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Pediatrics- Noelle > Immunology > Flashcards

Immunology Flashcards

1
Q

List 10 warning signs of primary immunodeficiency

A

4 or more new ear infections in one year
2 or more serious sinus infections in one year
2 or more months on antibiotics with little effect
2 or more pneumonias in one year
Failure of an infant to gain weight or grow normally **
recurrent deep skin or organ abscesses
persistent thrush in mouth or fungal infection on skin
need for IV antibiotics to clear infection *
two or more deep seated infections including septicaemia
a family history of primary immunodeficiency
*** means the top 3 warning signs of PID

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2
Q

what cells are associated with humoral assessment

A

CD 19

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3
Q

what cells are associated with t cell

A

CD4 (helper T cells)

CD8 (cytotoxic T cells)

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4
Q

what cells are measurements of adhesion markers

A

CD11

CD18

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5
Q

Name 2 humoral immune deficiencies

A

x- linked agammaglobulinemia (XLA)

common variable immune deficiency (CVID)

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6
Q

what mutation is associated with x-linked agammaglobulinemia

A

BTK

mutation in bruton tyrosine kinase

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7
Q

what is an important clue on physical exam for x-linked agammaglobulinemia

A

absent lymphoid tissue (no lymph nodes or tonsils)

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8
Q

what types of infections are associated with x-linked agammaglobulinemia

A

recurrent sinopulmonary infections
encapsulated bacteria (strep pneumonia, h influenza)
enterovirus meningoencephalitis

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9
Q

what are the laboratory findings associated with x-linked agammaglobulinemia

A

no immunoglobulins
absent B cells (CD19)
no antibodies to vaccines

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10
Q

what is the treatment for x-linked agammaglobulinemia

A

IVIG- IV monthly or SC weekly
400-600mg/kg IV monthly
Monitor trough IgG levels to maintain above lower limit of normal for age

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11
Q

what investigations need to be done for someone with agammaglobulinemia

A

CT chest, monitor PFTs- risk of bronchiectasis

genetic testing for BTK mutation

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12
Q

List 6 combined immune deficiencies

A 
SCID
Ataxia Telangiectasia
DiGeorge
Wiskott Aldrich
Hyper IgM
Cartilage Hair hypoplasia
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13
Q

When does severe combined immunodeficiency present

A

2-6 months

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14
Q

what is the most common cause of SCID

A

mutation in common γ chain of IL2 receptor on X chromosome (X-linked SCID)

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15
Q

what are the laboratory features of SCID

A 
lymphopenia
Severely reduced T cell numbers
B and NK cell numbers can be low, normal or elevated
low or absent T cell function
absent antibodies to vaccines
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16
Q

what is the treatment for SCID

A 
HSCT
gene therapy (ADA deficiency)
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17
Q

what is the triad for wiskott Aldrich syndrome?

A

thrombocytopenia
eczema
recurrent pyogenic infections

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18
Q

wiskott Aldrich is associated with increased risk of which malignancies

A

B cell lymphoma

leukaemia

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19
Q

what are the laboratory findings for wiskott aldrich

A 
thrombocyopenia
small platelets
*high IgA
*high IgE
variable IgG
low IgM
can have lymphopenia
decreased T cell function
poor antibody responses to some vaccines
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20
Q

how do you manage someone with wiskott Aldrich?

A

IgG replacement
PJP prophylaxis
HSCT
monitor for autoimmunity/malignancy

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21
Q

what types of infections are associated with wiskott aldrich

A 
encapsulated bacteria (Recurrent otitis media, Pneumonia, Skin infections)
opportunistic infections- PJP
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22
Q

What is the most appropriate screening test for ataxia telangiectasia?

A

alpha-fetoprotein

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23
Q

what is the mutation associated with AT

A

ATM mutation

autosomal recessive

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24
Q

what are the features of AT

A
  1. ataxia (cerebellar)
  2. progressive neurodegeneration
  3. telangiectasia- age 4-5, prominent on face, conjunctiva and ears
25
Q

ataxic child with lymphoma= what?

A

ataxia telangiectasia until proven otherwise

26
Q

children with AT should avoid what medical treatment

A

radiation (CT, Xray)

abnormal DNA repair, cells have increased sensitivity to irradiation

27
Q

what are the laboratory features of AT

A

increased alphafetoprotein
absent IgA in 80%

Decreased T cell numbers
Low or normal IgG
Sometimes elevated IgM
Decreased T cell function
Can have poor antibody responses to some vaccines
28
Q

what is the treatment for AT

A

supportive care- HSCT does not tx progressive neurodegeneration
IVIG if necessary
avoid radiation

29
Q

what should you recommend for the parents of a child with AT

A

genetic testing

ATM gene carriers have increased risk of breast and gi ca

30
Q

what does CATCH 22 stand for

A

C- cardiac
A-abnormal facial features
T- thyme hypopasia
C- cleft palate and midline abnormalities
H- hypocalcemia
22- 22q11 deletion (3rd &4th pharyngeal pouch)

31
Q

what are the laboratory features associated with DGS

A

mildly decreased CD4 and CD 8 cells
hypocalcemia
abnormal T cell function
may have abnormal antibody response to some vaccines (uncommon)

32
Q

what is the inheritance pattern of DGS

A

autosomal dominant

remember to screen the parents!

33
Q

list 3 phagocytic deficiencies

A
  1. Hyper IgE (Job’s)
  2. chronic granulomatous disease (CGD)
  3. leukocyte adhesion defects (LAD)
34
Q

what is the defect associated with chronic granulomatous disease?

A

defect in NADPH oxidase

35
Q

Patients with CGD are susceptible to catalase positive pathogens. List the catalase positive pathogens (6)

A 
S. aureus**
Aspergillus** 
Nocardia
Serratia marcescens
Burkholderia cepacia
Salmonella

** 2 most common

36
Q

inflammatory bowel disease is associated with what primary immunodeficiency

A

up to 40% of x linked chronic granulomatous disease

37
Q

what is the laboratory finding associated with chronic granulomatous disease

A

normal or elevated neutrophils

abnormal neutrophil oxidate burst index (NOBI)

38
Q

what is the management of someone with CGD

A 
aggressive treatment of infections
prophylaxis:
s aureus: TMP/SMX
aspergillus: itraconazole
HSCT
39
Q

what is the mutation associated with HyperIgE syndrome

A

autosomal dominant mutation in STAT 3

40
Q

what infections are associated with HyperIgE syndrome

A

Recurrent abscesses in skin, lungs
• “Cold boils” (no pain, heat, or redness)
• Caused by S. aureus
• Can have pneumatoceles

41
Q

which primary immunodeficiency is associated with delayed shedding of teeth

A

hyper IgE syndrome

42
Q

what are the laboratory features associated with Hyper IgE syndrome

A

very high IgE (>20, 000)
eosinophilia
Normal IgG, IgA, IgM
Can have poor antibody responses to vaccines

43
Q

what is the management for someone with hyper IgE

A

treat infections

anti- staphylococcal prophylaxis (TMP/SMX)

44
Q

what is considered delayed separation of the umbilical cord?

A

> 4 weeks

45
Q

which PID is associated with delayed separation of the umbilical cord

A

Leukocyte adhesion defect

46
Q

what type of infections are seen with LAD

A

staph infections in first year of life (dental, gingivitis, intestinal)

47
Q

what are the laboratory features associated with LAD

A

neutrophila (can be >100, 000 even without an infection)

absent surface adhesion molecules CD11 and CD18

48
Q

what is the treatment for LAD

A

antibiotic treatment
prophylaxis
HSCT

49
Q

what are the 3 complement pathways?

A

classical pathway
lectin pathway
alternative pathway

50
Q

what is the laboratory finding associated with complement deficiency

A

abnormal CH50
Check alternative pathway (AH50) if CH50 normal &
still suspect complement deficiency

51
Q

Deficiency of what is seen in patients with recurrent Neisseria meningitidis?

A

Deficiency of one of the terminal components (C5-9) that compose the MAC predisposes patients to infection with Neisseria meningitidis.

52
Q

Congenital deficiency of C1-inhibitor results in what?

A

hereditary angioedema

53
Q

early complement components (C1, C2, C3, C4, factor I and factor H) have an association with ?

A

early complement components (C1, C2, C3, C4, factor I and factor H) have an association with autoimmune diseases and infection with encapsulated organisms.

54
Q

penicillin anaphylaxis is mediated by what?

A

IgE against a major determinant

55
Q

Deficiency of what may lead to anaphylaxis in a patient given IVIG:

A

IgA

56
Q

what is the test for T cell function

A

mitogen proliferation studies
antigen proliferation studies
T cell excision circles (TRECs)- Measured in newborn screening for severe combined immunodeficiency

57
Q

CVID is associated with what malignancies

A 
non- Hodgkins lymphoma
GI malignancies (gastric carcinoma)
58
Q

What are the side effects of IVIG? (9)

A 
Headaches
fevers
chills
Aseptic meningitis
Anaphylaxis*- IgA
Hemolytic anemia *- patients with high doses more then for immunology
Thrombosis
Renal complications
Risk of transmission of blood borne illness (screen yearly for HepB/C, HIV)
59
Q

what immunodeficiency is associated with giardia?

A

CVID

Pediatrics- Noelle (28 decks)

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