10 Warning Signs of Primary Immunodeficiency

1 Four or more new ear infections within 1 year.
2 Two or more serious sinus infections within 1 year.
3 Two or more months on antibiotics with little effect.
4 Two or more 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 on skin.
8 Need for intravenous antibiotics to clear infections.
9 Two or more deep-seated infections including septicemia.
10 A family history of PI.


10 Warning Signs of Primary Immunodeficiency For Adults

1 Two or more new ear infections within 1 year.
2 Two or more new sinus infections within 1 year, in the absence of allergy.
3 One pneumonia per year for more than 1 year.
4 Chronic diarrhea with weight loss.
5 Recurrent viral infections (colds, herpes, warts, condyloma).
6 Recurrent need for intravenous antibiotics to clear infections.
7 Recurrent, deep abscesses of the skin or internal organs.
8 Persistent thrush or fungal infection on skin or elsewhere.
9 Infection with normally harmless tuberculosis-like bacteria.
10 A family history of PI.


4 Stages of Testing for Primary Immunodeficiency

Stage 1
History and physical examination, height and weight
CBC and differential
Quantitative Immunoglobulin levels lgG, lgM, IgA (related to age)

Stage 2
Specific antibody responses (tetanus, diphtheria)
Response to pneumococcal vaccine (pre/post) (for ages 3 and up)
IgG subclass analysis

Stage 3
Candida and Tetanus skin tests
Lymphocyte surface markers CD3/CD4/CD8/CD19/CD16/CD56
Mononuclear lymphocyte proliferation studies(using mitogen and antigen stimulation)
Neutrophil oxidation burst (if indicated)

Stage 4
Complement screening CH50, C3, C4
Enzyme measurements adenosine deaminase, purine nucleoside phosphorylase)
Phagocyte studies (surface glycoproteins, mobility, phagocytosis)
NK cytotoxicity studies
Further complement studies AH50
Neo antigen to test antibody production
Other surface/cytoplasmic molecules
Cytokine receptor studies
Family/genetic studies

Reference
http://info4pi.org/