Allergy and Immunology

Question 1

Type I reaction

Answer 1

Immediate

IGE mediated

Examples angioedema and anaphylaxis

Question 2

Type 2

Answer 2

Cytotoxic

Antibody depended killing of a cell

Example auto immune hemolytic anemia

Question 3

Type 3

Answer 3

Immune complex

Example serum sickness, lupus, post strep glomerular nephritis

Question 4

Type 4

Answer 4

Cell mediated

T cells example dress Stevens-Johnson and intersitial nephritis

Question 5

Serum sickness what type of reaction and labs?

Answer 5

Type 3 immune complex
Elevated inflammatory markers
Low complement
Low albumin
Proteinuria and hematuria

Question 6

Serum sickness symptoms

Answer 6

Fever
Rash urticaria or erythema multiforme
Malaise
Arthlagias and arthritis

Question 7

DRESS what is it and what type reaction?

Answer 7

Drug reaction with eosinophils and systemic systems
Type IV

Question 8

Symptoms of Dress
What meds cause and viruses reactivate
Tx

Answer 8

Rash- morbilliform
Fever
Entire facial edema
LN
Multiple system involvement
+/- viral reactivating HHV 6, CMV, EBV
Minicycline
Need to treat with steroids

Question 9

Brutons X linked Agammablobinemja

Answer 9

Early onset
Boys- X linked
No B cells- No IgG, IgA, IgM
No LN/tonsils

Question 10

Hyper IgM

Answer 10

Boys, X linked
Only IgM

Question 11

CVID

Answer 11

Diagnose >2 years old
B cells fail
recurrent infxns
Low IgG, low IgA
B cells low
Tx replace

Question 12

SCID

Answer 12

T cells and B cells fail
Viral, fungal and bactreial
No CD4 cells
TREC abnormal, lymph subpop
Tx stem cell transplant

Question 13

Job Syndrome

Answer 13

Hyper IgE
Abnormal coarse facies
eczema
cold abscesses

Question 14

Wiskott Alrich

Answer 14

Low platets
eczema
encapulated bacterial infections

Question 15

Ataxia-Telegiectasia

Answer 15

AR
recurrent infxns
elevated alpha 1 fetoprotein
Bloom syndrome- short statue

Question 16

CGD

Answer 16

recurrent skin abcesses
+NBT
X linked

Question 17

SJS- common meds and what type of reaction

Answer 17

Type VI
bactrim, phenytoin, NSAIDS, valproic acid

Question 18

Shwachman-Diamond Syndrome

Answer 18

AR
Pancytopenia
FTT- short stature, bone abnormalities
Fat malabsoprtion- pancreatic insufficiency
Risk of leukemia

Question 19

Fanconi anemia

Answer 19

Aplastic anemia- macrocytic anemia
Short stature
Absent thumbs
VACTERL

Question 20

VACTRL

Answer 20

Vertebral
Anal atresia
Cardiac defects
TE fistula
Renal anomalies
Limb anomalies

Question 21

Diamond Blackfin Anemia

Answer 21

Macrocytic anemia ONLY (no pancytopenia)
Microcephaly, hypertelorism, horseshoe kidney
AV septal defects
Blue sclera/cataracts
thumb abnormalies

Question 22

Primary ciliary dyskineisa

Answer 22

Frequent mucopurluent sinus infections
Abnormal nitric oxide test

Question 23

Galactosemia- symptoms

Answer 23

Metabolic decompensaton following lactose formula
Liver dysfunction, janudice, coagulopathy
Increased bilirubin, plasma amino acids, +urien reducing substances

Question 24

Galactsoemia associated with?

Answer 24

E. coli sepsis and cataracts

Question 25

Maple syrup urine disease- what amino acid decreased?

Answer 25

Alanine

Question 26

Maple syrup urine disease what amion acid increased

Answer 26

Valine, Leucine, Isoleucine- Braanching ones;

Question 27

Only amion acid diseae with hyperammonia

Answer 27

Maple syrup urine disease

Question 28

Acidemias

Answer 28

Increase in AA
Increase in urine organic acids
Metabolic acidosis
Ketosis
Hyperammonia

Question 29

Urea Cycle Defects

Finding which causes…

Answer 29

HYPERAMMONIA only

Hyyperventialation cause respiratory alklaosi

Question 30

Lipid disorders
which is beta oxidation of FA and derangment of cartinine metabolism

Answer 30

Hypoketotic hypoglycemia with prolonged fasting

Question 31

Labs to check in Lipid disorders

Answer 31

Urine ketones- low
Beta hydroxybutyrate- low
Blood glucose- low

Question 32

Urea cycle tx

Answer 32

Sodium benzoate is a nitrogen scavengerse to help with nitrogen excretion

Question 33

Duchenne Muscular Dystrophy

Answer 33

X linked recessive
Genetic testing
Elevated CK