Chronic Liver Disease 1 and 2: Cause and Clinical Features
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Questions and Answers

What characterizes chronic liver disease compared to acute liver injury?

  • Shorter duration than six months
  • Complete recovery is typically expected
  • More rapid onset of symptoms
  • Progressive deterioration of liver function for over six months (correct)

    • Which of the following is a known cause of chronic liver disease?

  • Hepatitis A
  • Ischaemic hepatitis
  • Paracetamol overdose
  • Fatty liver disease (correct)

    • What process leads to cirrhosis in chronic liver disease?

  • Increased bile production
  • Acute liver injury
  • Chronic inflammation and fibrosis (correct)
  • Rapid cell division

    • Which symptom is NOT typically associated with chronic liver disease?

    <p>Acute abdominal pain</p> Signup and view all the answers

    How can chronic liver disease potentially be reversed in its early stages?

    <p>By addressing the underlying cause and fibrosis</p> Signup and view all the answers

    Which of the following liver diseases can progress from acute to chronic?

    <p>Acute alcohol-associated hepatitis</p> Signup and view all the answers

    What is the primary focus of investigations in chronic liver disease?

    <p>To identify the underlying cause and extent of liver damage</p> Signup and view all the answers

    What role does fibrosis play in chronic liver disease?

    <p>It represents the scarring from chronic liver injury</p> Signup and view all the answers

    What marks the transition point to irreversible fibrosis in liver disease?

    <p>Activation of hepatic stellate cells</p> Signup and view all the answers

    Which complication is most commonly associated with cirrhosis?

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

    How does portal hypertension affect the hepatic sinusoids?

    <p>Leads to blood vessel constriction and high pressure</p> Signup and view all the answers

    Activation of hepatic stellate cells in response to liver injury leads to what change?

    <p>Increased fibrogenesis</p> Signup and view all the answers

    Which of the following factors leads to an increase in blood volume in the portal system during portal hypertension?

    <p>Activation of renin-angiotensin-aldosterone system</p> Signup and view all the answers

    What is the main consequence of the liver's failure to synthesize coagulation factors?

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

    Which of the following describes a significant sign of hepatic encephalopathy?

    <p>Neurological symptoms from toxin buildup</p> Signup and view all the answers

    What results from the increased resistance to blood flow in the portal circulation during cirrhosis?

    <p>Backflow and alternative circulation routes</p> Signup and view all the answers

    Which symptom is indicative of jaundice in a patient with cirrhosis?

    <p>Accumulation of bilirubin in skin</p> Signup and view all the answers

    What is a potential effect on hormone metabolism in a failing liver?

    <p>Accumulation of aldosterone leading to edema</p> Signup and view all the answers

    What is the appropriate treatment to address confusion in a patient with chronic liver disease?

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

    Why is Lorazepam not suitable for managing confusion in patients with liver disease?

    <p>It causes sedation that can worsen encephalopathy.</p> Signup and view all the answers

    Which of the following statements about prednisolone therapy is accurate?

    <p>Bone protection should be considered with prolonged steroid use.</p> Signup and view all the answers

    What is a common side effect of prolonged steroid use like prednisolone?

    <p>Increased susceptibility to infections.</p> Signup and view all the answers

    What condition does lactulose primarily treat in patients with liver disease?

    <p>Hepatic encephalopathy.</p> Signup and view all the answers

    What is a concern for patients receiving long-term steroid therapy?

    <p>Increased risk of adrenal crisis.</p> Signup and view all the answers

    What is an inappropriate use of Senna in patients with liver disease?

    <p>To reduce ammonia levels.</p> Signup and view all the answers

    What monitoring should be considered for patients on prolonged steroid treatment?

    <p>Bone density.</p> Signup and view all the answers

    What neurological symptoms are part of the clinical triad associated with Wernicke's encephalopathy?

    <p>Ophthalmoplegia and ataxia</p> Signup and view all the answers

    Which treatment is primarily used for managing Wernicke's encephalopathy and Korsakoff Psychosis?

    <p>Thiamine supplementation</p> Signup and view all the answers

    What is the main risk associated with Hepatitis C infections?

    <p>Cirrhosis and hepatocellular carcinoma (HCC)</p> Signup and view all the answers

    Which of the following statements is true regarding Hepatitis B?

    <p>There is a vaccine available</p> Signup and view all the answers

    What symptom can indicate haemochromatosis along with chronic iron overload?

    <p>Bronze skin hyperpigmentation</p> Signup and view all the answers

    What is the primary method used for the management of hereditary haemochromatosis?

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

    Which of the following is a characteristic of Wilson's disease?

    <p>Copper accumulation</p> Signup and view all the answers

    What is the common treatment for patients diagnosed with Wilson's disease?

    <p>D-Penicillamine</p> Signup and view all the answers

    Primary biliary cholangitis is predominantly found in which demographic?

    <p>Females of childbearing age</p> Signup and view all the answers

    What laboratory findings are typically associated with primary biliary cholangitis?

    <p>Elevated alkaline phosphatase and GGT</p> Signup and view all the answers

    What factor can delay the onset of hereditary haemochromatosis symptoms in women?

    <p>Menstrual loss of blood</p> Signup and view all the answers

    Which condition is characterized by a T-lymphocyte-mediated attack on bile ducts?

    <p>Primary biliary cholangitis</p> Signup and view all the answers

    What is a common misconception regarding Hepatitis C?

    <p>It is easily preventable by vaccination</p> Signup and view all the answers

    What is the function of D-penicillamine in Wilson's disease treatment?

    <p>Promotes urinary excretion of copper</p> Signup and view all the answers

    What is the primary cause of confusion in patients with liver disease such as Alcohol-Related Liver Disease?

    <p>Reduced ammonia excretion</p> Signup and view all the answers

    Which symptom is caused by excess bilirubin deposition in the skin?

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

    What condition leads to ascites in chronic liver disease patients?

    <p>Salt and fluid retention</p> Signup and view all the answers

    Which of the following best describes the metabolic syndrome associated with steatotic liver disease?

    <p>Insulin resistance, central obesity, and hypertension</p> Signup and view all the answers

    What is the significant role of thiamine deficiency in alcohol withdrawal?

    <p>Leads to neurological complications</p> Signup and view all the answers

    What is the primary diagnostic feature to differentiate MASLD from other liver diseases?

    <p>Obesity and metabolic syndrome features</p> Signup and view all the answers

    Which lab finding is typically seen in cases of alcohol-related liver disease?

    <p>AST:ALT ratio greater than 1.5</p> Signup and view all the answers

    What is a common cause of jaundice in chronic liver disease?

    <p>Hepatocyte damage and reduction in bilirubin conjugation</p> Signup and view all the answers

    Which lifestyle change is most recommended for managing chronic liver disease associated with metabolic dysfunction?

    <p>Weight loss and exercise</p> Signup and view all the answers

    What type of liver disease is associated with the consumption of high saturated calorie fats?

    <p>Metabolic dysfunction-associated steatotic liver disease</p> Signup and view all the answers

    Which condition is a common extra-hepatic manifestation of alcohol-related liver disease?

    <p>Chronic pancreatitis</p> Signup and view all the answers

    Which symptom is primarily associated with portal hypertension?

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

    What condition results from hepatic encephalopathy in liver disease patients?

    <p>Reduced GCS and asterixis</p> Signup and view all the answers

    Which statement is true regarding the diagnosis of Non-Alcoholic Fatty Liver Disease (NAFLD)?

    <p>Diagnosis involves ruling out other liver diseases.</p> Signup and view all the answers

    What is the potential risk associated with chronic alcohol use on brain function?

    <p>Cerebellar dysfunction</p> Signup and view all the answers

    What is a hallmark histological finding in autoimmune hepatitis?

    <p>Interface hepatitis with a lymphoplasmacytic infiltrate</p> Signup and view all the answers

    Which liver function test pattern indicates hepatocellular damage?

    <p>Elevated ALT and AST</p> Signup and view all the answers

    What is a critical diagnostic criterion for alcoholic liver disease?

    <p>AST/ALT ratio greater than 2:1</p> Signup and view all the answers

    Which treatment is considered first-line for autoimmune hepatitis?

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

    What imaging study is most appropriate to evaluate liver morphology for possible cirrhosis?

    <p>Liver ultrasound</p> Signup and view all the answers

    Which condition is characterized by interface hepatitis with lymphocytic infiltration?

    <p>Autoimmune hepatitis</p> Signup and view all the answers

    What does the MELD score predict?

    <p>Short-term mortality in cirrhotic patients</p> Signup and view all the answers

    Which sign is noted in a physical examination of a patient with chronic liver disease?

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

    What defines a liver biopsy as the 'gold standard' investigation?

    <p>It can provide specific diagnostic features of liver disease.</p> Signup and view all the answers

    What is a common cause of hypergammaglobulinemia in autoimmune hepatitis?

    <p>Presence of antibodies</p> Signup and view all the answers

    In diagnosing chronic liver disease, which blood test would most likely be abnormal?

    <p>Bilirubin levels</p> Signup and view all the answers

    What is the purpose of paracentesis in liver disease management?

    <p>To drain ascitic fluid and exclude spontaneous bacterial peritonitis</p> Signup and view all the answers

    What management strategy is crucial for a patient experiencing variceal hemorrhage?

    <p>Sengstaken-Blakemore tube for tamponade</p> Signup and view all the answers

    Which feature indicates cholestatic liver damage?

    <p>Elevated ALP and GGT</p> Signup and view all the answers

    What is the primary characteristic of Chronic Liver Disease (CLD)?

    <p>Progressive deterioration of liver functions for more than six months</p> Signup and view all the answers

    Which of the following liver diseases is specifically known to present acutely?

    <p>Hepatitis A</p> Signup and view all the answers

    Which condition is classified under Chronic Alcohol Related Liver Disease (ALD)?

    <p>Fatty liver disease</p> Signup and view all the answers

    Haemochromatosis is an example of a liver disease that can present in which manner?

    <p>Only chronic</p> Signup and view all the answers

    Which of the following is a common cause of Acute Liver Injury?

    <p>Alcohol-associated hepatitis</p> Signup and view all the answers

    Which of the following can progress from an acute to a chronic state?

    <p>Acute alcohol-associated hepatitis</p> Signup and view all the answers

    What is fibrosis in the context of liver disease?

    <p>Deposition of extracellular matrix (ECM) in response to chronic liver injury</p> Signup and view all the answers

    Which of the following statements about fibrosis is true?

    <p>It can be reversible in the initial stages of development.</p> Signup and view all the answers

    What is cirrhosis?

    <p>A final stage of chronic liver disease with disruption of liver architecture</p> Signup and view all the answers

    Which of the following features is associated with cirrhosis?

    <p>Formation of widespread nodules and vascular reorganization</p> Signup and view all the answers

    What is the significance of neo-angiogenesis in cirrhosis?

    <p>It contributes to the reorganization of vascular structures in the liver.</p> Signup and view all the answers

    Which statement best describes the transition from reversible fibrosis to irreversible fibrosis?

    <p>The transition point is still not completely understood.</p> Signup and view all the answers

    Which of the following best describes the extracellular matrix (ECM) in chronic liver disease?

    <p>It plays a role in the pathogenesis of fibrosis.</p> Signup and view all the answers

    Which cells initiate the injury pathway in the liver?

    <p>Hepatic stellate cells (HSC)</p> Signup and view all the answers

    Where are hepatic stellate cells (HSCs) typically located?

    <p>In the space between sinusoids and hepatocytes</p> Signup and view all the answers

    What is the primary function of quiescent hepatic stellate cells?

    <p>To metabolize Vitamin A</p> Signup and view all the answers

    What occurs in hepatic stellate cells (HSCs) in response to liver injury?

    <p>They are activated and transform into fibroblasts.</p> Signup and view all the answers

    What is a significant consequence of the pro-inflammatory phase in liver injury?

    <p>Increased fibrosis due to HSC activation</p> Signup and view all the answers

    What percentage of cardiac output does the liver receive?

    <p>25%</p> Signup and view all the answers

    What is the primary function of hepatic sinusoids?

    <p>To filter blood and facilitate nutrient exchange</p> Signup and view all the answers

    In cirrhosis, what do sinusoidal endothelial cells (SECs) produce that leads to vasoconstriction?

    <p>Endothelin-1 (ET-1)</p> Signup and view all the answers

    What is the result of increased production of nitric oxide (NO) and endothelin-1 (ET-1) in cirrhosis?

    <p>Constriction of blood vessels leading to portal hypertension</p> Signup and view all the answers

    What term describes the increased pressure in the portal vein?

    <p>Portal hypertension</p> Signup and view all the answers

    How does portal hypertension affect the splanchnic circulation?

    <p>It increases nitric oxide levels, causing dilation of blood vessels.</p> Signup and view all the answers

    Activation of which system is a compensatory response to portal hypertension?

    <p>Renin-angiotensin-aldosterone system (RAAS)</p> Signup and view all the answers

    What is a consequence of activating the renin-angiotensin-aldosterone system (RAAS) in the context of portal hypertension?

    <p>Increased sodium and water retention</p> Signup and view all the answers

    What range of cardiac output does the liver receive?

    <p>800-1200 mL/min</p> Signup and view all the answers

    What is portal hypertension primarily caused by in the context of cirrhosis?

    <p>Increased resistance to blood flow due to fibrosis</p> Signup and view all the answers

    What happens to blood pressure when resistance to blood flow increases in the portal circulation?

    <p>Blood pressure increases and backs up into other veins.</p> Signup and view all the answers

    What condition may develop due to pressure backing up into the gastro-oesophageal veins?

    <p>Oesophageal or gastric varices</p> Signup and view all the answers

    What is the clinical consequence of splenic vein hypertension due to portal hypertension?

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

    Which condition is characterized by distended veins around the umbilicus due to portal hypertension?

    <p>Caput medusae</p> Signup and view all the answers

    What is a potential risk associated with oesophageal or gastric varices in the context of portal hypertension?

    <p>Life-threatening variceal bleeding</p> Signup and view all the answers

    Which of the following proteins is primarily synthesized by the liver and is crucial for maintaining oncotic pressure?

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

    What is the primary role of thrombopoietin, a protein produced by the liver?

    <p>To stimulate platelet production</p> Signup and view all the answers

    Which substance is involved in the detoxification of endogenous nitrogenous compounds by the liver?

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

    What is the role of caeruloplasmin in the liver?

    <p>It transports copper in the bloodstream.</p> Signup and view all the answers

    What characterizes the acute deterioration of liver function in a patient with cirrhosis?

    <p>Development of complications such as jaundice or ascites</p> Signup and view all the answers

    Which of the following is the most common complication of cirrhosis?

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

    What is the underlying cause of jaundice in patients with decompensated cirrhosis?

    <p>Impaired excretion of bilirubin</p> Signup and view all the answers

    What neurological condition is associated with hepatic encephalopathy in cirrhosis?

    <p>Build-up of toxins such as ammonia</p> Signup and view all the answers

    Which complication of cirrhosis is primarily due to portal hypertension?

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

    Which of the following describes the term 'decompensation' in the context of liver cirrhosis?

    <p>Acute deterioration leading to complications</p> Signup and view all the answers

    What is the main reason patients with decompensated cirrhosis are at high risk of in-hospital death?

    <p>They require complex medical management for multiple complications.</p> Signup and view all the answers

    What is the mechanism thought to contribute to hepatic encephalopathy?

    <p>Accumulation of ammonia and other toxins crossing the blood-brain barrier</p> Signup and view all the answers

    What is the recently renamed term for Non-Alcoholic Fatty Liver Disease (NAFLD)?

    <p>Metabolic dysfunction-associated steatotic liver disease</p> Signup and view all the answers

    Which condition is the most common cause of chronic liver disease in the Western world?

    <p>Non-Alcoholic Fatty Liver Disease (NAFLD)</p> Signup and view all the answers

    Which type of liver disease is most strongly associated with diabetes?

    <p>Non-Alcoholic Fatty Liver Disease (NAFLD)</p> Signup and view all the answers

    What is a major cause of liver disease and a leading cause of liver-related mortality?

    <p>Alcoholic Liver Disease (ALD)</p> Signup and view all the answers

    Which viral hepatitis can have a chronic course and potentially lead to cirrhosis?

    <p>Hepatitis B</p> Signup and view all the answers

    Which of the following is classified as an autoimmune liver disease?

    <p>Primary biliary cholangitis</p> Signup and view all the answers

    Which condition is characterized by copper accumulation in the liver?

    <p>Wilson's Disease</p> Signup and view all the answers

    What is the primary defect in Alpha-1 Antitrypsin Deficiency?

    <p>Deficiency of a protein that protects the liver and lungs</p> Signup and view all the answers

    Which autoimmune condition is characterized by the destruction of bile ducts?

    <p>Primary sclerosing cholangitis</p> Signup and view all the answers

    What is the most common cause of chronic liver disease in Ireland and the Western World?

    <p>Metabolic Dysfunction-Associated Steatotic Liver Disease (MASLD)</p> Signup and view all the answers

    Which of the following conditions is part of the metabolic syndrome associated with MASLD?

    <p>Insulin Resistance</p> Signup and view all the answers

    Which of the following is NOT associated with Metabolic Dysfunction-Associated Steatotic Liver Disease (MASLD)?

    <p>Crohn's Disease</p> Signup and view all the answers

    Which of the following is a key component in the diagnosis of MASLD?

    <p>Fatty liver on ultrasound</p> Signup and view all the answers

    Which of the following is the primary treatment for Metabolic Dysfunction-Associated Steatotic Liver Disease?

    <p>Lifestyle Modification (diet, exercise, weight loss)</p> Signup and view all the answers

    Which of the following medications is increasingly used for managing MASLD?

    <p>GLP-1 Analogues</p> Signup and view all the answers

    Which of the following is the primary enzyme responsible for ethanol metabolism in hepatocytes?

    <p>Alcohol dehydrogenase (ADH)</p> Signup and view all the answers

    Which of the following complications is commonly associated with chronic alcohol use and cerebellar damage?

    <p>Cerebellar atrophy leading to chronic cerebellar ataxia</p> Signup and view all the answers

    What is the primary toxic byproduct of ethanol metabolism that contributes to liver injury in alcohol-related liver disease?

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

    Which of the following is a key neurological complication associated with thiamine deficiency in chronic alcoholics?

    <p>Both B and C</p> Signup and view all the answers

    What complication is associated with abrupt alcohol withdrawal and changes to neurotransmitter channels?

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

    Which vitamin deficiency is most commonly associated with alcohol use disorder and can lead to Wernicke-Korsakoff syndrome?

    <p>Vitamin B1 (Thiamine)</p> Signup and view all the answers

    What is the classic triad of symptoms seen in Wernicke's encephalopathy?

    <p>Confusion, ataxia, ophthalmoplegia</p> Signup and view all the answers

    Korsakoff psychosis, the chronic phase of Wernicke-Korsakoff syndrome, is primarily characterized by which of the following?

    <p>Memory disturbances and confabulation</p> Signup and view all the answers

    What is the mainstay of treatment for Wernicke's encephalopathy in patients with alcohol use disorder?

    <p>Intravenous thiamine (Pabrinex)</p> Signup and view all the answers

    In alcohol withdrawal, what role does thiamine (Vitamin B1) play in cerebral metabolism?

    <p>It is essential for neuronal energy metabolism and prevents neuronal cell death.</p> Signup and view all the answers

    Which brain structure is most affected by thiamine deficiency, classically seen in Wernicke-Korsakoff syndrome?

    <p>Mammillary bodies</p> Signup and view all the answers

    Which of the following is NOT a typical manifestation of Korsakoff psychosis?

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

    What is the genetic mutation most commonly responsible for hereditary haemochromatosis (HH)?

    <p>C282Y mutation on the HFE gene on chromosome 6</p> Signup and view all the answers

    Which of the following is NOT a common organ affected by iron accumulation in hereditary hemochromatosis?

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

    What is the first-line treatment for hereditary hemochromatosis?

    <p>Venesection (phlebotomy)</p> Signup and view all the answers

    In Wilson's disease, which organ shows Kayser-Fleischer rings, a diagnostic feature of copper deposition?

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

    What is the most commonly used medication to promote copper excretion in Wilson's disease?

    <p>D-Penicillamine</p> Signup and view all the answers

    Which of the following is a key distinguishing feature of Wilson's disease in neurological presentations?

    <p>Dysarthria, tremor, parkinsonism</p> Signup and view all the answers

    In Wilson's disease, which lab finding would you expect?

    <p>High serum copper, low caeruloplasmin</p> Signup and view all the answers

    What is the key distinguishing factor between Wernicke's encephalopathy and Wilson's disease when considering neurological symptoms?

    <p>Wernicke's is due to thiamine deficiency, Wilson's is due to copper accumulation.</p> Signup and view all the answers

    What is the hallmark diagnostic antibody for Primary Biliary Cholangitis (PBC)? A) Anti-Smooth Muscle Antibody (SMA) B) Antimitochondrial Antibody (AMA) C) Anti-Liver-Kidney Microsomal Antibody (Anti-LKM) D) Anti-Nuclear Antibody (ANA)

    <p>Antimitochondrial Antibody (AMA)</p> Signup and view all the answers

    Which liver enzyme is typically elevated in cholestatic liver diseases like PBC and PSC?

    <p>Alkaline Phosphatase (ALP)</p> Signup and view all the answers

    Which of the following conditions is most frequently associated with Primary Sclerosing Cholangitis (PSC)?

    <p>Ulcerative Colitis</p> Signup and view all the answers

    Which imaging modality is typically used to diagnose Primary Sclerosing Cholangitis (PSC) by revealing the characteristic 'beaded' appearance of the bile ducts?

    <p>MRCP (Magnetic Resonance Cholangiopancreatography)</p> Signup and view all the answers

    Which of the following is a characteristic feature of Autoimmune Hepatitis on liver biopsy?

    <p>Interface hepatitis with lymphoplasmacytic infiltrate</p> Signup and view all the answers

    What is the first-line treatment for Primary Biliary Cholangitis (PBC)?

    <p>Ursodeoxycholic Acid</p> Signup and view all the answers

    Which of the following autoimmune liver diseases has a strong association with xanthomas and xanthelasma due to raised cholesterol?

    <p>Primary Biliary Cholangitis (PBC)</p> Signup and view all the answers

    Which autoantibodies are commonly positive in Autoimmune Hepatitis (AIH)?

    <p>Anti-Liver-Kidney Microsomal Antibodies (Anti-LKM), ANA, and SMA</p> Signup and view all the answers

    What is a major long-term complication of Primary Sclerosing Cholangitis (PSC)?

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

    What is the main method for diagnosing Autoimmune Hepatitis (AIH)?

    <p>Blood tests for specific autoantibodies</p> Signup and view all the answers

    What demographic is most commonly affected by chronic inflammatory liver disease?

    <p>Middle-aged women</p> Signup and view all the answers

    Which symptom is least likely to present in a patient with chronic inflammatory liver disease?

    <p>Anorexia nervosa</p> Signup and view all the answers

    What radiological feature is characteristic of primary sclerosing cholangitis?

    <p>Beaded appearance of the bile duct</p> Signup and view all the answers

    What is a common risk associated with chronic inflammatory liver disease?

    <p>Progression to hepatocellular carcinoma</p> Signup and view all the answers

    What is a common clinical feature of chronic inflammatory liver disease?

    <p>Weight loss and malaise</p> Signup and view all the answers

    Which liver enzyme elevation indicates hepatocyte damage?

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

    What is the major component assessed in the CHILD-PUGH score?

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

    Which test is considered the gold standard for assessing liver fibrosis?

    <p>Liver Biopsy</p> Signup and view all the answers

    An AST to ALT ratio greater than 2:1 typically indicates what condition?

    <p>Alcohol-associated hepatitis</p> Signup and view all the answers

    Which imaging technique is best suited for evaluating the biliary tree?

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

    What laboratory finding is most indicative of chronic liver disease regarding synthetic function?

    <p>Raised INR</p> Signup and view all the answers

    What is the primary role of serum A1AT in liver evaluations?

    <p>Screen for A1AT deficiency</p> Signup and view all the answers

    What does a raised level of alkaline phosphatase (ALP) primarily indicate?

    <p>Cholestatic/obstructive liver injury</p> Signup and view all the answers

    Which medication is primarily used to lower ammonia levels in patients with hepatic encephalopathy?

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

    What is the recommended target hemoglobin level during resuscitation in cases of variceal hemorrhage?

    <p>7-9 g/dL</p> Signup and view all the answers

    In chronic liver disease, what is a significant role of beta blockers in patient management?

    <p>Preventing variceal hemorrhage</p> Signup and view all the answers

    Which sign on a physical examination would be indicative of portal hypertension?

    <p>Caput medusae</p> Signup and view all the answers

    Among the following, which is NOT a pharmacological treatment for portal hypertension?

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

    What is the primary role of lactulose in the management of hepatic encephalopathy?

    <p>Acidify intestinal contents</p> Signup and view all the answers

    Which of the following complications is most concerning with each episode of variceal hemorrhage?

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

    For a patient receiving long-term prednisolone therapy, which precaution should be emphasized?

    <p>Bone protection should be considered</p> Signup and view all the answers

    Which of the following represents an inappropriate treatment in the context of hepatic encephalopathy management?

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

    What is a potential benefit of early involvement in palliative care for chronic liver disease patients?

    <p>Optimization of symptoms</p> Signup and view all the answers

    Study Notes

    Chronic Liver Disease (CLD) and Cirrhosis

    • CLD is a progressive deterioration of liver function lasting more than six months.
    • Cirrhosis is the final stage of CLD, leading to irreversible liver damage and disruption of its architecture.
    • CLD and Cirrhosis can be caused by alcohol, viral hepatitis, metabolic dysfunction, genetic disorders, and autoimmune diseases.

    Pathophysiology of CLD

    • Hepatic stellate cells (HSCs), normally dormant, activate in response to liver injury.
    • Activated HSCs transform into fibroblasts and produce extracellular matrix (ECM), leading to fibrosis.
    • Fibrosis is the deposition of ECM in response to chronic liver injury.
    • Fibrosis in the initial stage might be reversible.
    • Portal hypertension occurs due to increased resistance to blood flow within the portal circulation.
    • Portal hypertension leads to an increase in nitric oxide (NO) and endothelin-1 (ET-1) production, causing blood vessel constriction and high pressure in the liver vascular bed.
    • The liver receives 25% of the cardiac output.

    Clinical Features of CLD

    • Fatigue and weakness are common due to malnutrition, inflammation, and poor diet.
    • Nausea and vomiting are common due to poor gastric emptying and ascites.
    • Jaundice (yellowing of the skin) is due to the accumulation of bilirubin.
    • Ascites (accumulation of fluid in the abdomen) is due to portal hypertension, reduced oncotic pressure, and fluid retention.
    • Hepatic encephalopathy is a range of neurological symptoms thought to be caused by a build-up of toxins.
    • Variceal hemorrhage is a life-threatening complication of portal hypertension where esophageal varices rupture.

    Causes of Chronic Liver Disease

    Metabolic Dysfunction-Associated Steatotic Liver Disease (MASLD)

    • It is the leading cause of chronic liver disease in Ireland and the Western world.
    • Steatosis refers to the accumulation of lipid droplets within hepatocytes.
    • MASLD can be caused by a combination of factors like insulin resistance, central obesity, hypertension, and hyperlipidaemia.
    • Lifestyle modifications, including diet, exercise, and weight loss, are crucial for managing MASLD.
    • Ethanol is metabolized by alcohol dehydrogenase (ADH) in hepatocytes, creating acetaldehyde, a highly reactive and toxic compound.
    • Chronic alcohol use can lead to cerebellar dysfunction, peripheral neuropathy, pancreatitis, and cardiomyopathy.
    • Diagnosis of ALD includes documentation of alcohol consumption, clinical or biochemical features of liver injury, and a specific LFT pattern.

    Viral Hepatitis

    • Hepatitis A (HAV) and E (HEV) are usually acute, while Hepatitis B (HBV) and C (HCV) can become chronic.
    • Hepatitis B can be prevented through vaccination.
    • Hepatitis C is curable with direct-acting antivirals.
    • Hepatitis B is managed by viral suppression with nucleoside analogues.

    Haemochromatosis

    • It is an autosomal recessive disorder where excessive iron is absorbed via the intestine, leading to total-body iron overload.
    • This disorder can lead to cirrhosis, cardiomyopathy, diabetes, pituitary hypogonadism, arthritis, and osteoporosis.
    • Symptoms include fatigue, erectile dysfunction, bronze skin hyperpigmentation.
    • Treatment involves venesection and iron chelators.

    Wilson's Disease

    • It is an autosomal recessive disorder of copper metabolism due to ATP7B mutations.
    • Impairment of biliary copper excretion leads to copper accumulation, most notably in the liver, brain, and cornea.
    • Symptoms include Kayser-Fleischer rings around the cornea, cirrhosis, and neurological symptoms.
    • Treatment focuses on promoting copper excretion and interfering with copper uptake from the digestive tract.

    Primary Biliary Cholangitis (PBC)

    • It is an autoimmune liver disease characterized by T-lymphocyte-mediated attack on small bile ducts, leading to their destruction and cholestasis.
    • Associated conditions include Sjogren's syndrome, autoimmune thyroid disease, rheumatoid arthritis, and celiac disease.
    • Symptoms include fatigue, pruritis, and signs of CLD.
    • It is a progressive disease that can lead to end-stage liver disease.

    Autoimmune Hepatitis (AIH)

    • It is a clinical diagnosis based on exclusion of other CLD causes, hypergammaglobulinaemia, hepatocellular LFT pattern, positive autoantibodies, and specific liver biopsy findings.
    • AIH responds well to corticosteroids and immunosuppressants like azathioprine or mycophenolate mofetil.

    Liver Function Tests (LFTs)

    • Liver enzymes (ALT, AST, ALP, GGT) indicate damage to different parts of the liver.
    • Liver function tests measure bilirubin, albumin, prothrombin time, and platelets.
    • Raised ALT/AST: indicate hepatocyte damage.
    • Raised GGT/ALP: indicate biliary tree injury.
    • Raised bilirubin: visible jaundice when levels exceed 50.
    • Alcohol-associated hepatitis: AST>ALT ratio is >2:1.

    Investigations - Bloods

    • Coagulation profile: Raised INR and reduced coagulation factor production indicate impaired liver synthetic function.
    • Albumin: Reduced levels indicate impaired synthetic function.
    • FBC:
      • Anaemia may be present.
      • Thrombocytopenia (low platelets) may be present.
    • Viral Hepatitis Screen: Identifies exposure to Hep A, B, C, EBV, CMV, VZV, HIV, and VDRL.
    • Autoantibody screen:
      • ANA, SMA, Anti-LKM: used to diagnose Autoimmune Hepatitis (AIH).
      • Anti Smooth muscle Ab: used to diagnose Primary Sclerosing Cholangitis (PSC).
      • AMA: Anti Mitochondrial Ab: used to diagnose Primary Biliary Cholangitis (PBC).
    • Iron studies and ferritin:
      • Raised Ferritin and T sat %; HFE genotype are suggestive of Haemochromatosis.
    • Caeruloplasmin and serum copper: Reduced levels may indicate Wilson's Disease.
    • Serum A1AT Level: Reduced levels may indicate Alpha 1 Antitrypsin Deficiency.

    Investigations - Imaging

    • Liver Ultrasound: Evaluates the liver morphology for nodularity or coarse texture of cirrhosis, ascites, hepatomegaly/splenomegaly, and HCC.
    • CT/MRI Liver/MRCP: Provides high-quality images to further evaluate liver tumours, morphology, and the biliary tree to investigate PSC/PBC.
    • OGD: To screen for or monitor oesophageal varices.

    Special Tests

    • Liver elastography (Fibroscan): Can quantify hepatic steatosis and fibrosis.
    • Liver Biopsy: Gold standard investigation for liver disease, but invasive and may not always be required.

    Scoring Systems in Cirrhosis

    • CHILD-PUGH SCORE: Estimates cirrhosis mortality and classifies patients as A, B, or C based on total bilirubin, albumin, INR, ascites, and hepatic encephalopathy.
    • MELD (Mayo End-Stage Liver Disease): Predicts 3-month mortality among cirrhotic patients listed for liver transplantation by measuring serum bilirubin, serum creatinine, and INR.

    Management

    • Prevention:
      • Alcohol cessation.
      • Avoid hepatotoxic medications (paracetamol, NSAIDs, opiates).
      • Weight loss and diet management.
      • Optimisation of DM for hepatic steatosis.
      • Hep A and B vaccination.
    • Pharmacological:
      • Alcohol cessation pharmacotherapy.
      • GLP1 analogues for metabolic syndrome.
      • Beta Blockers for portal hypertension/varices.
      • Lactulose for encephalopathy (increases bacterial uptake of ammonia).
      • Rifaxamin for encephalopathy.
      • Diuretics and low salt diet for ascites and oedema management.
    • Procedures:
      • Paracentesis to exclude SBP and to drain ascitic fluid.
      • OGD banding of varices.
      • Trans-jugular Intrahepatic Portosystemic Shunt.
    • Surgical: Liver transplant is the definitive management.
    • MDT: Involves dietician, physiotherapy, and pharmacy.
    • Palliative care: Can be involved early for symptom optimisation and advanced care planning.

    Medical Emergency: Variceal Haemorrhage

    • Rupture of oesophageal varices.
    • Overall mortality per episode is approximately 15-25% at six weeks.
    • Sengstaken-Blakemore Tube: A balloon tamponade inserted into the oesophagus to manually apply pressure to the bleeding vessel. Used in rare cases of unstable, uncontrolled haemorrhage when endoscopy is not possible.
    • Resuscitation: ABCs, IV fluids, blood transfusion aiming for Hb 7-9g/dL.
    • Pharmacological Management:
      • Terlipressin.
      • Antibiotic prophylaxis (Ceftriaxone). -Source Control:
      • Endoscopy.
      • Band ligation.
      • Sengstaken-Blakemore Tube (if necessary). -Long-term:
        • Prophylaxis: Non-cardioselective beta-blockers: propranolol.

    Case Presentation - Planning Management

    • Case 1: A 65-year-old gentleman with a history of haemochromatosis and chronic liver disease presents to the emergency department with acute confusion and behavioural disturbance. Physical examination reveals asterixis, distended abdomen with shifting dullness, and caput medusae.
    • Answer: Lactulose is an appropriate treatment for this gentleman's confusion, as it lowers ammonia levels and is indicated in the treatment of hepatic encephalopathy. Aim for 3 bowel motions per day.
    • Additional information: Rifaximin is also indicated in addition to lactulose. Senna is a stimulant laxative and has no role in ammonia reduction. Lorazepam sedation does not address the underlying cause and will cause encephalopathy. Cefotaxime is an antibiotic used to treat spontaneous bacterial peritonitis.

    Case Presentation - Providing Information

    • Case 2: A 55-year-old lady presents to the hepatology service with jaundice and hepatocellular derangement of her liver function tests. She is a non-drinker with no metabolic risk factors and a normal ferritin level. Her ANA and SMA antibodies are positive. The hepatology team prescribes a prolonged tapering course of prednisolone.
    • Answer: Bone protection should be considered with prolonged steroid use.
    • Additional Information: Prolonged steroid use can lead to osteoporosis, gastric ulceration, hypertension, diabetes, and an adrenal crisis if stopped abruptly.

    Autoimmune Hepatitis

    • Chronic, inflammatory disease of the liver.
    • Characterized by circulating autoantibodies and elevated serum globulin levels.
    • Challenging to diagnose and manage due to its rarity and heterogeneity.
    • More common in females (4:1 ratio).

    Clinical Features

    • Wide range of symptoms, from asymptomatic to severe liver disease.
    • Classically presents in middle-aged women, but can occur at any age in both sexes.
    • Common symptoms include fatigue, malaise, weight loss, nausea, itching (pruritis), jaundice, joint pain (polyarthralgia), and irregular periods (oligomenorrhea).
    • Can progress to liver cancer (HCC).

    Primary Sclerosing Cholangitis (PSC) Diagnosis

    • Characterized by distinctive radiological features.
    • Multifocal, short, ring-shaped strictures (narrowings) in the bile ducts, alternating with normal or slightly widened segments.
    • This creates a "beaded" appearance on imaging.
    • Long strictures can also occur and raise concern for bile duct cancer (cholangiocarcinoma).

    Liver Function Tests (LFTs)

    • Elevated ALT/AST: indicate hepatocyte damage
    • Elevated GGT/ALP: indicate biliary tree injury
    • Elevated bilirubin: can indicate both hepatocellular and cholestatic/obstructive damage, visible jaundice when >50
    • AST>ALT ratio >2:1: may suggest alcohol-associated hepatitis

    Investigations

    • Bloods:

      • LFTs: assess liver function and damage
      • Coagulation profile: Raised INR, reduced coagulation factor production - measure of liver synthetic function
      • Albumin: Reduced- measure of synthetic function
      • FBC: assess for anemia and thrombocytopenia (low platelets)
      • Viral Hepatitis Screen: Hep A, B, C, EBV, CMV, VZV, HIV, VDRL
      • Autoantibody screen: ANA, SMA, Anti-LKM- AIH, Anti Smooth muscle Ab- PSC AMA: Anti Mitochondrial Ab-PBC
      • Iron studies and ferritin: raised ferritin and T sat %; HFE genotype
      • Caeruloplasmin and serum copper: reduced in Wilson's
      • Serum A1AT Level: reduced in A1AT deficiency

    • Imaging:

      • Liver Ultrasound: evaluate for nodularity, cirrhosis, ascites, hepatomegaly, splenomegaly, HCC
      • CT/MRI Liver: higher quality images, evaluate for liver tumors and morphology
      • MRCP: evaluate the biliary tree, PSC/PBC
      • OGD: screen/surveillance of esophageal varices

    • Special Tests:

      • Liver elastography (Fibroscan): evaluate for hepatic steatosis and fibrosis
      • Liver Biopsy: gold standard, invasive, may not be required if other investigations suggest CLD/Cirrhosis

    Scoring Systems in Cirrhosis

    • CHILD-PUGH SCORE: estimates cirrhosis mortality, classified A, B, or C, based on:
      • Total bilirubin
      • Albumin
      • INR
      • Ascites
      • Hepatic encephalopathy
    • MELD Score: Mayo End-Stage Liver Disease (MELD):
      • Predictor of 3-month mortality in cirrhotic patients
      • Based on serum bilirubin, creatinine, and INR
      • Used for liver transplant prioritization

    Management

    • Prevention:
      • Alcohol cessation
      • Avoid hepatotoxic medications (paracetamol, NSAIDs, opiates)
      • Weight loss and diet management
      • Hep A and B vaccination
    • Pharmacological:
      • Alcohol cessation pharmacotherapy
      • GLP1 analogues for metabolic syndrome
      • Beta Blockers for portal hypertension/varices
      • Lactulose for encephalopathy (increases bacterial uptake of ammonia)
      • Rifaximin for encephalopathy
      • Diuretics and low salt diet for ascites and edema management
    • Procedures:
      • Paracentesis to exclude SBP and to drain ascitic fluid
      • OGD banding of varices
      • Trans-jugular Intrahepatic Portosystemic Shunt
    • Surgical: Liver transplant - definitive management
    • MDT: Dietician, physiotherapy, pharmacy involvement
    • Palliative care: can be involved for symptom optimization and advanced care planning

    Medical Emergency: Variceal Haemorrhage

    • Rupture of esophageal varices:
      • Overall mortality: 15-25% at 6 weeks
    • Sengstaken-Blakemore Tube: balloon tamponade, only indicated in rare cases of uncontrolled bleeding when endoscopy is not possible
    • Resuscitation: ABCs, IV fluids, blood transfusion
    • Pharmacological Management: Terlipressin, antibiotic prophylaxis (Ceftriaxone)
    • Source Control: Endoscopy, band ligation, or Sengstaken-Blakemore tube
    • Long-term Prophylaxis: Non-cardioselective beta blockers (propranolol)

    Case Presentations

    • Case 1: Patient with acute confusion, asterixis, distended abdomen, and caput medusae.
      • Treatment for confusion: Lactulose
      • Other treatment options: Rifaximin
      • Lactulose lowers ammonia levels, treating hepatic encephalopathy
      • Senna is a stimulant laxative, not indicated
      • Lorazepam sedation does not address the underlying cause and can worsen encephalopathy
      • Cefotaxime is used for spontaneous bacterial peritonitis
    • Case 2: Patient with jaundice, hepatocellular derangement, negative risk factors, positive ANA/SMA antibodies
      • Treatment: Prolonged tapering course of prednisolone
      • Important information:
        • Bone protection is crucial with prolonged steroid use
        • Gastric protection is recommended with prednisolone
        • Patients can experience hypotension on steroids
        • Steroids can increase the risk of diabetes
        • Abruptly stopping steroids can be dangerous

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    Description

    This quiz explores Chronic Liver Disease (CLD) and its progression to cirrhosis. It covers the causes, pathophysiology, and complications associated with liver damage. Test your knowledge on how liver injury leads to fibrosis and portal hypertension.

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