Endocrinology, infectious disease

Question 1

How does someone with 21 alpha hydroxylate deficiency present

Answer 1

Presents with salt wasting: Hyponatremia and Hyperkalemia, with hypotension

Elevated 17-hydroxyprogesterone

Treatment

1. Glucocorticoid replacement with hydrocortisone
2. Mineralocorticoid replacement with fludrocortisone

Question 2

How often should you monitor 17-hydroxyprogesterone level in infants and in children

Answer 2

EVery 3 months in infants
Every 4-12 months in children
Bone age films taken every 6 months

Question 3

What is the definition of precocious puberty

Answer 3

Puberty before 8 in girls, before 9 in boys

Question 4

What are two main differentials for precocious puberty

Answer 4

1. Gonadotropin dependant precocious puberty: HPG axis gets activated on own - requires brain imaging
2. Gonadotropin independent precocious puberty: Activates by adrenals and gonads - CAH, tumors, and McCune-Albright syndrome

Question 5

What should you do for diagnosis of precocious puberty

Answer 5

Obtain X-rays of wrist to determine bone age

Labs: Serum estradiol or testosterone level, 17-OHP, basal and GnRH stimulated LH, DHEA

Question 6

What is the treatment for precocious puberty

Answer 6

GnRH agonists only for GDPP (the one activated by HPG axis activation)

Question 7

How does treatment differ for fever without a source for infants 0-28 days, 1-3 months, and 3-36 months

Answer 7

1. 0-28 days: See previous cards
2. 1-3 months: Treat with ceftriaxone if WBC > 15 or < 5
3. 3-36 months: If vaccinated, bacteremia and meningitis risk is low, treat with ceftriaxone if WBC > 15

For fever, UTI is most common cause, and less than 3 months it is uncircumscribed boys, over 3 months it’s girls

Question 8

What is Kawasaki’s disease

Answer 8

Medium vessel vasculitis of childhood that develops coronary artery aneurysms and subsequent myocardial ischemia, happens mainly in asians

Question 9

How do you diagnose Kawasaki’s disease

Answer 9

Fever for >5 days and 4/5 of

1. bilateral conjunctivitis
2. strawberry tongue
3. adenopathy
4. truncal rash
5. Hand/foot desquamation

Will see elevated ESR/CRP

Question 10

What is the treatment for Kawasaki’s disease

Answer 10

High dose aspirin during acute phase to reduce cardiac abnormalities

IVIG to prevent coronary aneurysms

When you enter into convalescence stage, switch to low dose aspirin for antiplatelet effect

Question 11

What must you monitor regularly in Kawasaki’s disease

Answer 11

The heart - regular echocardiograms

Question 12

What are complications of Kawasaki’s disease

Answer 12

Myocardititis
Pericarditis
Coronary artery aneurysm