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Paeds BPT Written Exam > Immunology > Flashcards

Immunology Flashcards

(79 cards)

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1
Q

Which infections are associated with terminal complement deficiency (C5-9)?

A

Meningococcal septicaemia, meningitis, arthritis (mostly)
Sinopulmonary
OM, septic arthritis
Skin and soft tissue infection

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2
Q

Transmission of complement deficiencies?

A

Autosomal recessive (Except properdin deficiency which is X-linked)

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3
Q

Which complement deficiency is most common?

A

C2 deficiency most common (followed by C4 deficiency)

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4
Q

Which primary immunodeficiency is due to impaired B cell differentiation into plasma cells?

A

CVID

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5
Q

Th2 cells secrete which cytokines in response to an allergen?

A

IL-4, IL-5, IL-13

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6
Q

Which cytokine stimulates acute phase reactants from the liver?

A

IL-6

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7
Q

The action of Treg cells (which down-regulate immune response to antigens) are mediated predominantly by which 2 cytokines?

A

IL-10
TGF-Beta (transforming growth factor

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8
Q

Which 2 cytokines are involved in anti-viral response?

A

IFN alpha and IFN beta

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9
Q

Liver abscesses are pathognomonic for which inborn error of immunity?

A

Chronic granulomatous disease (CGD)

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10
Q

Investigations for C1 esterase deficiency?

A

C4 will be low
C1 esterase level and function

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11
Q

Criteria for diagnosing HLH

A

Need 5 of:
- fever
- splenomegaly
- cytopenias (2 cell lines affected)
- hypertriglyceridemia and/or hypofibrinogenemia
- haemophagocytosis on bone marrow or LN or spleen biopsy
- low or absent NK cell activity
- Ferritin >500
- Soluble CD25 > 2400

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12
Q

Which cytokines is protective against candidal infection?

A

IL-17 and IL-22

IL-17 is a pro-inflammatory cytokine which provides mucosal immunity and defence against candidal and fungal infection

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13
Q

Features of Wiskott-Aldrich syndrome?

A

X-linked (so will be male)
Small platelets
Severe, early eczema
T and B cell dysfunction - encapsulated bacteria and opportunistic infections

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14
Q

Which pathway is important for mycobacterial immunity?

A

IFN gamma /IL-12 pathway

infected macrophage with mycobacteria -> IL-12 secretion -> IFN gamma secreted by T cell or NK cell in response to IL-12

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15
Q

MHC class 1 presents antigen to?

A

CD8 T cells. It binds peptides derived from degdraded INTRACELLULAR (cytosolic) proteins unlike MHC II which bind peptides from extracellular proteins

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16
Q

MHC class 2 is expressed on which cells?

A

“Professional” antigen presenting cells
- B cells
- Macrophages
- Dendritic cells

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17
Q

Presentation of IgA deficiency

A

Often asymptomatic
May have recurrent URTIs/GIT infections if severe deficiency or absence

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18
Q

Difference between XLA and CVID?

A

CVID: reduced B cell numbers and hypogammaglobulinemia

XLA: absent B cells and agammaglobulinemia (due to deficiency of BTK which is required for B cell maturation)

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19
Q

Dose of adrenaline for anaphylaxis?

A

0.01mg/kg
1 in 1000 for IM use (= 1mg/ml)

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20
Q

Which vaccines are live vaccines?

A

MMR
Rotavirus (oral)
Typhoid (oral)
Yellow fever
BCG
One of the VZV vaccines (Zostavax) and one of the Japanese encephalitis vaccines

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21
Q

MHC class I is expressed on which cells?

A

~ all cells except mature RBCs

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22
Q

CD56 is a marker of which type of cell?

A

NK cells

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23
Q

CD19 and CD 20 is a marker of which type of cell?

A

B cells

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24
Q

CD3, CD4, CD8 are markers of which type of cell?

A

T cells
CD4= T helper
CD8= Cytotoxic T cells

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25
Main antibody secreted in breastmilk?
IgA
26
What are the 3 ways the complement pathway is activated?
1. Classical pathway (C1q binds to IgG or IgM) -> cascade resulting in activation of C3 2. Lectin (mannose binding) pathway: MBLs bind mannose on the surface of pathogens 3. Alternative pathway: C3 is spontaneously cleaved by bacterial cell wall hydroxyl groups
27
What are the three main functions of complement?
1. Opsonisation by coating with C3b 2. Formation of the membrane attack complex (C5-9) 3. Release of inflammatory mediators "Anaphylotoxins" including C3a and C5a
28
What are T cell receptor excision circles (TRECs)?
They are a marker of naive T cell development and are low/absent in SCID. TREC levels are tested as part of newborn screening for SCID Develop during T cell receptor gene rearrangement in thymus where the variable (V), Diverse (D) and Joining (J) segments of DNA are rearranged to form unique T cells with diverse specificities
29
What is tested for on newborn screening for x-linked agammaglobulinemia?
kappa-deleting recombination excision circles (KRECs). Low levels suggest low/absent B cells
30
Examples of type 2 hypersensitivity reaction?
Autoimmune condition such as ITP, autoimmune haemolytic anaemia, Graves disease, acute transfusion reaction autoantibodies (IgG or IgM) bind to antigen -> cell destruction via complement and phagocytes
31
Examples of type 3 hypersensitivity reaction?
SLE Rheumatoid arthritis Serum sickness
32
Examples of type 4 hypersensitivity reaction
Allergic contact dermatitis Granulomatous disease (eg Crohn's or Sarcoidosis)
33
Differences between urticarial vasculitis and chronic idiopathic urticaria?
In urticarial vasculitis, the lesions last >24hrs in a fixed location There is also often associated purpura, ecchymosis or hyperpigmentation
34
What % of children will outgrow milk, egg and peanut allergies?
Milk and egg: 80% Peanut: 20%
35
What defines a positive skin prick test?
Size 3mm+ greater than saline control (length+width/2) - the larger the size, the MORE LIKELY an IgE mediated reaction is to occur (doesn't tell you about severity or non-IgE mediated reactions)
36
Best test post ?IgE mediated penicillin reaction
Skin prick testing - has good NPV. Specific IgE has poor sensitivity
37
Risk of cephalosporin allergy in child with penicillin allergy?
<2% 1st generation more likely than 3rd generation to have cross reactivity
38
Features of SJS/TEN
- Onset 4-28d post exposure - painful macular erythematous blisters - erosive mucositis - palmoplantar tender erythema - prodrome of flu-like symptoms, fever
39
Features of DRESS
- Onset 2-8wks post exposure - Itchy exanthem, or urticarial papules or plaques, erythroderma - non erosive mucositis - BSA >50% Fever, oedema, lymphadenopathy - Eosinophilia - Atypical lymphocytes - Hepatitis - Renal impairment
40
Features of acute generalised exanthematous pustulosis (AGEP)?
- Onset <3days - Pustules on erythematous background, with flexural accentuation - High fever, oedema - Neutrophilia, eosinophilia 90% provoked by medications, most commonly beta lactams
41
Common causes of DRESS?
Sulfa drugs eg Bactrim Minocyclin Vancomycin Some anti epileptics eg carbamazepine Allopurinol
42
Which infections can be reactivated in DRESS?
HHV6, EBV, VZV, HHV7
43
Common causes of SJS/TEN
*Sulfonamides *Penicillin *Lamotrigine, carbamazepine and other AEDs (*Hans ChinesE) *NSAIDS * Allopurinol * Infections incl: HSV, EBV, Mycoplasma, HBV
44
Features of Ataxia-Telangiectasia
Ataxia Telangiectasia (oculocutaneous distribution) Recurrent sinopulomary infections Sensitivity to ionising radiation
45
Infections in X-linked agammaglobulinemia?
- Recurrent sinopulmonary infections with encapsulated bacteria - Giardia - Enterovirus ** Have small/absent lymph nodes and tonsils
46
Cause of X-linked agammaglobulinemia?
Bruton Tyrosine Kinase (BTK) deficiency -> pre-B cell arrest
47
Presentation of SCID
- Unwell by 3months - FTT - Persistent diarrhoea - Opportunistic infections eg candida and PJP - More severe common infections
48
What causes Hyper IgM syndrome?
Defect in gene encoding CD40 ligand on the T cell which is important in T to B cell signalling -> B cells unable to undergo class switching
49
Presentation of Hyper-IgM syndrome?
Unwell early (first 1-2yrs) PJP infection* Giardia * Recurrent sinopulmonary infections Hepatosplenomegaly Neutropenia -> gingivitis and ulcers *In the X-linked type which has a T cell defect in CD40L
50
Bloods in HyperIgM syndrome?
High IgM, Low IgA and IgG Reduced vaccine responses T cell numbers normal
51
Common organisms causing infection in phagocyte disorders (Eg CGD)?
S aureus Serratia Klebsiella Aspergillus Nocardia Burkholderia
52
Causes of DiGeorge syndrome besides 22q11
22q11 deletion accounts for 90-95% Other causes - CHARGE syndrome - TBX1 mutations - 10p13-14 mutations
53
Defect in NAPDH oxidase causes what disease?
Chronic granulomatous disease
54
In which immune deficiency are perianal abscesses common
Leukocyte adhesion defect (a phagocyte chemotaxis disorder)
55
Bloods in leukocyte adhesion defect?
- Neutrophilia (but neutrophils don't accumulate at sites of infection) - CD18 (and CD11) absence on flow cytometry
56
Investigations for CGD
- Dihydrododamine oxidation (DHR) with flow cytometry - Nitroblue tetrazolium (NBT) reduction - Direct measures of superoxide production
57
Which complement deficiency results in severe pyogenic infections with encapsulated bacteria?
C3 deficiency (C1, 2, 4 deficiency can also result in recurrent pyogenic infections)
58
Neisseria infection, which immune deficiency?
Terminal complement deficiency (C5-9) - involved in the formation of the membrane attack complex (MAC) Properdin deficiency (X-linked)
59
What does CH50 measure?
Complement classical pathway - a normal test shows that all factors (C1-9) are present
60
Which cytokine causes T cell proliferation?
IL-2
61
Most common type of SCID
Common gamma chain (X-linked recessive) T- , B+, NK -
62
What does AH50 measure?
Alternative complement pathway. Will be low with factor B, D or properdin deficiency
63
Which diseases are very common with C1 and C4 deficiency?
SLE (and occasionally get pyogenic infections) ~100% in C1q def ~57% in Cr and Cs def ~75% in C4 def
64
CRP production is stimulated by which inflammatory cytokine?
IL-6
65
Fever is mediated by which pyrogenic cytokines?
IL-1, IL-6, TNF, IFN
66
Weight for epipens?
7.5-20kg: epipen jnr (150microg) 20-50kg: epipen (300microg) >50kg: anapen (500microg)
67
Prophylaxis in hereditary angioedema
C1 inhibitor Lanadelumab (mab against kallikrein) Berotralstat (kallikrein inhibitor) TXA Attenuated androgens (post pubertal)
68
Management in acute hereditary angioedema attack?
Recombinant C1 inhibitor Lanadelumab (mab against kallikrein) Berotralstat (kallikrein inhibitor) TXA and FFP
69
What is gluten found in?
Wheat, barley, oats, rye
70
Which disease is a phagocyte chemotaxis defect?
Leukocyte adhesion defect
71
Which types of leukocyte adhesion defect have delayed umbilical cord separation
Type 1 and 3 Type 1: Deficiency or dysfunction of beta 2 integrin family Type 2:
72
What is the risk of subsequent systemic reaction to a insect sting after a) cutaneous reaction b) anaphylaxis
Future risk of anaphyalxis/systemic reaction = 10% after cutaneous reaction, 40% after previous systemic reaction
73
MOA of eculizumab?
Antibody against C5 complement protein Prevents cleavage of C5 to C5a (anaphylotoxin) and C5b, preventing formation of the MAC Used in atypical HUS
74
T-cell independent vaccinations?
Polysaccharide vaccines eg pneumococcal and meningococcal polysaccharide vaccines
75
Distinguishing features of FMF
- Febrile episodes lasting 1-3 days with serositis, mono arthritis and erysipelas like rash - Present age <20yrs
76
Distinguishing features of Hyper IgD syndrome?
- Presents age <1 - Episodes of fever lasting 3-7 days with abdo pain, diarrhoea, N,V, cervical lymphadenopathy, diffuse macular rash + ulcers
77
Distinguishing features of TRAPS?
- Presents age <10yrs - Long episodes of fever (up to 21 days) with severe pain - get periorbital oedema and conjunctivitis
78
Distinguishing features of CAPS?
- NOMID presents in neonatal period - Get urticarial rash - Muckel Wells causes SNHL
79

Paeds BPT Written Exam (18 decks)

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