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
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
3
Q
angioedema - look for
A
recent start of ACE inhibitors preceding symptoms
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
5
Q
a special characteristic of hereditary angioedema
A
it does not respond to glucocorticoids
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
7
Q
urticaria - definition
A
form of allergic reaction that causes sudden swelling of the superficial layers of the skin
8
Q
urticaria - caused by
A
- insects
2, medications - physical agents
9
Q
urticaria - physical agents
A
- pressure (DERMATOGRAPHISM)
- cold
- vibration
10
Q
urticaria - treatment
A
- antihistamines: hydroxizine, diphenhydramine, fexofenadine, loratadine, cetirizine, ranitidine
- leukotriene receptor antagonist; monelukast or zafirlukast
11
Q
allergic rhinitis - definition / etiology
A
- seasonal allergies such as hay fever are comon
- IgE depended triggering of mast cells
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
13
Q
allergic rhinitis - treatment
A
- prevention with avoidance of the precipitating allergen
- intranasal corticosteroid sprays
- antihistamines
4 intranasal anticholinerics (ipratropium) - desenitazation to allergens that cannot be avoided
14
Q
Immunodeficiencies - B-cells disorders - types
A
- X-linked (Bruton) agammaglobulinemia
- Selective IgA deficiency
- Common variable immunodeficiency
15
Q
X-linked (Bruton) agammaglobulinemia - defect
A
Defect in BTK, a tyrosine kinase gene –> no B cell maturation (X-linked recessive –> boys)
16
Q
X-linked (Bruton) agammaglobulinemia - presentation
A
Recurrent bacterial and enteroviral infection after 6 months (after maternal IgG protection)
17
Q
X-linked (Bruton) agammaglobulinemia - findings
A
- Absent B cells in peripheral blood
- Low Ig of classes
- Absent/scanty lymph nodes and tonsils
18
Q
Selective IgA deficiency - presentation
A
5 As
- Majority asymptomatic
- airway and GI infections
- autoimmune diseases
- Atopy
- Anaphylaxis to IgA-containing products
19
Q
Common variable immunodeficiency - defect
A
defect in B-cells differentiation (many causes)
20
Q
Common variable immunodeficiency - presentation
A
Can be acquired in 20s-30s
- high risk of autoimmune disease
- bronchiectasis 3. lymphoma
- sinopulmonary infections
21
Q
Common variable immunodeficiency - findings
A
- low plasma cells
2. low immunoglobulins
22
Q
T-cell disorders - types
A
- Thymuc aplasia (DiGeorge syndrome)
- IL-12 receptor deficiency
- Autosomal dominant hyper-IgE syndrome (Job syndrome)
- Chronic mucocutaneous candidiasis
23
Q
Thymuc aplasia (DiGeorge syndrome) - presentation
A
- Tetany (hypocalcemia)
- reccurent viral/fungal infections
- conotruncal abnormalities (ef. tetralogy of Fallot, truncus arteriosus)
24
Q
IL-12 receptor deficiency - defect / mode of inheritance
A
low Th1 response / AR
25
IL-12 receptor deficiency - presentation
Disseminated mycobacterial and fungal infections
- may present after BCG vaccine administration
LOW INF-γ
26
Autosomal dominant hyper-IgE syndrome (Job syndrome) - defect / mode of inheritance
Deficiency of Th17 cells due to STAT3 mutation --> impaired recruitment of neutrophils to site of mutation
AD
27
Autosomal dominant hyper-IgE syndrome (Job syndrome) - presentation
FATED
1. coarse Facies
2. cold (noninflammed) staphylococcal Abscess
3. retained primary Teeth
4. IgE
5. Demratoligic problems (eczema)
28
Autosomal dominant hyper-IgE syndrome (Job syndrome) - fingings
high IgE
| low IFN-γ
29
Chronic mucocutaneous candidiasis - defect
T-cell dysfunction (many causes)
30
Chronic mucocutaneous candidiasis - presentation
Noninvasive Candida ablicans infection of skin and mucous membranes
31
Chronic mucocutaneous candidiasis - findings
1. absent in vitro T-cell proliferation in response to Candida ablicans
2. Absent cutaneous reaction to Candida ablicans
32
MC immunodeficiency
Selective IgA deficiency
33
B and T cell disorders - types
1. Severe combined immunodeficiency
2. Ataxia-telangiectasia
3. Hyper-IgM syndrome
4. Wiskott-Aldrich syndrome
34
Severe combined immunodeficiency - defect
several types:
a. defective IL-2R gamma chain (MC, X-linked)
b. adenosine deaminase deficinecy (AR)
35
Severe combined immunodeficiency - presentation
1. Failure to thrive
2. chronic diarrhea
3. thrush
4. Reccurent viral, bacterial, fungal and protozoal infections
36
Severe combined immunodeficiency - treatment
bone marrow transplant (no concern for rejection)
37
Severe combined immunodeficiency - findings
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)
38
Ataxia-telangiectasia - defect
defects in ATM gene --> failure to repair DNA double strand breaks --> cell cycle arrest
39
Ataxia-telangiectasia - presentation
triad: 1. cerebellar defects (Ataxia)
2. spider angiomas (telangiectasia)
3. IgA deficiency
40
Ataxia-telangiectasia - findings
1. increased AFP
2. low IgA, IgG and IgE
3. Lymphopenia
4. cerebellar atrophy
5. normal level of IgM
41
Hyper-IgM syndrome - defect
```
MC due to defective CD40L on Th cells --> class switching defect
XR
```
42
Hyper-IgM syndrome - presentation
1. severe puogenic infection early in life
| 2. opportunistic infection with Pneumocystis, Cryptospridium, CMV
43
Hyper-IgM syndrome - findings
1. normal of increased IgM
| 2. low IgG, IgA, IgE
44
Wiskott-Aldrich syndrome - defect
mutation of was gene --> T cells unable to reorganize actin cytoskeleton
XR
45
Wiskott-Aldrich syndrome - presentation
Mneominic: WATER + autoimmune + malignancy
1. Thrmobocytopenia
2. Eczema
3. Reccurent infections
4. High risk of autoimmune disease and malignancy
46
Wiskott-Aldrich syndrome - findings
1. low/normal IgG, IgM
2. High IgE, IgA
3. Fewer and smaller platelets
47
Phagocyte dysfunction - types and mode of inheritance
1. Phagocyte adhesion deficiency (type 1) --> AR
2. Chediak-Higashi --> AR
3. Chronic granulomatous disease --> XR
48
Leukocyte adhesion deficiency (type 1) - defect
defect in LFA-1 integrin (CD18) protein on phagocytes --> impaired migration and chemotaxis
AR
49
Leukocyte adhesion deficiency (type 1) - presentation
1. reccurent bacterial skin and mucosa infection
2. absent pus formation
3. impaired wound healing
4. delayed separation of umbilical cord (>30 days)
50
Leukocyte adhesion deficiency (type 1) - findings
1. increased neutrophils
| 2. no neutrophils at infection site
51
Chediack-Hisgashi syndrome - mechanism
Defect in lysosomal trafficking regulator gene (LYST) --> microtubule dysfunction in phagosome-lysosome fusion
AR
52
Chediack-Hisgashi syndrome - presentation
1. reccurent pyogenic infections by staphyococci and streptococci
2. partial albinism
3. peripheral neuropathy
4. progressive neurodegeneration
5. infiltrative lymphohistiocytosis
53
Chediack-Hisgashi syndrome - findings
1. giant granules in granulocytes and platelets
2. pancytopenia
3. Mild coagulation defects
54
Chronic granulomatous disease - mechanism
Defect of NADPH oxidase --> decreased ROS and respiratory burst in neutrophils
XR
55
Chronic granulomatous disease - presentation
high susceptibility to CAT + organism
56
CAT + bugs?
CATs Need PLACESS to Belch their Hairballs
| Nocardia, Pseudomonas, Listeria, Aspergillus, Candida, E. coli, Staphylococci, Seratia, B. cepacia, H. pylori
57
Chronic granulomatous disease - findings
```
Abnormal dihydrorhodamine (flow cytometry) test (decreased green fluorescence)
Nitroblue tetrazolium dye reduction test obsolete (if + --> less blue)
```
58
parents refuse vaccination
document in the medical chart the the risks and benefits have been discussed
59
contraindications of rotavirus vaccination
1. anaphylaxis to vaccine ingredients
2. history of intussusception
3. SCID
4. history of uncorrected congenital malformation of GI
60
live-vaccines - pregnancy
not in pregnancy
| it is ok to administrate them in other members of the house
61
standard pediatric immunisations in US
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
vaccinations in preterm - when
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
DTaP - how many doses and when / contains?
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
transient hypogammaglobulinemia of infancy
decreased IG, variable IgM, normal IgA and B cell concentrations. --> increased sinopulmonary and GI infections (usually mild) --> normalise by age 9-15 months
65
lapsed immunization
does not require reinstitution of the entire series
66
active immunization (vaccines) in patients who recently received gamma globulin
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
egg allergy - MMR / other vaccines
not contraindication --> MMR is derived from chick embryo fibroblast tissue culures
on the other hand, influenza + yellow fever contain
68
measles immunization after exposure
0-6 months: IV globulin if mother not immune
pregnant or immunocompromised: immune serum globulin
all others: vaccine within 72 h of exposure
69
HAV immunization after exposure
if not vaccinated or receive 1 dose within the past 4 weeks --> immunoglobulins within 2 weeks
70
HBC immunization after exposure
nonimmune patient: vaccination + globulins, repeat vaccine at 1 and 6 months
71
Mumps + rubella immunization after exposure
not protected
72
Varicella immunization after exposure
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
HBV vaccination
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
Rotavirus - doses, if missed
3 doses: 2, 4, 6 months
no catch-up if behind (no after 8 months)
NO IN INTUSSESUCEPTION)
75
MMR doses
2 does:
1st 12-15 months
2nd: 4-6 years
76
HAV vaccine - who / doses
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
Meningococcal Conjucate Vaccine
all children 11-12 years old + booster at 16
| all freshmen living in dorms if not vaccinated
78
Diptheria tetanus - contraindication
anaphylaxis
79
pertussis - contraindications
- anaphylaxis
- progressive neurologic disorder (uncontrolled epilepsy, infantile spasms)
- encephalopathy within a week of previous vaccine dose
80
MCC of acquired anigoedema
ACEi
| can occur at ANYTIME
81
angioedema - management
epinephrine --> if no response --> tracheostomy
82
PPSV 23 vs PCV 13 regarding immune response
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
transplantation - MCC of infection in the first 6 months
0-1 bacterial
1-6: opportunistic
(more than 6 months: community acquired pathogens)
84
MC vaccine preventable disease among travelers
HAV
85
vaccines for traveling to N. Africa
HAV
HBV
POLIO
TYPHOID FEVER
86
major toxicity of mycophenolate
bone marrow supppression
87
major tocity of azathioprine
dose related diarrhea
leukopenia
hepatotoxicity
88
immunosuppressants - drugs
1. cyclosporine
2. tacrolimus
3. Sirolimus (Rapamycin)
4. Daclizumab
5. Basiliximab
6. Azathioprine
7. Mycophenolate mofetil
8. Corticosteroids
89
Cyclosporine - toxicity
1. Nephrotoxity
2. hypertension
3. hyperlipidemia
4. neurotoxicity
5. gingival hyperplasia
6. hirsutism
90
Tacrolimus - side effects
1. diabetes
2. neuphrotoxic
3. neuroticity
4. hypertension
91
Tacrolimus and cyclosporine are both highly .... (side effect)
nephrotoxic
92
Sirolimus (Rapamycin) - side effects
1. pancytopenia
2. insulin resistance
3. Hyperlipidemia
NOT NEPHROTOXIC
93
Sirolimus (Rapamycin) is NOT (toxicity)
nephrotoxic
94
Mycophenolate mofetil - toxicity
1. GI upset
2. pancytopenia
3. hypertension
4. hyperglycemia
5. Associated with invasive CMV infecion
LESS NEPHROTOXIC AND NEUROTOXIC
95
HCV - maternal management (in pregnancy)
1. Ribavirin is teratogenic (avoid)
2. no indication for barrier protection in serodiscordant, nonogamous couples
3. HAV + HBV vaccination
96
contraindicated vaccines during pregnancy
1. HPV
2. MMR
3. live attenuated infl
4. VARICELLA
97
recommended vaccines during pregnancy
1. Tdap
2. inactivated inf
3. Rho (D) immunogl
98
Vaccines during pregnancy - for high risk patients
1. HBV
2. HAV
3. Pneumococcus
4. H. infl
5. Meningococ
5. Varicella zoster immunogl
99
All pregnant should receive screening for
1. HIV
2. HBV
3. Chlamydia
4. syphilis
100
hyper IgM syndrome - treatment
- antibiotic prophylaxis
| - interval administration of IV immunoglobulin
