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Pediatrics > Immunology > Flashcards

Immunology Flashcards

1
Q

Extracellular bacteria

A

B cells - immunoglobulins

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2
Q

Intracellular bacteria, viruses, and fungi

A

T cells

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3
Q

Present after 6mo with recurrent sinopulmonary, GI, and UTIs with encapsulated organisms

A

B-cell deficiencies

Encapsulated: H flu, Strep pneumo, Neisseria meningitidis

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4
Q

Tx: B cell deficiencies

A

IVIG (except IgA def)

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5
Q

Present 1-3mo with opportunistic and low-grade fungal, viral, and intracellular bacterial infections (mycobacteria)

A

T-cell deficiencies

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6
Q

Mucous membrane infections, abscesses, poor wound healing. Infections with Catalase+ org (S aureus), fungi, and gram- enteric organisms are common

A

Phagocyte deficiencies

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7
Q

Children with recurrent bacterial infections with encapsulated organisms. In children with congenital asplenia or splenic dysfunction

A

Complement deficiencies

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8
Q

Bruton’s congenital agammaglobulinemia

A

XLR (found only in boys)
B-Cell disorder
Apparent after 6 mo (when maternal IgG is inactive.
Encapsulated infections: Pseudomonas, Strep pneumo, Haemophilus influ.

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9
Q

Dx: Bruton’s

A

Quantitative immunoglobulin levels
-if low, confirm with B and T cell subsets
-B cells absent, T cells often high
Absent tonsils and other lymphoid tissue

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10
Q

Tx: Bruton’s

A

Prophylactic Abx

IVIG

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11
Q

Common variable immunodeficiency (CVID)

A 
Usually combined B and T cell defect
ALL Ig LEVELS LOW (20s and 30s)
Normal B cell numbers
Dec plasma cells
Usually present later in life (15-35y)
Inc pyogenic and URI and lower resp infections.
Inc risk of lymphoma and autoimmune dz
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12
Q

Dx: CVID

A

Quantitative immunoglobulin levels

Confirm with B and T cell subsets

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13
Q

Tx: CVID

A

IVIG

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14
Q

IgA Deficiency

A

MC immunodeficiency
B-cell disorder
Dec IgA levels only
Usually asymptomatic
May have recurrent resp or GI infections (Giardia)
Prone to anaphylactic transfusion reactions 2/2 anti-IgA antibodies

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15
Q

Tx: IgA deficiency

A

DO NOT GIVE IVIG, can lead to the production of anti-IgA antibodies
Treat infections

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16
Q

DiGeorge Syndrome (Thymic aplasia)

A 
CATCH 22
Cardiac abnormalities
Abnormal facies
Thymic aplasia
Cleft palate
Hypocalcemia
Chromosome 22
Presents: tetany 2/2 hypocalcemia in first days of life
Variable risk of infection
Much INC risk with viruses, fungi and PCP
17
Q

Dx: DiGeorge

A

XRay may show absent thymic shadow

Absolute lymphocyte count

18
Q

Tx: DiGeorge

A

Bone marrow transplant
IVIG
PCP prophylaxis
Alternative - thymus transplant

19
Q

Ataxia-telangiectasia

A

Combined (B and T) disorder
Progressive cerebellar ataxia and oculocutaneous telangiectasias.
Defect: DNA repair
INC incidence of malignancies (non-Hodgkin’s, leukemia, and gastric carcinoma).

20
Q

Tx: ataxia-telangiectasia

A

No specific treatment, maybe IVIG depending on the severity

21
Q

Severe Combined Immunodeficiency (SCID)

A

Combined (B and T) disorder
Defect in stem cell maturation
Dec in adenosine deaminase
Severe, frequent bacterial infections, chronic candidiasis, opportunistic organisms

22
Q

Tx: SCID

A

Bone marrow or stem cell transplant
IVIG for ab deficiency
PCP prophylaxis

23
Q

Wiskott-Aldrich Syndrome

A 
XLR (only seen in males)
Combined (B and T) disorder
Symptoms at birth
INC IgE/IgA
DEC IgM
DEC Plt (Thrombocytopenia)
INC risk of atopic disorders, lymphoma/leukemia, and infection from encapsulated org (S pneumo, S aureus, H influ)
24
Q

Bleeding, eczema, recurrent otitis media

A 
Wiskott-Aldrich Syndrome
W.I.P.E.
Wiskott-Aldrich
Infections
Purpura (thrombocytopenia)
Eczema (atopic disorders)
25
Q

Chronic Granulomatous Dz

A

XL (2/3), AR (1/3)
Phagocytic disorder
Def. of Superoxide production in PMNs and macrophages
May see: anemia, lymphadenopathy, and hypergammaglobulinemia
Chronic infections with CATALASE+ org
S aureus, E coli, Candida, Klebsiella, Pseudomonas, Aspergillus
Chronic skin, lymph node, pulm, GI, urinary tract, osteomyelitis, and hepatitis
May have granulomas of the skin, GI/GU tracts

26
Q

Dx: CGD

A

Abs PMN count with PMN assay

NITROBLUE tetrazolium test - dx of CGD

27
Q

Tx: CGD

A

Daily TMP-SMX
INF-gamma can DEC incidence of serious infection
New therapies: bone marrow transplant and gene therapies

28
Q

Leukocyte Adhesion Def

A

Phagocytic disorder
defect in chemotaxis of leukocytes
Recurrent skin, mucosal, and pulm infections
DELAYED SEPARATION OF THE UMBILICAL CORD
Omphalitis in the newborn
NO PUS with MINIMAL INFLAMMATION in wounds
HIGH WBC in blood

29
Q

Tx: LAD

A

Bone marrow transplant is curative

30
Q

Chediak-Higashi Syndrome

A

AR
Phagocytic disorder
Defect in PMN chemotaxis and microtubule polymerization.
PARTIAL OCULOCUTANEOUS ALBINISM, PERIPHERAL NEUROPATHY AND NEUTROPENIA
Greatly INC incidence of overwhelming pyogenic infections with S pyogenes, S aureus, and Pseudomonas sp

31
Q

Giant granules in PMNs

A

Chediak-Higashi Syndrome

32
Q

Tx: Chediak-Higashi

A

Bone marrow transplant in ToC

33
Q

Job’s Syndrome

A 
Phagocytic disorder
Defect in PMN chemotaxis
RECURRENT S AUREUS INFECTIONS AND ABSCESSES
F.A.T.E.D
coarse Facies
Abscesses (S aureus)
retained primary Teeth
hyper-IgE (eosinophilia)
Dermatologic (severe eczema)
34
Q

Tx: Job’s Syndrome

A

Penicillinase-resistant abx

IVIG

35
Q

Fever
Bilat, nonexudative, painless conjunctivitis
Polymorphous rash
Unilateral cervical lymphadenopathy
Strawberry tongue; dry, red, cracked lips
Erythema of palms and soles
Indurative edema of the hands and feet
desquamation of the fingertips
Sterile pyuria, gallbladder hydrops, hepatitis, and arthritis

A

Kawasaki’s Dz

36
Q

Kawasaki dz: Subacute phase

A 
begins after abatement of fever
lasts 2-3 wks
Thrombocytosis, elevated ESR
Untreated may begin to develop coronary artery aneurysm
Asses with ECHO
37
Q

Kawasaki dz: Chronic phase

A

begins when clinical symptoms have disappeared

Lasts until ESR returns to baseline

38
Q

Tx: Kawasaki

A

High-dose ASA
IVIG
Low-dose ASA is continued, usually for 6 wks
If coronary aneurysm, chronic anticoag with ASA
(corticosteroids may be used in IVIG-refractory cases)

Pediatrics (7 decks)

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