Head And Neck

Question 1

Symptoms of Kawasaki (mucocutaneous lymph node syndrome)

Answer 1

<5 years

Fever at least 5 days plus 4 of following: 
Conjunctivitis
Oral cavity changes
Rash
Erythema palms/soles
Cervical adenopathy > 1.5 cm

Question 2

What is chance of developing coronary abnormalities in untreated Kawasaki?

Answer 2

20%

0.5% MI

Question 3

What causes chronic granulomatous disease?

Answer 3

Primary immunodeficiency disorder
Disorder of NADPH oxidase complex - failure to generate superoxide,H2O2, hypochlorite needed for leukocyte respiratory burst

Question 4

How does chronic granulomatous disease manifest?

Answer 4

Recurrent infections and granulomatous formations- usu skin, lung, soft tissue

Question 5

What is Chediak-Higashi syndrome?

Answer 5

Immunodeficiency disorder, defect in neutrophil function

LYST gene deficiency, impaired I travel pulse vesicles, chemotacis, degranulation

Question 6

Where does castle man disease commonly occur?

Answer 6

Most common level V but can be level 2-3.

Lymphoproliferative dz

Question 7

Describe histology for castleman dz.

Answer 7

2 types
Most common- hyaline vascular-low recurrence after excision
Or
Plasma cell- multi centric, systemic symptoms, treat with chemo

Question 8

What percent of medullary thyroid carcinoma is hereditary in children?

Answer 8

95% is hereditary

Medullary ca is 5% of peds thyroid ca

From parafollicular c cells

CEA and calcitonin related to c cell mass- great tumor markers

Question 9

Cause of MEN 2A and MEN 2 B

Answer 9

RET protooncogene