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pediatrics > endoped > Flashcards

endoped Flashcards

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
Q

conditions causing precocious puberty

A

gonadtropin-dependent puberty; idiopathic; brain tumors; severe head trauma; hydrocephalus; prolonged and untreated hypothyroidism; ovarian tumors; exogenous estrogen

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26
Q

breast development in the firts 2 yrs of life; regress after 2yrs and rarely progressive

A

premature thelarche

27
Q

pubic hair; early maturational event of adrenal androgen production

A

premature adrenarche

28
Q

Tx of precocious puberty

A

Leuprolide acetate 0.25-3 mg/kg IM every 4wks for true precocious puberty; to decrease serum sex hormones to prepubertal levels

29
Q

pathophysiology of Graves dse

A

autoantibodies stimulate hypersecretion of thyroid hormones

30
Q

why is there exophthalmos in Graves dse

A

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
Q

earliest sign of Graves dse

A

emotional disturbance with motor hyperactivity

32
Q

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

A

thyroid storm

33
Q

Tx of thyroid storm

A

Methimazole

34
Q

Definitive Tx of Graves dse

A

radioactive iodine ablation or thyroidectomy

35
Q

most common cause of hypothyroidism

A

thyroid dysgenesis

36
Q

most common cause of thyroid dse in children and adolescents

A

thyroiditis

37
Q

who are at more risk of having hypocalcemia in NB with hypoparathyroidism

A

premature infants; in infants with asphyxia at birthy; and infants of diabetic mothers

38
Q

management of hypoparathyroidism

A

calcium gluconate IV for neonatal tetany; calcitriol

39
Q

most common cause of hyperparathyroidism in children

A

single benign adenoma manifested after 10 years of age

40
Q

symptoms of hypercalcemia

A

painful bones; renal stones; abdominal groans; psychic moans; fatigue overtones

41
Q

managemtn of hyperparathyroidism

A

parthyroidectomy

42
Q

what part of the adrenal cortex secretes aldosterone

A

zona glomerulosa

43
Q

what part of the adrenal cortex secretes cortisol and androgens

A

zona fasciculata

44
Q

what part of the adrenal cortex secretes androgen

A

zona reticularis

45
Q

what enzyme deficiency accounts ofr 90% of px with CAH

A

21-hydroxylase deficiency

46
Q

deficient production of cortisol or aldosterone due to congenital or acquired lesions of the hypothalamus pituitary gland or adrenal cortex

A

Addison disease

47
Q

most prominent clinical manifestation in Addison dse

A

increased skin pigmentation

48
Q

Lab findings in Addison dse

A

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
Q

Management of Addison dse

A

D5 0.9 NSS IV to correct hypoglycemia and Na loss; Hydrocortisone succinate IV for 48hr then oral

50
Q

characteristic pattern of obesity associated hypertension which is the result of high blood levels of cortisol resulting from hyperfunction of the adrenal cortex

A

Cushing’s sundrome

51
Q

rounded face; prominent cheeks; moon facies; buffalo hump; generalized obesity; hypertension; impaired growth; purplish striae on hips abdomen and thighs

A

Cushing syndrome

52
Q

cathecolamine-secreting tumor arising from the chromaffin cells

A

pheochromocytoma

53
Q

where is the tumor in pheochromocytoma more often located

A

on the right side

54
Q

Dx of pheochromocytoma

A

elevated blood and urinary levels of cathecolamines and their metabolites; urinary excretion of VMA is increased; CT UTZ or MRI for tumors

55
Q

short stature; webbing of the neck; pectus carinatum/excavatum; cubitus valgus; right-sided CHD; hypertelorism; ptosis; micrognathia; moderate MR;delayed puberty; cryptorchidism

A

Noonan syndrome

56
Q

most common sex chromosomal aneuploidy in males

A

Klinefelter syndrome

57
Q

Tall; slim; underweight; long legs; small testes and penis; gynecomastia; azoospermia; associated with leukemia and lymphoma(15-30yo)

A

Klinefelter syndrome

58
Q

management of Klinefelter syndrome

A

replacement therapy tiwh a long-acting testosterone preparation at 11-12 yrs old; Enanthate ester

59
Q

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

A

Turner syndrome

60
Q

most effective in inducing puberty in px with Turner syndrome

A

Premarin(conjugated estrogen)

61
Q

glucose >300mg/dL; ketonemia; acidosis; glucosuria; ketonuria; precipitated by trauma infections vomiting and psychologic disturbances in px with type I DM

A

DKA

62
Q

represents the fraction of Hgb to which glucose has been nonenzymatically attached in the blood stream

A

Glycohemoglobin(HBA1c)

63
Q

predicts risk of progression of diabetic complications

A

glycated Hgb

pediatrics (16 decks)

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