Brainscape's Knowledge GenomeTM

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


See full index

Block 6 Week 1 > Hogan- Primary immunodeficiency Diseases > Flashcards

Hogan- Primary immunodeficiency Diseases Flashcards

1
Q

What is the most likely failure of immunity?
What about second most likely?
Least likely?

A

Antibody defect (B cell)
T cell disorders
innate and complements

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

Leukocyte development has passed from (blank) to bone marrow BEFORE birth

A

liver/spleen

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

Is phagocyte functionality complete in neonates?

A

yes

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

Is complement function available to neonates?

A

yes via maternal passive transfer of IgG

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

(Blank) will lage until antibody production is initiated.

A

Antibody mediated complement fixation (self)

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

Is NK function available in neonates?

A

yes

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

When is T cell function complete?

A

at birth :)

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

T cell maturation is required to complete B cell maturation to functional antibody function. What does this mean?

A

It means that T cell function is available at infancy, but B cell function lags for 6-24 months. (i.e if T cells to develop, B cell functionality will suffer)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Autoimmune problems in patient/family members suggest some difficulty with (blank) at possibly a genetic level.

A

B cell maturation/or tolerance issues

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

Does immunodeficiency develop before or after autoimmunity?

A

either

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

Most immune defenses are (blank) in nature as a significant number of autoimmue issues are failure of B cell maturation/isotope switiching/ or tolerance

A

humoral

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

What are some severe infections of the innate immunity?

A

lymphadenitis, osteomyelitis, pneumonia, and sepsis

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

Are phagocytes nondiscriminatory? WHat does this mean

A

yes

they attack both gram + and gram - and yeast/fungal organisms are attacked

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

When you have a catalase producing organism, what kind of disorder should you be thinking about?

A

CGD (neutrophil disorder)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

What are some CGD organisms?

A 
staph aureus
pseudomona aeroginosa
aspergillus fumigatus
candida
enterbacteriiaceae
nocardia/listeria
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

What are common problems in B cell disorders?

A

otitis media
sinusitis
pulmonary infections

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

What organisms are associated with B cell disorders?

A

moraxella
haemophilus influenzae
strep pneumoniae

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

When is getting otitis media worrisome?

A

after middle school

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

When you get an infection from a rar or odd organisms, what kind of problem should you suspect?

A

T cell

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

What are some of the weird organisms associated with T cell problems?

A

pneumocystis carinii
candida: invasive (lung/esophagus)
system viral illness (CMV etc.)
mycobacterial infections: systemic

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

What are the clinical characteristics of T cell deficiency?

A 
Family history
onset before 6 months
opportunistic infection
no lymph nodes
cutaneous lesions
increased chance of cancer
failure to thrive
GvH disease after transfusion
Severe fungal/viral infection
fatality after BCG
diarrhea
hepatosplenomegaly
22
Q

What are the clinical characteristics of B cell deficiency?

A 
Famiy history (autoimmune/immunodeficiency)
onset after 6 months
recurrent virulent bacterial infections
allergy/autoimmune disease
vaccine failure
sinopulmonary infections
failure to thrive
23
Q

What are the clinical characteristics of phagocytic deficiencies?

A

severity range mild to severe
susceptible to low grade bacteria, fungus
severe infections (pneumonia/osteomyelitis)
skin infections/ furunculosis
lymphadenitis
abscesses
delayed separation of the umbilical cord

24
Q

What complement will be deficient if you get recurrent bacterial infections?

A

C3

25
Q

What complement will be deficient if you have nesseria infections?

A

C5-C8

26
Q

What disease has neutrophils that have azurophilic granules?

A

chediak higashi

27
Q

What disease has bi-lobed nucleus in the neutrophils?

A

specific granule deficiency

28
Q

If you have neutrophilia what should you consider?

A

LAD d/o or infection

29
Q

If you have neutropenia what should you consider?

A

congenital absence, autoantibody, cyclic neutropenia

30
Q

If you have small platelets, and decreased number of platelets what should you consider?

A

WAS

Wiskott aldrich syndrome

31
Q

If you have an RBC abnormality what should you consider?

A

autoimmune anemia and associated G6PD deficiency

32
Q

If you have lymphopenia what should you consider?

A

SCID in an infant

33
Q

If you have a genetic deficiency somewhere in the complement cascade what will the CH50 be? how else can you get this number

A

zero

profound sepsis with DIC: consumptive and will take 4-6 weeks to replenish cascade

34
Q

If you have a genetic deficiency somewhere in the complement cascade what wil the AH50 be?

A

zero

could be consumption

35
Q

If you AH50 and CH50 are both zero. where could you deficiency be?

A

C3 or C5-9 because these are shared by both assays

36
Q

What are the 5 immunology labs you want to order?

A 
IgG
IgA
IgM
IgE
Albumin
37
Q

What does albumin tell you?

A

it determines if IgG loss is secondary

38
Q

What IgA level is considered good?

A

any number greater than botom of measurable assay is good despite statistical reporting.

39
Q

If a child has nasal polyposis what should you look for?

A

CF test

40
Q

Before you look for a B cell deficiency in chidren, what 2 things might you look into?

A

nasal polyposis

immotile cilia syndrome

41
Q

What can functional responses to vaccines tell you?

A

can tell you what antibodies arent being produced.

I.e if you respond to protein components but not polysaccharide componenets etc.

42
Q

If you have IgM, what does this tell you?

A

that you have titer function

43
Q

If you have failure in a vaccine you can do an advance test which is what?

A

its a booster dose that will check for memory cel function and plasma cell production.

44
Q

If you give an advanced test and al the Igs are decreased what should you be thinking?

A

CD3, CD4, CD8, CD19, CD56

ie check the CDs

45
Q

If CD19 (which is B cells) is low, what should you be thinking is the culprit?

A

XLA

if all Bs and Cs are messed up think SCID

46
Q

If you are suspecting T cell disorder, what should you look into?

A

HIV (secondary immunodeficiency)

CD3 CD4 CD8 CD19 (B cell), CD56 (NK cells)

47
Q

What are some ways you can check T cell function?

A

lymphocyte stimulation with mitogens
specific antigen studies (PPD if BCG vaccinated)
TREC analysis of thymic output
FISH: 22q11 (DiGeorge)

48
Q

What is the sailboat that you see on a neonatal CXR?

A

the thymus :)

49
Q

If your suspicious of a phagocyte disorder what do you look up?

A 
CD11/18 flow cytometry
serial neutrophil counts
G6PD
MPO
Bactericidal assay (function)
IgE
50
Q

When does pneumonia in childhood become a red flag?

A

the third !!!

51
Q

If someone says they had pneumonia what do you want to ask?

A

was there a chest xray done

52
Q

There are 14 serotype pneumococcal titers and (blank) out of 14 is considered normal

A

8

Block 6 Week 1 (13 decks)

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