ALLERGY & IMMUNOLOGY Flashcards by Bao Truong

Boot-shaped heart + absent thymic shadow + hypocalcemia in neonate

What is the heart condition? What is the underlying condition?

Booth-shaped heart = tetralogy of Fallot

Condition = DiGeorge syndrome (22q11.2 deletion)

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When does X-linked agammaglobulinemia present?
What types of infections patient tend to get?

Presents late in the 1st year of life (after maternal IgG has been consumed)

Encapsulated bacterial infections of the respiratory tract
1 - Pneumonia
2 - Otitis media
3 - Sinusitis

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Recurring skin infections, pneumonias, lymphadenitis

What is the condition? What is the diagnostic test to confirm?

Chronic granulomatous disease (CGD)

Dihydrorhodamine oxidation test (DHR test)

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Small grey grape-like shape mass in nasal cavity

What is the mass? What is the most likely cause? First-line treatment for the mass?

Nasal polyp

Most common cause = allergic rhinitis

1st line treatment = nasal steroids

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Small grey grape-like shape mass in nasal cavity

What is the mass? What is the most likely cause? First-line treatment for the mass?

Nasal polyp

Most common cause = allergic rhinitis

1st line treatment = nasal steroids

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What is the infection that is potentially associated with DRESS syndrome?

HHV6 reactivation

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Reddish-brown macules that wheal with gentle stroking of the skin + Hepatosplenomegaly and/or lymphadenopathy

What is the sign called? What is the condition? What are 2 tests that help confirm this diagnosis?

Darier sign

Mastocytosis

Tests: (1) serum tryptase; (2) bone marrow biopsy

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Reddish-brown macules that wheal with gentle stroking of the skin. No systemic finding

What is the condition?

Urticaria pigmentosa

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Delayed separation of the cord associated with omphalitis (inflammation around the cord) in an infant

What is the underlying diagnosis? What are 2 organisms these patients are particularly at risk?

Leukocyte adhesion deficiency type 1 (LAD1)

1 - Staph aureus
2 - E.coli

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Recurring episodes of facial swelling, eyelid swelling, and abdominal pain / vomiting

What is going on? Which is the characteristic lab finding?

Recurring cutaneous & GI angiodema.

Decrease C4 levels

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Deep abscess with Staph aureus + atopic picture (eczema etc.)

What is the underlying condition? What is the problem?

Hyper-IgE syndrome (Job syndrome)

Failure of neutrophils to adhere to endothelium and enter the tissues - causing secondary stimulation of IgE production from eosinophils that are recruited to the area

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What is the most common immunodeficiency in children?

Selective IgA deficiency

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Anaphylaxis to blood product despite no evidence of incompatibility

Which immunodeficiency that is responsible?

Selective IgA deficiency

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Mechanism for anaphylaxis reaction to bee-sting venom

IgE-mediated reaction

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Mechanism for anaphylaxis reaction to radiocontrast media

Mast-cell and basophils mediated

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Mechanism for anaphylaxis reaction during dialysis

Complement activation

Mechanism for serum sickness

Immune-complex deposition in organ tissues

Mechanism for anaphylaxis against penicillin and other antibiotics

IgE recognition of protein-hapten conjugates

Drug that is associated with linear IgA dermatosis, and treatment of choice

Vancomycin (should be discontinued)

Treatment = dapsone

What are components of CHARGE syndrome?

1 - Coloboma
2 - Heart abnormalies (especially aortic arch defects)
3 - Atresia of choaenae
4 - Restricted growth and mental disability
5 - Genital hypoplasia
6 - Ear anomalies

What is the syndrome that is commonly associated with CHARGE syndrome?

DiGeorge

Classical triad of thrombocytopenia, eczema, and recurrent infections

What is the underlying syndrome? Underlying cause? Mode of inheritance

Wiskott-Aldrich syndrome (WAS)

Defect in platelet-leukocyte surface glycoprotein

X-linked recessive

Prolonged diarrhea with Giardia infection
What is the immunodeficiency?

Selective IgA deficiency

Which syndrome is at high risk for developing anaphylaxis to IVIG?

Selective IgA deficiency

What are 4 autoimmune diseases associated with selective IgA deficiency

1 - Celiac disease 2 - Chronic autoimmune thyroiditis (Hashimoto) 3 - Diabetes type 1 4 - Inflammatory bowel disease

Chronic pulmonary infections, unexplained bronchiectasis, recurrent Giardia, splenomegaly What is the condition? What is the issue? What are typical lab tests? Treatment?

Common variable immunodeficiency (CVID) B cells can recognize antigen and multiply but fail to differentiate to plasma cells -> **panhypogammaglobulinemia** Treatment = monthly IVIG

Sterile abscess, delayed separation of the umbilical cord What is the condition

X-linked (Bruton) agammaglobulinemia

Urticaria wheals that persist in the same areas for days after taking an antibiotic, also accompanied by joint pain What is the condition? What are other associated exam findings ? (3)

Serum sickness-like reaction (SSLR) 1 - Fever 2 - Lymphadenopathy 3 - Facial edema

Patient with lupus and recurring pyogenic infection What is going on ?

C2 complement deficiency

Fair skin, blonde hair, blue eyes, multiple oral aphthous lesion. What is the condition? What is the unique lab finding?

Chediak-Higashi syndrome Neutropenia with giant granules in the neutrophils, eosinophils, and granulocytes

Streak of blood in stools of an infant, but normal growth, no other symptoms What is going on?

Protein-induced proctitis (no other sx, no growth issue, no malabsorptive diarrhea)

Systemic symptoms (fever, headache etc.) + bad skin rashes + oral mucosa inflammation What is going on?

Stevens-Johnson syndrome (SJS)

Which classes of drugs are lead triggers of SJS ? (3)

1 - Bactrim 2 - Antiseizure meds (phenobarb, carbamazepine) 3 - NSAIDs

Which infectious agents are lead triggers of SJS ? (4)

1 - HSV 2 - EBV 3 - Mycoplasma pneumoniae 4 - Hep B

What is the latency of DRESS ? (length from exposure to onset)

2-6 weeks

Large protruding ears, long limbs, malar rash with sun exposure What is the condition? What is the type of immunodeficiency associated with this ?

Bloom syndrome Decreased IgA, IgM, IgG

Recurring stress-induced edema of lips and tongue - normal between episodes What is the condition? What is the underlying cause?

Hereditary angiodema C1-esterase inhibitor

What is a characteristic lab finding in ataxia telangiectasia?

Elevated alpha-fetoprotein

Low IgM, high IgA and IgE, normal IgG What is the immunodeficiency?

Wiskott-Aldrich Syndrome