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SEM 1 > SEM 1 - (CC) LIVER FUNCTION TEST 2 > Flashcards

SEM 1 - (CC) LIVER FUNCTION TEST 2 Flashcards

1
Q

2nd liver function test

A

TEST MEASURING CONJUGATION AND EXCRETION FUNCTION

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2
Q

One of the most important functions of the liver is the excretion of endogenous and exogenous substances into the ____ or ____ such as the major heme product, ______

A

bile or urine ; bilirubin.

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3
Q

The _____ is the only organ that has the capacity to rid the body of heme waste products.

A

liver

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4
Q

hence ______ measures the capacity of the liver to conjugate and excrete.

A

bilirubin

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5
Q

the end product of hemoglobin metabolism

A

Bilirubin

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6
Q

principal pigment in bile

A

Bilirubin

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7
Q

Bilirubin is also formed from destruction of heme-containing proteins such as ______, ________, and ________

A

myoglobin, catalase, and cytochrome oxidase.

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8
Q

WHAT TYPE OF BILIRUBIN?

Water-insoluble
Non-polar bilirubin
Indirect reacting
Hemobilirubin
Slow reacting
Prehepatic bilirubin

A

Unconjugated Bilirubin

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9
Q

WHAT TYPE OF BILIRUBIN?

Water-soluble
Polar bilirubin
Direct reacting
Cholebilirubin
One minute/Prompt Bilirubin
Posthepatic bilirubin/Hepatic Bilirubin/Obstructive and Regurgitative Bilirubin

A

Conjugated Bilirubin

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10
Q

Ref Range of conjugated bilirubin

A

0-0.2 mg/dL (0-3 umol/L)

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11
Q

Ref Range of unconjugated bilirubin

A

0.2-0.8 mgdL (3-14 umol/L)

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12
Q

Ref Range of Total Bilirubin

A

0.2-1.0 mg/dL (3-17 umol/L)

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13
Q

(BILIRUBIN METABOLISM)

Red cell lysis after 120 days separates the ___ and ____

A

heme and globin

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14
Q

(BILIRUBIN METABOLISM)

heme is converted to unconjugated bilirubin in __ hours after lysis, the globin is digested to ______ and reused in the body.

A

3 ; amino acids

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15
Q

(BILIRUBIN METABOLISM)

while the iron is bound to _______ and becomes part of the iron pool in the______ and ______

A

transferrin ; liver and bone marrow

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16
Q

(BILIRUBIN METABOLISM)

The ________ is insoluble in water and the only mechanism to remove it from the plasma is through
______ in the liver.

A

unconjugated bilirubin ; conjugation

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17
Q

(BILIRUBIN METABOLISM)

To reach the site of conjugation, the hemobilirubin binds to ______ and
transport it up to the ________, followed by dissociation of the
_______ before entering the core of the liver.

A

albumin ; hepatic sinusoid ; hemobilirubin

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18
Q

(BILIRUBIN METABOLISM)

The _________ of the hepatic cell is the actual site of conjugation, and the _______ is the common isoform of UDPGT that is involved in the process. Another set of carrier proteins brings the
hemobilirubin to the SER.

A

smooth endoplasmic reticulum (SER) ; UDPGTIA1

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19
Q

(BILIRUBIN METABOLISM)

The intracellular conjugation of
________ onto two sites of the bilirubin molecule confers negative charge to it, making conjugated bilirubin soluble in _____ phase.

A

glucuronic acid ; aqueous

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20
Q

(BILIRUBIN METABOLISM)

Only small amounts of cholebilirubin circulates in blood because of minor leakage of the _______ in directions
away from the formation and excretion of bile; only _______ can enter the bile .

A

hepatocytes ; cholebilirubin

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21
Q

(BILIRUBIN METABOLISM)

______ and the early segment of the colon are the major sites of deconjugation with minimal deconjugation in the duodenum,

and the colon enzymes from the indigenous flora catalyze the process, resulting in the formation of _______, also known as the ________.

A

Ileum ; urobilinogen ; stercobilinogen

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22
Q

(BILIRUBIN METABOLISM)

______ or ______ is the oxidized form of urobilinogen which imparts stool its color;

A

Stercobilinogen or urobilin

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23
Q

(BILIRUBIN METABOLISM)

however, once the feces is exposed to air, the color of the stool turns dark due to the conversion of residual ______ to ______

A

urobilinogen to urobilin.

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24
Q

(BILIRUBIN METABOLISM)

_______ is responsible for the normal color of the urine;

A

Urobilin

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25
Q

(BILIRUBIN METABOLISM)

most of the urobilinogen is excreted in the feces as _____ while the remaining is either eliminated as _____ or _____ in the urine.

A

stercobilin ; urobilinogen or urobilin

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26
Q

conjugated bilirubin tightly bound to albumin.

A

Delta Bilirubin

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27
Q

Delta Bilirubin has longer half-life (_________) than other forms of bilirubin (_______).

A

12-14days ; 2-4hours

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28
Q

It is formed due to prolonged elevation of conjugated bilirubin in the case of biliary obstruction.

A

Delta Bilirubin

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29
Q

helps in monitoring the decline of serum bilirubin following surgical removal of gallstones.

A

Delta Bilirubin

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30
Q

Delta Bilirubin reacts with __________, specifically in the direct bilirubin assay

A

diazo reagent

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31
Q

Delta Bilirubin is not calculated on _________ (≤14 days ).

A

neonatal patients

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32
Q

Delta Bilirubin calculation and ref. range

A

Calculation: T B DB + IB = Delta bilirubin

Ref. Range: < 0.2 mg/dL (<3 pmol/L)

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33
Q

The plasma concentration of bilirubin increases upon ______ and reaches its peak on the ____ day.

A

birth ; 5th

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34
Q

Bilirubin comes from the normal lysis of the erythrocytes with less contribution from heme-containing proteins and enzymes such as myoglobin, cytochrome, and catalase.

A

myoglobin, cytochrome, and catalase.

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35
Q

Albumin binding reduces _______, restricts bilirubin’s ability to leave the _______, and inhibits it from ______ and ______ in tissues.

A

glomerular filtration ; vascular space ; precipitating and accumulating

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36
Q

Bilirubin conjugation to its water-soluble form involves the dissociation of _______, which is a necessary step in its disposal by the liver and the kidneys.

This is accomplished by _______ conjugation of the bilirubin’s propionic acid side chains.

A

hydrogen bonds ; glucuronic acid

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37
Q

The intestinal mucosa does not reabsorb conjugated bilirubin due to its __________ and _________

A

hydrophilicity and large molecular size

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38
Q

If the rate of bilirubin formation exceeds the rate of liver clearance (i.e., state of overproduction of bilirubin), there will be a rise in the bilirubin level in ______

A

serum.

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39
Q

Jaundice is also called ____ or _____

A

icterus or hyperbilirubinemia.

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40
Q

characterized by yellow discoloration of the skin, sclerae, and mucus membranes.

A

Jaundice

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41
Q

Jaundice is clinically evident when bilirubin level exceeds ________

A

3 mg/dL

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42
Q

3 classification of Jaundice or hyperbilirubinemia:

A

Pre-hepatic Jaundice or Hemolytic Jaundice

Post-hepatic Jaundice or Obstructive Jaundice

Hepatocellular Combined Jaundice or Hepatic Jaundice

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43
Q

Cause: Too much destruction of red blood cells (premature RBC lysis)

Clinical cases: Malaria, hemolytic anemia, and hemolytic disease of the newborn

Serum Bilirubin: Elevated B1

Urine Bilirubin: Negative

Urine Urobilinogen: Normal or increased

A

Pre-hepatic Jaundice or Hemolytic Jaundice

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44
Q

Cause: Failure of bile to flow or reach the intestine

Clinical cases: Cholelithiasis, bilateral pancreatic tumor, and colon parasitism

Serum Bilirubin: Elevated B2

Urine Bilirubin: Positive

Urine Urobilinogen: Decreased or Undetectable

A

Post-hepatic Jaundice or Obstructive Jaundice

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45
Q

Cause: Hepatocyte injury caused by viruses, alcohol, and parasites

Clinical cases: Cirrhosis, viral hepatitis, toxic hepatitis, and fascioliasis

Serum Bilirubin: Elevated B1 and B 2

Urine Bilirubin: Positive

Urine Urobilinogen: Decreased or Undetectable

A

Hepatocellular Combined Jaundice/Hepatic Jaundice

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46
Q

Derangements of Bilirubin Metabolism

A
  1. Gilbert’s Syndrome/Bilirubin Transport Deficit
  2. Crigler-Najjar Syndrome/Conjugation Deficit Syndrome
  3. Dubin-Johnson Syndrome/Bilirubin Excretion Deficit
  4. Lucey-Driscoll Syndrome/Conjugation Inhibitor Syndrome
  5. Rotor Syndrome/Mixed Hyperbilirubinemia
  6. Kernicterus: Bilirubin-Induced Brain Dysfunction
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47
Q

characterized by impaired cellular uptake of bilirubin

A

Gilbert’s Syndrome/Bilirubin Transport Deficit

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48
Q

Gilbert’s Syndrome/Bilirubin Transport Deficit is characterized by a molecular deficit within the gene involved in bilirubin metabolism such as the insertion of 2 bases into the promoter region of the _____ gene that encodes for UDGPT, resulting in lower transcriptional rates and diminished enzymatic activity.

A

UGT1A1

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49
Q

Gilbert’s Syndrome/Bilirubin Transport Deficit is diagnosed in _______

A

young adults ; 20-30 years old

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50
Q

In Gilbert’s Syndrome/Bilirubin Transport Deficit, affected individuals may have no symptoms but may have _________

A

mild icterus

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51
Q

In Gilbert’s Syndrome/Bilirubin Transport Deficit, the serum bilirubin is

A

Elevated B1 (< 3 mg/dL)

52
Q

a derangement of bilirubin metabolism characterized by the absence of UDGPT

A

Crigler-Najjar Syndrome/Conjugation Deficit Syndrome

53
Q

has been observed that multiple mutations in UGT1A1 gene, resulting in formation of dysfunctional proteins, which may lead to this abnormal condition.

A

Crigler-Najjar Syndrome/Conjugation Deficit Syndrome

54
Q

Crigler-Najjar Syndrome/Conjugation Deficit Syndrome is common among ________ and the treatment is by ______

A

infants ; phototherapy

55
Q

In Crigler-Najjar Syndrome/Conjugation Deficit Syndrome, the serum bilirubin is

A

Elevated B1

56
Q

Two types of Crigler-Najjar Syndrome/Conjugation Deficit Syndrome

A
  1. Type 1 Crigler-Najjar Syndrome
  2. Type 2 Crigler-Najjar Syndrome
57
Q

A type of Crigler-Najjar Syndrome with Absolute deficiency of the enzyme UPDGT

A

Type 1

58
Q

In type 1 Crigler-Najjar Syndrome, there’s a _____ of B2 production

A

total absence

59
Q

distinct feature of type 1 Crigler-Najjar Syndrome

A

(+) kernicterus
bile is colorless

60
Q

a type of Crigler-Najjar Syndrome that is characterized by partial deficiency of UDPGT and theres a small amount of B2 is produced

A

Type 2 Crigler-Najjar Syndrome

61
Q

associated with the blockade of the excretion of bilirubin into the canaliculi caused by a hepatocyte membrane defect

A

Dubin-Johnson Syndrome/Bilirubin Excretion Deficit

62
Q

In Dubin-Johnson Syndrome/Bilirubin Excretion Deficit, the defect in the canalicular organic anion transport, contributes to ________, specifically the mutation in the human _____ gene.

A

pathogenicity ; cMOAT

63
Q

Dubin-Johnson Syndrome/Bilirubin Excretion Deficit is observed with ________ and the total bilirubin level is not exceeding __mg/dL.

A

mild jaundice ; 5 mg/dL

64
Q

distinct feature and serum bilirubin of Dubin-Johnson Syndrome/Bilirubin Excretion Deficit

A

Distinct feature: Intense dark pigmentation of the liver due to accumulation of lipofuscin pigment

Serum bilirubin: Elevated B2

65
Q

a familial form of unconjugated hyperbilirubinemia (transient familial hyperbilirubinemia) and may be caused by a circulating inhibitor of bilirubin conjugation in the baby’s or mother’s blood

A

Lucey-Driscoll Syndrome/Conjugation Inhibitor Syndrome

66
Q

Lucey-Driscoll Syndrome/Conjugation Inhibitor Syndrome may also be due to a genetic change in the _____ gene enhancing _______

A

UGT1A1 ; hyperbilirubinemia.

67
Q

an autosomal recessive disease ; it is an inherited disorder where the exact cause is unknown though it may be caused by having mutations in both the SLCO1B1 and SLCO1B3 genes

A

Rotor Syndrome/Mixed Hyperbilirubinemia

68
Q

Rotor Syndrome/Mixed Hyperbilirubinemia may also be a defect of ________ of conjugated bilirubin.

A

sinusoidal reuptake

69
Q

serum bilirubin and distinct feature of Rotor Syndrome/Mixed Hyperbilirubinemia

A

Serum bilirubin: ElevatedB1 and B2 (conjugated bilirubin is prominent)

Distinct feature: (+) Porphyrinuria

70
Q

a derangement of Bilirubin Metabolism that is due to immature blood brain barrier among infants and is characterized byelevated unconjugated bilirubin

A

Kernicterus: Bilirubin-Induced Brain Dysfunction

71
Q

type of brain damage that can result from high levels of bilirubin in a baby’s blood

A

Kernicterus: Bilirubin-Induced Brain Dysfunction

72
Q

Kernicterus: Bilirubin-Induced Brain Dysfunction can cause _____ and _____ also creates problems with ________, _______ and ________.

A

athetoid cerebral palsy and hearing loss.

vision and the teeth, and intellectual disabilities

73
Q

When B1 rises in neonates due to deficient ________, it cannot be processed and bilirubin may be deposited in the nuclei of the brain and degenerative nerve cells producing kernicterus.

A

UDPGT

74
Q

A fetus with hemolytic disease does not develop ___________ because the placenta normally removes the bilirubin.

A

hyperbilirubinema (jaundice)

75
Q

Some of the bilirubin may appear in the amniotic fluid, and monitoring the concentration by spectrophotometer at _________ provides a means of determining the degree and progression of fetal hemolytic disease.

A

450 nm wavelength (Liley’s test)

76
Q

The ____ capacity of albumin for unconjugated bilirubin is the basic defense for prevention of kernicterus in HDN and infantile jaundice.

A

binding

77
Q

Kernicterus, as seen in Crigler-Najjarsyndrome, is the deposition of bilirubin in the brain, particularly affecting the ______, mainly the _______, causing severe _______ and _______.

A

basal ganglia ; lenticular nucleus ; motor dysfunction and retardation

78
Q

_____________ may cause the blood-brain barrier resulting in kernicterus.

A

Unbound bilirubin (free bilirubin)

79
Q

Measurement of ____ is a sensitive and specific marker for hepatic and post-hepatic jaundice because it is not elevated in hemolytic anemia.

A

B2

80
Q

In adults, _____________ is the most common cause of hyperbilirubinemia

A

cholelithiasis (presence of gallstones)

81
Q

In biliary obstruction, ________ will re-enter the circulation and accumulate and spontaneously form a covalent bond with albumin, thereby prolonging its half-life in the circulation.

A

conjugated bilirubin

82
Q

Obstruction of the biliary tract also causes elevated _________ and is associated with the normal excretion of cholesterol in ____.

A

total cholesterol ; bile

83
Q

______ injury causes loss of conjugation of transported bilirubin, so that unconjugated bilirubin also rises in plasma.

A

Hepatocyte

84
Q

In _________, because insufficient viable liver tissue remains, and because fibrosis destroys the cholangioles, both indirect and direct bilirubin tend to be elevated

A

panhepatic cirrhosis

85
Q

_______ is caused by infection with the hepatitis D virus (HDV) and is considered to be the most severe form of viral hepatitis in humans a s it occurs only in individuals positive for the ________

A

Hepatitis D ; HBV surface antigen(HBsAg).

86
Q

a defective RNA viroid that requires HBsAg for transmission.

A

HDV

87
Q

In Rotor syndrome, caused by a mutation in the SLC01B1 and SLCO1B3 genes, block in the excretion of conjugated bilirubin may occur where both forms of bilirubin is increased in plasma, but the serum level of ___ is higher than ___

A

B2 ; B1

88
Q

_______ should be avoided since it may affect the reaction of bilirubin with the diazo reagent, causing: false decrease.

A

Hemolysis

89
Q

The sample should be protected from ___________ as it would cause significant reduction in the actual concentration of bilirubin and it should be analyzed within ______
after collection.

A

light exposure (fluorescent lamp, indirect and direct sunlight) ; 2 to 3 hours

90
Q

Visible icterisia occurs when bilirubin is ______

A

25 mg/L.

91
Q

Principle: Van den Bergh reaction is the diazotization of bilirubin to produce azobilirubin.

A

Bilirubin Assay

92
Q

Bilirubin Assays

A

Evelyn and Malloy Method
Jendrassikand Grof

93
Q

Coupling Accelerator: Methanol

Main reagent: Diazo reagents
Diazo A 0.1% Sulfanilic Acid + HCI Diazo B = 0.5% Sodium Nitrite
1.5% HCI

Final reaction: Pink to purple azobilirubin at 560nm

A

Evelyn and Malloy Method

94
Q

Main reagent in Evelyn and Malloy Method is Diazo reagent what are those?

A

Diazo A 0.1% Sulfanilic Acid + HCI

Diazo B = 0.5% Sodium Nitrite

Diazo Blank = 1.5% HCI

95
Q

a bilirubin assay that is most commonly used method and is popular technique for the discreet analyzers.

A

Jendrassik and Grof

96
Q

a bilirubin assay that is more sensitive compared to Evelynand Malloy method.

A

Jendrassik and Grof

97
Q

Jendrassik and Grof is not affected by ___ changes and the presence of hemoglobin is up to _____

A

pH ; 750 mg/dL

98
Q

Jendrassik and Grof main reagent

A

Diazo reagent

99
Q

Jendrassik and Grof coupling accelerator

A

caffeine sodium benzoate

100
Q

Jendrassik and Grof buffer

A

sodium acetate

101
Q

In Jendrassik and Grof, __________ Terminates the initial reaction and destroys the excess diazo reagent

A

Ascorbic acid:

102
Q

In Jendrassik and Grof, _______ Provides an alkaline pH

A

Alkaline tartrate

103
Q

Final reaction In Jendrassik and Grof:

A

Blue azbilirubin at 600 nm

104
Q

The purpose of adding a methanol or caffeine benzoate solution is to allow indirect bilirubin to react (solubilize) with the _______.

A

color reagent

105
Q

________ is preferred over _____ because the latter promotes protein precipitation and increases turbidity.

A

Caffeine-benzoate ; methanol

106
Q

Conjugated bilirubin produces color in ______ solution.

A

aqueous

107
Q

In the measurement of the total bilirubin, the unconjugated fraction produced a color only after the addition of the ______

A

accelerant

108
Q

Total bilirubin is measured ______ after adding methanol or caffeine solution.

A

15 minutes

109
Q

Bilirubin absorbs light maximally at _____ and imparts a yellow color to ________

A

450nm ; amniotic fluid.

110
Q

disease associated with increased B1

A
  1. Gilbert’s Syndrome
  2. Crigler-Najjar Syndrome
  3. Hemolytic anemia
  4. Hepatocellular disease
  5. Lucey Driscoll Syndrome
  6. G-6-PD deficiency
111
Q

disease associated with increased B2

A

1 . Biliary obstruction (gall stones)
2 . Pancreatic (head) cancer
3. Dubin-Johnson Syndrome
4. Alcoholic and viralhepatitis
5. Biliary atresia
6. Hepatocellular disease
7. Septicemia
8. Rotor syndrome

112
Q

a colorless end product of bilirubin metabolism that is oxidized by intestinal bacteria to the brown pigment stercobilin (urobilin).

A

Urobilinogen

113
Q

Urobilinogen is either excreted in ____ and _____ or reabsorbed in to the ______ and returned to the _____.

A

urine and feces, ; portal blood ; liver

114
Q

Absence of this substance in urine or stool denotes complete biliary obstruction

A

Urobilinogen

115
Q

In Urobilinogen, the collection of the sample, avoid exposure to ______

A

direct light.

116
Q

In Urobilinogen, the sample preparation the sample should be collected ______ after a meal (preferably ____ meal)

A

2 hours ; noon

117
Q

Presence of drugs in urobilinogen (methyldopa and nitrofurantoin)

A

False increased

118
Q

If urobilinogen was exposed to light and air.

A

False decreased

119
Q

method in urobilinogen

A

Ehrlich’s method (p-dimethyl aminobenzaldehyde reagent)

120
Q

ref range of urobilinogen in urine

A

Urine = *0.3-1.0Ehrlich units/2hour or 0.5-4 Ehrlichunits/day

121
Q

ref range of urobilinogen in feces

A

F e c e s = *80-280 Ehrlich units/24 hours

122
Q

A test for hepatocellular function and potency of bile duct, however, rarely used

A

Bromsulphthalein (BSP) Dye Excretion Test

123
Q

It determines the ability of protein albumin to transport the exogenous dye to the liver where it is excreted in the bile.

A

Bromsulphthalein (BSP) Dye Excretion Test

124
Q

Bromsulphthalein (BSP) Dye Excretion Test procedure

A

BSP dye is administered intravenously.

125
Q

Dose: 5 mg/kg body weight of the patient

Sample Collection: After 45 minutes of IV dose

Normal/Expected Results: After 45 minutes: +/-5% dye retention

A

Mac Donald
(Single Collection Method)

126
Q

Dose: 2 mg/kg body weight of the patient

Sample Collection: After 5 minutes and after 30 minutes of IV dose

Normal/Expected Results:
After 5 minutes: 50% dye retention After 30 minutes: 0% dye retention

A

Rosenthal W hite (Double Collection Method)

SEM 1 (4 decks)

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