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Royal Super 4th Year Brain > Allergy/Immunology > Flashcards

Allergy/Immunology Flashcards

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1
Q

Angioedema - classification by mechanism

A

+Urticaria –> activation of mast cells –> IgE mediated, direct mast cell activation, or non-IgE mediated
-Urticaria –> unknown or generation of bradykinin –> hereditary or acquired

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2
Q

Diagnosis to r/o for delayed umbilical cord seperation

A

Leukocyte adhesion defect (type 1)

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3
Q

Common bugs and presentations suggestive of phagocytic (neut) defects

A 
Bugs = catalase positive organisms (aspergillus, S. aureus)
Prez = umbilical separation delay, poor wound healing, severe gingivitis, SSTI, solid organ infection
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4
Q

Dx - potentially life threatening, non-pruritic swelling (with no urticaria), and recurrent abdominal pain

A

Hereditary C1 inhibitor deficiency = recurrent angioedema to face/body, GI tract, and airway

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5
Q

Common bugs and presentation for complement defect immunodeficiency

A
  • Bugs = encapsulated organisms (Neisseria*)

- Prez = rheum disorders/autoimmune, increased susceptibility to infection, angioedema

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6
Q

Mneumonic for hypersensitivity reactions

A

ACID = acute, cytotoxic, immune complex mediated, delayed

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7
Q

4 types of hypersensitivity with x1 example

A
  • Type 1: IgE, anaphylaxis
  • Type 2: IgG/IgM cytotoxic, hemolytic anemia
  • Type 3: Immune complex mediated, serum sickness/SLE
  • Type 4: T-cell, SJS
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8
Q

When (timing wise) is the best time to swab an asymptomatic neonate following C/S for a mother with HSV lesions?

A

> 24 hours from birth

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9
Q

Spectrum of presentations for a parvovirus infection

A
  • Asymptomatic
  • Erythema infectiosum (5th disease, slapped cheek)
  • Transient aplastic crisis with hemolytic anemia
  • Chronic anemia in child with immunodeficiency
  • Hydrops fetalis
  • Arthritis/arthralgia
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10
Q

GAS pharyngitis - tx of choice, duration, when child can go back to daycare?

A

10 days of penicillin or amox

-Go back after 24 hours of Abx

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11
Q

GAS pharyngitis - time window when can treat with Abx

A

For 9 days since onset of symptoms

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12
Q

GAS pharyngitis - why treat with Abx

A
  • Shorten illness course
  • Prevent ARF
  • Prevent suppurative complications
  • Decrease spread of infection
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13
Q

Scarlet fever - what is rash caused by and how does the rash present?

A
  • Caused by strep pyrogenic exotoxin
  • Begins on neck, face, and upper trunk then spreads to rest of body within 1-2 days
  • Palm/sole sparing
  • Sandpaper texture, erythematous, blanchable papules
  • Pastia lines =erythema in skin folds
  • Desquamation in 5-7 days
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14
Q

Indications for tonsillectomy

A
  • OSA secondary to adenotonsillar hypertrophy
  • Recurrent throat infection: >7 past year, >5 last 2 years, >3 last 3 years
  • History of peritonsillar abscess
  • Multiple Abx allergy/intolerance
  • PFAPA
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15
Q

Basic screen of labs for immunodeficiency (x7)

A
  • CBC
  • Immunoglobulins
  • Complement: CH50, C3, C4
  • Vaccine titres
  • Infectious w/u
  • Immunodeficiency panel
  • CXR
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16
Q

x9 red flags for immunodeficiency

A
  • 4 or more AOM in 1 year
  • 2 or more serious PNA in 1 year
  • 2 or more serious sinus infection in 1 year
  • 2 or more deep seated infections
  • Recurrent deep skin or organ abscesses
  • Recurrent thrush when >1 year of age
  • Required IV Abx to clear infections +/- >2 months total of Abx with little effect
  • FTT
  • Family history
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17
Q

Clinical presentation for IgE Syndrome

A
  • Coarse facial features
  • Atypical eczema
  • Recurrent infections - S.aureus, PNA with pneumatoceles
  • Delayed tooth exfoliation
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18
Q

Common bugs for B-cell deficiency (x6)

A
  • Gram pos + neg bacteria, encapsulated
  • Giardia
  • Cryptosporidium
  • Mycoplasma
  • Enterovirus
  • Campylobacter
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19
Q

Classic presentation for B-cell deficiency + other symptoms

A
  • Sinopulmonary infections
  • Bronchiectasis unexplained
  • Other: malabsorption, diarrhea, ileitis/colitis, arthritis
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20
Q

Bugs + presentation of predominant T-cell disorder

A
  • Bugs = viruses, fungal

- Presentation = oral candidiasis, opportunistic infections, other (GVHD, FTT, skin rash, sparse hair)

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21
Q

x2 examples of Ab-deficiencies (humoral or B-cell)

A
  • Common variable immunodeficiency

- X-linked agammaglobulinemia

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22
Q

x5 examples of combined deficiencies

A
  • SCID
  • Wiskott-Aldrich Syndrome
  • 22q11 deletion syndrome
  • Hyper IgM syndrome
  • Ataxia telangiectasis
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23
Q

x2 examples of phagocytic defects

A
  • Chronic granulomatous disease

- Leukocyte adhesion defect

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24
Q

Secondary causes for recurrent infections

A
  • Meds: steroids, chemo, anti-epileptics
  • Infection: HIV, TB, measles, influenza, herpes group
  • Structural: anatomic, impaired membrane integrity, immotile cilia, CF, indwelling catheter
  • Malignancy: lymphoma, leukemia, solid tumors, myeloma
  • Other: DM, renal insufficiency, PLE, malnutrition, lymphangiectasia, asplenia
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25
Q

Work-up for humoral assessment

A
  • Number: lymphocyte subsets (flow cytometry) + immunoglobulins (check albumin to screen for secondary loss)
  • Function: Vaccine titres, isohemagglutinins (IgM Ab to blood group A/B)
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26
Q

Work-up for cellular assessment

A
  • Number: lymphocyte total count and subsets

- Function: specialized testing

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27
Q

Work-up for phagocytic assessment

A
  • Number: neutrophil number

- Function: adhesion markers (CD11+CD18 for leukocyte adhesion defect), neutrophil oxidative burst index (for CGD)

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28
Q

Work-up for complement assessment

A
  • Number: C1 esterase inhibitor levels

- Function: total hemolytic complement (CH50), alternate pathway (AH50), C1 esterase inhibitor function

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29
Q

X-linked agammaglobinemia: what is it and when does it present

A
  • Caused by mutations in the tyrosine kinase that is important for B cell maturation
  • Absent B lymphocytes in peripheral blood = no Ig production
  • Presents 6-24 months when maternal IgG disappears
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30
Q

What to look for on exam in X-linked agammaglobinemia?

A

No tonsils or LNs

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31
Q

What will you find on Ix for X-linked agammaglobinemia?

A
  • No B cells
  • No immunoglobulins
  • No Abs to vaccines
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32
Q

Therapy for X-linked agammaglobinemia?

A

IgG replacement for life

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33
Q

What are children with X-linked agammaglobinemia at risk for?

A

Bronchiectasis

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34
Q

What are the x3 classic features of Common Variable Immunodeficiency?

A
  • Recurrent sinopulmonary infections
  • Autoimmunity
  • Malignancy
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35
Q

Difference on exam between Common Variable Immunodeficiency and XLA?

A

Presence of lymphatic tissue in CVID

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36
Q

What do labs show in Common Variable Immunodeficiency?

A
  • Number: Decreased serum IgG, variable B cells

- Function: absent/reduced Ab vaccines, low isohemagglutinins

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37
Q

What is SCID?

A

Profoundly defective T + B cell function

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38
Q

When does SCID present?

A

2-6 months old

39
Q

Are LN’s and tonsils present on exam in SCID?

A

No

40
Q

What would Ix show in SCID?

A
  • CXR: no thymus
  • Lymphopenia with low/absent T cell function
  • Absent Ab to vaccines
41
Q

Mgmt for SCID

A
  • New immune system = HSCT, gene therapy
  • Prevent/tx infections = Abx, PJP proph, protective isolation, immunoglobulin replacement tx, blood products (CMV neg + irradiated), avoid live vaccines
42
Q

Triad for Wiskott-Aldrich Syndrome

A
  • Thrombocytopenia
  • Eczema
  • Recurrent infections
43
Q

What would investigations show for Wiskott-Aldrich Syndrome

A
  • Number: thrombocytopenia (small), eosinophilia, +/- lymphopenia, variable IgG, low IgM, high IgA/IgE
  • Function: poor Ab response to some vaccines, decreased T cell function
44
Q

Management for Wiskott-Aldrich Syndrome

A
  • Immunoglobulin replacement therapy
  • PJP prophylaxis
  • HSCT
  • Monitor for autoimmune/malignancy
45
Q

What autoimmune conditions (x3) are associated with Wiskott-Aldrich Syndrome?

A
  • Hemolytic anemia
  • Vasculitis
  • IBD
46
Q

What malignancies (x2) are associated with Wiskott-Aldrich Syndrome?

A
  • B cell lymphoma

- Leukemia

47
Q

What immunodeficiency must you rule out in a patient with PJP?

A

SCID

48
Q

What to think of with ataxia + lymphoma?

A

Ataxia telangiectasia

49
Q

Where to look on exam for telangiectasia in AT?

A

Conjunctiva, ears, face

50
Q

Mode of inheritance for ataxia telangiectasia

A

Autosomal recessive

51
Q

What immunodeficiency do you need to avoid ionizing radiation?

A

Ataxia telangiectasia

52
Q

What malignancy are patients with Ataxia telangiectasia most at risk for?

A

Lymphoma

53
Q

What x1 lab is specific for Ataxia telangiectasia?

A

Increased AFP

54
Q

What immunodeficiency has progressive neurodegeneration?

A

Ataxia telangiectasia

55
Q

What part of the immune system is impacted in Ataxia telangiectasia?

A

T-cell (cellular)

56
Q

What organisms to think of for Chronic Granulomatous Disease?

A

Catalase positive = S. aureus, aspergillus, serratia, burkholderia, salmonella

57
Q

What autoimmune condition is associated with Chronic Granulomatous Disease?

A

IBD

58
Q

What would investigations show for Chronic Granulomatous Disease?

A
  • Normal number of neutrophils

- Abnormal function on oxidative burst

59
Q

Features of Hyper IgE syhndrome

A
  • Recurrent abscesses in skin + lungs from S. aureus
  • Mucocutaneous candidiasis
  • Eczema
  • Delayed shedding of primary teeth
  • Bone #, scoliosis, joint laxity
60
Q

What would you find on labs for hereditary angioedema?

A

-Deficiency in C1 inhibitor

61
Q

Mode of inheritance in hereditary angioedema

A

Autosomal dominant

62
Q

What is the more likely cause (rather than a B-lactam allergy) for maculopapular exanthems in children?

A

Viral infection

63
Q

Type 1 immune reaction

A
  • IgE mediated

- <2 hours to symptoms

64
Q

Type 2 immune reaction

A
  • Cytotoxic
  • 10h to a week to symptoms
  • Anemia, thrombocytopenia
65
Q

Type 3 immune reaction

A
  • Immune complex
  • 1-3 weeks to symptoms
  • Serum sickness-like reaction
66
Q

Type 4 immune reaction

A
  • T-cell mediated
  • 2-14 days
  • SJS, DRESS
67
Q

What is the gold standard for ruling out a beta-lactam IgE-mediated allergy - when thought to be unlikely

A

Provocative drug challenge

68
Q

Serratia - what immunodeficiency do you think of?

A

CGD

69
Q

Most common use of immunotherapy?

A

Bee sting allergy

70
Q

Match C3 deficiency and C5 deficiency with (a) early vs late complement component and (b) associated with autoimmune vs meningococcal

A

C3 - early, autoimmune

C5 - late, meningococcal

71
Q

What immunoglobulin deficiency has an increased risk of anaphylaxis with IVIG?

A

IgA

72
Q

Most common food allergens

A
  • Wheat
  • Fish
  • Shellfish
  • Peanuts
  • Tree nuts
  • Sesame
  • Soy
  • Milk
  • Egg
73
Q

x2 ways to test allergies

A
  • Skin prick testing

- IgE blood test = RAST

74
Q

What is the benefit of RAST vs skin prick testing?

A

Can stay on medications like montelukast

75
Q

Tx for serum sickness like reaction

A
  • Stop offending agent

- PO steroids

76
Q

Presentation of serum sickness like reaction

A
  • Rash
  • Arthralgia
  • Fever
  • Edema
  • LAD
  • No mucous membrane involvement
77
Q

What are the x2 indications for allergy testing and possible immunotherapy for insect venom reaction/allergy?

A
  • If airway involvement
  • If anaphylaxis

-Otherwise, do not refer for urticaria or localized reaction

78
Q

What is the most effective use for immunotherapy in setting of allergies?

A

Bee sting allergy

79
Q

Most important aspects of FPIES management

A
  • IV fluid resuscitation
  • Ondansetron
  • Strict avoidance
80
Q

x2 main types of mastocytosis

A
  • Cutaneous

- Systemic

81
Q

Derm sign for cutaneous mastocytosis

A

Darier’s sign

82
Q

Lab finding in systemic mastocytosis

A

Elevated serum tryptase

83
Q

Physical exam findings of allergic rhinitis

A
  • Allergic shiners
  • Dennie-Morgan lines
  • Transverse nasal crease + allergic salute
  • Allergic faces = high-arched palate, open, dental malocclusion
84
Q

What medications to avoid with hereditary angioedema?

A

No OCP or ACEi’s

85
Q

What test to do for hereditary angioedema?

A

C1 esterase inhibitor levels + function

86
Q

What is characteristic of a rituximab specific serum sickness reaction?

A

Palpable purpura along lateral aspect of feet

87
Q

Serum sickness management

A
  • Remove inciting agent
  • Supportive care
  • Antihistamines
  • NSAIDs
  • Analgesics
  • If severe = steroids
88
Q

What may you see on labs in serum sickness?

A
  • Thrombocytopenia
  • Proteinuria
  • Elevated CRP/ESR
  • Decreased C3/C4
89
Q

What PID do you think of with serratia?

A

CGD

90
Q

What PID do you think of with N.meng?

A

Complement

91
Q

What is the dx = hilar lymph nodes, eosinophilia, elevated Ca, elevated ALP?

A

Sarcoidosis

92
Q

What is the component that is affected in penicillin allergy?

A

Minor component

93
Q

What does a candida skin test do?

A

Test T-cell function

94
Q

What organ does chronic GVHD not affect (entirely…may be some unhappiness but no dysfunction)? …oh RC questions

A

Liver

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