Immunodeficiency Flashcards
1
Q
recurrent infections
A
-normal
-6 per year
-9 for daycare
12 second hand smoke
-cold 4-12 days
2
Q
when should immune eval be considered?
A
- two or more invasive bacterial infections
- persistant or recurrent sinopulmonary infections
- unusual etiologic agents or unusual severity of common infection
3
Q
evaluation for immundeficiency
A
-history
physical exam
labs
4
Q
history
A
growth and development types and sites of infections severity of infections, complications specific organisms hiv risk factors diarrhea, malabsorption ai or allergic phenomenon family history
5
Q
physical exam
A
physical and anatomic barriers growth and development tonsils, nodes, liver, spleen thrush eczyma, seborrhea, petechiae, others
6
Q
nonimmunologic lines of defense
A
- skin and mucous membranes-eczyma, burns, open fractures, sinus tracts
- adequate drainage- eustachian tube dysfunction, ciliary immotility, CF, ureteral reflux
- foreign bodies-medical/accidental
7
Q
four limbs of the immune system
A
- cellular-T, NK, cytokines
- Humoral- B, Ig
- complement
- phagocytes
8
Q
distribution of primary immnodeficiencies
A
cellular 7% complement 2% phagocyte 20% combined 25% humoral 46%- IgA
9
Q
SCID
A
- FTT, thrush, skin rash, diarrhea
- male 75%
- x linked and AR
- early death without bone marrow transplant
- no t cells/ b cells
- common IL2 gamma chain defect- which is present in a lot of cell types
- adenosine deaminase deficiency
- purine nt phosphorylase deficiency
- T cell signal transduction defects
- others
10
Q
common gamma chain
A
- b cell maturation
- NK development
- hematopoeisis
- t cell development
- class switch recombination
- peripheral t cell homeostasis
11
Q
SCIDs 2
A
- CBC-lymphopenia
- enumeration of specific lymphocytes subsets assists in diagnosis
- analysis of lymphocyte proliferation in response to mitogens (stim T and B), non self HLA, specific antigens
- most need bone marrow transplant
- invasive, life threatening viral, fungal, bacterial infections
- thrush is common
- aggressive treatment with antibiotics, replacement Ig, often antivirals and antifungals while awaiting transplant
12
Q
humoral immunodeficiency
A
- bronchiectasis, recurrent sinusitis
- R/O CF, immotile cilia syndrome and HIV
- B cell enumeration (CD19 and/or 20)
- quantitative Ig- A, G, M, G subclasses, pre and post vaccine titers, isohemmagglutinins
- infectious risks-encapsulated bacteria, pneumococcus
- susceptibility to chronic enterovirus meningoencephalitis
- AI disease common
- lifelong risk of lymphoma
- common variable immunodeficiency
- selective defect in anti-polysaccharide antibody production
13
Q
x linked agammaglobulinemia
A
-absence of b cells due to mutation in brutons tyrosine kinase
14
Q
cd40 L deficiency
A
- hypogammaglobulinemia with hyper IgM- can’t react with TH2 to class switch
- no germinal centers
- no TH1 to macrophage-intracellular pathogens
15
Q
humoral immunodeficiency management
A
- Ig replacement
- early id and treatment of infections, including enteroviral disease
- aggressive pulmonary F/U
