Allergy and Immunology Flashcards
Type I reaction
Immediate
IGE mediated
Examples angioedema and anaphylaxis
Type 2
Cytotoxic
Antibody depended killing of a cell
Example auto immune hemolytic anemia
Type 3
Immune complex
Example serum sickness, lupus, post strep glomerular nephritis
Type 4
Cell mediated
T cells example dress Stevens-Johnson and intersitial nephritis
Serum sickness what type of reaction and labs?
Type 3 immune complex
Elevated inflammatory markers
Low complement
Low albumin
Proteinuria and hematuria
Serum sickness symptoms
Fever
Rash urticaria or erythema multiforme
Malaise
Arthlagias and arthritis
DRESS what is it and what type reaction?
Drug reaction with eosinophils and systemic systems
Type IV
Symptoms of Dress
What meds cause and viruses reactivate
Tx
Rash- morbilliform
Fever
Entire facial edema
LN
Multiple system involvement
+/- viral reactivating HHV 6, CMV, EBV
Minicycline
Need to treat with steroids
Brutons X linked Agammablobinemja
Early onset
Boys- X linked
No B cells- No IgG, IgA, IgM
No LN/tonsils
Hyper IgM
Boys, X linked
Only IgM
CVID
Diagnose >2 years old
B cells fail
recurrent infxns
Low IgG, low IgA
B cells low
Tx replace
SCID
T cells and B cells fail
Viral, fungal and bactreial
No CD4 cells
TREC abnormal, lymph subpop
Tx stem cell transplant
Job Syndrome
Hyper IgE
Abnormal coarse facies
eczema
cold abscesses
Wiskott Alrich
Low platets
eczema
encapulated bacterial infections
Ataxia-Telegiectasia
AR
recurrent infxns
elevated alpha 1 fetoprotein
Bloom syndrome- short statue
CGD
recurrent skin abcesses
+NBT
X linked
SJS- common meds and what type of reaction
Type VI
bactrim, phenytoin, NSAIDS, valproic acid
Shwachman-Diamond Syndrome
AR
Pancytopenia
FTT- short stature, bone abnormalities
Fat malabsoprtion- pancreatic insufficiency
Risk of leukemia
Fanconi anemia
Aplastic anemia- macrocytic anemia
Short stature
Absent thumbs
VACTERL
VACTRL
Vertebral
Anal atresia
Cardiac defects
TE fistula
Renal anomalies
Limb anomalies
Diamond Blackfin Anemia
Macrocytic anemia ONLY (no pancytopenia)
Microcephaly, hypertelorism, horseshoe kidney
AV septal defects
Blue sclera/cataracts
thumb abnormalies
Primary ciliary dyskineisa
Frequent mucopurluent sinus infections
Abnormal nitric oxide test
Galactosemia- symptoms
Metabolic decompensaton following lactose formula
Liver dysfunction, janudice, coagulopathy
Increased bilirubin, plasma amino acids, +urien reducing substances
Galactsoemia associated with?
E. coli sepsis and cataracts
Maple syrup urine disease- what amino acid decreased?
Alanine
Maple syrup urine disease what amion acid increased
Valine, Leucine, Isoleucine- Braanching ones;
Only amion acid diseae with hyperammonia
Maple syrup urine disease
Acidemias
Increase in AA
Increase in urine organic acids
Metabolic acidosis
Ketosis
Hyperammonia
Urea Cycle Defects
Finding which causes…
HYPERAMMONIA only
Hyyperventialation cause respiratory alklaosi
Lipid disorders
which is beta oxidation of FA and derangment of cartinine metabolism
Hypoketotic hypoglycemia with prolonged fasting
Labs to check in Lipid disorders
Urine ketones- low
Beta hydroxybutyrate- low
Blood glucose- low
Urea cycle tx
Sodium benzoate is a nitrogen scavengerse to help with nitrogen excretion
Duchenne Muscular Dystrophy
X linked recessive
Genetic testing
Elevated CK
