rheum.txt

Onset within minutes
cross-linking of IgE on mast cells
anaphylaxis, angioedema, urticaria
treat with epinephrine and steroids

Onset within hours
cytotoxic antibody
hemolytic anemia

Onset within hours
Immune complex deposition with activation of complement
Serum sickness (fever, urticaria, arthritis)
Low C3, C4, proteinuria
treat with antihistamines, NSAIDs or prednisone

Onset within days
T-cell mediated
Contact dermatitis

FPIES
bloody diarrhea or profuse diarrhea
usually due to soy or milk protein
diagnose with double blind placebo-controlled food challenge

Vaccines to avoid if egg allergic

4 Allergic causes of eosinophilia

Endocrine causes of eosinophilia

3. Infectious causes of eosinophilia

5 Immune dysfunction causes of eosinophilia

4 Neoplastic causes of eosinophilia

3 Idiopathic causes of eosinophilia

Endomyocardial fibrosis secondary to the toxic effects of eosinophilic granules on the heart

Congenital abscence of B cells
commonly X linked
very low IgA, IgM and IgG
absent vaccine responses
pyogenic bacterial and enteroviral infections after six months of age

acquired disorder of antibody production
unkonwn mechanism
Low IgA, IgG or IgM
absent vaccine responses
B cells are present
presents after age 2 with severe infections
associated with autoimmune disease and lymphoma

Most commonly inherited immunodeficiency
unknown mechanisms
may have relatives with CVID
Isolated low IgA
Minor infections or asymptomatic
associated with celiac disease

Slow to develop normal levels of antibodies
Low IgA, IgG, or IgM
present vaccine responses
minor infections or asymptomatic
resolves by four years of age

presents in infancy, FTT
both cellular and humoral deficiency
low antibody levels
abscent vaccine responses
opportunistic infections
require bone marrow transplant
IVIG until transplant

thymic hypoplasia
association with conotruncal anomalies
dysmorphic face, cleft palate and hypoparathyroidism
22q deletions

X-linked
eczema
thrombocytopenia
severe infections

DNA breakage repair deficiency
affects T and B cell gene rearrangement
ataxia, telangiectasias, leukemia, lymphona, immunodeficiency

neutrophils lack NADPH oxidase
NBT or DHR fluorescence test
NOBA neutrophil oxidative burst activation test
skin and organ abscesses

neutrophils cannot migrate out of vessels
high neutrophil count
abscesses without pus

abnormal phagosome trafficking
giant granules in PMNs
partial albinism

mutations to elastase gene ELA2
neutropenia in cycles of 3 weeks
stomatitis, OM, abscesses

ELA2 and HAX1 mutations
low absolute neutrophil count
stomatitis, OM, abscesses, pneumonias

SBDS gene mutations
ineffective hematopoiesis
neutropenia
fat malabsorption due to pancreatic insufficiency
respiratory infections, diarrhea, FTT, metaphyseal dysplasia

a failure of lymphocyte apoptosis
lymphadenopathy
splenomegaly
anemia
thrombocytopenia

staphylococcal microabscesses
pneumonia with pneumatoceles
coarse facies
dermatitis
markedly increased IgE

dry eyes, dry mouth, recurrent parotitis
positive ANA, anti Ro, anti La, RF and IgG
increased risk of JIA, SLE, scleroderma, thyroid, PN, lymphoma

regular fevers, abdominal pain
occaisional pericarditis, pleuritis and arthitis
long-standing inflammation leads to amyloidosis
treat with prophylactic colchicine

fevers every 4-8 weeks lasting 4-6 days
aphthous ulcers
lymphadenopathy
pharyngitis
tonsillitis

oral ulcers
genital ulcers
eye disease
Pathergy test-pustules form at needlestick sites