Ch. 25 - Liver Function Alterations During Disease

Question 1

The word juandice comes from the French word _____, which means “yellow”.

Answer 1

Jaune

Question 2

The term used to describe the yellow discoloration of the skin, eyes, and mucous membranes most often resulting from the retention of bilirubin; however it may also occur due to retention of other substances.

Answer 2

Jaundice

Question 3

A type of jaundice that is not visible to the naked eye.

Answer 3

Overt Jaundice

Question 4

At what bilirubin levels will jaundice be visible?

Answer 4

3.0 - 5.0 mg/dL

Question 5

This term is most commonly used in the clinical laboratory to refer to serum or plasma sample with a yellow discoloration due to an elevated bilirubin level.

Answer 5

Icteric

Question 6

Enumerate the different classifications of jaundice based on the site of the infection.

Answer 6

Prehepatic
Hepatic
Posthepatic

Question 7

A type of jaundice that is due to a problem with the liver itself (an intrinsic liver defect or disease).

Answer 7

Hepatic jaundice

Question 8

This is a type of jaundice occurring prior to liver metabolism. Most commonly caused by an increased amount of bilirubin being presented to the liver (seen in acute and chronic hemolytic anemias)

Answer 8

Prehepatic jaundice

Question 9

This disease causes an increased amount of red blood cell destruction and the subsequent release of increased amounts of bilirubin presenting to the liver for processing.

Answer 9

Hemolytic anemia

Question 10

People that have prehepatic jaundice rarely have bilirubin levels that exceed ___ mg/dL because the liver is capable of handling the overload

Answer 10

5.0

Question 11

Prehepatic jaundice may also be referred to as _____ because the fraction of bilirubin increased in this disease is the _____ fraction.

Answer 11

Unconjugated hyperbilirubinemia

Unconjugated

Question 12

This fraction of bilirubin is:
Not water soluble
Bound to albumin
Not filtered by the kidneys 
Not seen in the urine

Answer 12

Unconjugated fraction

Question 13

This type of jaundice occurs when the primary problem causing the jaundice resides in the liver (Intrinsic liver defect or disease).

Answer 13

Hepatic jaundice

Question 14

These are disorders of bilirubin metabolism and transport defects that can cause hepatic jaundice.

Answer 14

Crigler-Najjar syndrome
Dubin-Johnson syndrome
Gilbert’s disease
Neonatal physiologic jaundice of the newborn

Question 15

These are hepatic causes of jaundice that result in elevations in unconjugated bilirubin.

Answer 15

Crigler-Najjar syndrome
Gilbert’s disease
Neonatal physiologic jaundice of the newborn

Question 16

These are hepatic causes of jaundice that result in elevations in conjugated bilirubin.

Answer 16

Dubin-Johnson syndrome

Rotor syndrome

Question 17

This is a benign autosomal recessive hereditary disorder that results from a genetic mutation in the gene UGT1A1.

Answer 17

Gilbert’s syndrome

Question 18

This gene is located on chromosome 2; it produces UDPGT. It is the hepatic 1A1 isoform of UDPGT

Answer 18

UGT1A1

Question 19

It is known as the most common cause of jaundice but has no morbidity or mortality in the majority of those affected and carries generally no clinical consequences.

Answer 19

Gilbert’s syndrome

Question 20

The total serum bilirubin of patients with Gilbert’s syndrome fluctuates between ___ and 3.0 mg/dL, and rarely exceeds___ mg/dL.

Answer 20

1.5; 4.5

Question 21

The liver’s conjugation system in this disease/syndrome is working at approximately 30% of normal.

Answer 21

Gilbert’s syndrome

Question 22

Described as a syndrome of chronic nonhemolytic unconjugated hyperbilirubinemia and an inherited disorder of bilirubin metabolism resulting from a molecular defect within the gene involved with bilirubin conjugation.

Answer 22

Crigler-Najjar syndrome

Question 23

A type of Crigler-Najjar syndrome where there is a complete absence of enzymatic bilirubin conjugation.

Answer 23

Type 1

Question 24

A type of Crigler-Najjar syndrome where there is a mutation causing a severe deficiency of the enzyme responsible for bilirubin conjugation.

Answer 24

Type 2

Question 25

Diseases that are characerized as primarily unconjugated hyperbilirubinemias.

Answer 25

Gilbert's syndrome | Crigler-Najjar syndrome

Question 26

Diseases that are characerized as primarily conjugated hyperbilirubinemias.

Answer 26

Dubin-Johnson syndrome | Rotor syndrome

Question 27

This syndrome is a rare autosmoal recessive inherited disorder caused by a deficiency of the canalicular multidrug resistance/multispecifc organic anionic transporter protein (MDR2/cMOAT).

Answer 27

Dubin-Johnson syndrome

Question 28

In this disease, the liver's ability to uptake and conjugate bilirubin is functional; however, the removal of conjugated bilirubin from the liver cell and the excretion into the bile are defective. Results in accumulation of conjugated and unconjugated bilirubin.

Answer 28

``` Dubin-Johnson syndrome Posthepatic jaundice (Bilirubin is conjugated, but not excreted properly) ```

Question 29

Conjugated bilirubin bound to albumin is referred to as _____. This type of bilirubin reacts as conjugated bilirubin in the laboratory method to measure conjugated or direct bilirubin.

Answer 29

Delta bilirubin

Question 30

On a liver biopsy sample, this syndrome would have the appearance of dark-stained granules. It is also obstructive in narture.

Answer 30

Dubin-Johnson syndrome

Question 31

What is the level of total bilirubin for a person with Dubin-Johnson syndrome?

Answer 31

Between 2 and 5 mg/dL

Question 32

A syndrome that is clinically similar to Dubin-Johnson syndrome but its has an unknown cause. It is hypothesized to be due to a reduction in the concentration or activity or intracellular binding proteins such as ligandin.

Answer 32

Rotor syndrome

Question 33

On a liver biopsy sample, this syndrome would have NO appearance of dark-stained granules.

Answer 33

Rotor syndrome

Question 34

This disease is a result of a deficiency in the enzyme glucoronyl transferase, one of the last liver functions to be activated in prenatal life since bilirubin processing is handled by the mother of the fetus.

Answer 34

Physiologic jaundice of the newborn

Question 35

It is the enzyme responsible for bilirubin conjugation. The deficiency of this enzyme results in rapid build-up of unconjugated bilirubin, which can be life threatening.

Answer 35

Glucoronyl transferase

Question 36

When unconjugated bilirubin builds up in the neonate, it cannot be processed and it is deposited in the nuclei of the brain and degenerate nerve cells, causing _____. This often results in cell damage and death in the newborn.

Answer 36

Kernicterus

Question 37

What are ways to treat infants suffering from Physiologic jaundice of the newborn?

Answer 37

Ultraviolet radiation to destroy the bilirubin as it passes through the capillaries of the skin Exchange transfusion in extreme cases.

Question 38

This classification of jaundice occurs as result from biliary obstructive disease, usually from physical obstructions (gallstones or tumors) that prevent the flow of conjugated bilirubin into the bile canaliculi.

Answer 38

Posthepatic jaundice

Question 39

In posthepatic jaundice, bile is not being brought to the intestines therefore stool loses its source of normal pigmentation and becomes _____ colored.

Answer 39

Clay

Question 40

A clinical condition in which scar tissue replaces normal, healthy liver tissue. The scar tissue blocks the flow of blood through the organ an prevents the liver from functioning properly.

Answer 40

Cirrhosis

Question 41

What are the signs and symptoms of cirrhosis that would appear after liver function deteriorates?

Answer 41

Fatigue, nausea, unintended weight loss, jaundice, bleeding from the gastrointestinal tract, intense itching and swelling in the legs and abdomen.

Question 42

In the US, what is the most common cause of cirrhosis?

Answer 42

Chronic alcoholism

Question 43

Enumerate other causes of cirrhosis aside from chronic alcoholism.

Answer 43

Chronic hepatitis B (HBV), C (HCV), and D (HDV) virus Autoimmune hepatitis Inherited disorders (α1-antitrypsin deficiency, Wilson disease, hemochromatosis, and galactosemia) Nonalcoholic steatohepatitis Blocked bile ducts Drugs, toxins, and infections

Question 44

What are the classifications of liver cancer?

Answer 44

Primary | Metastatic

Question 45

_____ liver cancer is cancer that begins in the liver cells; | _____ cancer occurs when tumors from other parts of the body spread (metastasize) to the liver.

Answer 45

Primary; | Metastatic

Question 46

Metastatic cancer is (more/less) common than primary liver cancer.

Answer 46

More

Question 47

From what site does cancer in the liver usually originate from?

Answer 47

Colon Lung Breast

Question 48

A type of benign tumor of the liver that occurs almost exclusively in females of child-bearing age.

Answer 48

Hepatocellular adenoma

Question 49

A type of benign tumor of the liver that has masses of blood vessels with no known etiology.

Answer 49

Hemangiomas

Question 50

A type of malignant tumor of the liver that is also known as hepatocarcinoma, and hepatoma.

Answer 50

Hepatocellular carcinoma (HCC)

Question 51

Aside from HCC, it is another form of malignant tumor of the liver.

Answer 51

Bile duct carcinoma

Question 52

This is the most common type of malignant tumor of the liver.

Answer 52

Hepatocellular carcinoma (HCC)

Question 53

This is an uncommon hepatic malignancy of children.

Answer 53

Hepatoblastoma

Question 54

It is a term used to describe a group of disorders caused by infectious, metabolic, toxic, or drug-induced disease found almost exclusively in children.

Answer 54

Reye syndrome

Question 55

A condition wherein the cause is unknown.

Answer 55

Idiopathic

Question 56

Although idiopathic, it is often preceded by a viral syndrome such as varicella (chicken pox), gastroenteritis, or an upper respiratory tract infection such as influenza.

Answer 56

Reye syndrome

Question 57

It is an acute illness characterized by non-inflammatory encephalopathy and fatty degeneratioin of the liver, with a clinical presentation of profuse vomiting accompanied with varying degrees of neurological impairment such as fluctuating personality changes and deterioration in consciousness.

Answer 57

Reye syndrome

Question 58

In Reye syndrome, the degeneration of the liver is characterized by a mild hyperbilirubinemia and threefold increases in _____ and _____.

Answer 58

Ammonia (NH3) | Aminotransferases (Aspartate aminotransferase (AST) and Alanine aminotransferase (ALT))

Question 59

It is the most common mechanism of toxicity in drug and alcohol related disorders. In this type of mechanism, the drug induces an adverse immune response directed against the liver itself and results in hepatic and/or cholestatic disease.

Answer 59

Immune-mediated injury to the hepatocytes

Question 60

Of all the drugs associted with hepatic toxicity, what is the most important/valuble? It causes very mild, transient, and unnoticed injury to the liver; however, with heavier and prolonged consumption, it can lead to alcoholic cirrhosis.

Answer 60

Ethanol

Question 61

Approximately how much of the alcohol absorbed from the stomach and small intestine is transported to the liver for for metabolism?

Answer 61

90%

Question 62

What enzymes are required by the liver for the elimination of alcohol?

Answer 62

Alcohol dehydrogenase | Acetaldehyde dehydrogenase

Question 63

The enzymes alcohol dehydrogenase and acetaldehyde dehydrogenase converts alcohol to acetaldehyde and subsequently to _____. This can then be oxidized to water and carbon dioxide, or it may enter the citric acid cycle

Answer 63

Acetate

Question 64

Alcohol induced liver injury may be categorized into three stages which are:

Answer 64

Alcoholic fatty acid liver Alcoholic hepatitis Alcoholic cirrhosis

Question 65

This represents the mildest category of alcohol induced liver injury where very few changes in liver function are measurable. Characterized by slight elevations in AST, ALT and gamma-glutamyl transferase (GGT) On biopsy, fatty infiltrates are noted in the vacoules of the liver. Affects young to middle aged people with moderate alcohol consumption.

Answer 65

Alcoholic fatty liver

Question 66

An alcohol induced liver injury that presents with common signs and symptoms including fever, ascites, proximal loss Laboratory evidence of liver damage: moderately elevated AST, ALT, GGT, and alkaline phosphate (ALP) and elevations in total bilirubin greater than 5 mg/dL Serum proteins (albumin) is decreased, International normalized ratio is elevated

Answer 66

Alcoholic hepatitis

Question 67

The most severe stage in an alcohol induced liver injury Symptoms tend to be nonspecific and include weight loss, weakness, hepatomegaly, splenomegaly, jaundice, ascites, fever, malnutrition, and edema Increased liver function tests (AST, ALT, GGT, ALP, and total bilirubin), decreased albumin, and prolonged prothrombin time Liver biopsy is the only method by which a definitive diagnosis may be made.

Answer 67

Alcoholic cirrhosis

Question 68

Alcoholic cirrhosis is (more/less) common in males than in females.

Answer 68

More

Question 69

Give one of the most common drugs associated with serious hepatic injury.

Answer 69

Acetaminophen

Question 70

A liver abnormality that is characteristic of alcoholic fatty liver with inflamation.

Answer 70

Steatohepatitis

Question 71

A liver abnormality characterized by scar tissue formation

Answer 71

Hepatic fibrosis

Question 72

A liver abnormality characterized by destruction of normal liver structure

Answer 72

Hepatic cirrhosis

Question 73

An alcohol induced liver injury wherein the elevations in AST are more than twice the upper reference of normal but rarely exceed 300 IU/mL. ALT are lower than AST, resulting in ALT/AST ratio (De Ritis ratio) greater than 2 Serum proteins, specially albumin, is decreased International normalized ratio is decreased

Answer 73

Alcoholic hepatitis

Question 74

The AST/ALT ratio is referred to as the _____.

Answer 74

De Ritis ratio

Question 75

This is the ratio of the coagulation time in the patient compared with a normal coagulation time

Answer 75

International Normalized ratio (INR)