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Neonatology Boards > Immunology > Flashcards

Immunology Flashcards

1
Q

Clinical features of neonatal lupus

A
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2
Q

Neonatal myasthenia gravis

A

neonates born to mother’s with MG have a 10-20% chance of being affected
most newborns have normal muscle tone and activity

Transient neonatal myasthenia gravis:
- transplacental passage of IgG autoantibodies
- no sxs in first few days: transplacental passage of maternal meds and maternal concentration of alpha-fetoprotein (inhibits autoantibody binding to acetylcholine receptors)
- symptoms: hypotonia, dec spont movements, poor feeding, respiratory insufficiency
- Dx: high concentrations of acetylcholine receptor antibodies
- prognosis is good- recovery by ~ 2 months

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3
Q
A

Good Morning America
IgG
IgM
IgA (colostrum)

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4
Q

Function of the spleen

A

-synthesis of antibodies against carbohydrate antigens
- clears microorganisms from bloodstream

Effect of splenectomy:
- increased susceptibility to infection: E.coli, H flu, Staph, strep pyogenes, malaria, viruses
- usually rapid onset of infection- more likely to be fatal

most important feature for immunologic function = malpighian bodies (secondary follicle)

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5
Q

Describe T cell lymphopoiesis

A

8.5 wks: percursors in fetal liver
10 wks: thymus becomes lyphoid
11-12 wks: T cells emigrate from thymus to spleen, nodes
16-18 wks: Hassall’s bodies in thymus

Hassall's corpuscle
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6
Q

Describe B cell lymphopoiesis

A

~ 8 wks: Pre-B cells in fetal liver
8-10 wks: in fetal bone marrow
18-22 wks: liver, lung, kidney
>30 wks: B cell production solely in bone marrow

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7
Q

Natural immune system

A

First-line defense
non-specific
Rapid availability
Does not rely on T/B cell
Does not require prior epxosure to organism or antigen to function

Components:
- neutrophils: chemotaxis, phagocytosis, bacterial killing
- monocytes: chemotaxis phagocytosis, bacterial killing, wound repair
- complement: opsonization, chemoattraction, inflammation

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8
Q

Neutrophil function in neonate

A
  • decreased migration
  • normal bacterial killing if healthy, decreased if ill
  • majority of neutrophils in bone marrow
  • neonates < 1500g have lower total neutrophil concentration
  • longer period of neutrophilia following infection
  • higher baseline proliferationrate (doesnt increase much with infection)
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9
Q

Disorders of neutrophil FUNCTION

A
  1. abnormalities of chemotaxis: hyperimmunoglobulin E (hyper-IgE, Job’s syndrome)
  2. adhesions abnormalities: Leukocyte adhesion deficiency
  3. intracellular killing: myeloperoxidate deficiency, Chediak-Higashi, chronic granulomatous disase
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10
Q

Disorders of neutrophil PRODUCTION

A
  1. decreased production: Shwachmann-Diamond syndrome, Kostmann syndrome, Reticular dysgenesis
  2. excessive margination: pseudoneutropenia, endotoxemia
  3. accelerated usage or destruction: autoimmune/alloimmune neutropenia, Chediak Higasi
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11
Q

Hyperimmunoglobulin E
Job’s syndrome
Hyper IgE

A

abnormal neutrophil chemotaxis
STAT 3 mutation

recurrent infections- especially of skin
coarse facial features
broad nasal bridge
eczema

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12
Q

Early complement vs later complement deficiencies associated with which infections

A
  1. Early: C1-4 = pneumococcal
  2. Late: C5-9 = neiserria
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13
Q

Leukocyte adhesion deficiency

A

Type 1: issue with integrin
- very low/absent CD11/CD18
- delayed separation of umbilical stump (beyond 21 days), leukocytosis

Type 2: issue with selectin
- normal CD11/CD18, low CD15

NO PUS

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14
Q

When do you reach adult levels of complement

A

3-6 months

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15
Q

chronic granulomatous disease

A

ABSENCE of NADPh oxidase function

X-linked recessive (M»>F)

Severe, recurrent infections with CATALASE POSITIVE bacteria/fungi (ex. staph, pseudomonas, aspergillus, candida, enterobacter)

Dx: Nitroblue tetrazolium test (NBT)
- remains colorless = absent/deficiency respiratory burst in phagocytes

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16
Q

myeloperoxidase deficiency

A

recurrent candidal infections

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17
Q

Chediak-Higashi

A

Abnormal degranulation = giant intracellular granules

  • recurrent infections
  • albinism
  • nystagmus
  • peripheral neuropathy
18
Q

Wiskott Aldrich

A

Triad: eczema, thrombocytopenia, susceptibility to infection (dec lymphocyte count)

Eczema: caused by high IL-4 levels –> incr IgE

NORMAL IgG
DEC IgM
INCR IgA, IgE

X-linked

19
Q

Hyper-IgM syndrome

A

X-linked- CD40 ligand mutations
- abnormal T helper cell regulation of B cell Ig isotype switching

Symptoms start ~6 mo as maternal IgG decreases
peri-rectal/oral abscesses
Sinopulmonary infections, chronic diarrhea
neutropenia
Pneumocystic carinii pneumonia, encapsulated organisms (H flu, Strep pneumo)

High rate of malignancy- lymphomas, GI adenomas

LOW IgG, IgA, IgE
NORMAL/INC IgM

20
Q

Which immunoglobulin crosses the placenta?

A

IgG!

provides passive protection to the neonate

21
Q

What produces the immunoglobulins?

A

B-cells!

22
Q

Describe the different immunoglobulins

A

IgA: secretory; main component in colostrum, resp/GI secretions

IgG: binds and activates complement, binds Fc receptor to facilitate action

IgM: binds and activates complement, FIRST antibody expressed after infection

IgE: ALLERGIC reaction- binds to basophils/mast cells leading to histamine/leukotriene release

IgD: ? antigen receptor, importnat in regulating B-cell development

23
Q

Agammaglobulinemia

A

X-linked

severe deficiency of B cells leading to hypogamma-globulinemia
recurrent infections 4-12 mo
- pneumonia, otitis, sepsis, meningitis
- ENCAPSULATED bacteria: H flu, Strep pneumo

total protein - albumin = immunoglobulins
- if this number is low, think agammaglobulinemia

Could be due to mutation in Bruton tyrosine kinase

24
Q

Absent TRECs on newborn screen- next step?

A

Flow cytometry- lymphocyte subset eval

Could be SCID, DiGeorge

25
Q

SCID

A

Failure of T lymphocytes to develop
B lymphocytes compromised/absent

26
Q

Acquired immune system

A

specific response that relies on T and B lymphocytes

27
Q

Type and function of T lymphocytes

A

CD4 T cells: cytokine secreting helper cells
- present antigen with MACROPHAGES to B-lymphocytes

CD8 T cells: cytotoxic killer cells

28
Q

Function of T lymphocytes

A

MUST travel to thymus to complete development
activate macrophages
cell-mediated
present antigen to B-lymphocytes (CD4 T helper with macrophages)

T = THYMUS

29
Q

Function of B lymphocytes

A

Mature with bone marrow
Humoral-mediated
Produce immunoglobulins!

Preterm infants with poor antibody response to infection

B = BONE MARROW

30
Q

Triad: enteropathy, dermatitis, hyperglycemia

Family history of other siblings dying at early age

A

IPEX

Immune dysregulation
Polyendocrinopathy
Enteropathy
X-linked

FOXP3 mutation affecting control of T-regulatory cells

Tx: bone marrow or allogeneic hematopoietic stem cell transplantation

31
Q

Shwachman-Diamond

AR

A
  • generalized bone marrow dysfunction
  • dysmorphic features
  • malabsorption–> steatorrhea–> failure to thrive
  • skeletal defects

Increased risk of myelodysplastic syndromes

32
Q

Kostmann syndrome

Severe congenital neutropenia

A

AR

frequent infections beginning in first months of age
severe neutropenia, low ANC since birth

HIGH RISK for AML or myelodysplasia

33
Q

Development from the blood stem cell

A
34
Q

Classical vs alternative complement pathway

A
35
Q

Preterm vs term immunity

A

Decreased complement
Lower neutrophil stores/adhesion
Decreased CD4/CD8 activity
Decreased maternal IgG transfer (most 3rd tri)

36
Q

Immature B cells produce which type of immunoglobulin

A

IgM

Mature B cells switch from IgM to other isotypes
Switch is antigen driven and requires signaling between CD40 on B cell with CD40 ligand on T cell

37
Q

Make up of Ig molecule

A

Heavy chain: determines class/sub-class
Light chain:

Fab (fragment antigen binding): binds antigens
Fc: primary effector domain, changes here after antigen binding cause host response

38
Q

Synagis targets which part of the RSV virus?

A

F protein

39
Q

Part of the lung most affected by RSV

A

Bronchiole

think: RSV causes bronchiolitis

40
Q

triad of watery diarrhea, endocrinopathy (hypothyroidism, neonatal insulin dependent DM), eczematous dermatitis

A

IPEX

X-linked

BMT or suppress autoimmunity is the only treatment

Neonatology Boards (19 decks)

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