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COMAT - Peds > Immunology > Flashcards

Immunology Flashcards

1
Q

What is Kawasaki disease and what patient population is affected?

A

Multi-system vasculitis that affects Asian heritage children < 5 years old

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2
Q

What is the pneumonic to remember the signs of Kawasaki disease?

A

CRASH and Burn

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3
Q

Symptoms of Kawasaki Disease?

A

CRASH and Burn
- Conjunctivitis
- Rash
- Adenopathy (unilateral)
- Strawberry tongue
- Hands/feet are red/swollen/flaking
- Fever for > 5 days

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4
Q

Symptoms of Kawasaki Disease?

A

CRASH and Burn
- Conjunctivitis
- Rash
- Adenopathy (unilateral)
- Strawberry tongue
- Hands/feet are red/swollen/flaking
- Fever for > 5 days

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5
Q

What are children with Kawasaki disease at risk for?

A

Coronary A. Aneurysm/MI

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6
Q

What 2 changes may be seen on labs with Kawasaki disease?

A

Elevated ESR and platelet count

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7
Q

What 2 things are used in the treatment for Kawasaki disease?

A

Aspirin
IVIG – prevents Coronary A. Aneurysm

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8
Q

Aspirin is part of the treatment for Kawasaki disease, although it is rarely given to children. Why?

A

Reye Syndrome

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9
Q

What is Reye syndrome?

A

Aspirin in children
–> Mitochondrial injury and liver failure
–> Hyperammonemia and Encephalopathy

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10
Q

What immunologic change is present with Bruton X-linked Agammaglobulinemia?

A

Low levels of B cells and immunoglobulins

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11
Q

When will CVID present in life? What defect is present?

A

Presents later in life > 15 years of age
– Combined B and T cell defect to cause low Ig levels

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12
Q

IgA deficiency often presents how? Why?

A

Anaphylactic reaction upon administration of transfusion because of the patient’s Anti-IgA antibodies!

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13
Q

What causes Hyper-IgM syndrome?

A

Absent CD40 ligand = NO class switching for the B cells

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14
Q

If a person is missing a CD40 ligand, what syndrome will they have?

A

Hyper-IgM

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15
Q

How is DiGeorge Syndrome inherited?

A

Autosomal Dominant

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16
Q

What is the pneumonic to remember the signs of DiGeorge Syndrome?

A

CATCH 22

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17
Q

Symptoms of DiGeorge syndrome?

A

CATCH 22
- Cardiac anomalies
- Abnormal facies
- Thymic aplasia
- Cleft palate
- Hypocalcemia
- Deletion of 22q11

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18
Q

What immunologic change will be present with DiGeorge syndrome and why?

A

Low T cells because of the thymic aplasia

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19
Q

Since DiGeorge syndrome has low levels of T cells, what types of infections are they more at risk for?

A

Viruses
Fungi
Pneumocystis Jirovecii

20
Q

What mutation causes Ataxia-Telangiectasia?

A

Mutation in a gene responsible for repair of dsDNA breaks

21
Q

If a person cannot repair dsDNA breaks, what symptoms will they have?

A

Cerebellar ataxia
Oculocutaneous telangiectasias

22
Q

How is SCID inherited?

A

X-linked recessive – boys only

23
Q

What defects cause SCID?

A

Stem cell maturation defect and low levels of Adenosine Deaminase

24
Q

What immunologic change is present with SCID?

A

LOW levels of B AND T cells

25
Q

How is Wiskott - Aldrich inherited?

A

X-linked recessive

26
Q

What immunologic changes are present with Wiskott-Aldrich?

A

Low levels of platelets and IgM

27
Q

What pneumonic is used with Wiskott-Aldrich?

A

WIPE

28
Q

Signs of Wiskott-Aldrich?

A

WIPE
- Wiskott-Aldrich
- Infections
- Purpura/bleeding
- Eczema

29
Q

What is deficient with Chronic Granulomatous Disease (CGD)?

A

Deficient Superoxide (NADPH) production

30
Q

What 2 tests are used to diagnose Chronic Granulomatous Disease?

A

Dihydrorhodamine test
Nitroblue Tetrazolium test

31
Q

What are the 2 main signs of Leukocyte Adhesion Deficiency?

A

Absent pus
Omphalitis = delayed separation of the umbilical cord

32
Q

What are the signs of Chediak-Higashi?

A

Albinism
Peripheral neuropathy
Neutropenia

33
Q

If a child is albino, has peripheral neuropathy and recurrent infections, what do they have?

A

Chediak-Higashi

34
Q

Those with Chediak-Higashi are at risk for what types of infections?

A

Pyogenic bacteria – staph aureus/s. pyogenes

35
Q

What will be seen in the neutrophils with Chediak-Higashi?

A

Giant granules

36
Q

What pneumonic is used to remember Job syndrome?

A

FATED

37
Q

Signs of Job syndrome?

A

FATED
- Facies are coarse
- Abscesses
- Teeth are retained
- Elevated IgE/Eosinophilia
- Dermatologic conditions

38
Q

Signs of Job Syndrome?

A

FATED
- Facies are coarse
- Abscesses
- Teeth are retained
- Elevated IgE
- Dermatologic conditions

39
Q

How is Hereditary Angioedema inherited?

A

Autosomal Dominant

40
Q

What is deficient with Hereditary Angioedema?

A

C1 Esterase Inhibitor is deficient

41
Q

What is the treatment for Hereditary Angioedema?

A

C1 inhibitor

42
Q

What is the patient unable to form with Terminal Complement Deficiency (C5-C9)?

A

Membrane Attack Complex (MAC)

43
Q

What are those with a Terminal Complement Deficiency at an increased risk for?

A

Neisseria infections

44
Q

What are those with a Terminal Complement Deficiency at an increased risk for?

A

Neisseria infections

45
Q

Many of the immunologic disorders are treated with what 2 things?

A

Prophylactic antibiotics + IVIG

46
Q

What 2 medications should be given for Chronic Granulomatous Disease prophylaxis?

A

TMP-SMX
Itraconazole

COMAT - Peds (12 decks)

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