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Immunodeficiency disorders - resp > immunodeficiency disorders > Flashcards

immunodeficiency disorders Flashcards

1
Q

what does SPUR stand for?

A 
Serious 
Persistent 
Unusual 
Recurrent
[regarding infections]
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2
Q

what are 3 common upper resp tract infections?

A
  • sinusitis
  • otitis media
  • laryngeal angioedema
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3
Q

what are 4 common lower resp tract infections?

A
  • pneumonia
  • ILD
  • malignancies
  • bronchitis/bronchiectasis
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4
Q

what is sinusitis and otitis media associated with?

A

primary antibody deficiency

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5
Q

what 4 things are under the bracket of primary antibody deficiency?

A
  • selective IgA deficiency
  • specific antibody deficiency
  • common variable immunodeficiency
  • x-linked agammaglobulinaemia
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6
Q

what is laryngeal angioedema associated with?

A

complement system disorder

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7
Q

what is a complement system disorder?

A

hereditary angioedema

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8
Q

what is pneumonia associated with?

A
  • primary antibody deficiency
  • complement system disorder
  • congenital phagocytosis disorder
  • combined immunodeficiency
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9
Q

what happens to risk of infection as neutrophil rises?

A

risk of infection decreases

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10
Q

what condition is associated with severe congenital neutropenia?

A

kostmann syndrome

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11
Q

what happens in Kostmann syndrome?

A

= low neutrophils count, therefore there is recurrent bacterial/fungal infections occurring after 2 weeks from birth

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12
Q

what gene is associated with Kostmann?

A

ELANE gene on chromosome 19p13.3

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13
Q

how do you treat Kostmann syndrome?

A

G-CSF gene

growth factor

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14
Q

what is leukocyte adhesion deficiency?

A

high neutrophil count as neutrophils fail to bind to adhesion molecules, then therefore cannot undergo trans-endothelial margination out the cell

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15
Q

what gene is associated with leukocyte adhesion deficiency?

A

CD18 integrin gene

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16
Q

what is chronic granulomatous disease?

A

= the failing of intra-cellular phagocytosis due to lack of NADPH oxidase and as a result granulomas form

17
Q

what gene is associated with chronic granulomatous disease?

A

p47phox gene
also
a defect on IL-12 INFgamma gene

18
Q

describe why a defect in IL-12 INFgamma gene would result in chronic granulomatous disease.

A
  • macrophages produces IL-12
  • IL-12 stimulates T cells
  • T cells activate INF
  • INF produce TNF
  • TNF produce NADPH oxidase

If there is a defect in this, then no/less NADPH oxidase is produced resulting in granuloma formation

19
Q

what bacteria is associated with infections in chronic granulomatous disease?

A
  • staph aureus
  • aspergillus
  • pseudomonas

Internal bacteria;

  • salmonella
  • mycobacterium
20
Q

describe the neutrophil levels in chronic granulomatous disease?

A

acutely = neutrophil count is high

latterly once granulomas are formed = neutrophil count gos to normal

21
Q

how would you treat chronic granulomatous disease?

A
  • stem cell transplant
  • gene therapy
  • gamma interferon therapy
22
Q

where do B cells mature?

A

bone marrow

23
Q

where do T cells mature?

A

thymus

24
Q

where do T cells arise?

A

haematopoetic stem cells in bone marrow

25
Q

what are 3 jobs of antibodies?

A
  • identifying the antigen
  • recruiting immune cells to clear the antigen
  • neutralising the toxins produced by antigens
26
Q

what is reticular dysgenesis a defect of?

A

= defect in haematopoetic stem cells

27
Q

what does reticular dysgenesis fail to produce?

A

= all leukocytes

28
Q

what are symptoms of reticular dysgenesis?

A
  • fatal septicaemia

- recurrent bacterial infections

29
Q

how would you treat reticular dysgenesis?

A

haematopeotic stem cell transplant

30
Q

what is severe combined immunmodeficieicny a defect of?

A

= lymphocyte precursor molecules

31
Q

what does severe combined immunmodeficieicny fail to produce?

A

= all lymphocytes

32
Q

what are symptoms of severe combined immunmodeficieicny?

A
  • infections by 3 months
  • failure to thrive
  • diarrhoea
  • unusual skin infections
33
Q

why do infections in severe combined immunmodeficieicny not arise until 3 months?

A

as mothers help protect them by using IgG antibody (in placenta)

34
Q

how would you treat severe combined immunmodeficieicny?

A

prophylactically

  • antibiotics
  • antibody infusion/replacement

definitvely

  • stem cell transplant
  • gene transplant
35
Q

what is Di George syndrome a defect of?

A

= thymocyte development

36
Q

what does Di George syndrome fail to produce?

A

= CD4+ and CD8+ T cells

37
Q

what chromosome is involved in Di George syndrome?

A

22q11

38
Q

what is X-linked agamma globulianemia a defect of?

A

= defect in B cell development

39
Q

what does x-linked gamma globulinaema fail to produce?

A

= mature B cells

Immunodeficiency disorders - resp (1 decks)

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