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Infection A > Session 10 > Flashcards

Session 10 Flashcards

1
Q

What does PID stand for?

A

Primary immunodeficiency disease

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2
Q

What is a PID?

A

Group of more than 300 rare, chronic disorders in which part of the body’s immune system is missing or functions improperly

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3
Q

Define an “immunocompromised” host

A

State in which the immune system is unable to respond appropriately and effectively to infectious microorganisms

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4
Q

An is a immunodeficiency due to?

A

A defect in one or more components of the immune system

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5
Q

A defect in which part of the immune system is the most damaging?

A

Defect in B cells of T cells

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6
Q

Why is a host “immunocompromised”?

A

Primary immunodeficiency is congenital

Secondary immune deficiency is acquired

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7
Q

What causes primary immunodeficiency?

A

Due to intrinsic gene defect - missing protein, missing cell or non functional components

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8
Q

What causes secondary immunodeficiency?

A

Due to underlying disease/treatment - dec production/function of immune components or inc in catabolism of immune components

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9
Q

When do you suspect an immunodeficiency?

A

Infections suggesting underlying immune deficit defined as SPUR

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10
Q

What does SPUR stand for?

A

Severe
Persistent
Unusual
Recurrent

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11
Q

Give some examples of warning signs of PID for children

A

Family history of PID, two or more pneumonia in 1 year, recurrent deep skin or organ abscess, persistent thrush, need for IV antibiotics to clear infections

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12
Q

What are some warning signs of PID for adults?

A

Family history of PID, chronic diarrhoea with weight loss, recurrent viral infections, thrush

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13
Q

What are the limitations of the “10 warning signs” used to detect a PID?

A
  • Lack of population based evidence
  • PID patients with different defects/presentations
  • PID patients with non-infectious manifestation
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14
Q

What does immunodeficiency make you more susceptible to?

A

Infection and cancer

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15
Q

What are most important PIDs?

A

Selective IgA deficiency, IgG subclass deficiency, SCID and phagocytic defects

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16
Q

What is SCID?

A

Sever combined immunodeficiency - combined T and B cell

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17
Q

What will the age of symptom onset tell you about the immunodeficiency?

A

It will tell you what type of PID the patient is suffering from

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18
Q

If the onset of symptoms is <6 months what does this tell you about the immunodeficiency?

A

Suggests T cell or phagocyte defect

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19
Q

What does the age of onset of >6month and <5 years tell you about the immunodeficiency?

A

Suggests a B-cell/antibody or phagocyte defect

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20
Q

What does the age of symptom onset of >5 years tell you about the immunodefincy?

A

Cell/antibody/complement or secondary immunodeficiency

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21
Q

What bacterial infections might lead to complement deficiency?

A

Neisseria, streptococci, encapsulated bacteria

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22
Q

What infections cause a defiency in which complement proteins?

A

Pyogenic infection - C3
Meningitis/Sepsis/Arthiritis - C5-C9
Angiooedema - C1

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23
Q

What infections are commonly associated with phagocytic defects?

A

Staph aureus, candida, aspergillus (skin.mucous infections, deep seating infections or invasive fungal infections)

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24
Q

What infections are commonly associated with antibody deficiency?

A

Strap, staph, enterovirus, Protozoa.

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25
What type of deficiency is commonly associated with upper respiratory tract infections?
Antibody deficiency
26
What infections are commonly associated with T cell defects?
All viruses, lots of bacteria, candida, aspergillus, lots of Protozoa
27
What are some important points regarding T cell defects?
This will not be missed because it will start at a very early age. Death is not treated
28
What is the presentation of patients with chronic granuloma to us disease (CGD)?
Pulmonary aspergillosis and skin infections
29
How is pulmonary aspergillosis seen?
Halo sign in HRCT scan
30
What is chronic granulomatous disease?
It is an inherited PID which increases the body's susceptibility to infections caused by certain bacteria and fungi
31
What is a granuloma?
Masses of immune cels that form at sites of infection or inflammation
32
What type of PID is CGD?
Phagocyte defect
33
What are the different types of management of PID?
Supportive, specific and comorbidities
34
What supportive treatment is used in management of PIDs?
Infection prevention, treat infections promptly and aggressively, nutrient support, avoid line attenuated vaccine in patient with sever PIDs.
35
What sort of specific treatment is used in the management of PIDs?
Regular immunoglobulin therapy (IVIG or SCIG). IN SCID - use hematopoitic stem cell therapy
36
How are comorbidities essential in PIDs?
Autoimmunity and malignancies, organ damage and avoid non-essential exposure to radiation
37
What is the goal of immunoglobulin replacement therapy?
Serum IgG>8g/L | Life long treatment
38
For what conditions is immunoglobulin replacement therapy used?
CVID, XLA and hyper IgM syndrome
39
What does XLA stand for?
X-linked agammaglobulinemia
40
What is XLA?
A condition that affects the immune system and occurs almost exclusively in males. They have very few B cells.
41
What is another name for XLA?
Brutons disease
42
What is the cause of secondary immune deficiencies?
Decreased production of immune components | Increased loss or catabolism of immune components
43
Why might you get decreased production of immune components?
Malnutrition, infection (HIV), liver diseases, lymphoproliferative diseases or splenectomy
44
Why might liver disease cause secondary immune deficiencies?
Cytokines and acute phase proteins are all produced in the liver
45
What is a splenectomy?
Surgical removal of the spleen
46
What are the causes of a splenectomy?
Infarction (sickle cell anaemia), trauma, autoimmune haemolytic disease, infiltration (tumour), coeliac disease, congenital
47
Why is the spleen so important?
Immune function - protects against blood borne pathogens, antibody production and splenic macrophages
48
Role of the splenic macrophages?
Removal of opsonised microbes and removal of immune complexes
49
How do asplenic/splenectomised patients present?
Inc susceptibility to encapsulated bacteria and OPSI
50
What is OPSI?
Overwhelming post-splenectomy infection.
51
Give examples of encapsulated bacteria?
Haemophilus influenzae, streptococcus pneumoniae and neisseria meningitids
52
What is the management for people who are splenic/spelnectomised?
Penicillin prophylaxis (life long), immunisation against encapsulated bacteria, medic alert bracelet
53
Which people are at an increased risk of getting haematological malignancies?
Chemotherapy induced neutropenia, chemotherapy induced damage to mucosal barriers and vascular catheter
54
How would you treat a person with haematological malignancy?
Treat suspected neutropenic sepsis as an acute medical emergency and offer empiric antibiotic therapy immediately. Assess patient's risk of septic complications
55
Why might you get increased loss or catabolism of immune components?
Protein-losing conditions eg neuropathy and enteropathy | Burns
56
What is the important thing to remember when recognised and diagnosing an immunodeficiency disease?
The pattern and type of infections always reflect the nature of immunological defect
57
What laboratory test would you do when looking for secondary immunodeficiency?
Full blood count and differential
58
What tests would you do for humoral (antibody) immunity?
IgG,IgA,IgM,IgE IgG levels to specific previous vaccines Measure antibody in response to "test" immunisation
59
What tests would you do for cell mediated immunity?
Lymphocyte count )FBC) Lymphocyte subset analysis (CD4+ etc) In vitro test for IV function
60
What are the test for phagocytic cells?
``` Neutrophil count (FBC) Neutrophil function tests Adhesion molecule expression ```
61
How can you test neutrophil function?
Oxidative burst for CGD
62
How can you test for complement?
Individual components, tests for complement function (CH50/AP50)
63
What definitive test would you do for immunodeficiency?
Molecular testing and gene mutations

Infection A (18 decks)

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