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Paeds BPT Written Exam > Immunology > Flashcards

Immunology Flashcards

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
Q

Main antibody secreted in breastmilk?

A

IgA

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26
Q

What are the 3 ways the complement pathway is activated?

A
  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
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27
Q

What are the three main functions of complement?

A
  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
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28
Q

What are T cell receptor excision circles (TRECs)?

A

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

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29
Q

What is tested for on newborn screening for x-linked agammaglobulinemia?

A

kappa-deleting recombination excision circles (KRECs). Low levels suggest low/absent B cells

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30
Q

Examples of type 2 hypersensitivity reaction?

A

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

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31
Q

Examples of type 3 hypersensitivity reaction?

A

SLE
Rheumatoid arthritis
Serum sickness

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32
Q

Examples of type 4 hypersensitivity reaction

A

Allergic contact dermatitis
Granulomatous disease (eg Crohn’s or Sarcoidosis)

33
Q

Differences between urticarial vasculitis and chronic idiopathic urticaria?

A

In urticarial vasculitis, the lesions last >24hrs in a fixed location
There is also often associated purpura, ecchymosis or hyperpigmentation

34
Q

What % of children will outgrow milk, egg and peanut allergies?

A

Milk and egg: 80%
Peanut: 20%

35
Q

What defines a positive skin prick test?

A

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
Q

Best test post ?IgE mediated penicillin reaction

A

Skin prick testing - has good NPV.
Specific IgE has poor sensitivity

37
Q

Risk of cephalosporin allergy in child with penicillin allergy?

A

<2%
1st generation more likely than 3rd generation to have cross reactivity

38
Q

Features of SJS/TEN

A
  • Onset 4-28d post exposure
  • painful macular erythematous blisters
  • erosive mucositis
  • palmoplantar tender erythema
  • prodrome of flu-like symptoms, fever
39
Q

Features of DRESS

A
  • 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
Q

Features of acute generalised exanthematous pustulosis (AGEP)?

A
  • Onset <3days
  • Pustules on erythematous background, with flexural accentuation
  • High fever, oedema
  • Neutrophilia, eosinophilia

90% provoked by medications, most commonly beta lactams

41
Q

Common causes of DRESS?

A

Sulfa drugs eg Bactrim
Minocyclin
Vancomycin
Some anti epileptics eg carbamazepine
Allopurinol

42
Q

Which infections can be reactivated in DRESS?

A

HHV6, EBV, VZV, HHV7

43
Q

Common causes of SJS/TEN

A

*Sulfonamides
*Penicillin
Lamotrigine, carbamazepine and other AEDs (Hans ChinesE)
*NSAIDS
* Allopurinol
* Infections incl: HSV, EBV, Mycoplasma, HBV

44
Q

Features of Ataxia-Telangiectasia

A

Ataxia
Telangiectasia (oculocutaneous distribution)
Recurrent sinopulomary infections
Sensitivity to ionising radiation

45
Q

Infections in X-linked agammaglobulinemia?

A
  • Recurrent sinopulmonary infections with encapsulated bacteria
  • Giardia
  • Enterovirus

** Have small/absent lymph nodes and tonsils

46
Q

Cause of X-linked agammaglobulinemia?

A

Bruton Tyrosine Kinase (BTK) deficiency -> pre-B cell arrest

47
Q

Presentation of SCID

A
  • Unwell by 3months
  • FTT
  • Persistent diarrhoea
  • Opportunistic infections eg candida and PJP
  • More severe common infections
48
Q

What causes Hyper IgM syndrome?

A

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
Q

Presentation of Hyper-IgM syndrome?

A

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
Q

Bloods in HyperIgM syndrome?

A

High IgM, Low IgA and IgG
Reduced vaccine responses
T cell numbers normal

51
Q

Common organisms causing infection in phagocyte disorders (Eg CGD)?

A

S aureus
Serratia
Klebsiella
Aspergillus
Nocardia
Burkholderia

52
Q

Causes of DiGeorge syndrome besides 22q11

A

22q11 deletion accounts for 90-95%

Other causes
- CHARGE syndrome
- TBX1 mutations
- 10p13-14 mutations

53
Q

Defect in NAPDH oxidase causes what disease?

A

Chronic granulomatous disease

54
Q

In which immune deficiency are perianal abscesses common

A

Leukocyte adhesion defect (a phagocyte chemotaxis disorder)

55
Q

Bloods in leukocyte adhesion defect?

A
  • Neutrophilia (but neutrophils don’t accumulate at sites of infection)
  • CD18 (and CD11) absence on flow cytometry
56
Q

Investigations for CGD

A
  • Dihydrododamine oxidation (DHR) with flow cytometry
  • Nitroblue tetrazolium (NBT) reduction
  • Direct measures of superoxide production
57
Q

Which complement deficiency results in severe pyogenic infections with encapsulated bacteria?

A

C3 deficiency
(C1, 2, 4 deficiency can also result in recurrent pyogenic infections)

58
Q

Neisseria infection, which immune deficiency?

A

Terminal complement deficiency (C5-9) - involved in the formation of the membrane attack complex (MAC)

Properdin deficiency (X-linked)

59
Q

What does CH50 measure?

A

Complement classical pathway - a normal test shows that all factors (C1-9) are present

60
Q

Which cytokine causes T cell proliferation?

A

IL-2

61
Q

Most common type of SCID

A

Common gamma chain (X-linked recessive)
T- , B+, NK -

62
Q

What does AH50 measure?

A

Alternative complement pathway. Will be low with factor B, D or properdin deficiency

63
Q

Which diseases are very common with C1 and C4 deficiency?

A

SLE
(and occasionally get pyogenic infections)

~100% in C1q def
~57% in Cr and Cs def
~75% in C4 def

64
Q

CRP production is stimulated by which inflammatory cytokine?

A

IL-6

65
Q

Fever is mediated by which pyrogenic cytokines?

A

IL-1, IL-6, TNF, IFN

66
Q

Weight for epipens?

A

7.5-20kg: epipen jnr (150microg)
20-50kg: epipen (300microg)
>50kg: anapen (500microg)

67
Q

Prophylaxis in hereditary angioedema

A

C1 inhibitor
Lanadelumab (mab against kallikrein)
Berotralstat (kallikrein inhibitor)
TXA
Attenuated androgens (post pubertal)

68
Q

Management in acute hereditary angioedema attack?

A

Recombinant C1 inhibitor
Lanadelumab (mab against kallikrein)
Berotralstat (kallikrein inhibitor)
TXA and FFP

69
Q

What is gluten found in?

A

Wheat, barley, oats, rye

70
Q

Which disease is a phagocyte chemotaxis defect?

A

Leukocyte adhesion defect

71
Q

Which types of leukocyte adhesion defect have delayed umbilical cord separation

A

Type 1 and 3
Type 1: Deficiency or dysfunction of beta 2 integrin family
Type 2:

72
Q

What is the risk of subsequent systemic reaction to a insect sting after
a) cutaneous reaction
b) anaphylaxis

A

Future risk of anaphyalxis/systemic reaction = 10% after cutaneous reaction, 40% after previous systemic reaction

73
Q

MOA of eculizumab?

A

Antibody against C5 complement protein

Prevents cleavage of C5 to C5a (anaphylotoxin) and C5b, preventing formation of the MAC

Used in atypical HUS

74
Q

T-cell independent vaccinations?

A

Polysaccharide vaccines eg pneumococcal and meningococcal polysaccharide vaccines

75
Q

Distinguishing features of FMF

A
  • Febrile episodes lasting 1-3 days with serositis, mono arthritis and erysipelas like rash
  • Present age <20yrs
76
Q

Distinguishing features of Hyper IgD syndrome?

A
  • Presents age <1
  • Episodes of fever lasting 3-7 days with abdo pain, diarrhoea, N,V, cervical lymphadenopathy, diffuse macular rash + ulcers
77
Q

Distinguishing features of TRAPS?

A
  • Presents age <10yrs
  • Long episodes of fever (up to 21 days) with severe pain
  • get periorbital oedema and conjunctivitis
78
Q

Distinguishing features of CAPS?

A
  • NOMID presents in neonatal period
  • Get urticarial rash
  • Muckel Wells causes SNHL
79
Q
A

Paeds BPT Written Exam (18 decks)

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