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Pediatrics > Allergy and Immunology > Flashcards

Allergy and Immunology Flashcards

1
Q

Cut off for making to intermittent vs. persistent asthma

A

Daytime sx >2 days a week
Nighttime sx >2 a month
SABA use >2 days a week
Steroid need >1 a year
FEV1/FVC <85%

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2
Q

Disorders of T-lymphocyte function include ____ (3 things)

A
  1. severe combined immunodeficiency,
  2. DiGeorge syndrome
  3. X-linked hyperimmunoglobulin M
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3
Q

The best study to assess vaccine response is _____

A

humoral immune panel (titers)

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4
Q

Defects of the humoral immune system present as ____

A

sinopulmonary infections with encapsulated bacteria

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5
Q

Disorders of humoral immune system: (2); and how you test it

A
  1. X-linked aggamaglobulinemia
  2. common variable immunodeficiency

Lab: serum immunoglobulins

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6
Q

Defects of the innate immune system (3) and how you test it

A
  1. chronic granulomatous disease (CGD),
  2. leukocyte adhesion defects, and
  3. complement deficiencies

Labs: Phagocyte oxidative response evaluates the neutrophil defect of CGD and total hemolytic complement (CH50) determines complement activity

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7
Q

Clinical presentation of T-lymphocyte immune disorders

A

failure-to-thrive, chronic diarrhea, and recurrent opportunistic infections, including CMV, CANDIDA, and PCP (think HIV) – not really bacterial

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8
Q

The most common etiology for atelectasis in status asthmaticus is ____

A

mucus plugging

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9
Q

Definition of “reversibility” in asthma

A

more than or equal to 12% increase in FEV1 from baseline or by an increase of 10% or more of predicted FEV1 after inhalation of a short-acting bronchodilator, in adults also 200 ml increase

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10
Q

clinical presentation of chronic granulomatous disease (CGD)

A

mucocutaneous candidiasis, mesenchymal lymphadenitis, deep granuloma formation, and infections with Staphylococcus aureus, Escherichia coli, Burkholderia, and other gram-negative organisms

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11
Q

Newborn screen showing low T-cell receptor excision circle (TREC). What does it mean and what is the next step?

A

Primary immunodeficiency (severe combined)
For premature infants, repeat until 37wk corrected GA

*start child on ppx abx: Bactrim (>2mo but ok for ppx) and fluconazole

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12
Q

When should mothers be cautioned in breastfeeding (immunodeficiency)

A

severe combined immunodeficiency
- can get CMV from mother

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13
Q

What does allergen immunotherapy do for asthmatics?

A

decrease ICS use and other controller therapy
Does NOT decrease rescue inhaler use

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14
Q

Sores in mucosa (mouth, lips, genitalia, conjunctiva) and palms and soles, history of using a drug, diagnosis and treatment?

A

SJS
Supportive care

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15
Q

Low IgG, Positive anti-granulocyte ab, Positive DAT IgG, multiple bacterial infections what’s the diagnosis?

A

common variable immune deficiency

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16
Q

X-linked agammaglobulinemia (Bruton). What’s the pathophysiology

A

Mutation in Bruton’s tyrosine kinase
–> B-cell defect
–> decreased immunoglobulin

Shows up after 3 mo (maternal Ig protects infants until then)

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17
Q

Pathophysiology of allergic rhinitis

A

Ag-specificIgE on mucosal mast cells and basophils –> cascade of immunologic reactions releasing histamine and tryptase (mast cell granules) and generate leukotrienes

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18
Q

In patients not adherent to controller meds, gets frequent steroids, still worsening, hypoxic to low 90s, RML atelectasis, what’s the next step?

A

admit to inpatient for systemic steroids

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19
Q

After finding absence of TREC (t-cell receptor excision circles), what is the next step to confirm diagnosis?

A

Lymphocyte subsets via flow cytometry

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20
Q

What does flow cytometry for dihydroxy-rhodamine123 reduction test for

A

neutrophil function
Dx for chronic granulomatous disease (CGD)

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20
Q

What does flow cytometry for dihydroxy-rhodamine123 reduction test for

A

neutrophil function
Dx for chronic granulomatous disease (CGD)

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21
Q

Child with foul odor and worse rhinitis of one nare more than the other in a young, mobile child, what do you think of?

A

Nasal foreign body

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22
Q

Major factors of asthma (outside of respiratory risk factors)

A

Obesity and vitamin D deficiency
Residency in farm is PROTECTIVE

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23
Q

Frequency of infections that would make you think immunodeficiency

A

> =4 ear infx a year
=2 serious sinus infection ever
=2 pneumonias a year
=2 systemic bacterial infections per year

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24
Q

Severity of infection that is a red flag for immunodeficiency

A

need for prolonged IV abx/hospitalizations to clear infections
Deep skin or organ abscess
>=2 months of antibiotics without improvement

25
Q

Organisms that are red flags for immunodeficiency

A

PJP
CMV
obtained from live vaccines

26
Q

Sx of combined immunodeficiency

A

bacterial, viral, fungal, opportunistic infections
Failure to thrive
chronic diarrhea

27
Q

Sx of humoral deficiency

A

after 4-6mo
recurrent resp infections upper and lower
FTT
chronic diarrhea
hepatomegaly/splenomegaly
abnl lung exam
digital clubbing

28
Q

Sx of phagocytic disorders

A

soft tissue infections req surgical debridement
dental infections leading to premature tooth loss
Recurrent anorectal infections

29
Q

Sx of complement disorders

A

recurrent severe bacterial infections
autoimmune manifestations

30
Q

DiGeorge

A

hypoCa
congenital cardiac disease
dysmorphic facies
T-cell disorder

31
Q

Hyper IgE syndrome

A

coarse facial features
chronic-infected eczema
cold abscesses

32
Q

Wiskott-Aldrich syndrome

A

petechiae
easy bleeding
eczema

33
Q

Ataxia telangiectasia

A

ataxia, telangictasias, loss of developmental milestones

34
Q

CGD mode of inheritance

A

X-linked or autosomal recessive

35
Q

CGD pathophys

A

Dysfunctional neutrophils and monocytes
unable to kill catalase-positive organisms
Unable to generate oxygen species by NADPH oxidase

Genes: gp91, p22, p47, p67

36
Q

Concerning bacteria for those with CGD

A

pseudomonas, staph aureus, serratia, nocardia, aspergillus, burkholderia

37
Q

Recurrent infection, osteo, inflammatory bowel disease with granulomas, fistula forming wound complication. Dx?

A

CGD

38
Q

Modes of testing for NADPH oxidase function

A

nitroblue tetrazolium dye (NBT) – stays yellow
Dihydrorhodamine test (DHR) – DHR does not convert to rhodamine

39
Q

Leukocyte adhesion disorder genes and sx

A

Diagnose with flow cytometry for CD18 (type 1), CD15 (type 2 - benefits from dietary fructose)

Impaired pus formation

40
Q

Delayed umbilical cord separation >4 weeks, dx?

A

Leukocyte adhesion defect

41
Q

How do you diagnose myeloperoxidase deficiency (MPO)

A

flow cytometry for neutrophil MPO

clinically BENIGN
Can have disseminated candida in patients with diabetes

42
Q

What is Chediak-Higashi syndrome

A

abnormal fusion of intracellular granules with vesicle trafficking (AR defect in LYST gene)

43
Q

Recurrent staph and strep infetions, oculocutaneous albanism, tooth loss, progressive neurologic abnormalities (periph neuropathy), coag defect w/ plt dysfunction, HLH-like syndrome. Dx?

A

Chediak-Higashi syndrome

44
Q

How do you diagnose Chediak-Higashi

A

smear: giant neutrophil granules
hair shaft: melanin clumping

45
Q

Diagnosis of agammaglobulinemia

A

B-cells are absent (CD19, CD20, T cells are intact)
Absent/low IgG
Genetic testing for BTK in males

46
Q

Treatment of agammaglobulinemia

A

immunoglobulin replacement therapy
Can only give inactivated vaccinations for T-cell immunity
ppx antibiotics

47
Q

Recurrent sinopulmonary infections with variable onset >2 yo
GI sx (giardia, bacterial overgrowth), increased risk of autoimmune disorder, may present with malignancies. Dx?

A

Common Variable Immunodeficiency

48
Q

How to diagnose CVID

A

IgG +/- IgA or IgM
Normal lymphocytes
Poor response to vaccines

49
Q

When does transient hypogammaglobulinemia of infancy resolves? What does this mean for vaccines?

A

6 yo

Normal vaccine response, normal B-cells

50
Q

What’s happening with B-cells and T-cells in Severe Combined Immunodeficiency?

A

B-cells impaired
T- cells absent

51
Q

lymphadenopathy, hepatosplenomegaly, diffuse erythroderma. Dx and what is it associated with?

A

Omenn syndrome
SCID

52
Q

Absent thymus on imaging, ddx?

A

SCID
DiGeorge

53
Q

Tx of SCID

A

HSCT
Enzyme replacement for adenosine deaminase deficiency
Transfusion of blood products with irradiation, leukoreduction, CMV neg
Abx ppx

54
Q

Triad of petechiae, eczema, immunodeficiency

A

Wiskott-Aldrich

  • also has elevated IgE
55
Q

Hyper IgM syndrome, what happens?

A

defect in class switching (low IgG, IgA, IgE
CD40L mutation
Need Ig replacement, prophylactic abx, HSCT

56
Q

Hyper IgM syndrome, what happens?

A

defect in class switching (low IgG, IgA, IgE
CD40L mutation
Need Ig replacement, prophylactic abx, HSCT

57
Q

Recurrent infection to encapsulated orgs like N. meningitides, N. gonorrhoeae

A

complement disorder

58
Q

Angioedema, severe

A

C1-esterase inhibitor deficiency

59
Q

How do you assess classical complement pathway, what about alternative complement pathway?

A

CH50
AH50

Pediatrics (26 decks)

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