Allergy and Immunology - 2019 Updated! Flashcards
1
Q
What are clinical findings of acute GVHD?
A
Within 3 months of transplant
Skin (rash), GI (anorexia, D&V), liver (transaminitis, hyperbili)
Prevent with immunosuppression (cyclosporine, tacrolimus), treat with steroids
2
Q
What are clinical findings of chronic GVHD?
A
Occurs more then 3 months post transplant
Skin (malar rash, sica syndrome), MSK (arthritis, joint contractures), lung (bronchiolitis obliterans), liver (cholestasis, bile duct degeneration), eyes (dry eyes, conjunctivitis)
3
Q
An 18 month old boy has had 3 pneumonias, 2 episodes of OM and now presents with purulent nasal
discharge. On examination he has a tender 1x1.5cm cervical node with overlying redness. His mother
states that he had an episode of varicella a month ago that was fairly typical in course. Which of the
following investigations will most likely lead to the diagnosis:
a. ADA level
b. NBT
c. Immunoglobulins
A
c. Immunoglobulins
PID: recurrent sinopulmonary infections suspicious for innate issues (complement defect), adaptive immunity (B cell defect).
Ix: B cells: look at immunoglobulins and antibody titres
- complement (C3, C4, CH50)
ALSO, CVID and ataxia-telangiectasia both present with recurrent sinopulmonary infections and low immunoglobulins
4
Q
- Which allergy can be treated with immunotherapy:
a) food allergy
b) bee sting allergy
c) penicillin allergy
d) atopic dermatitis
e) cholinergic urticarial
A
b) bee sting allergy
a) food allergy is now also correct (peanuts)
Allergies that respond to immunotherapy:
- seasonal or perennial allergic rhinoconjunctivitis
- asthma triggered by allergen exposures
- insect venom sensitivity
5
Q
- IVIG can be used in all of the following EXCEPT:
a) immune thrombocytopenic purpura
b) bone marrow transplant
c) nephrotic syndrome
d) Kawasaki disease
e) Guillain-Barré syndrome
A
c) nephrotic syndrome (you’ll just pee it out)
Can be used in: ITP, bone marrow transplant (patients exposed to measles), KD, Guillain-Barre
6
Q
- A child has received IVIG in the course of their treatment. How long should you wait before giving
them vaccines to ensure adequate response:
a. 1 mo
b. 3 mo
c. 6 mo
d. 11 mo
A
d. 11 mo
Varies between 3-11 months depending on product and dose used
7
Q
- Which is true regarding RAST:
a) It is more sensitive than skin testing (similar)
b) It is affected by corticosteroids
c) false positives occur in children with hyper-IgE states
d) Preferred test for food allergies
Concept: comparison of RAST to skin testing
A
c. false positives in children with hyper-IgE states
RAST = testing for allergen specific IgE (now more commonly called allergy specific IgE or sIgE test)
some facts: less sensitive than skin testing, not preferred method for food allergies (skin testing is), BUT not affected by corticosteroids or antihistamines, while skin testing is (neither are affected by montelukast)
8
Q
13mo child with diaper rash and diarrhea with the introduction of new foods over the
past couple months. Which of the following is most consistent with this presentation:
a) arrange for skin testing and RAST
b) it is due to sensitization to cow’s milk protein
c) carbohydrate intolerance is a common cause
d) citrus fruits and tomatoes can cause this via an immune mediated mechanism
A
c. carbohydrate intolerance is a common cause
- Sucrase-Isomaltase Deficiency- high amongst Canadian natives- when feeding milk and lactose- no symptoms- once exposed to sucrose and starches (juice, fruits,
crackers, other starches) develop diarrhea, diaper rash, FTT, abdominal distensionavoid
sucrose and maltose- Rx sacrosidase (Sucraid) - They might be describing toddler’s diarrhea where high amounts of osmotically active carbohydrates (ie. Juice) lead to diarrhea. This could cause diarrhea, which would lead to diaper rash.
- no role for RAST in diagnosis because this is only good for suspected IgE mediated CMPI
9
Q
5 year old had a severe reaction to peanut at 1 year of age. He also has asthma. Now, allergist has ordered an IgE level for peanut. Mom is wondering why. What do you tell them?
- The IgE level will determine how allergic to peanuts she is
- She will not have to go off of her daily montelukast
- This test negates the risk of possible anaphylaxis
- This test is more sensitive than skin testing
A
- it prevents systemic reactions that could result from the skin test
10
Q
Kid to have RAST for possible peanut allergy: 2 advantages over skin test; 1 disadvantage compared to
skin test.
A
advantages: ● Better safety ● Results are not influenced by skin disease or medications disadvantages: ● less sensitive
11
Q
Recurrent meningococcal meningitis. Most common immunodeficiency is:
A
a. C5 deficiency
Consider complement deficiency if:
o Recurrent angioedema
o Autoimmune disease (SLE, nephritis, HUS, partial lipdystrophy) - C1, C3, C4
o Recurrent pyogenic infections
o Disseminated meningococcal or gonococcal infection - C5-9
o 2 episodes of bacteremia - C2
o Meningitis with uncommon serotype (other than A, B or C)
12
Q
You suspect a 6 year old of having a T-cell defect. The best screen is:
a. Schick test
b. TB skin test
c. Candida skin test
d. CD4 count
e. gamma-globulin electrophoresis
A
c. Candida skin test
Screening tests for T cell defect: lymphocyte count, CXR for thymus size, delayed skin tests (candida, tetanus toxoid)
13
Q
Deficiency of which of the following may lead to anaphylaxis in a patient given IVIG:
a. IgA
b. IgD
c. IgG
d. IgE
e. IgM
A
a. IgA
44% have antibodies to IgA (which if they are IgE mediated can cause anaphylaxis to blood products including IVIG)
14
Q
In which condition is the IgE level normal:
a) Wiskott-Aldrich syndrome
b) immune thrombocytopenic purpura
c) selective IgA deficiency
d) Kawasaki disease
e) ascariasis
A
b) immune thrombocytopenic purpura
15
Q
What is the expected IgE level in Wiskott-Aldrich?
A
high
16
Q
What is the expected IgE level in selective IgA deficiency?
A
low
17
Q
What is the expected IgE level in Kawasaki?
A
high
18
Q
A 6 year old presents with eczema, decreased platelets and recurrent OM and pneumonias. Increased IgA and IgE. Decreased IgG. Your diagnosis is:
a. Wiskott-Aldrich
b. SCID
c. IgA deficiency
d. atopic dermatitis
e. normal
A
a. Wiskott-Aldrich
It’s a TIE! (TCP, immunodeficiency, eczema)
- Low T cell percentages
- treat with IVIG, killed vaccines, BMT curative
Mnemonic: IgG&M low, IgA&E high - it’s a tie
19
Q
3 year old with a history of fevers. Occur every 4 to 12 weeks for 1-4 days. Growing well. Treated for numerous otitis and pharyngitis. a) Viral illnesses b) FMF c) CVID
A
A) multiple viral infections
20
Q
A 15-year-old boy has had 5 episodes of documented pneumonia, 3 RML, 1 RLL, and 1 LLL.
What investigation would be most helpful:
a) immunoglobulins with IgG subclasses
b) pulmonary function tests-
c) CT chest
d) TB skin test
e) sputum culture
A
a) immunoglobulins with IgG subclasses
X-linked agammaglobulinemia versus CVID: first have small or no tonsils and lymph nodes, second have normal tonsils and nodes
21
Q
What is true about chronic granulomatous disease:
a) all girls with the disease must be 45 XO karyotype
b) suppurative lymphadenopathy is a characteristic feature
c) there are decreased lymphocytes in 90% of cases
d) there are a decreased number of absolute neutrophils
e) rarely present before 5 years of age
A
b) suppurative lymphadenopathy is a characteristic feature
Normal number of neutrophils but abnormal function (can eat catalase producing bugs but not kill them)
- features include pneumonia, lymphadenitis, abscesses, onset in infancy
- infections with staph, gram negative enterics, candida, aspergillus
- X Linked Recessive or AR
Dx: flow cytometry and genetic testing (used to use nitroblue tetrazolium)
22
Q
- In chronic granulomatous disease, all of the following features are present EXCEPT:
a) usually present in males
b) leukocytosis
c) lymphadenopathy
d) hepatomegaly
e) hypogammaglobulinemia
A
e) hypogammaglobulinemia - no, can have hypergammaglobulinemia
23
Q
A 6mos male has several abscesses (S. aureus) now and in the past. He has also had recurrent
respiratory infections and Serratia UTI. Now has butt abscess. Which diagnosis is most likely?
CGD
Wiskott-Aldrich
CVID
Hypogammoglobinemia
A
c. CGD
Common bugs of CGD:
- staph aureus most common
- serratia marcescens
- B cepacia
- aspergillus
- candida albicans
- nocardia
- salmonella
- mycobacterium
24
Q
Which of the following is true in Wiskott-Aldrich Syndrome?
a) autosomal recessive inheritance
b) poor response to protein antigens
c) decreased IgG and IgE
d) increased risk of malignancy
e) leukopenia
A
d) increased risk of malignancy
25
Which immune deficiencies are associated with increased risk of leukaemia and lymphoma?
Wiskott-Aldrich, SCID, CVID, X-linked lymphoproliferative syndrome - all are associated with defects in immune surveillance
26
Which of the following is true of patients with X-linked agammaglobulinemia?
a. respond to protein antigens, but not to polysaccharide ones
b. should not get live vaccines
c. prone to PCP as infants
d. Risk of lymphoma
e. Risk of EBV associated diseases
b. should not get live vaccines
All immunoglobulins are low; they have no circulating B cells
27
31. Which lab abnormality is seen in Ataxia-telangiectasia?
decreased IgA
decreased IgE
increased IgG
a. decreased IgA (selective absence of IgA in 50-80% of patients), IgE and IgG can also be low
28
18 mo M with knee arthritis, aspirate grew N. meningitidis. This is the same organism that grew in his
CSF when he had meningitis at 10 months. Which of the following tests is most likely to be abnormal in
this child?
a) Immunoglobulins
b) C3,C4, total hemolytic complement
c) Lymphocyte differentiation
b. C3,C4, total hemolytic complement
C3, C4 - assesses number
CH50 - assesses classical pathway function
AH50 - assesses alternate pathway function
29
39. A 4 month old baby presents with eczema, recurrent pneumonias and absent thymus shadow on CXR.
What is the most likely diagnosis?
a) SCID
All SCIDs lack T cells, some also have no B or NK cells
- lack all Ig classes and subclasses
- extreme susceptibility to bacterial, viral and parasitic infections
- DO NOT give live vaccines
30
44. What is true for X linked agammaglobulinemia?
a. respond to protein antigens (no they do not)
b. no live vaccines
c. at risk for PCP (rare)
d. risk of lymphoma, LIP (no lymph tissue)
e. risk of EBV associated diseases (EBV infect B cell thus immune)
b. no live vaccines
Risk for paralytic polio
31
S. aureus. What finding would confirm your diagnosis?
a. absent radius
b. no LNs/tonsils
c. draining ears
d. hypoplastic patellae
c. draining ears
| Wiskott-Aldrich
32
Patients with hyperIgE syndrome are classically infected with which bacteria:
● Recurrent severe staphylococcal abscesses of skin, lungs, other + elevated serum IgE
33
```
10 year old Lebanese girl with recurrent self limited fevers and abdominal pains. Her albumin is low, and her AST and ALT are slightly elevated (ie: 60-80 range). The remainder of investigations are negative (they don’t tell you what those investigations are!). What is the most likely
diagnosis?
a. Familial Mediterranean Fever
b. SLE
c. Cyclic Neutropenia
```
a. Familial Mediterranean Fever (more in keeping w/ abdo pain + fever)
- fever for 1-2 days every 1-2 months + serositis, arthritis, erysipeloid rash (over dorsum of foot)
o ESR, CRP, fibrinogen, serum AA (amyloid A) often increased
34
What are some diagnostic criteria for SLE?
```
SOAP BRAIN MD
serositis (pleuritis, pericarditis)
oral ulcers
arthritis
photosensitivity
blood low (anemia, TCP, leukopenia)
renal - protein loss
ANA positive
Immune markers (dsDNA) positive
Neuro (psych, seizures)
Malar or discoid rash
```
35
You are seeing a 14 month old boy with a history of severe atopic dermatitis and frequent infections.
On his CBC you find that he has a platelet count of 80.
What is the most likely diagnosis?
Wiskott Aldrich Syndrome
36
6. After how many invasive infections would you be concerned about an immunodeficiency?
Nelson’s- 1 o r more systemic (sepsis, meningitis) bacterial infections; 2 or more serious or
documented bacterial infections in 1 year.
37
b.) List 4 investigations for work-up of a patient with suspected immunodeficiency
1. CBC + Diff
2. Immunoglobulins
3. Vaccine Titres
4. Flow cytometry
Note:
- normal ESR rules out chronic bacterial or fungal infection
- normal neuts rules out congenital and acquired neutropenias and leukocyte adhesion defects
- normal lymphs rules out severe T cell defect
- normal platelet size and count rules out Wiskott
38
6 week old baby with erythema and induration around his umbilical cord which is still firmly attached.
What underlying condition should you be suspicious of?
LAD I – (Leukocyte Adhesion defect type I)
- recurrent bacterial and fungal infections, despite lots of neutrophils
- note: more than 3 weeks is considered delayed separation of umbilical cord,but 10% of normal infants take >3 weeks
- diagnosis: flow cytometry
39
Name two organ systems and two organisms that are typically affected by granulocyte defects.
Skin (skin & soft tissue infections) and lungs (pneumonia)
Staphylococcus spp, Serratia marcescens , Klebsiella spp, other Gram negative organisms
(Granulocytes are the precursor to neutrophils, eosinophils & basophils)
40
Kid has had several infections. What other 4 findings would suggest immunodeficiency?
Failure to thrive
Family history of primary immune deficiency
Need for IV antibiotics to clear infections
Two or more months on antibiotics with little effect
Persistent thrush
41
Description of a child who is having multiple episodes of otitis media and pneumonia. Lab results given are IgG 8(normal), IgM normal and IgA 0.01(decreased).
What important piece of advice would you warn
this child about? (and what do they have?)
Selective IgA deficiency
Anaphylaxis to blood transfusions → Serum antibodies to IgA are reported in as many as 44%
of patients with selective IgA deficiency
42
1. Which is true regarding allergic rhinitis:
a. indoor mould is not a trigger
b. long term follow up shows that symptoms persist into adulthood
c. these children go on to develop asthma
d. ingested substances are more significant than inhaled substances in perennial rhinitis
e. it always starts less than 5 years of age
b. long term follow up shows that symptoms persist into adulthood
o Rare if <1 year (need two seasons of exposure)
43
15. The best therapy for perennial allergic rhinitis:
a) avoid exposure to allergens
b) intranasal corticosteroids
c) oral antihistamines
d) oral corticosteroids
e) sodium cromolyn
As PER HAMILTON -> Intranasal steroids
This is a shit question Nasal steroids also very effective
44
5. Boy with runny nose and itchy eyes in summertime. List 3 signs on physical exam that you’d see with this diagnosis.
b. ) Initial treatment x1
Allergic rhinitis
1. allergic shiners
2. nasal crease
3. pale boggy nasal turbinates
Also:
allergic conjunctivitis
dry skin (xerosis)
keratosis pilaris
Tx: avoidance of trigger, antihistamines, inhaled steroids
45
Name 2 advantages of second generation anti-histamines
● Typically lipophobic – therefore less likely to have CNS effects
o i.e. less sedation, better school outcomes in children
● Quick onset of action (w/in 1 hr) and peak levels w/in 2-3 hrs
● Longer acting and less frequency
46
6 year old Mom concerned about chronic nasal congestions.
What 4 things on history would suggest allergic rhinitis?
3 Physical finding which support allergic rhinitis.
Hx: ● Seasonal appearance of symptoms
● Specific environmental triggers
● Positive family history - risk of allergic disease in a child approaches 50% when one parent is allergic and 66% when both parents are allergic.
● History of particular maneuvers: rubbing eyes, nose, allergic cluck
```
PE:
● Nasal crease
● Pale to purple/blue mucosa
● Thin, clear nasal secretions
● Cheilitis or dry lips → mouth breathing
Allergic shiners
```
47
A child is brought to the emergency room after being stung by a bee. He is wheezing
and moderately tachypneic and tachycardic. BP=100/70. First step in management:
a. 0.01mg/kg SC epinephrine
b. 0.01 mg/kg IV epinephrine
c. oxygen
d. methylprednisolone
e. Benadryl
c. oxygen
Definitely to also need to give epi but IM
O2 should be given to all patients with suspected anaphylaxis
48
A child who is known to be allergic to peanuts presents to emergency after having eaten some 30
minutes ago. He is very itchy and has hives all over his body. His vitals including BP are stable and
there is no wheezing. Which of the following is correct?
a) IV epinephrine would be the preferred medication
b) Benadryl can be given IV, IM or PO
c) ventolin and Pulmicort should be administered
d) hydrocortisone does not prevent the late onset effects
e) desensitization therapy should be undertaken
b) Benadryl can be given IV, IM or PO
49
A possible anaphylactic reaction. Where will you give it:
a. SC in the abdomen
b. IM
c. SC in the thigh
d. IV
b. IM
50
Child with signs of anaphylaxis after the transition to cow milk formula.
a) Most important medication in the immediate management and route?
b) When can the patient go home (i.e. how long does she need to be observed)?
c) List 4 preventative recommendations for the non acute management.
a) IM Epinephrine
b) More than 90% of biphasic responses occur within 4 hr, so patients should be observed
for at least 4 hr before being discharged from the ED.
c) 1. Allergen avoidance
2. Education of symptoms of anaphylaxis
3. Carrying epinephrine (an EpiPen)
4. Written emergency plan should be prepared ( www.foodallergy.org )
51
Kid ate Chinese food at birthday party. Since then has had 2 weeks of angioedema, urticaria. Rx? (and diagnosis?)
IM epi
subcut epi
po steroids
po antihistamine
b. Antihistamine (second generation like cetirizine best option)
If H1 antihistamine does not achieve sufficient control, then can add H2 blocker (ranitidine)
Prednisone for severe angioedema or unresponsive to H1 and H2 blockers
acute urticaria (<6 weeks) - wheals or angioedema
52
Causes of acute urticaria?
```
● Acute < 6 weeks
o Food
o Meds
o Insect Stings
o Infection
o Contact allergy
o Transfusion Reaction
```
53
Causes of chronic urticaria?
```
● Chronic > 6 weeks
o Idiopathic (2/3 of cases)
o Physical (1/3 of cases)
o AI (Thyroid, JIA, SLE, Celiac etc.)
o Periodic fever syndromes
o Neoplastic
```
54
Differential diagnosis of urticaria? (important stuff not to miss)
● KEY DDX: Erythema Multiforme (targetoid, central necrosis or vesicles, duration> 7 d on palms
and soles ++, no dermographism, MM can be involved)
● Key DDX: utrticaria pigmentosa (type of mastocytosis; reddish brown macules that may wheal
when stroked)
55
Child has recurrent episodes of face and ear swelling. Father has a similar history. This has stopped in the past four weeks as he has stopped drinking milk and eating cheese. He still eats ice cream. The rash is not itchy. What is the diagnosis?
a) acute intermittent urticarial (just means < 6 weeks and NYD)
b) chronic urticarial (just means > 6 weeks)
c) cow’s milk protein allergy ( but has ice cream)
d) C1 esterase deficiency
d) C1 esterase deficiency (aka hereditary angioedema)
C1 esterase inhibits the complement system to prevent spontaneous activation -without it get complement activation and leaky blood vessels causing edema
Tx: replace C1 esterase
56
4. Which is true regarding penicillin anaphylaxis
a. it is mediated by IgG against a major determinant
b. it is mediated by IgG against a minor determinant
c. it is mediated by IgE against a minor determinant
d. it is mediated by IgE against a major determinant
e. it is mediated by IgM against a major determinant
d. it is mediated by IgE against a major determinant
57
13 month old with diarrhea and diaper rash what is cause?
a. cow milk protein allergy
b. carbohydrate intolerance
c. immunodeficiency
b. carbohydrate intolerance
58
Investigations for carbohydrate malabsorption (12 month old with diarrhea and diaper rash)
● TESTING
o Maldigested carb emptied into colon = osmotic effect
o + colonic bacteria ferment these carb= gas + acids
o Testing therefore = stool pH < 5.5, hydrogen breath test (+)
59
4. Picture of a 7 year old boy’s thigh, linear vesicles with some excoriation. He comes to your office, in
July, with a rash on his left thigh, It is itchy and has seemed to spread where he scratches.
a)What is your diagnosis
b)what is your treatment
● Washing the skin and other items that may have come in contact with the plant
● If patient develops skin lesions, systemic antihistamines are usually needed.
● Mild to moderate strength (class 7 to 4) topical steroids are appropriate.
● Topical pramoxine (an antihistamine) is safe and can be used over large areas of the body 2–3
times a day
● If lesions are more extensive, systemic steroid therapy for 2 weeks at a beginning dose of
0.5–1.0 mg/kg, followed by a rapid taper over the subsequent 2 weeks.
60
Neutropenic child with central line site red.
| Which antibiotics?
● Anti-pseudomonal beta-lactam: cefepime, ceftazidime, meropenem or pip-tazo
● Anti staph: vancomycin
61
A mother has come in with her 12 month old, and is concerned by the “number of shots” that is getting today. She has read in the paper about how is it bad to expose a child to a large number of “antigens” all at once, and is worried about overwhelming his immune system.
What advice can you give to help
alleviate this motherʼs worries? (2 points)
- Children are often exposed to more antigens from routine daily activities as compared to the routine vaccine schedule
- Few are aware of Canada’s robust vaccine safety system or that vaccines are held to a higher safety standard than drugs.
- Vaccines are scheduled to be given before children are at high risk of getting an infection.
- Combination vaccines help reduce pain and discomfort associated with vaccines.
- We give more vaccines at once but with better technology there are fewer antigens used than before
-
62
What are THREE indications for giving the conjugated quadrivalent vaccine for meningococcus?
Risk increased because of underlying medical conditions
● Asplenia or functional asplenia, including those with sickle cell anemia
● Properdin, factor D or complement deficiency (including those with acquired complement
deficiency from eculizumab (Soliris); primary antibody deficiency)
● HIV
Risk increased because of the potential for exposure
● Travellers to endemic areas (currently, travellers to sub-Saharan Africa and Hajj pilgrims)
● Close contacts of a case of invasive meningococcal disease
63
Child with egg allergy, what do you tell her about the flu shot (list 3)
- It is safe to give your child the flu shot
- The amount of ovalbumin in influenza vaccines is a/w low risk of adverse events
- Standard monitoring of child after vaccines
- If adverse rxn was to occur, health care provider giving vaccine could administer EPI
- There is no need for vaccine skin testing
- They can have the full dose
64
Child with anaphylaxis, weight is 25kg.
a) What dose epi autoinjector will you give him:
b) Where is the best place to inject the epi in anaphylaxis:
a) I would give him a regular epipen (0.3mg) but technically he could still use the Junior.
b) Intramuscular - mid-outer part of the thigh, hold for at least 3 seconds (ideally 10)
EpiPen (Adult) - has 0.3 mg per dose -> Good for children >25 kg
EpiPen Jr - has 0.15mg per dose - > Good for kids 15 to 30 kg as per manufacture guidelines
65
Dose of EpiPen Jr and EpiPen Adult
Adult 0.3 mg (for >25 kg)
| Junior 0.15 mg (for 15-30 kg)
66
Two reasons to do a RAST over a skin test?
Cannot take off antihistamines for some reason
Terrible eczema
Dermatographia
?high risk of anaphylaxis
67
A mother is concerned about the possibility of future development of food allergies in her infant. Name four pieces of advice you would give her.
- do not restrict mom’s diet when pregnant or lactating; ok to eat milk, egg, peanut
- exclusive Breastfeed the first 4-6 months
- if need to use formula, consider hydrolyzed cow milk formula - to prevent development of FA in AT RISK babies
- do not delay introducing solids to the baby beyond 4 - 6 months old, allergenic foods may be introduced at this time
- Soy milk is NOT recommended over Cows milk as a strategy to prevent FA
- Once tolerates a food, keep it in diet regularly
68
2 things to prevent infections in patients with SCID ?
- Mom should have recommended booster vaccines prior to delivery
- Protective Isolation
- Prophylaxis prior to SCT - TMP/SMX, fluconazole, synagis, acyclovir (if mom had herpes),
- Immune globulin
- Give vaccines but No Live vaccines
- Irradiate blood products (this is also to prevent GVH), leukodepleted and CMV negative
- Transplant
- Might not breastfeed if mom has CMV IgG or IgM
- Health care providers should have up to date vaccines
- Immunization of Care providers (esp VZV, MMR, Influenza)
- Health care facilities should have infection control and prevention policies'
- Hand hygiene
69
Child post bone marrow transplant. Significant exposure to varicella.
a) What therapy do you give him?
b) What is the time frame for this therapy to be effective?
c) How long do you isolate this patient from other immunocompromised patients?
What therapy do you give him?
VZIG
What is the time frame for this therapy to be effective?
Should give immediately (within 96 hours) but can give within 10 days
How long do you isolate this patient from other immunocompromised patients?
From days 8- 21
70
Oral thrush not responding to topical Rx … what is the Tx and the associated conditions?
Fluconazole 6mg/kg day 1, followed by 3mg/kg/day for 7-14 days if responsive.
Culture the lesions if not responding (see chart in Candida infections in children)
- (SCID) B cell, t cell defects,Hyper IgE
71
4 vaccines to avoid in T cell ID? Which vaccines are CI to a household member of immunocompromised patient? Which vaccines are CI to be given to a pregnant women? CI in immunocompromised patients? CI to vaccination in patient with egg allergy?
Tcell Dysfunction: All live attenuated vaccines are contraindicated - MMR, Flumist, Rotavirus, varicella, shingles, Yellow fever, typhoid
Household members: Oral polio, live attenuated influenza - avoid close contact due to theoretical risk of transmission, small pox
These vaccines you want: Non-immunized close contacts of immunocompromised people should be immunized against pertussis, Hib, rotavirus, pneumococcus, measles, mumps, rubella, varicella, zoster and influenza as appropriate for age. Non-immune household or close contacts of immunocompromised people should be given hepatitis B vaccine.
Pregnant - Avoid live vaccines
Immunocompromised - Depends on the immunodef, can get killed vaccines, depends
72
Minimal observation time following systemic anaphylactic reaction?
4 - 6 hours
73
What is the treatment for chronic urticaria who failed H1 antihistamines?
a) H2 antihistamine
b) Steroids
a) H2 antihistamines
2014 American guidelines recommend as an adjunct if failed normal-dose H1 antihistamines. Studies show modest benefit. 2018 international guidelines recommend going to 4x-dose H1 antihistamines and then omalizumab.
74
Child with eczema and recurrent pneumonia. Hepatomegaly, petechiae, otitis media and low platelets. What do you expect?
a. elevated IgA and IgE
b. Immune response to polysaccharide vaccine
c. oral Candida
d. Abnormal mitogen proliferation
a. Elevated IgA and IgE
75
2-week old child with a 1 week history of stool mixed with blood in an infant? Bottlefeeding well, passed stool in first 24 hours. Most likely dx?
a. anal fissure
b. cow milk protein allergy enterocolitis
c. Meckel’s diverticulum
b. cow milk protein allergy enterocolitis
76
Which of the following is the most helpful measure to decrease risk of asthma?
a. dust mite covers
b. elimination of environmental smoke exposure
c. removing pets from the home
d. breastfeeding
b. elimination of environmental smoke exposure
77
Eczema, thrombocytopenia, multiple infections.
What would you look for to make the diagnosis?
Wistcott Aldrich - Xlinked, can see small platelets on smear “microthrombocytes”, low IgM and IgG but high IgE, low CD8
WAS gene
78
A child has recurrent infections and a liver abscess. He grew Serratia. What is the diagnosis?
a) CGD
b) Wiskott-Aldrich
c) CVID
d) Hypogammoglobinemia
CGD
79
A child has tingling sensation after eating fruits with peels, but does not have the same problem once the fruit is cooked. What test will help you with the diagnosis?
- RAST for specific IgE
- Skin prick testing for pollens
- Oral challenge
- Serum tryptase
Skin prick testing for pollens
80
A girl presents with abdominal pain and hives. On abdominal ultrasound, there is thickened colon with some free fluid. She tells you she has had previous episodes of facial swelling and hives, which resolve on their own. Which test will give you the diagnosis?
a) C1-Esterase
b) RAST
c) skin testing
d) C3 and C4
a) C1- Esterase
Hereditary Angioedema:
- Inherited autosomal dominant
- Low functional levels of plasma protein C1 inhibitor
- Cutaneous non-pitting and non-pruritic edema NOT associated with urticaria most common
- Swelling occurs over 1.5 days then resolves over same time
- Often preceded by rash (erythema marginatum) that is erythematous, NOT RAISED, NOT PRURITIC
- Second symptom - attacks of severe abdo pain, can look like and acute abdomen caused by edema of mucosa of any portion of GI tract
- Often have prodrome of tightness and tingling
Provocation:
- Dental work w/ Novocain injection
- Trauma
- Emotional Stress
- ACE-I (inhibit degradation of braykinin making the diesease worse)
- Estrogens
- Menstruation
Tx:
- Doesn’t respond well to Epi, Antihistamines or Glucocorticoids
- Purified C1-INH for proph - Cinryze
- Gonadotropin inhibitor (Danzol)
- Weak Androgens (but ++ SE like premature closure of epiphyses)
- Fibrinolysis inhibitor ε-aminocaproic acid but also ++ SE
81
In the setting of anaphylaxis, the epinephrine concentration and route to be used is:
1: 1000 IV
1: 1000 IM
1: 10000 IV
1: 10000 IM
1:1000 IM
82
When can you introduce egg to diet. Child’s sibling had eczema at 6 months of age.
a) Not until 12 months
b) Introduce yolk only at 6 months
c) Introduce white only at 6 months
d) Introduce whole egg at 6 months.
d) Introduce whole egg at 6 mos
83
Boy with egg allergy. What to do about MMR vaccine?
a) Give it in office
b) Allergy consult
c) Delay?
a) Give it in office
Best answer would be just to give it like any other child.
84
in what instances is RAST better than SPT?
a) not affected by medication
b) cheaper
c) more sensitive
A) Not affected by medication
*We dont do RAST anymore (that’s an outdated term): now use fluorescence enzyme labeled assays
You can still be on antihistamines and do sIgE
Also consider when pt has extensive dermatitis or dermatographism
sIgE have comparable sensitivity to SPTs
85
3 year old kid with multiple recurrent sinopulmonary infections. One pneumonia with lung abscess cultured Strep pneumo. Previous episode of stomatitis with secondary facial cellulitis. History of recurrent oral ulcers. Concern with what part of the immune system?
T cell
B cell
Granulocytes
complements
Granulocytes
86
8 year old boy presents with itchy mouth and angioedema of lips and tongue after eating an apple. He is otherwise well. What do you do?
- Recommend avoiding raw apples.
- Recommend avoiding raw apples and prescribe EpiPen autoinjector.
- Recommend avoiding raw and cooked apples.
- Recommend avoiding all raw fruit.
- Recommend avoiding raw apples.
| Oral Allergy Syndrome
Only give epi pen if RF
87
A child is admitted to hospital with pneumonia, previously on Ceftriaxone. Culture grew ampicillin-sensitive gram positive organism. Switched to IV Ampicillin, then suddenly develops urticarial rash, swelling and lethargy.
Vitals: HR 160, sBP 60. In addition to giving a fluid bolus, that is the most appropriate next step in management?
Methylpred 30mg/kg IV
Diphenhydramine 1.25mg/kg IV
Epi 0.01mg/kg IV of 1:10,000 (IM not an option!!!)
Dopamine 10mcg/kg/min
?diphenhydramine (Benadryl can be given IM/IV/PO) -- different iteration of other crappy question
don't give epic IM
88
2 year old recently admitted with Staph aureus lung abscess. Has a history of recurrent sinusitis, mucositis and recurrent skin/soft tissue infections. Which immunologic function is affected?
T-cells
B-cells
Granulocytes
Complement
Granulocytes
89
Child is admitted with strep pneumo bacteremia and started on ampicillin. Shortly after receiving antibiotics has decreased LOC and urticaria. Tachycardic and hypotensive. What would you do?
- IV epinephrine (**it really said IV, not IM!)
- IV benadryl
- Normal saline bolus
- Oral cetirazine
Bolus (out of these options)
90
Child with signs of anaphylaxis after the transition to cow milk formula.
A) Most important medication in the immediate management and route?
B) When can the patient go home (i.e. how long does she need to be observed)?
C) List 4 preventative recommendations for the non acute management.
a) IM epinephrine, 1:1000 0.01mg/kg (max 0.5mg)
b) 4- 6 hours
c) - allergen avoidance
- epi-pen junior for home
- can prescribe 3 day course of antihistamines (cetirizine and ranitidine) and PO corticosteroids for ‘added benefit in resolution of symptoms’ CPS statement
- medic alert bracelet
- referral to allergist
- provide information on allergies/anaphylaxis
91
Picture of a 7 year old boy’s thigh, linear vesicles with some excoriation. He comes to your office, in July, with a rash on his left thigh, It is itchy and has seemed to spread where he scratches.
What does he have?
Name one treatment you can give.
a) Poison Ivy
b)
Wash without scrubbing, remove affected clothes
Topical lotions like calamine
Oatmeal baths, cool compresses
High potentency topical steroids
Systemic corticosteroids for several weeks (don't give short course = rebound)
Antihistamines not helpful unless sedating
92
5. Boy with runny nose and itchy eyes in summertime.
a. ) List 3 signs on physical exam that you’d see with this diagnosis
b. ) Initial treatment x1
a)
- epistaxis from frequent picking of the nose
- allergic salute
- frequent nose blowing or sniffing
- nasal crease
- decreased sense of smell on neurological exam (lol)
- mouth breathing ‘the allergic gape’
- chapped lips
- dental malocclusion
- allergic shiners (venous stasis)
- conjunctival edema, tearing and hyperemia
- clear nasal secretions
- edematous, boggy, and bluish mucus membranes with minimal erythema on nasal exam
- swollen turbinates
b) Avoid allergen exposure
Oral antihistamines or Nasal antihistamines
Nasal corticosteroid
93
Kid with perennial allergic rhinitis - 4 environmental avoidance factors (Q1)
→ ie. list 4 (or 5) avoidant strategies for child with perennial allergic rhinitis
Reduce dust mites :
- put bedding in airtight, allergen impermeable covers
- wash bedding weekly at >130F
- replace wall-to-wall carpets
- replace curtains with blinds
- reduce indoor humidity
- minimize clutter
Animal Dander:
- avoid furred pets
- keep animals out of bedroom (?? probably not going to help enough)
Mold:
- avoid high humidity in bedroom
- HEPA filters
- Repair water leaks
- Check basement /crawl space for standing water
Pollen:
- Windows closed
- AC in car
- HEPA filter
- Restrict camping/hiking/leaf raking
94
In asthmatic youth, give 3 risk factors that will lead to complications during anesthesia.
Overall, poor asthma control: Including frequent asthma symptoms, frequent use of bronchodilators, and recent respiratory infections.
95
Description of a young boy with asthma. He has an elevated IgE level and worsening disease. What is his most likely diagnosis?
? Hyper IgE : recurrent skin infections, pulmonary infections, eczema, high IgE. JOB syndrome: coarse facial features, failure to shed primary teeth, fractures, hyper mobile joints, scoliosis.
? Allergic asthma. Most will have IgE in 300s.
? If SEVERE asthma - think allergic bronchopulmonary aspergillosis. IgE > 1000 and sensitization to Apergillus, eosinophilia CXR opacities.
Treat with 3-6 mos steroids and 4 mos itoa/voriconazole.
96
What is Heiner syndrome?
Food hypersensitivity pulmonary disease or Food induced pulmonary hemosiderosis
Sx: Cough, recurrent fevers, wheeze, hemoptysis, FTT, dyspnea, colic, anorexia, vomiting, diarrhea, hematochezia
Cow’s milk protein most common cause, but also egg and pork reported
97
What are the 2 most common allergies in infants?
Cow's milk
| Egg
98
A mom asks about OTC cough and cold remedies for her child who has an URTI. Do you recommend this? Why or why not?
Not recommended. Multiple adverse effects from combination products (Ex. Drowsiness). Potential for unintentional overdose, and many ED visits and deaths have been reported from their use. Recommend Honey, NSAIDs and fluid intake.
