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Undeleted > Powell: Immunodeficiency Diseases > Flashcards

Powell: Immunodeficiency Diseases Flashcards

1
Q

The first primary immunodeficiency disease to be described was what and by whom?

A

WHAT: Agammaglobulinemia
WHO: Bruton

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2
Q

In _____, Bruton noted the absence of ________ in a boy w/ history of pneumonias and bac. infections.

A

1952

immunoglobulins

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3
Q

Burton (the 1st physician to provide specific immunotherapy for agammagloculinemia; X linked disorder) administered _______ ______ of _____.

A

intramuscular injections

IgG

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4
Q

Passive Immunity

A

given the cell product (not the cell; B cell/Tcell)

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5
Q

Why did it take until 1952 to describe an immunodeficiency?

A
  • didn’t know what immunity was until 1918 Spanish Flu

- HIV: 1980

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6
Q

Primary or congenital immunodeficiencies:

-are ____ _____ that results in an increased susceptibility to infection

A

genetic defects

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7
Q

Primary or congenital immunodeficiencies:

-frequently manifested in ____/_____

A

infancy and childhood

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8
Q

Primary or congenital immunodeficiencies:

-such diseases affect about 1 in ___ ppl in US

A

500

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9
Q

Secondary or acquired immunodeficiencies:

  • develop as a _____ of:
  • -_______
  • -disseminated _______
  • -treatment w/ _______ _______
  • -_______ of cells or immune system
A 
consequences
malnutrion
cancer
immunosuppressive drugs
infection (HIV)
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10
Q

_______ of the immune system is ______ for defense against ________ organisms and their toxic ______.
-Immune System is important for our survival.

A

integrity
essential
infectious
products

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11
Q

_______ are conserved across widely diverse species.

Any loss of function mutation affecting a TLR has neg. consequences for survival.

A

Toll-Like Receptors

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12
Q

Primary Immunodeficiencies:

  • ______ determined
  • disorders may affect _/__ components of the immune system
  • -including T,B lymphocytes, NK cells, phagocytic/complement proteins
A

genetically

1 or more

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13
Q

Primary Immunodeficiencies:

-may result from defects in _______ maturation or _______/______ in effector mechanism of innate and adaptive immunity

A

leukocyte
activation
or defects

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14
Q

Primary Immunodeficiencies:

  • innate defects= _______/______ ect.
  • adaptive defects= ______
A

cytokines/neutrofils

genes

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15
Q

The principal consequences of an immuno-deficiency is an ______ ______ to ________

A

increased susceptibility to infection

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16
Q

The nature of the infection in a particular patient depends largely on the ________ of the immune system that is ______

A

component

defective

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17
Q

The types of _______ infections can predict the type of _________

A

recurring

immunodeficiency

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18
Q

Deficient _____ immunity usually results in increased susceptibility to infection by _____ _____

A

humoral
pyogenic bacteria
(B cells/antibody infection)

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19
Q

X-linked Agammaglobulinemia (XLA):

-all antibody isotypes are very ___= not even IgM or IgD

A

low

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20
Q

X-linked Agammaglobulinemia (XLA):

-circulating __ cells are usually absent

A

B

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21
Q

X-linked Agammaglobulinemia (XLA):

-____ cells are present in reduced numbers in the ____ _____

A

Pre-B

bone marrow

22
Q

X-linked Agammaglobulinemia (XLA):

-______ are usually very small and ___ ____ are rarely palpable due to absence of germinal centers (contain B cells)

A

tonsils

lymph nodes

23
Q

X-linked Agammaglobulinemia (XLA):

-____ architectures is normal, as are ___ cell-dependent areas of spleen and lymph nodes

A

thymus

T cell-dependent

24
Q

X-linked Agammaglobulinemia (XLA):
-boys remain well during first __ to __ months of life by virtue of maternally transmitted ___ antibodies—repeatedly acquire infections w/ extracellular pyogenic organisms

A

6-9 months

IgG

25
Q

X-linked Agammaglobulinemia (XLA):
-defects are associated w/ loss of function of ____ ____ _____ that is important for ___ ___ cell expansion and maturation into Ig-expressing __ ___

A

Bruton Tyrosine Kinase
Pre-B
B cells

26
Q

X-linked immunodeficiency w/ Hyper-IgM:

  • very low serum Ig__, Ig__, and Ig__
  • elevated concentrations of ________ ig__
A

IgG
IgA
IgE
polyclonal IgM

27
Q

X-linked immunodeficiency w/ Hyper-IgM:

  • like boys with XLA, patients may become symptomatic during the ___ and ___ year of life w/ recurrent _____ _____
  • otitis media, sinusitis, pneumonia, tonsillitis
A

first and second

pyogenic infections

28
Q

X-linked immunodeficiency w/ Hyper-IgM:

-in contrast to pt with XLA, pts have____ ____ (circulating B cells but only make IgM)

A

lymphoid hyperplasia

29
Q

X-linked immunodeficiency w/ Hyper-IgM:
-defects in this disease is associated w/ a loss of function of _____ ligand (aka CD 154) that is expressed on ____ ___ ____

A

CD40

helper T cells

30
Q

X-linked immunodeficiency w/ Hyper-IgM:

-loss of _____ prevents __ ____ from co-stimulating _____ ______ __ ______.

A

CD40
T cell
antigen specific B cells

31
Q

X-linked immunodeficiency w/ Hyper-IgM:

-___ ____ are not signaled by the ___ ___ to go through isotype switching and only produce ____

A

B cells
T cell
IgM

32
Q

Treatment for Immunodeficiencies:
-it is ______
–_____ ______ and ____ _____ _____ (IgG therapy)
by Bruton pioneered this therapy

A

routine
prophylactic antibiotics
gamma-globulin therapy

33
Q

Deficient ________ immunity usually results in increased susceptibility to ____ and other ______ pathogens.

A

cell-mediated
viruses
intracellular

34
Q

Cell-Mediated Deficiencies:

  • ___ treatments for defects associated with deficient T cell responses
  • -rare for pts w/ absolute defects in T cell function survive beyond infancy/childhood
A

few

35
Q

DiGeorge’s Syndrome:

-a _______ related disease associated with tissue morphogenesis– the _____ does not develop

A

developmentally

thymus

36
Q

DiGeorge’s Syndrome:

  • _____ ____ results from defects in morphogenesis of the ___ and ____ pharyngeal pouches during early embryogenesis
  • -other structures forming at the same age are frequently affected: anomalies of great vessels, esophageal atresia, bifid uvuala, upper limb malformations, congenital heart disease, short philtrum of upper lip, hypertelorism, antimongoloid slant to eye, mandibular hypoplasia, low set notched ears
A

thymic hypoplasia

3rd and 4th

37
Q

DiGeorge’s Syndrome:
-percentage of T cells is variably _______
–there is a relative increase in percent of ___ ___
=B cell function is impaired only to the extend of needing helper T cells

A

decreased

B cells

38
Q

DiGeorge’s Syndrome:

  • most infants die from infections, cardiovascular defects or seizures within the _____/_____ of life= if survive infancy are mentally retarded
  • —similar with ___ ___ syndrome
A

first few months/2nd yr
fetal alcohol

(not just immune problems=other anomalies)

39
Q

X-linked Recessive Severe Combined Immunodeficiency Disease (SCIDs):
-severe combined immunodeficiency is a ____, fatal syndrome characterized by profound deficiencies of __ ___ and __ ___ function

A

rare
T cell
B cell

40
Q

X-linked Recessive Severe Combined Immunodeficiency Disease (SCIDs):
-__-linked is the most common form= ~42% of cases

A

X

41
Q

X-linked Recessive Severe Combined Immunodeficiency Disease (SCIDs):
-affected infants present w/in ___ ___ months of life= frequent episodes of diarrhea, pneumonia, otitis, sepsis, cutaneous infections

A

first few

42
Q

X-linked Recessive Severe Combined Immunodeficiency Disease (SCIDs):

  • growth appears ____ initially
  • extreme ____ usually after infections and diarrhea begins
A

normal

wasting

43
Q

X-linked Recessive Severe Combined Immunodeficiency Disease (SCIDs):

  • persistent infections w/ _____ ____
  • -candida albicans, pneumocystis, carinii, varicella, measles, parainfluenzae, cytomegalovirus, EBV
A

opportunistic organisms

44
Q

X-linked Recessive Severe Combined Immunodeficiency Disease (SCIDs):

  • infants lack the ability to reject _____ ____ and are therefore at risk for ________
  • -can result from maternal __ ___ that cross into the fetal circulation while the SCID infant is in utero
A

foreign tissue
GVHD
T cells

45
Q

X-linked Recessive Severe Combined Immunodeficiency Disease (SCIDs):
-_____ pts have few or no ___ ____ and ____ ____

A

XSCID
T cells
NK cells (susceptible to any infection)

46
Q

X-linked Recessive Severe Combined Immunodeficiency Disease (SCIDs):

  • XSCID pts usually have elevated % of __ ___
  • -however these __ ___ do not produce immunoglobulin normally, even after ___ ___ reconstitution by ____ ____ ______
A

B cells
Bcells
T cell
bone marrow transplatation

47
Q

Treatment of Immunodeficiencies:

    1. to minimized and control _____
    1. to replace the defected or absent components of the immune system by ____ ____ and/or _____
A

adoptive transfer

transplantation

48
Q

Treatment of Immunodeficiencies:

-____ ____ w/ pooled ___ ___ is enormously valuable for agammaglobulinemic patients and have been life saving for many

A 
passive immunization
gamma globulin (IgG)
49
Q

Treatment of Immunodeficiencies:
-___ ____ ____ is currently the treatment of choice for various immunodeficiency diseases and has been successful in treatment of ___ and other similar diseases

A

bone marrow transplants

SCID

50
Q

Viruses:

  • must infect the ____ cell
  • -HIV can get into a cell via ____ ____
A

host

chemokine receptor

51
Q

Progression of HIV disease:

  1. primary infection of cells in ____, ____
  2. infection established in ____ ___ (lymph node)
  3. acute HIV syndrome= spread of infection throughout body (_____)
  4. immune response- partial control of viral replication
  5. clinical latency= establishment of chronic infection–virus trapped in lymphoid tissues by follicular dentric cells; low level virus production–>increaed viral replication—->AIDS (destruction of lymphoid tissue; depletion of CD4+ T cells)
A

blood, mucosa
lymphoid tissues
viremia

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