Immunodeficiency Flashcards

(53 cards)

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
what is HAART a combination of?
nucleoside analog reverse transcription inhibitors | and non-nucleoside RT inhibitor or protease inhibitor
26
when is HAART recommended?
- 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
what does HIV/AIDS cause for oral health?
- herpes (fever blisters) - hairy leukoplakia - oral candidiasis - aphthous ulcers - oral warts - dry mouth
28
how rare are defects in complement proteins?
-rare and often inherited
29
what is complement important in fighting?
infection anf dissolving immune complexes
30
how do complement defences often present as?
- Recurrent infections or | - Immune complex disease (SLE-like)
31
what causes hereditary angiodema (HAE)?
Due to deficiency (absence or dysfunction) in | main inhibitor of classical pathway, C1 inhibitor
32
As well as the inherited form (HAE), C1 inhibitor defiecny may arise later in life due to what?
- autoantibody | - certain B cell leukaemias
33
what are the symptoms of hereditary angioedema (HAE)?
recurrent episodes of angioedema (swelling)
34
where can angiodema occur?
- intestine-excruitating pain, cramps, vomiting - periphery- unsightly swelling - upper airways - respiratory obstruction which can be fatal
35
what are long term treatments include?
- Danazol (attenuated androgen) - Antifibrinolytic agents - C1 Inhibitor (C1 INH
36
why is HAE very important for dentists?
- 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
what is treatment of oedema?
C1 INH concentrate should resolve oedema in 30 mins - 2 hrs
38
what is prevention of oedema?
Daily Danazol 100-600mgs, for 5 days before & 2 days after or C1 INH concentrate (500-1500 Units)- up to 24 hrs beforehand
39
what are phagocytes (neutrophils/monocytes/macrophages) imporant for?
defence vs bacteria
40
what does general deficiency in neutrophils cause?
Overwhelming bacterial infection
41
what are 3 important genetic defects in phagocytes?
i) Chronic granulomatous disease (CGD) ii) Leucocyte adhesion deficiency iii) Chédiak-Higashi syndrome
42
what do these genetic defects lead to?
- Susceptibility to severe infections | - Can be fatal
43
who does chronic granulomatous disease affect?
- 1-4 people in every million | - defective gene on X chromosome so boys are more commonly affected
44
what do patients with chronic granulomatous disease present with?
a lot of dental disease, gum abscesses and scarring
45
what is chronic granulomatous disease due to?
-defective NADPH oxidase (enzyme responsible for generation of superoxide anions) -These anions normally help phagocytes kill ingested bacteria (Staphylococcus aureus) and fungi
46
What happens due to the defectiveness of NADPH?
-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
what is the treatment pf chronic granulomatous disease?
Prophylactic daily antibiotics and IFN
48
what causes leukocyte adhesion deficiency?
-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
what does this result in (leukocyte adhesion deficiency)?
repeated pyogenic infections
50
what are the oral manifestations of leukocyte adhesion deficiency?
- Early childhood periodontitis - Oral ulcerations - Delayed wound healing
51
How does Chediak- hibachi syndrome arise?
Arises through rare autosomal recessive disorder due to mutation in gene that regulates lysosome trafficking
52
what does this mutation result in?
- 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
what are the oral manifestations of Chediak- higashi syndrome?
early childhood periodontitis , possibly increased bleeding