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CKPeds > Immune DO > Flashcards

Immune DO Flashcards

1
Q

Common Organisms for B cell deficiency

A

Recurrent bacterial: streptococci, staphylococci, Haemophilus, Campylobacter;

Viral:enteroviruses;

Uncommon: giardia, cryptosporidia

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2
Q

Common Organisms for T cell deficiency

A

Opportunistic organisms: CMV, EBV, varicella, Candida,

Pneumocystis jiroveci, mycobacteriwa

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3
Q

Common Organisms for Complement deficiency

A

Pneumococci, Neisseria

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4
Q

Common Organisms for neutrophil deficiency

A

Bacteria: Staphylococci, Pseudomonas, Serratia, Klebsiella, Salmonella;

Fungi: Candida, Aspergillus

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5
Q

Age onset

B-Cell

T-Cell

Complement

Neutrophil

A

5-7 months of age or later childhood to adult

Usually 2-6 months of age

Any age

Early onset

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6
Q

Types of infections wth

B-Cell deficiency

A

Most are recurrent sinopulmonary infections and recurrent

enteroviral meningitis

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7
Q

Types of infections wth

T Cell deficiency

A

Mucocutaneous candidiasis; pulmonary and GI infections

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8
Q

Types of infections wth

Complement deficiency

A

Meningitis, arthritis, septicemia, recurrent sinopulmonary

infections

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9
Q

Types of infections wth

Neutrophil deficiency

A

Skin abscesses, impetigo, cellulitis, suppurative adenitis,

gingivitis, oral ulcers, osteomyelitis, internal organ abscesses

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10
Q

Other findings with:

B-Cell deficiency

A

Autoimmunity, lymphoreticular malignancy

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11
Q

Other findings with:

T-Cell deficiency

A

Chronic diarrhea and failure-to-thrive;

postvaccination dissemination - varicella, BCG;

hypocalcemia in infancy;

graft-versushost from transplacental maternal engraftment or nonirradiated blood

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12
Q

Other findings with:

Complement deficiency

A

Autoimmune disorders, vasculitis, glomerulonephritis,

angioedema

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13
Q

Other findings with:

Neutrophil deficiency

A

Prolonged attachment of umbilical cord, poor wound healing, decreased signs of infection

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14
Q

Best initial test

B-Cell deficiency

A

Screen with IgA→if low, measure IgG and IgM (quantitative

immunoglobulins

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15
Q

Best initial test

T-Cell deficiency

A

Lymphocyte count (low)

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16
Q

Best initial test complement deficiency

A

Screen is total hemolytic complement (CH50)—will be

depressed if any component is consumed

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17
Q

Best initial test neutrophil deficiency

A

Neutrophil count

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18
Q

Best cost-effective test for T-cell function –

A

Candida skin test

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19
Q

Complemend deficiency

all are autosomal except for _______

A 
properdin deficiency
(X-linked
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20
Q

Neutrophil respiratory burst after phorbol ester stimulation;
most reliable now uses_________

A

rhodamine fluorescence (replaced the NBT test

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21
Q

Specific test

B-Cell deficiency

A

Enumerate B-cells with flow cytometry (monoclonal antibodies to B-cell-specific CD antigens): B cell absent or
present and number

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22
Q

Specific tests:

T cell deficiency

A

Flow cytometry using monoclonal antibodies recognizing
T-cell CD antigens (phytahemmaglutinin,
concanavalin A, pokeweed mitogen

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23
Q

profound defect in B-cell development
which leads to an absence of circulating B cells and thus leads to severe hypogammaglobulinemia
with small-to-absent tonsils and no palpable lymph nodes

A

X-linked (Bruton) agammaglobulinemia

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24
Q

> 500 known mutations of the _______ which is necessary for pre-B-cell expansion and maturation; long arm of X-chromosome

A

Btk gene (Bruton tyrosine kinase),

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25
Q

Findings for X-linked (Bruton) agammaglobulinemia

A

boys with pyogenic sinopulmonary infections

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26
Q

X-linked (Bruton) agammaglobulinemia Tx

A

appropriate use of antibiotics + regular monthly IVIG

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27
Q

The only 2 B-cell defects for which stem cell transplantation is recommended are
________ and ______

A

CD40 ligand defect (extremely rare; one of the known mutations on the X-chromosome for
hyper IGM syndrome) and X-linked lymphoproliferative disease

28
Q

Common Variable Immunodeficiency (CVID) is _____________

A

hypogammaglobulinemia with phenotypically

normal B-cells;

29
Q

What is the problem with CVID

A

Common Variable Immunodeficiency (CVID) is hypogammaglobulinemia with phenotypically
normal B-cells;

30
Q

CVID

clinical presentation + serum IG and antibody deficiencies as profound or less than in XLA; normal sized lymphoid tissue; later autoimmune disease and malignancy __________

A

(lymphoma

31
Q

Whast is the Tx of CVID

A

therapy consists of the one IG preparation available that contains no IgA.

32
Q

__________ is the most common immunodeficiency. It is caused by the absence or near absence of serum and secretory IgA with phenotypically normal B-cells

A

Selective IgA deficiency

33
Q

Selective IgA deficiency

Clinical findings: same bacteria as others with most infections in respiratory, GI and urogenital tracts;_________ is common

A

giardiasis

34
Q

serum antibodies to IgA can cause severe _______ if any blood product with IgA is administered (NOT a transfusion reaction)

A

anaphylactic reactions

35
Q

Tx of IgA Def

A

Treatment: IVIG is not indicated (95−99% is IgG) because if usual IVIG (containing IgA) product is given, patients are at risk for severe reaction. Additionally, because it is specifically an IgA deficiency, the IVIG product with the IgA removed cannot be used. Treat the infections (generally milder).

36
Q

________is thymic and parathyroid hypoplasia to aplasia from dysmorphogenesis of the 3rd and 4th pharyngeal pouches

A

DiGeorge syndrome

37
Q

Other structures involved in Di Georg

A

great vessel anomalies (right-sided aortic arch, interrupted aortic arch), esophageal atresia, bifid uvula, congenital heart disease (conotruncal malformations, septal defects), facial dysmorphism (short philtrum, thin
upper lip, hypertelorism, mandibular hypoplasia, low-set, often notched ears), and cleft palate

38
Q

What is the CATCH 22 syndrome

A

Cardiac, Abnormal facies, Thymic hypoplasia, Cleft palate, Hypocalcemia); partial DiGeorge is more common, with
variable thymic and parathyroid hypoplasia.

39
Q

About 1/3 with complete DiGeorge have the ____

A

CHARGE association.

40
Q

Initial presentation of Di George is?

A

neonatal hypocalcemic seizures

41
Q

Tx of Di Georg

A

complete form correctable with either culture unrelated thymic tissue transplants or bone marrow or peripheral blood transplantation from HLA-identical
sibling

42
Q

_________ is the absence of all adaptive immune function, and in some, natural killer cells due to diverse mutations. It is the most severe immunodeficiency
known

A

Severe Combined Immunodeficiency (SCID)

43
Q

Clinical findings associated with SCID

A

first 1-3 months of life with recurrent/persistent diarrhea and
opportunistic infections that may lead to death;

44
Q

Pts with SCID

also at risk for graft-versus-host disease from ________that crossed the placenta in
utero

A

maternal immunocompetent T-cells

45
Q

the presence of low but not absent T-cell function and low
but not absent antibodies; patients survive longer but have failure-to-thrive and still die relatively
early in life

A

Combined immunodeficiency

46
Q

_________ is an impaired humoral immune response and highly variable concentrations of the IGs with moderately reduced T-cells and variable mitogen responses.

A

Wiskott-Aldrich Syndrome

47
Q

Clinical findings of WAS

A

(1) thrombocytopenia presenting in neonatal period
(2) atopic dermatitis, and
(3) recurrent infections in first year of life

48
Q

Dx of WAS

A

most common IG pattern is low IgM,

high IgA and IgE and normal to slightly low IgG and variably reduced T-cells.

49
Q

WAS

Rare survival beyond adolescence (bleeding, infections and EBV associated malignancies and autoimmune complications) without a _______

A

bone marrow

transplant

50
Q

______ is a moderately depressed response to T and B-cell mitogens, moderately reduced CD3 and CD4 T-cells with normal or increased percentages of CD8, T-helper cell and intrinsic B-cell defects, and hypoplastic thymus

A

Ataxia-telangiectasia

51
Q

Mutation of AT

A

AT mutation (ATM) at 11.22-23

52
Q

AT

(1) ataxia evident with onset of walking and progresses until
age______ when confined to a wheelchair

(2) ______ develop at 3-6 years of age and

(3) recurrent sinopulmonary infections most with
common viruses and occasional fatal _____

A

10-12 years

oculocutaneous telangiectasias

varicella

53
Q

___________ is a rare disorder of leukocyte function causing recurrent bacterial and fungal infections and decreased inflammatory responses in the presence of neutrophilia (increased counts

A

Leukocyte adhesion deficiency

54
Q

MC infections associated with Leukocyte Adhesion Deficiency

A

most common organisms are S. aureus, gram-negatives and Candida and Aspergillus

55
Q

Leukocyte Adhesion Deficiency Dx

A

paucity of neutrophils in affected tissue but circulating neutrophil count is significantly elevated

56
Q

Tx of Leukocyte Adhesion Def

A

early allogenic stem-cell transplantation for severe forms otherwise supportive care

57
Q

_________is when neutrophils and monocytes phagocytize but cannot kill catalase-positive microorganisms as a result of a defect in production of oxidative metabolites

A

Chronic granulomatous disease (CGD)

58
Q
  • Autosomal recessive

* Abnormal secretory/storage granules lead to large and irregular seen in neutrophils

A

Chediak-Higashi Syndrome

59
Q

Associations of Chediak-Higashi Syndrome

A

Oculocutaneous albinism from birth, prolonged bleeding time, peripheral neuropathy, recurrent infections

60
Q

All components are autosomal recessive or co-dominant, except for ______ which is X-linked recessive

A

properdin deficiency

61
Q

Decrease in both _____ and ____suggests activation of the alternative pathway; this is most useful in distinguishing nephritis secondary to immune complex deposition
from that due to nephritic factor

A

C3 and C4

62
Q

Results of Defect in complement function

A

recurrent angioedema, autoimmune disease,
chronic nephritis, HUS, recurrent pyogenic infections, disseminated meningococcal or gonococcal infections or a second episode of bacteremia at any age; high
incidence of pneumococcal and meningococcal infections

63
Q

Major cause of morbidity and mortality after allogenic stem cell transplantation

A

Graft-Versus-Host Disease (GVHD)

64
Q

__________ 2-5 weeks post-transplant; erythematous maculopapular rash, persistent anorexia, vomiting and/or diarrhea and abnormal liver enzymes and LFTs;

A

Acute GVHD:

65
Q

primary prevention of GVHD is with ________

A

post-transplant immunosuppressive drugs and corticosteroids

66
Q

_______develops or persists >3 months after transplant; major cause of non-relapse morbidity and mortality in long-term transplant survivors

A

Chronic GVHD:

CKPeds (20 decks)

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