First Aid, Chapter 8, Immunologic Disorders, Congenital (primary) immunodeficiencies)

Question 1

What are warning signs of PIDs?

Answer 1

Warning Signs of PIDs.
1. ≥4 new ear infections within 1 year
2. ≥2 serious sinus infections within 1 year
3. ≥2 months on antibiotics with little effect
4. ≥2 pneumonias within 1 year
5. Failure of an infant to gain weight or grow normally
6 Recurrent, deep skin or organ abscesses
7. Persistent thrush in mouth or fungal infection of skin
8. Need for IV antibiotics to clear infections
9. ≥2 deep-seated infections, including septicemia
10. A family history of PIDs

Question 2

What viruses, bacteria, mycobacteria, fungi, and protozoa are those with antibody deficiency susceptible to?

Answer 2

Viruses: Enterovirus (XLA)

Bacteria: Streptococcus pneumoniae, Haemophilus influenzae, Moraxella catarrhalis, Pseudomonas aeruginosa

No mycobacteria or fungi

Protozoa: Giardia

Question 3

What viruses, bacteria, mycobacteria, fungi, and protozoa are those with combined immune deficiencies susceptible to?

Answer 3

Viruses: All, esp CMV, RSV, EBV

Bacteria: Streptococcus pneumoniae, Haemophilus influenzae, Moraxella catarrhalis, Pseudomonas aeruginosa, Salmonella typhi, Listeria monocytogenes, enteric flora

Mycobacteria: Nontuberculous including BCG

Fungi: Candida, Aspergillus, Cryptococcus, Histoplasma capsulatum

Protozoa: Pneumocystis jiroveci, Toxoplasma, Cryptosporidium parvum

Question 4

What viruses, bacteria, mycobacteria, fungi, and protozoa are those with phagocyte defects susceptible to?

Answer 4

Viruses: none

Bacteria:
Staphylococcus aureus, Burkholderia cepacia, Nocardia, Serratia

Mycobacteria: Nontuberculous including BCG

Fungi: Candida, aspergillus

Protozoa: no

Question 5

What viruses, bacteria, mycobacteria, fungi, and protozoa are those with complement deficiency susceptible to?

Answer 5

Viruses: none

Bacteria: Staphylococcus aureus, Burkholderia cepacia, Nocardia, Serratia, Neisseria meningitidis (late component

Mycobacteria: none

Fungi: none

Protozoa: none

Question 6

What is the initial quantitative and qualitative work-up for humoral immunity (B cell)?

Answer 6

Quantitative: Flow cytometry: CD19 and CD20 IgG, IgA, and IgM

Qualitative: Isohemagglutinin titer Specific antibody titers to: Protein antigens (diphtheria and tetanus) and Polysaccharide antigens (Pneumococcus and Meningococcus)

Question 7

What is the initial quantitative and qualitative work-up for Cellular immunity (T and NK cell)?

Answer 7

Quantitative: Absolute lymphocyte count (CBC with differential counts) Exclude HIV Flow cytometry: CD3CD4, CD3CD8, and CD16CD56

Qualitative: Delayed-type hypersensitivity Enzyme assays (ADA and PNP) NK cytolysis assay Mitogen or antigen stimulation Cytokine production Cytotoxic assays

Question 8

What is the initial quantitative and qualitative work-up for phaogcytic deficiencies (macrophage and monocyte)

Answer 8

Quantitative:Absolute neutrophil count (CBC with differential counts) Flow cytometry: CD11 and CD18 (LAD type1) and CD15a (LAD type2)

Qualitative: Oxidative function (DHR, NBT, or chemiluminescence) Enzyme assays (MPO and G6PD) Phagocyte function Antineutrophil antibodies

Question 9

What is the initial quantitative and qualitative work-up for complement systems?

Answer 9

Quantitative: C3 and C4

Qualitative:
CH50 (classical pathway) AH50 (alternative pathway)