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Infection > Immunodeficiencies > Flashcards

Immunodeficiencies Flashcards

0
Q

How can you recognise patients who are immunocompromised?

A

S - severe ie life threatening
P - persistent
U - unusual - opportunist pathogens
R - recurrent

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1
Q

Define immunocompromised

A

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

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2
Q

Features of a primary immunodeficiency?

A

Caused by an intrinsic (immune defect)
Congenital or acquired
Relatively rare

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3
Q

Features of secondary immunodeficiencies?

A

Relatively common
Secondary to another condition
Acquired

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4
Q

What can a secondary immunodeficiency be caused by?

A 
Infection (HIV)
Malnutrition
Liver disease
Splenectomy 
Drug-induced neutropenia
Lymphoproliferative diseases
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5
Q

What is neutropenia?

A

Abnormally low neutrophils in the blood, leading to increased susceptibility to infection

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6
Q

What can cause neutropenia?

A 
Drugs
Autoimmune neutropenia
Bone marrow infiltration with malignancy
B12/folate/iron deficiency 
Chemo-cytotoxic/immunosuppressant
Chemical agents
Radiotherapy
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7
Q

Management of suspected neutropenic sepsis?

A

Treat as acute medical emergency
Empiric antibiotics immediately
Assess risk of septic complications

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8
Q

What are asplenic patients more susceptible to? Give examples

A

Encapsulated bacteria
H influenzae
Streptococcus pneumoniae
N meningitidis

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9
Q

What are asplenic patients more at risk of?

A

Overwhelming post-splenectomy infection

-sepsis and meningitis

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10
Q

What do asplenic patients have to have?

A

Lifelong penicillin prophylaxis
Immunisation against encapsulated bacteria
Medical alert bracelet

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11
Q

What does the spleen do?

A

Immune function against blood-borne pathogens

Antibody production

  • acute response: IgM
  • long-term protection: IgG

Splenic macrophages

  • remove opsonised microbes
  • remove immune complexes
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12
Q

What can cause loss of catabolism of immune components and the fore secondary immunodeficiency?

A

Protein losing conditions eg nephropathy, enter ovary

Burns

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13
Q

What can primary immunodeficiencies be classified into?

A

B cell (50%)
Phagocytes (18%)
T cell (30%)
Complement (2%)

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14
Q

When are primary deficiencies normally seen?

A

First few months of life

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15
Q

What are the B cell deficiency types?

A

Common variable immunodeficiency (CVID)
IgA deficiency
Bruton’s disease (X-linked agammaglobulinaemia)

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16
Q

What is common variable immunodeficiency?

A

Inability of B cels to mature into plasma cells.

Low IgG

17
Q

How does IgA deficiency happen?

A

B cells are unable to switch to IgA

18
Q

What is Bruton’s disease?

A

Impaired B cell development

Present in first 6 months

19
Q

What are people with Bruton’s disease at increased risk of?

A

Streptococcus pneumoniae
Mycoplasma amphoriforme
Non-capsulated Haemophilus influenzae

20
Q

What is hyper IgM syndrome?

A

CD40 ligand on activated T cells prevents them from switching from IgG to IgM
Therefore keep making IgM

21
Q

Presentation and management of patients with primary antibody (B cell?) deficiencies?

A

Recurrent upper and lower respiratory bacteria infections causing bronchiectasis

GI complications including infections

Anthropathies (joints - including Mycoplasma spp and Ureaplasma spp)

Increased incidence of autoimmune disease

Increased incidence of lymphoma and gastric carcinoma

22
Q

Management of primary antibody deficiencies?

A

Prompt/prophylactic antibiotics
Immunoglobulin replacement therapy (gold standard)
Management of respiratory function - physiotherapist
Avoid unnecessary exposure to radiation eg CT scan

23
Q

Goal of immunoglobulin replacement therapy?

A

Serum IgG > 8g/L

Life long

24
Q

What is immunoglobulin replacement therapy given for?

A

CIVD
XLA (Bruton’s)
Hyper-IgM syndrome

25
Q

What are some clinically important phagocyte deficiencies?

A

Cyclic neutropenia - cause unknown
Leukocyte Adhesion deficiency
Chronic granulomatous disease
CH syndrome

26
Q

What is leukocyte adhesion deficiency?

A

Lack of CD18 protein on phagocytes - defects adhesion to endothelium

27
Q

What is chronic granulomatous disease?

A

Lack of respiratory burst

28
Q

What is CH syndrome?

A

Failure of phagolysosome formation

29
Q

Presentation of phagocyte deficiencies?

A

Prolonged and recurrent infection

  • ulcers
  • osteomyelitis
  • deep abscesses
  • commonly staphylococcal (catalase positive)
  • invasive aspergillosis
  • inflammatory problems
30
Q

Management of phagocyte deficiencies?

A

Prophylactic antibiotics and antifungals
Immunisation
Surgical management
Stem cell transplantation

31
Q

Clinically important T cell deficiencies?

A

DiGeorge syndrome

Severe combined immunodeficiency

32
Q

What is DiGeorge syndrome caused by?

A

Defect in thymus embryogenesis and incomplete development

33
Q

Signs and symptoms of DiGeorge syndrome?

A 
Cardiac abnormalities
Abnormal face (low ears, wide eyes)
Thymic hypoplasia
Cleft palate
Hypocalcaemia

22 - chromosome abnormality

34
Q

Management of DiGeorge syndrome?

A

Neonatal cardiac surgery
Correct hypocalcaemia
Low T cell number
Bone marrow transplant

35
Q

Presentation of SCID?

A 
Failure to thrive
Long term antibiotic therapy
Deep skin and organ abscesses
Low lymphocyte count
High susceptibility to fungal and viral infections
36
Q

Management of SCID?

A

Antibiotics and prevention
IV Ig

Long term

  • bone marrow/stem cell transplant
  • gene therapy
37
Q

Where can Aspergillus be found?

A

Soil

AC systems

38
Q

What can Aspergillus do in immunocompromised patients?

A

Breathed into upper respiratory tract
Causes invasive aspergillosis
Starts in lungs and may involve other organs through haematogenous spread

39
Q

Why is Aspergillus difficult to fight?

A

Combination of low neutrophils (caused by immunodeficiency) and toxic metabolites produced by the fungi

40
Q

Symptoms of Aspergillus?

A 
Cough
Fever
Shortness of breath
Pleuritic chest pain
Haemoptysis
Nasal congestion
Pain
41
Q

What is Aspergillus treated with?

A

Amphotericin

Echinocandin derivatives

Infection (14 decks)

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