Immunology Flashcards
Most common B-cell defect
Selective IgA deficiency
Hypocalcemia, unusual facies and ears, heart disease, diagnosis?
DiGeorge anomaly
Delayed umbilical cord detachment, leukocytosis, recurrent infections, diagnosis?
Leukocyte adhesion defect
Persistent thrush, failure to thrive, pneumonia, diarrhea, diagnosis?
Severe combined immunodeficiency
Bloody stools, draining ears, atopic eczema, diagnosis?
Wiskott-Aldrich syndrome (excluded if with normal platelet size and count
P. jiroveci pneumonia, neutropenia, recurrent infections, diagnosis?
X-linked hyper-IgM syndrome
Severe progressive infectious mononeucleosis, diagnosis?
X-linked lymphoproliferative syndrome
Recurrent staphylococcal abscesses, staphylococcal pneumonia with pneumatocele, coarse facial features, pruritic dermatitis, diagnosis?
Hyper-IgE syndrome
Persistent thrush, nail dystrophy, endocrinopathies, diagnosis?
Chronic mucocutaneous candidiasis
Short stature, fine hair, severe varicella, diagnosis?
Cartilage hair hypoplasia with short-limbed dwarfism
Oculocutaneous albinism, recurrent infection, diagnosis?
Chediak-Higashi syndrome
Abscesses, suppurative lymphadenopathy, antral outlet obstruction, pneumonia, osteomyelitis, diagnosis?
Chronic granulomatous disease
Progressive dermatomyositis with chronic enterovirus encephalitis, diagnosis?
X-linked agammaglobulinemia
Sinopulmonary infections, neurologic deterioration, talengiectasia, diagnosis?
Ataxia-telangiectasia
Recurrent neisserial meningitis, diagnosis?
C6, C7 or C8 deficiency
Sinopulmonary infections, splenomegaly, autoimmunity, malabsorption, diagnosis?
Common variable immunodeficiency
T-cell, B-cell, granulocyte or complement defect?
Early onset, usually 2-6 mo of age
T-cell
T-cell, B-cell, granulocyte or complement defect?
Onset after 5-7 mo of age
B-cell defect (onset after maternal antibodies diminish)
T-cell, B-cell, granulocyte or complement defect?
Pathogens: common gram (+) and (-) bacteria and mycobacteria, CMV, EBV, adenovirus, parainfluenza 3, varicella, enterovirus, Candida and P. jiroveci
T-cell
T-cell, B-cell, granulocyte or complement defect?
Pathogens: pneumococci, streptococci, staphylococci, Haemophilus, Campylobacter, Mycoplasma, enteroviruses
B-cell defect
T-cell, B-cell, granulocyte or complement defect?
Pathogens: Staphylococci, Pseudomonas, Serratia, Klebsiella, Salmonella, Candida, Nocardia, Aspergillus
Granulocyte
T-cell, B-cell, granulocyte or complement defect?
Pathogens: Pneumococci, Neisseria
Complement
T-cell, B-cell, granulocyte or complement defect?
Affected organs: mucocutaneous candidiasis, lungs, failure to thrive, protracted diarrhea
T-cell
T-cell, B-cell, granulocyte or complement defect?
Affected organs: Sinopulmonary infections, chronic gastrointestinal symptoms, malabsorption, arthritis, enteroviral meningoencephalitis
B-cell
T-cell, B-cell, granulocyte or complement defect?
Graft vs. host disease caused by maternal engraftment or nonirradiated blood transfusion
T-cell
T-cell, B-cell, granulocyte or complement defect?
Postvaccination disseminated BCG or varicella
T-cell
T-cell, B-cell, granulocyte or complement defect?
Autoimmunity, lymphoreticular malignancy, postvaccination paralytic polio
B-cell
T-cell, B-cell, granulocyte or complement defect?
Prolonged attachment of umbilical cord, poor wound healing
Granulocyte
T-cell, B-cell, granulocyte or complement defect?
Autoimmune disorders: SLE, vasculitis, dermatomyositis, scleroderma, glomerulonephritis, angioedema
Complement
Screening tests for phagocytic cell defects
Absolute neutrophil count
Respiratory burst assay
Screening test for complement deficiency
CH50
Intermittent allergic rhinitis
<4 days/week or <4 weeks at a time
Mild allergic rhinitis
Not troublesome, sleep is normal, no impairment in daily activities, no incapacity at work or school
Allergic conjunctivitis is reported in __% of patients with allergic rhinitis
70
Up to __% of patients with asthma have allergic rhinitis and __% of patients with allergic rhinitis have asthma
78, 38
To avoid false negatives in epicutaneous skin test, montelukast should be withheld for __ day, most sedating antihistamines for __ day/s and nonsedating antihistamines for __ day/s
1, 3-4, 5-7
In order to avoid rebound nasal congestion, intranasal decongestants should be used for less than __ days, not to be used more than __ a month
5, once
Most effective therapy for allergic rhinitis
intranasal corticosteroids
Beclomethasone (dosing)
6-12 yr: 1 spray in each nostril BID
>12 yr: 1 or 2 sprays in each nostril BID
Flunisolide (dosing)
6-14 yr: 1 spray in each nostril TID daily or 2 sprays in each nostril BID, not to exceed 4 sprays/day in each nostril
≥15 yr: 2 sprays in each nostril BID, may increase to 2 sprays TID, max dose 8 sprays/day in each nostril
Triamcinolone (dosing)
2-6 yr: 1 spray in each nostril OD
6-12 yr: 1-2 sprays in each nostril OD
≥ 12: 2 sprays in each nostril OD
Fluticasone proprionate (dosing)
≥ 4 yr: 1-2 sprays in each nostril OD
Fluticasone furoate (dosing)
2-12 yr: initial dose - 1 spray per nostril OD, may increase to 2 sprays, but once symptoms controlled, reduced to 1 spray (55mcg), max dose 110 mcg/nostril/d
≥12: initial dose - 2 sprays per nostril OD, once controlled, reduce to 1 spray, max dose 110 mcg/nostril/d
Mometasone (dosing)
2-12 yr: 1 spray in each nostril OD
≥12 yr: 2 sprays in each nostril OD
Budesonide (dosing)
6-12 yr: 2 sprays in each nostril OD
>12 yr: 4 sprays in each nostril OD (max dose)
In asthmatic patients, FEV1 typically decreases by __% after exercise (6-8 min of running)
> 15
Onset of xercise-induced bronchospasm is 15 min after exercise challenge and can spontaneously resolve within 30-60 min
Diurnal variation in PEF of __% is consistent with asthma
20
FEV1:FVC __ indicates severe airflow obstruction
<0.8
Improvement in FEV1 by __ or __ is consistent with asthma
> /= 12%, >200 mL
Asthma severity Intermittent Daytime symptoms: \_\_ Nighttime awakenings: (0-4 yr) \_\_, (≥5) \_\_ SABA use \_\_ Interference with normal activity FEV1 % predicted \_\_ FEV1:FVC: (5-11) \_\_, (≥12) \_\_ Exacerbations: (0-4) \_\_, (≥5) \_\_
Asthma severity Intermittent Daytime symptoms: ≤2 days/wk Nighttime awakenings: (0-4 yr) 0, (≥5) ≤2x/mo SABA use: ≤2 days/wk Interference with normal activity: none FEV1 % predicted: >80%, normal between exarcerbations FEV1:FVC: (5-11) >85%, (≥12) Normal Exacerbations: (0-4) 0-1/yr, (≥5) 0-1/yr
Asthma severity Mild Daytime symptoms: \_\_ Nighttime awakenings: (0-4 yr) \_\_, (≥5) \_\_ SABA use \_\_ Interference with normal activity FEV1 % predicted \_\_ FEV1:FVC: (5-11) \_\_, (≥12) \_\_ Exacerbations: (0-4) \_\_, (≥5) \_\_
Asthma severity Mild
Daytime symptoms: >2 days/wk but not daily
Nighttime: (0-4 yr) 1-2x/mo, (≥5) 3-4x/mo
SABA use: >2 days/wk but not daily and not more than once a day
Interference with normal activity: minor
FEV1 % predicted: ≥80%
FEV1:FVC: (5-11) >80%, (≥12) Normal
Exacerbations: (0-4) ≥2 in 6 mos or
≥4 wheezing episodes/yr lasting >1 day and risk factors for persistent asthma
(≥5): ≥2/yr
Asthma severity Moderate Daytime symptoms: \_\_ Nighttime awakenings: (0-4 yr) \_\_, (≥5) \_\_ SABA use \_\_ Interference with normal activity FEV1 % predicted \_\_ FEV1:FVC: (5-11) \_\_, (≥12) \_\_ Exacerbations: (0-4) \_\_, (≥5) \_\_
Asthma severity Moderate
Daytime symptoms: Daily
Nighttime: (0-4 yr) 3-4x/mo, (≥5) >1x week but not nightly
SABA use: Daily
Interference with normal activity: some
FEV1 % predicted: 60-80% predicted
FEV1:FVC: (5-11) 75-80%, (≥12) Reduced 5%
Exacerbations: (0-4) ≥2 in 6 mos or
≥4 wheezing episodes/yr lasting >1 day and risk factors for persistent asthma
(≥5): ≥2/yr
Asthma severity Severe Daytime symptoms: \_\_ Nighttime awakenings: (0-4 yr) \_\_, (≥5) \_\_ SABA use \_\_ Interference with normal activity FEV1 % predicted \_\_ FEV1:FVC: (5-11) \_\_, (≥12) \_\_ Exacerbations: (0-4) \_\_, (≥5) \_\_
Asthma severity Severe Daytime symptoms: Throughout the day Nighttime: (0-4 yr) >1x/wk, (≥5) >7x/wk SABA use: Several times a day Interference with normal activity: exteme FEV1 % predicted: <60% predicted FEV1:FVC: (5-11) <75%, (≥12) Reduced >5% Exacerbations: (0-4) ≥2 in 6 mos or ≥4 wheezing episodes/yr lasting >1 day and risk factors for persistent asthma (≥5): ≥2/yr
Asthma control: Well-controlled Symptoms: \_\_ Nighttime awakenings 0-4 yr \_\_ 5-11 yr \_\_ ≥12 yr \_\_ SABA use \_\_ Interference with normal activity \_\_ Lung function 5-11 yr: FEV1% \_\_; FEV1/FVC \_\_ ≥12 yr: FEV1% \_\_ Exacerbation 0-4 yr \_\_ ≥5 yr \_\_
Asthma control: Well-controlled Symptoms: ≤2 days/wk but not more than once a day Nighttime awakenings 0-4 yr: ≤1x/mo 5-11 yr: ≤1x/mo ≥12 yr: ≤2x/mo SABA use: ≤2x/wk Interference with normal activity: none Lung function 5-11 yr: FEV1%: >80% predicted or personal best; FEV1/FVC: >80% ≥12 yr: FEV1% >80% predicted or personal best Exacerbation 0-4 yr: 0-1/yr ≥5 yr: 0-1/yr
Asthma control: Not Well-Controlled Symptoms: \_\_ Nighttime awakenings 0-4 yr \_\_ 5-11 yr \_\_ ≥12 yr \_\_ SABA use \_\_ Interference with normal activity \_\_ Lung function 5-11 yr: FEV1% \_\_; FEV1/FVC \_\_ ≥12 yr: FEV1% \_\_ Exacerbation 0-4 yr \_\_ ≥5 yr \_\_
Asthma control: Not Well-Controlled Symptoms: ≥2 days/wk or multiple times on ≤2 days/wk Nighttime awakenings 0-4 yr: >1x/mo 5-11 yr: ≥2x/mo ≥12 yr: 1-3x/wk SABA use: >2 days/wk Interference with normal activity: some Lung function 5-11 yr: FEV1%: 60-80% predicted or personal best; FEV1/FVC: 75-80% ≥12 yr: FEV1% 60-80% predicted or personal best Exacerbation 0-4 yr: 2-3/yr ≥5 yr: ≥2/yr
Asthma control: Very Poorly Controlled Symptoms: \_\_ Nighttime awakenings 0-4 yr \_\_ 5-11 yr \_\_ ≥12 yr \_\_ SABA use \_\_ Interference with normal activity \_\_ Lung function 5-11 yr: FEV1% \_\_; FEV1/FVC \_\_ ≥12 yr: FEV1% \_\_ Exacerbation 0-4 yr \_\_
Asthma control: Very Poorly Controlled Symptoms: Throughout the day Nighttime awakenings 0-4 yr: >1x/wk 5-11 yr: ≥2x/wk ≥12 yr: ≥4x/wk SABA use: Several times per day Interference with normal activity: extreme Lung function 5-11 yr: FEV1%: <60% predicted or personal best; FEV1/FVC: <75% ≥12 yr: FEV1% <60% predicted or personal best Exacerbation 0-4 yr: >3/yr
Asthma pharmacotherapy Step 1
0-4 yr: __
5-11 yr: __
≥12 yr: __
Asthma pharmacotherapy Step 1
0-4 yr: SABA prn
5-11 yr: SABA prn
≥12 yr: SABA prn
Intermittent Asthma
Asthma pharmacotherapy Step 2
0-4 yr: __
5-11 yr: __
≥12 yr: __
Asthma pharmacotherapy Step 2
0-4 yr: Low-dose ICS
OR cromolyn or montelukast
5-11 yr: Low-dose ICS
OR cromolyn, LTRA, nedocromil or theophylline
≥12 yr: Low-dose ICS
OR Cromolyn, LTRA, nedocromil or theophylline
Asthma pharmacotherapy Step 3
0-4 yr: __
5-11 yr: __
≥12 yr: __
Asthma pharmacotherapy Step 3
0-4 yr: Medium-dose ICS
5-11 yr: Either Low-dose ICS ± LABA, LTRA or theophylline, or Medium-dose ICS
≥12 yr: Either Low-dose ICS + LABA or Medium-dose ICS
OR Low-dose ICS + LTRA, theophylline, or zileuton
Asthma pharmacotherapy Step 4
0-4 yr: __
5-11 yr: __
≥12 yr: __
Asthma pharmacotherapy Step 4
0-4 yr: Medium-dose ICS + either LABA or LTRA
5-11 yr: Medium-dose ICS + LABA
OR Medium-dose ICS + either LTRA or theophylline
≥12 yr: Medium-dose ICS + LABA
OR Medium-dose ICS + LTRA, theophylline or zileuton
Asthma pharmacotherapy Step 5
0-4 yr: __
5-11 yr: __
≥12 yr: __
Asthma pharmacotherapy Step 5
0-4 yr: High-dose ICS + either LABA or LTRA
5-11 yr: High-dose ICS + LABA
OR High-dose ICS + either LTRA or theophylline
≥12 yr: High-dose ICS + LABA
and consider omalizumab for patients with allergies
Asthma pharmacotherapy Step 6
0-4 yr: __
5-11 yr: __
≥12 yr: __
Asthma pharmacotherapy Step 6
0-4 yr: High-dose ICS + either LABA or LTRA
and oral corticosteroid
5-11 yr: High-dose ICS + LABA
and corticosteroid
OR High-dose ICS + either LTRA or theophylline and oral corticosteroids
≥12 yr: High-dose ICS + LABA + oral corticosteroid
and consider omalizumab for patients with allergies
Hallmark of atopic dermatitis
Severely dry skin
Cardinal features of atopic dermatitis
Intense pruritus (especially at night) and cutaneous reactivity
First-line therapy for atopic dermatitis
moisturizers
Cornerstone of anti-inflammatory treatment for acute exacerbations fo atopic dermatitis
topical corticosteroids
Food allergy is comorbid in approximately __ of infants and young children with moderate to severe AD
40%
Key components to optimal asthma management
- Assessment and monitoring
- Education
- Control of environmental factors and comorbid conditions
- Medications
- Management of exacerbations
Recommended number of asthma checkups per year for assessing and maintaining good asthma control __
Lung function testing should be done at least __ (frequency)
2-4, annually
Gold standard test for sinus disease
Coronal “screening” or “limited” CT scan of the sinuses
Management of sinusitis
Nasal saline irrigation, intranasal corticosteroids, 2-3 week course of antibiotics
Second generation ICSs
fluticasone propionate, mometasone furoate, ciclesonide, budesonide
A good response to asthma rescue medication (inhaled SABA up to 3 treatments in 1 hr) is characterized by:
resolution of symptoms within 1 hour
no further symptoms over the next 4 hr
improvement in PEF value to at least 80% of personal best
Dose of prednisone for exacerbations
0.5-1 mg/kg q6-12 for 48 hr, then 1-2 mg/kg/d BID (not to exceed 60 mg/day)
Dose of terbutaline for exacerbations
Continuous IV infusion, 2-10 mcg/kg LD, followed by 0.1-0.4 mcg/kg/min
Titrate in 0.1-0.2 mcg/kg/min increments every 30 min
Patient may be discharged to home after an exacerbation if:
There has been sustained improvements in symptoms Bronchodilators are at least 3 hr apart Physical findings are normal PEF >70% of predicted or personal best O2sat >92% on room air
Preferred SABA and ICS for pregnant women
albuterol, budesonide
Gastrointestinal manifestation of food allergy. Manifests in the first several months of life as irritability, intermittent vomiting (occurs 1-3 hr after feeding), protracted diarrhea, bloody diarrhea, abdominal distention, failure to thrive. Provoked by cow’s milk or soy protein-based formulas
Food protein-induced enterocolitis syndrome (FPIES)
Gastrointestinal manifestation of food allergy.
Presents in the first few months of life as blood-streaked stools in otherwise healthy infants. 60% of cases in breastfed infants.
Food protein-induced proctocolitis
Gastrointestinal manifestation of food allergy.
Manifests in the first several months of life as diarrhea, often with steatorrhea and poor weight gain, protracted diarrhea, vomiting, failure to thrive, abdominal distention, early satiety, malabsorption.
Food protein-induced enteropathy
Examples or Cow’s milk sensitivity and Celiac disease
Before a food challenge is initiated, the suspected food should be eliminated from the diet for ___ days for IgE-mediated food allergy and up to ___ for some cell-mediated disorders
10-14 days; 8 weeks
Drug of choice for cold-induced urticaria
Cyproheptadine
Most feared complication of hereditary angioedema
layrngeal edema
Inherited autosomal dominant disease caused by low functional levels of the plasma protein C1 inhibitor presenting with cutaneous nonpitting and nonpruritic edema not associated with urticaria
Hereditary angioedma
Epinephrine dose for anaphylaxis
1:1000, 0.01 mg/kg IM (max 0.5 mg) repeated 2-3 times at intervals of 5-15 min if epi IV drip is not yet started
Diagnostic criteria of anaphylaxis
Any 1 of 3:
- Acute onset with mucocutaneous involvement and at least 1 of the ff:
a. respiratory compromise
b. Reduced BP/end-organ dysfunction - At least 2 of the ff that occur rapidly after a likely allergen:
a. mucocutaneous involvement
b. respiratory compromise
c. Reduced BP/end-organ dysfunction
d. persistent GI symptoms - Reduced BP following exposure to known allergen