Allergy and Immunology Flashcards

1
Q

Recurrent bacterial infections, X-linked inheritence, small tonsils

A

Bruton or X-linked agammaglobulinemia

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2
Q

Bruton agamaglobulinemia genetics

A
  • X linked
  • BTK gene
  • Complete defect in B cell production
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3
Q

Bruton agamaglobulinemia symptoms

A
  • Bacterial infections (respiratory and skin) starting around 6 months of age (protected by maternal antibodies prior to that)
  • Small tonsils
  • Require life long IVIG replacement or stem cell transplant
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4
Q

Atopic march order in kids

A
  • Atopic dermatitis in infants
  • Allergic rhinitis in children
  • Asthma in children/adolescents
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5
Q

Most important risk factor for developing atopic disease

A

Parent with atopic disease

50% risk if 1 parent, 70% risk if both

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6
Q

Asthma epidemiology

A
  • Mortality is increasing

- MC in boys, African Americans, and Hispanic children

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7
Q

Spirometry measurements

A
  • Inspiratory and expiratory flow rate
  • Decreased FEV1 in asthma
  • It DOES NOT measure total lung capacity or residual volume
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8
Q

Asthma symptoms: symptoms and albuterol < 2 days/week, zero nighttime awakenings

A

Intermittent

Tx: SABA PRN

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9
Q

Asthma symptoms: symptoms and albuterol > 2 days/week but not daily, nighttime symptoms 1-2 times per month

A

Mild persistent

Tx: Low dose inhaled steroids, can add leukotriene inhibitor

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10
Q

Asthma symptoms: symptoms and albuterol daily, nighttime 3-4 times per month

A

Moderate persistent

Tx: Medium dose inhaled steroids, can add LABA or montelukast

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11
Q

Asthma symptoms: symptoms all day long, albuterol multiple times a day, night symptoms more than once a week

A

Severe persistent

Tx: High dose inhaled steroids plus LABA, montelukast

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12
Q

Side effects of beta blockers

A

Tachycardia, tremors, hypokalemia, hyperglycemia

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13
Q

How do inhaled steroids help asthma

A

Decrease bronchial inflammation and also reduce bronchial hyperresponsiveness

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14
Q

Signs of hypercapnia in asthma

A
  • Fatigue, CO2 retention

- Agitation, flushing, mental status change, headache, tachycardia

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15
Q

Symptoms of poorly controlled asthma

A

Rule of 2s:

  • Symptoms 2 or more days a week
  • Waking up at night 2 times a month
  • Albuterol 2 or more times a week
  • Steroids more than 2 times a year
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16
Q

Four types of allergic reactions

A
  • Type 1 = Anaphylactic (A) - IgE mediated
  • Type 2 = Mediated by AntiBodies (B)
  • Type 3 = Immune complex
  • Type 4 = Delayed hypersensitivity (poison ivy)
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17
Q

What interferes with skin allergy testing

A

Any antihistamine use will interfere with the results of skin testing but not IgE testing

  • Contraindications to skin testing: urticaria, mastocystosis (skin conditions), high anaphylactic risk (poorly controlled asthma), recent anaphylactic event
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18
Q

When is in vitro IgE allergy testing preferred

A
  • Kids on chronic antihistamines (it doesn’t affect the testing)
  • Children with extensive eczema or skin infections (limited area to do skin testing)
  • Limitations: higher cost and higher false positive rate
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19
Q

Penicillin allergy testing

A
  • The only antibiotic that can be skin/IgE tested
  • IgE mediated reaction starts within 24 hours of penicillin exposure
  • Chance of having reaction to cephalosporins is less than 10%
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20
Q

Non allergic rhinitis with eosinophilia syndrome

A

Allergy symptoms and eosinophils on nasal smear but skin tests are negative and serum IgE levels would be normal

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21
Q

Symptoms of infectious rhinitis

A

Presents in younger children with nasal congestion worse in the winter

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22
Q

Symptoms of vasomotor rhinitis

A

Congestion, rhinorrhea, post-nasal drainage without any specific trigger
- But can be associated with emotions, pollution, cold drafts, rapid temperature changes, or changes in humidity

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23
Q

Symptoms of rhinitis medicamentosa

A
  • Rebound reaction to adrenergic nose drops (leads to severe nasal congestion)
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24
Q

Symptoms of aspirin triad

A
  • Nasal polyps
  • Aspirin intolerance
  • Asthma
  • Have chronic nasal congestion and anosmia
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25
Q

Nasal complication of cocaine abuse

A

Nasal septum perforation

26
Q

At what age can seasonal allergic rhinitis be diagnosed

A

Not until age 3, requires repeated exposure

27
Q

Who needs to be sent for food allergy testing

A

Kids with severe eczema and persistent asthma

28
Q

Most common childhood allergies

A

Milk, eggs, peanuts, tree nuts, soybeans, wheat, fish

- IgE mediated allergy reactions occur within minutes, food poisoning happens 6 or more hours after

29
Q

At what age are most food allergies outgrown

A

Age 5 - milk, egg, and soy are usually outgrown (the others are not)

30
Q

Treatment of anaphylaxis

A
  • Epinephrine 0.01 mg/kg 1:1,000 IM every 15 minutes

- 0.3 mg for patients > 30 kg, 0.15 mg for patients < 30 kg

31
Q

Chronic urticaria cause and treatment

A
  • If > 6 weeks then it is chronic
  • Most likely cause is food
  • Treatment: antihistamines (2nd or 3rd generation) for long term, can use benadryl in short term
  • Allergy testing has no role, no need for chronic steroids
32
Q

Cause of reaction to contrast

A

Osmolality-hypertonicity reaction that triggers degranulation of mast cells and basophils with release of mediators

33
Q

Allergic reaction to stings symptoms

A
  • Localized non-systemic symptoms (even hives) don’t require allergy testing
  • Triad of systemic reactions: hypotension, wheezing, laryngeal edema
  • Tx: remove stinger as fast as possible
  • If systemic reaction, need to have epi-pen and if life threatening reaction need treated with allergy shots
34
Q

Normal amount of infections in childhood

A

It’s okay for a kid to have one infection a month especially if self limited GI or respiratory infections –> don’t need immunodeficiency workup

35
Q

Chronic Granulomatous Disease cause

A
  • Phagocytic dysfunction
  • Can’t undergo respiratory burst needed to kill bacteria and fungi (get infected with those)
  • 2/3 are X-linked, 1/3 are autosomal recessive
36
Q

CGD symptoms

A
  • Bacterial infections, present in the first 5 years of life
  • MC infections are deep abscesses, pneumonia, lymphadenitis, osteomyelitis, and systemic infections
  • S. aureus, B. cepacia, Serratia, and fungi
37
Q

CGD diagnosis and treatment

A
  • Nitroblue tetraolium test (NBG) - assays the phagocytic oxidase activity
  • Okay to get live viral vaccines but not bacterial
  • Ppx with bactrim and itraconazole
38
Q

Leukocyte adhesion deficiency cause

A
  • Defect in chemotaxis (WBCs can’t get to where they need to go or adhere to the endothelium)
39
Q

LAD symptoms

A
  • High WBC count
  • Perirectal abscess, indolent skin infections, omphalitis
  • Infections have NO PUS and minimal inflammation, delayed wound healing
  • Can present with delayed umbilical separation
40
Q

LAD diagnosis and treatment

A
  • Dx is flow cytometry

- Tx: bone marrow and stem cell transplantation

41
Q

Chediak-Higashi syndrome cause

A
  • WBCs contain lysosomal granules and have abnormal chemotaxis
  • Autosomal recessive
42
Q

Chediak-Higashi syndrome symptoms

A

Frequent infections, easy bruisability, oculocutaneous albinism

  • Infections occur in the lungs and skin
  • MC pathogens are Staph aureus, strep pyogenes, pneumococcus
43
Q

Chediak-Higashi syndrome diagnosis and treatment

A
  • Diagnose with giant granules in neutrophils on blood smear

- Tx with bone marrow transplant

44
Q

Humoral immune deficiency general symptoms

A
  • Antibody deficiencies are the most common
  • Recurrent sinopulmonary infections with ENCAPSULATED BACTERIA
  • Symptoms begin at 4-6 months of age due to initial protection with maternal antibodies
  • NO LIVE VACCINES
45
Q

IgA deficiency symptoms

A
  • MC primary immunodeficiency, over 80% are asymptomatic
  • Recurrent sinopulmonary infections
  • NOT an indication for IVIG replacement
46
Q

Bruton’s X-linked agammaglobulinemia symptoms

A
  • Mostly affects B cells (T cell count may be elevated)
  • Serum levels of IgG, IgA, IgM, and IgE are very low with small lymphoid tissue and small spleen
  • Baby boy with recurrent infections with encapsulated pyogenic bacteria (Strep pneumo and H flu)
  • Require IVIG
  • Risk for bronchiectasis and chronic pulmonary insufficiency
47
Q

CVID symptoms

A
  • MC clinically significant immunodeficiency
  • B lymphocytes don’t differentiate into plasma cells so deficiency of immunoglobulin subtypes
  • T cells are also often affected
  • HAVE NORMAL CBC
  • Recurrent respiratory and GI infections, also risk for recurrent herpes/zoster infections
  • Associated with autoimmune diseases and increased risk of lymphoma
  • Require IVIG
48
Q

X-linked Hyper IgM Syndrome

A
  • Disruption of B cell differentiation, can’t convert IgM to IgG
  • Male infant age 6-12 months with frequent otitis and sinopulmonary infections as well as diarrhea
  • LYMPHOID HYPERTROPHY
  • Think of this with opportunistic infections (PCP with no HIV)
  • Labs: low IgG, IgA, IgE with high IgM
  • Tx with IVIG
49
Q

Job Syndrome (Hyper IgE) symptoms

A
  • Eosinophilia, Eczema (recurrent skin infections), elevated IgE
  • Staph aureus, sinopulmonary infections, chronic thrush, abnormal facies, multiple fractures, skeletal abnormalities
  • Can be mistaken for atopic dermatitis or Wiskott-Aldrich
  • Tx with steroids and antibiotics
50
Q

Transient hypogammaglobulinemia of infancy

A
  • Decreased T helper function leads to low IgG and IgA

- Manifests by 6 months or so and outgrow by 3-6 years of age

51
Q

Cellular immune deficiency general rules

A
  • Present with opportunistic infections
  • Low lymphocyte count
  • Don’t give live vaccines
52
Q

SCID symptoms

A
  • Complete absence of B and T cell function
  • Present in first 3 months of life with failure to thrive, chronic diarrhea, recurrent opportunistic infections
  • Low WBC, complete abscence of T cell function
  • Tx: supportive but need BMT
53
Q

Wiskott Aldrich Syndrome symptoms

A
  • X linked (presents in males)
  • Triad is: eczema, thrombocytopenia (unusual bleeding), cellular immunodeficiency
  • Low platelet count with SMALL PLATELETS
  • Sinopulmonary infections with encapsulated bacteria –> opportunistic infections with PCP and herpes
  • High rate of lymphoma (MCC death)
  • Tx with BMT or stem cells
54
Q

Complement deficiency symptoms and management

A
  • C1-C4 deficiency: recurrent sinopulmonary infections due to encapsulated bacteria
  • C5-C9 deficiency: recurrent Neisseria infections and increased risk of meningitis
  • Autosomal recessive
  • Screen with CH50 assay
  • Okay to give all vaccines, no therapy
55
Q

Humoral vs Cellular immunodeficiency symptoms

A
  • Humoral (B cell): present AFTER 6 months, sinopulmonary and GI tract infections, test for quantitative immune globulins
  • Cellular (T cell): sick AT BIRTH, severe infections, test for lymphocyte subset analysis via flow cytometry
56
Q

Symptoms of systemic mastocytosis

A
  • Darier sign (formation of a wheal upon gentle stroking of reddish-brown macules on the skin)
  • GI ulcers
  • Small itchy bumps on skin
57
Q

Immunodeficiency with increased risk of infections from giardia

A
  • Selective IgA deficiency
58
Q

Immunodeficiency with increased risk of anaphylaxis after blood transfusion

A
  • Selective IgA deficiency
59
Q

What type of reaction can occur after initial exposure to a foreign antigen and doesn’t require prior exposures

A

Serum sickness

60
Q

Risk factors for persistent asthma

A

Early RSV infection, maternal asthma, smoke exposure, elevated IgE, atopic disease