Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

Online Med Ed - Pediatrics > Immunodeficiency > Flashcards

Immunodeficiency Flashcards

1
Q

B cell immunodeficiency dz

A
  • Brutons
  • IgA def
  • Hyper IgM
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

T cells immunodeficiency dz

A

digeorge

HIV( aquired)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

phagocytosis dz 3

A
  • chronic granulomatous dz
  • leukocyte adhesion def
  • chediac hidashi
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

3 dz that are from problem with both T and B cells

A

wiskott aldrich
Ataxic telectantasia
SCID mega aids w/o virus

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Complement dz

A

C1 esterase deficiency (edema)

C9 and C5 mac attack predispose to infxn

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

when think pt has a immuno deficiency?

A
  • FTT with diarrhea
  • recurrent infxn w/o immunity
  • severe infection when typically not severe
  • unusual pathogens
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

when do immuno deficiency dz show up?

A

after 6 months once mom’s antibodies wear off and baby isnt making their own

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

workup first two steps

A
  1. CBC with diff
  2. quantitative IgG level (show you G,A,M)
  3. then dz specific
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

big idea main immunodef treatment goals

A
  1. avoid antigens kid cant fight off

2. give them what they dont have

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Bruton’s aka

A

X linked A Ig -enemia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Brutons def

A

x linked dz
no immunoglobulins
B cells, Brutons, Boys

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

6 mo old sinopulmonary infections, boy

A

Brutons XLA

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Brutons workup

A
  1. CBC normal
  2. quant Ig: zero
  3. flow cytometry shows no B cells
  4. confirm dx with RTK gene
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

burton’s tx

A

schedule Iv IG treatments lifetime

  • or BM transplant yet usually too severe
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

combined variable immunodeficiency def

A

mild form of burtons

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

recurrent sinopulmonary dz in older kid… think?

A

CVID

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

CVID workup

A

CBC normal
QIG decrease in 2/3 of the Ig

less severe burtons

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

CVID tx

A

no BM transplant necessary

schedule IV Ig

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

IgA deficieny patho

A

low IgA cannot fight mucosal infections

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

sinopulmonary infxn
GI bugs
or asx till get blood transfusion then anaphylaxis

A

IgA deficiency

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

IgA dx

A

cbc normal

quant low IgA yet higher igM and IgG

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

IgA tx

A
  • no tx
  • watch out for anaphylaxis for blood transfusions
  • screen future blood transfusions and remove IgA (looks like a foreign protein to body = anaphylaxis)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

HyperIgM

A

B cell deficiency cannot convert IgM (nonspecific) to IgG (specific)

24
Q

hyper IgM workup

A

cbc normal

quant Ig: low IgA, low IgG, high IgM

25
Q

hyper IgM tx

A

none

26
Q

XLA vs CVID

A

young 6 mo vs older child / teen

27
Q

Digeorge syndrome caused by

A

22q11.2 deletion gene

problem of 3rd pharyngeal pouch

28
Q

Digeorge sx

A
  • wide spaced eyes
  • low set ears
  • absent thymic shadow on xray
  • small face
29
Q

digeorge common infxn

A

fungal infxn or PCP pneumonia

30
Q

Dx digeorge

A

clinical

Cbc: reduced absolute lymphocyte count

31
Q

Digeorge tx

A

tmp/smx prophylaxis PCP

IvIG bridge to thymic transplant

32
Q

digeorge f/u

A

watch out for hypocalcemia due to loss of PTH hormone

sx: seizure, tetany

33
Q

wiskott aldrich patho

A

x linked - boys

34
Q

ezema, low plts, normal infections boy

A

wiskott aldrich

35
Q

WA dx

A

CBC: low WBC, low plt

QIgG: big IgM and IgG

36
Q

WA tx

A

bone marrow transplant

37
Q

ataxia, telectantisia, ID, starts prior age 5

A

Ataxia telangectasia

38
Q

AT f/u

A

increase risk of:

  • leukemia
  • lymphoma
  • sensitive to radiation (including xrays)
39
Q

bubble boy

A

SCID

40
Q

SCID patho

A

no immune sx

  • no b cells
  • no t cells
  • def adenosine deoninate
41
Q

ID immediately post birth, any exposure = infxn

A

SCID

like mega aids but HIV negative

42
Q

SCID dx

A

CBC: low WBC
QIG: zero

43
Q

SCID tx

A

isolation
tmp/smx
BM transplant

44
Q

chronic granulomatis dz pathophys

A

no respiratory burst

macrophage problem ..can eat bacteria but cannot kill it if it is catalase +

45
Q

staph abscesses

A

think chronic granulomatis dz

46
Q

CGD dx

A

nitro blue
CBC: increased WBC
QIG: elevated IGM, IGG

47
Q

CGD tx

A

BMT

48
Q

leukocyte adhesion def

A

WBC cannot get out of the blood stream to get onto cell wall etc.

49
Q

high fever, high WBC yet no pus, delayed seperation of the cord

A

LAD

50
Q

LAD tx

A

bone marrow transplant

51
Q

CH

A

autosomal recessive dz

52
Q

giant granules in neutrophils PMN

A

dx

53
Q

CH associated with

A

neuropathy
albinoism
neutropenia

54
Q

complement

A

C1 esterase def

angioedema

55
Q

pt with angioedema h/o complement def

A

give FFP

56
Q

pt with neisseria

A

think C5-9 mac attack

Online Med Ed - Pediatrics (16 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions