Respiratory - Immune Deficiencies 1 - Week 4 Flashcards Preview

Immunology > Respiratory - Immune Deficiencies 1 - Week 4 > Flashcards

Flashcards in Respiratory - Immune Deficiencies 1 - Week 4 Deck (40):
1

Which acronym is used to prompt consideration of immune deficiencies?

SPUR

2

What does SPUR stand for?

Serious infections, Persistent infections, Unusual infections, Recurrent infections

3

What is meant by a 'serious' infection?

Not responsive to antibiotics

4

What is meant by a persistent infection?

Early structural damage or chronic infections

5

How is 'recurrent infection' classified?

2 major or one major and many minor infections in one year

6

How do chronic infections develop?

Degranulation of neutrophils releases destructive enzymes which create the niche for chronic infection to develop

7

What is the single greatest indicator of immune deficiency?

Family history of immune deficiency - they're usually genetically encoded

8

Give 3 symptoms (outwith SPUR) that may suggest immunodeficiency.

Failure to thrive, chronic diarrhoea, weight loss

9

What is meant by secondary immunodeficiency?

Another condition/factor causes the immunodeficiency rather than the body itself

10

Give 5 examples of conditions which may cause secondary immunodeficiency?

Extremes of age, infection (such as HIV), treatments (eg. immunosuppressants), malignancy, nutritional disorders

11

What is the role of TNF-alpha in the innate immune response?

Triggers vasodilation & production of heat.

12

Which of the leukocytes are phagocytes?

Macrophage & Neutrophil

13

What are the typical clinical features of phagocyte deficiency?

Infection at unusual sites, common & unusual bacteria, typical & atypical mycobacterium, fungal infections

14

State 2 reasons which can cause Neutrophils to not be produced or not be produced properly.

Failure to differentiate along the myeloid lineage or failure of naive neutrophils to mature

15

Give a primary defect which would cause failure of a cell to differentiate along the myeloid lineage.

Reticular dysgenesis

16

What is reticular dysgenesis?

Most severe form of Inborn Severe Combined Immunodeficiency (SCID) - no neutrophils & few lymphocytes --> Fatal septicaemia within days of birth

17

Give a secondary defect which would cause failure of a cell to differentiate along the myeloid lineage.

After stem cell transplant

18

Give 2 examples of conditions caused by failure of neutrophils to be able to mature.

Kostmann Syndrome, Cyclic neutropaenia

19

What is the mechanism of reticular dysgenesis?

Stem cell cannot differentiate into common myeloid progenitor or common lymphoid progenitor

20

What is the mechanism of Kostmann Syndrome?

Granulocyte-Monocyte progenitor cannot differentiate into a neutrophil as there is a defect in G-CSF cytokine or receptor

21

State 4 clinical presentations of Kostmann Syndrome.

Severe chronic neutropenia, recurrent bacterial infections usually 2 weeks after birth, fever, irritability, failure to thrive

22

State 2 supportive treatments of Kostmann Syndrome.

Prophylactic antibiotics & prophylactic anti-fungals

23

State 2 definitive treatments of Kostmann Syndrome.

Stem cell transplantation (if problem in Neutrophil pre-cursor), G-CSF (if defect in cytokine)

24

If a patient's phagocytes were unable to bind to adhesion molecules, what clinical signs would be seen?

High neutrophil numbers in blood, no pus formation in tissue infections, recurrent bacterial & fungal infections

25

Failure to recognise activation markers expressed on endothelial cells is called...

Leukocyte Adhesion Deficiency

26

Leukocyte adhesion deficiency is caused by...

A defect in the CD-18 leukocyte integrin

27

What kind of receptors do phagocytes express?

Fc

28

What is the role of Fc receptors in phagocytes?

Allows any antibody-antigen complex to attach

29

Which receptor on phagocytes binds to complement fragments bound to an antigen?

Complement receptor 1 (CRI)

30

State 2 potential reasons behind defective phagocytosis.

Defect in opsonin receptors (Fc or CRI) or defect in complement or antibody production

31

Failure of oxidative killing mechanisms is called...

Chronic Granulomatous Disease

32

Chronic Granulomatous Disease results in...

inability of phagocytes to kill by oxidative killing as they cannot produce free radicals

33

State 5 clinical features of Chronic Granulomatous Disease.

Recurrent deep bacterial infections, recurrent fungal infection, failure to thrive, Lymphadenopathy and hepatosplenomegaly (enlarged spleen & liver), granuloma formation

34

Which test is used to test for Chronic Granulomatous Disease?

NBT test

35

What does the NBT test test for?

Whether neutrophils can kill via production of oxidative free radicals

36

Give 2 examples of supportive treatments for Chronic Granulomatous Disease.

Prophylactic antibiotics & anti-fungals

37

Give 2 examples of definitive treatments for Chronic Granulomatous Disease.

Stem cell transplantation or Gene therapy

38

Explain the immunological pathway involved in TB.

Infected macrophage produces IL-12 which stimulates Th1 cells to produce interferon gamma which acts on neutrophils & macrophages to produce TNF-alpha. This triggers oxidative killing mechanisms.

39

IFNg receptor deficiency, IL-12 deficiency and
IL-12 receptor deficiency can all cause...

Increased susceptibility to mycobacterial infections

40

Which tests may be involved with diagnosing phagocyte function deficiency?

Full blood count, presence of pus, Expression of neutrophil adhesion markers, Chemotactic assays, Phagocytosis assays, NBT test