Immunodeficiency Flashcards

1
Q

what is immunodeficiency?

A

Absence or failure of the normal function of the

immune system

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2
Q

what results of immunodeficiency?

A

increased susceptibility to infection

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3
Q

why are dentists expected to treat increasing numbers of immunodeficient patients?

A

as people with once-fatal diseases continue to

survive them

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4
Q

what are the classification of immunodeficiency?

A
  • specific-involving abnormalities of B and T cells-problem with adaptive immune system
  • non-specific - involving abnormalities in complement or phagocytes- problems with innate immune system
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5
Q

Describe primary immunodeficiency .

A

primary - due to intrinsic defects,often genetic (age-related decline)

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6
Q

Describe secondary immunodeficiency.

A

Results from extrinsic factors:

  • drug therapies for cancer or autoimmune disease
  • irradiation (e.g. in cancer treatment)
  • organ or bone marrow transplantation protocols
  • malnutrition, alcoholism
  • certain infections
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7
Q

Describe B cell immunodefiences.

A
  1. Low serum levels (hypogammaglobulinaemia)
  2. recurrent pyogenic infections i.e sinusitis - ifutreated, severe obstructive lung disease develops from recurrent pneumonia
  3. mostly occur as primary (born with)
    immunodeficiencies
  4. rare with exception of IgA deficiency
  5. Treatment -intravenous replacement therapy with Ig
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8
Q

Name the infectious susceptibility and oral manifestations of No Ig production.

A

IS-Respiratory infections with extracellular bacteria,
Giardia infection in GI tract
Enterovirus infections
OM-Possible sepsis from abscessed teeth

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9
Q

Name the infectious susceptibility and oral manifestations of severely decreased or No IgG production.

A

IS-Respiratory infections with extracellular bacteria
Some patients asymptomatic
OM-Possible sepsis from abscessed teeth ,
Some patients asymptomatic

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10
Q

Name the infectious susceptibility and oral manifestations of severely decreased or No IgA production.

A
IS-Respiratory and GI tract infections 
Some patients asymptomatic
OM-Candidiasis ,
Oral ulcerations, 
Some patients asymptomatic
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11
Q

what are patients with no T cells or poor T cell function susceptible to?

A

opportunistic infections e.g herpes

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12
Q

what is T cell immunodeficiencies due to?

A
  • MHC defects
  • CD40 ligand deficiency
  • CD3 mutations
  • Decreased T cell number
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13
Q

since B cell function depends on T cell function , what does T cell immunodeficiencies also result in?

A

humoral

deficiency i.e. combined immunodeficiency

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14
Q

how severe are primary T cell immunodeficiencies?

A

rare and often fatal e.g severe combined immunodeficiency (SCID)

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15
Q

What are side effects of patients with SCID ?

A

-Failure to thrive, repeated infections i.e herpes simplex
-During first 6-9 months maternally acquired antibodies offer some
protection. Thereafter bacterial infections much
more common

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16
Q

Name some oral manifestations in SCID.

A
  • Candidiasis
  • Herpes infections
  • Recurrent ulcerations of tongue and buccal mucosa
  • Severe necrotizing gingivostomatitis
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17
Q

what does AIDS stand for?

A

Acquired Immunodeficiency Syndrome
or
Acquired Immune Deficiency Syndrome

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18
Q

how is secondary T cell immunodeficiency caused by?

A

caused by infection with HIV (human immunodeficiency virus)

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19
Q

how is HIV transmitted?

A
  1. Sexual contact
  2. Transfer via placenta or milk from mother
    to infant
  3. Blood transfusion
  4. Needle sharing - intravenous drug use
  5. Needlestick injuries
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20
Q

Describe how HIV infiltrates cell.

A

-HIV interacts with our helper T cells , major receptor of HIV to bind to CD4( found in helper t cells)
-Release of RNA
-Reverse transcibred –double stranded DNA forms -integrated into host DNA
Cell activation –HIV rna is transcibred
Proteins for extrerior part of virus translated and released

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21
Q

Describe clinical stages of AIDS.

A

-Infection with HIV -
many individuals asymptomatic
some develop transient fever, swollen lymph nodes,
sore throat, rash

-Abs against HIV proteins take 2-6 weeks to develop
Standard HIV infection tests detect these

-After 1o infection, period of latency (no or few
symptoms)
Progression to AIDS takes 2-15 years

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22
Q

when is HIV then classified as AIDS?

A

Classified as AIDS when CD4+ T cells <200 cells/l

23
Q

what are the clinical stages of AIDS?

A

a) Major opportunistic infectionsE.g. Pneumocystis jirovecii pneumonia,Cryptosporidial diarrhoea
b) malignancy e.g. Kaposi’s sarcoma (tumour of endothelial cells)
c) Thrombocytopenia (low platelet count)
d) nervous system disease - dementia and paralysis

24
Q

how can HIV be treated?

A

-HIV infection can be controlled with highly active antiretroviral therapy (HAART)

25
Q

what is HAART a combination of?

A

nucleoside analog reverse transcription inhibitors

and non-nucleoside RT inhibitor or protease inhibitor

26
Q

when is HAART recommended?

A
  • when CD4+ T cell count is below 350 cells/l
  • in all patients with AIDS defining illness
  • when serum HIV RNA is >50,000-100,000 copies/ml
27
Q

what does HIV/AIDS cause for oral health?

A
  • herpes (fever blisters)
  • hairy leukoplakia
  • oral candidiasis
  • aphthous ulcers
  • oral warts
  • dry mouth
28
Q

how rare are defects in complement proteins?

A

-rare and often inherited

29
Q

what is complement important in fighting?

A

infection anf dissolving immune complexes

30
Q

how do complement defences often present as?

A
  • Recurrent infections or

- Immune complex disease (SLE-like)

31
Q

what causes hereditary angiodema (HAE)?

A

Due to deficiency (absence or dysfunction) in

main inhibitor of classical pathway, C1 inhibitor

32
Q

As well as the inherited form (HAE), C1 inhibitor defiecny may arise later in life due to what?

A
  • autoantibody

- certain B cell leukaemias

33
Q

what are the symptoms of hereditary angioedema (HAE)?

A

recurrent episodes of angioedema (swelling)

34
Q

where can angiodema occur?

A
  • intestine-excruitating pain, cramps, vomiting
  • periphery- unsightly swelling
  • upper airways - respiratory obstruction which can be fatal
35
Q

what are long term treatments include?

A
  • Danazol (attenuated androgen)
  • Antifibrinolytic agents
  • C1 Inhibitor (C1 INH
36
Q

why is HAE very important for dentists?

A
  • Oedema may occur spontaneously or after very slight trauma e.g. minor dental innveration
  • dental procedures can easily provoke oedema of upper airways
  • rapid action to maintain an open airway
37
Q

what is treatment of oedema?

A

C1 INH concentrate should resolve oedema in 30 mins - 2 hrs

38
Q

what is prevention of oedema?

A

Daily Danazol 100-600mgs, for 5 days before & 2 days after or C1 INH concentrate (500-1500 Units)- up to 24 hrs beforehand

39
Q

what are phagocytes (neutrophils/monocytes/macrophages) imporant for?

A

defence vs bacteria

40
Q

what does general deficiency in neutrophils cause?

A

Overwhelming bacterial infection

41
Q

what are 3 important genetic defects in phagocytes?

A

i) Chronic granulomatous disease (CGD)
ii) Leucocyte adhesion deficiency
iii) Chédiak-Higashi syndrome

42
Q

what do these genetic defects lead to?

A
  • Susceptibility to severe infections

- Can be fatal

43
Q

who does chronic granulomatous disease affect?

A
  • 1-4 people in every million

- defective gene on X chromosome so boys are more commonly affected

44
Q

what do patients with chronic granulomatous disease present with?

A

a lot of dental disease, gum abscesses and scarring

45
Q

what is chronic granulomatous disease due to?

A

-defective NADPH oxidase (enzyme responsible
for generation of superoxide anions)
-These anions normally help phagocytes kill ingested bacteria
(Staphylococcus aureus) and fungi

46
Q

What happens due to the defectiveness of NADPH?

A

-instead they stay alive and cell-mediated response develops
-This leads to Granuloma formation that can obstruct GI & urogenital systems
(Rarely intraoral granulomas can form)

47
Q

what is the treatment pf chronic granulomatous disease?

A

Prophylactic daily antibiotics and IFN

48
Q

what causes leukocyte adhesion deficiency?

A

-Lack of integrin subunit, important in adhesion and homing
-Phagocytic cells are unable to bind properly to endothelial
cells and emigrate through
vessel walls to infected tissue

49
Q

what does this result in (leukocyte adhesion deficiency)?

A

repeated pyogenic infections

50
Q

what are the oral manifestations of leukocyte adhesion deficiency?

A
  • Early childhood periodontitis
  • Oral ulcerations
  • Delayed wound healing
51
Q

How does Chediak- hibachi syndrome arise?

A

Arises through rare autosomal recessive disorder due to mutation in gene that regulates lysosome trafficking

52
Q

what does this mutation result in?

A
  • giant lysosomal granules in neutrophils, monocytes and lymphocytes
  • Phagocytes are defective in chemotaxis, phagocytosis
    and bactericidal activity
  • Infections with intra- and extracellular bacteria,
    granuloma formation
53
Q

what are the oral manifestations of Chediak- higashi syndrome?

A

early childhood periodontitis , possibly increased bleeding