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Clinical Science > Serum Proteins > Flashcards

Serum Proteins Flashcards

1
Q

What are the three main types of plasma proteins?

A
  • albumins
  • fibrinogen
  • globulins
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2
Q

What are the different globulins?

A
  • alpha and beta globulins
  • gamma globulins (Ig)
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3
Q

What are the different alpha and beta globulins?

A

alpha-1 globulins
- alpha-1-antitrypsin
alpha-2 globulins
- haptoglobin
beta-globulins
- transferrin, C3 and C4 complement

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4
Q

What are gamma globulins produced by?

A

lymphoid tissue and major component of the immune system

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5
Q

What are the different gamma globulins?

A
  • IgG
  • IgM
  • IgA
  • IgD
  • IgE
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6
Q

What plasma protein inhibits the action of many key enzymes that are released during inflammatory reactions in the lungs?

A

alpha-1-antitrypsin

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7
Q

When a pt is deficient or has abnormal alpha-1-antitrypsin, what can this lead to?

A

early-onset lung disease (COPD)

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8
Q
  • When alpha-1-antitrypsin is diminished, these enzymes are no longer inhibited, and (…) is damaged
  • Can also cause (…) due to toxic accumulation of mutant alpha-1-antitrypsin in the cells of the (…)
A
  • lung parenchyma
  • liver disease
  • liver
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9
Q

In what type of deficiency are the lungs not protected from the harmful effects of neutrophil elastase, resulting in damage to the lung parenchyma? (it can also result in liver toxicity as well and pt may have elevated liver function tests)

A

alpha-1-antitrypsin deficiency

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10
Q

Decreased/deficiency alpha-1-antitrypsin manifestations include COPD in adults that have an onset that often occurs when?

A

occurs before the age of 40

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11
Q
  • Who should you evaluate for an A1AT deficiency?
  • What should you ask these individuals?
A
  • any young pt who manifests signs of COPD at an early age
  • never-smokers or light smokers with COPD
  • Is there a family history of COPD? Liver disease?
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12
Q

What other manifestation is there other than COPD with A1AT deficiencies? What are the symptoms?

A

liver disease
- prolonged jaundice or hepatitis is infants
- chronic hepatitis
- cirrhosis
- hepatocellular carcinoma

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13
Q

What accounts for <1% of COPD cases in the US (earlier onset)?

A

A1AT deficiency

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14
Q
  • A1AT deficiency results from a mutation in which gene?
  • How is it inherited?
  • What are the different alleles that exist?
  • What combination of alleles causes the worst deficiency?
A
  • SERPINA1 gene
  • codominant manner (more than 2 alleles)
  • M (normal), Z (deficient), S (deficient)
  • ZZ
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15
Q

What is synthesized mainly in the liver and is composed of amino acids which are gained through proper diet intake?

A

prealbumin

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16
Q
  • What does prealbumin function as a transport protein for?
  • What is this called?
A
  • thyroxine and vitamin A
  • transthyretin
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17
Q

What can be used in conjunction with albumin (on metabolic panel) in the assessment of patient’s nutritional status?

A

prealbumin

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18
Q

What type of half-life does prealbumin have in comparison to albumin?

A
  • shorter half life
  • may be more sensitive to rapid changes in nutrition
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19
Q

Prealbumin is not included in any panel and must be (…)

A

ordered independently

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20
Q

Prealbumin may be falsely (…) in some patients, as its value falls during (…), even in an appropriately nourished individual

A
  • falsely low
  • inflammation and illness
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21
Q

Prealbumin values of what level indicate malnutrition?

A

<10 mg/dl

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22
Q

What increases prealbumin?

A
  • pregnancy
  • long term renal disease
  • steroid or alcohol use
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23
Q

What decreases prealbumin?

A
  • liver disease (decreased production)
  • eating disorders
  • malabsorption
  • malnutrition
  • severe infection, inflammation
  • low protein diets
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24
Q
  • Where is haptoglobin produced?
  • The function of haptoglobin is to bind to (…) when RBCs are destroyed?
  • From here, haptoglobin transports (…) back to the liver where heme is converted to (…)
A
  • liver
  • free hemoglobin
  • hemoglobin
  • bilirubin
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25
Q

If there is increased RBC destruction, what happens to haptoglobin?

A

haptoglobin becomes depletes and its levels decrease (because so much haptoglobin is being used, there is only a finite amount)

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26
Q

Haptoglobin is very useful when looking for signs of (…)

A

hemolytic anemia

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27
Q

Haptoglobin is an (…)

A

acute phase reactant

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28
Q

What increases haptoglobin levels?

A
  • infection
  • inflammation
  • neoplastic disease
  • pregnancy
  • trauma
  • acute MI
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29
Q

What decreases haptoglobin levels?

A
  • hemolytic anemia
  • transfusion reaction
  • artificial heart valves (shear stress damage)
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30
Q

What supplement or “complement” the action of antibodies?

A

complement proteins

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31
Q

What is the goal of complement proteins?

A

to destroy and eliminate pathogens from the body

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32
Q
  • Where are complement proteins synthesized?
  • How many total proteins are there?
  • What do they act as?
A
  • the liver
  • 31 total
  • enzymes, cofactors, and inhibitors
33
Q
  • Activation of complement proteins can occur by what?
  • What do these pathways trigger?
A
  • presence of an antigen-antibody complex (classic pathway)
  • alternative pathway
  • lectin mannose pathway (certain organisms have mannose and glucose in cell wall)
  • trigger C1 which results in a cascade of reactions
34
Q

Deficiencies of complement proteins can result in what?

A

frequent disseminated, recurrent infections, especially with:
- S. pneumoniae
- N. meningitidis
- N. gonorrhea

35
Q
  • Complement levels rise as much as 50% with many (1) and (2) conditions
  • C3 and C4 are considered what?
A
  • infectious and inflammatory conditions
  • acute phase reactants
36
Q

Low complement proteins can be (…) or (…)

A

inherited or acquired

37
Q

Acquired complement protein loss can be seen in what conditions?

A
  • liver disease (factory shutting down) - alcoholic cirrhosis
  • renal disease (losing proteins during filtration)
  • autoimmune disease - immune complexes formed against own tissues triggers complement cascade
38
Q

What are some indications for complement testing?

A
  1. evaluating immune system in a pt w/ repeated infections, particularly aggressive bacterial infections
  2. monitor complement activity in autoimmune disease
39
Q

How can we monitor complement levels?

A
  • testing specific complement factors
  • performing CH50 test (entire complement system)
40
Q

What test measures the ability of human serum to lyse sheep RBCs that have been coated with antibodies (when 50% of cells are lysed)?

A

CH50 test

41
Q

What must be present in order to have a normal CH50 test result?

A

all nine complement proteins, C1- C9

42
Q
  • What does a decreased CH50 result mean?
  • What does a CH50 result of zero mean?
A
  • decreased complement activity
  • one of pathway components are totally absent
43
Q

If we have an undetectable CH50 level, what are most commonly ordered?

A

C3 and C4

44
Q

C3 and C4 are often used primarily in investigating what?

A
  • a pt with an undetectable CH50 level
  • some autoimmune diseases, such as lupus
45
Q

C3 and C4 can be tested to evaluate pts who have repeated/severe infections or appear to have an underlying poorly functioning immune system such as what type of infections?

A
  • severe fungal infections
  • gram-negative sepsis
46
Q

C3 and C4 tests can be included when?

A
  • initial screening for complement abnormality
  • after an abnormal CH50 result
47
Q

What leads to decreased C3,C4 complement protein levels?

A
  • systemic lupus erythematosus (body constantly targeting itself)
  • cirrhosis and hepatitis (synthesis occurs in liver)
  • complement deficiencies
48
Q

What leads to increased C3,C4 complement protein levels (due to being APR)?

A
  • cancer
  • acute infectious processes
49
Q

What are the most significant of the gamma globulins?

A

immunoglobulins (antibodies)

50
Q

What are antibodies developed to target?

A

specific foreign invaders, specifically bacteria, viruses and toxins

51
Q
  • What are immunoglobins produced by?
  • What do they develop to become very specific against?
A
  • B lymphocytes
  • individual infectious or foreign agents
52
Q
  • Which immunoglobin is usually found in secretions and along the mucosal epithelium?
  • What is this the defender of?
  • What does it allow for?
A
  • IgA
  • defender of mucosal or epithelial surfaces against pathogens
  • allows for clearance of pathogens by cilia or toxins in the GI tract
53
Q

Where is IgA present?

A
  • saliva
  • tears
  • colostrum
  • mucus
    think secretions, or “secretory IgA”
54
Q

What symptoms can an IgA deficiency cause?

A
  • no symptoms
  • frequent respiratory infections
  • inflammation of GI tract
  • unexplained asthma symptoms
55
Q

Which immunoglobulin has an exact function that is unknown and clinical significance is uncertain?

A

IgD

56
Q
  • IgD represents only about what percent of circulating immunoglobulins?
  • What is IgD found to activate?
  • Concentration of IgD increases with what?
A
  • 0.25%
  • basophils and mast cells
  • chronic infections
57
Q

Higher levels of IgD have been seen with multiple infections such as?

A
  • leprosy
  • TB
  • malaria
  • AIDS
  • hepatitis
  • staph infections
58
Q

Which immunoglobulin is the key factor in allergic reactions and parasitic infections?

A

IgE

59
Q

What are examples of where IgE is involved?

A
  • allergic asthma
  • allergic rhinitis
  • food allergies
  • atopic dermatitis
  • some forms of drug allergy
  • insect sting allergy
  • helminth (parasitic worm) infections
60
Q

What does IgE bind to and what does this initiate?

A

binds to mast cells which initiates a chain of immune responses

61
Q

Increased total IgE levels mean what?

A

a person likely has allergies

62
Q
  • What test can determine specific IgE antibodies against allergens, such as dog, cat, mold, dust mites, and common foods?
  • What does this test also provide?
  • What can you use this test in place of?
A
  • immunocap test (blood test)
  • total IgE test
  • in place of a skin allergy test
63
Q
  • What is the major antibody produced when an antigen is encountered?
  • What is the initial antibody secreted after an immune challenge with half-life of 10 days?
A
  • IgG
  • IgM
64
Q

What is the most prevalent antibody in serum and has the longest half-life of all immunoglobulins (23 days)?

A

IgG

65
Q

What immunoglobulin can persist and be detected for life in some cases? What infection does this happen in?

A
  • IgG
  • mononucleosis
66
Q
  • What is IgG responsible for?
  • What can IgG cross so that the fetus can be protected from infection?
A
  • immunity to bacteria and other organisms
  • crosses the placenta
67
Q

What does an increased IgM antibody usually indicate?

A

a recent infection

68
Q

Which antibody shows up first in an infection? Which one shows up later?

A
  • IgM
  • IgG
69
Q

IgG and IgM testing are commonly used when testing for what?

A
  • Epstein Barr Virus
  • Cytomegalovirus
  • Herpes I/II
  • Varicella
  • Measles, Mumps, and Rubella
70
Q

What are some indications for serum protein electrophoresis?

A
  • detecting some forms of cancer/pre-cancer
  • immune abnormalities
  • kidney/liver dysfunction
71
Q

What makes up 10% of all hematologic cancers? How are the survival rates?

A
  • multiple myeloma
  • poor survival rates
72
Q

Multiple myeloma is a neoplastic disorder which causes proliferation of a monoclonal immunoglobulin, usually (1) and (2)

A
  1. IgG
  2. IgA
73
Q
  • In multiple myeloma, clones of a single structurally-identical antibody multiplies rapidly and now becomes labelled the (…)
  • The proliferation of these clones end up infiltrating organs such as (…)
  • This can also happen in other (…)
A
  1. M protein (monoclonal protein)
  2. the bones, and cause end-organ damage
  3. similar premalignant disorders
74
Q

What is useful in identifying pts with multiple myeloma and other potentially malignant disorders?

A

serum protein electrophoresis

75
Q
  • Electrophoresis is used to separate the (…) based on their physical properties
  • After separation, a special machine uses a gel to produce a tracing that indicated the level of each (…)
  • This test can be done with (…)
A
  • plasma proteins
  • proteins
  • serum or urine
76
Q
  • What is the largest component of serum and represents the largest peak that is closest to the positive electrode is the protein electrophoresis?
  • What are smaller proportions but are where the focus lies the most?
A
  • albumin
  • globulins
77
Q

What are the peaks from positive to negative in the protein electrophoresis?

A
  • albumin (largest)
  • alpha 1
  • Alpha 2
  • beta
  • gamma (Ig’s)
78
Q
  • In a protein electrophoresis, if there is a big spike in the gamma portion, what is this called?
  • Where else can these occur?
  • What is this protein associated with?
  • What does this possibly signify?
A
  • M spike (M protein - monoclonal)
  • alpha 2 or beta region
  • plasma cell disorder
  • a neoplastic process, such as multiple myeloma
79
Q

What are some of the conditions associated with M protein on protein electrophoresis?

A
  • multiple myeloma (most common)
  • chronic lymphocytic leukemia
  • any B- or T- cell lymphomas
  • breast cancer
  • colon cancer
  • cirrhosis

Clinical Science (18 decks)

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