Immunology/ID Flashcards
(29 cards)
Presentation of Primary immunodeficiency:
What 3 recurrent infections?
- OM
- Sinusitis
- PNA
(“POMS”)
Primary Immunodeficiency: Recurrent OM/Sinusitis/PNA are infected w/ what type of bacteria
encapsulated bacteria
T/F: IgA Deficiency is MC
true
Dx of IgA deficiency?
Low IgA
normal IgG/IgM
Age of diagnosis of IgA Deficiency?
>4y/o
When should you work up pt for IgA deficiency (3 reasons)
- Giardiasis
- Fam hx
- Celiac Disease
- Impaired B cell and T cell and dendrite production
- Impaired Ig production
CVID (Common Variable Immunodeficiency)
When does CVID (Common Variable Immunodeficiency) usually present
around puberty
What is the 3 criteria for Dx of CVID (Common Variable Immunodeficiency)
- decreased IgA, IgG, IgM
- Presence of B cells
- Poor Vaccine response
5 Sxs of CVID?
- Chronic/recurrent URI/LRTI
- GI infection
- Diarrhea, weight loss
- FTT
5. Atopic Triad
How do you tx CVID?
IVIG
Refer
Which condition?
- T and B lymphocyte systems
- Death from overwhelming infection w/in first yr of life
SCID (Severe combined Immunodeficiency)
“Bubble Boy”
How is SCID inherited?
X linked recessive- males
T/F: SCID is part of NB screen
True
Which condition
- 1 or more severe infection in 1st few mon (P. jirovecci, Lymphoma)
- +/- ill from live vaccine
SCID (Severe combined immunodeficiency)
How do you tx SCID?
Stem Cell Transplant
Genetic variation of DiGeorge Syndrome
Deletion of Chromosome 22
Which syndrome is the Mose prevalent microdeletion syndrome
DiGeorge Syndrome
- Cardiac defects (TOF, TGA)
- Cleft Palate
- Hypocalcemia (Parathyroid hypoplasia)
- Cyanosis (b/c of cardiac defects)
DiGeorge Syndrome
- X-linked
- WASp Protein (boys)
Wiskott-Aldrich Syndrome
Clinical presentation
- Susceptibility to infection
- Thrombocytopenia
- Eczema
- Malignancies
Wiskott-Aldrich Syndrome
How do you manage Wiskott-Aldrich Syndrome? (3)
- Prophylactic Abx/antiviral
- Platlet transfusion PRN
- Ig if deficient
How do you tx Wiskott-Aldrich Syndrome?
SCT is curative
