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CSP1 > CSP3 > Flashcards

CSP3 Flashcards

1
Q

Define hypersensitvity

A

Damage to a patient caused by the immune system

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2
Q

Define allergy

A

Subdivision of hypersensitivity

- unecessary immune response against foreign antigen (type 1 hypersensitvity)

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3
Q

Type 1 hypersensitvity

A

IgE mediated
Mast cells
Anaphylaxis

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4
Q

Type 2 hypersensitvity

A

Autoantibodies against self structures

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5
Q

Type 3 hypersensitivity

A

Immune complex mediated

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6
Q

Type 4 hypersensitvity

A

T cells and macrophages

With out without granulomas

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7
Q

Type 5 hypersensitivity

A

Stimulatory autoantibodies

Graves’

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8
Q

What stimulates mast cells?

A

Antigen binding to IgE on surface
Complement activation = C5a, C3a
Nerves = axon reflex sensory nerves, substance P
Direct stimulation

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9
Q

Diagnostic test for anaphylaxis

A

Tryptase
C4 not consumed (low)
D dimers high

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10
Q

Causes of anaphylaxis

A
  • arthropod venoms (bee, wasp, stings)
  • drugs (ABs, insulin, ACTH, suxamethonium)
  • foods (peanute, shellfish, egg, milk, latex, kiwi, alpha gliadin)
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11
Q

Differentials of anaphylaxis

A 
MI
PE
Hyperventilation
Hypoglycaemia
Phaeo
Angioedema
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12
Q

Define anaphylactoid reactions

A

Direct or indirect activation of mast cells without IgE

Still tryptase positive as involve mast cells

Same treatment as anaphylaxis

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13
Q

Triggers of anaphylactoid reactions

A

Vancomycin, NSAIDs, opiates, anaesthetic agents
Srawberries
Exercise, cold, trauma
Immune complex reactions to blood products

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14
Q

Scromboid reactions define

A

Massive ingestion of histamine from decayed mackerel/other oily fish
Tryptase negative as mast cells not involved

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15
Q

Urticaria

A

Chronic if >1 month but can be acute
Wheals/hives/raised itchy
Mast cells, histamine, leukotrienes
Dermis inflammation

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16
Q

Causes of urticaria

A

Allergic triggers
Direct contact - grass, latex
Allergic normally acute
Infections = viral, parasites
Autoimmune = vasculitis, SLE, type 3 hypersensitivity (normally chronic)
Autonomic = with heat/sweating, adrenergic with stress
Physical = sunlight/pressure/vibration/heat
Exercise: within hours of a meal
Hormonal: menstrual cycle link, steroid use
Mast cell disorders: urticaria pigmentosa
Iron/folate/B12 deficiency

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17
Q

Cold urticaria

A

Cryoglobulinaemia
Other autoimmune = SLE, leukaemia
Can cause Raynaud’s phenomenon
Can be inherited = C1AS1 gene mutation

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18
Q

Treatment of urticaria

A

Antihistamines - up to 4x recommended dose
Can add H2 antagonist (ranitidine)
Add montelukast
Progress to omalizumab (before was cyclosporin)
Treat lymphomas/autoimmune diseases accordingly

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19
Q

Non sedating antihistamines

A

Cetirizine
Loratidinde
Fexofenidine

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20
Q

Sedating antihistamines

A

Chloramphenamine

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21
Q

Define angioedema

A

Swelling of subcutaneous tissues

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22
Q

Causes of angioedema

A

Allergic (accompanied by urticaria)
Anaphylaxis/anaphylactoid syndromes
Hereditary

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23
Q

Hereditary Angioedema Type 1

A

C1 esterase inhibitor

Restraint on complement and bradykinin pathways

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24
Q

Hereditary Angioedema Type 2

A

C1 esterase inhibitor mutation

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25
Q

Hereditary Angioedema Type 3

A

Mutation of factor XII initiating bradykinin pathway and intrinsic clotting pathway

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26
Q

Acquired C1 esterase inhibitor deficiency

A

Angioedema but not hereditary as acquired

Autoimmune, haem malignancy, cryoglobulins, Heb C/B/Helicobacter

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27
Q

Idiopathy angioedema

A

Normal C1 esterase inhibitor level

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28
Q

treatment of angioedema

A

If allergic - same as anaphylaxis and urticaria
C1 inhibitor deficiency = anabolic steroids (tranexamic acid)/C1 inhibitor IV or SC
Idiopathic = tranexamic acid, icatibant SC inhibits bradykinin pathway

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29
Q

Allergic conjunctivitis, rhinitis, sinusitis

A

Type 1 hypersensitivity

IgE mediated

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30
Q

Antigens for allergic conjunctivitis/rhinitis/sinusitis

A 
Grass pollens
Tree pollens
Seasonal exposure
Animal danders
House dust mite
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31
Q

Diagnosis of allergic conjunctivitis/rhinitis/sinusitis

A 
Mainly History
Seasonal = pollens, hayfever
Skin prick
IgE specific test
May develop nasal polyps
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32
Q

Treatment for allergic conjunctivtis, rhinitis, sinusitis

A

Topical/systemic antihistamines
Topical mast cell stabilisers
Topical steroids
Antigen exclusion

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33
Q

Causes of rhinitis & sinusitis

A 
Infections - common
Vaso-motor rhinitis
Non ellergic rhinitis with eosinophilia (NARES)
Drusg = alpha agonist sprays, cocaine
Irritant fumes/solvents
Wegner's
Septal deviation/foreign bodies
Late pregnancy oestrogens
CSF leak
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34
Q

Define atopy

A

Genetic predisposition to make IgE antibody against common environmental antigens
Associated with hayfever, childhood asthma and atopic dermatitis

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35
Q

Atopic dermatitis

A 
Dry cracked itchy raised lesion
Extensor surfaces first then flexures and cheeks
Genetic component
Filaggrin mutation
IgE>1000 raised
Staph infections of affected areas
Increased eosinophils and mast cells
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36
Q

Aspirin sensitivity

A

Can cause angioedema
Triad = asthma, nasal polyps, sinusitis
Can enhance ability of other allergens to cause anaphylaxis

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37
Q

Nasal polyps

A

May need surgery
Topical steroids
Avoid aspirin and salicylates in food(tea/coffee/spices/tomatoes/cider/wine/mints)

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38
Q

Oral allergy syndrome

A

Itching and local swelling in oropharynx within minutes of eating
Rarely progresses to urticaria & angioedema and maybe anaphylaxis
IgE Type 1

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39
Q

Antigens for oral allergy syndrome

A

Fresh fruit/veg and cross react with pollens so often have hayfever too

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40
Q

treatment for oral allergy syndrome

A

Avoid foods

Cooked normally ok

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41
Q

Food allergy symptoms

A 
Gut pain, bowel changes, vomiting
Urticaria
Angioedema
Within minutes
Anaphylaxis?
Enteritis if eosinophillic gastropathy
HISTORY crucial
Skin prick tests
avoidance
Steroids
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42
Q

Latex allergy

A 
Gloves, medical products, condoms
Cross reactions = bananas/avacado/kiwi
IgE mediated against latex protein (plant based)
Contact urticaria
Asthma
Angioedema
Anaphylaxis
Rhino-conjunctivitis
Skin prick
Specific IgE testing
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43
Q

Drug allergies - penicillin

A

type 1 = anaphylaxis
2 = haemolytic anaemia
3 = serum sickness
4 = interstitial nephritis

+ erythema multiforme & Stevens Johnson

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44
Q

other drug allergies

A 
Co-trimoxazole
Sulphonamide
Insulin = urticaria, anaphylactic like
Anesthetics = measure tryptase
DRESS syndrome = weeks after rash/fever/lymph, eosinophillia
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45
Q

Extrinsic allergic alveolitis

A 
Type 3
inhaled foreign antigens
occupational
6 hours after, worst at 24-48
fever, cough, SOB
asthma
may develop type 4
chronic exposure - interstitial pulmonary disease
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46
Q

Allergic bronchopulmonary aspergillosis

A

Type 3 & 1
Wheeze, cough, fever, haemoptysis
Can dev. bronchiectasis
High IgE and IgE

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47
Q

Serum sickness

A 
Infused human or serum products
Monoclonal AB
Type 3
Fever, urticaria, vasc., lymph, poly-arthritis
7-14 days post primary exposure
1-3 days after secondary exposure
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48
Q

Contact hypersensitivity

A 
Type 4
Nickel allergy
topical drugs
leather dyes
chromium cement
latex and rubber
hair dyes/fragrance
plants
sunlight may be required to trigger sensitivity
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49
Q

Special tests overall

A 
HISTORY!!!
Skin prick tests
Specific IgE in vitro tests
Autoantibody screen
Patch testing
Tryptase
D-dimers
Complement - C3, C4, C1q, C2, C1q autoantibodies
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50
Q

Extracellular pathogen targets

A

Blood
Lymph
Interstitial spaces
Epithelial surfaces

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51
Q

Intracellular Pathogen targets

A

Cytoplasmic

Vesicular

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52
Q

When does adaptive immune response begin?

A

Innate immune response begins first

Then reaches threshold where pathogen level increases where adaptive is activated

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53
Q

Immune response from a cute

A
  • wound bleeds washing out foreign bodies
  • resident macrophages and injured tissue initiate inflammatory response
  • interleukins and cytokines activated- specificaly IL1,6,8 and TNFalpha
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54
Q

How are interleukins actiavted?

A

TLR recognise pathogen features

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55
Q

TLR1 & 2

A

Gram positive
bacterial products
yeast

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56
Q

TLR3

A

dsRNA

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57
Q

TLR4

A

LPS

Gram negative

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58
Q

TLR5

A

Flagellin

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59
Q

TLR6

A

Complex

With TLR2

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60
Q

TLR7 & 8

A

ss RNA

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61
Q

TLR9

A

bacterial DNA

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62
Q

What do interleukins then do?

A 
Inflammation
Driving innate immune response
Fluid enters site
Clot forms sealing wound
NK cells, neutrophils, macrophages and IgM attracted
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63
Q

How does innate immunity initiate adaptive

A

Dendritic cells phagocytose bacterial antigen

Become APC

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64
Q

How does a dendritic cell mature?

A

Immature releases LPS which is detected by TLR4

Moves from tissues to lymph nodes

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65
Q

What do dendritic cells do once matured?

A

Activate naive T cells binding to them via MHC

CD4+ differentiate to either Th1 or Th2 effector cells

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66
Q

CD4+ to Th1

A

Via IL12

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67
Q

CD4+ to Th2

A

Via IL4

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68
Q

What produces IL4

A

Parasitic worms via IgE

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69
Q

What produces IL12

A

Dendritic cell

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70
Q

Function of Th1 cells?

A

Produce IFNgamma which:
cell mediated immunity
helps cytotoxic T cells and macrophages

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71
Q

Function of Th2 cells?

A

Produce IL4,5,13
Humoral Immunity
Help B cells for antibody production via plasma cell production

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72
Q

TB Leprosy

A 
Th1 dominant response
IFN-gamma
Activated macrophages
Low organism number
Limited disease
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73
Q

Lepromatous Leprosy

A 
Th2 dominant response
IL4,5,13
Hyperglobulinemia
High no. of organisms
Disseminated disease
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74
Q

Cutaneous leishmaniasis

A

boils
Th1 dominant
limited

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75
Q

Visceral lieshmaniasis

A

Black Fever
Th2 dominant
Disseminated

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76
Q

Miliary TB

A

Th2 dominant

Disseminated

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77
Q

Memory cells

A

Fraction of activated T and B cells become memory cells
Provide long lasting immunity
Stored in bone marrow and lymph nodes

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78
Q

Features of bronchial asmtha

A

Mucus hypersecretion
Airway inflammation
Bronchial hyper-responsiveness
Acute attacks of SOB and airway obstruction due to SM contraction

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79
Q

2 phases of asthms

A 
1 = early bronchospasm
2 = late inflammation
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80
Q

Spasmogens examples asthma early phase

A

Histamine

Methacholine

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81
Q

Histological asthma chages

A

Goblet cell hyperplasia
Thick sub-basement membrane
Cellular infiltrate

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82
Q

IgE production is asthma

A

Dendritic cells present allergen to T and B cells at LN

- T helper switch to B cells via IL4,13 and CD14 on B cells bind to ligand on T cells

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83
Q

Role of IgE in asthma

A

Binds to IgE receptors on mast cells via FcR1

Binds to low affinity receptors on lymphocytes, eosinophils, platelets and macrophages via FcR2

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84
Q

Role of mast cells

A

Release mediators:

  • histamine
  • leukotrienes
  • cytokines
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85
Q

Early effects of mediators

A

bronchospasm
OEdema
Airflow obstruction

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86
Q

Late effects of mediators

A

Airway inflammation
Airway obstruction
Airway hyper-responsiveness

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87
Q

Eosinophils

A
  • originate in bone marrow
  • IL3 and 5 regulate
  • leukotriene source
  • contain proteins which damage epithelium
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88
Q

Terminal differentiation of eosinophils

A

By GM-CSF & IL5

  • IL5 prolongs survival
  • Increased IL5 associated with tissue eosinophilia
  • Anti-IL5 therapy = mepolizumab, reslizumab
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89
Q

Lymphocytes

A

Surface activation markers
CD4+ Th1 - IL2 and IFNgamma
Th2 - IL4,5,6,9,13

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90
Q

GATA-3 mRNA

A

TF confined to Th2 in asthmatics

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91
Q

Factors favouring Th1 phenotype

A

Presence of older siblings
Early exposure to day care
TB, measles, Hep A
Rural environment

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92
Q

Factors favoring Th2 phenotypes

A 
Widespread AB use
Western lifestyles
Urban environment
Diet
Sensitization to house dust mites and cockroaches
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93
Q

Define immunodeficiency

A

Suboptimal resistance to infectious disease

Primary & secondary

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94
Q

Primary immunodeficiency

A

Genetically mediated = X linked agammaglobulaemia, Di George

Idiopathic = Common variable immune deficiency

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95
Q

Secondary Immunodeficiency

A 
Infections = AIDS
Malignant = lymphoma, myeloma, leukaemia
Metabolic = DM
Trauma = burns
Treatment caused = cytotoxic drugs, glucocorticoids, irradiation
AB loss = nephrotic syndrome
  • more common than primary
96
Q

B lymphocyte & AB deficiency

A 
Pneumococcus
Haemophilus
Staphylococcus
Moraxella
Strep
Neisseria
Mycoplasma
Enteroviruses 
Norovirus
Rhinovirus
Otitis Media
Sinusitis
LRTI
Meningitis
Septicaemia
Septic arthritis
Osteomyeltiis
Abscess
97
Q

X Linked Agammaglobulaemia

A
  • no antibodies
  • no B lymphocytes
  • defective tyrosine kinase gene for B lymphocytes
  • very rare
  • boys>
  • 2nd 6m of life with chest infections/ear/sinusitis
  • will get worse infections if not treated -> bronchiectasis
  • die in childhood or early adulthood if not treated
  • can also get viruses
98
Q

Treatment of X linked agammaglobulaemia

A

AB replacement - almost all IgG
AB when necessary
Then normal lifespan

99
Q

Common Variable Immune Deficiency

A
  • not common
  • any time presents
  • small degree of inherited
  • B lymph normal/low/absent
  • low AB
  • impaired immunisation response
  • T lymph may be affected
  • autoimmunity associated?/granulomatous disease?
  • get infections like X linked
  • severity depends on AB levels
  • Need AB replacement ASAP
100
Q

Isolated AB class deficiency

A

IgA = low or absent with normal IgG and IgM

  • at any time
  • often healthy otherwise
  • RTI, intestinal infections
  • v. rare IgM inherited deficiencies, aut recessive in consanguineous families
101
Q

Secondary AB deficiency causes

A
  • drugs
  • nephrotic syndrome = urine low
  • protein losing enteropathy = loss into bowel lumen
  • malignancies = lymphomas (with T lymp defect)
  • cytotoxic therapy + immunosuppressive = with T lymph deficiency and neutrophil deficiency
  • rituximab = B lymphocyte depletion so specific AB deficiency
102
Q

Fungi causing T lymphocyte deficiency

A 
Pneumocystis jeroveci
Candida
Cryptococcus
Histoplasma
Skin
103
Q

Protozoa causing T lymphocyte deficiency

A

Cryptosporidium = diarrhoea

Toxoplasma

104
Q

Viruses causing lymphocyte deficiency

A

Herpes Simplex, Zoster
CMV
EBV
HHV 8

105
Q

Mycobacteria causing T lymphocyte deficiency

A

Atypical

106
Q

Di George Syndrome

A

Primary T lymph deficiency cause

  • chromosome 22 partial deletion
  • pharyngeal 3rd & 4th arch defects
  • no thymus or parathyroids
  • vessel abnormalities
  • facial abnormalities
  • complete thymus absence = complete T lymph absence
  • often incomplete thymus = T lymph gradually improves with age
107
Q

Nucleoside phosphorylase and adenine deaminase deficiency

A

T lymph deficiency primary cause

- enzymes specific to T lymph

108
Q

AIDS

A
  • caused by HIV
  • T lymph deficiency
  • retrovirus
  • CD4 targeted
  • now antiretroviral treatment
109
Q

Diagnostic test of AIDS

A

AB to HIV
p24 viral antigen
HIV RNA

110
Q

Neutropenia

A
  • oral soreness/gingivitis/pharyngitis
  • then URTI/LRTI
  • soft tissue infections and abscess
  • septicaemia
111
Q

Bacteria causing neutropenia

A

Gram negative aerobic rods = pseudomonas, E Coli, entrobacteria, enterococci
Non beta haemolytic strep
Staph - epidermidis, saprophyticus

112
Q

Fungi causing neutropenia

A

Candida spp.
Aspergillus spp.
Dermatophyte fungi

113
Q

Primary causes of neutropenia

A 
Rare genetic syndromes:
Cyclical neutropenia
Severe congenital neutropenias
Kostmann syndrome
Chronic benign neutropenia
114
Q

Secondary causes of neutropenia

A 
Marrow aplasia
Haem malignancies
Drugs - cytotoxic therapy
Autoimmune - anti-neutrophil AB
Infections
115
Q

Chronic granulomatous disease

A

Neutrophil function defects

  • X linked recessive
  • boys
  • childhood presentation
  • pneumonia/infective arthirits/abscess/skin infections
  • end as granulomas
116
Q

Test for chronic granulomatous disease

A

Nitroblue tetrazolium test
OR
Dihydrorhodamine test

Normal phagocytes decolourise dyes, patients’ do not

117
Q

Treatment fro chronic granulomatous disease

A

AB
IFN
Bone marrow transplant

118
Q

Leukocyte Adhesion Deficiency (LAD)

A 
Very rare
aut recessive
CD18 deficiency
Associates with CD11 forming beta 2 integrin (neutrophil adhesion molecule)
Failure of umbilical cord to slough
119
Q

Test for LAD

A

CD11/18 expression measured

120
Q

Treatment for LAD

A

AB

Bone marrow or stem cell transplant

121
Q

Possible mechanisms for diabetes causing infections

A

Neutrophil function impaired
Microcirculation impaired
Body fluids more nutritious for bacteria

122
Q

Complement defects

A

Genetic
Lupus like picture
Meningitis danger
Often healthy individual

123
Q

Low C3

A

Bacterial infections

124
Q

Tests for complement defects

A

C3 and C4 routine
C1q
C2
Factors H,I,B

125
Q

Angioedema

A

ACEi can cause

126
Q

Absent/small spleen

A

Congenital
Infarcted in SC
Hyposplenism in coeliac
Removed for hereditary spherocytosis and lymphoma, trauma, idiopathic thrombocytopenia, hypersplenism

127
Q

Typical infections causing absent/small spleen

A 
Septicaemia
Pneumococcus
Haemophilus
Meningococcus
Malaria
128
Q

Treatment for absent/small spleen

A

Prophylaxis AB = pen V or macrolide
Vaccines - pneumococcal, meningococcal, Haem Infl.
Better before splenectomy
Carry INFO CARD

129
Q

Defects of anatomical innate immune system

A 
CF
Bronchiectasis
Urinary Outflow Obsrtuction
Cilia defects = Kartagener's syndrome and smoking
Burns/wounds
Poor phlebotomy, catheterisation
Indwelling lines
130
Q

Reticular Dysgenesis

A
  • severe combined immune deficiency
  • rare genetic syndrome
  • only red cells and platelets present in blood
  • few days of life before overwhelming infection
  • sterile isolation needed until bone marrow transplant
131
Q

SCID

A

Severe combined immune deficiency

  • have phagocytes
  • no B or T lymph
  • 2 years of life with AB support in sterile isolation
  • need bone marrow transplant
132
Q

Hyper IgM syndrome

A
  • very rare
  • inherited defect
  • loss of signal from activated CD4 T helper cells to APCs
  • no B cell class switching
  • High IgM, low G and A
  • pneumocystis carinii
  • mutation of CD40 ligand on T lymph is commonest cause, X linked
  • CD40 lack on APC is rarer cause, aut recessive
133
Q

Hyper |gE syndrome

A

Job’s syndrome if STAT3 mutation

  • eosinophilia
  • facial and skeletal abnormalities
  • eczema, staph infec, candida, pneumonia
134
Q

Wiskott-Aldrich syndrome

A

X linked

  • defective cytoskeletal protein found in haemoapoetic cells
  • thrombocytopenia = bleeding
  • affects B and T lymph & phagocytes
  • ear, sinuses infections, lymphomas
135
Q

Chediak-Higashi syndrome

A
  • defect moving material into lysosomes
  • phagocyte affected
  • affects production of cytolytic granules in T lymph and NK cells
  • neutropenia
  • peripheral neuropathy
  • bacterial infections and fungi
136
Q

Ataxia telangectasia

A 
aut recessive
defect in ATM gene for DNA repair
- whites of eyes = telangectasia
- T & B lymph affected
- infections, lymphoma, leukaemia
137
Q

Secondary causes of combined defects

A
  • common
  • gluco-corticosteroids
  • azathioprine
    mycophenolate
    MTX
    Ciclosporine
    Tacrolimus
    Inflixmab block TNF
138
Q

Ix for immunodeficiency diseases

A

FBC
ESR & CRP
U&E, LFT, bone profile, TFT
C3 & C4, C1q, C2, C1 esterase inhibitor
Mannose binding ligand
Immunoglobulins - IgG, A, M
Specific AB to infections before and after immunisations,
CD3,4,8,19,56
Measure T lymph proliferation response to stimulation
Neutrophils number
genetic tests
Infections = cultures, swabs, HIV, Hep B and C, EBV, CMV, imaging

139
Q

Define commensal organism

A

Micro-organism that derives benefit from another organism without causing damage

140
Q

Define primary pathogen

A

Cause of disease in healthy hosts

141
Q

Iatrogenic causes of immunodeficiency

A

2ry cause

Treatments - steroids, chemo

142
Q

Non iatrogenic causes of immunodeficiency

A

2ry cause

due to disease process

143
Q

Which organisms damage skin barrier and how?

A

Flora
Perineal flora
Bacillus fungi
Via surgery, trauma, burns, cannula

144
Q

Which organisms damage mucosa barrier and how?

A 
Normal commensal flora translocate
Gram negative
Candida
Aerobes
Via chemo, broad spectrum AB
145
Q

Which organisms damage resp tract barrier and how?

A

Intubation
From GI and URT secretions
Candida
Gram -ve

146
Q

Which organisms damage urinary tract barrier and how?

A

Catheter
Perineal flora
Gram -ve
Candida

147
Q

Which organisms damage vaginal barrier and how?

A

Broad spectrum AB

Candida

148
Q

What diseases cause primary caused phagocyte defects?

A

Chronic granulomatous disease

Neutrophil migration defects

149
Q

What diseases cause secondary cause phagocyte defects?

A 
AML
Aplastic anaemia
Diabetes
Chemo associated neutropenia
Corticosteroids - impair neutrophil function
150
Q

Which organisms usually cause disease in phagocyte defects?

A 
S. aureus
Pseudomonas
Aspergillus
Zygomycetes
Normal commensals
151
Q

Prevention of disease due to phagocyte defects

A

Prophlactic AB early = quinolones
Antifungals
IFNgamma
Vaccinations

152
Q

What things cause secondary complement defects?

A

Eculizumab

Myeloma auto antibodies

153
Q

Impact of complement defects

A

Increased risk of neisseria meningitis
Meningococcal meningitis
Menigococcal sepsis

154
Q

Prevention of disease due to complement defects

A

Proph AB

Vaccinations

155
Q

Causes of primary humoral immune defects

A

IgA defciiency

Hyper IgM

156
Q

Causes of secondary humoral immune defects

A

CLL
Splenectomy
Corticosteroids
Rituximab

157
Q

Impact of humoral immune defects

A

Strep pneumoniae
H. influenzae
Neisseria meningitis
(encapsulated organisms if IgG deficiency)
GI pathogens (protozoa) if IgA deficiency

158
Q

Prevention of disease in humoral immune defects

A

Proph AB

AB replacement = immunoglobulins from blood

159
Q

Splenectomy infection prevention

A

Vaccination - before splenectomy better

Prop AB = pen V

160
Q

Causes of primary cell mediated immune defects

A

Combined
Di-George
Hyper IgM

161
Q

Causes of secondary cell mediated immune defects

A 
HIV
Malignancy
Anti-rejection therapy
Steroids
Anti-TNF
Stem cell transplant
Radiotherapy
Malnutrition
162
Q

Impact of cell mediated immune defects

A 
HIV targets CD4 cells = T helper response poor
Mycobacteria, salmonella, listeria
Cryptococcus, PCP, histoplasma
CMV
Toxoplasma
163
Q

Prevention of disease in cell mediated immune defects

A

Aciclovir for viral
Cotrimoxazole for fungal and latent TB
Immunisations
Avoid live vaccines!

164
Q

When to suspect primary immunodeficiency

A
  • infectiosn requiring IV AB
  • FH of it
  • 4 or more new ear infections in 1 year
  • 2 or more new sinus infections in 1 year
  • 2 or more months on at least 2 AB
  • 2 or more pneumonias within 3 yrs
  • frequent deep skin abscesses
  • thrush/skin fungal infection >6m
  • 2 or > deep infections within 3 yrs
165
Q

Chronic persistent infection define

A

Infection maintained by continuous replication of a virus

166
Q

Chronic latent infection define

A

Persistent or life long infection maintained by pool of latently infected cells (non replicating)

167
Q

Viral infections in cell mediated immune deficiency

A 
Herpes = Simplex, Varicella zoster, CMV, EBV
Paramyxoviruses = RSV, measles, parainfluenza
Papillomarviruses = HPV
Polyomaviruses = BK, JK
168
Q

HSV

A

Herpes Simplex Virus

  • nearly always HSV-1
  • genital herpes HSV-2
  • direct contact spread with lesions
  • swab lesion PCR
  • Tx = aciclovir
169
Q

HSV Primary infection

A

asymptomatic

Maybe = fever, pharyngitis, ulceration and lymphadenopathy

170
Q

HSV Secondary recurrence

A

Very common
Prodromal tingling
Localised painful blisters
Resolve 5-7 days

171
Q

Varicella Zoster

A

Most have had exposure

Diagnosis = swab area

172
Q

Varicella Zoster Primary Infection

A 
Chickenpox
Fever + generalised rash
Sporadic
Spring-summer peak
Highly infectious via resp droplets and lesion shedding
- most infectious 1-2 days before rash
173
Q

Varicella Zoster Reactivation

A 
Shingles
In immunosuppressed
May be multidermatomal
Encephalitis
Infectious until all lesions crusted
Uncomplicated in healthy
Severe with pneumonitis otherwise
174
Q

Treatment of HSV and VZV

A

Treat immunocompromised always
Antivirals = reduce symp, duration, shedding, complications
If VZV have pneumonitis, encephalitis, eye disease = IV treatment

175
Q

ACE Viruses

A

Adenovirus, CMV, EBV

  • major hazard for allogeneic HSCT
  • primary infection and reactivation
  • transmitted from donor
176
Q

CMV

A
  • virus in saliva, urine, breast milk
  • common in childhood
  • usually minimal symptoms and self limiting
  • mononucleosis like
  • remains latent in blood and bone marrow cells and can reactivate when suppressed
  • important causes of congenital abnormalities
  • pathogen of solid organ and bone marrow transplant patients
177
Q

CMV diagnosis

A

Pre transplant serology

Post transplant monitoring for viraemia/infection

178
Q

CMV infection vs. disease

A 
Infection
- lab evidence of virus activity
- primary and re-infection
Disease
- clinical evidence of disease
- syndrome
- organ specific
179
Q

CMV management

A

PROPH - anti-viral after transplant regardless
Pre-emptive = start antiviral therapy at first indication of active CMV replication after transplant (if bone marrow and SCT)

180
Q

EBV

A
  • herpes virus establishes latency
  • most of pop have past infection
  • in immunosuppressed = lymphoma (transplant and HIV patients) and post transplant lymphoproliferative disorder (first year)
181
Q

Risk Factors of PTLD

A 
<5 yrs
Antirejection Therapy
Tacrolimus >ciclosporin
CMV seromismatch
Small bowel/heart/lung/liver/renal/BM/pancreas transplants
EBV seronegative prior to transplant
182
Q

PTLD Symptoms

A 
Fever unexplained
GI upset
Lymphadenopathy
Tonsillar hypertrophy
IM
Hepatic/splenic enlargement
Anaemia
Graft dysfunction
183
Q

Adenovirus

A

3 main syndrome - resp (mild, self limiting), keratoconjunctivitis, gastroenteritis

  • immunosuppressed
  • transplant (RF = child, severe GvH disease, cord blood transplant, liver, heart)
184
Q

Adenovirus in immunosuppressed

A

Asymptomatic viral shedding/ end organ disease/ disseminated more than 2 sites

185
Q

Management of adenovirus in immunosuppressed

A

Screening via blood = urine PCR

Reduction of immune suppressed

186
Q

Polyomarviruses

A

JC and BK
Early life acquired
Initial viraemia = kidney seeding = latency
Reactivation due to viruria = viraemia = end organ disease
Asymptomatic

187
Q

3 syndromes of polomarviruses

A

BK virus associated haemorrhagic cystitis
BK virus associated nephropathy
JC-PML

188
Q

Define confidentiality

A

Agreement that gives the confider the right to expect discretion from the confidant as well as guardianship of the information received

189
Q

Define confidential information

A

Info disclosed to the professional about the patient before or after death which has a nature of confidence or seen by the patient as having such a nature
Patient identifiable data

190
Q

When does confidentiality cease to exist

A

Only when no representatives can give consent or refuse disclosure

191
Q

How long should health records be retained for?

A

8 years min.

25 for maternity/obstetric

192
Q

Why do we have duty of confidentialtiy?

A
  • no trust otherwise = no info. = no care = no business
  • unpleasant legal/professional consequences
  • precondition of autonomy
193
Q

When can confidentiality be relaxed?

A
  • patient gives explicit consent to disclose for purposes of epidemiology/research/education, to relatives
  • must be informed of purpose and consequences and whom
  • may refuse
  • if in patient’s best interest = young patient abuse tell guardians
  • in society’s best interest = dangerous drivers, harm to individuals
  • professional has dual responsibility = member of armed forces, prison worker
  • in case of serious crime/assault = disclose to police
  • court of law
  • tax inspectors for financial info
194
Q

What is not a notifiable disease?

A

AIDS

Notifiable to PH Act

195
Q

Statutory Duties

A 
Abortions
Drug Addicts
Births
Deaths
Certain infections
196
Q

Fitness to drive

A
  • depends on vehicle
  • up to age of 70 years then renews every 3 years, automatically done unless driver reports problem
  • must inform DVLA of potential medical disability
197
Q

Doctor concerns with patient informing DVLA

A
  • inform patient they have legal duty to
  • if refuse = suggest second opinion of diagnosis and risks and advice not to drive until obtained
  • if continue to drive = persuade not to, involve next of kin
  • if still drives = tell them that DVLA will be informed
  • discuss matter with patient’s defence organisation
  • give medical info to medical adviser at DVLA
  • write to patient informing you have done
198
Q

Prescribed drugs affecting fitness to drive

A 
Transquillisers
Antiepileptics
Antidepressants
Antipyschotics
Antihistamines
Analgesics
Anaesthetics
Opoiates, cannabis, LSD, alcohol
199
Q

When is it justified to withhold truth from patient?

A
  • if will cause real predictable harm =make them suicidal

- if patient states informed preference not to be told the truth

200
Q

Helsinki Principles

A

1 - must conform to accepeted scientific principles and based on adequate lab and animal experiments
2 - Independent committee which conform with local laws should be able to guide/comment
3 - conducted by scientifically qualified people and under supervision of clinically competent medical people
4 - importance of research objective must be in proportion to inherent subject risk
5 - Assessment of risk and benefits must be done & concern for interests of subject must outweight interest of science and society
6 - respect subject’s autonomy and privacy, minimise harm
7 - docotrs cease investigation if hazards outweight risks
8 - physician obliged to preserve accuracy, reports should concord with principles laid down otherwise not published
9 - subject correctly informed and can withdraw at any time, consent needed in writing
10 - consent to be free from coercion
11 - informed consent obtained from legal guardian in case of incompetence

201
Q

Who should patient sue in what scenario?

A

If GP = direct claim

If hospital doctor = against health authority and doctor

202
Q

Define negligence

A
  • duty of care
  • breach of that duty
  • person claiming suffered harm/damage
  • damage caused by breach
203
Q

Duty of care

A

Good Samaritans - ethical duty not a legal one

204
Q

breach of duty of care

A

Failure to reach level of proficiency of peers

205
Q

Bolitho Modification

A
  • ignorance is not a defence
  • genuine errors of clinical judgement not negligence if based on reasonable skill
  • breach done (commission) or not done (ommission - failure to attend to a patient)
  • misdiagnosis if history taking was poor, investigation poor, compared to reasonable doctor, not referred to specialist if doubt
206
Q

Actionable Harm or Damage

A

Disability
Injury
Loss of earnings
Reduced quality of life

207
Q

Legal Process

A

Letter of claim - must be acknowledged within 14 days and copies of requested notes within 40d

  • letter of response within 3m
  • claim form by civil court
  • denial by defendant
  • proofing
  • assessment of quantum, settlement reached by trust leaving doctors to feel that justice was not done
  • 5% go to court, decision based on expert opinion
208
Q

Examples of neonatal single gene disorders

A 
Sickle Cell
Cystic fibrosis
Hypothyroidism
Phenylketonuria
Medium chain Acyl CoA dehydrogenase deficiency
Maple Syrup Urine Diseasse
Isovaleric Acidaemia
Glutaric aciduria type 1
Homocystinuria
209
Q

Define single gene disease

A

Genetic disorder caused by a change affecting only 1 gene

210
Q

Inheritance pattern of single gene diseases

A 
Simple Mendelian patterns
Auto dominant, recessive etc.
X linked recessive
X linked dominant
Y linked dominant
Imprinting
Mitochondrial
211
Q

Define epigenetics

A

Study of changes in organisms caused by modification of gene expression rather than alteration of genetic code itself

212
Q

Type of DNA mutations

A

Missense
Nonsense (stop codons)
Fram Shift

213
Q

Transcriptional control

A

Mutations in regulatory elements

Epigenetics = DNA methylation/chromatin modification, deletions, insertions, inversions, translocations

214
Q

X linked recessive disorders

A

Haemophilia A
X linked severe combined immune disorder
Duchenne muscular dystrophy
Becker muscular dystrophy

215
Q

X linked recessive inheritance mechanism

A

Males born to carrier mothers
1/2 sons affected
Daughters of affected fathers are carriers
Never father to son

216
Q

Duchenne muscular dystrophy

A

Dystrophin mutation
Dystrophin - stabilises sarcolemma, protects muscle fibres from LT damage, links microfilament network of actin to proteins
Stem cells regenerate muscle to some extent but eventually die and get replaced by fat and CT

217
Q

DMD vs Backer Muscular Dystrophy

A

DMD = dystrophin gene absent or non functional vs. partially functional in Becker
DMD greater incidence
Becker later onset 12 yrs vs. 3-5 yrs in DMD
Longer LE in Becker = 40s vs. 20s in DMD
Cardiomyopathy may present before skeletal symptoms in Becker vs. after in DMD

218
Q

X linked dominant disorders

A

Some forms of retinitis pigmentosa
Chorndrodysplasia Punctata (cartilage and bone dev.)
Hypophosphatemic rickets

219
Q

Mechanism of X linked dominant inheritance

A

Both sexes
More females
Affected sons have affeted mother
All daughters from affected father are affected

220
Q

Autosomal recessive diseases

A

Pheylketonuria
Tay-Sachs
Hemochromatosis
Cystic Fibrosis

221
Q

Autosomal Dominant Diseases

A

Huntingtons
Achondroplasia
Polycystic Kidney Disease
Werner mesomelic syndrome

222
Q

Werner mesomelic syndrome

A
  • hypo or aplasia of the tibiae
  • preaxial polydactyly of hands and feet
  • five fingered hand with absence of thumbs
  • overexpression of SHH
223
Q

Y linked dominant diseases

A

Retinitis pigmentosa

224
Q

Y linked dominant inheritance mechanism

A

Only males

From father to all sons

225
Q

Examples of imprinting conditions

A

Beckwith-Wiedemann syndrome
Prader-Willi
Angelman syndrome

226
Q

Imprinting inheritance mechanism

A

Certain genes expressed in a parent of origin specific manner
If allele inherited from father is imprinted it is silenced and only the allele from the mother is expressed

227
Q

Angelman syndrome

A

Neuro-genetic
Severe intellectual and developmental disability
Sleep disturbance
Seizures
Both sexes - only when mutated chromosome is maternally inherited
If mother is carrier = chance is 50%

228
Q

Examples of mitochondrial conditions

A

Mitochondral myopathy
DM and deafness
Leber’s hereditary optic neuropathy
Leigh syndrome, subacute sclerosing encephalopathy

229
Q

What can gene therapy help with?

A

Recessive disorders
Loss of function mutations
Small gene
Tissue is accessible = skin/blood

230
Q

Treatment options

A

Gene therapy
Genome Engineering = CRISPR-Cas9 technique
Gene repair of double strand breaks

231
Q

Complex disease = multi gene diseases

A
  • common
  • polygenic
  • high frequencies of variants in pop
  • variants have small affects on gene function
  • unclear modes of inheritance
232
Q

How to determine if genes are important?

A

Familial aggregation
Twin studies
Adoption studies

233
Q

Examples of complex diseases

A

Essential HTN
CHD
Diabetes
Asthma, epilepsy, cancer

234
Q

Genetic causes of HTN

A
  • rare inherited syndromes of HTN
  • majority of cases = polygenic complex
  • 30-50% genes, 50-70% lifestyle factors
235
Q

miR-425

A

Present in atria and ventricles

  • expressed at levels similar to miR-208a which is known to have significant biological functions in the heart
  • inhibiting it could enhance atrial ANP release in response to salt excess and HF

CSP1 (20 decks)

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