AMINOACIDOPATHIES.3 Flashcards

1
Q

_____: a2-glycoprotein synthesized in hepatocytes

  1. used to evaluate _____
  2. reported as independent risk factor for _______ with ______
  3. function:
    - binds _____ to prevent loss of hgb
    - used primarily to help detect and evaluate _____
    - evaluate degree or intravascular hemolysis that has occurred in _____ or _____
A

HAPTOGLOBIN

  1. RHEUMATIC DISEASE
  2. CVD; TYPE 2 DM
  3. function:
    - FREE HGB
    - HEMOLYTIC ANEMIA
    - TRANSFUSION RXN or HEMOLYTIC DISEASE OF NEWBORN
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2
Q

_____: copper containing a2 glycoprotein enzyme synthesized by liver

  1. ___ or more of total serum copper is found in ceruplasmin; ___% is bound to albumin
  2. _____ inherited disorder associated with decreased levels
    - typically ____
  3. copper also deposit is in cornea producing characteristic of ______
  4. decreased causes what syndrome
  5. ceruplasmin determination (3)
A

CERULOPLASMIN

  1. 90%; 10%
  2. AUTOSOMAL RECESSIVE
    - 0.1g/L
  3. KAYSER-FLEISCHER SYNDROME
  4. MENKES SYNDROME (KINKY HAIR DISEASE)
    5.
    - COPPER OXIDASE ACTIVITY
    - RADIAL IMMUNODIFFUSION
    - NEPHELOMETRY
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3
Q

______: Major component of beta globulin fraction and appears distinct bond on HRE

  1. iron bound to transferrin is _____ (__mg) of total body iron, most important iron pool
A

TRANSFERRIN

  1. 0.1% (4mg)
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4
Q

______: migrates electrophoretically in beta globulin region

  1. ____ cells
  2. induces _____ activities
  3. increased (4)
  4. decreased (1)
A

HEMOPEXIN

  1. PARENCHYMAL cells
  2. INTRACELLULAR ANTIOXIDANT activities
  3. INFLAMMATION, DM, DUCHENNE TYPE MUSCULAR DYSTROPHY, MELANOMAS
  4. DIAGNOSTIC HEMOLYTIC ANEMIA
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5
Q

_______: light chain component of major histocompatibility complex (HLA)

  1. found on surfaces of most _______
  2. present in high conc of _____
  3. increased
A

B2-MICROGLOBULIN

  1. NUCLEATED CELLS
  2. LYMPHOCYTES
  3. RHEUMATOID ARTHRITIS & SLE
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6
Q

______: natural defense mechanism that protects human body from infections

  1. ____ most abundant complement protein in human serum
  2. 2nd most abundant because of its pivotal position
  3. decreased C3 (5)
  4. decreased C4 (4)
A

COMPLEMENT

  1. COMPLEMENT C3
  2. COMPLEMENT C4
  3. ABC NT = Autoimmune disease, Bacteremia, Chronic hepatitis, Neonatal Respiratory Distress, Tissue injury
  4. DACS = DIC, Acute glomerulonephritis, Chronic hepatitis, SLE
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7
Q

_______: one of largest proteins in blood plasma
1. Distinct band between ____ & _____
2. Forms a ____ when activated by ______

A

Fibrinogen
1. Beta and Gamma globulins
2. Fibrin clot, thrombin

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8
Q

______: first acute phase proteins to rise in response to inflammatory disease

  1. received its first na,e because it precipitates with ____, a polysaccharide of pneumococci
  2. CRP bound to ______ and ______ promotes binding of complement
  3. result above ____ is usually considered high
  4. most infections & inflammations result in CRP levels above _____
  5. in cases of inflammatory rheumatic disease, such as _____ & _____
A

C REACTIVE PROTEIN
1. C SUBSTANCE
2. BACTERIA and FUNGI
3. >1mg/dL
4. >10mg/dL
5. RHEUMATOID ARTHRITIS and SLE

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9
Q

IG

  1. composed of _____ proteins
  2. composed of ______ carbohydrates
  3. produced by wbc known as ____ (_____ immunity)
  4. two identical ____ & _____
  5. ratio of k and gamma
A
  1. 82-96%
  2. 4-18%
  3. B CELLS (HUMORAL IMMUNITY)
  4. HEAVY & LIGHT
  5. 2:1
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10
Q

____: acts on bacteria, fungi, viruses, and foreign particles by agglutination and opsonization

  1. most abundant class of Ab found in _____ and ______
  2. Increased in ______, ______,____,_____ and _____
  3. Decrease in _____, _______, ______, _____ and ______
A

IgG
1. BLOOD PLASMA and LYMPH
2. INCREASED
- Liver Disease
- Infections
- IgG Myeloma
- Parasitic Disease
- Rheumatic Disease
3.DECREASED
- Acquired Immunodeficiency
- Protein Losing States
- Non-IgG Myeloma

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11
Q

____: resistant to enzyme degradation and remains active in digestive and respiratory tracts to provide antibody protection in body secretions

  1. found on
  2. Increased in (3)
  3. Decreased in (2)
A

IgA
1. Mucous secretions
2. INCREASED:
- Liver Disease
- Infections
- Autoimmune disease
3. DECREASED:
- Depressed Protein Synthesis
- Immunodeficiency

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12
Q

____: first to appear in response to antigenic stimulation

  1. monoclonal increase is seen in _______
    - spike in vicinity of late _____ zone on protein electrophoresis
  2. Increased in (6)
  3. Present in all _____
A

IgM

  1. WALDENSTROM MACRO
    - B ZONE
  2. TOXOPLASMIS, PRIMARY BILIARY CIRRHOSIS, CYTOMEGALOVIRUS, RUBELLA, HERPHES, SYPHILIS, and various bacterial and fungal diseases
  3. B CELLS
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13
Q

____: help regulate B cell function

  1. Present on surface of most but not all
  2. Increased in (3)
A

IgD
1. B cells
2. Infections, liver diseases, CT disorders

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14
Q

____: associated with allergic and anaphylactic reactions

  1. Monoclonal increases are seen in ____, a rare disease
  2. Increased in (2)
A

IgE
1. IgE myeloma
2. Inflammatory Infectious Diseases inc ASTHMA and HAY FEVER

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