Immunodeficiencies and Fungal Infections Flashcards

1
Q

What are primary immunodeficiencies?

A
  • Group of > 300 rare, chronic disorders in which part of the body’s immune system is missing or functions improperly
  • Can affect a single part of the immune system or multiple components
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2
Q

What are primary immunodeficiencies caused by?

A

Single gene defects

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3
Q

What are secondary immunodeficiencies?

A
  • Acquired diseases affecting the immune system and/or treatments negatively influencing the immune system.
  • Components of the immune system itself are all present and functional
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4
Q

Give examples of secondary immunodeficiency.

A
  • HIV infection

- Patients receiving treatment for malignancy

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5
Q

What types of primary immunodeficiencies are there?

A
  • Antibody deficiency
  • Cellular immunodeficiency
  • Innate immune disorders
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6
Q

What are antibody deficiencies characterised by?

A
  • Characterised by a deficiency of 1 or more subclasses of antibodies due to defective B cell function
  • Absence of mature B-cells
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7
Q

What are cellular immunodeficiencies characterised by?

A

Characterised by impaired T-cell function or the absence of normal T-cells

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8
Q

What are innate immune disorders characterised by?

A
  • Defects in phagocyte function
  • Complement deficiencies
  • Absence or polymorphisms in pathogen recognition receptors
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9
Q

What infections are associated with antibody deficiencies?

A
  • Recurrent bacterial infections of the upper and/or lower respiratory tract
  • S pneumonia, H influenzae
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10
Q

What infections are associated with cellular immunodeficiencies?

A
  • Unusual or opportunistic infections often combined with failure to thrive
  • Pneumocystic jirvecii, CMV(pneumonia)
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11
Q

What infections are associated with defects in phagocyte function?

A
  • Staph aureus (sepsis, skin lesions, abscesses in internal organs)
  • Aspergillus infections (lung, bone and brain )
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12
Q

What infection is associated with complement deficiency?

A

N. meningitidis

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13
Q

How can primary immunodeficiencies be recognised?

A

Be suspicious of infection that is:

  • Severe: requires hospitalisation or IV antibiotics
  • Persistent: won’t completely clear up or clears very slowly
  • Unusual: caused by an uncommon organism
  • Recurrent: keeps coming back
  • Runs in the family
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14
Q

How can HIV/AIDS present in paediatrics?

A
  • Recurrent common childhood RTIs
  • Persistent oral thrush
  • Erythematous papilar rash
  • Generalised lymphadenopathy
  • recurrent/disseminated VZV/HSV infections
  • Failure to thrive
  • Developmental delay
  • Opportunistic infections: CMV, pnumoniae/retinitis, PCP( pneumocystic jirovecii pneumonia)
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15
Q

What is the inheritance of chronic granulomatous disease?

A
  • 65% X-linked

- 35% autosomal recessive

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16
Q

How does chronic granulomatous disease present?

A

-Life-threatening recurrent severe bacterial and fungal infections

17
Q

What is a curative treatment option in chronic granulomatous disease?

A

Haematopoietic Stem Cell Transplant

18
Q

What is the main cause of death in chronic granulomatous disease?

A

Invasive aspergillosis
-Life-time incidence 25-40%
Mortality 35%

19
Q

How is chronic granulomatous disease diagnosed?

A

DHR test

-Non-fluorescent rhodamine derivative oxidised by NADPH oxidase to green fluorescent compound

20
Q

In what circumstances can invasive fungal infections present?

A
  • Symptom of primary immunodeficiency
  • Children with neutropenia due to leukaemia and/or chemotherapy
  • Invasive candidiasis in premature neonates due to immature immune system
  • Children admitted to PICU, treated with broad-spectrum antibiotics and abdominal surgery
21
Q

What are the features of candida infection?

A
  • Endogenous species
  • Transmitted by birth canal, hands of health care workers
  • Positive blood cultures
  • Presents with candidemia
  • Budding of yeast cells
  • Pseudohyphae in tissue
  • Metastatic foci
22
Q

What are the features of aspergillus infection?

A
  • Exogenous species
  • Transmitted by air, water and environment
  • Negative blood cultures
  • Presents with pulmonary disease
  • No sporulation in vivo
  • Hyphal growth in tissue
  • Angio-invasive
23
Q

What is the incidence of neonatal candidaemia?

A
  • > 1500g <1%
  • 750-1500g 3%
  • <750g 12%
24
Q

What is the mortality of neonatal candidaemia?

A

20-40%

25
Q

How does neonatal candidaemia present?

A
  • Presents in the 2nd/3rd week of life
  • Sepsis syndrome
  • Thrombocytopenia
  • Hyperglycaemia
26
Q

Give examples of risk factors for neonatal candidaemia.

A
  • Extreme prematurity
  • Extremely low birth weight
  • Indwelling catheters
  • Hyperglycaemia
  • Immunosuppression
  • Broad-spectrum antibiotics