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pediatrics > allergy and immunology > Flashcards

allergy and immunology Flashcards

1
Q

anaphylactoid reactions?

A

non-IgE related –> clinically identixal and treated the same way and not preceding sensitization ot the antigen

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2
Q

angiodema - mechanism

A

it can be from deficiency of C1 esterase inhibitor (hereditary). There is a characteristic association with the onset with minor physical trauma. It often has idiopathic origin

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3
Q

angioedema - look for

A

recent start of ACE inhibitors preceding symptoms

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4
Q

angioedema - presentation

A

heredtary angioedema is characterized by sudden facial swelling and stridor with absence of pruritus and urticaria
maybe also colicky abd pain, diarrhea, vomiting

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5
Q

a special characteristic of hereditary angioedema

A

it does not respond to glucocorticoids

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6
Q

angioedema - best initial test

A

test for decreased C2 and C4 in the complement pathway as well as deficiency of C1 esterase inhibitor

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7
Q

urticaria - definition

A

form of allergic reaction that causes sudden swelling of the superficial layers of the skin

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8
Q

urticaria - caused by

A
  1. insects
    2, medications
  2. physical agents
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9
Q

urticaria - physical agents

A
  1. pressure (DERMATOGRAPHISM)
  2. cold
  3. vibration
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10
Q

urticaria - treatment

A
  1. antihistamines: hydroxizine, diphenhydramine, fexofenadine, loratadine, cetirizine, ranitidine
  2. leukotriene receptor antagonist; monelukast or zafirlukast
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11
Q

allergic rhinitis - definition / etiology

A
  • seasonal allergies such as hay fever are comon

- IgE depended triggering of mast cells

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12
Q

allergic rhinitis - diagnostic tests

A
  • most often clinical diagnosis with recurrent episodes
  • skin testing and blood testing for reactions to antigen may e useful to identify a specific etiology
  • allergen-specific IgE levels may be elevated
  • nasal smear: eosinophils
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13
Q

allergic rhinitis - treatment

A
  1. prevention with avoidance of the precipitating allergen
  2. intranasal corticosteroid sprays
  3. antihistamines
    4 intranasal anticholinerics (ipratropium)
  4. desenitazation to allergens that cannot be avoided
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14
Q

Immunodeficiencies - B-cells disorders - types

A
  1. X-linked (Bruton) agammaglobulinemia
  2. Selective IgA deficiency
  3. Common variable immunodeficiency
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15
Q

X-linked (Bruton) agammaglobulinemia - defect

A

Defect in BTK, a tyrosine kinase gene –> no B cell maturation (X-linked recessive –> boys)

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16
Q

X-linked (Bruton) agammaglobulinemia - presentation

A

Recurrent bacterial and enteroviral infection after 6 months (after maternal IgG protection)

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17
Q

X-linked (Bruton) agammaglobulinemia - findings

A
  1. Absent B cells in peripheral blood
  2. Low Ig of classes
  3. Absent/scanty lymph nodes and tonsils
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18
Q

Selective IgA deficiency - presentation

A

5 As

  • Majority asymptomatic
  • airway and GI infections
  • autoimmune diseases
  • Atopy
  • Anaphylaxis to IgA-containing products
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19
Q

Common variable immunodeficiency - defect

A

defect in B-cells differentiation (many causes)

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20
Q

Common variable immunodeficiency - presentation

A

Can be acquired in 20s-30s

  1. high risk of autoimmune disease
  2. bronchiectasis 3. lymphoma
  3. sinopulmonary infections
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21
Q

Common variable immunodeficiency - findings

A
  1. low plasma cells

2. low immunoglobulins

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22
Q

T-cell disorders - types

A
  1. Thymuc aplasia (DiGeorge syndrome)
  2. IL-12 receptor deficiency
  3. Autosomal dominant hyper-IgE syndrome (Job syndrome)
  4. Chronic mucocutaneous candidiasis
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23
Q

Thymuc aplasia (DiGeorge syndrome) - presentation

A
  1. Tetany (hypocalcemia)
  2. reccurent viral/fungal infections
  3. conotruncal abnormalities (ef. tetralogy of Fallot, truncus arteriosus)
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24
Q

IL-12 receptor deficiency - defect / mode of inheritance

A

low Th1 response / AR

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25
Q

IL-12 receptor deficiency - presentation

A

Disseminated mycobacterial and fungal infections
- may present after BCG vaccine administration
LOW INF-γ

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26
Q

Autosomal dominant hyper-IgE syndrome (Job syndrome) - defect / mode of inheritance

A

Deficiency of Th17 cells due to STAT3 mutation –> impaired recruitment of neutrophils to site of mutation
AD

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27
Q

Autosomal dominant hyper-IgE syndrome (Job syndrome) - presentation

A

FATED

  1. coarse Facies
  2. cold (noninflammed) staphylococcal Abscess
  3. retained primary Teeth
  4. IgE
  5. Demratoligic problems (eczema)
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28
Q

Autosomal dominant hyper-IgE syndrome (Job syndrome) - fingings

A

high IgE

low IFN-γ

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29
Q

Chronic mucocutaneous candidiasis - defect

A

T-cell dysfunction (many causes)

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30
Q

Chronic mucocutaneous candidiasis - presentation

A

Noninvasive Candida ablicans infection of skin and mucous membranes

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31
Q

Chronic mucocutaneous candidiasis - findings

A
  1. absent in vitro T-cell proliferation in response to Candida ablicans
  2. Absent cutaneous reaction to Candida ablicans
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32
Q

MC immunodeficiency

A

Selective IgA deficiency

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33
Q

B and T cell disorders - types

A
  1. Severe combined immunodeficiency
  2. Ataxia-telangiectasia
  3. Hyper-IgM syndrome
  4. Wiskott-Aldrich syndrome
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34
Q

Severe combined immunodeficiency - defect

A

several types:

a. defective IL-2R gamma chain (MC, X-linked)
b. adenosine deaminase deficinecy (AR)

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35
Q

Severe combined immunodeficiency - presentation

A
  1. Failure to thrive
  2. chronic diarrhea
  3. thrush
  4. Reccurent viral, bacterial, fungal and protozoal infections
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36
Q

Severe combined immunodeficiency - treatment

A

bone marrow transplant (no concern for rejection)

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37
Q

Severe combined immunodeficiency - findings

A
  1. decreased T-cell receptor excision circles (TRECs)
  2. absence of thymic shadow on CXR
  3. absence of germinal centers (lymph node biopsy)
  4. absence of T cells (flow cytometry)
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38
Q

Ataxia-telangiectasia - defect

A

defects in ATM gene –> failure to repair DNA double strand breaks –> cell cycle arrest

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39
Q

Ataxia-telangiectasia - presentation

A

triad: 1. cerebellar defects (Ataxia)
2. spider angiomas (telangiectasia)
3. IgA deficiency

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40
Q

Ataxia-telangiectasia - findings

A
  1. increased AFP
  2. low IgA, IgG and IgE
  3. Lymphopenia
  4. cerebellar atrophy
  5. normal level of IgM
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41
Q

Hyper-IgM syndrome - defect

A 
MC due to defective CD40L on Th cells --> class switching defect
XR
42
Q

Hyper-IgM syndrome - presentation

A
  1. severe puogenic infection early in life

2. opportunistic infection with Pneumocystis, Cryptospridium, CMV

43
Q

Hyper-IgM syndrome - findings

A
  1. normal of increased IgM

2. low IgG, IgA, IgE

44
Q

Wiskott-Aldrich syndrome - defect

A

mutation of was gene –> T cells unable to reorganize actin cytoskeleton
XR

45
Q

Wiskott-Aldrich syndrome - presentation

A

Mneominic: WATER + autoimmune + malignancy

  1. Thrmobocytopenia
  2. Eczema
  3. Reccurent infections
  4. High risk of autoimmune disease and malignancy
46
Q

Wiskott-Aldrich syndrome - findings

A
  1. low/normal IgG, IgM
  2. High IgE, IgA
  3. Fewer and smaller platelets
47
Q

Phagocyte dysfunction - types and mode of inheritance

A
  1. Phagocyte adhesion deficiency (type 1) –> AR
  2. Chediak-Higashi –> AR
  3. Chronic granulomatous disease –> XR
48
Q

Leukocyte adhesion deficiency (type 1) - defect

A

defect in LFA-1 integrin (CD18) protein on phagocytes –> impaired migration and chemotaxis
AR

49
Q

Leukocyte adhesion deficiency (type 1) - presentation

A
  1. reccurent bacterial skin and mucosa infection
  2. absent pus formation
  3. impaired wound healing
  4. delayed separation of umbilical cord (>30 days)
50
Q

Leukocyte adhesion deficiency (type 1) - findings

A
  1. increased neutrophils

2. no neutrophils at infection site

51
Q

Chediack-Hisgashi syndrome - mechanism

A

Defect in lysosomal trafficking regulator gene (LYST) –> microtubule dysfunction in phagosome-lysosome fusion
AR

52
Q

Chediack-Hisgashi syndrome - presentation

A
  1. reccurent pyogenic infections by staphyococci and streptococci
  2. partial albinism
  3. peripheral neuropathy
  4. progressive neurodegeneration
  5. infiltrative lymphohistiocytosis
53
Q

Chediack-Hisgashi syndrome - findings

A
  1. giant granules in granulocytes and platelets
  2. pancytopenia
  3. Mild coagulation defects
54
Q

Chronic granulomatous disease - mechanism

A

Defect of NADPH oxidase –> decreased ROS and respiratory burst in neutrophils
XR

55
Q

Chronic granulomatous disease - presentation

A

high susceptibility to CAT + organism

56
Q

CAT + bugs?

A

CATs Need PLACESS to Belch their Hairballs

Nocardia, Pseudomonas, Listeria, Aspergillus, Candida, E. coli, Staphylococci, Seratia, B. cepacia, H. pylori

57
Q

Chronic granulomatous disease - findings

A 
Abnormal dihydrorhodamine (flow cytometry) test (decreased green fluorescence) 
Nitroblue tetrazolium dye reduction test obsolete (if + --> less blue)
58
Q

parents refuse vaccination

A

document in the medical chart the the risks and benefits have been discussed

59
Q

contraindications of rotavirus vaccination

A
  1. anaphylaxis to vaccine ingredients
  2. history of intussusception
  3. SCID
  4. history of uncorrected congenital malformation of GI
60
Q

live-vaccines - pregnancy

A

not in pregnancy

it is ok to administrate them in other members of the house

61
Q

standard pediatric immunisations in US

A

inactivated: Polio, HAV, rabies
toxoid, diptheria, tetanus
live: MMR, rota, varicella, BCG, oral typhoid, nadal infl, smallpox
subuint: HBV, pertusis, Hemoph type B, Pneumoc, Meningococ, HPV, Influenza

62
Q

vaccinations in preterm - when

A

all according chronologic rather than gestational age in preterm infnats
THE ECXEPTION is that weight should be 2 or more Kg before the first HBV vaccine

63
Q

DTaP - how many doses and when / contains?

A

5 doses
from 6 months to 6 years
accelular pertusis antigen + diphtheria and tetanus toxoids
BOOSTER of Tdap during adolescence and then Td every 10 years

64
Q

transient hypogammaglobulinemia of infancy

A

decreased IG, variable IgM, normal IgA and B cell concentrations. –> increased sinopulmonary and GI infections (usually mild) –> normalise by age 9-15 months

65
Q

lapsed immunization

A

does not require reinstitution of the entire series

66
Q

active immunization (vaccines) in patients who recently received gamma globulin

A

live virus vaccine may have diminished immunogenecity when given shortly before or during the several months after so live vaccine is delated (3-11 months)

67
Q

egg allergy - MMR / other vaccines

A

not contraindication –> MMR is derived from chick embryo fibroblast tissue culures
on the other hand, influenza + yellow fever contain

68
Q

measles immunization after exposure

A

0-6 months: IV globulin if mother not immune
pregnant or immunocompromised: immune serum globulin
all others: vaccine within 72 h of exposure

69
Q

HAV immunization after exposure

A

if not vaccinated or receive 1 dose within the past 4 weeks –> immunoglobulins within 2 weeks

70
Q

HBC immunization after exposure

A

nonimmune patient: vaccination + globulins, repeat vaccine at 1 and 6 months

71
Q

Mumps + rubella immunization after exposure

A

not protected

72
Q

Varicella immunization after exposure

A

vaccine as soon as possible except immunocompromised, pregnant and children under 1 (VZIG)
also VGIZ for all newborn whose mother had onset of chicken pox within 5 days before delivery to 48 after

73
Q

HBV vaccination

A
  1. if mother is HBsAg (-): 3 doses until 18 months (1st soon after birth)
  2. if HBsAg (+) mother: 3 doses until month, with 1st soon after birth + globulin at 2 different sites the 1st 12 hours
74
Q

Rotavirus - doses, if missed

A

3 doses: 2, 4, 6 months
no catch-up if behind (no after 8 months)
NO IN INTUSSESUCEPTION)

75
Q

MMR doses

A

2 does:
1st 12-15 months
2nd: 4-6 years

76
Q

HAV vaccine - who / doses

A

all children more than 1 years old, chronic liver disease, homosexual, drugs, cloating factor disorder, risk for exposure
2 doses with 6 months apart

77
Q

Meningococcal Conjucate Vaccine

A

all children 11-12 years old + booster at 16

all freshmen living in dorms if not vaccinated

78
Q

Diptheria tetanus - contraindication

A

anaphylaxis

79
Q

pertussis - contraindications

A
  • anaphylaxis
  • progressive neurologic disorder (uncontrolled epilepsy, infantile spasms)
  • encephalopathy within a week of previous vaccine dose
80
Q

MCC of acquired anigoedema

A

ACEi

can occur at ANYTIME

81
Q

angioedema - management

A

epinephrine –> if no response –> tracheostomy

82
Q

PPSV 23 vs PCV 13 regarding immune response

A

PPSV23 –> polysaccharides (cannote presented to Tcells –> T cell independent B cell response (less effective in young and elderly)
PCV 13 –> conjugate –> T cell dependent B cell response

83
Q

transplantation - MCC of infection in the first 6 months

A

0-1 bacterial
1-6: opportunistic
(more than 6 months: community acquired pathogens)

84
Q

MC vaccine preventable disease among travelers

A

HAV

85
Q

vaccines for traveling to N. Africa

A

HAV
HBV
POLIO
TYPHOID FEVER

86
Q

major toxicity of mycophenolate

A

bone marrow supppression

87
Q

major tocity of azathioprine

A

dose related diarrhea
leukopenia
hepatotoxicity

88
Q

immunosuppressants - drugs

A
  1. cyclosporine
  2. tacrolimus
  3. Sirolimus (Rapamycin)
  4. Daclizumab
  5. Basiliximab
  6. Azathioprine
  7. Mycophenolate mofetil
  8. Corticosteroids
89
Q

Cyclosporine - toxicity

A
  1. Nephrotoxity
  2. hypertension
  3. hyperlipidemia
  4. neurotoxicity
  5. gingival hyperplasia
  6. hirsutism
90
Q

Tacrolimus - side effects

A
  1. diabetes
  2. neuphrotoxic
  3. neuroticity
  4. hypertension
91
Q

Tacrolimus and cyclosporine are both highly …. (side effect)

A

nephrotoxic

92
Q

Sirolimus (Rapamycin) - side effects

A
  1. pancytopenia
  2. insulin resistance
  3. Hyperlipidemia
    NOT NEPHROTOXIC
93
Q

Sirolimus (Rapamycin) is NOT (toxicity)

A

nephrotoxic

94
Q

Mycophenolate mofetil - toxicity

A
  1. GI upset
  2. pancytopenia
  3. hypertension
  4. hyperglycemia
  5. Associated with invasive CMV infecion
    LESS NEPHROTOXIC AND NEUROTOXIC
95
Q

HCV - maternal management (in pregnancy)

A
  1. Ribavirin is teratogenic (avoid)
  2. no indication for barrier protection in serodiscordant, nonogamous couples
  3. HAV + HBV vaccination
96
Q

contraindicated vaccines during pregnancy

A
  1. HPV
  2. MMR
  3. live attenuated infl
  4. VARICELLA
97
Q

recommended vaccines during pregnancy

A
  1. Tdap
  2. inactivated inf
  3. Rho (D) immunogl
98
Q

Vaccines during pregnancy - for high risk patients

A
  1. HBV
  2. HAV
  3. Pneumococcus
  4. H. infl
  5. Meningococ
  6. Varicella zoster immunogl
99
Q

All pregnant should receive screening for

A
  1. HIV
  2. HBV
  3. Chlamydia
  4. syphilis
100
Q

hyper IgM syndrome - treatment

A
  • antibiotic prophylaxis

- interval administration of IV immunoglobulin

pediatrics (16 decks)

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