Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

2.3 T1: Immune system > T1 L21: Immunodeficiency diseases > Flashcards

T1 L21: Immunodeficiency diseases Flashcards

1
Q

What is primary immunodeficiency syndrome (PID)?

A

Immune defect that is intrinsic to the immune system

Often genetic but not always

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

What is secondary immunodeficiency?

A

Immune defect secondary to another disease process

Very common
Eg. malignancies, metabolic, drugs, infection

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Which cells make up the adaptive immune system?

A

B and T-cell

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

What are combines immunodeficiencies?

A

Those affecting antibody production and T-cells

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

What are the manifestations of immunodeficiency?

A
  • Recurrent infections

- Immune dysregulation Eg. uncontrolled inflammation, autoimmune disease

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

What is immunosenescence?

A

A combination of age-related factors that cause a greater susceptibility to infection and reduced response to vaccination

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

What are some immunological aspects of immunosenescence?

A
  • Thymic involution
  • Telomere shortening in the stem cells reducing quality and quantity of lymphocyte output
  • Reduced T and B-cell receptor diversity
  • Reduced vaccine responses
  • Reduced neutrophil function
  • Reduced self-tolerance
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

What is thymic involution?

A

Shrinking of the thymus with age

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

What factors make the elderly more susceptible to infection?

A
  • Reduced immunity, motility, nutrition, wound healing, physiological reserve
  • Increased co-morbidities
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

What is bronchiectasis?

A

When the walls of the bronchi are thickened from inflammation and infection

It causes irreversible lung damage

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

What causes antibody deficiency?

A
  • Transient hypogammaglobulinemia of infancy
  • Loss of IgG through nephrogenic syndrome or excessive burns
  • Impaired production of IgG due to immunosuppressive drugs
  • X-linked agammaglobulinemia
  • X-linked hyper-IgM-syndrome
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

What is transient hypogammaglobulinemia of infancy?

A

In healthy infants, there is a period at about 6 months old when there is relative antibody deficiency

This is a physiological state but can be correlated with increased infections

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

When do infants with immunodeficiency present?

A

After 3-6 months because up until then they are protected by maternal IgG

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

What is X-linked agammaglobulinemia?A

A

Also known as Bruton’s disease

Caused when there is no signalling via Bruton’s tyrosine kinase (btk) which is required for transduction at pro-B stage of B-cell maturation . It causes maturation arrest

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

What is maturation arrest?

A

When there is no heavy chain rearrangement, no B-cells leave the bone marrow, and there is no immunoglobulin production

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

What is X-linked hyper-IgM-syndrome?

A

A CD40L deficiency that causes failure of B-cell maturation from primary to secondary. It results in low IgA and IgG but raised or normal IgM

This results in recurrent bacterial infections at about 3-6months old

17
Q

What is cellular immunodeficiency?

A

Affects CD4 T-cells

Manifests with opportunistic, viral, fungal, and mycobacterial infections

The secondary cause of this is HIV infection

18
Q

What is severe combined immunodeficiency (SCID)?

A

Absent T-cell and B-cells may be present but they’re non functional

19
Q

What are the causes OF severe combined immunodeficiency (SCID)?

A
  • Common gamma chain deficiency
  • JAK3 deficiency
  • RAG1/2 deficiency
20
Q

What are the characteristics of common gamma-chain deficiency as a cause of SCID?

A

It’s X-linked SCID. The function of the gamma chain is to be part of a membrane receptor for several cytokines, some of which are needed for T-cell maturation

There are no T-cell and the B-cell that are there are non-functional

21
Q

What are the characteristics of JAK3 deficiency as a cause of SCID?

A

It’s an autosomal recessive SCID. JAK3 is used downstream of the common gamma chain so deficiency prevents signalling

There are no T-cell and the B-cell that are there are non-functional

22
Q

What are the characteristics of RAG1/2 deficiency as a cause of SCID?

A

It’s an autosomal recessive SCID . RAG1/2 are needed for somatic recombination between V(D)J segments

No RAG1/2 means there are no T or B-cell receptors

23
Q

How is SCID treated?

A

Stem cells are harvested from HLA-matched donors and given to the patient into the bone marrow

This results in reconstitution of T and B-cells

24
Q

What is DiGeorge syndrome?

A

A failure migration of the 3rd/4th brachial arches

Most patients have micro-deletions in chromosome 22 resulting in absent parathyroids, cleft palate, congenital heart defects, thymic aplasia

Presentation: immunodeficiency, autoimmunity

25
Q

What is terminal complement deficiency?

A

A deficiency of terminal complement components C5-C9 leading to specific susceptibility to Neisseria species

It can be diagnosed by functional complement assays

2.3 T1: Immune system (21 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions