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Pediatrics > Pediatric Immunology/Allergology > Flashcards

Pediatric Immunology/Allergology Flashcards

1
Q

[Genetic bases of atopy]

gene region associated with atopy

A

5q23-35

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2
Q

[Risk of allergic disease]

One parent is allergic

A

50%

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3
Q

[Risk of allergic disease]

both parents are allergic

A

66%

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4
Q

Age at which allergic rhinitis can be generally established

A

6 years old

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5
Q

[Allergic Rhinitis]

Early phase reactions

A
  1. Degranulation of mast cells and release of inflammatory mediators
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6
Q

[Allergic Rhinitis]

Late phase reaction

A
  1. arises 4-8 hours following allergen exposure
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7
Q

[Allergic Rhinitis]

Cut off value for intermittent symptoms

A

<4 days per week

OR

4 weeks

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8
Q

[Allergic Rhinitis]

Cut off value for persistentsymptoms

A

> 4 days per week

AND

> 4 weeks

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9
Q

[Allergic Rhinitis]

Moderate-severe symotoms

A
  1. Abnormal sleep
  2. Impairment of daily activities
  3. Impaired work and school
  4. Troublesome symtoms
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10
Q

[Allergic Rhinits: Treatment]

Moderate-severe

A
  1. Intranasal CS

2. H1 antihistamine or LTRA

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11
Q

[Allergic Rhinits: Treatment]

If with conjunctivatis

A

Add

  1. Oral h1 antihitamine OR
  2. Intraocular H1 antihistamine OR
  3. Intraocular cromone
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12
Q

What are the 10 warning signs of immunideficiency

A
  1. Four or more new ear infections within 1 year
  2. Two or more serious sinus infections within 1 year
  3. Two or more months on antibiotics with little effect
  4. Two or more pneumonias within 1 year
  5. Failure of an infant to gain weight or grow normally
  6. Recurrent, deep skin or organ abscesses
  7. Persistent thrush in mouth or fungal infections
  8. need for IV antibiotics to clear infection
  9. Two or more deep-seated infections including septicemia
  10. Family history of PI
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13
Q

___ disease wherein preBcell to Bcell development is blocked in heavy chain gene rearrangement

A

Bruton Disease

btk gene

Healthy infants until 6 months old

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14
Q

What is the treatment for Bruton disease

A

Gamma globulin IM once a month at 2-3 weeks

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15
Q

[Diagnose]

Fungal inection at 4 months of age, no visible tonsillar tissue, no lymphadenopathies, no thymic shadow, low IgG, IgA and IgE

A

SCID

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16
Q

What enzyme deficiency is associated with SCID?

A

Adenosine deaminase

17
Q

What are the components of DiGeorge Syndrome?

A
  1. Cardiac defect (TOF)
  2. Abnormal facies
  3. Thymic aplasia
  4. Cleft palate
  5. Hypocalcemia

22q11.2 chromosomal deletion

18
Q

What are the components of Wiskott-Aldrich Syndrome?

A
  1. Thrombocytopenia
  2. Infections
  3. Eczema
  4. WASP mutation
19
Q

[Diagnosis]

Prolonged bleeding from circumcision site
Bloody diarrhea during infancy

Normal appearing megakaryocyte but defective platelets

A

Wiskot-Aldrich Syndrom

wasp mutation

20
Q

What are the effective treatment for primary B cell defects?

A
  1. Antibiotics for infection

2. regular IVIG 400mg/kg per month SQ

21
Q

[Diagnose]

Small to absent tonsils, no palpable LN

No antibody production,
Total Ig <100
lack B cells

A

Bruton Agammaglobinemia

22
Q

[Diagnose]

Low IgG
Normal Bcells
normal nodes and tonsils

increased granuloma risk

A

CVID

23
Q

[Diagnose]

Low IgA (<10mg/dL)

Urogenital infections, Giardiasis

A

Selective IgA deficiency

6p21.3

24
Q

[Diagnose]

Normal to High IgM
No IgG
No IgA
no IgE

neutropenic, P. jirovecii pneumonia

A

Hyper IgM syndrome

Tx: BMSCT

25
Q

[Diagnose]

Low Ig
Lack anti-EBNA
Lack long-lived T cell immunity

Inadequate immunity to EBV leading to fatal infectious mononucleosis

A

X-linked lympho-proliferative disease or Duncan disease

26
Q

What is the most severe immunodeficiency?

A

SCID

27
Q

What are the components of Job Syndrome

A
  1. Coarse facies
  2. Coronary artery aneurysm
  3. Pathologic fractures
  4. Mortality in adulthood
28
Q

[Disorders in Neutrophil Function]

Recurrent pyogenic infections, neutropenia, hepatosplenomegaly, partial oculo-cutaneous albinism, photophobia, light skin, silvery hair, roatry nystagmus

Decreased chemotaxis, impaired NK function

A

Cheidak-Higashi

29
Q

[Disorders in Neutrophil Function]

Impaired neutrophil adhesion and platelet activation, decreased binding of C3b to neutrophils

delayed umbilical cord separation, slow healing ulcers, bleeding tendency

A

Leukocyte adhesion deficiency

30
Q

[Disorders in Neutrophil Function]

recurrent pyogenic infections, osteomyelitis, lympahdenitis, granuloma formation

Failure to activate neutrophil respiratory burst, failure to kill catalase-positive microbes

A

CGD

Pediatrics (15 decks)

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