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Terms in this set (109)
Both NARES and allergic rhinitis have eosinophils on nasal smear. How do you tell them apart?Nares: negative skin test and non elevated serum IgE
Allergic rhinitis: positive skin test and elevated serum IgEKids with SCID usually present by this age?3 monthsWhat diagnosis would give you hypogammaglobulinemia and lymphopenia?SCIDWhat cell line(s) are affected in SCID?B and T cell linesBest treatment for complete digeorge syndromeThymidine transplantMost SCID inheritance is this type?X linked6 frequent presenting symptoms with SCIDAcute Ottis media
Dermatitis
Diarrhea
FTT
Thrush
Life threatening infections4 face abnormalities with digeorgeLow ears
Smooth philtrim
Micrognathia
HypertelorismDigeorge syndrome results from defects in which pharyngeal pouches?3rd and 4thProblems associated with deletions of chromosome 22?Cardiac
Abnormal facies/atresia of esophagus
Thymic hypoplasia/thyroid
Cleft palate
Hypocalcemia
22What immune alteration do you see with ataxia telangiectasia?Low IgA and IgMConsider THIS with a patient with recurrent bacterial infections, eczema, and petechiaeWiskott-Aldrich syndromeWhat immunodeficiency will cause no lymphoid tissue to develop?SCIDWhat immune cell dysfunction usually presents with recurrent Phoenicians infections?B cell disordersWhat cell type dysfunction usually presents with chronic or recurrent candida?T cell dysfunctionName the 2 primary in vitro allergy tests?RAST
ELISAWhat's the most sensitive and cheapest allergy testing method?Skin testingAn IgE mediated allergy to penicillin will present withing how long?24 hours of administration
Otherwise it is non IgE mediated Nd May simply be a viral ex anthemWhat allergy test is not affected by antihistamines?RASTWhat should you do if a 3-4 ,onto old gets urticaria after exposure to cows milk?Go to the allergistWhat should you do if a 3-4 month old child develops eczema after exposure to cows milk?Remove the offending agent (T cell mediated, not IgE)How long after a Hymenoptera sting do you need to wait before doing allergy testing4-6 weeksWhich type of allergy is IgE mediated?Type 1 hypersensitivity
Worst comes first (anaphylaxis)Name 5 types of type 1 hypersensitivity reactions?Anaphylaxis
UrticariaFood allergy
Stings
LatexHow are type 2 hypersensitivity reactions mediated?Antibody mediated (b is "2")Name the most common type of type 2 hypersensitivity reaction?Hemolytic anemiaWhat's the mechanism of hoe 3 sensitivity reactions?Immune complex (c is 3)Name a type of type 3 hypersensitivity reactionSerum sicknessWhat's the mechanism of oen4 hypersensitivity reactions?Delayed type (slowest is 4)Name one type 4 hypersensitivityPoison ivyHow effective is venom immunotherapy?98% effective at preventing future reactionsHow do you differentiate local Hymenoptera sting reaction from a surrounding cellulitis?Surrounding redness won't be tender with a sting, and the patient won't be febrile.What is the risk for future anaphylaxis if a patient has a significant local reaction to a sting?5-10%A child with a life threatening. Reaction to a bee sting should subsequently get THIS (aside from and Epi pen)Venom immunotherapyMost common cause of chronic urticaria?Food!Should patients with chronic urticaria get allergy testing?No!Allergy immunotherapy may take this long to produce symptom relief?2 years!At what weight do you graduate from 0.15mg Epi to 0.3mg?30kgHow far out can you experience late phase anaphylaxis symptoms?24 hoursVasodilation
Vascular leak
Smooth muscle spasm
Are all parts of which phase of anaphylaxis?Initial responseMucosal edema
Mucus secretion
WBC infiltrates
Epithelial damage
Bronchodpasm
Are all parts of which phase of anaphylaxisLate5 most common food allergies in PedsCows milk
Eggs
Peanuts
Tree nuts
Soy
6th is wheatWhich food allergies are usually outgrown by 5 years old?Wheat
Milk
Egg
SoyAllergy to which 3 foods are usually not outgrown?Peanuts
Tree nuts
SeafoodWhat medication has peanut in it?Atrovent!Name the four types of rhinitisAllergic
Infective
Vasomotor
Rhinitis medicomentosa (rebound)Top 4 causes of anaphylaxis in PedsFood
Stings
Vaccines
MedicationsThe cutaneous manifestation of mastocytosis is urticaria pigmentosa. What does that look like?Red brown macules and a positive darier sign (urticaria on stroking)What is the mechanism behind contrast allergy?Osmolality/hypertonicity reaction triggering direct degranulation of mast cells and basophils.
It is NOT IgE mediated!Is pretreatment against contrast allergy with Benadryl and/or steroids effective?Yes!What age group does transient hypogammaglobulinemia of infancy affect?6 months to 3-6 yearsWhat labs are low In Transient hypogammaglobulinemia of infancy?Low IgG and IgAName 6 humoral immune deficienciesBrutons disease
CVID
X linked hyper IgM syndrome
Iga deficiency
Job syndrome (hyper IgE)
Transient hypogammaglobulinemia of infancyCGD usually presents before what age?5 yearsBest test for CGDNitroblue tetrazolium testWhat does the NBT test measure?Phagocytize oxidase activityWhat type of bacteria can't CGD pts kill?Catalase positive organismsCGD inheritance:2/3 x linked
1/3 a rThis disorder has delayed healing and doesn't make pusL A DThis disorder is most commonly associated with umbilical cord separation after 45 daysL A DTreatment for CGDAggressive antibiotic use3 innate immunodeficienciesCgd
LAD
Complement deficiencyTreatment for L A DBMT is curativeWorst two complement deficienciesC5 def and C9 def
Recurrent or overwhelming meningococcemiaAll complement deficiencies are autosomal recessive except THISProperdin deficiency, which is x linked recessiveWhat immune test should you get if a person gets recurrent serious bacterial infectionsCH50What test(s) can be used to test for cell mediated immunity defects associated with t cell dysfunctionTB or candida skin testTest of choice for LADRoebuck skin windowThis test evaluates the humoral systemIg levelsThis defect usually presents with recurrent "less serious" infectionsB cell (humoral) defectsWhat should you order if you really suspect humoral defects but the IgG levels are normal?Specific immune antibodies (tetanus, rubella, pneumococcus)Which immune deficiency is associated with skeletal abnormalities and fracturesJob syndromeBest treatment for job syndrome (hyper IgE syndrome)Abx
Steroids5 findings with job syndromeEosinophilia
Eczema and skin infections
Elevated IgE
Sink pulmonary infections
Coarse faciesMost humoral immunity defects present in what age range6 months to 1 yearIn what immunodeficiency is the treatment immunoglobulin replacement?Hyper IgM syndromePatients with THIS are exceptionally susceptible to coldsIga deficiency
But it is usually asymptomatic!What test do you do for SCID?Fluorometric analysis of b t and nk cellsTreatment for SCIDSupportive early on, then bmtWhat do you worry about if a kid with SCID gets varicella?PoliomyelitisWhat us the cellular defect in Wiskott-Aldrich syndrome?Actin filament assembly problemWhat ig is low in Wiskott-Aldrich syndrome?IgMWhat is the inheritance pattern of Wiskott-Aldrich syndrome?X linkedThis immune disorder has a tendency to develop hematologist malignancies and autoimmune diseases?Wiskott-Aldrich syndrome.2 treatment options for WAS?BMT or cord blood transplant
Monthly ivigName 4 disorders of cellular immunitySCID
WAS
A.T.
Digeorge syndromeWhat is the mechanism of the disorder with hyper IgM syndrome?Absent CD-40 ligand in T cells disrupts B cell differentiationWhat diagnosis should you consider if a patient gets recurrent:
Otitis
Sinopulmonary infections
PCP PNA
diarrhea
Opportunistic infectionsHyper IgM syndromeThese two immune disorders increase the risk of malignancy if you get ebv infectionWAS
CVIDPatients with these two disorders need to get icing regularly to prevent recurrent bacterial infectionsCVID
Brutons diseaseWhat's the most common primary immunodeficiencyCVIDWhat's the mechanism of CVID?Defective antibody response (B cells don't become plasma cells so they don't make the right amount of immunoglobulins - there is some but not enough)Rheumatoid arthritis
Cytopenias
Thyroid disease
These are associated with this immunodeficiency?CVIDWhat's the diagnostic test for Brutons?IgG levels first
If all levels are low, get B and T cell subsetsThis x linked disease makes you more likely to die if infected with EBVDuncan's disease
Primary infection is 80% fatalMechanism of disease with Duncan's diseaseUncontrolled proliferation of T cells during infectionMost common presentation of Brutons disease?Recurrent infections with encapsulated organisms (pseudomonas, strep pneumoniae, h flu)What age do most Brutons patients present at?Less than 1 year but after 6 months.What is the gene associated with Brutons?BLK gene
X linked
1/2