AI Flashcards
what is the risk of atopic disease in a child with one parent with atopy
50%
what is the risk of atopic disease in a child with 2 parents with atopy
70%
define the asthma classification & treatment:
sx 2 or less day per wk
no nighttime sx
no interference with activity
intermittent
short acting bronchodilator prn
define the asthma classification & treatment:
sx more than 2 days per week but not daily
nighttime sx 1-2x/mo
minor limitation with normal activity
requiring SABA more than 2d/wk, not daily
mild persistent
low dose inhaled corticosteroid
2nd line tx- leukotriene inhibitor
plus SABA prn
define the asthma classification & treatment:
sx daily
nighttime sx 3-4x/mo
SABA daily
some limitation with normal activity
moderate persistent
low-medium inhaled corticosteroid + LABA or monteleukast
define the asthma classification & treatment:
sx throughout the day
nighttime sx more than 1x/wk
SABA several times/d
extreme limitation with normal activity
severe persistent
high dose inhaled corticosteroid + LABA or monteleukast
risk factors for asthma persisting into adulthood (4)
1- onset before age 3
2- IgE elevation
3- maternal h/o asthma
4- eosinophilia
what % of children w/ mild asthma will outgrow it
60%
what % of infants with severe RSV bronchiolitis will develop recurrent wheeze
50%
type 1 allergic rxn
IgE mediated
anaphylaxis
type 2 allergic rxn
Ab mediated
type 3 allergic rxn
immune complex mediated
type 4 allergic rxn
delayed hypersensitivity
poison ivy
medications that interfere with skin testing
antihistamines
also drugs with anti-histamine effects (think anti depressants)
fruits children with latex allergy should avoid (6)
avocado banana chestnut kiwi peach tomato
what is the management of contrast media reactions
2/2 reaction from hyperosmolality –> degranulation of mast cells and basophils
pretreat with prednisone and diphenhydramine
nitroblue tetrazolium test
tests for neutrophil activity
normal turns blue
abnormal stays colorless
tests for chronic granulomatous disease (CGD)
CH50 test
tests the complement system
order this if they describe repeated serious bacterial infections
tests for complement deficiencies
Candida skin test
tests for cell mediated immunity associated with T cell defects such as in AIDS
TB skin test
tests for cell mediated immunity associated with T cell defects such as in AIDS
what is the best therapeutic approach for pt with digeorge
thymic transplantation
what is the dx
2 mo old p/wFTT, chronic diarrhea and recurrent opportunistic infections and thrush
also what lab?
SCID- absent of T and B cell fxn
CBC shows low WBC
what is the tx for SCID
BM transplant
what is the dx
eczema
thrombocytopenia
cellular immunodeficiency
wiskott aldrich
what is the dx
male child with eczema, umltiple sinopulmonary infections, and bruising
wiskott aldrich
what is the inheritance of ataxia telangiectasia
AR
what is the inheritance of wiskott aldrich
x linked
what are the CBC findings in wiskott aldrich
thrombocytopenia with small plt
what is the dx
9 month old boy with reccurrent infection with S pneumo and H flu, noted to have small tonsils, adenoids, and spleen. Labs show decrease of all Igs
Brutonsx linked agammaglobulinemia
what is the treatment for bruton XLA
IVIG
what is the dx
9 month old boy with reccurrent sinopulmonary infections, OM, and diarrhea. On exam pt has lymphoid hypertrophy. Labs show decrease of all Igs except IgM which is high
x linked hyper IgM
what is the dx
eczema
eosinohilia
and elevated IgE
with chronic thrush and skeletal abnormalities!
hyper IgE/Jobs
what is the inheritance of CGD
xlinked and AR
what is the dx
child with h/o delayed separation of umbilical cord now with multiple abscesses, although not filled with pus and CBC notable for elevated WBC
LAD
defective chemotaxis
what is the dx
CBC that shows WBC with lysosomal granules
chediak higashi
what is the inheritance of chediak higashi
AR
what is the dx
fair child with blonde hair, blue eyes and frequent skin infections
chediak higashi
what is the inheritance of complement deficiencies
AR
on which chromosome is the immune system (HLA) encoded on
chromosome 6
CD8 cells recognize antigens presented with ___ antigen
class I HLA
CD4 cells recognize antigens presented with ___ antigen
class II HLA
what is the best Ab for complement activation
IgM- think of this because it reacts first
which Ig is secreted in BM
IgA
early complement deficiencies in the classical pathway result in
increase in sinopulmonary infection, pyogenic infections
C2 complement deficiency results in
increase risk SLE
late complement deficiencies result in
increased risk for recurrent N meningitis
what are the catalase positive organisms that results from a phagocytic d/o like CGD (6)
staph serratia aspergillus chromobacterium burkholderia nocardia
what immuno deficiency results in no mature B cells and no Ab present
brutons XL agammaglobulinemia
what infections are those with B cell deficiency prone to
bacteria- encapsulated- strep, H flu, staph
virus- entero
protozoa- giardia
recurrent sinopulmonary infections
what infections are those with T cell deficiency prone to
intracellular organisms
opportunistic infections
bacteria- salmonella, syphilis
mycobacterium
virus- CMV, HSV, VZV, EBV
fungi- candida, aspergillus, cryptococcus
protozoa- PCP, toxo, isosporiasis, microsporidiosis
flow cytometry findings in XL agammaglobulinemia
no mature B cells (CD19)
what is the defect in XL hyperIgM syndrome
CD40L defect
- important in T-to-B cell signaling
- important in T-to-macrophage signaling
what are the 2 types of SCID
x linked SCID (still have some B cells)
ADA- adenosine deaminase deficiency- AR, no T B or NK cells
what are the signs of ataxia telangienctasia
combined deficiency of cellular and humoral immunity
first ataxia –> telangiectasia –> immunodeficiency
AR
high risk of Ca
what is a marker of ataxia telangiectasia
increased AFP
what is Bloom syndrome
chromosomal instability d/o humoral and cellular immunodeficiency AR small stature, telangiectasias, CNS abnormalities high s/w leukemia
what is Nijmegen Breakage syndrome
combined cellular and humoral immunodeficiency
AR
bird-like facies and microcephaly
normal/near normal IQ
what disease is 2/2 defect in C1 inhibitor fxn
hereditary angioedema
AD
recurrent episodes of angioedema, abdominal pain, extremity swelling, laryngeal edema
+ erythema marginatum
NO urticaria