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1

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

2

definitive diagnostic test for hypopituitarism

absent or low levels of GH in response to stimulation

3

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

pathologic short stature

4

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

constitutional short stature

5

height stay parallel to the growth curve

familial short stature

6

height parallel to the growth curve but is much more marked

prenatal short stature

7

management of hypopituitarism

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

8

when to stop hGH in hypopituitarism

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

9

cardinal clinical feature of gigantism

longitudinal growth acceleration due to GH excess

10

half-life of GH

22mins

11

Dx laboratory modality in hyperpituitarism

serum somatomedin C(IGF-I)

12

mgt of hyperpituitarism

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

13

cardinal features of diabetes insipidus

polyuria and polydipsia

14

vasopressin deficiency

central DI

15

vasopressin insensitivity

nephrogenic DI

16

how to differentiate central vs nephrogenic DI

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

17

what secretes vasopressin

posterior pituitary

18

where is vasopressin synthesized

paraventricular and supraoptic nuclei of the thalamus

19

management of central DI

fluids; lon-acting vasopressin analog(dDAVP)

20

etilogy of central DI

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

21

management of nephrogenic DI

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

22

etiology of nephrogenic DI

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

23

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

precocious puberty

24

true precocious puberty

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

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

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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

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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