pituitry disorders Flashcards
gigantism and diabetes insipidus
what is gigantism
abnormal linear growth during childhood due to growth hormone excess while the epiphyseal growth plates are still open.
rf
associated with an ↑ incidence of pituitary tumors
Multiple endocrine neoplasia (MEN) type I
McCune-Albright syndrome
Neurofibromatosis
PP of gigantism
↑ growth hormone (GH) secretion from the anterior pituitary
→ GH induces IGF-1 synthesis
→ IGF-1 stimulates cell growth and proliferation and inhibits apoptosis
GHRH secretion from the hypothalamus (i.e., tumors)
increased IGF-1 binfing protein which increases IGF-1 half life
sx
Tall
↑ Growth of distal limbs (i.e., hands, feet, fingers, toes)
macroencephaly
Coarse facial features
frontal bossing (rihanna bosses fenty)
tumor mass sx
blurred vision, headache
dg
elevated IGF-1
elevated GH w/ OGTT shows pituitary causes
MRI
rx
transphenpoidal adeneectomy
somatostatin analog (ocreotide/ lanreotide)
complications
carpal tunnel
CDV
LFH, HF, Aneurysm
diabetes insipidus
secretion of or response to the vasopressin(ADH) is impaired, resulting in the production of very large quantities of dilute urine, (polyuria) often with dehydration and insatiable thirst.(polydipsia)
class of DI
Central diabetes insipidus (CDI) (most common form)
- lack of hypothalamal synthesis of ADH
- lack of ADH secretion from post pituitary
Nephrogenic diabetes insipidus (NDI) (rare)
-defective ADH receptors in the distal tubules and collecting ducts
causes of CDI
Primary defect in hypothal/ pituitary (1/3 cases)
secondaary 2/3
- cranial tumors/ metastasis
- post op removal of adenoma
- pituitaru ischemia in sheehan syndrome and meningitis
causes of NDI
hereditary defects ofdistal tubules & collecting duccts
acquired
- lithium medication
- hypokalemia
- hypercalcemia
- polycystic kidney disease
sx
Polyuria with dilute urine
Nocturia → Restless sleep, daytime sleepiness
Polydipsia
dehydration w/ altered mental status
dg
plasma & urine osmalality test
- mild hyponatremia
- high/normal plasma osmolality
- low urine osmolality
water deprivation test
- plasma osmolality increases
- urirne osmolality stays the same
desmopressin administration test (dx CDI vs NDI)
CDI:
- plasma osmolality becomes normal
- urine osmolality increases
NDI:
- plasma osmolality stays high
- urine osmolality stays low
describe water deprivation test
obtain baseline labs
pt doesnt drink for 2-3 hrs
test urine vol and osmolaltiy every hour
test plasma osmolality every 2hrs
continue until
-urine osmolality becomes normal RULES OUT DI as ADH release and effect on kidneys both func = primamry polydipsia
-no change in urine osmolality while plasma osmolality rises
plasma osmolality
if no change in urine and plasma osmolality increases apply desmopressin (synthetic ADH)
to dx betwen CDI vs NDI
monitor urine osmalilty every 30 mins for 2 hours
how does desmopressin dx between CDI & NDI
CDI: Urine osmolality rises after desmopressin administration (renal ADH receptors are intact).
NDI: Urine osmolality remains low after desmopressin administration (defective renal ADH receptors).