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NICU Board Review > Immune > Flashcards

Immune Flashcards

1
Q

Inflammatory Response Mediators

A

Vasodilation
Histamine, prostaglandin, nitric oxide, bradykinin
Increased vascular permeability
Histamine, complement, bradykinin, leukotrienes, nitric oxide

Leukocyte adhesion
- Cytokines (IL-1, TNF-a), complement, eicosanoids (prostaglandins, leukotrienes), selectins

Chemotaxis
Chemokines, complement, eicosanoids (prostaglandins, leukotrienes),

Fever
• IL-1, TNF-a, prostaglandins

Tissue necrosis
- Neutrophil granules, free radicals
Platelet aggregation
Eicosanoids (prostaglandins, leukotrienes)

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2
Q

Complement crosses the placenta (T/F)

A

False. They don’t cross

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3
Q

Role of neutrophils

A

Phagocytic and microbicidal function

Function reduced with premature GN sepsis indomethacin and intrapartum mag

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4
Q

Which molecules allow for cell adhesion rolling?

A

Decreased L selectin and B 2 integrity

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5
Q

Macrophages present which cells?

A

T cells and B cells

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6
Q

Macrophages have decreased activation and migration in neonates secondary to?

A

Poor response to IFN gamma

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7
Q

Adaptive immune system has which type of response?

A

Antigen specific

Takes 4-7 days to develop

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8
Q

What’s the role of CD8 T cells

A
  • Cytotoxic lymphocytes
  • Release perforins, degradative enzymes,
    and cytokines
  • Cytokine production is lower compared with adults
    -Cytotoxic activity is limited in neonates
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9
Q

IgG

A

Crosses the placenta

IgM (pentameter) and IgA (dime)
levels are low at birth as they do
not cross the placenta

Infections with CMV, rubella, and
Toxo can raise levels

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10
Q

Clinical features of immunodeficiency

A

Delayed umbilical cord separation
Infection - particularly repeated
Failure to thrive
Chronic diarrhea
Symptomatic infection due to live vaccines (eg, rotavirus, Bacille Calmette-Guérin [BCG], oral polio)
Heart or lung disease (low qxygen saturation suggests one of these problems)
Congenital asplenia
Mucosal abnormalities such as thrush, mouth sores, and ulcerations
Petechiae, melena, bleeding
Cutaneous rashes, pigmentary abnormalities
Syndromic appearance (abnormal faces or habits) including facial or craniofacial anomalies
Lymphadenopathy and/or hepatosplenomegaly
Autoimmunity

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11
Q

Absence in NADPh oxidase function. Dx?

A

Chronic granulomatous disease

Deficiency or absent NADPH oxidase function in
phagocytic cells
80-90% MALE- usually X-linked recessive
Severe, recurrent infections with catalase-positive
bacteria or fungi
Diagnosis made by NBT ->remains colorless
demonstration of absent or severely deficient respiratory
burst activity in phagocytes
Difficult diagnosis in neonates-reduced respiratory burst at baseline

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12
Q

Leukocyte Adhesion Deficiency

A

• LAD-1 (predominant type)
Deficiency of leukocyte integrins
(very low to absent surface
CD11b/CD18 on PMNs)

• LAD-2
Deficiency of selectin function
(normal CD11b/CD18, decreased
CD15 on PMNs)

Clinical presentation (LAD-1)
Recurrent infections
Delayed separation of umbilical
stump
Leukocytosis
-
NO PUS!!
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13
Q

Disorders of Neutrophil production and function

A

Myeloperoxidase deficiency
• MC deficiency (1 in 2-4K people), can be clinically silent
• Recurrent candidal infections

Chédiak-Higashi syndrome
• Abnormal degranulation, recurrent infections, albinism, giant intracellular granules

HyperimmunoglobulinE
STAT3 mutation alters PMN chemotaxis

G6PD
Decreased NADPH activity > decreased oxygen dependent killing

Galactosemia
Inhibitory effects of galactose on PMNs

Neutropenia
Glycogen Storage Disease 1B, Shwachman-Diamond syndrome, Kostmann syndrome, Reticular
dysgenesis

Trisomy 21
Depressed chemotactic activity

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14
Q

Not much difference between albumin and total protein. Which immune disorder?

A

Agammaglobulinemia

Profoundly diminished lg levels of all isotypes
- IgM levels are undetectable
B cell-specific src-associated (Bruton’s) tyrosine
kinase mutation >prevents pre-B cells from
becoming mature B cells/plasma cells
Absence of B cells in the blood and lymphoid tissue
•
Plasma cells are absent in Gl tract
A
• Normal T cell numbers but dysplastic lymph nodes
due to no B cells

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15
Q

Peri rectal abscess and neutropenia in a boy?

A

Hyper IgM syndrome

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16
Q

Absent thymic tissue, interrupted aortic arch, problems with Ca retention. Dx?

A

Chromosome 22q11 deletion

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17
Q

Boy with eczema, thrombocytopenia and infections. Dx?

A

Wiskott-Aldrich Syndrome
Eczema, thrombocytopenia, and susceptibility to
infection (decreased lymphocyte count)
• Platelets are small, defective, and abnormally
shaped
• Lymphocyte numbers are decreased and T cell
function is abnormal
• High IL-4 levels lead to eczema (increases IgE)
• Decreased IgM, Elevated IgA and IgE
• Normal IgG
• X-linked

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18
Q

Lymphadenopathy on vignette. MCC Dx?

A

HIV

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19
Q

How do you diagnosis SCID?

A

Absence of TREC

SCID - Deficiency of both antibody and cell-mediated immunity
(low/normal # B cells, decreased T cells/NK cells)
• Diarrhea, pneumonia, otitis, sepsis, cutaneous infections,
eosinophilia
20-30% risk of maternal cell-mediated GVHD (placental transfer of
maternal T cells)

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20
Q

Which pathogen has a 1 reservoir outside of humans and primates

A

Toxo

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21
Q

Granulomatosis Infectiosum is seen with which disease?

A

Listeria

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22
Q

Highest sensitivity for maternal chorio

A

Maternal fever (80%)

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23
Q

What grows in Thayer Martin medium

A

Gonorrhea

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24
Q

Name single stranded DNA virus

A

Parvo

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25
Q

Single stranded RNA

A

Enterovirus

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26
Q

What is associated with
Celery stalking long bone
Salt and pepper retina

A

Rubella

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27
Q

Infant with infancy with the classic triad of enteropathy, dermatitis, and hyperglycemia. Other siblings died at early age. Dx?

A

IPEX

Immune dysregulation, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome is a systemic autoimmune disease caused by immune dysregulation because of a mutation of FOXP3 affecting control of T-regulatory cells.

Bone marrow and allogeneic hematopoietic stem cell transplantation are the only potential cures presently for IPEX syndrome.

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28
Q

If low TREC on NBS, what is next test?

A

Flow cytometry of lymphocyte subset

IgG levels not indicated until after 6 mo (bc of maternal antibodies)

Think SCID or Di George or false positive in premie.

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29
Q

What is the MOA of interferon alpha?

A

Activated in response to infection and act via toll like teceptors.
Limit viral replication

30
Q

Development of T cell (weeks and location)

A

8.5 weeks fetal liver
10 weeks thymus becomes lymphoid
11-12 weeks T cells emigrate from thymus to spleen, nodes
16-18 weeks hassall’s bodies in the thymus

31
Q

B cell development (location and weeks)

A

8 weeks pre-B cells in fetal liver
8-10 weeks in fetal bone marrow
18-22 weeks in liver lung and kidney
>30 weeks Solely in bone marrow

32
Q

Natural immune system comprises of?

A

Neutrophils for chemotaxis, phagocytosis, bacterial killing

Monocytes for chemotaxis, phagocytosis, bacterial killing, wound repair

Complement for opsonization, chemoattraction

33
Q

Infant with recurrent infections, eczema, coarse facial features and broad nasal bridge. Etiology of this disorder?

A

Abnormality in neutrophil chemotaxis

Hyperimmunoglobulin E (Jobs syndrome)

34
Q

Neonate with omphalitis without pus formation and delayed separation of cord. What is the underlying reason for this disorder?

A

Leukocyte adhesion deficiency

Defective neutrophil adhesion and migration

Mutation in the B2 integrin gene

35
Q

Infant with partial oculocutaneous albinism, nystagmus, Peripheral neuropathy and recurrent infections. Whats the underlying problem?

A

Chediak-Higashi

Abnormal neutrophil degranulation (afecta intracellular killing) and monocyte chemotaxis

36
Q

Infant with recurrent abscesses, poor wound healing and granulomas. What is the underlying cause of this disease?

A

Chronic granulomatous disease

Dysfunctional NADPH oxidase and abnormal phagocytic microbial ability

X-linked

Test: nitroblue tetrazolium test (neutrophils in pts with CGD cannot produce superoxide and thus test negative(

37
Q

Pale infant with steatorrhea, failure to thrive, and generalized bone marrow dysfunction. Dx?

A

Schwachman Diamond Syndrome

AR- decreased neutrophil production

May also have skeletal defects and dysmorphic features

Increased risk for myelodysplastic syndrome

38
Q

Infant noted with severe neutropenia since 1 month of life with recurrent infections. Dx?

A

Kostman syndrome

AR

39
Q

Most neonates reach adult complement levels by what age?

A

3-6 months

40
Q

Early (C1-4) components vs late (C5-9) component deficiencies of the complement system are associated with which infections?

A

Early C1-4 pneumococcal

Late C5-9 neiserria

41
Q

IL 1 and TNF alpha role

A

Cytokines involved in leukocyte adhesions and mediating fever response

42
Q

Infant with h/o pneumonia, multiple bouts of itits, diarrhea, sinusitis between 4-12 months of life. Dx?

A

X linked agammaglobulinemia

Severe deficiency of B cells leading to hypogamma-globulinemia

43
Q

Infant with episodic fever triggered by immunizations with athralgias, rash, diarrhea, vomiting, oral ulcers. Dx?

A

Hyperimmunoglobulin D Syndrome

Mutation in mevalonate kinase gene

Treatment with 3 hydroxy 3 methylglutaryl co enzyme A reductase inhibitors or TNF inhibitors or IL 1 inhibitors

Severe form is mevalonic aciduria

44
Q

Alloimmune neonatal neutropenia are mediated by antibodies that bind to human neutrophil antigen (HNA)

A

HNA 1a

45
Q

Neonate born to a mother w lupus and now 7 days old w renal vein thrombosis. Mother with prior pregnancy losses. Which is the most common autoantibody associated with neonate’s condition?

A

Antiphospholipid syndrome

Neonate without other findings of SLE and 30% of mother’s w SLE have antiphospholipid antibodies which increases risk for clots and pregnancy loss.

46
Q

Most common thoracic duct anatomy involves a

A

Single duct

47
Q

When does the stratum corneum keratinization begin?

A

32-34 weeks gestation and matures within 2 weeks after birth

Skin keratinization is delayed is delayed in premies because of this

48
Q

Difference between preterm and term immunity

A

Decreased complement
Lower neutrophils stores and adhesion
Decreased activity CD4 and CD8
Decreased maternal transfer of IgG that happens in 3rd trimester

49
Q

Most common manifestation of primary infantile systemic lupus erythematosus?

A

Glomerulonephritis, then pneumonitis

50
Q

What marker is used to diagnose SCID with low TREC present?

A

Beta actin levels

Term
Low TREC + normal beta actin = abnormal result —> obtain flow cytometry

Preemie
Low TREC + normal beta actin = abnormal result —> repeat TREC by 40 weeks

51
Q

What is the initial event in pathogenesis of congenital heart block in neonatal lupus syndrome?

A

Cardiocyte apoptosis

52
Q

Most common cause of neutropenia in early neonatal period is

A

Pregnancy-induced hypertension

53
Q

What amino acid is essential for disulfide covalent bonding in chemokines?

A

Cysteine

54
Q

The most common manifestation of neonatal SLE is?

A

Thrombocytopenia (MC)

then rash and direct hyperbilirubinemia

55
Q

MCC of apnea after immunizations in preemies

A

Having apneic episodes before immunization

56
Q

How can phagocytosis be more effective?

A

By complement component 3 and IgG coating

57
Q

Role of follicular B 2 cells

A

Provide adaptive immune response against thymus dependent antigens

High affinity Ab - highly specific to Ag

58
Q

B1 cells are part of the ___ immune response

A

Innate

Found primarily in the peritoneal and pleural cavities

Low affinity antibodies with broad antigen specificity

59
Q

Role of Marginal zone B cells

A

Found in spleen

Innate immune response against thymus independent Ag

Create low affinity Ab with briad Ag specificity

60
Q

Patients with X linked agammaglobulinemia is characterized by mutation in which enzyme?

A

Beuton tyrosine kinase

Near abscence of all immunoglobulins

Present after 2 months as maternal IgG is still present in newborn period

61
Q

CD 40 ligand mutations lead to ______.

A

X linked Hyper IgM

High to normal IgM
Low IgG, IgA, IgE

This mutation leads to abnormalities in IG class switch recombination

62
Q

What decreases neutrophil chemotaxis?

A

GCSF

Granulocyte macrophage colony stimulating factor

63
Q

What decreases neutrophil chemotaxis?

A 
Mag sulfate (intrapartum)
Excessive theophylline [ ]
Indomethacin administration
64
Q

By what postnatal age does maternal IgG typically disappear in neonatal circulation?

A

9 months

65
Q

Neutrophils of term neonates compared to adults

A

Decreased migration
Poor adherence to endothelium
Decreased chemotactic response
Comparable phagocytosis and de granulation

66
Q

Most common complement decifiency

A

C2

67
Q

Where do the classical and alternative pathways converge ?

A

C3

68
Q

Differences between classical and alternative pathways?

A

Classical complement pathway requires Ag-Ab reaction (immune complex formation)

Alternative pathway may be Ab independent

69
Q

Patients with splenic dysfunction show a peripheral blood smear with

A

Howell jolly bodies

70
Q

Name 4 collectins

A

Surfactant protein A, SP-D, conglutinin, mannose binding lectin

NICU Board Review (19 decks)

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