Pediatric Immunology/Allergology Flashcards
[Genetic bases of atopy]
gene region associated with atopy
5q23-35
[Risk of allergic disease]
One parent is allergic
50%
[Risk of allergic disease]
both parents are allergic
66%
Age at which allergic rhinitis can be generally established
6 years old
[Allergic Rhinitis]
Early phase reactions
- Degranulation of mast cells and release of inflammatory mediators
[Allergic Rhinitis]
Late phase reaction
- arises 4-8 hours following allergen exposure
[Allergic Rhinitis]
Cut off value for intermittent symptoms
<4 days per week
OR
4 weeks
[Allergic Rhinitis]
Cut off value for persistentsymptoms
> 4 days per week
AND
> 4 weeks
[Allergic Rhinitis]
Moderate-severe symotoms
- Abnormal sleep
- Impairment of daily activities
- Impaired work and school
- Troublesome symtoms
[Allergic Rhinits: Treatment]
Moderate-severe
- Intranasal CS
2. H1 antihistamine or LTRA
[Allergic Rhinits: Treatment]
If with conjunctivatis
Add
- Oral h1 antihitamine OR
- Intraocular H1 antihistamine OR
- Intraocular cromone
What are the 10 warning signs of immunideficiency
- Four or more new ear infections within 1 year
- Two or more serious sinus infections within 1 year
- Two or more months on antibiotics with little effect
- Two or more pneumonias within 1 year
- Failure of an infant to gain weight or grow normally
- Recurrent, deep skin or organ abscesses
- Persistent thrush in mouth or fungal infections
- need for IV antibiotics to clear infection
- Two or more deep-seated infections including septicemia
- Family history of PI
___ disease wherein preBcell to Bcell development is blocked in heavy chain gene rearrangement
Bruton Disease
btk gene
Healthy infants until 6 months old
What is the treatment for Bruton disease
Gamma globulin IM once a month at 2-3 weeks
[Diagnose]
Fungal inection at 4 months of age, no visible tonsillar tissue, no lymphadenopathies, no thymic shadow, low IgG, IgA and IgE
SCID
What enzyme deficiency is associated with SCID?
Adenosine deaminase
What are the components of DiGeorge Syndrome?
- Cardiac defect (TOF)
- Abnormal facies
- Thymic aplasia
- Cleft palate
- Hypocalcemia
22q11.2 chromosomal deletion
What are the components of Wiskott-Aldrich Syndrome?
- Thrombocytopenia
- Infections
- Eczema
- WASP mutation
[Diagnosis]
Prolonged bleeding from circumcision site
Bloody diarrhea during infancy
Normal appearing megakaryocyte but defective platelets
Wiskot-Aldrich Syndrom
wasp mutation
What are the effective treatment for primary B cell defects?
- Antibiotics for infection
2. regular IVIG 400mg/kg per month SQ
[Diagnose]
Small to absent tonsils, no palpable LN
No antibody production,
Total Ig <100
lack B cells
Bruton Agammaglobinemia
[Diagnose]
Low IgG
Normal Bcells
normal nodes and tonsils
increased granuloma risk
CVID
[Diagnose]
Low IgA (<10mg/dL)
Urogenital infections, Giardiasis
Selective IgA deficiency
6p21.3
[Diagnose]
Normal to High IgM
No IgG
No IgA
no IgE
neutropenic, P. jirovecii pneumonia
Hyper IgM syndrome
Tx: BMSCT
