Term
| partial or complete deficiency of one or any combination of anterior pituitary hormones |
|
Definition
|
|
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
|
|
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
|
|
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
|
|
Term
| Pituitary adenomas are usually __________, but may be associated with _______. |
|
Definition
|
|
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
|
|
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
|
|
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
|
|
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
|
|
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
|
|
Term
| What is the inheritance pattern of Kal 2? |
|
Definition
|
|
Term
| What is the inheritance pattern of Kal 3? |
|
Definition
|
|
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
|
|
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
|
|
Term
| What does GH deficiency cause in childhood? |
|
Definition
|
|
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
|
|
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
|
|
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
|
|
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
|
|
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
|
|
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
|
|
Term
| What would you expect to see with the ACTH levels in a patient with secondary hypoadrenalism? |
|
Definition
|
|
Term
| What would you expect the DHEA levels to be in a pt. with anterior hypopituitarism? |
|
Definition
|
|
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
|
|
Term
| What is the mainstay of substitution therapy for hypopituitarism? |
|
Definition
|
|
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
|
|
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
|
|
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
|
|
Term
| What is used to treat pts. with Laron syndrome? |
|
Definition
|
|
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
|
|
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
|
|
Term
| What do adults with congenital nephrogenic DI also often have? |
|
Definition
|
|
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
|
Definition
| high dose corticosteroids (increase renal free water clearance) |
|
|
Term
| What urine volume rules out DI? |
|
Definition
|
|
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
|
|
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
|
|
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
|
|
Term
| What drug choices do you have in treating nephrogenic DI? |
|
Definition
indomethacin-HCTZ
indomethacin-desmopressin
indomethacin-amiloride |
|
|
