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2023 RC Blue's Clues > Allergy/Immuno > Flashcards

Allergy/Immuno Flashcards

1
Q

Number of infections concerning for IEI

A

Frequency red flags
4 new ear infections per year
2 serious sinus infectionsper year
2 pneumonia per year
2 serious deep seated systemic bacterial infections (total)

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2
Q

Loss of developmental milestones
Low IgA!

A

Ataxia telangectasia

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3
Q

Congenital cardiac disease
Hypocalcemia
Dysmorphic facies

A

DiGeorge

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4
Q

Wiskott Aldrich

A

Petechiae (thrombocytopenia), Bleeding
Eczema
high IgA and IgE
FTT

poor response to polysaccharide vaccines ex. pneumococcal

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5
Q

9 year old male with recurrent AOM, sinusitis, hearing loss. Has a history of hydrocephalus, and required CPAP at birth for respiratory distress. What is the most likely diagnosis?

A

primary ciliary dyskinesia

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6
Q

CVID

A

most common IEI
inability to produce specific antibodies - IgG MUST BE LOW
increased susceptibility to respiratory and GI infections (due to IgG and IgA deficiency)

Investigations show:
- Hypogammaglobulinemia with low serum levels of all class switched Ig’s (IgA, IgG, and IgE)
- Lack of vaccine titres despite vaccination
- Normal B/T cell numbers, but lack of class switched B cells

Tx: IVIG

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7
Q

HyperIgE Syndrome (Job Syndrome)

A

Triad: Recurrent staph skin abscesses, pneumonia, and very high IgE

“cold abscesses” - no fever

Other features:
Recurrent bacterial skin (staph boils) and pulmonary infections,
Chronic mucocutaneous candidiasis (non-invasive fungal infection),
very high serum IgE,
chronic eczematous dermatitis
Skeletal abnormalities (facial asymmetry, broad nose, deep set eyes, prominent forehead, scoliosis, delayed shedding of primary teeth)

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8
Q

primary ciliary dyskinesia

A

Autosomal recessive Genetic disorder resulting in either function of structure of cilia being compromised

Characterized by:
Recurrence U/LRTI
Bronchitis, recurrent rhinosinusitis,
frequent middle ear infections → hearing loss
Infertility
Aberrant organ laterality

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9
Q

CGD

A

x linked (males)

Presents with multiple abscesses and oral ulcers.
you have neutrophils but they don’t work (the neutrophils go to site of infection and just sit there creating an abscess)

At risk for Catalase positive organisms (staph aureus, serratia, pseudomonas, norcadia) and encapsulated organisms (Pseudomonas, Streptococcus pneumoniae, Haemophilus Influenzae type B, Neisseria meningitidis, Escherichia coli, Salmonella, Klebsiells, and group B Strep)

aspergillus is classic fungal infections
lymph nodes present

neutrophil oxidative burst (or DHR - dihyroxy rutabine)

Tx:
- Antibiotic prophylaxis
- HSCT is curative

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10
Q

Types of Immune reactions

A

Type 1 - IgE
Type 2 - Cytotoxic
Type 3 - Immune Complex
Type 4 - t cell mediated

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11
Q

Diagnosis of amoxicillin allergy

A

drug challenge test

skin testing for IgE poor predictive value

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12
Q

Ataxia Telangiectasia

A
  • ataxia (progressive) & telangiectasia (around age 3-6) & frequent infections
  • autosomal recessive; problems with DNA repair
  • combined cellular and humoral imunodef –> recurrent sinopulm infections (most commonly absent IgA)
  • high serum AFP
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13
Q

Bad reaction to live vaccines

A

T cell problem
–> look at lymphocytes

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14
Q

Description of a child with multiple abscesses, including lymph nodes and hepatic with serratia. which immunodeficiency?

A

CGD

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15
Q

Oral food allergy

A
  • class 2 food allergen
  • foods are cross reactive with pollens
  • symptoms with raw food only (can tolerate cooked/processed foods)
  • oropharyngeal symptoms only
  • anaphylaxis is rare
  • do a skin prick test for pollen
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16
Q

SCID

A

combined immunodef - both B and T cell function
x linked (male)
recurrent bacterial, viral and fungal infxn (opportunistic infxn - PJP)
usually presents early

Classics
- bad candida
- diarrhea
- FTT
- no lymph tissue (LNs or tonsils)
- no thymus on CXR

Labs
- CBC: lymphopenia
- flow cytometry: low t cells, now B cells
- abnormal mitogen stimulation assay (function of lymphocytes t cells)
- on NMS
CXR: no thymus

No live vaccines (ex. rota, MMRV)

17
Q

Hereditary Angioedema

A
  • autosomal dominant
  • low function of C1 inhibitor leads to increased bradykinin production –> smooth muscle relaxation –> edema
  • be cautious of laryngeal edema!!!
  • don’t respond to epi, antihistamines or glucocorticoids
  • often worsened in trauma, stress, menstruation, ACEi

Test:
- C4 will be low
- low C1 esterase inhibitor level and function

Treat:
- C1 INH or Bradykinin type 2 receptor antagonist

18
Q

Serum sickness like reaction

A

type 3 hypersensitivity
Don’t form immune complex in serum sickness LIKE (you do form complexes in serum sickness)
Common triggers: amoxicillin, septra, vaccines)

Symptoms:
7-12 days post exposure
- rash
- fever
- arthralgias
- edema

Tx:
- stop the offending agent
- analgesis, NSAIDs, and antihistamine
- if severe, give steroids

19
Q

10 signs of immunodeficiency

A
  • 4+ ear infxn / yr
  • 2+ sinus infxn / yr
  • 2+ pneumonia / yr
  • 2+ deep infxn/serious bacterial infxn
  • 2+ months of abx with little effect
  • Recurrent deep skin or organ abscesses
  • Persistent fungal infxn (skin, thrush)
  • IV abx
  • FTT
  • Family hx of primary immunodef
20
Q

allergy testing

A

skin prick testing or specific IgE blood test (Aka RAST)

DONT test IgG

21
Q

anaphylaxis epi dosing

A

0.01mg/kg of 1:1000 (1mg/ml) Intramuscular

22
Q

tryptase level in anaphylaxis

A

should be elevated
if doing labs, send tryptase for patient with first episode of anaphyalxis from the ED

23
Q

serum sickness like reactions

A

fever, rash and polyarthritis
typically sulfa drugs
less migratory compared to urticaria
usually 5-10 days after exposure
no mucous membrane involvement

management: stop the agent and do not rechallenge

24
Q

skeeter syndrome

A

exquisite sensitivity to mosquito bites
bites can become inflamed or infected
worse at onset of season
responds to antihistamines

25
Q

what is the most effective use for allergy immunotherapy?

A

bee stings

26
Q

management FPIES

A

acute:
- ondans
- volume resus

Long term
- strict food avoidance

ex. stem child eats rice cereal for first time

27
Q

confirming diagnosis of food allergy syndrome

A

skin prick testing to pollen
not life threatening
don’t need epi pen

28
Q

poison ivy management

A

high potency topical steroids or po steroids if really bad
antihistamines not helpful

29
Q

10 yo girl w/abdominal pain, u/s shows edema of the sigmoid colon & moderate free fluid in lower quadrants. On history, has had intermittent facial & left arm swelling over the last 5 years. Which test would reveal the diagnosis?

A

c1 esterase inhibitor level

30
Q

9 year old male with recurrent AOM, sinusitis, hearing loss. Has a history of hydrocephalus, and required CPAP at birth for respiratory distress. What is the most likely diagnosis?

A

PCD
- hydrocephalus
- CPAP at birth (?TTN)
- hearing loss
- sinopulm infxn

31
Q

A 6-year-old girl has a history of frequent infections. She also is off balance when walking and has skin telangiectasias. What is the most likely finding?

A

low IgA and high AFP

ataxia telangectasia
T cell disorder

classic triad:
Progressive cerebellar ataxia
Oculocutaneous telangiectasias
Recurrent sinopulmonary infections (immunodeficiency)

32
Q

A new refugee with adenitis that grew S. aureus. You find out on history that he also had adenitis after BCG. What is the test to reveal the diagnosis?

A

infxn after vaccine - think T

T cell defect
- testing: lymphocytes (low), flow cytometry for t cells

33
Q

4 year old with Staph aureus pneumonia, lung abscess, stomatitis, impetigo on the face and mouth ulcers. Which PID and how do you test

A

CGD
granulocyte disorder
oxidative burst / DHR / BHT

34
Q

autoimmune polyglandular syndrome type 1

A

t cells attack endocrine glands
- parathyroid
- adrenals
- gonads

severe cutaneous candidiasis

35
Q

is recurrent AOM in PCD or CF?

A

PCD

36
Q

definitive management of SCID

A

preferably HLA matched sibling stem cell transplant
if no sibling, can use HLA matched unrelated donor

2023 RC Blue's Clues (20 decks)

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