Lecture 93 - Peds Endo

Question 1

what is the main mediator of growth inutero

meditor of growth post natal

Answer 1

insulin

Growth hormone

Question 2

what is an environtal cause for abd tall stature as a child

Answer 2

obesity

Question 3

5 syndormes associated with abn tall stature in children

Answer 3

® Klinefelter Syndrome -- XXY 
Marfans 
Beckwith Wiedmann Syndrome
Soto's Syndrome 
					® Weaver's Syndrome --

Question 4

what is Beckwith Wiedmann Syndrome

Answer 4

Wilm’s tumor, Macroglossia, Organomegaly, Macrosomia,

Question 5

who should be evalulated for short stature in children?

Answer 5

patient is crossing percentiles, below 3rd percentile, outside of genetic expectations (>2 STD), child/family is concerned

Question 6

karyotype of Turner syndrome -

what other important gene is also impicated

clinical featurse

Answer 6

Turner Syndrome – Chromosomes: 45X;
– Webbing of the neck, wide nipples, lymphedema

							– SHOX is also implicated

Question 7

what is SHOX?

assocaitd with what causes of shortness?

Answer 7

SHOX = Short Stature Homeobox Gene

Turner syndrome
Idiopathic short stature

Question 8

Other causes of short statures

Answer 8

Endocrine – hypothyroid, GH def, GH rsistance,

Small for gestational Age

Prader willin

Question 9

Short stature inteventions

Answer 9

® Treat underyling cause of the short stature

Medical: GH

				® Other Medical Therapies to delay puberty and closure of growth plates

Question 10

what significes onset of puberty in boys and girls

Answer 10

boys -testicular enlargement

Girls – breast buds, with menarch

Question 11

threshold of age for dx of delayed puberty?

Answer 11

13 yo

Question 12

ddx of delayed puberty in girls

Answer 12

Turners
Gonadal failure 
Pituitary
hypothalamsu
Adrongen Insensitivity syndrome -- 

Constitutional delay –late bloomer

Question 13

ddx of delayed puberty in boys

Answer 13

late bloomer 
Gonadal failire (Klinefetlers, Cryptorchidism, XY gonadal dysgenes) 

Pitutiary
Hypothalamus

Question 14

treatment of dleayed puberty boys vs grils

Answer 14

□ Girls – low dose estrogen, then increase dose, then add cyclin progesterone
□ Boys – Low dose testosterone, increase dose overtime

Question 15

what is true central Precocious puberty?

more common in which sex?

Answer 15

Gonadotropin Dependent;

Girls – Breast and Pubic Hair
Boys – Testicular growth and pubic hair

Question 16

what is peripheral precocious puberty?

Answer 16

gonadotropin Independent

Question 17

Girls – causes of precocious puberty

Answer 17

Exogenous steroids
Ovarian cyst,
ectropic estrogen, hypothyroidism, non classic CAH

Question 18

Triad of symptoms of Mccune Albright Syndrome

-

Answer 18

◊ Triad: Ovarian hypersecretion, Polyostotic Fibrous Dysplasia of bone, and “Coast of Maine” Café au Lait Spots (doesn’t cross midline)

Question 19

how does primary hypothyroid cause PP in girls?

Answer 19

If Primary — TSH is elevated. TSH has same subunit as FSH and may appear to ovaries as FSH and trigger estrogen secretion

Question 20

etiologies of PP in boys?

Answer 20

Testicular Hypersecretion
exogenous/topical testosterone,

Tumor of testical or adrenal gland, McCune Albright Syndrome, Ectopic Tumor

Question 21

treatment of Central PP

Answer 21

GNRH Agonists – stops the pulsing by overwhelming the receptor
Leuprolide Depot
Histrelin

Question 22

treatment of Peripheral PP

Answer 22

® Aromatase Inhibitors –
® Ketoconazole
® Spironolactone
® Estrogen Receptor Blockers

Question 23

Mneumonic for digeorge syndrome endocrine and non endocrime manifestations

Answer 23

(CATCH 22)

Question 24

(CATCH 22)

Answer 24

◊ Cardiac Abnormalities 
						◊ Abnormal Facies 
						◊ Thymic Aplasia 
						◊ Cleft Palate
						◊ HypoPTH/HypoCalcemia 
						◊ 22q Deletion on Chr 22