Immunodeficiency Flashcards

1
Q

What are the clinical features associated with immunodeficiency?

A
Chronic infection
Recurrent infections (more than expected)
Unusual microbial agents
Incomplete clearing of infection
Incomplete response to treatment
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2
Q

What are the types of immunodeficiency?

A

Primary

Secondary

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3
Q

Describe the features of primary immunodeficiency

A

Mostly genetically determined (from birth)

Involves intrinsic defects in
lymphocytes
complement components
or phagocytic cells

Specific (abnormalities of B or T cells) or non-specific (deficiency in innate components)

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4
Q

The type of infection can indicate which part of the immune system is immune deficient.

What deficiencies are Pyogenic infections linked to?

A

Deficiencies in:
Phagocytosis
Complement
Antibody

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5
Q

The type of infection can indicate which part of the immune system is immune deficient.

What deficiencies are opportunistic infections linked to?

A

Defects in cell-mediated immunity

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6
Q

Give examples of pyrogenic infections

A

Streptococcus pneumoniae
Haemophilus influenzae
Staphylococcus aureus

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7
Q

What symptoms are pyrogenic infections linked to?

A

Pneumonia, sinusitis, otitis media

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8
Q

Give examples of opportunistic infections

A

Gut parasite- Giardia lamblia
Candida albicans
Chickenpox

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9
Q

What symptoms are opportunistic infections linked to?

A

Diarrhoaea, common yeast & viral infections

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10
Q

What is the most common inheritance pattern of congenital primary immunodeficiencies?

A

X-linked

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11
Q

Give examples of X-linked congenital primary immunodeficiencies

A
Chronic Granulomatous Disease (CGD)
Wiskott-Aldrich Syndrome (WAS) 
Severe Combined Immunodeficiency (SCID)
X-Linked Agammaglobinaemia (X-LA)
X-Linked Immunodeficiency with High IgM (HIgM)
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12
Q

What can cause congenital primary immunodeficiencies?

A

The cause of a primary deficiency may vary

Mutations – rare, affect any part of immune system, cause severe disease

Polymorphisms – very common, moderate increased risk of infection

Polygenic disorders- relatively common, affect mainly antibodies, usually cause severe disease

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13
Q

Describe the features of polymorphisms for congenital primary immunodeficiencies

A

Multiple alleles of the same gene occurring at a single locus -in at least 1% of population

Polymorphisms in promoter for TNF – susceptibility to cerebral malaria, septic shock

Chemokines & receptors – susceptibility to HIV

Mannan binding lectin & complement components – susceptibility to fungal and bacterial infections

TLR polymorphisms – susceptibility to various infections

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14
Q

Describe the features of polygenic disorders for congenital primary immunodeficiencies

A

Involves interaction of several genes + environmental factors

CVID, 1 in 20,000 people – mild to severe

IgA, IgG subclass deficiencies - moderate

IgA deficiency, 1 in 700 people - mild

IgG2 deficiency – moderate (pneumococcal, haemophilus infections)

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