endoped Flashcards
deficiency of growth hormone with or without a deficiency of other pituitary hormones; may be congenital or acquired; atrophy of adrenal cortex thyroid and gonads result in weight loss asthenia sensitivity to cold and absence of sweating
hypopituitarism
definitive diagnostic test for hypopituitarism
absent or low levels of GH in response to stimulation
normal range for height; over time it starts falling off the height curve
pathologic short stature
normal range for height; normal final adult height is reached but the growth spurt and puberty are delayed
constitutional short stature
height stay parallel to the growth curve
familial short stature
height parallel to the growth curve but is much more marked
prenatal short stature
management of hypopituitarism
human growth hormone 0.18-0.3 mg/kg/wk SC in 6-7 divided doses
when to stop hGH in hypopituitarism
growth rate < 1in/yr and BA>14yrs in girls and >16yrs in boys
cardinal clinical feature of gigantism
longitudinal growth acceleration due to GH excess
half-life of GH
22mins
Dx laboratory modality in hyperpituitarism
serum somatomedin C(IGF-I)
mgt of hyperpituitarism
if with well circumscribed pituitary adenoma-transsphenoidal surgery; pituitary radiation and medical therapy; somatostatin analog(Octreotide)
cardinal features of diabetes insipidus
polyuria and polydipsia
vasopressin deficiency
central DI
vasopressin insensitivity
nephrogenic DI
how to differentiate central vs nephrogenic DI
water deprivation test(vasopressin insensitivity) and ADH administration(vasopressin deficiency)
what secretes vasopressin
posterior pituitary
where is vasopressin synthesized
paraventricular and supraoptic nuclei of the thalamus
management of central DI
fluids; lon-acting vasopressin analog(dDAVP)
etilogy of central DI
congenital; trauma; tumors; autoimmune; infection; drugs(ethanol; phenytoin)
management of nephrogenic DI
treat underlying disorder; thiazides(decrease urine flow to DCT; induce formation of functional receptors)
etiology of nephrogenic DI
congenital; hypercalcemia; hypokalemia; renal dse(PCKD; CRF); drugs(lithium; amphotericin; rifampicin)
onset of secondary sexual characteristics before 8 yrs old in girls and 9yo in boys
precocious puberty
true precocious puberty
gonadotropin-dependent puberty(increased hormone secretion leading to maturation of gonads)
conditions causing precocious puberty
gonadtropin-dependent puberty; idiopathic; brain tumors; severe head trauma; hydrocephalus; prolonged and untreated hypothyroidism; ovarian tumors; exogenous estrogen
breast development in the firts 2 yrs of life; regress after 2yrs and rarely progressive
premature thelarche
pubic hair; early maturational event of adrenal androgen production
premature adrenarche
Tx of precocious puberty
Leuprolide acetate 0.25-3 mg/kg IM every 4wks for true precocious puberty; to decrease serum sex hormones to prepubertal levels
pathophysiology of Graves dse
autoantibodies stimulate hypersecretion of thyroid hormones
why is there exophthalmos in Graves dse
due to antibodies against antigens shared by the thyroid and eye muscle(TSH receptors identified in reto-orbital adipocytes and may be a target for antibodies)
earliest sign of Graves dse
emotional disturbance with motor hyperactivity
acute life-threatening surge of thyroid hormone usually precipitated by surgery; trauma; infection; acute iodine load; or long-standing hyperthyroidism; presents with HR>140bpm; heart failure; agitation; delirium; psychosis; stupor; and/or coma
thyroid storm
Tx of thyroid storm
Methimazole
Definitive Tx of Graves dse
radioactive iodine ablation or thyroidectomy
most common cause of hypothyroidism
thyroid dysgenesis
most common cause of thyroid dse in children and adolescents
thyroiditis
who are at more risk of having hypocalcemia in NB with hypoparathyroidism
premature infants; in infants with asphyxia at birthy; and infants of diabetic mothers
management of hypoparathyroidism
calcium gluconate IV for neonatal tetany; calcitriol
most common cause of hyperparathyroidism in children
single benign adenoma manifested after 10 years of age
symptoms of hypercalcemia
painful bones; renal stones; abdominal groans; psychic moans; fatigue overtones
managemtn of hyperparathyroidism
parthyroidectomy
what part of the adrenal cortex secretes aldosterone
zona glomerulosa
what part of the adrenal cortex secretes cortisol and androgens
zona fasciculata
what part of the adrenal cortex secretes androgen
zona reticularis
what enzyme deficiency accounts ofr 90% of px with CAH
21-hydroxylase deficiency
deficient production of cortisol or aldosterone due to congenital or acquired lesions of the hypothalamus pituitary gland or adrenal cortex
Addison disease
most prominent clinical manifestation in Addison dse
increased skin pigmentation
Lab findings in Addison dse
hyponatremial; hypochloremia; hyperkalemia; inc urinary excreation of Na and Cl; hypoglycemia; measurement of plasma or serum level of cortisol before and after administration of ACTH
Management of Addison dse
D5 0.9 NSS IV to correct hypoglycemia and Na loss; Hydrocortisone succinate IV for 48hr then oral
characteristic pattern of obesity associated hypertension which is the result of high blood levels of cortisol resulting from hyperfunction of the adrenal cortex
Cushing’s sundrome
rounded face; prominent cheeks; moon facies; buffalo hump; generalized obesity; hypertension; impaired growth; purplish striae on hips abdomen and thighs
Cushing syndrome
cathecolamine-secreting tumor arising from the chromaffin cells
pheochromocytoma
where is the tumor in pheochromocytoma more often located
on the right side
Dx of pheochromocytoma
elevated blood and urinary levels of cathecolamines and their metabolites; urinary excretion of VMA is increased; CT UTZ or MRI for tumors
short stature; webbing of the neck; pectus carinatum/excavatum; cubitus valgus; right-sided CHD; hypertelorism; ptosis; micrognathia; moderate MR;delayed puberty; cryptorchidism
Noonan syndrome
most common sex chromosomal aneuploidy in males
Klinefelter syndrome
Tall; slim; underweight; long legs; small testes and penis; gynecomastia; azoospermia; associated with leukemia and lymphoma(15-30yo)
Klinefelter syndrome
management of Klinefelter syndrome
replacement therapy tiwh a long-acting testosterone preparation at 11-12 yrs old; Enanthate ester
edema of the dorsa of the hands and feet; loose skin folds at the nape; LBW: decreased length; webbed neck; low posterior hairline; small mandible; prominent ears; epicanthal folds; high arched palate; widely spaced nipples; hyperconvex fingernails; delayed sexual maturation
Turner syndrome
most effective in inducing puberty in px with Turner syndrome
Premarin(conjugated estrogen)
glucose >300mg/dL; ketonemia; acidosis; glucosuria; ketonuria; precipitated by trauma infections vomiting and psychologic disturbances in px with type I DM
DKA
represents the fraction of Hgb to which glucose has been nonenzymatically attached in the blood stream
Glycohemoglobin(HBA1c)
predicts risk of progression of diabetic complications
glycated Hgb
