Medicine Marrow Pg No 1025-1034 (Hepatology)
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Questions and Answers

Which HLA type is associated with Type-II autoimmune hepatitis?

  • HLA DR-9
  • HLA DR-7 (correct)
  • HLA DR-4
  • HLA DR-5

    • Type-II autoimmune hepatitis is commonly seen in adults.

    False

    Name one disease association with Type-II autoimmune hepatitis.

    Diabetes mellitus Type-1

    Treatment for diagnosed AIH includes steroid _____ mg/kg/day for 4 weeks.

    <p>Img</p> Signup and view all the answers

    Match the following treatment outcomes with their descriptions:

    <p>Symptomatic improvement = Reduction in symptoms like fatigue and jaundice Histological improvement = Changes observed in liver tissue indicating recovery Decreased S. bilirubin = Lower levels of bilirubin in the blood Liver enzyme back to normal = Restoration of normal liver function tests</p> Signup and view all the answers

    What is the cirrhogenic dose for females in grams per day?

    <p>20-40g/day</p> Signup and view all the answers

    Alcoholic fatty liver is irreversible after 3-5 years.

    <p>False</p> Signup and view all the answers

    What is the best serum marker for alcoholic hepatitis?

    <p>Carbohydrate deficient transferrin</p> Signup and view all the answers

    The primary enzyme responsible for converting acetaldehyde into less toxic substances is __________.

    <p>Aldehyde dehydrogenase</p> Signup and view all the answers

    Match the following liver conditions with their characteristics:

    <p>Alcoholic Hepatitis = High mortality rate of 70% Cirrhosis = Long-term damage from alcohol Alcoholic Fatty Liver = Reversible condition HCC = Risk associated with excessive alcohol consumption</p> Signup and view all the answers

    Which of the following symptoms is commonly associated with Nonalcoholic Steatohepatitis (NASH)?

    <p>Vague RUQ pain</p> Signup and view all the answers

    Elevated ALT and AST levels suggest a stage with cirrhosis in NASH.

    <p>True</p> Signup and view all the answers

    What is the significance of a biopsy in the investigation of NASH?

    <p>It shows inflammatory changes.</p> Signup and view all the answers

    The sign indicating liver enlargement in NASH is called _____ .

    <p>Hepatomegaly</p> Signup and view all the answers

    Match the following findings with their possible interpretations in the context of NASH:

    <p>ALT &lt; AST = Normal ALT &gt; AST = Stage with cirrhosis Normal ALT and AST + Normal IFL = NASH without cirrhosis Elevated ALT and AST + Elevated IFL = NASH with risk of cirrhosis</p> Signup and view all the answers

    Which of the following is a classical histopathological feature of alcoholic hepatitis?

    <p>Centrilobular fatty infiltration</p> Signup and view all the answers

    A Discriminant Factor score of 32 or more indicates a poor prognosis.

    <p>True</p> Signup and view all the answers

    What is the initial treatment dosage of steroids for patients with a Discriminant Factor score of 32 or higher?

    <p>1 mg/kg x 7 days tapering over 3 weeks</p> Signup and view all the answers

    Pentoxifylline inhibits _____ to help manage alcoholic hepatitis.

    <p>TNF-α</p> Signup and view all the answers

    Match the following complications of alcoholic hepatitis with their corresponding symptoms:

    <p>Encephalopathy = Confusion, altered mental state Coagulopathy = Increased bleeding tendency Portal HTN = Increased pressure in the portal vein Fulminant hepatitis = Rapid onset liver failure</p> Signup and view all the answers

    What is the most common site affected in Budd Chiari Syndrome?

    <p>Hepatic vein</p> Signup and view all the answers

    Myeloproliferative neoplasm is a cause of Budd Chiari Syndrome.

    <p>True</p> Signup and view all the answers

    Name one type of involvement classified under Budd Chiari Syndrome.

    <p>Extrahepatic, Pre-sinusoidal, Intrahepatic, Post-sinusoidal, or Post Hepatic</p> Signup and view all the answers

    Budd Chiari Syndrome is classified as a ______ disease.

    <p>primary</p> Signup and view all the answers

    Which of the following is a common risk factor for Nonalcoholic Steatohepatitis (NASH)?

    <p>Obesity</p> Signup and view all the answers

    Match the following causes of Terminal IVC involvement with their descriptions:

    <p>Polycythemia rubra vera = Condition associated with increased red blood cell mass Paroxysmal nocturnal hemoglobinuria = Rare disease causing episodes of dark urine due to hemolysis Pregnancy/postpartum = Physiological changes during and after pregnancy affecting venous structures</p> Signup and view all the answers

    What is a significant pathological finding in alcoholic liver disease?

    <p>Centrilobular necrosis</p> Signup and view all the answers

    NASH is characterized by macrovesicular fat in less than 25% of hepatocytes.

    <p>False</p> Signup and view all the answers

    Fatty liver disease is indicated by fat in more than 5% of hepatocytes.

    <p>True</p> Signup and view all the answers

    What is a potential progression pathway for NASH?

    <p>NASH leads to cirrhosis, which can progress to portal hypertension, liver failure, and potentially hepatocellular carcinoma.</p> Signup and view all the answers

    What is the primary treatment option recommended for improving Nonalcoholic Steatohepatitis (NASH)?

    <p>Vitamin E</p> Signup and view all the answers

    Weight loss of 5% over 6 months is recommended as supportive treatment for NASH.

    <p>False</p> Signup and view all the answers

    What are Mallory bodies indicative of in alcoholic liver disease?

    <p>Endoplasmic reticulum stress</p> Signup and view all the answers

    One drug potentially associated with NASH is __________.

    <p>Valproate</p> Signup and view all the answers

    Match the following conditions with their associations to NAFLD:

    <p>Obstructive sleep apnea = Other conditions associated with NAFLD Obesity = Common conditions associated with NASH Hypothyroidism = Other conditions associated with NAFLD Dyslipidemia = Common conditions associated with NASH</p> Signup and view all the answers

    The toxic effect of acetaldehyde results in increased NADH and decreased ________.

    <p>glutathione</p> Signup and view all the answers

    What is the duration of exercise recommended per week for supportive treatment in NASH?

    <p>30 - 45 minutes /day x 5 day / wk</p> Signup and view all the answers

    The drug known to have unknown efficacy in cirrhosis but improves NASH is __________.

    <p>Vitamin E</p> Signup and view all the answers

    Match the following laboratory findings with their corresponding features:

    <p>Neutrophilia = Increase in total count AST:ALT = 73:1 ratio indication Macrocytosis = Increased mean corpuscular volume S. Albumin = Negative acute phase reactant</p> Signup and view all the answers

    Match the following drugs with their classifications:

    <p>Vitamin E = Supportive treatment for NASH Saroglitazar = Dual PPAR-α + PPAR-γ agonist Pioglitazone = PPAR-γ agonist Oral Semaglutide = GLP-1 receptor agonist</p> Signup and view all the answers

    What is the most common cause of cirrhosis?

    <p>Non-alcoholic steatohepatitis (NASH)</p> Signup and view all the answers

    Macrovesicular steatosis is primarily caused by Reye's syndrome.

    <p>False</p> Signup and view all the answers

    What condition is characterized by swollen mitochondria with reduced numbers?

    <p>Reye's syndrome</p> Signup and view all the answers

    Microvesicular steatosis can be associated with _____ liver disease.

    <p>alcoholic</p> Signup and view all the answers

    Match the types of cirrhosis with their causes:

    <p>Micronodular cirrhosis = Indian childhood cirrhosis (ICC) Macronodular cirrhosis = Wilson's disease</p> Signup and view all the answers

    What is the prognosis for a patient with hepatic venous outflow obstruction?

    <p>3-year survival rate of less than 10%</p> Signup and view all the answers

    Increased sinusoidal portal pressure is one of the possible mechanisms for hepatic venous outflow obstruction.

    <p>True</p> Signup and view all the answers

    What condition may lead to hypoxic damage to hepatocytes?

    <p>Zone 3 involvement due to hepatic venous outflow obstruction</p> Signup and view all the answers

    Patients presenting with refractory ascites are typically noted to have a high SAAG and high protein _____ .

    <p>ascites</p> Signup and view all the answers

    Match the symptoms with their respective presentations:

    <p>Abdominal pain = Acute hepatitis Hepatomegaly = Acute hepatitis Ascites = Subacute presentation Refractory ascites = Subacute presentation</p> Signup and view all the answers

    Study Notes

    Autoimmune Hepatitis

    • Type-II AIH is seen in children and has a poor prognosis.
    • Type-II AIH is associated with HLA DR-7.
    • Type-II AIH is associated with Diabetes mellitus Type-1, Vitiligo, and Autoimmune polyendocrine syndrome Type-1.
    • Treatment for diagnosed AIH includes steroids (1 mg/kg/day for 4 weeks) and azathioprine after 3-4 months.
    • AIH treatment involves tapering the steroid dosage over 12 weeks and regular liver function monitoring through Fibroscan.
    • AIH remission is characterized by symptom improvement, histological improvement, decreased serum bilirubin levels, and normalization of liver enzymes.
    • AIH relapse may require a liver transplant, which carries a risk of recurrence.

    Alcoholic Liver Disease

    • Cirrhogenic Dose: 40-80g/day for males and 20-40g/day for females (1 peg = 60ml alcohol, 40% ABV).
    • Cirrhosis development: Consuming the cirrhogenic dose 5 days a week for 15 years can lead to cirrhosis.
    • Alcoholic Hepatitis: 70% mortality rate, best serum marker is carbohydrate-deficient transferrin.
    • Alcoholic Hepatitis Progression: 3-5 years: Alcoholic fatty liver (reversible) → Cirrhosis.
      • Progressing cirrhosis characterized by micronodules.
      • Macronodules develop when the nucleus is pushed to the periphery.
    • Alcohol Metabolism: Enzymes involved include aldehyde dehydrogenase, Cytochrome P450, and catalase.
    • Toxic Effects: Poor alcohol metabolizers are prone to alcoholic hepatitis due to toxic acetaldehyde buildup.
    • Efficient metabolizers are less susceptible to alcoholic hepatitis.
    • Alcoholic Hepatitis (High Mortality): Caused by binge drinking and increases the risk of Hepatocellular Carcinoma (HCC).

    Nonalcoholic Steatohepatitis (NASH)

    • Clinical Features: Vague right upper quadrant (RUQ) pain, fatigue, malaise, hepatomegaly, splenomegaly, spider angiomata, palmar erythema, ascites (decompensation) in advanced cases.
    • Investigations:
      • Biopsy: Shows inflammatory changes and elevated serum ferritin.
      • Imaging: Ultrasound (USG) to MR elastography.
      • Enzymes: Elevated ALT and AST.
      • Liver Fibroscan: Used to assess fibrosis stages (F0-F4), with time (ms) and depth (mm) scales.
    • NASH Management:
      • ALT < AST: Normal liver function.
      • ALT > AST: Suggests cirrhosis.
      • Normal ALT and AST + Normal IFL (inflammatory fibrosis levels): NASH without cirrhosis.
      • Elevated ALT and AST + Elevated IFL: NASH with increased risk of cirrhosis.

    Hepatology - 1028

    • Histopathological Features: Centrilobular fatty infiltration (Zone III involvement), ballooning degeneration, and alcoholic hyaline surrounding polymorphonuclear infiltrate.
    • Note: Hepatitis C can cause fatty liver-like changes or hepatic steatosis.
    • Complications: Alcoholic hepatitis → Fulminant hepatitis or decompensation; Alcoholic hepatitis with nodules and fibrous septation → Encephalopathy, Coagulopathy, Portal hypertension, increased intracranial pressure (ICT), and death.
    • Prognostic Scores:
      • MELD: 4.6 x (PT(ರೆ) - PT(c)) + S.bilirubin.
      • Glasgow: Not specified.
    • Poor Prognosis: MELD score ≥ 32.
    • Treatment:
      • Steroid indication: DF (discriminant factor) Score ≥ 32.
      • Steroid start: 1 mg/kg x 7 days, tapered over 3 weeks.
      • Steroid contraindications: Sepsis, renal failure, ongoing gastrointestinal (GI) disease.
    • Medication:
      • Pentoxifylline: Inhibits TNF-α, 400 mg TDS x 4 weeks.

    Alcoholic Liver Disease - Adverse Effects

    • Mnemonic: HESST (Hypoxia, Endoplasmic reticulum stress, Superoxide, Isam/Isah, Toxic effect of acetaldehyde)
    • Hypoxia: Mitochondrial injury in Zone 3 (centrilobular hepatocyte involved).
    • Endoplasmic Reticulum Stress: Mallory hyaline bodies.
    • Superoxide: Reactive oxygen species produced by Cyt P450.
    • Isam/Isah: TNFα, the most important pathogenic factor.
    • Ex: Example: Pentoxifylline.
    • Toxic Effect of Acetaldehyde: Increased NADH, decreased glutathione, leaky gut.
    • Assinophilic Body Inside Hepatocyte: Mallory's hyaline bodies (endoplasmic reticulum stress).

    Alcoholic Liver Disease - Pathological Findings

    • Centrilobular Necrosis: Balooning degeneration of hepatocytes and Mallory bodies.
    • Fat in >5% Hepatocytes: Fatty Liver Disease.
    • Fat Pushing Nucleus to Periphery: Macronodules.

    Alcoholic Liver Disease - Presentation

    • Laboratory Features: Neutrophilia (increased total count), elevated liver enzymes (300-500 U/L, AST:ALT).
    • Clinical Features: Jaundice, soft and painful abdomen, hepatomegaly.
    • Likely Diagnosis: 73:1 Likely and highly suggestive of Alcoholic Hepatitis.
    • Additional Findings: Non-significant serum albumin (negative acute phase reactant), PT/INR 1, increased mean corpuscular volume (MCV) (macrocytosis), thiamine depletion.
    • Biochemical Pathway: Pyruvate ↔ Acyl-CoA, with a downward arrow to transketolase reaction.

    Alcoholic Liver Disease - Note

    • Most Common Cause of Cirrhosis: NASH.
    • Causes of Micronodular Cirrhosis: Alcoholic hepatitis, biliary cirrhosis, Indian childhood cirrhosis (ICC).
    • Causes of Macronodular Cirrhosis: Wilson's disease.
    • Micro vs. Macrovesicular Steatosis: Mnemonic: Pretty visits Jamaica with Alcohol and Acid.

    Microvesicular Steatosis

    • Pregnancy:
    • Reye's Syndrome:
    • Tetracycline:
    • Valproate:
    • Jamaican Vomiting Sickness:
    • Alcoholic Liver Disease:
    • Acid Lipase Deficiency:

    Macrovesicular Steatosis

    • HCV:
    • NASH:
    • Wilson's Disease:

    Other Observations

    • Reye's Syndrome: Swollen mitochondria with reduced numbers.
    • Nutmeg Liver: Chronic venous condition.
    • Mallory Denk Bodies: Endoplasmic reticulum stress (eosinophilic inclusion bodies) in hepatocytes.
    • Mallory Denk Bodies are Seen in: Wilson's disease, ICC, NASH, Alcohol/Alpha-1-antitrypsin deficiency, primary biliary cholangitis (PBC), Hepatocellular Carcinoma (HCC), cholestasis.
    • Mallory Denk Bodies are Not Seen in: HBV/HCV.

    Vascular Diseases of the Liver: Budd Chiari Syndrome (BCS)

    • Pathogenesis:
      • Vasculature: Efferent ductule of the right atrium.
      • Site: Most commonly affecting the hepatic vein.
      • Causes: Factor V Leiden mutation, deficiency of antithrombin III, protein C, or protein S, myeloproliferative neoplasm (MPN).
    • Terminal IVC: Caused by polycythemia rubra vera (PRV), paroxysmal nocturnal haemoglobinuria (PNH), pregnancy/postpartum.
    • Classification:
      • Primary: Inherited.
      • Secondary: Acquired.
    • Types of Involvement:
      • Extrahepatic: First-order portal vein involvement, extrahepatic portal vein occlusion (EHPVO), non-cirrhotic.
      • Pre-sinusoidal: Third-order vein involvement, non-cirrhotic portal fibrosis (NCPF).
      • Intrahepatic: Sinusoidal: Cirrhosis, portal hypertension.
      • Post-sinusoidal: Sinusoidal obstruction syndrome (veno occlusive), hepatic venous occlusion, thrombosis anywhere.
      • Post Hepatic: Hepatic venous obstruction, Budd-Chiari syndrome, cirrhosis.

    Hepatology - 1032

    • Treatment for NASH:
      • Supportive Treatment: Weight loss (10% over 6 months), exercise (30-45 minutes/day, 5 days/week), omega-3 fatty acids, caffeine.
      • DOC (Drug of Choice): Vitamin E: 800-1000 units/day. Improves NASH, unknown efficacy in cirrhosis. Takes ~2 years to show effect.
      • Saroglitazar (4mg): Dual PPAR-α + PPAR-γ agonist.
      • Pioglitazone: PPAR-γ agonist (may be tried).
      • Oral Semaglutide: GLP-1 Receptor agonist (RA).

    Nonalcoholic Steatohepatitis (NASH)

    • Features of Fatty Liver Disease (FLD):
      • NAFLD (Non-alcoholic fatty liver disease): Macrovesicular fat in >25% hepatocytes.
      • 80%: Isolated fatty liver (IFL).
      • 20%: Fatty liver + inflammation (NASH):
        • Ballooning change, degree of steatosis.
        • Lobular inflammation.
        • Macrovesicular fat in NAFLD.
        • Chicken-wire fibrosis (zone 3).

    Risk Factors for NASH

    • Common Conditions with Established Association: Obesity, type 2 diabetes mellitus (T2DM)/insulin resistance, dyslipidemia, metabolic syndrome, polycystic ovary syndrome, starvation/rapid weight loss, decreased adiponectin levels, leptin resistance.
    • Other Conditions Associated with NAFLD: Hypothyroidism, obstructive sleep apnea, hypopituitarism, hypogonadism, pancreatoduodenal resection, psoriasis.
    • Drugs Potentially Associated with NAFLD: L-asparaginase, bleomycin, tetracycline, griseofulvin, valproate, amiodarone.

    Progression of NASH

    • Hypothesis: "Hit" hypothesis: Risk factors impact a normal liver, leading to NASH progression.
    • Progression: Normal liver → risk factors → NASH → cirrhosis (10-15% of patients over 15 years) → portal hypertension → liver failure → hepatocellular carcinoma (HCC) (only in NASH and Hep B virus infection).

    Hepatology - Case Presentation

    • Patient: 30-40 years post-partum female.
    • Presentation: Ascites with zone 3 involvement, sinusoidal distension, and pooling.
    • Diagnosis (Prognosis): Very poor, 3-year survival rate < 10%. Hepatic venous outflow obstruction.
    • Possible Mechanisms:
      • Increased sinusoidal portal pressure.
      • Sinusoidal distension.
      • Extravasation of fluid from hepatic veins into sinusoids.
      • High SAAG (serum ascites albumin gradient), high protein ascites.
      • Venous stasis and congestion.
      • Hypoxic damage to hepatocytes (zone 3 involvement).
      • Hepatocyte necrosis (centrilobular), leading to lobar collapse.
      • Fibrosis, nodular regenerative hyperplasia.
      • Cirrhosis.
    • Subacute Presentation: Refractory ascites, high SAAG, high protein ascites, collaterals.
    • Acute Hepatitis Presentation: Abdominal pain, hepatomegaly, ascites, jaundice.

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    Test your knowledge on Autoimmune Hepatitis, particularly Type-II AIH, and its treatment options. Additionally, explore the impacts of Alcoholic Liver Disease including cirrhogenic doses and risk factors. This quiz covers essential concepts related to liver diseases.

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