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endoped Flashcards

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1
Q

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

A

hypopituitarism

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2
Q

definitive diagnostic test for hypopituitarism

A

absent or low levels of GH in response to stimulation

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3
Q

normal range for height; over time it starts falling off the height curve

A

pathologic short stature

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4
Q

normal range for height; normal final adult height is reached but the growth spurt and puberty are delayed

A

constitutional short stature

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5
Q

height stay parallel to the growth curve

A

familial short stature

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6
Q

height parallel to the growth curve but is much more marked

A

prenatal short stature

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7
Q

management of hypopituitarism

A

human growth hormone 0.18-0.3 mg/kg/wk SC in 6-7 divided doses

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8
Q

when to stop hGH in hypopituitarism

A

growth rate < 1in/yr and BA>14yrs in girls and >16yrs in boys

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9
Q

cardinal clinical feature of gigantism

A

longitudinal growth acceleration due to GH excess

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10
Q

half-life of GH

A

22mins

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11
Q

Dx laboratory modality in hyperpituitarism

A

serum somatomedin C(IGF-I)

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12
Q

mgt of hyperpituitarism

A

if with well circumscribed pituitary adenoma-transsphenoidal surgery; pituitary radiation and medical therapy; somatostatin analog(Octreotide)

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13
Q

cardinal features of diabetes insipidus

A

polyuria and polydipsia

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14
Q

vasopressin deficiency

A

central DI

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15
Q

vasopressin insensitivity

A

nephrogenic DI

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16
Q

how to differentiate central vs nephrogenic DI

A

water deprivation test(vasopressin insensitivity) and ADH administration(vasopressin deficiency)

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17
Q

what secretes vasopressin

A

posterior pituitary

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18
Q

where is vasopressin synthesized

A

paraventricular and supraoptic nuclei of the thalamus

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19
Q

management of central DI

A

fluids; lon-acting vasopressin analog(dDAVP)

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20
Q

etilogy of central DI

A

congenital; trauma; tumors; autoimmune; infection; drugs(ethanol; phenytoin)

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21
Q

management of nephrogenic DI

A

treat underlying disorder; thiazides(decrease urine flow to DCT; induce formation of functional receptors)

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22
Q

etiology of nephrogenic DI

A

congenital; hypercalcemia; hypokalemia; renal dse(PCKD; CRF); drugs(lithium; amphotericin; rifampicin)

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23
Q

onset of secondary sexual characteristics before 8 yrs old in girls and 9yo in boys

A

precocious puberty

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24
Q

true precocious puberty

A

gonadotropin-dependent puberty(increased hormone secretion leading to maturation of gonads)

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25
conditions causing precocious puberty
gonadtropin-dependent puberty; idiopathic; brain tumors; severe head trauma; hydrocephalus; prolonged and untreated hypothyroidism; ovarian tumors; exogenous estrogen
26
breast development in the firts 2 yrs of life; regress after 2yrs and rarely progressive
premature thelarche
27
pubic hair; early maturational event of adrenal androgen production
premature adrenarche
28
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
29
pathophysiology of Graves dse
autoantibodies stimulate hypersecretion of thyroid hormones
30
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)
31
earliest sign of Graves dse
emotional disturbance with motor hyperactivity
32
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
33
Tx of thyroid storm
Methimazole
34
Definitive Tx of Graves dse
radioactive iodine ablation or thyroidectomy
35
most common cause of hypothyroidism
thyroid dysgenesis
36
most common cause of thyroid dse in children and adolescents
thyroiditis
37
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
38
management of hypoparathyroidism
calcium gluconate IV for neonatal tetany; calcitriol
39
most common cause of hyperparathyroidism in children
single benign adenoma manifested after 10 years of age
40
symptoms of hypercalcemia
painful bones; renal stones; abdominal groans; psychic moans; fatigue overtones
41
managemtn of hyperparathyroidism
parthyroidectomy
42
what part of the adrenal cortex secretes aldosterone
zona glomerulosa
43
what part of the adrenal cortex secretes cortisol and androgens
zona fasciculata
44
what part of the adrenal cortex secretes androgen
zona reticularis
45
what enzyme deficiency accounts ofr 90% of px with CAH
21-hydroxylase deficiency
46
deficient production of cortisol or aldosterone due to congenital or acquired lesions of the hypothalamus pituitary gland or adrenal cortex
Addison disease
47
most prominent clinical manifestation in Addison dse
increased skin pigmentation
48
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
49
Management of Addison dse
D5 0.9 NSS IV to correct hypoglycemia and Na loss; Hydrocortisone succinate IV for 48hr then oral
50
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
51
rounded face; prominent cheeks; moon facies; buffalo hump; generalized obesity; hypertension; impaired growth; purplish striae on hips abdomen and thighs
Cushing syndrome
52
cathecolamine-secreting tumor arising from the chromaffin cells
pheochromocytoma
53
where is the tumor in pheochromocytoma more often located
on the right side
54
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
55
short stature; webbing of the neck; pectus carinatum/excavatum; cubitus valgus; right-sided CHD; hypertelorism; ptosis; micrognathia; moderate MR;delayed puberty; cryptorchidism
Noonan syndrome
56
most common sex chromosomal aneuploidy in males
Klinefelter syndrome
57
Tall; slim; underweight; long legs; small testes and penis; gynecomastia; azoospermia; associated with leukemia and lymphoma(15-30yo)
Klinefelter syndrome
58
management of Klinefelter syndrome
replacement therapy tiwh a long-acting testosterone preparation at 11-12 yrs old; Enanthate ester
59
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
60
most effective in inducing puberty in px with Turner syndrome
Premarin(conjugated estrogen)
61
glucose >300mg/dL; ketonemia; acidosis; glucosuria; ketonuria; precipitated by trauma infections vomiting and psychologic disturbances in px with type I DM
DKA
62
represents the fraction of Hgb to which glucose has been nonenzymatically attached in the blood stream
Glycohemoglobin(HBA1c)
63
predicts risk of progression of diabetic complications
glycated Hgb

pediatrics (16 decks)

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