Immunology

Question 1

1 Make immunoglobulins and are responsible for immunity against extracellular bacteria.

2 Responsible for immunity against intracellular bacteria, viruses, and fungi.

Answer 1

B cells: Make immunoglobulins and are responsible for immunity against extracellular bacteria.

T cells: Responsible for immunity against intracellular bacteria, viruses, and fungi.

Question 2

• Most common (50%) immunodeficiency
• Typically present after 6 months of age with recurrent sinopulmonary, GI, and urinary tract infections with encapsulated organisms (H influenzae, Streptococcus pneumoniae, Neisseria meningitidis)
• Treat with IVIG (except for IgA deficiencies).

Answer 2

B cell deficiencies

Question 3

Tend to present earlier (1–3 months) with opportunistic and low-grade fungal, viral, and intracellular bacterial infections (eg, mycobacteria). 2° B-cell dysfunction may also be seen.

Answer 3

T-cell deficiencies:

Question 4

Most common immunodeficiency

Answer 4

IgA deficiency

Question 5

Kawasaki Disease Phases
Acute phase:
Subacute phase
Chronic

Answer 5

Acute phase: Lasts 1–2 weeks

Subacute phase: Begins after the abatement of fever and typically lasts for an additional 2–3 weeks.

Chronic phase: Begins when all clinical symptoms have disappeared; lasts until ESR returns to baseline

Question 6

Kawasaki Disease Criteria
Fever plus 4 or more of the following

1. Fever temp of ____ for at least __ days.
2. Bilateral/unilateral, nonexudative, painless/painful conjunctivitis sparing the limbic area.
3. A polymorphous rash (primarily truncal).
4. Cervical lymphadenopathy (often painful and unilateral/bilateral, with at least 1 node > ____ cm).
5. Diffuse mucous membrane erythema (eg, “strawberry tongue”); dry, red, chapped lips.
6. Erythema of the palms and soles; indurative edema of the hands and feet; late desquamation of the fingertips (in the subacute phase).

Answer 6

1. Fever (usually > 40°C [> 104°F]) for at least 5 days.
2. Bilateral, nonexudative, painless conjunctivitis sparing the limbic area.
3. A polymorphous rash (primarily truncal).
4. Cervical lymphadenopathy (often painful and unilateral, with at least 1 node > 1.5 cm).
5. Diffuse mucous membrane erythema (eg, “strawberry tongue”); dry, red, chapped lips.
6. Erythema of the palms and soles; indurative edema of the hands and feet; late desquamation of the fingertips (in the subacute phase).

Other manifestations include sterile pyuria, gallbladder hydrops, hepatitis, and arthritis.

Question 7

Kawasaki disease symptoms—

CRASH and BURN

Answer 7

Conjunctivitis

Rash

Adenopathy (unilateral)

Strawberry tongue

Hands and feet (red, swollen, flaky skin)

BURN (fever > 40°C [> 104°F] for > 5 days

Question 8

Kawasaki Disease versus scarlet fever

Answer 8

Kawasaki disease and scarlet fever may both present with “strawberry tongue,” rash, desquamation of the hands and feet, and erythema of the mucous membranes. However, children with scarlet fever have normal lips and no conjunctivitis.

Question 9

An autoimmune disorder manifesting as arthritis with “morning stiffness” and gradual loss of motion that is present for at least 6 weeks in a patient

Answer 9

JUVENILE IDIOPATHIC ARTHRITIS (JIA)

Question 10

: Most common; involves 4 or fewer joints (usually weight-bearing); usually ANA ⊕ and RF ⊖.
Involves young females;
uveitis is common and requires slit-lamp examination for evaluation.
No systemic symptoms.

Answer 10

Pauciarticular (oligoarthritis)

JIA

Question 11

JIA with systemic onset
May present with recurrent high fever (usually > 39°C [> 102.2°F]), 
hepatosplenomegaly, 
and a salmon-colored macular rash; 
usually RF and ANA negative

Answer 11

Still’s Disease