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
Severity of infection that is a red flag for immunodeficiency
need for prolonged IV abx/hospitalizations to clear infections Deep skin or organ abscess >=2 months of antibiotics without improvement
25
Organisms that are red flags for immunodeficiency
PJP CMV obtained from live vaccines
26
Sx of combined immunodeficiency
bacterial, viral, fungal, opportunistic infections Failure to thrive chronic diarrhea
27
Sx of humoral deficiency
after 4-6mo recurrent resp infections upper and lower FTT chronic diarrhea hepatomegaly/splenomegaly abnl lung exam digital clubbing
28
Sx of phagocytic disorders
soft tissue infections req surgical debridement dental infections leading to premature tooth loss Recurrent anorectal infections
29
Sx of complement disorders
recurrent severe bacterial infections autoimmune manifestations
30
DiGeorge
hypoCa congenital cardiac disease dysmorphic facies T-cell disorder
31
Hyper IgE syndrome
coarse facial features chronic-infected eczema cold abscesses
32
Wiskott-Aldrich syndrome
petechiae easy bleeding eczema
33
Ataxia telangiectasia
ataxia, telangictasias, loss of developmental milestones
34
CGD mode of inheritance
X-linked or autosomal recessive
35
CGD pathophys
Dysfunctional neutrophils and monocytes unable to kill catalase-positive organisms Unable to generate oxygen species by NADPH oxidase Genes: gp91, p22, p47, p67
36
Concerning bacteria for those with CGD
pseudomonas, staph aureus, serratia, nocardia, aspergillus, burkholderia
37
Recurrent infection, osteo, inflammatory bowel disease with granulomas, fistula forming wound complication. Dx?
CGD
38
Modes of testing for NADPH oxidase function
nitroblue tetrazolium dye (NBT) -- stays yellow Dihydrorhodamine test (DHR) -- DHR does not convert to rhodamine
39
Leukocyte adhesion disorder genes and sx
Diagnose with flow cytometry for CD18 (type 1), CD15 (type 2 - benefits from dietary fructose) Impaired pus formation
40
Delayed umbilical cord separation >4 weeks, dx?
Leukocyte adhesion defect
41
How do you diagnose myeloperoxidase deficiency (MPO)
flow cytometry for neutrophil MPO clinically BENIGN Can have disseminated candida in patients with diabetes
42
What is Chediak-Higashi syndrome
abnormal fusion of intracellular granules with vesicle trafficking (AR defect in LYST gene)
43
Recurrent staph and strep infetions, oculocutaneous albanism, tooth loss, progressive neurologic abnormalities (periph neuropathy), coag defect w/ plt dysfunction, HLH-like syndrome. Dx?
Chediak-Higashi syndrome
44
How do you diagnose Chediak-Higashi
smear: giant neutrophil granules hair shaft: melanin clumping
45
Diagnosis of agammaglobulinemia
B-cells are absent (CD19, CD20, T cells are intact) Absent/low IgG Genetic testing for BTK in males
46
Treatment of agammaglobulinemia
immunoglobulin replacement therapy Can only give inactivated vaccinations for T-cell immunity ppx antibiotics
47
Recurrent sinopulmonary infections with variable onset >2 yo GI sx (giardia, bacterial overgrowth), increased risk of autoimmune disorder, may present with malignancies. Dx?
Common Variable Immunodeficiency
48
How to diagnose CVID
IgG +/- IgA or IgM Normal lymphocytes Poor response to vaccines
49
When does transient hypogammaglobulinemia of infancy resolves? What does this mean for vaccines?
6 yo Normal vaccine response, normal B-cells
50
What's happening with B-cells and T-cells in Severe Combined Immunodeficiency?
B-cells impaired T- cells absent
51
lymphadenopathy, hepatosplenomegaly, diffuse erythroderma. Dx and what is it associated with?
Omenn syndrome SCID
52
Absent thymus on imaging, ddx?
SCID DiGeorge
53
Tx of SCID
HSCT Enzyme replacement for adenosine deaminase deficiency Transfusion of blood products with irradiation, leukoreduction, CMV neg Abx ppx
54
Triad of petechiae, eczema, immunodeficiency
Wiskott-Aldrich - also has elevated IgE
55
Hyper IgM syndrome, what happens?
defect in class switching (low IgG, IgA, IgE CD40L mutation Need Ig replacement, prophylactic abx, HSCT
56
Hyper IgM syndrome, what happens?
defect in class switching (low IgG, IgA, IgE CD40L mutation Need Ig replacement, prophylactic abx, HSCT
57
Recurrent infection to encapsulated orgs like N. meningitides, N. gonorrhoeae
complement disorder
58
Angioedema, severe
C1-esterase inhibitor deficiency
59
How do you assess classical complement pathway, what about alternative complement pathway?
CH50 AH50