
Definition
short stature
Term
What does GH deficiency cause in adulthood?
Definition
increased systolic pressure
increased LDL
decreased cardiac output
Term
What other things does GH deficiency cause?
Definition
-reduced muscle and bone mass
-reduced physical and mental energy
-impaired concentration and memory
-depression
-Laron syndrome
Term
What is the inheritance pattern for Laron syndrome?
Definition
autosomal recessive
Term
What causes Laron syndrome?
Definition
mutations in GH receptor gene

resistance to GH and IGF-1
Term
What clinical manifestations are associated with Laron syndrome?
Definition
dwarfism
prominent forehead
depressed nasal bridge
small mandible
central obesity
recurrent hypoglycemic seizures
Term
deficiency of several anterior pituitary hormones
Definition
combined pituitary hormone deficiency (CPHD)
Term
What gene mutation is present with CPHD?
Definition
PROP 1 gene mutation
Term
What are the clinical manifestations of CPHD?
Definition
short stature and growth failure due to GH and TSH deficiency
Term
What symptoms are common in patients with long-standing hypopituitarism?
Definition
dry, pale, finely textured skin
fine wrinkles
apathetic countenance
Term
What do you expect to see with the fasting glucose level in a pt. with anterior hypopituitarism?
Definition
may be low
Term
Why does hyponatremia often occur in a pt. with anterior hypopituitarism?
Definition
hypothyroidism
hypoadrenalism
Term
Why does hyperkalemia usually not occur with anterior hypopituitarism?
Definition
aldosterone production is not affected
Term
What tests should be done in older men to get a more accurate level for serum total testosterone?
Definition
testosterone and sex hormone binding globulin

calculate free testosterone
Term
What tests need to be done if testosterone is low and why?
Definition
FSH and LH to distinguish between gonad dysfunction and pituitary dysfunction
Term
What do you expect to see on a thyroid panel with a pt. that has anterior hypopituitarism?
Definition
free T4 is low, but TSH is not elevated
Term
What would you expect the level of sex hormones to be in a pt. with anterior hypopituitarism?
Definition
low
Term
What would you expect to see with the prolactin levels in a pt. with anterior hypopituitarism?
Definition
high if pt. has prolactinoma, acromegaly, or hypothalamic disease
Term
ACTH deficiency causes adrenal cortex atrophy in ___ weeks.
Definition
2
Term
What diagnostic tool is used to diagnose secondary hypoadrenalism?
Definition
cosyntropic stimulation test (synthetic ACTH)

measure cortisol--if 20 or greater, they are normal
Term
What would you expect to see with the ACTH level in a patient with primary hypoadrenalism?
Definition
high
Term
What would you expect to see with the ACTH levels in a patient with secondary hypoadrenalism?
Definition
low
Term
What would you expect the DHEA levels to be in a pt. with anterior hypopituitarism?
Definition
low
Term
What hormone deficiency is hard to diagnose and is usually just based on presentation?
Definition
GH deficiency

present in 96% of pts. who have 3 or more anterior pituitary hormone deficiencies
Term
What things might decreased IGF-1?
Definition
oral estrogen
prolonged fasting
hypothyroidism
uncontrolled DM
liver failure
Term
What needs to be done in a pt. with hypopituitarism when you can't establish an etiology?
Definition
screen for hemochromatosis with serum iron and transferrin saturation or ferritin
Term
What conditions can cause reversible hypogonatoropic hypogonadism?
Definition
serious illness
malnutrition
anorexia nervosa
Term
What surgical procedure is sometimes used in the treatment of anterior hypopituitarism?
Definition
transphenoidal removal of pituitary tumors
Term
What needs to be checked frequently for 2 weeks after surgery in a pt. who has a transphenoidal removal of a pituitary tumor and why?
Definition
sodium because the surgery can result in hyponatremia
Term
What treatment is given for excess prolactin?
Definition
dopamine agonist (cabergoline, bromocriptine, quinagolide)
Term
What treatment do you give for a GH secreting tumor?
Definition
octreotide
Term
What is the mainstay of substitution therapy for hypopituitarism?
Definition
hormone replacement
Term
What lab needs to be monitored while pt. is on hydrocortisone therapy for hypopituitarism and why?
Definition
WBC with diff

neutrophilia and lymphopenia can indicate over-replacement with steroids and vice versa
Term
What do you need to base your thyroid hormone replacement therapy on in a pt. with hypopituitarism and why?
Definition
free T4 levels, because their TSH will always be low
Term
What can be given to women with hypopituitarism and secondary adrenal insufficiency to increase pubic and axillary hair, improve libido, alertness, stamina, and psychological well being?
Definition
compounded DHEA (if her level is <400 ng/ml)
Term
What is an equivalent to LH that can be given to men to increase spermatogenesis?
Definition
hCG

(d/c testosterone)
Term
If after 6 months of hCG therapy the sperm count is still low, what treatment can be initiated?
Definition
FSH injections (follitropin B)
Term
What hormone replacement therapy improves body morphology and lipid profiles but is associated with an increase in carotid plaques?
Definition
recombinant human growth hormone (rhGH)
Term
How long should you give rhGH to see effects and if not, discontinue?
Definition
3-6 months
Term
How can you use rhGH during pregnancy?
Definition
1st trimester--can be taken
2nd trimester--tapered
3rd trimester--discontinued
Term
What needs to potentially change in a women before initiating rhGH therapy and why?
Definition
oral estrogen needs to be changed to transdermal or transvaginal because oral estrogen decreases IGF-1 production
Term
What do you use to guide treatment with rhGH?
Definition
serum IGF-1 levels
Term
What is used to treat pts. with Laron syndrome?
Definition
IGF-1
Term
What causes most cases of primary central diabetes insipidus?
Definition
autoimmune reaction against hypothalamic AVP secreting cells
Term
What would you see on an MRI with a pt. that has primary central DI?
Definition
pituitary thickening
Term
What might also be a cause of primary central DI besides an autoimmune reaction?
Definition
genetic (dominant trait)
symptoms begin at age 2
Term
What autosomal recessive condition can DI occur with?
Definition
Wolfram syndrome (DIDMOAD)
Term
What does DIDMOAD stand for?
Definition
diabetes insipidus
type 1 DM
optic atrophy
deafness

(Wolfram syndrome)
Term
What causes secondary central diabetes insipidus?
Definition
tumor causing damage to hypothalamus or pituitary stalk

metastases is more likely to cause DI than pituitary adenomas
Term
When does vasopressinase-induced diabetes insipidus occur?
Definition
last trimester of pregnancy and in puerperium
Term
What happens in vasopressinase-induced DI?
Definition
vasopressinase (circulating enzyme) destroys native vasopressin
Term
What is the treatment for vasopressinase-induced DI?
Definition
synthetic desmopressin

(vasopressinase only destroys native vasopressin)
Term
What happens in nephrogenic DI?
Definition
defect in renal tubules interferes with water reabsorption

normal levels of vasopressin; polyuria is just unresponsive to it
Term
What causes congenital nephrogenic DI?
Definition
defective expression of renal vasopressin V2 receptors or vasopressin sensitive water channels
Term
What is the inheritance pattern of congenital nephrogenic DI?
Definition
familial x-linked trait
Term
What do adults with congenital nephrogenic DI also often have?
Definition
hyperuricemia
Term
What conditions may cause acquired forms of vasopressin resistant DI?
Definition
diuretic/corticosteroid use
pyelonephritis
renal amyloidosis
myeloma
potassium depletion
Sjogren syndrome
sickle cell anemia
chronic hypercalcemia
Term
What are the symptoms of diabetes insipidus?
Definition
-intense thirst
-polyuria (2-20 L of fluid per day)
-hypernatremia and dehydration
Term
What aggravates DI?
Definition
high dose corticosteroids (increase renal free water clearance)
Term
What urine volume rules out DI?
Definition
less than 2 L/24 hours
Term
What challenge test may be given to diagnose central DI?
Definition
vasopressin challenge test

pts. with central DI have a marked reduction in thirst and polyuria
Term
How do you diagnose non-familial central DI?
Definition
MRI to look for lesions
Term
How do you diagnose nephrogenic DI?
Definition
measure serum vasopressin during fluid restriction

typically the vasopressin is high
Term
What is the drug of choice in treating central DI?
Definition
desmopressin acetate
Term
What side effects does desmopressin have?
Definition
hyponatremia
depression
agitation
increased suicide rates
Term
What type of diuretics work well for central and nephrogenic DI?
Definition
HCTZ
Term
What drug choices do you have in treating nephrogenic DI?
Definition
indomethacin-HCTZ
indomethacin-desmopressin
indomethacin-amiloride
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