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Abbie Year 5 Pathology > Primary immunodeficiency 1 > Flashcards

Primary immunodeficiency 1 Flashcards

1
Q

Innate immune system

A

Cells - polymorphonuclear cells, monocytes, macrophages, dendritic cells and NK cells… Soluble component -

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2
Q

Phagocytes express?

A

Pathogen recognition receptors …

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3
Q

Polymorphonuclear cells release

A

Releas enzymes, histamines …

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4
Q

Mononuclear cells

A

produced in bone marrow, differentiate to macrophages

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5
Q

What happens when pathogen infects tissue?

A

Endothelial cell activation …

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6
Q

Phagocyte deficiency types

A

Reticular dysgenesis, Kostmann Syndrome, cyclic neutropenia, defect of phagocyte migration, cytokine deficiency

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7
Q

What is reticular dysgenesis

A

autosomal recessive severe SCID. Mutation in mitochondrial energy metabolism enzyme adenylate kinates 2 (AK2)

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8
Q

What is Kostmann syndrome

A

Autosomal recessivce severe congenital neutropenia

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9
Q

What is cyclic neutropenia?

A

…

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10
Q

Defect of phagocyte migration

A

Leukocyte adhesion deficiency - deficiency of CD18 (beta-2 integrin suubunit)

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11
Q

Luekocyte adhesion deficienyc feautures

A

… absence of pus formation …

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12
Q

Investigation of chronic granulomatous disease

A

Nitroblue tetrzolium (NBT) test, dihydrorhodamine (DHR) flow cytometry test

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13
Q

Cytokine deficiency - failure to produce what?

A

IFN gamma, IFN gamma receptor, IL12, IL12 receptor

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14
Q

Recurrent infections in skin or mouth bacterial

A

staph aureus, …

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15
Q

Investigations of phagocyte deficiencies

A

neutrophil count, leukocyteadhesion markers, NBT or DHR test

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16
Q

Recurrent infections with high neutrophil count on FBC but no abscess formation

A

Leukocyte adhesion deficiency … neutrophils can get into bloodstream but not out

17
Q

Child with recurrent infections with hepatosplenomegaly and abnormal dihydrorhodamine test (does not fluoresce)

A

chronic granulomatous disease

18
Q

Draw complement activation pathway

A

…

19
Q

What pathways of complement activation

A

classical, MBL, alternate

20
Q

Complement deficiency - susceptibility to bacterial infections, especially encapsulated bacteria (NHS)

A

Neisseria meningitides, haemophilus influenzae, strep pneumoniae

21
Q

C3 nephritic factors lead to consumption of C3 - true or false?

A

True

22
Q

Functional complement tests

A

CH50 classical pathway and AP50 alternative pathway

23
Q

C1q deficiency investigation results

A

CH50 abnormal (low?)

24
Q

Properidin deficiency investigation results

A

AP50 abnormal (low)

25
Q

C9 deficiency investigation results

A

CH50 and AP50 low/abnormal

26
Q

SLE invstigation rsults

A

C4 LOW/abnormal, may have reduction/abnormalities in C3 AND or CH50

27
Q

Management of complement deficiencies…

A

vaccinations …

28
Q

Q. no features..

A

…

29
Q

Q. 9 y/o girl …

A

…

Abbie Year 5 Pathology (18 decks)

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