(F) L3: Liver Function Tests (Liver Diseases)

Question 1

• Refers to elevated bilirubin in the blood
• A yellowish discoloration of skin and sclera

Answer 1

Jaundice/Hyperbilirubinemia

Note: Jaundice is NOT a disease, just a manifestation

Question 2

Hyperbilirubinemia

Jaundice will only start manifesting once the level of bilirubin in the blood reaches what?

Answer 2

3-5mg/dL or GREATER

Question 3

Hyperbilirubinemia

TOF: Jaundice is always associated with hyperbilirubinemia

Answer 3

False (some people can be diagnosed with hyperbilirubinemia but manifest no signs of jaundice yet)

Question 4

Hyperbilirubinemia

Refers to a serum/plasma specimen with increased bilirubin/yellowish discoloration

Answer 4

Icterus/Icteric

Note: Jaundice is for describing the person, icteric is for the sample

Question 5

Hyperbilirubinemia

Refers to hyperbilirubinemia in newborns/infants wherein the BBB is not yet intact leading to bilirubin deposition in the brain

Answer 5

Kernicterus

Question 6

Hyperbilirubinemia

Kernicterus level according to Henry’s?

Answer 6

> 20mg/dL (15-20mg/dL is borderline)

Question 7

Classifications of Hyperbilirubinemia

• Happens when there is hemolysis/hemolytic disease, malaria, and autoimmune disease
• There is elevated B1
• There is no problem with the liver

Answer 7

Pre-hepatic

Question 8

Classifications of Hyperbilirubinemia

• There is elevated bilirubin due to liver disease
• May be secondary to intrinsic defects due to disorders of bilirubin metabolism and transport defects
• Diseases resulting to hepatocellular injury/destruction (e.g. hepatitis)

Answer 8

Hepatic

Question 9

Classifications of Hyperbilirubinemia

• There is a problem with bilirubin excretion (biliary obstruction)
• May be due to gallstones (cholelithiasis) and tumors
• There is elevated B2

Answer 9

Post-hepatic

Question 10

Hyperbilirubinemia (Instrinsic Liver Defects)

• Due to immaturity of the liver (deficient UDPGT)
• Elevated B1 levels are observed
• Patient will undergo phototherapy until the liver matures (bilirubin is light sensitive)

Answer 10

Physiologic Jaundice of the Newborn

Question 11

Hyperbilirubinemia (Instrinsic Liver Defects)

• A genetic disorder wherein the UGT1A1 gene (for UDPGT) is mutated
• B1 is elevated while B2 is decreased

Answer 11

Crigler-Najjar Syndrome

Question 12

Hyperbilirubinemia (Instrinsic Liver Defects)

Types of Crigler-Najjar Syndrome (answer all):
1. A complete deficiency of UDPGT (elevated B1 and absent B2)
2. A partial deficiency of UDPGT (elevated B1 and decreased B2)

Answer 12

1. Type 1
2. Type 2

Question 13

Hyperbilirubinemia (Instrinsic Liver Defects)

• An impaired cellular uptake of bilirubin due to a defect in bilirubin transport
• Can be secondary to a benign deficiency of UDPGT
• Elevated B1

Answer 13

Gilbert Syndrome

Question 14

Hyperbilirubinemia (Instrinsic Liver Defects)

• There is a circulating inhibitor of bilirubin conjugation
• Elevated B1 and decreased B2
• A familial form of unconjugated hyperbilirubinemia (inherited disorder)

Answer 14

Lucey-Driscoll Syndrome

Question 15

Hyperbilirubinemia (Instrinsic Liver Defects)

• A genetic disorder wherein there is a deficiency of MDR2/cMOAT (Canalicular Multi-Specific Organic Anion Transporter) which is important for the excretion of bilirubin
• Elevated B2 (no problem with the conjugation, only transport)
• Risk for developing delta-bilirubin
• There is a deposition of lipofucsin in the liver (dark pigmentation)

Answer 15

Dubin-Johnson

Question 16

Hyperbilirubinemia (Instrinsic Liver Defects)

• A genetic defect in the excretion of bilirubin but with unknown etiology
• Elevated B2
• There is no dark pigmentation of the liver

Answer 16

Rotor Syndrome

Question 17

• Usually occurs secondary to chronic hepatocellular damage
• Scar tissues replace normal healthy tissue
• Conjugation and excretion functions are affected
• Both B1 and B2 are elevated
• Secondary to viral hepatitis, autoimmune hepatitis, inherited disorders, non-alcoholic steatohepatitis, blocked bile ducts, drugs, toxins, and infections

Answer 17

Cirrhosis

Question 18

• Includes viral, bacterial, and parasitic infections
• Also includes non-infections (radiation, drugs, chemical, and autoimmune diseases and toxins)
• Refers to the inflammation of the liver
• There is elevated B1 and B2

Answer 18

Hepatitis

Question 19

In hepatitis, bilirubin is increased in what percent of cases for the following?

1. Hepatitis A
2. Hepatitis B
3. Acute Hepatitis C

A. 20%
B. 30%
C. 70%

Answer 19

1. Hep. A - 70%
2. Hep. B - 30%
3. Acute Hep. C - 20%

Question 20

• Refers to gallstones, cholelithiasis, and pancreatic tumors
• There is post-hepatic jaundice caused by elevated B2

Answer 20

Biliary Obstruction

Question 21

Benign or Malignant Tumor?
1. Hepatocellular adenoma and hemangiomas
2. Hepatocellular and bile duct carcinoma

Answer 21

1. Benign
2. Malignant

Question 22

• An acute illness characterized by non-inflammatory encephalopathy
• Neurologic functions are affected
• There is fatty degeneration of the liver (hyperbilirubinemia)
• A threefold increase of ammonia in blood due to the insufficient conversion of ammonia to urea
• More common in children with unknown etiology

Note: Doctors suspect the etiology to be the ingestion of aspirin

Answer 22

Reye’s Syndrome

Question 23

Alcohol and Drug Related Diseases

• Slight elevation in AST, ALT, and GGT
• Fatty infiltrates are noted with vacuoles on liver biopsy
• Affects young to middle-aged people with a history of moderate alcohol consumption

Answer 23

Alcoholic Fatty Liver

Question 24

Alcohol and Drug Related Diseases

• Presents with fever, ascites, and proximal damage
• Moderately elevated AST, ALT, GGT, ALP
• Total bilirubin is greater than 5mg/dL

Answer 24

Alcoholic Hepatitis

Question 25

# Alcohol and Drug Related Diseases - Most severe form (all functions are affected) - More common in males than in females - Increased liver function tests (AST, ALT, GGT, ALP, and Total Bilirubin) - There is decreased albumin and prolonged prothrombin time - A biopsy is the only method for definitive diagnosis

Answer 25

Alcoholic Cirrhosis

Question 26

This enzyme is the marker for occult and chronic alcoholism

Answer 26

Gamma-Glutamyl Transferase (GGT)

Question 27

TOF: Intrinsic liver defects are inherited

Answer 27

True

Question 28

Give the intrinsic liver defects that present with elevated B1

Answer 28

1. Physiologic Jaundice of Newborn 2. Crigler-Najjar Syndrome 3. Gilbert Syndrome 4. Lucey-Driscoll Syndrome

Question 29

Give the intrinsic liver defects that present with elevated B2

Answer 29

1. Dubin-Johnson 2. Rotor Syndrome

Question 30

Unknown etiology? A. Rotor Syndrome B. Reye's Syndrome C. Both D. Neither

Answer 30

C. Both

Question 31

B1 is elevated while B2 is decreased A. Crigler-Najjar Type 1 B. Crigler-Najjar Type 2 C. Lucey-Driscoll Syndrome D. A and B E. B and C

Answer 31

E. LD Syndrome and Type 2 only (Type 1 has absent B2)

Question 32

Elevated B1 only A. Cirrhosis B. Hepatitis C. Both D. Neither

Answer 32

D. Neither (both B1 and B2 are elevated)