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Immunology Flashcards

1
Q

What is the triad for ataxia telangiectasia

A
  1. Telangiactasia - not present at birth
  2. progressive ataxia - wheelchair bound as teen
  3. B and T cell def
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2
Q

What are Dx criteria for AT

A

low Ig A and IgG2
elevated AFP
Def = sequecing the ATM gene

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3
Q

CP of familial Mediterranean fever

A

typical acute episode lasts 1-4 days;
fever and 1 or more symptoms of sterile peritonitis, manifested as abdominal pain (90%), arthritis or arthralgia (85%), or pleuritis manifested by chest pain (20%).

Other serosal tissues, such as the pericardium and tunica vaginalis testis (acute scrotum), are rarely affected.

Erysipelas-like rash, myalgia, splenomegaly, scrotal involvement in boys, neurologic involvement, Henoch-Schönlein purpura, and hypothyroidism are other, less common clinical manifestations.

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4
Q

what are the 2 types of CMPA

A

Ig E mediated:urticaria, angioedema, resp, GI features

non IgE - GI issues

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5
Q

who should not have soy formula?

A

prems

congenital hypothyroidism - ? inbiits thyroid peroxidase

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6
Q

what formula is recommended for CMPA

A

hydrolysed protein

not soy, hard to diff Ig E med vs not, non IgE medated high allergy to soy too

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7
Q

what might be the issues if child has recurrent sinopulmonary infections with encapsulated organisms?

A

antibody mediated immunity issues - they evade phagocytosis

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8
Q

what are encapsulated organisms

A 
SHINE SKis
Strep pneumo
H. Influenza
Neisseria meningitis
E. Coli

Salmonella
Klebsiella
GAS

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9
Q

FTT, diarrhea, malabsorption, and fungal infection - where is the issue

A

T cell immunodef

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10
Q

eczema, petechia, easy bruising, bleeding disorder?

A

Wiskott-Aldrich syndrome

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11
Q

what is the immunologic defect of SCID

A

NO T cell +/- B cells

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12
Q

how does agammablobulinemia present

A 
No B cells
No lymphoid tissue
get encapsulated bacteria infections
usually ok with Viruses except entero
present at 4-6 month when mat immunity i s gone
No live vaccines!
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13
Q

what are the most common B-cell diseases

A

X linked agammaglobulinemia
common variable immunodef
Hyper igM
Ig A def

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14
Q

what are features of common variable immunodef?

A

initially normal
gradual decrease in Ig
GI - malabsorption, diarrhoea, splenomegaly - Looks like IBD
ID - recurrent resp infect
AI - low plt, hemolytic anemia, neutropenia
liver dysfunction
High risk of LYMPHOMA - 400x inc

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15
Q

What are clinical features of IgA def

A

Can only Dx after 4 yr when level at adult level
recurrent sino-pulm infection
food allergies
AI disease
celiac disease
can become CVID
TRANSFUSION RISK - need IiA A poor blood bc might have some of their own

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16
Q

What are clinical features of SCID

A 
severe infection - viral, bacterial, fungal, protozoa
FTT
chronic diarrhea
Severe eczema
could present with a GVHD
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17
Q

what lab results would make you worry about SCID

A

lymphopenia

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18
Q

what are important management

A

No blood transfusion - need CMV-, irradiated

No live vaccines

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19
Q

when should you worry about a primary immunodeficiency?

A 
presence of 1-2 should raise suspicion:
>=4 new AOM per year
>= 2 sinus infec per year
>= 2 pneumonias in one year
>= 2 MONTHS on Abx with little effect
>= 2 deep seated infections
recurrent abscesses
persistent thrush
need for IV Abx to clear and infection
FTT
Family HX of PID
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20
Q

if a child has no tonsils and no LN, what should you think off?

A

agammaglobulinemia
SCID

NO lymphatic tissue anywhere

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21
Q

if someone is doing a nitroblue tetrazolium test or a neutrophil oxidative burst suppression test, what are they looking for?

A

chronic granulomatous disease

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22
Q

what test would you do to confirm a complement def?

A

CH 50 - classical pathway
AH 50- alternative
C3 and C4

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23
Q

what common PID had NO Ig?

24
Q

a pt with XLA is at risk of what type of infections?

A 
ENCAPSULATED!
ENTEROVIRUS
sinopulmonary
GI
sepsis
meningitis
25
how do we manage a pt with XLA
IV IG monthly monitor trough IgG regular PFT NO live vaccine
26
what PID has lymphatic tissue but does not make enough IG
CVID
27
what lab findings would you expect with CVID
"NORMAL" amount of B cells on flow cytometry Low Ig G but does not work well Low/N - Ig A and M Low or NO Ab to vaccines
28
what PID has high risk of developing autoimmune issues?
CVID | 20-25 %
29
what PID has high risk of malignancy
CVID High risk for lymphoma and gastric malignancies
30
what are the most common features of SCID
``` Infections of all types no lymphoid tissue no thymus oral thrush chronic diarrhea = FTT severe eczema ```
31
if you think a patient has SCID, what on initial investigations will help confirm your DDX
LOW lymphocytes | may have low Ig G and or IgM
32
how do we treat SCID?
``` Abx Iv Ig PJP prophylaxis avoid transfusion - can lead to GVHD - or CMV neg, irridiated Protective isolation NO live vaccine but killed are OK BONE MARROW TRANSPLANT! ```
33
what is the triad for Wiskott-Aldrich syndrome?
eczema low Plt recurrent pyogenic infections
34
what combined ID can present with bloody diarrhea and petechia?
WAS
35
what combinedID will have LOW Ig Mbut high Ig A and E
WAS
36
if a 2 year old presents with progressive ataxia and BW suggestive of lymphoma. Dx?
AT
37
what is the disease progression of AT
ataxia starts at 18 month telangiectasia starts at 2-4 yrs wheelchair by early teens
38
what is the mgnt of AT?
supportive only no role for BMT if have humural def - can do IV Ig
39
what are the clinical features of DiGeorge syndrome
``` Cardiac defect - interrupted aortic arch Abnormal facies Thymus hypoplasia Cleft palate - bifid uvula, high arch Hypocalcemia - parathyroid issue ```
40
what are the MC immune issues with DiGeorge syndrome?
Low T cell number and function but gets better over time NO live vaccine until T cell number normal if need transfusion - CMV neg, irr to avoid GVHD
41
what are 3 phagocytic defects?
CGD hyper Ig E LAD
42
what are 2 complement defects
Complement deficiencies | hereditary angioedema
43
if a patient presents with recurrent bacterial and fungal infections, what should you consider as Dx?
CGD
44
what bacteria are pt with CGD particularly at risk for?
catalase +,PLACES for CATS ``` Pseudomonas, Listeria, Aspergillus, Candida, E-coli, S. aureus, Serratia ```
45
what is the MC presentation of patient with CGD?
1. Cat + bacterial infection or fungal 2. abcesses and granulomas 3. IBD like symptoms
46
what is the defect in CGD
defect in NADPH oxidase which is required to kill certain bugs 65 % X linked rest is AR
47
how do we manage CGD?
antibacterial and antifungal prophylaxis | BMT****
48
patient presents with recurrent abscesses but they don't seem to hurt or turn red. Dx
Hyper IgE syndrome Neutrophils can't get to the site of infection therefore have COLD abscesses
49
what are CF of hyper IgE syndrome
``` coarse facial features delayed teeth shedding eczema cold boils bone fractures/scoliosis/jt hyperlaxity ```
50
if a baby has delayed cord separation, what investigation will help you make the Dx
a CBC with very HIGH Neutrophils if Neutrophils normal - Not LAD
51
patient presents with recurrent episodes of swelling of the hands and feet, especially after stress. It is non painful. investigations and Dx
1. C 1 esterase inhibitor def - AD | Dx- hereditary angioedema
52
how do you manage an attacke of hereditary angioedema
C1 esterase inhibitor infusion | Can use androgens for prophylaxis
53
what primary immonodef gets fungal infections
T cell defects: SCID, di george, AT, WAS + hyper Ig E + CGD
54
who will have no response to vaccines
CVID
55
who should not recieve live vaccines
Digeorge SCID Agammaglobulinemia
56
who will NOT benefit from a BMT
AT

Allergy & immunology (2 decks)

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