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Inflammation and Wound Healing Exam 1 > Immunodeficiency > Flashcards

Immunodeficiency Flashcards

1
Q

what are the two types of immunodeficiency disorders?

A
  • primary: congenital-inherited as autosomal recessive genes
  • secondary: comprised of outside factors ( cancer therapy, HIV, immuno-suppression therapy etc)
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2
Q

what are the types of primary immune deficiency disorders?

A
  • B cell disorders
  • T cell disorders
  • T and B cell disorders
  • complement deficiency disorders
  • phagocytic dysfunctions
  • leukocyte adhesion molecule deficiencies
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3
Q

signs that an immunodeficiency disorder is present?

A
  • recurrent infection or severe infections
  • poor response to treatment
  • delayed or incomplete recovery from illness
  • certain types of cancers
  • certain infections
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4
Q

a deficiency in B cells would cause what disease
susceptibility?

A
  • recurrent bacterial infections (otis media and pneumonia)
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5
Q

a deficiency in T cells would cause what disease
susceptibility?

A

intracellular viral, bacterial, fungal or protozoal infections

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6
Q

a deficiency in phagocytes would cause what disease susceptibility?

A

systemic infection with bacteria that are normally of low virulence

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7
Q

a deficiency in complements would cause what disease susceptibility?

A

bacterial infections and autoimmune diseases

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8
Q

a deficiency in leukocyte adhesion molecules would cause what disease
susceptibility?

A

recurrent bacterial infections, impaired pus formation, impaired wound healing

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9
Q

what are the common B cell deficiency diseases ?

A
  • X-linked Infantile Agammaglobulinemia (XLA) / Bruton Disease
  • Common Variable Immunodeficiency (CVID) and Transient Hypogammaglobulinemia
  • selective deficiency of IgA
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10
Q

what are the common T cell deficiency diseases ?

A
  • congenital thymic aplasia (DiGeorge syndrome)
  • chronic mucocutaneous candidiasis
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11
Q

what are the common B and T cell deficiency diseases ?

A
  • Ataxia-telangiectasia
  • Severe combined Immunodeficiency
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12
Q

what are the common phagocytic deficiency diseases ?

A
  • chediak-higashi syndrome
  • chronic granulomatous diseases
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13
Q

what are the common complement deficiency diseases ?

A

hereditary angioneurotic edema

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14
Q

what are the tests used in diagnosis of immunodeficiency disorders?

A
  • immunoelectrophoresis
  • low serum antibodies against vaccine
  • white blood cell count
  • T cell function - mixed lymphocyte culture
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15
Q

what does immunoelectrophoresis measure?

A

absence or abnormal levels of antibody/immunoglobulin or complement proteins

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16
Q

what are ways to manage or treat immunodeficiency disorders?

A
  • intravenous immunoglobulin
  • antibiotics
  • anti-inflammatory
  • gene therapies
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17
Q

what are the characteristics of X-linked infantile agammaglobulinemia (Bruton’s disease)?

A
  • no circulating B cells and all classes of Ig are absent
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18
Q

what are the characteristics of transient hypogammaglobulinemia?

A
  • more likely to occur in premature babies
  • temporary
19
Q

what are the characteristics of common variable hypogammaglobulinemia?

A
  • B cells fail to mature into plasma cells
  • variable (some have a decrease in IgA and IgG but others may have decrease in all three IgA, IgG and IgM)
20
Q

what are the characteristics of selective immunoglobulin deficiency?

A

absence of certain subclasses (ex IgA)

21
Q

what is the most common selective immunoglobulin deficiency and what does it cause?

A
  • IgA deficiency
  • decreased immune function in the mucosal surfaces of the mouth, GI tract and lungs causing an increase risk for respiratory and GI infections
22
Q

what is IgA also associated with ?

A

autoimmune diseases

23
Q

how do you diagnose IgA deficiency ?

A

symptoms
- family history of IgA deficiency or Bruton’s
- mouth infections
- frequent respiratory infections
- unexplained asthma or bronchiectasis
- chronic diarrhea
tests
- serum immunoelectrophoresis
- quantitative immunoglobulins

24
Q

how do you treat IgA deficiency ?

A
  • antibiotics and anti-inflammatories
25
Q

why are intravenous immunoglobulin not recommended as a treatment for IgA deficiencies?

A

increased risk of life threatening allergic reactions or anaphylaxis

26
Q

what does DiGeorge syndrome cause?

A
  • abnormal thymus: poor T cell development
  • abnormal parathyroid: blood calcium is low
  • infants may be jittery or have seizures
27
Q

how do you diagnosis DiGeorge syndrome?

A
  • signs and symptoms present at birth
  • in teen years: speech delays or problems
  • genetic tests
28
Q

what is the treatment for DiGeorge syndrome?

A

variable - calcium replacement if T cell function is low but sufficient

29
Q

what is chronic mucocutaneous candidiasis?

A

selective defect in protection against candida albicans causing Thrush

30
Q

what is ataxia telangiectasia?

A
  • Autosomal recessive chromosomal breakage disorder of the ATM (ataxia telangiectasia mutated) protein, an essential enzyme for DNA repair
31
Q

what are the symptoms of ataxia telangiectasia ?

A
  • progressive decline in motor neurologic function
  • wobbly gait
  • tremors
  • abnormal eye movemnts
  • corkscrew-shaped blood vessels
32
Q

is there a cure for ataxia telangietasia?

A

no cure or therapy

33
Q

what causes severe combined deficiency disease (SCID) “bubble boy syndrome” ?

A

mutations in genes that encode proteins necessary for developing T and B lymphocytes
- adenosine deaminase
- recombinase activating genes 1 and 2 deficiency

34
Q

what does severe combined deficiency disease (SCID) “bubble boy syndrome” cause ?

A

extreme susceptibility to severe infections

35
Q

what are the signs of SCID in infants?

A
  • severe infections from organisms that are usually not harmful
  • susceptible to vaccines
  • fungal (yeast) infections may be hard to treat
  • persistant diarrhea
  • severe weight loss and malnutrition
  • rash caused by mothers T cells
36
Q

how do you diagnosis SCID?

A
  • family history
  • count of peripheral blood lymphocytes and examine T lymphocytes through lab tests
37
Q

how do you treat SCID?

A
  • isolation
  • replacement therapy (ADA gene therapy)
  • bone marrow transplant
38
Q

what causes chediak-hagashi syndrome?

A
  • phagocytosed bacteria are not destroyed by the lysosome’s enzymes.
    due to mutation in the lysosomal trafficking regulator gene (LYST)
39
Q

what are the symptoms of chediak-higashi syndrome?

A
  • partial albinism
  • platelete disfunction
  • associated with aggressive periodontisis
40
Q

what causes chronic granulomatous disease?

A

The body’s phagocytic cells are unable to make hydrogen peroxide and other oxidants making patients susceptible to infections with bacteria and fungi that usually are of low virulence

41
Q

what are the symptoms of chronic granulomatous disease?

A
  • in infancy, recurrent unusual infection with bacteria or fungus
  • prolonged drainage, delayed healing, and residual scaring
  • fungal pneumonia
  • major risk in handling garden mulch
42
Q

how is chronic granulomatous disease managed?

A
  • antibiotic prophylaxis, an antifungal drug, interferon gamma and no gardening
43
Q

what are the consequences of a deficiency in the complement cascade?

A
  • increased susceptibility to bacterial infections and autoimmune diseases
  • failure to clear immune complexes
  • deficiencies of C5,6,7,8,or 9, late acting complement proteins involved in forming holes
  • susceptible to the bacteria neisseria
  • deficiencies in regulators of complement - hereditary angioneurotic edema

Inflammation and Wound Healing Exam 1 (15 decks)

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