PGH

Question 1

What is the predominant inheritance pattern of patients diagnosed with chronic granulomatous diseases (CGD)?

Answer 1

X-linked recessive

Question 2

Why are patients with LAD III deficiency prone to develop bleeding?

Answer 2

Nonfunctional B2 integrin in platelets

Question 3

A 30/F was having recurrent folliculitis for six years now. She was eventually diagnosed to have chronic granulomatous disease based on wound culture. Which of the following bacteria would you least expect to find if cultures were done on her skin lesions?

a. Burkholderia cepacia
b. Serratia marcescens
c. Staphylococcus aureus
d. Streptococcus mitis

Answer 3

Streptococcus mitis

Question 4

A 28/F was diagnosed to have chronic granulomatous disease after presenting with recurrent skin infections. Which of the following medications will you prescribe to her to prevent bacterial infections?

Answer 4

Trimethoprim/sulfamethoxazole

Question 5

Which of the following is the most frequent type of severe combined immunodeficiency?

Answer 5

Absence of T and NK cells

Question 6

Which of the following is the most common primary immunodeficiency disease?

Answer 6

IgA deficiency

Question 7

Caused by defect in FAS mediated apoptosis of the lymphocytes

Answer 7

ALPS

Question 8

Hallmark is oresence of aB double negative T cells

Answer 8

ALPS

Question 9

What is the key effector cell in allergic rhinitis and asthma

Answer 9

Mast cells

Question 10

Patients diagnosed with Wiskott-Aldrich Syndrome is characterized by

Answer 10

Typically characterized by recurrent bacterial infections, eczema, bleeding caused by thrombocytopenia

Question 11

For chronic urticaria that failed to respi d to combi H1 and CystLT1

Answer 11

Omalizumab

Question 12

First line treatment for most forms of urticaria

Answer 12

H1 antihistamins- chlorpheniramine or diphenhydramine

May inc up to 4x

Question 13

For chronic idiopathic urticaria that is severe and poorly responsive to tx OR if gluc is required

Answer 13

Cyclosporine

Question 14

Impt add-on therapy to H1 antihistamine for urticaria

Answer 14

CystLT1 rx antagonist- montelukast, zafirlukast

Question 15

Systemic Glucocorticoids is avoided in

Answer 15

1. Idiopathic u
2. Allergen-induced u
3. Physical u

P-I-A

Question 16

Among the early mediators of anaphylaxis, which of the following causes cutaneous flushing and attracts eosinophils and basophils to the site of mast cell activation?

Answer 16

Prostaglandin D2