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Flashcards in Pedia Deck (50):
1

elevated serum 17-hydroxyprogesterone

Congential adrenal hyperplasia - usually due to deficiency 21-hydroxylase-imparied corticol synthesis; cannot negatively inhibit hypothalamus or pituitary; inc ACTH; inc adrenal androgens

2

symptoms of congenital adrenal hyperplasia, female

ambiguous genitalia at birth or hirsutism and virilism w/ norm menstruation

3

symptoms of congenital adrenal hyperplasia, male

adrenal crisis at birth or precocious puberty

4

Treatment of CAH

cortisol

5

MCC of genital ambiguity

CAH

6

Places newborns at risk for neonatal adrenal crisis due to sodium loss due to absence of aldosterone

CAH

7

what is the cause of irregular menstrual cycles in pubertal females

anovulatory cycles due to immaturity of hypothalamic-pituitary-gonadal axis, not producing adequate proportions of FSH, LH to induce ovulation

8

Pituitary gland location

Sella turcica

9

Rathke's pouch gives rise to

Anterior pituitary gland

10

MC type of pituitary tumor in children

Rathke pouch craniopharyngioma

11

MC pituitary tumor in adults

Prolactinoma

12

DOC for prolactinoma

Bromocriptine (dopamine agonist)

13

Posterior lobe hormones

OxytocinADH

14

Definitive diagnosis of hypopituitarism

Absent or low levels of GH

15

dz as w/ asymmetric septal hypertrophy and transposition of the gerat vessels

maternal diabetes

16

Polyuria in children

>5cc/kg/hour

17

DI: Vasopressin deficiency

Central

18

DI: Vasopressin insensitive kidney

Nephrogenic

19

ADH acts on which part of kidneys

CD

20

Etiology of 50% of DI

Brain tumor

21

SG of urine in DI

1.001-1.005

22

Urine osmolality in DI

50-200 mosm/kg

23

Electrolyte derrangement in DI

HypernatremiaHyperchloremia

24

3 P'sPrecocious pubertyPigmentation (unilateral cafe au lait spots - large, irregular borders, no freckling)Polyostotic fibrous dysplasia (multiple bone fractures)

McCune Albright syndrome

25

GI tract polyposis, mucocutaneous pigmentation-estrogen secreting tumor: precocious puberty

Peutz-Jeghers syndrome

26

T/F Peutz-Jeghers polyps are neoplasms

F, hamartomas

27

disorder due to autonomous stimulation of aromatase enzyme prod of estrogens by the ovaries

McCune-Albright syndrome

28

T/F There is an increased risk for cancer in Peutz-Jeghers syndrome

F

29

Most common polyp in GIT

Hypoplastic polyp

30

T/F Hypoplastic polyp is a neoplasm

F, hamartoma

31

MCC of precocious puberty

Hypothalamic hamartoma

32

Precocious puberty in girls

Breast bud before 8 years

33

Precocious puberty in boys

Testicular enlargement before 9

34

Coast of maine pattern

Cafe-au-laite spots of McCune Albright syndrome

35

Treatment for McCune Albright syndrome

Leuprolide acetate

36

47XXY

Klinefelter's syndrome

37

Klinefelter is phenotypically male vs female

Male

38

-hypogonadism-testicular atrophy, tall stature, long extremities, gynecomastia, female hair distribution

Klinefelter's syndrome

39

tall stature, emaciated extremities, arachnodactyly, hypermobility of joints, *upward lens dislocation*, *aortic root dilation*

Marfan

40

Marfan: Inheritance and mutation

Aut dom; Fibrillin-1 gene

41

stroke/thrombotic event; *fair complexion, *lens dislocated downward, *Mentally retarded

Homocystinuria

42

Homocystinuria: Inheritance and deficiency

Aut rec; cystathionine synthase deficiency

43

Homocystinuria: Elevated in blood

HomocysteineMethionine

44

Homocystinuria: Treatment

High doses Vit B6

45

Tall stature is defined as

Taller than 2SD above mean

46

Syndromes associated with tall stature

Sotos syndrome (cerebral gigantismMarfan syndrome

47

Short stature is defined as

>3SD below mean for age and gender

48

Causes of short stature

ConstitutionalHypopituitarism (absent or low GH)Hypothyroidism

49

Syndrome's associated with short stature

Turner syndrome (females)Noonan syndrome (males)

50

MCC of tall stature

Familial/normal variant