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43 Cards in this Set

  • Front
  • Back
What % of all primary immunodef is Adaptive?
What percent are Bcell?
What percent are Tcell or combined B/Tcell?
Bcell = 50%
T or combined = 30%
When is the onset of Bcell defects?
4-6 mo of age (when maternal Ab wears off)
When is the onset of Tcell defects?
Usually before 6 months old
What does a Bcell defect present with?
-Recurrent sinupulmonary infections
-Chronic GI infections showing malabsorption and poor growth
What types of infections will babies with Bcell defects get?
Bacterial - especially encapsulated, and viral
What does a Tcell defect present with?
Poor growth, failure to thrive, and chronic diarrhea
What types of infections will Tcell-defective patients get?
Viral, fungal, and opportunistic infections
What percentage of all PID are innate?
What are the 4 types of innate immune system deficiencies?
1. Neutrophil defect
2. Macrophage defect
3. NK cell defect
4. Complement defect
When is the onset of most of the innate deficiencies?
In infancy or childhood
How does an neutrophil deficiency present?
With common pathogens causing severe infections
What are the typical agents of infection in neutrophil deficiency?
Bacterial - Staph, pseudomonas
What are the common presentations in macrophage defects?
-Recurrent fevers
-Disseminated mycobacterial infections
What is the common disease associated with macrophage defects?
Mycobacterial disease
What is the typical presentation of a NK cell defect?
Disseminated viral infections or Cancer
What is the common presentation of a Complement defect?
Rheumatoid disorders like lupus or scleroderma
What infection is typically seen in complement defects?
What type of disorders will exhibit an abnormal immune response that never really gets up and goes?
Digeorge syndrome
What type of disorders will exhibit an abnormal immune response that sort of gets up, but then wavers up and down?
Autoinflammatory disorders
What type of disorders will exhibit an abnormal immune response that gets up and goes and goes and goes?
Immune dysregulation syndromes
What is the normal number of otitis media infections per year?
What is considered an abnormal number of sinus infections per year?
How many episodes of pneumonia per year would be abnormal?
When is thrush abnormal?
Over the age of 1 yr old
Why is thrush common in children under 1 yrs old?
-Nipple colonization
-Freq use of antibiotics for ear infections decreases the normal protection against thrush.
What do the majority of PIDs have some degree of?
GI complications
What are 3 GI infections that are suggestive of PID?
-Recurrant/refractory C.diff
If there are recurrent sinupulmonary infections
poor growth
and diarrhea... think:
Humoral (Bcell) defect
What bacteria will cause infections in Bcell defects?
3 types of encapsulated bacteria:
If you see abcesses, open sores without pus, or poor wound healing, oral ulcers, recurrent pneumonias, think:
Neutrophil defect
Bacteria, fungi, and parasites that will cause infections in neutrophil defects:
Staph aureus
Serratia, Klebsiella

Failure to thrive
Chronic diarrhea
Opportunistic infections
Thrush... THINK:
Tcell or combined T/Bcell defect
What will the family history show in Tcell or combined T/B cell defect?
History of early childhood deaths
You suspect a humoral deficiency; what lab test will you order?
HISS panel
What is a HISS panel?
Humoral Immune Status Survey
What does a HISS panel give you?
Antibody levels and vaccine titers
You suspect a Tcell or combined immune defect; what would you order?
-Lymphocyte subsets
-HISS panel
-Skin testing
You suspect a phagocyte defect; what would you order?
1. CBC with diff
2. NBT/oxidative burst test
You suspect a complement defect; what would you order?
Total Hemolytic Complement assay
What does a Total Hemolytic Complement assay measure?
Both the classical and alternative pathways
Why is flow cytometry an important assay?
Because a CBC only gives the basic numbers and is not complete.