Exam 3 - Hepatic/Biliary

Question 1

Given the crucial role of the liver in synthesizing coagulation factors, which of the following factors is NOT predominantly produced by the liver?

A. Factor V
B. Factor IX
C. Factor VIII
D. Factor X

Answer 1

Correct Answer: C. Factor VIII

Rationale: The liver is responsible for the synthesis of most coagulation factors, including factors V, IX, and X. However, Factor VIII is an exception as it is synthesized in the endothelial cells lining the blood vessels.

Question 2

Which of the following is NOT a primary function of the liver?
A. Detoxification of blood
B. Synthesis of digestive enzymes
C. Metabolism of fats, proteins, and carbohydrates
D. Storage of vitamins and iron

Answer 2

Correct Answer: B. Synthesis of digestive enzymes

Question 3

The falciform ligament is an important anatomical landmark in the liver. It separates the:
A. Right and left lobes
B. Anterior and posterior segments
C. Superior and inferior parts
D. Quadrate and caudate lobes

Answer 3

Answer: A. Right and left lobes
Rationale: The falciform ligament attaches the liver to the anterior abdominal wall and separates the larger right and smaller left lobes of the liver. This is essential knowledge for a CRNA when considering the hepatic blood flow and possible implications during liver resections or transplantations.

Question 4

The liver is divided into how many segments?
A. 2
B. 4
C. 8
D. 10

Answer 4

Answer: C. 8
Rationale: The liver is divided into 8 segments based on blood supply and bile drainage.

Question 5

The hepatic ducts drain bile into the:
A. Pancreas
B. Spleen
C. Gallbladder & common bile duct
D. Stomach

Answer 5

Answer: C. Gallbladder & common bile duct
Rationale: Bile produced by the liver drains through the hepatic duct into the gallbladder and common bile duct.

Question 6

Which veins are responsible for draining blood from the liver into the inferior vena cava?
A. Hepatic portal vein
B. Right, middle, and left hepatic veins
C. Cystic vein
D. Renal veins

Answer 6

Answer: B. Right, middle, and left hepatic veins
Rationale: The three hepatic veins (right, middle, left) are responsible for draining deoxygenated blood from the liver into the inferior vena cava.

Question 7

The liver receives what percentage of the cardiac output (COP)?
A. 10%
B. 25%
C. 50%
D. 75%

Answer 7

Answer: B. 25%
Rationale: The liver receives 25% of the cardiac output, which is the highest proportionate blood flow received by any organ, amounting to 1.25-1.5 liters per minute.

Question 8

What is the source of the blood that flows through the portal vein to the liver?
A. Hepatic artery
B. Aorta
C. Splenic vein and superior mesenteric vein
D. Inferior vena cava

Answer 8

Answer: C. Splenic vein and superior mesenteric vein
Rationale: The portal vein is formed by the confluence of the splenic vein and the superior mesenteric vein, carrying deoxygenated blood from the gastrointestinal organs, pancreas, and spleen.

Question 9

The blood supplied by the portal vein to the liver is characterized by:
A. Being fully oxygenated
B. Containing nutrients from gastrointestinal absorption
C. Carrying waste products to be filtered by the liver
D. B and C are correct

Answer 9

Answer: D. B and C are correct
Rationale: The portal vein carries blood that is rich in nutrients from the gastrointestinal tract and the spleen but is partially deoxygenated. This blood also contains waste products that the liver will process.

Question 10

How is oxygen delivered to the liver?
A. 100% through the hepatic artery
B. 75% through the portal vein and 25% through the hepatic artery
C. 50% through the portal vein and 50% through the hepatic artery
D. 25% through the portal vein and 75% through the hepatic artery

Answer 10

Answer: C. 50% through the portal vein (deoxygenated) and 50% through the hepatic artery

Question 11

Hepatic arterial blood flow compensates for changes in which of the following?
A. Systemic arterial pressure
B. Portal venous blood flow
C. Kidney perfusion
D. Heart rate

Answer 11

Answer: B. Portal venous blood flow
Rationale: Hepatic arterial blood flow inversely relates to portal venous blood flow, meaning that when portal venous flow decreases, hepatic arterial flow increases, and vice versa. This is part of the hepatic arterial buffer response, which is essential for maintaining a consistent hepatic blood flow (HBF).

Question 12

Increased portal venous pressure can lead to the development of:
A. Renal stones
B. Pulmonary embolism
C. Esophageal and gastric varices
D. Pancreatic insufficiency

Answer 12

Answer: C. Esophageal and gastric varices
Rationale: When portal venous pressure rises, it can cause blood to back up into other areas of the systemic circulation. This often results in the development of esophageal and gastric varices, which are enlarged veins in the esophagus and stomach that pose a risk for bleeding.

Question 13

Autoregulation of hepatic blood flow ensures:
A. An increase in hepatic artery dilation with an increase in portal venous flow
B. A decrease in hepatic artery dilation with an increase in portal venous flow
C. Consistent hepatic arterial flow despite fluctuations in portal venous flow
D. An increase in portal venous flow with a decrease in hepatic arterial flow

Answer 13

Answer: C. Consistent hepatic arterial flow despite fluctuations in portal venous flow
Rationale: Hepatic blood flow is autoregulated, which means the liver can maintain a constant blood flow even when there are changes in portal venous flow. When portal flow is low, the hepatic artery dilates to compensate, ensuring the liver receives sufficient blood supply.

Question 14

The hepatic venous pressure gradient (HVPG) is a clinical measurement used to assess:
A. Kidney function
B. Heart function
C. Severity of portal hypertension
D. Lung perfusion

Answer 14

Answer: C. Severity of portal hypertension
Rationale: HVPG is the gradient between the portal vein and hepatic veins and is used specifically to determine the severity of portal hypertension, with higher values indicating more severe disease.

Question 15

Which HVPG range indicates clinically significant portal hypertension that may lead to complications such as cirrhosis and esophageal varices?
A. 1-5 mmHg
B. >5-10 mmHg
C. >10 mmHg
D. >12 mmHg

Answer 15

Answer: C. >10 mmHg
Rationale: An HVPG measurement greater than 10 mmHg is considered clinically significant portal hypertension and is associated with complications like cirrhosis and esophageal varices.

Question 16

When HVPG exceeds 12 mmHg, there is an increased risk for which serious complication?
A. Cholecystitis
B. Variceal rupture
C. Hepatic encephalopathy
D. Ascites

Answer 16

Answer: B. Variceal rupture
Rationale: An HVPG greater than 12 mmHg is associated with an increased risk of variceal rupture, which is a life-threatening event requiring immediate medical attention.

Question 17

In the assessment of liver function, which of the following is considered a major risk factor?
A. Heavy ethanol (ETOH) consumption
B. High calcium intake
C. Physical inactivity
D. High carbohydrate diet

Answer 17

Answer: A. Heavy ethanol (ETOH) consumption
Rationale: Heavy alcohol consumption is a well-known risk factor for liver disease and is a critical aspect to consider when assessing liver function.

Question 18

Which of the following symptoms is likely to be observed in the early stages of liver disease - select all ?
A. Pruritis
B. Jaundice
C. Disrupted sleep
D. Hepatomegaly
E. disrupted sleep

Answer 18

answer: ABCDE, Even later stages may only have vague sx s/a disrupted sleep,↓appetite

Question 19

The presence of asterixis, a flapping tremor of the hands, is associated with:
A. Renal dysfunction
B. Severe metabolic acidosis
C. Advanced liver disease
D. Diabetes mellitus

Answer 19

Answer: C. Advanced liver disease
Rationale: Asterixis is typically associated with advanced liver disease and is an important sign during a physical examination indicating potential hepatic encephalopathy.

Question 20

Which laboratory tests are most liver-specific according to the provided information?
A. Aspartate aminotransferase (AST) and Alanine aminotransferase (ALT)
B. Blood Urea Nitrogen (BUN) and Creatinine
C. Hemoglobin and Hematocrit
D. Platelet count and White Blood Cell (WBC) count

Answer 20

Answer: A. Aspartate aminotransferase (AST) and Alanine aminotransferase (ALT)
Rationale: AST and ALT are enzymes that are found in high levels within liver cells and are released into the bloodstream when liver cells are damaged, making them specific indicators for liver function.

Question 21

What imaging modality is useful for assessing portal blood flow in the liver?
A. X-ray
B. Doppler Ultrasound
C. MRI without contrast
D. Standard Ultrasound

Answer 21

Answer: B. Doppler Ultrasound
Rationale: Doppler Ultrasound is specifically mentioned for evaluating portal blood flow because it can measure the speed and direction of blood flow, providing valuable information about the liver’s vascular system.

Question 22

A. 1:1
B. At least 2:1
C. Less than 1:1
D. Equal to the level of alkaline phosphatase

Answer 22

Answer: B. At least 2:1
Rationale: The slide indicates that in alcoholic liver disease, the aspartate aminotransferase (AST) to alanine aminotransferase (ALT) ratio is usually at least 2:1, which is a pattern seen in this condition due to the specific effect of alcohol on the liver.

Question 23

Which condition is associated with a significant increase in AST/ALT, potentially up to 25 times the normal value?
A. Non-Alcoholic Fatty Liver Disease (NAFLD)
B. Chronic hepatitis B
C. Acute Liver Failure (ALF)
D. Cholestasis

Answer 23

Answer: C. Acute Liver Failure (ALF)
Rationale: Acute Liver Failure may show a dramatic increase in liver enzymes, such as AST and ALT, which can be elevated up to 25 times above the normal levels due to extensive liver damage.

Question 24

What lab findings are indicative of cholestasis?
A. Decreased albumin and decreased prothrombin time/international normalized ratio (PT/INR)
B. Elevated alkaline phosphatase, gamma-glutamyl transferase (GGT), and bilirubin
C. Elevated AST/ALT with normal bilirubin
D. Normal alkaline phosphatase with elevated bilirubin

Answer 24

Answer: B. Elevated alkaline phosphatase, gamma-glutamyl transferase (GGT), and bilirubin
Rationale: Cholestasis, a condition where bile flow from the liver is reduced or blocked, is often indicated by elevated levels of alkaline phosphatase, GGT, and bilirubin.

Question 25

Which blood test typically shows increased levels in both hepatocellular injury and cholestasis?
A. Serum albumin
B. Aminotransferases
C. Alkaline phosphatase
D. Prothrombin time

Answer 25

Answer: C. Alkaline phosphatase
Rationale: Alkaline phosphatase levels are usually increased in both hepatocellular injury and cholestasis, indicating a possible obstruction in bile flow or damage to the hepatocytes.

Question 26

In the case of bilirubin overload due to hemolysis, what is the predominant form of bilirubin found?
A. Conjugated
B. Unconjugated
C. Both conjugated and unconjugated
D. Neither, as bilirubin levels are not affected by hemolysis

Answer 26

Answer: B. Unconjugated
Rationale: In hemolysis, red blood cells are destroyed, releasing unconjugated bilirubin into the bloodstream. The liver has not had a chance to convert it into its conjugated form.

Question 27

A patient with acute liver failure is likely to exhibit which laboratory changes?
A. Increased aminotransferases and decreased serum albumin
B. Normal aminotransferases and increased prothrombin time
C. Increased serum albumin and normal prothrombin time
D. Normal aminotransferases and normal serum albumin

Answer 27

Answer: A. Increased aminotransferases and decreased serum albumin
Rationale: Acute liver failure often results in increased aminotransferases due to hepatocyte injury and decreased serum albumin due to reduced synthetic function of the liver.

Question 28

What is the typical presentation of cholelithiasis in symptomatic patients?
A. Lower abdominal pain
B. Diarrhea and flatulence
C. Right upper quadrant pain, nausea/vomiting, and fever
D. Left upper quadrant pain and jaundice

Answer 28

Answer: C. Right upper quadrant pain, nausea/vomiting, and fever
Rationale: The typical symptoms of cholelithiasis when present include right upper quadrant abdominal pain that may refer to the shoulders, nausea/vomiting, and fever, especially if there’s acute obstruction.

Question 29

Which procedure is the definitive treatment for symptomatic cholelithiasis?
A. Endoscopic retrograde cholangiopancreatography (ERCP)
B. Percutaneous transhepatic cholangiography (PTC)
C. Laparoscopic Cholecystectomy
D. Medical management with bile acid pills

Answer 29

Answer: C. Laparoscopic Cholecystectomy
Rationale: The definitive treatment for symptomatic cholelithiasis is the surgical removal of the gallbladder, known as laparoscopic cholecystectomy.

Question 30

After a cholecystectomy, where does the bile flow directly?
A. It is stored in the liver until meals
B. It flows through the cystic duct into the duodenum
C. It is directly secreted into the stomach
D. It flows through the common bile duct into the duodenum

Answer 30

Answer: D. It flows through the common bile duct into the duodenum
Rationale: Once the gallbladder is removed, bile flows directly from the liver through the common bile duct into the duodenum. There is no longer a storage function provided by the gallbladder.

Question 31

Choledocholithiasis is characterized by the presence of a stone in which part of the biliary system?
A. Gallbladder
B. Common bile duct (CBD)
C. Cystic duct
D. Pancreatic duct

Answer 31

Answer: B. Common bile duct (CBD)
Rationale: Choledocholithiasis refers to a stone obstructing the common bile duct, leading to biliary colic and potentially other serious symptoms like cholangitis.

Question 32

In an ERCP procedure, glucagon may be administered to:
A. Induce sleep
B. Relieve biliary colic
C. Counteract the effects of general anesthesia
D. Relieve a spasm of the Sphincter of Oddi

Answer 32

Answer: D. Relieve a spasm of the Sphincter of Oddi
Rationale: Glucagon may be used during an ERCP procedure to relax the Sphincter of Oddi, which can help in retrieving the stone and ease the procedure.

Question 32

What is the standard treatment for a stone in the common bile duct?
A. Surgical removal
B. Shock wave lithotripsy
C. Endoscopic Retrograde Cholangiopancreatography (ERCP)
D. Medication to dissolve the stone

Answer 32

Answer: C. Endoscopic Retrograde Cholangiopancreatography (ERCP)
Rationale: The endoscopic removal of the stone via ERCP is the standard treatment, where a guidewire is threaded through the Sphincter of Oddi into the Ampulla of Vater to retrieve the stone.

Question 33

Unconjugated hyperbilirubinemia is often a result of:
A. An obstruction in the biliary system.
B. Imbalance between bilirubin synthesis and conjugation.
C. Congenital infections.
D. Postoperative complications.

Answer 33

Answer: B. Imbalance between bilirubin synthesis and conjugation
Rationale: Unconjugated (indirect) hyperbilirubinemia occurs due to an imbalance between the production and conjugation of bilirubin. This could be due to increased production (as in hemolysis) or decreased conjugation (as in Gilbert’s syndrome).

Question 34

Which of the following conditions is associated with conjugated hyperbilirubinemia?
A. Sickle cell anemia
B. Hemolytic disease of the newborn
C. Dubin-Johnson syndrome
D. Physiologic jaundice of the newborn

Answer 34

Answer: C. Dubin-Johnson syndrome
Rationale: Conjugated (direct) hyperbilirubinemia is seen in conditions where there is an issue with bile excretion, such as Dubin-Johnson syndrome, leading to the reflux of conjugated bilirubin into the circulation.

Question 35

Which type of bilirubin is typically water-soluble and directly excreted into bile?
A. Unconjugated (indirect) bilirubin
B. Conjugated (direct) bilirubin
C. Both A and B
D. Neither A nor B

Answer 35

Answer: B. Conjugated (direct) bilirubin
Rationale: Conjugated bilirubin is water-soluble, allowing it to be excreted directly into the bile. Unconjugated bilirubin is not water-soluble and must first be conjugated in the liver before excretion.

Question 36

Which types of viral hepatitis are most commonly associated with chronic infection?
A. Hepatitis A and E
B. Hepatitis B and C
C. Hepatitis C and D
D. Hepatitis D and E

Answer 36

Answer: B. Hepatitis B and C
Rationale: Hepatitis B and C are known for their potential to cause chronic infections, which can lead to long-term liver damage and are significant health concerns worldwide.

Question 37

What is the impact of new treatments on Hepatitis C Virus (HCV) in the U.S. population?
A. Increased the rate of liver transplantation due to HCV
B. Significantly reduced the need for liver transplantation due to HCV
C. Have had no significant impact on HCV rates
D. Led to an increase in the types of HCV genotypes

Answer 37

Answer: B. Significantly reduced the need for liver transplantation due to HCV
Rationale: Newer treatments, such as a 12-week course of Sofosbuvir/Velpatasvir, have significantly reduced Hepatitis C Virus in the U.S. population and have greatly increased the clearance rates of HCV genotype 1A/1B, consequently reducing the need for liver transplantation.

Question 38

The decline in viral hepatitis is attributed to which of the following factors?
A. The natural progression of the disease
B. Improvements in hygiene practices only
C. Vaccines and newer treatments
D. The increase in natural immunity within the population

Answer 38

Answer: C. Vaccines and newer treatments
Rationale: The decline in the incidence of viral hepatitis is largely due to the introduction of vaccines, especially for Hepatitis B, and the development of new antiviral treatments that have been effective in managing the disease.

Question 39

Which progression of disease is commonly associated with chronic Hepatitis C Virus infection?
A. Acute HCV Asymptomatic → Chronic Infection → Cirrhosis → Hepatocellular Carcinoma
B. Acute HCV Asymptomatic → Hepatocellular Carcinoma → Cirrhosis → Chronic Infection
C. Chronic Infection → Acute HCV Asymptomatic → Hepatocellular Carcinoma → Cirrhosis
D. Cirrhosis → Chronic Infection → Acute HCV Asymptomatic → Hepatocellular Carcinoma

Answer 39

Answer: A. Acute HCV Asymptomatic → Chronic Infection → Cirrhosis → Hepatocellular Carcinoma
Rationale: Chronic HCV infection can lead to cirrhosis and increase the risk for developing hepatocellular carcinoma over time. Initially, acute HCV may be asymptomatic, which can progress to chronic infection if not treated.

Question 40

Common symptoms of acute and chronic hepatitis include all the following EXCEPT:
A. Fatigue
B. Hypertension
C. Dark urine
D. Jaundice

Answer 40

Answer: B. Hypertension
Rationale: Common symptoms of hepatitis typically include fatigue, dark urine, and jaundice, but not hypertension, which is a condition related to high blood pressure and not directly a symptom of hepatitis.

Question 41

Hepatitis C Virus is primarily transmitted through:
A. Respiratory droplets
B. Contaminated food and water
C. Blood
D. Insect bites

Answer 41

Answer: C. Blood
Rationale: The primary route of HCV transmission is through blood. It can occur via transfusion of contaminated blood products, sharing of needles, or through other exposures to infected blood.

Question 42

Which type(s) of viral hepatitis is primarily transmitted through blood transfusions?
A. Type A
B. Type B and C
C. Type D
D. Type E

Answer 42

Answer: B. Type B and C

Question 43

What is the primary approach to treating Alcoholic Liver Disease (ALD)?
A. Aggressive medical intervention with antibiotics
B. Surgical intervention
C. Abstinence from alcohol and management of liver failure symptoms
D. Liver transplant is the first-line treatment

Answer 43

Answer: C. Abstinence from alcohol and management of liver failure symptoms

Rationale: The cornerstone of treatment for ALD is abstinence from alcohol. Management also focuses on supportive care and addressing complications of liver failure, such as with transfusions if needed.

Question 44

ALD is the most common cause of which liver condition in America?
A. Hepatitis
B. Cirrhosis
C. Fatty liver
D. Hepatocellular carcinoma

Answer 44

Answer: B. Cirrhosis

Rationale: According to the slide, ALD is cited as the most common cause of cirrhosis, which is the severe scarring of the liver and poor liver function seen at the terminal stages of chronic liver disease.

Question 45

In patients with ALD, at what platelet count is a blood transfusion typically required?
A. Platelet count below 150,000
B. Platelet count below 100,000
C. Platelet count below 50,000
D. Platelet count is not a consideration for transfusion in ALD

Answer 45

Answer: C. Platelet count below 50,000

Rationale: As stated on the slide, a platelet count below 50,000 in the context of ALD requires a blood transfusion, which is a common management step for significant thrombocytopenia.

Question 46

Which lab value is specifically noted to increase in the later stages of Alcoholic Liver Disease (ALD)?
A. Mean corpuscular volume (MCV)
B. Liver enzymes
C. Gamma-glutamyl-transferase (GGT)
D. Bilirubin

Answer 46

Answer: C. Gamma-glutamyl-transferase (GGT)- but all are elevated

Rationale: The slide indicates that Gamma-glutamyl-transferase (GGT) is particularly elevated in the later stages of ALD, which can be a marker of chronic alcohol use and liver injury.

Question 47

In the context of Alcoholic Liver Disease, an increased Mean corpuscular volume (MCV) may indicate:
A. Dehydration
B. Malnutrition
C. Overhydration
D. Kidney disease

Answer 47

Answer: B. Malnutrition

Rationale: An increased MCV can be a sign of macrocytic anemia, which may result from malnutrition, often associated with chronic alcohol misuse.

Question 48

What does an elevated bilirubin level signify in the context of ALD?
A. Kidney failure
B. Increased bone activity
C. Poor liver function
D. High levels of physical activity

Answer 48

Answer: C. Poor liver function

Rationale: Bilirubin is a product of the normal breakdown of red blood cells, usually processed by the liver. High levels of bilirubin in ALD suggest the liver is not functioning properly to clear bilirubin from the blood.

Question 49

What is the gold standard for diagnosing Non-Alcoholic Fatty Liver Disease?
A. Blood tests
B. Ultrasound
C. Liver biopsy
D. MRI

Answer 49

Answer: C. Liver biopsy

Rationale: The slide identifies a liver biopsy as the gold standard in diagnosing NAFLD because it allows for direct observation of fat in hepatocytes and assessment of liver tissue structure.

Question 50

Which of the following is a risk factor for Non-Alcoholic Fatty Liver Disease?
A. Excessive alcohol consumption
B. Low body mass index
C. Insulin resistance
D. Viral infection

Answer 50

Answer: C. Insulin resistance

Rationale: Risk factors for NAFLD include obesity, type 2 diabetes (DM2), insulin resistance, and metabolic syndrome, which are all related to the body’s metabolism and not related to alcohol consumption or viral infections.

Question 51

Non-Alcoholic Fatty Liver Disease may progress to all the following EXCEPT:
A. Nonalcoholic steatohepatitis (NASH)
B. Hepatocellular carcinoma
C. Alcoholic cirrhosis
D. Cirrhosis

Answer 51

Answer: C. Alcoholic cirrhosis

Rationale: NAFLD can progress to NASH, cirrhosis, and hepatocellular carcinoma, but it does not lead to alcoholic cirrhosis, which is caused by chronic alcohol abuse, not NAFLD.

Question 52

What percentage of severely obese individuals are affected by Non-Alcoholic Fatty Liver Disease (NAFLD), according to the 2019 prevalence data?
A. 25%
B. 50%
C. 75%
D. 90%

Answer 52

Answer: D. 90%

Question 53

In the progression of liver disease, what is the stage following Non-alcoholic Steatohepatitis (NASH)?
A. Liver fibrosis
B. Alcoholic hepatitis
C. Non-alcoholic fatty liver (NAFL)
D. Cirrhosis of the liver

Answer 53

Answer: A. Liver fibrosis

Question 54

What percentage of patients with autoimmune hepatitis (AIH) typically achieve remission with treatment?
A. 10-20%
B. 30-50%
C. 60-80%
D. 90-100%

Answer 54

Answer: C. 60-80%

Question 55

Which medication is commonly associated with drug-induced liver injury due to overdose?
A. Ibuprofen
B. Acetaminophen
C. Aspirin
D. Naproxen

Answer 55

Answer: B. Acetaminophen

Question 56

In acute cases of autoimmune hepatitis, how much higher than normal can the AST/ALT levels be?
A. 2-5 times normal
B. 5-10 times normal
C. 10-20 times normal
D. More than 20 times normal

Answer 56

Answer: C. 10-20 times normal

Question 57

How common are inborn errors of metabolism according to the provided information?
A. 1 in 100 births
B. 1 in 500 births
C. 1 in 2500 births
D. 1 in 5000 births

Answer 57

Answer: C. 1 in 2500 births

Rationale: Inborn errors of metabolism are rare genetic disorders, occurring in approximately 1 in 2500 births, highlighting the relative rarity but significant risk for metabolic issues in the population.

Question 58

Which of the following disorders is an example of an inborn error of metabolism that can lead to an excess of iron accumulation in the body?
A. Wilson’s Disease
B. Alpha-1 Antitrypsin Deficiency
C. Hemochromatosis
D. Phenylketonuria

Answer 58

Answer: C. Hemochromatosis

Rationale: Hemochromatosis is a condition characterized by excessive iron accumulation in the body, which is one of the listed disorders under inborn errors of metabolism. It is a genetic disorder affecting iron metabolism.

Question 59

When is the onset for most severe forms of inborn errors of metabolism?
A. Neonatal period
B. Early childhood
C. Adolescence
D. Adulthood

Answer 59

Answer: A. Neonatal period

Rationale: The most severe forms of inborn errors of metabolism often present in the neonatal period, which is a critical time for identifying and managing these conditions to reduce associated high mortality risks.

Question 60

What is the inheritance pattern of Wilson’s Disease?
A. Autosomal dominant
B. Autosomal recessive
C. X-linked dominant
D. X-linked recessive

Answer 60

Answer: B. Autosomal recessive

Rationale: Autosomal recessive disease characterized by impaired copper metabolism

Question 61

What is a common diagnostic lab test used for Wilson’s Disease?
A. Serum ceruloplasmin
B. Hemoglobin A1c
C. Serum calcium
D. Thyroid-stimulating hormone (TSH)

Answer 61

Answer: A. Serum ceruloplasmin

Rationale: Lab tests (serum ceruloplasmin,aminotransferases,urine copper level)
Possible liver biopsy for copper level

Question 62

Which therapeutic approach is used to treat Wilson’s Disease?
A. Insulin therapy
B. Copper-chelation therapy
C. Hormone replacement therapy
D. Beta-blocker therapy

Answer 62

Answer: B. Copper-chelation therapy

Rationale: Treatment for Wilson’s Disease includes copper-chelation therapy to remove excess copper from the body and oral zinc to block copper absorption in the gastrointestinal tract. This is aimed at reducing the oxidative stress caused by copper buildup.

Question 63

What is the role of alpha-1 antitrypsin in the body?
A. To increase inflammation
B. To protect tissues from enzymes like neutrophil elastase
C. To assist in blood clotting
D. To aid in the digestion of dietary fats

Answer 63

Answer: B. To protect tissues from enzymes like neutrophil elastase

Rationale: Alpha-1 antitrypsin is a protein that protects the liver and lungs from enzymes like neutrophil elastase, which can cause tissue damage. In the deficiency state, lack of this protection leads to tissue damage, inflammation, cirrhosis, and hepatocellular carcinoma (HCC).

Question 64

How is Alpha-1 Antitrypsin Deficiency diagnosed?
A. Liver function tests
B. Alpha-1 antitrypsin phenotyping
C. Ultrasound
D. CT scan

Answer 64

Answer: B. Alpha-1 antitrypsin phenotyping

Rationale: The diagnosis of Alpha-1 Antitrypsin Deficiency is confirmed through phenotyping, which identifies the specific type of alpha-1 antitrypsin protein the liver is producing. This test is crucial as it helps distinguish the deficiency from other liver disorders.

Question 65

What is the only curative treatment for liver disease caused by Alpha-1 Antitrypsin Deficiency?
A. Antiviral medication
B. Steroid treatment
C. Liver transplant
D. Pooled alpha-1 antitrypsin infusion

Answer 65

Answer: C. Liver transplant

Rationale: While pooled alpha-1 antitrypsin is effective for treating lung-related symptoms, the only curative treatment for the liver disease associated with this condition is a liver transplant. This is a significant factor considering that alpha-1 antitrypsin deficiency is a leading genetic cause of liver transplantation in children.

Question 66

What is the primary cause of hemochromatosis?
A. Vitamin C deficiency
B. Excessive calcium intake
C. Genetic mutation leading to increased iron absorption
D. Lack of dietary iron

Answer 66

Answer: C. Genetic mutation leading to increased iron absorption

Rationale: Hemochromatosis is typically associated with a genetic mutation that causes the body to absorb an excessive amount of iron from the diet. This condition can lead to the accumulation of iron in body tissues, causing various symptoms and complications.

Question 67

Which of the following diagnostic tests is used to identify hemochromatosis?
A. Complete blood count (CBC)
B. Liver function test
C. Genetic mutation testing
D. Lipid panel

Answer 67

Answer: C. Genetic mutation testing

Rationale: Genetic mutation testing is used to diagnose hemochromatosis by identifying mutations in genes that regulate iron metabolism, such as the HFE gene.

Question 68

What is a common treatment approach for managing hemochromatosis?
A. Regular insulin injections
B. Weekly phlebotomy sessions
C. Daily calcium supplements
D. Bariatric surgery

Answer 68

Answer: B. Weekly phlebotomy sessions

Rationale: Phlebotomy, the process of drawing blood from a patient, is a common treatment for hemochromatosis. It helps to reduce iron levels in the body. Iron-chelating drugs may also be used, and a liver transplant may be necessary in cases of advanced liver disease due to iron overload.

Question 69

Which autoimmune condition is characterized by the destruction of bile ducts and presents with symptoms like jaundice, fatigue, and itching?
A. Primary Biliary Cholangitis (PBC)
B. Primary Sclerosing Cholangitis (PSC)
C. Autoimmune Hepatitis (AIH)
D. Wilson’s Disease

Answer 69

Answer: A. Primary Biliary Cholangitis (PBC)

Rationale: PBC is an autoimmune disease formerly known as biliary cirrhosis, and it involves the gradual destruction of bile ducts within the liver. This leads to symptoms like jaundice, fatigue, and itching due to the buildup of bile and associated inflammatory response.

Question 70

What is the diagnostic lab test indicative of PBC?
A. Antinuclear antibodies (ANA)
B. Antimitochondrial antibodies (AMA)
C. Anti-smooth muscle antibodies (ASMA)
D. Anti-liver kidney microsomal antibodies (LKM)

Answer 70

Answer: B. Antimitochondrial antibodies (AMA)

Rationale: Antimitochondrial antibodies are typically present in PBC and serve as a key diagnostic marker. Their presence along with symptoms and other lab findings such as elevated alkaline phosphatase and gamma-glutamyl transferase can confirm the diagnosis.

Question 71

In the context of PBC, why might the administration of exogenous bile acids require careful consideration?
A. They can exacerbate symptoms of jaundice.
B. They are known to be caustic to the stomach lining.
C. They increase the risk of autoimmune reactions.
D. They lead to a rapid progression of liver fibrosis.

Answer 71

Answer: B. They are known to be caustic to the stomach lining.

Rationale: Treatment for PBC includes the administration of exogenous bile acids to help with bile flow. However, these acids can be harsh on the stomach lining, requiring cautious use, especially in patients with comorbid conditions like diabetes with gastroparesis where delayed gastric emptying could increase the risk of stomach irritation or damage.

Question 72

What is the imaging modality of choice for diagnosing Primary Sclerosing Cholangitis?
A. X-Ray
B. Magnetic Resonance Cholangiopancreatography (MRCP)
C. Ultrasound
D. CT Scan

Answer 72

Answer: B. Magnetic Resonance Cholangiopancreatography (MRCP)

Rationale: MRCP is a non-invasive imaging modality that can show biliary strictures with dilated bile ducts, characteristic of PSC.

Question 73

Why is liver transplantation considered the only long-term treatment for PSC?
A. PSC does not respond well to pharmaceutical interventions.
B. PSC can lead to end-stage liver disease (ESLD) requiring transplantation.
C. Liver transplantation can prevent the autoimmune process.
D. PSC is always immediately life-threatening.

Answer 73

Answer: B. PSC can lead to end-stage liver disease (ESLD) requiring transplantation.

Rationale: PSC is a progressive disease that can lead to liver scarring, fibrosis, cirrhosis, and ultimately ESLD, for which a liver transplant may be the only curative option. However, due to its autoimmune nature, PSC can recur in the transplanted liver.

Question 74

Which lab findings are typically elevated in PSC?
A. Alkaline phosphatase and gamma-glutamyl transferase
B. Hemoglobin and hematocrit
C. Blood urea nitrogen and creatinine
D. Sodium and potassium

Answer 74

Answer: A. Alkaline phosphatase and gamma-glutamyl transferase

Rationale: In PSC, the liver enzymes alkaline phosphatase and gamma-glutamyl transferase are typically elevated due to cholestasis and liver inflammation.
Sx: Fatigue, itching, deficiency of fat-soluble vitamins (A,D,E,K), cirrhosis

Can happen intra - and extra hepatically

Question 75

What is the leading cause of drug-induced acute liver failure?
A. Antibiotics
B. Nonsteroidal anti-inflammatory drugs (NSAIDs)
C. Acetaminophen overdose
D. Antiviral medications

Answer 75

Answer: C. Acetaminophen overdose

Rationale: The slide indicates that nearly 50% of acute liver failure cases are drug-induced, with the majority being due to acetaminophen overdose.

Question 76

Which of the following symptoms is commonly associated with acute liver failure?
A. Peripheral edema
B. Cerebral edema
C. Constipation
D. Rash

Answer 76

Answer: B. Cerebral edema

Rationale: Symptoms of acute liver failure include jaundice, nausea, right upper quadrant pain, and cerebral edema. Cerebral edema is particularly concerning because it can lead to increased intracranial pressure and is associated with encephalopathy.

Question 77

In the context of acute liver failure, what is the significance of hepatic encephalopathy?
A. It is a rare complication that has little impact on the prognosis.
B. It is a minor symptom that often resolves without treatment.
C. It is a sign of severe liver dysfunction and can be life-threatening.
D. It only occurs post-liver transplant.

Answer 77

Answer: C. It is a sign of severe liver dysfunction and can be life-threatening.

Rationale: Hepatic encephalopathy is a serious complication of acute liver failure that indicates significant liver dysfunction. It results from the liver’s inability to remove toxins from the blood, leading to brain dysfunction and can have dire consequences if not treated promptly.

Question 78

What characterizes the pathology of cirrhosis?
A. Regeneration of liver tissue
B. Replacement of normal liver parenchyma with scar tissue
C. Inflammation of the bile ducts
D. Accumulation of fat in liver cells

Answer 78

Answer: B. Replacement of normal liver parenchyma with scar tissue

Rationale: Cirrhosis represents the final stage of liver disease where the normal liver parenchyma is replaced with scar tissue, leading to impaired liver function. This scarring process disrupts the liver’s normal structure and the vital functions it performs.

Question 79

Which lab findings are typically elevated in a patient with cirrhosis?
A. Alkaline phosphatase, aspartate aminotransferase (AST), alanine aminotransferase (ALT), bilirubin, and prothrombin time/international normalized ratio (PT/INR)
B. Hemoglobin, calcium, and sodium
C. Creatinine, blood urea nitrogen (BUN), and glucose
D. Vitamin B12 and folic acid

Answer 79

Answer: A. Alkaline phosphatase, AST, ALT, bilirubin, and PT/INR

Rationale: In cirrhosis, liver damage is indicated by elevated levels of liver enzymes (AST, ALT), bilirubin, and Alk phosphatase. Coagulopathy is reflected in prolonged PT/INR. These values together help diagnose the extent of liver damage.

Question 80

Which condition is considered the definitive treatment for cirrhosis?
A. Antiviral therapy
B. Steroid administration
C. Liver transplant
D. Antibiotic treatment

Answer 80

Answer: C. Liver transplant

Rationale: When cirrhosis progresses to end-stage liver disease, the only curative treatment is a liver transplant. This procedure replaces the damaged liver with a healthy one from a donor, addressing the root of the cirrhosis and potentially restoring normal liver function.

Question 81

What is the Hepatic Venous Pressure Gradient (HVPG) value that indicates portal hypertension (P-HTN) in cirrhosis?
A. HVPG > 5
B. HVPG < 5
C. HVPG > 10
D. HVPG < 10

Answer 81

Answer: A. HVPG > 5

Rationale: Portal hypertension is diagnosed when the HVPG exceeds 5 mmHg, reflecting increased vascular resistance within the portal venous system, which can lead to complications such as ascites.

Question 82

Which procedure is indicated for reducing portal hypertension and managing ascites in cirrhosis patients?
A. Endoscopic variceal ligation
B. Paracentesis
C. Transjugular Intrahepatic Portosystemic Shunt (TIPS)
D. Liver resection

Answer 82

Answer: C. Transjugular Intrahepatic Portosystemic Shunt (TIPS)

Rationale: TIPS is a procedure that creates a pathway within the liver for blood to flow easier, reducing portal pressure and consequently ascites and the risk of variceal bleeding.

Question 83

Spontaneous Bacterial Peritonitis (SBP) is a complication of cirrhosis that requires immediate treatment with what type of medication?
A. Antihypertensives
B. Antipyretics
C. Antibiotics
D. Anticoagulants

Answer 83

Answer: C. Antibiotics

Rationale: SBP is an infection of the fluid in the peritoneal cavity, a serious complication of cirrhosis that requires prompt antibiotic therapy to treat the infection.

Question 84

What medication class is used to prevent variceal hemorrhage in cirrhosis patients?
A. Antibiotics
B. Anticoagulants
C. Beta-blockers
D. Diuretics

Answer 84

Answer: C. Beta-blockers

Rationale: Beta-blockers are used to reduce the risk of variceal hemorrhage in patients with cirrhosis. They lower the blood pressure within the veins of the esophagus and stomach, reducing the risk of bleeding from varices.

Question 85

In the case of hepatic encephalopathy, what is the primary treatment goal?
A. To reduce blood ammonia levels
B. To increase protein intake
C. To improve renal function
D. To enhance cardiac output

Answer 85

Answer: A. To reduce blood ammonia levels

Rationale: The primary treatment goal in hepatic encephalopathy is to reduce the levels of ammonia and other nitrogenous waste products that the liver is unable to detoxify due to impaired function. Medications like lactulose and rifaximin are used to decrease ammonia-producing bacteria in the gut.

Question 86

What is the purpose of prophylactic endoscopic variceal banding in patients with cirrhosis?
A. To improve liver function
B. To prevent the formation of varices
C. To prevent bleeding from existing varices
D. To treat hepatic encephalopathy

Answer 86

Answer: C. To prevent bleeding from existing varices

Rationale: Endoscopic variceal banding is performed to place bands around the varices, which can help prevent them from bleeding. It is a prophylactic measure to reduce the risk of one of the most dangerous complications of cirrhosis.

Question 87

What is a distinctive feature of Hepatopulmonary Syndrome?
A. Platypnea
B. Hypertension
C. Ascites
D. Jaundice

Answer 87

Answer: A. Platypnea

Rationale: Hepatopulmonary Syndrome is characterized by the presence of platypnea—hypoxemia when upright—which improves when lying down, and is associated with intrapulmonary vascular dilatation.

d/t R to L intrapulmonary shunt

Question 88

Which treatments are used to manage Portopulmonary Hypertension?
A. Beta-blockers
B. Diuretics
C. Prostacyclin analogs and endothelin receptor antagonists
D. Antiviral medication

Answer 88

Answer: C. Prostacyclin analogs and endothelin receptor antagonists

Rationale: For Portopulmonary Hypertension, treatments include medications that promote vasodilation and inhibit vasoconstriction within the pulmonary circulation, such as prostacyclin analogs and endothelin receptor antagonists, which help to lower pulmonary pressure.

Question 89

Why is liver transplantation considered the only cure for complications like Portopulmonary Hypertension associated with cirrhosis?
A. It is the only way to restore normal pulmonary circulation.
B. Medications can manage symptoms but do not address the underlying liver disease.
C. Liver transplantation resolves both hepatic and pulmonary issues.
D. Other treatments have proven to be ineffective in reversing the condition.

Answer 89

Answer: C. Liver transplantation resolves both hepatic and pulmonary issues.

Rationale: Liver transplantation is considered the only cure for Portopulmonary Hypertension because it addresses the root cause of the condition, which is the cirrhotic liver. By transplanting a healthy liver, the abnormalities in the production of substances that affect the lung circulation can be corrected, resolving the pulmonary hypertension.

Question 90

In which of the following conditions is elective surgery contraindicated?
A. Acute cholecystitis
B. Chronic gastritis
C. Acute hepatitis
D. Chronic pancreatitis

Answer 90

Answer: C. Acute hepatitis

Rationale: Elective surgery is contraindicated in acute hepatitis due to the risk of exacerbating liver injury and potentially leading to liver failure.

Question 91

Which scoring system includes points based on bilirubin, albumin, prothrombin time, encephalopathy, and ascites to determine the severity and prognosis of liver disease?
A. Child-Pugh Score
B. APACHE II Score
C. Ranson’s Criteria
D. Glasgow Coma Scale

Answer 91

Answer: A. Child-Pugh Score (CTP)

Rationale: The Child-Pugh Score, also known as the CTP score, incorporates bilirubin, albumin, prothrombin time, encephalopathy, and ascites to assess the severity of liver disease and predict prognosis.

Question 92

Which scoring system is based on bilirubin, INR, creatinine, and sodium levels to evaluate the severity of liver disease and predict survival in patients awaiting liver transplantation?
A. Child-Turcotte-Pugh (CTP) Score
B. Model for End-Stage Liver Disease (MELD) Score
C. Maddrey’s Discriminant Function
D. Rockall Score

Answer 92

Answer: B. Model for End-Stage Liver Disease (MELD) Score

Rationale: The Model for End-Stage Liver Disease (MELD) Score utilizes bilirubin, INR, creatinine, and sodium levels to estimate the severity of liver disease and predict survival in patients awaiting liver transplantation.

Question 93

Which type of fluid is preferred over crystalloids for resuscitation in patients with liver disease?
A. Crystalloids
B. Hypertonic saline
C. Colloids
D. Albumin

Answer 93

Answer: C. Colloids

Rationale: Colloids are preferred over crystalloids for resuscitation in patients with liver disease due to their ability to maintain intravascular volume more effectively and reduce the risk of fluid overload and ascites formation.

Question 94

Why is succinylcholine (Succs) or cisatracurium considered ideal for neuromuscular blockade in patients with liver disease?
A. They have rapid onset of action
B. They are not metabolized by the liver
C. They have a short duration of action
D. They do not cause histamine release

Answer 94

B. They are not metabolized by the liver,

However Plasmacholinesterasemay be decreased in severe liver dz

Question 95

What is the primary purpose of the Transjugular Intrahepatic Portosystemic Shunt (TIPS) procedure?
A. To increase portal vein pressure
B. To decrease systemic circulation
C. To reduce the portosystemic pressure gradient
D. To induce portal vein thrombosis

Answer 95

Answer: C. To reduce the portosystemic pressure gradient

Rationale: The TIPS procedure is performed to create a shunt between the hepatic vein and the portal vein, effectively diverting blood flow away from the liver and reducing the pressure in the portal system, thus decreasing the portosystemic pressure gradient.

Question 96

What are the indications for performing a TIPS procedure?
A. Hypertension and ascites
B. Variceal hemorrhage and ascites
C. Heart failure and tricuspid regurgitation
D. Pulmonary hypertension and variceal hemorrhage

Answer 96

Answer: B. Variceal hemorrhage and refractory ascites

Rationale: TIPS procedure is indicated in cases of refractory variceal hemorrhage and refractory ascites, where conservative management has failed to control bleeding or fluid accumulation.

Question 97

Which of the following conditions is a contraindication for TIPS procedure?
A. Heart failure
B. Hypertension
C. Cirrhosis
D. Ascites

Answer 97

Answer: A. Heart failure

Rationale: Heart failure, tricuspid regurgitation, and severe pulmonary hypertension are contraindications for TIPS procedure due to the risk of exacerbating cardiac dysfunction or increasing pulmonary vascular resistance.

Question 98

What is the primary purpose of maintaining a low central venous pressure (CVP) by fluid restriction prior to liver resection?
A. To increase blood loss during surgery
B. To reduce blood loss during surgery
C. To induce hypotension in the patient
D. To minimize postoperative pain

Answer 98

Answer: B. To reduce blood loss during surgery

Rationale: Maintaining a low CVP by fluid restriction prior to liver resection helps to reduce blood loss during surgery by minimizing hepatic venous pressure and intraoperative bleeding.

Question 99

Why is it essential for patients undergoing partial hepatectomy resection to have invasive monitoring?
A. To measure cardiac output and systemic vascular resistance
B. To monitor blood pressure and heart rate only
C. To assess respiratory function
D. To measure urine output

Answer 99

Answer: A. To measure cardiac output and systemic vascular resistance

Rationale: Invasive monitoring, such as arterial line placement, allows continuous monitoring of blood pressure and enables measurement of cardiac output and systemic vascular resistance, which are crucial in managing hemodynamic stability during liver resection.

Question 100

What is a common postoperative pain management method for patients undergoing liver resection?
A. Epidural analgesia
B. Patient-controlled analgesia (PCA)
C. Intramuscular injections
D. Topical analgesic creams

Answer 100

Answer: B. Patient-controlled analgesia (PCA)

Rationale: Patients undergoing liver resection often require postoperative pain management, and PCA is a common method used to provide controlled pain relief while minimizing opioid-related side effects.

Question 101

What is the most common indication for liver transplantation in patients with end-stage liver disease (ESLD)?
A. Fatty liver disease
B. Hepatocellular carcinoma (HCC)
C. Alcoholic liver disease
D. Viral hepatitis

Answer 101

Answer: C. Alcoholic liver disease

Rationale: Alcoholic liver disease is the most common indication for liver transplantation in patients with ESLD, followed by hepatitis C, fatty liver disease, and hepatocellular carcinoma.

Question 102

What is a key consideration in intraoperative management during liver transplantation to ensure organ perfusion in brain dead donors?
A. Monitoring heart rate
B. Monitoring blood glucose levels
C. Maintaining hemodynamic stability
D. Monitoring urine output

Answer 102

Answer: C. Maintaining hemodynamic stability

Rationale: Intraoperative management during liver transplantation focuses on maintaining hemodynamic stability, including blood pressure and perfusion pressure, to ensure adequate organ perfusion in brain dead donors.

Question 103

Which monitoring modalities are commonly used during liver transplantation surgery?
A. MRI and PET scans
B. Echocardiography and CT angiography
C. Arterial line (A-line), central venous catheter (CVC), pulmonary artery (PA) catheter, transesophageal echocardiography (TEE)
D. EEG and EMG

Answer 103

Answer: C. Arterial line (A-line), central venous catheter (CVC), pulmonary artery (PA) catheter, transesophageal echocardiography (TEE)

Rationale: Intraoperative monitoring during liver transplantation commonly includes the placement of an arterial line (A-line), central venous catheter (CVC), pulmonary artery (PA) catheter, and transesophageal echocardiography (TEE) for hemodynamic monitoring and assessment of cardiac function.

Question 104

during liver transplant, clamping and reperfusion

Answer 104

worried about hypocalcemia, hyperkalemia, hypoglycemia and pulm emboli