acromegaly Flashcards
what is acromegaly
condition in which benign pituitary adenomas lead to an excess secretion of growth hormone (GH) and insulin-like growth factor 1 (IGF-1)
3rd decade of life (mean age at diagnosis usually 40–45 year
etio of acromegaly
Benign growth hormone-secreting pituitary adenoma (> 95% of cases)
hypothalamic tumors
paraneoplastic syndromes
NORMAL AXIS OF GH
hypothalamus secretes GhRH
pituitary secreses GH
liver synthesises
IGF-1
acts on IGF-1 and insulin receptors
effect of IGF-1 on insulin receptors:
pathological glucose tolerance caused by binding to insulin receptors
effect of IGF-1 on IGF-1 receptors:
causes growth of bone, cartilage, skeletal muscle, skin, soft tissue, and organs
↑ secretion of somatostatin from the hypothalamus → ↓ serum GH and IGF-1 (negative feedback)
pathophys of gh in pit adenoma
1)acromegaly sx
Overproduction of GH →
abnormally high serum IGF-1 levels → overstimulation of cell growth and proliferation
2) mass effect
-Tumor mass compresses optic nerve→ blurred vision/ bitemporal hemianopsia
-hyperprolactinemia
d/2 damage to pit stalk by tumor
3)hypogondism
hypopituitarism of other pituitary hormones:
gonadotropins → ↓ LH and FSH → ↓ estrogen and testosterone → infertility & amenorhoea &
sx of acromegaly
MASS EFFECT
- (bitemporal hemianopsia),
♀: secondary amenorrhea, galactorrhea,
♂: Erectile dysfunction, decreased libido, ↓ testicular volume
SOFT TISSUE
hyperhidrosis by enlarged sweat glands
Deepening of the voice, macroglossia & obstructive sleep apnea
Carpal tunnel syndrome from connective tissue proiferation in tendon sheath
SKELETAL sx
Large skull, coarsened features: enlarged nose, forehead, and jaw
diastema (gap tooth)
Widened hands, fingers, and feet
CDV sx
- LVH
- cardiomyopathy
ORGAN ENLARGEMENT
-kidneys & thyroid
dg
hormone test
-elevated serum IGF-1
OGTT
- measure baseline GH after 2hrs of intake of 75g glucose
- UNSUPRESSED GH indicates acromegaly
- supressed gh rules out acromegaly
MRI
-for visible mass on pituitary
abscence shows extrapituitary cause
rx of pituitary adenoma
Surgery: transsphenoidal adenomectomy (thru the nose)
RADIATION THERAPY W/ GAMMA KNIFE
medication:
SOMATOSTATIN ANALOG
(ocreotide/ lanreotide)
dopamine agonist for hyperprolactinoma
complication of pituitary adenoma
Cardiovascular complications (cause death) (CHF, hypertension, arrhythmia, valvular disease, hypertrophy)
Diabetes mellitus