Liver and Biliary System Diseases II

Question 1

Symptoms of liver disease occur late and are characterised by:

Answer 1

i. Jaundice
or features of hepatic decompensation, such as:
ii. Ascites
iii. Peripheral oedema
iv. Portal hypertensive GI bleeding
v. Encephalopathy

JAPEP

Question 2

Mention 4 examples of Liver disease

Answer 2

i. Acute liver disease
ii. Chronic liver disease
iii. Cirrhosis
iv. Viral hepatitis (A, B, C, D, E, G)

Question 2

What are the symptoms that characterise biliary disorders?

Answer 2

i. Upper abdominal pain
ii. Jaundice
iii. Fever
iv. Weight loss

Question 3

Mention 4 examples of Biliary disorders.

Answer 3

i. Gallstone disease
ii. Cholecystitis: inflammation of the gallbladder, often due to gallstones blocking the cystic duct
iii. Mirizzi syndrome: a rare condition where a gallstone becomes impacted in the cystic duct or neck of the gallbladder, causing compression of the common bile duct.
iv. Cholangitis: an infection of the bile ducts, often due to bile duct obstruction, commonly by gallstones
v. Biliary tract malignancies, cysts and others.

Question 4

Mention 3 examples of pancreatic disorder.

Answer 4

i. Pancreatitis
ii. Pancreatic cancer
iii. Exocrine pancreatic insufficiency (EPI)

Question 5

What is Acute Liver Failure (ALF)?

Answer 5

This is an uncommon condition characterised by rapid deterioration of liver function, resulting in coagulopathy (with INR greater than 1.5) and encephalopathy (altered mental status ) in previously healthy individuals.

Question 6

What are the symptoms of ALF?

Answer 6

i. Encephalopathy
ii. Cerebral edema / signs Increased intracranial pressure e.g. papilledema, hypertension, bradycardia
iii. Jaundice
iv. Ascites
v. Tenderness of the abdominal upper right quadrant
vi. Change in liver size:
shrinking due to liver necrosis or enlargement due to heart failure, viral hepatitis
vii. Hematemesis or melena
viii. Hypotension and tachycardia: due to reduced systemic vascular resistance

Question 7

The most important step in assessing patients with ALF is _________.

Answer 7

Identifying the cause

Question 8

What evaluations should be carried out on patients with laboratory evidence of moderate or severe hepatitis?

Answer 8

Immediate measurement of Prothrombin time (PT) and careful evaluation of mental status.

Question 9

The presence of PT prolongation or mental status change is not enough grounds for hospitalisation.

True or False.

Answer 9

False.

The presence of these two is in fact grounds for hospitalisation.

Question 10

Mention 8 laboratory tests that are conducted in the investigation of ALF.

Answer 10

1. Complete blood count: to reveal thrombocytopenia
2. Coagulation studies: PT time or INR
3. Liver function tests:
   i. AST
   ii. Serum glutamic-oxaloacetic transaminase (SGOT)
   iii. ALT
   iv. Serum glutamic-pyruvic transaminase (SGPT)
   v. ALP
4. Serum bilirubin level: Elevated
5. Acetaminophen and acetaminophen-protein adducts levels
6. Drug screening
7. Blood cultures: For patients with suspected infection
8. Viral serologies: hepatitis A, B, C, D, E

Question 11

What medications may be used in emergency response to ALF?

Answer 11

1. Antidotes
2. Osmotic diuretics (e.g. mannitol) to decrease cerebral edema
3. Barbiturates (e.g. pentobarbital, thiopental) to check unresponsive intracranial hypertension
4. Benzodiazepines (e.g. midazolam) used during intubation
5. Anesthetics (e.g. propofol) decreases cerebral blood flow and intracranial hypertension

Question 12

What are the antidotes for acetaminophen poisoning?

Answer 12

i. N-acetylcysteine
ii. Activated charcoal (if less than 4 hrs)

Question 13

What would you use as an antidote for mushroom (Amanita phalloides) poisoning?

Answer 13

Activated charcoal
Penicillin G

Question 14

_________ possesses antioxidant properties that benefit liver disease management and is used as an antidote.

Answer 14

Silymarin

Question 15

What is Cirrhosis?

Answer 15

It is a diffuse hepatic process characterised by fibrosis and the conversion of normal liver architecture into structurally abnormal nodules.

In most patient, cirrhosis is not a reversible process.

Question 16

With appropriate examples, mention the causes of cirrhosis.

Answer 16

1. Drugs and Toxins e.g. Alcohol, Methotrexate, Isoniazid, Methyldopa, Amiodarone.
2. Infections e.g. Hepatitis B and C, Schistosomiasis.
3. Immune-mediated e.g. Autoimmune hepatitis, Primary sclerosing cholangitis.
4. Metabolic e.g. Hemochromatosis, Porphyria, α1-antitrypsin deficiency, Wilson’s disease
5. Biliary Obstruction e.g. Cystic fibrosis, Strictures, Gallstones.
6. Cardiovascular e.g. Chronic Right-sided Heart Failure, Budd-Chiari syndrome, Veno-occlusive disease.
7. Cryptogenic

Question 17

Cirrhosis results in the elevation of portal blood pressure. How does this occur?

Answer 17

i. Fibrotic changes with the hepatic sinusoids

ii. Increased vascular resistance in the liver as a result of a change in the level of vasodilatory (e.g. Nitrous oxide) and vasoconstrictive mediators .

iii. Increase in systemic artery vasodilation or in blood flow to the splanchnic vasculature

Question 18

List 7 signs and symptoms (complications) of cirrhosis?

Answer 18

1. Portal hypertension
2. Ascites: pathological accumulation of lymph fluid in the peritoneal cavity. Progressive systemic vasodilation results in decreased arterial blood volume, triggering baroreceptors in the kidney and activation of RAAS, resulting in plasma volume expansion, which is translocated from the liver sinusoids and splanchnic capillaries to the peritoneum.
3. Hepatic encephalopathy
4. Hepatic fibrosis: caused by the activation of stellate cells into collagen-forming cells by paracrine factors.
5. Hepatorenal syndrome: constriction of renal arteries and impaired perfusion that occurs in patients with cirrhosis and ascites
6. Coagulopathy: reduction in the synthesis of coagulation factors and the clearance of activated clotting factors due to cirrhosis. Vitamin K–dependent clotting factors, including factor VII, are affected early.
7. Oesophageal varices: the pressure build-up in the liver results in blood drainage from alternative routes, which manifest as varices. Oesophageal varices can only be identified by endoscopy.
8. Hepatopulmonary Syndrome
9. Endocrine dysfunction
10. Other signs and symptoms include: anorexia, fatigue, jaundice, weight loss, muscle wasting, pruritus, spider angioma, skin telangiectasis, finger clubbing etc.

Question 19

American Association for the study of Liver Diseases (AASLD) suggested that measurement of _________ is the gold-standard method to assess the presence of clinically significant portal hypertension (CSPH) - defined as an HVPG of ___________

Answer 19

hepatic venous pressure gradient (HVPG)
at least 10 mm Hg

Question 20

The primary strategy for the treatment of esophageal varices is __________.

Answer 20

the reduction of portal hypertension

Question 21

The major complications of cirrhosis that require therapeutic intervention include:

Answer 21

i. Portal hypertension and variceal bleeding
ii. Ascites and spontaneous bacterial peritonitis
iii. Hepatic encephalopathy
iv. Other systemic complications

Question 22

In patients with cirrhosis, the goal of treatment is to:

Answer 22

i. Prevent the development of clinically significant portal hypertension (CSPH), decompensation and even to achieve regression of cirrhosis.

ii. Eliminate the etiologic agent (the current mainstay of therapy)

Question 23

What is the general approach to treatment of cirrhosis?

Answer 23

1. Identify and eliminate, where possible, the causes of cirrhosis (e.g., alcohol abuse).
2. Assess the risk for variceal bleeding and begin pharmacologic prophylaxis when indicated.
3. Evaluate the patient for clinical signs of ascites and manage with pharmacologic and non-pharmacological therapy (e.g., diuretics and paracentesis).
4. Careful monitoring for spontaneous bacterial peritonitis should be employed in patients with ascites who undergo acute deterioration.
5. Clinical vigilance and treatment with dietary restriction, elimination of central nervous system depressants, and therapy to lower ammonia, to counter encephalopathy

Question 24

___________________ is the recommended treatment of choice in patients with acute bleeding.

Answer 24

Variceal obliteration with endoscopic techniques

Question 25

Highlight the management of PHT & Variceal Bleeding in Cirrhosis.

Answer 25

i. Primary prophylaxis: non-selective beta- blockers (e.g. propranolol 40mg or nadolol). Used in patients with medium to large varices.

ii. Synthetic prodrugs of vasopressin e.g. Octreotide, Terlipressin, Somatostatin
(cause selective mesenteric/splanchnic vasoconstriction)

iii. Others:
-Endoscopic intervention: sclerotherapy, band ligation
- Surgical treatment approaches

Question 26

Highlight the management of Ascites in Cirrhosis.

Answer 26

i. Use of diuretics e.g. Spironolactone (50-100mg/day), Furosemide
ii. Paracentesis: drainage of ascitic fluid by needle or catheter
iii. Low salt diet
iv Alcohol abstinence

Question 27

Highlight the management of Hepatic Encephalopathy in Cirrhosis.

Answer 27

i. Diet therapy to lower blood ammonia: Low protein diet
ii. Lactulose syrup: reduces the amount of ammonia in the blood, reducing fluid imbalance and encephalopathy
iii. Avoiding precipitating factors, such as GI bleeding, infection, drugs (e.g. opioids)

Question 28

Highlight the management of Pruritus in Cirrhosis.

Answer 28

1. Anion exchange resins: First line of therapy in pruritus. (e.g. Cholestyramine, Colestipol (4g once or twice daily) act by binding bile acids and preventing their reabsorption in the GIT.
2. Antihistamines – Cetirizine 10mg, Loratadine.
3. Ursodeoxycholic Acid (UDCA) -10mg/kg daily in two divided doses (indicated for long term treatment of pruritus)
4. Opioid Antagonists – e.g. Naloxone, Naltrexene, Nalmefene

Question 29

What therapy is used in the management of autoimmune hepatitis?

Answer 29

i. Prednisolone
ii. Azathioprine

Question 30

What therapy is used in the management of primary biliary cholangitis?

Answer 30

Ursodeoxycholic Acid (UDCA)

Question 31

What therapy is used in the management of Hepatitis B and C?

Answer 31

Interferon (IFN α) and other antiviral agents e.g. Tenofovir

Vaccination

Question 32

Mention 3 non-pharmacological approaches in the treatment of cirhosis.

Answer 32

i. Nutrition and exercise - Multiple feedings per day, including breakfast and a snack at night, are specified. Regular exercise is encouraged in patients with cirrhosis, to prevent them from slipping into inactivity and muscle wasting.

ii. Transjugular intrahepatic portosystemic shunt (TIPS)

iii. Liver Transplantation:
Some contraindications for liver transplant include severe cardiovascular or pulmonary disease, active drug or alcohol abuse, malignancy outside the liver, sepsis, or psychosocial problems that might jeopardize patients’ abilities to follow their medical regimens after transplant

Question 33

Formula for MELD Scoring system.

Answer 33

MELD Score = 3.78 loge (serum bilirubin [mg/dL]) + 11.2 loge (INR) + 9.57 loge (serum creatinine [mg/dL]) + 6.43

Question 34

What are the Child-Turcotte-Pugh (CTP) classifications?

Answer 34

Child A - Score (5 – 6) Well Compensated
Child B - Score (7 – 9) Significant functional Compromised
Child C - Score (10 – 15) Decompensated

Question 35

What are the dosage adjustments for the CTP classifications?

Answer 35

Child A - Normal dose
Child B - 25% decrease
Child C - 50% decrease

Question 36

What are the parameters used in the CTP scoring system?

Answer 36

i. Ascites
ii. Encephalopathy
iii. Bilirubin level (mg/dL)
iv. Albumin level (g/dL)
v. Prothrombin time or INR