Peds E2 -Allergy & Immunology

What are immunodeficiencies classified as?

cellular and humoral immunodeficiency

combined immunodeficiencies w/ syndromic features

antibody deficiencies

complement deficiencies (AI)

What disease is the CATCH 22 mnemonic for?

DiGeorge syndrome

Catch 22 mneumonic:

Cardiac, Abnormal facies, Thymic hypoplasia, Cleft lip/palate, Hypocalcemia, 22 (chromosome)

What type of immunodeficiency is DiGeorge Syndrome?

T-cell

What cardiac abnormality is associated with DiGeorge syndrome?

VSD & Tetralogy of Fallot

What is Severe Combined Immunodeficiency (SCID)?

disorder of both humoral and cellular immunity

How does SCID present?

recurrent infxns, failure to thrive, thrush, typically first few months of life; death btwn 12-24 months

What is a major risk factor for Ataxia- Telangiectasia?

Lymphomas & Leukemias

What is Bruton's agammaglobulinemia?

X-linked agammaglobulinemia

How does Bruton’s agammaglobulinemia present?

recurrent ear infxns, sinsusitis, pneumonia

What is Common Variable Immunodeficiency (CVID)?

impaired B cell differentiation and defective immunoglobulin production

What are immune signs of CVID?

reduced IgG, IgM, ± IgA; poor or absent response to immunizations

What are clinical features of CVID?

recurrent sinopulmonary infxn, recurrent diarrhea, malabsorption, wt loss, noncaseating granulomas, splenomegaly

How do you dx CVID?

low serum IgG (< 400), low IgM ± IgA

poor anitbody function = non response to vaccines

What is the cause of Hyper-IgM syndrome?

CD40L defect

What is the curative tx for Hyper-IgM syndrome?

bone marrow transplant

What is Transient Hypogammaglobulinemia of Infancy?

drop in IgG levels d/t delay in production after mothers wean off ~4 months

What is Leukocytic Adhesion Deficiency?

leukocytes (esp Neutrophils) can’t leave vasculature to migrate into tissue where inflammation or infxn is present

How does LAD 1 present?

persistent neutrophilia, absents pus formation (hallmark), impaired wound healing

How does LAD 2 present?

severe intellectual disabilities, short stature, distinctive facial appearance (depressed nasal bridge)

How does LAD 3 present?

LAD 1 + bleeding

What is Job syndrome?

Hyper IgE syndrome

What is Job syndrome characterized by?

recurrent skin and pulmonary infections and eczema

What is Chronic Granulomatous disease?

recurrent, life-threatening bacterial and fungal infections and granuloma formation

HIV is acquired through ______ transmission

Vertical

What is given to HIV+ neonates for PJP prophylaxis?

Bactrim once they are 6 wks

Which vaccines can HIV+ neonates recieve? What can’t they receive?

receive ALL vaccines except Live virus ones (MMR, Varicella, intranasal flu)

What is a Type 1 hypersensitivity?

Anaphylaxis, urticaria, food allergies; IgE mediated

What is a Type 2 hypersensitivity?

antibody mediated cell destruction; ex - hemolytic disease of newborn, AI hemolytic anema

What is a Type 3 hypersensitivity?

antigen-antibody complexes; vasculitis, serum sickness

What is a Type 4 hypersensitivity?

delayed; T cells interact w/ antigen; PPD & poison ivy

What is the tx for anaphylaxis?

Epi

How do you differentiate Anaphylaxis from an Anaphylactoid rxn?

indistinguishable

What are common causes of anaphylaxis/anaphylactoid reactions?

Antibiotics (PCN), Foods (peanuts, shellfish, eggs), Anesthetic agents, Insect stings (bees)

Oral allergy syndrome does NOT occur with _______

Cooked food

How does Allergic conjunctivitis present?

bilateral pruritis, watery discharge, injection, cobblestone eyelid

What is the tx for Allergic conjunctivitis?

Cromolyn Na drops

What are the MC allergens contributing to Allergic rhinitis?

Dogs & cats, pollen, grass, ragweed

How does allergic rhinitis present?

nasal itching, sneezing, cobblestoning or posterior pharyngeal wall, nasal crease

What is the tx for Allergic rhinitis?

antihistamines, nasal cromolyn, nasal steroids

What is the cycle of Atopic dermatitis?

itch → scratch → itch

How does Atopic dermatitis present?

dry skin + scratch marks, erythematous papules/vesicles, plaques, lichenification, symmetrical distribution

Where does Atopic dermatitis affect infants?

cheeks, trunk, hands, feet, extremities

Where does Atopic dermatitis affect children?

hands, feet, extremities

What is the tx for Atopic dermatitis?

avoid triggers, moisturize, antihistamines, if severe - corticosteroids, Abx if secondary infxn

What is a food intolerance?

abnormal physiologic response to ingested food, NOT immunologic (therefore not an allergy)

What is an IgE mediated allergy?

oral allergy, anaphylaxis, acute urticaria, angioedema

What is a mixed IgE mediated allergy?

atopic dermatitis, eosinophilic esophagitis/gastroenteritis

What is a Non-IgE mediated allergy?

food PTN induct proctocolitis/entercolitis

What are common food allergens?

peanuts, milk, egg, soy, wheat, fish

What are bacterial causes of B cell deficiency?

S. pneumo, H. influenza, S. aureus

What are viral causes of B cell deficiency?

Enterovirus

What are protozoal causes of B cell deficiency?

Giardia

What is X-linked agammaglobulinemia?

Mutation of Bruton Tyrosine Kinase (BTK) gene on X chromosome, leading to arrest of B cells and failure to generate plasma cells

Which Igs are reduced in Hyper-IgM syndrome?

IgG, IgA, IgE

What disease is diagnosed with dihydrohodamine oxidation test w/ flow cytometry?

Chronic granulomatous disease

What is the most common type of anaphylaxis/anaphylactoid reaction?

Cutaneous

Which is IgE mediated? (Anaphylaxis/Anaphylactoid reaction)

Anaphylaxis

Which is NOT IgE mediated, results from mast cell degeneration?

(Anaphylaxis/Anaphylactoid reaction)

Anaphylactoid reaction