Acromegaly, Gigantism, and Dwarfism Flashcards
both acromegaly and gigantism will present with what levels of GH?
increased GH
excessive secretion of GH, usually due to a pituitary somatotroph
acromegaly
at the time of diagnosis, 75% of patients with acromegaly will usually present with what?
macroadenoma
what is the interval from the onset of symptoms until diagnosis of acromegaly?
12 years
a patient presents with acral and soft tissue overgrowth, skin thickening, enlarged jaw, enlarged/swollen hands and feet, coarse facial features, deepening of the voice, and hand paresthesia. what are they likely experiencing?
acromegaly
macrognathia
enlarged jaw
a patient with acromegaly will likely tell you what about them?
increase in shoe/glove size
due to pressure from the adenoma, what would a patient with acromegaly complain of? (2)
headaches
visual changes
what are 4 disease processes that a patient with acromegaly can begin to experience?
hypertension
sleep apnea
type 2 diabetes
musculoskeletal pain
what is the best test to diagnose a patient with acromegaly? and why?
serum IGF-1
it is stable throughout the day
an unequivocal or elevated serum IGF-1 should prompt us to order a …
oral glucose tolerance test (OGTT)
for testing acromegaly with an OGTT, what should happen to indicate that we have a normal response?
serum GH goes to 1 or less, 2 hours after ingesting 75g of glucose
for testing acromegaly with an OGTT, what should happen to indicate that the patient has acromegaly?
serum GH is greater than 1
if GH and glucose is not suppressed after the administration of OGTT in acromegaly, what is the next diagnostic to order?
pituitary MRI
what is the ideal treatment for acromegaly?
endoscopic transphenoidal surgery
what are 3 medications that can be used for patients with acromegaly who are not surgical candidates?
somatostatin analog (octreotide, lanreotide)
cabergoline
pegvisomant
what is the treatment goal for acromegaly?
lower serum IGF-1 and GH concentrations
what cannot be reversed once it has occurred, in patients with acromegaly?
bony changes
if surgical and medical therapy do not work for acromegaly, what can be done?
radiation therapy
GH excess that occurs before fusion of the epiphyseal growth plates in a child or adolescent
gigantism
what is the most common cause of gigantism?
pituitary adenoma that excretes too much GH, leading to increased IGF-1
a 13 yr old presents with large hands, feet, coarse facial features, excessive sweating, and macrocephaly. what are they likely experiencing?
gigantism
what is the treatment for gigantism?
endoscopic transphenoidal surgery
if surgery is not an option for a patient with gigantism, what medical therapy can we do?
octreotide (first line)
bromocriptine
pegvisomant
why is radiation therapy in a patient with gigantism not recommendeded?
could lead to panhypopituitarism (completely kill the pituitary gland)
a proportionate growth failure; downward deviation of growth curve across 2 major percentile curves
dwarfism
a child between 2-4 yrs old should have what height velocity to be diagnosed with dwarfism?
less than 2.2 in/yr
a child between 4-6 yrs old should have what height velocity to be diagnosed with dwarfism?
less than 2 in/yr
a child between 6 years to puberty should have what height velocity to be diagnosed with dwarfism?
less than 1.6 in/yr for boys
less than 1.8 in/yr for girls
what are 3 diagnostics used for dwarfism?
xray for bone age
serum IGF-1
GH provocation
what is the treatment for a patient with dwarfism?
growth hormone
what are 3 things to be monitored in patients with dwarfism?
height velocity q 4-6 mo
IGF-1 q 4-6 mo
bone age q 6-12 mo