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1 Path Unit > lecture 12 > Flashcards

lecture 12 Flashcards

1
Q

What form do the misshapen proteins take on during amyloidosis?

A

beta pleated sheets.

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2
Q

If you find unexplained kidney, heart, and brain problems what should you think?

A

Amyloidosis

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3
Q

How do you stain amyloid?

A

congo red

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4
Q

What happens to the amyloid appearance when you add polarized filters?

A

you see and apple-green birefringence.

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5
Q

Multiple myeloma and other B cell abnormalities are associated with the major febrile protein_________?

A

AL protein

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6
Q

Chronic inflammatory conditions are associated with the major febrile protein_________?

A

AA protein

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7
Q

Chronic renal failure is associated with the major febrile protein _______?

A

AB2m protein (B=Beta)

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8
Q

Alzheimers disease is associated with the major febrile protein_______?

A

AB protein (B=Beta)

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9
Q

Prion diseases is associated with the major febrile protein ________?

A

PrPsc (AKA miss folded prion protein)

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10
Q

what is the precursor protein to AL protein in multiple myeloma?

A

Immunoglobulin light chains

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11
Q

what is the precursor protein to AA protein in chronic inflammatory diseases?

A

SAA protein

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12
Q

what is the precursor protein to AB2m in chronic renal failure?

A

B2 microglobulin

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13
Q

what is the precursor protein to AB in Alzheimers disease?

A

APP protein

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14
Q

what is the precursor protein to misfolded prion protein?

A

Normal prion protein

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15
Q

What are 6 major causes of secondary amyloidosis?

A 
SLE
Tuberculosis
Rheumatoid arthritis
Ulcerarive colitis
Tumors
Sarcoidosis
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16
Q

If you have a heart that has been affected by amyloid, what changes will you generally see?

A 
coloration changes (yellow/pinking plaques)
Size if the organ increases
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17
Q

T/F Amyloidosis can cause brown spots on the skin that look like a bruise?

A

TRUE

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18
Q

Dark and swollen legs, enlarged tongue, and purpura around the eyes are all characteristic of what ?

A

amyloidosis

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19
Q

What happens to vessels when they collect a large amount of amyloid within them?

A

they become prone to aneurysm.

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20
Q

Why do bleeding disorders often accompany amyloidosis?

A

1) difficult to repair damage to vessel with so much amyloid

2) Amyloid absorbs factor 10 preventing normal clotting.

21
Q

Nephrotic syndrome, organomegly, bleeding disorders, and intracerebral hemorrhage define which disease?

A

amyloidosis.

22
Q

Reasons to consider immunodeficiency include (6)?

A

1) persistent/frequent infections or inflammation
2) opportunistic infections
3) atypical autoimmunity
4) unexplained lymphadenopathy/splenomegly.
5) family history of immunodeficiency
6) congenital abnormality with symptoms (Ex: digeorge)

23
Q

Once you think that the person has an immunodeficiency, you will want to determine if it is B cell or T cell deficiency. What are some signs that it is a B cell deficiency (2)?

A

1) recurrent infections with extracellular encapsulated bacteria.
2) recurrent pulmonary disease (every winter getting pneumonia)

24
Q

Once you think that the person has an immunodeficiency, you will want to determine if it is B cell or T cell deficiency. What are some signs that it is a T cell deficiency (5)?

A

1) recurrent infections with opportunistic infections. (fungi, viruses, pneumocystis)
2) Potentially fatal reactions to live virus vaccines
3) susceptibility to GVH disease
4) growth retardation, wasting, diarrhea
5) increased malignancy

25
Q

Where is the best source of information on the latest up to date info from immune deficiencies?

A

The international union of immunological societies expert committee.

26
Q

What are the two types of severe combined immune deficiency?

A

1) XLR SCID2) AR SCID

27
Q

CD-40 ligand is on what cells?

A

T cells

28
Q

50% of the SCID patients have what type of SCID?

A

X-linked SCID

29
Q

With X-linked SCID, there is a defect in _________, that is shared by which interleukin receptors?

A

Common gamma chain.IL 2,4,7,9, & 15

30
Q

T/F the IL 7 receptor can cause SCID alone?

A

True but this is very rare.

31
Q

What is the most common cause of autosomal recessive SCID?

A

Adenosine deaminase deficiency.

32
Q

Besides the adenosine deaminase deficiency, what else can cause autosomal recessive SCID?

A

A JAK3 deficiency (Janus Kinase 3)

33
Q

What do you see in hyper IgM syndrome? What is the most likely cause of this?

A

1) A lot of IgM and no IgA, IgG, and IgE

2) A deficiency in the CD-40 ligand is most common.

34
Q

What major effect does CD-40 have on immunoglobulin production?

A

It promotes class switching.

35
Q

What causes digeorge syndrome?

A

underdeveloped thymus

36
Q

What is highly associated with digeorge syndrome?

A

congenital heart defects.

37
Q

Which cells are mainly affected in digeorge syndrome?

A

CD 8 T cells

38
Q

Which chromosome is deleted in digeorge syndrome?

A

22q11

39
Q

Which 5 things are associated with digeorge syndrome?

A 
CATCH 22
Cardiac defects
Abnormal faces
Thymic hypoplasia
Cleft palate
Hypocalcemia
40
Q

What causes Wiskott-Aldrich syndrome?

A

A mutated WAS gene on the X chromosome.

41
Q

What effects does a mutated WAS gene on the X chromosome have?

A

It messes up actin polymerization, and decreases response to polysaccharide antigens.

42
Q

What signs do you see with Wiskott-Aldrich syndrome?

A

T cells declined. (IgM down, IgA and IgE up)

43
Q

What is X linked agammaglobulinemia and what causes it?

A

1) An x linked disorder marked by decreased B cells and immunoglobulins.
2) It is caused by a defect in a gene coding for brutons tyrosine kinase (BTK)

44
Q

Which enzyme is critical for B cell development?

A

Brutons tyrosine kinase.

45
Q

Which disease is described by severe reduction of at least 2 serum immunoglobulins, and IgM will never be increased? (Note: relatives sometimes have IgA deficiencies)

A

Common Variable immunodeficiency

46
Q

which disease is described as the most common immune deficiency of people of northern European origin, B-cells and IgA are present, but IgA is in very low quantity, and most individuals are asymptomatic?

A

IgA deficiency

47
Q

Mutations in TACI and CVID genes can lead to which disease?

A

IgA deficiency

48
Q

What happens if you are unable to form IFN?

A

You cant form granulomas

1 Path Unit (16 decks)

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