Flashcards in immunology Deck (29):
Second most common after CVID. Often asymptomatic. Increased allergy and eczema incidence, and celiac. Difficulty clearing rota. More sinopulmonary infections.
Icnreased risk of malignancy and autoimmune disease.
Look for repeated organ specific infections, especially pyogenic lower resp and sinupulmonary infections from repeated, often encapsulated/polysaccharide bacteria. May present as "asthma". Bronchiectasis. Otitis media. Sinopulmonary.
X-linked Immunodeficiency With Hyper IgM
X Linked disease. Abnormal CD40. Can't make IgA, IgE or igG, with consequent high levels of circulating IgM.
Chronic pneumonias (esp PCP but of all variations), lots of diarrhea (think cryptococcus). Failure to thrive.
Chronic or cyclic neutropenia. Sclerosing cholangitis. Autoimmune disease. Malignancy.
Common Variable ImmunoDeficiency
CVID is the most common deficiency. Affects ability of B cell to mature to plasma cell. IgA, IgG and sometimes IgM is lower. Recurrent infections, unusual organisms like mycoplasma, PCP and giardia.
Granulomatous disease, lymphoid hyperplasia, autoimmune diseae and malignancy risk.
X-linked agammaglobulinemia (Bruton's)
X linked. BTK mutation (needed for B cell maturation-Pre-B cell). Unlike CVID, has no rate of autoimmune disease. Low or absent mature B cells. No IgG, IgA or IgE. Absent/reduced lymphoid tissue.
Lots of in ifections, including skin infections.
Post vaccine polio infection. Fungal and viral infections handled well except enteroviruses.
Severe combined immunodeficiency
SCID is a life-threatening syndrome of recurrent infections, diarrhea, dermatitis, and failure to thrive. Untreated, usually dead by 2.
Usually lymphopenic (but may just be nonfunctional) and Ig's of all types decreased.
DiGeorge Syndrome is associated with what immune dysfunction?
T cell dysgenesis.
Wiskot Aldrich Syndrome
eczema-thrombocytopenia-immunodeficiency syndrome. X linked. Has bloody diarrhea because of thrombocytopenia.
Hyperimmunoglobulinemia E (Job) Syndrome
High IgE levels. Recurrent mucocutaneous candidiasis, lichenifications, eczema, wheeze. Abnormal facies.
Most common food allergies
egg, milk, soy, tree nuts, peanuts, shellfish, fish.
Prognosis of egg and milk allergies
85% outgrown by age 5.
peanut, tree nuts, fish, and shellfish allergy natureal history
Natureal history of non-IgE food "allergies"
The proctocolitis and enterocolitis resove usualyl in first year of life. Eosinophilic esophagitis is persistent.
Causes of secondary immunodeficiency...
HIV, CMV, starvation, metabolic disease, steroids, radiation and nutrients like zinc, copper, iron, and selenium.
• X-linked agammaglobulinemia (Bruton’s)
• Common Variable Immunodeficiency (CVID)
• Selective Ig-A Deficiency
• Transient Hypogammaglobulinemia of Infancy
• Functional Hypogammaglobulinemia
• IgG Subclasses deficiency
• Hyper Ig-M Syndrome
T cell Dysfunction : Clinical Characteristics - organisms
Infections with intracellular microorganisms
Viruses (HSV, VZ, CMV, EBV)
Protozoa (Cryptosporidium, toxoplasma)
Fungal (Candida, P. carinii )
Bacteria, gram negative enteric (T cell)
Bacteria, polysaccharide encapsulated
T Cell Dysfunction: Clinical Characteristics
• Anergy to recall antigens
• Graft versus host disease
• Failure to thrive (especially with
• Increased B-cell malignancies
• Eosinophilia, thrombocytopenia
• Eczema, alopecia
Classification of Food reactions
1) Autoimmune: ie celiac
2) Non-Immune: enzyme defects/deficiencies, drug reactions, food additive reactions, metabolic diseases (PKU, fructose intolerance, galactosemia... etc.)
3) Immune Mediated: IgE mediated (allergies), mixed IgE/non-IgE: eosinophilic esophagitis; non-IgE (enterocolitis, proctocolitis, chronic constipation).
Red flags with fevers
Inconsolable, poor tone, altered LOC, not feeding, Anuria, lethargy, seizure
Prematurity and infections
Good antiobiotics for fever without a source in a baby
Amp and cefotaxime
Antibacterial for first 6 months: DTaP, menC, pneumo, HiB (IPV though).
Influenza at 6 months.
12mo: mmr, vsv,
Grade 5 hep b and hpv.
People at greater risk of opv induced polio disease
B cell immunodefiencies.
Red flags for vaccines
Previous anaphylactic reactions, recent steroids, recent blood transfusion products
People that should really get vaccines
Splenectomy/hyposplenia, elderly, neonates, immunodeficiency, HIV, cops, heart failure,
Types of vaccines
Live attenuated (mmr, vsv, oral typhoid, oral polio, yellow fever, bcg).
Whole inactivated (Salk)
Parts/protein +\_ conjugate
Parts of vaccines
Additives to enhance immunogenicity (aluminum salt)
Support growth (egg, animal protein)
Education points for vaccines
Very safe, even multiple early in childhood. Most even with active mild disease.
No link to autism.
No safety reason to avoid thimerosal
Risks: common(pain, swelling, irritability, rash, fever); rare (fever, seizure, hypotonia, parotitis); life threat: GBS, allergic,