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Flashcards in peds16 Deck (25):
1

mixed gonadal dysgenesis

karyotype with 45XO/46XY mosaicism; ambig genitalia (usually); testes on one side and streak gonad on the other; fallop tubes may be present bilat

2

true hermaphroditism

ambig genitalia; both ovarian and testicular gonadal tissue; usually 46xx but can be 46xy

3

increased BP with ambig genitalia in a female suggests what?

CAH with 11b-OH def and decr bp suggests adrenal insuff

4

most common cause of female pseudohermaphroditism

CAH caused by 21-hydrox def, which causes incr 17-oh progesterone (11B and 3B- hydrox def are other causes)

5

what regulates each of the products of the adrenal cortex?

cortisol and androgens reguated by ACTH; aldosterone regulated by RAAS

6

most common cause of secondary adrenal insuff

iatrogenic caused by supp of the hypothalamus due to longterm exposure to steroids

7

3 different subtypes of 21-hydrox def CAH

classic salt wasting CAH (cortisol and aldo def); simple virilizing CAH (cortisol def); nonclassic CAH (late onset at 4-5 yo with mild cortisol def and no mineralocorticoid involvement)

8

11-b hydrox def

present like 21-hydrox kids byt they are hypertensive and hypokalemic; have incr levels of 11-deoxycortisol aka specific compound S

9

3-b hydrox dehyd def

rare; salt wasting crisis, glucocorticoid def, and ambig genitalia; incr leveles of DHEA and 17-hydroxypregnenolone

10

management of CAH

cortisone to suppress ACTH, mineralocorticoid replacement (fluorocortisol)

11

addison's disease

autoimmune destruction of the adrenal cortex; may be associated with other autoimmune diseases

12

waterhouse-friderichsen syndrome

septicemia associated with meningococcemia, cause of adrenal insuff

13

adrenal crisis

a medical emergency of adrenal insuff; treat with IV fluids with 5% dextrose in normal saline to correct hyptension and hyponatremia and to prevent hypoglycemia; parenteral steroids are given until the patient is stabilized

14

diff between cushing syndrome and cushing disease

disease is caused by excessive ACTH production from a pit tumor, such as microadenoma; cushing syndrome is caused by adrenal tumors

15

genetics of DM type 1

approx 95% of patients have HLA haplotype DR3 or DR$

16

antibodies that may be seen in type 1 dm patients

antibodies against insulin, glutamic acid decarboxylase, islet cell antibodies

17

DKA is initial presentation of type 1 dm in what percent?

25%

18

honeymoon period of type 1 DM

lasts months to a few years; transient recovery of your islet cells after dx but then goes away

19

somogyi phenomenon

evening dose of insulin is too high cuaseing hypoglycemia in the morning and release of counter-reg hormones (epi and glucagon) which causes high blood glucose and ketones in the morning

20

definition of DKA

hyperglycemia greater than 300 mg/dL with ketonuria and a serum bicard level less than 15 mmol/L or a serum pH less than 7.30

21

hyperkalemia in DKA caused by what?

potassium moves out of cells in the face of acidosis

22

management of DKA

fluids and electrolyte therapy; potassium repletion (even with normal serum potassium); insulin

23

complications of DKA

cerebral edema (due to drop in gluc with tx), severe hypokalemia, hypocalcemia (due to excessive use of potassium phosphate or osmotic losses)

24

presentation of hypothyroidism

suboptimal growth velocity (less than 2 inches per year) with delayed bone age; goiter, myxedema (or puffy skin or dry skin), amenorrhea or oligomenorrhea

25

most common metabolic disorder

congenital hypothyroidism; it is evaluated on new born screen