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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
Q

What type of deficiency is commonly associated with upper respiratory tract infections?

A

Antibody deficiency

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26
Q

What infections are commonly associated with T cell defects?

A

All viruses, lots of bacteria, candida, aspergillus, lots of Protozoa

27
Q

What are some important points regarding T cell defects?

A

This will not be missed because it will start at a very early age. Death is not treated

28
Q

What is the presentation of patients with chronic granuloma to us disease (CGD)?

A

Pulmonary aspergillosis and skin infections

29
Q

How is pulmonary aspergillosis seen?

A

Halo sign in HRCT scan

30
Q

What is chronic granulomatous disease?

A

It is an inherited PID which increases the body’s susceptibility to infections caused by certain bacteria and fungi

31
Q

What is a granuloma?

A

Masses of immune cels that form at sites of infection or inflammation

32
Q

What type of PID is CGD?

A

Phagocyte defect

33
Q

What are the different types of management of PID?

A

Supportive, specific and comorbidities

34
Q

What supportive treatment is used in management of PIDs?

A

Infection prevention, treat infections promptly and aggressively, nutrient support, avoid line attenuated vaccine in patient with sever PIDs.

35
Q

What sort of specific treatment is used in the management of PIDs?

A

Regular immunoglobulin therapy (IVIG or SCIG). IN SCID - use hematopoitic stem cell therapy

36
Q

How are comorbidities essential in PIDs?

A

Autoimmunity and malignancies, organ damage and avoid non-essential exposure to radiation

37
Q

What is the goal of immunoglobulin replacement therapy?

A

Serum IgG>8g/L

Life long treatment

38
Q

For what conditions is immunoglobulin replacement therapy used?

A

CVID, XLA and hyper IgM syndrome

39
Q

What does XLA stand for?

A

X-linked agammaglobulinemia

40
Q

What is XLA?

A

A condition that affects the immune system and occurs almost exclusively in males. They have very few B cells.

41
Q

What is another name for XLA?

A

Brutons disease

42
Q

What is the cause of secondary immune deficiencies?

A

Decreased production of immune components

Increased loss or catabolism of immune components

43
Q

Why might you get decreased production of immune components?

A

Malnutrition, infection (HIV), liver diseases, lymphoproliferative diseases or splenectomy

44
Q

Why might liver disease cause secondary immune deficiencies?

A

Cytokines and acute phase proteins are all produced in the liver

45
Q

What is a splenectomy?

A

Surgical removal of the spleen

46
Q

What are the causes of a splenectomy?

A

Infarction (sickle cell anaemia), trauma, autoimmune haemolytic disease, infiltration (tumour), coeliac disease, congenital

47
Q

Why is the spleen so important?

A

Immune function - protects against blood borne pathogens, antibody production and splenic macrophages

48
Q

Role of the splenic macrophages?

A

Removal of opsonised microbes and removal of immune complexes

49
Q

How do asplenic/splenectomised patients present?

A

Inc susceptibility to encapsulated bacteria and OPSI

50
Q

What is OPSI?

A

Overwhelming post-splenectomy infection.

51
Q

Give examples of encapsulated bacteria?

A

Haemophilus influenzae, streptococcus pneumoniae and neisseria meningitids

52
Q

What is the management for people who are splenic/spelnectomised?

A

Penicillin prophylaxis (life long), immunisation against encapsulated bacteria, medic alert bracelet

53
Q

Which people are at an increased risk of getting haematological malignancies?

A

Chemotherapy induced neutropenia, chemotherapy induced damage to mucosal barriers and vascular catheter

54
Q

How would you treat a person with haematological malignancy?

A

Treat suspected neutropenic sepsis as an acute medical emergency and offer empiric antibiotic therapy immediately. Assess patient’s risk of septic complications

55
Q

Why might you get increased loss or catabolism of immune components?

A

Protein-losing conditions eg neuropathy and enteropathy

Burns

56
Q

What is the important thing to remember when recognised and diagnosing an immunodeficiency disease?

A

The pattern and type of infections always reflect the nature of immunological defect

57
Q

What laboratory test would you do when looking for secondary immunodeficiency?

A

Full blood count and differential

58
Q

What tests would you do for humoral (antibody) immunity?

A

IgG,IgA,IgM,IgE
IgG levels to specific previous vaccines
Measure antibody in response to “test” immunisation

59
Q

What tests would you do for cell mediated immunity?

A

Lymphocyte count )FBC)
Lymphocyte subset analysis (CD4+ etc)
In vitro test for IV function

60
Q

What are the test for phagocytic cells?

A 
Neutrophil count (FBC)
Neutrophil function tests 
Adhesion molecule expression
61
Q

How can you test neutrophil function?

A

Oxidative burst for CGD

62
Q

How can you test for complement?

A

Individual components, tests for complement function (CH50/AP50)

63
Q

What definitive test would you do for immunodeficiency?

A

Molecular testing and gene mutations

Infection A (18 decks)

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