Hepatology and Friends Flashcards
What is the definition of acute liver failure?
Decline in hepatic function where the liver loses the ability to repair or regenerate, characterised by jaundice, coagulopathy, and hepatic encephalopathy
What is the aetiology of acute liver failure?
Paracetamol overdose, alcohol, medications, infections, neoplasms, metabolic conditions, vascular conditions, autoimmune hepatitis
What is the clinical presentation of acute liver failure?
Jaundice, hepatic encephalopathy (confusion, altered mental status, asterixis (flapping tremor), and coma), hepatomegaly, ascites
What are the investigations of acute liver failure?
o LFTs: hyperbilirubinemia, elevated liver enzymes
o Prothrombin time: elevated (INR > 1.5)
o FBC: leukocytosis, anaemia, thrombocytopenia
o Liver biopsy: hepatocellular necrosis
What is the treatment of acute liver failure?
Treat underlying cause, IV mannitol (if raised ICP), lactulose (increases ammonia uptake in encephalopathy), liver transplantation
What is the definition of cholelithiasis?
Formation of gallstones in the gallbladder
What is the aetiology of cholelithiasis?
Bile supersaturated with cholesterol, gallbladder hypomotility
What is the clinical presentation of cholelithiasis?
Biliary colic (Right upper quadrant pain, which is worse after fatty meals, and may radiate to the epigastrium and back)
What are the investigations of cholelithiasis?
o Serum LFTs: elevated alkaline phosphatase, elevated bilirubin
o Abdominal ultrasound: stones in the gallbladder or bile duct with or without bile duct dilation
What is the treatment of cholelithiasis?
Opioid analgesia, IV fluids and nil by mouth, lithotripsy (fragment stones), laparoscopic cholecystectomy
What is the definition of acute cholecystitis?
Acute inflammation of the gallbladder
What is the aetiology of acute cholecystitis?
Cystic duct obstruction, bile inspissation, bile stasis
What is the clinical presentation of acute cholecystitis?
Right upper quadrant pain, fever, Murphy’s sign (tenderness which is worse on inspiration)
What are the investigations of acute cholecystitis?
o FBC: elevated WCC
o CRP: elevated
o Serum LFTs: elevated alkaline phosphatase, gamma glutamyl transferase, and bilirubin
o Abdominal ultrasound: gallstones, pericholecystic fluid, distended gallbladder, thickened gallbladder wall
What is the treatment of acute cholecystitis?
Opioid analgesia, IV fluids and nil by mouth, prophylactic antibiotics, laparoscopic cholecystectomy
What is the definition of ascending cholangitis?
Infection of the biliary tree
What is the pathophysiology of ascending cholangitis?
Obstruction of the common bile duct results in bacterial seeding of the biliary tree
What is the clinical presentation of ascending cholangitis?
Right upper quadrant pain, fever, jaundice (dark urine, pale stools, pruritus), altered mental state, hypotension
What are the investigations of ascending cholangitis?
o FBC: elevated WCC
o CRP: elevated
o Serum LFTs: elevated alkaline phosphatase, gamma glutamyl transferase, and bilirubin
o Abdominal ultrasound: dilated bile duct, common bile duct stones
o Magnetic resonance cholangiography: mass impinging on biliary tree, stricture, and/or choledocholithiasis
What is the treatment of ascending cholangitis?
IV antibiotics, fluids, opioid analgesia, drainage of the biliary system with ERCP, stenting, laparoscopic cholecystectomy
What is the definition of primary biliary cholangitis?
Progressive bile duct damage and eventual loss
What is the pathophysiology of primary biliary cholangitis?
Autoimmune destruction of biliary epithelial cells lining the small intra-hepatic bile ducts
What is the clinical presentation of primary biliary cholangitis?
Asymptomatic, hepatomegaly, pruritus, fatigue
What are the investigations of primary biliary cholangitis?
o ESR: elevated
o Serum LFTs: elevated alkaline phosphatase, gamma glutamyl transferase, and bilirubin
o Anti-mitochondrial antibody immunofluorescence: present
o Liver biopsy: non suppurative cholangitis of interlobular and septal bile ducts, granulomatous lymphocytic infiltration of portal triad
What is the treatment of primary biliary cholangitis?
Ursodeoxycholic acid (slows progression), supplementation of fat-soluble vitamins (A/D/E/K), methotrexate, liver transplantation
How does primary sclerosing cholangitis differ with primary biliary cholangitis?
Primary sclerosing cholangitis is associated with pANCA and inflammatory bowel disease
What is the definition of acute pancreatitis?
Acute inflammation of the pancreas associated with acinar cell injury with local and systemic inflammatory responses
What is the aetiology of acute pancreatitis?
Idiopathic, gallstones, ethanol, trauma, steroids, mumps, autoimmune, scorpion venom, hyperlipidaemia, hypercalcaemia, ERCP, drugs
What is the pathophysiology of acute pancreatitis?
Trypsinogen is converted to trypsin within the pancreas due to increased intracellular calcium which leads to a cascade of zymogen activation. The enzymes then undergo autodigestion of the pancreatic tissue. This triggers the recruitment of inflammatory cells and the release of inflammatory mediators
What is the clinical presentation of acute pancreatitis?
Epigastric or central abdominal pain (radiates to the back, sitting forward may relieve), nausea and vomiting, anorexia
What are the investigations of acute pancreatitis?
o Serum amylase or lipase: 3 times the upper limit of the normal range
o CT: diffuse or segmental enlargement of the pancreas with irregular contour and obliteration of the peripancreatic fat, necrosis, or pseudocysts
What is the treatment of acute pancreatitis?
Opioid analgesia, IV fluids and nil by mouth
What is the definition of chronic pancreatitis?
Chronic inflammatory process of the pancreas, due to structure changes such as fibrosis, atrophy, and calcification
What is the aetiology of chronic pancreatitis?
Alcohol
What is the pathophysiology of chronic pancreatitis?
Inappropriate activation of enzymes within the pancreas leads to precipitation of protein plugs within the duct lumen, forming a nidus for calcification. This causes a ductal obstruction and leads to ductal hypertension, and therefore pancreatic damage
What is the clinical presentation of chronic pancreatitis?
Epigastric pain (radiates to the back, sitting forward may relieve, worse upon eating and alcohol consumption), steatorrhea, weight loss and malnutrition
What are the investigations of chronic pancreatitis?
o Serum amylase and lipase: may be elevated but in advance disease, there may not be sufficient residual acinar cells to produce elevation
o Faecal elastase: low
o CT: pancreatic calcifications, focal or diffuse enlargement of the pancreas, ductal dilation, and/or vascular complications
What is the treatment of chronic pancreatitis?
Analgesia, lifestyle modifications
What is the definition of alcoholic liver disease?
Three stages of liver damage: fatty liver (steatosis), alcoholic hepatitis (inflammation and necrosis), and alcoholic liver cirrhosis
What is the pathophysiology of alcoholic liver disease?
Ethanol metabolism in the liver generates high levels of NADH which stimulates fatty acid synthesis and production of triglycerides, leading to steatosis. TNF-alpha release from Kupffer cells causes the release of reactive oxygen species which leads to hepatocyte injury and necro-inflammatory activity. Ongoing, this causes liver fibrosis which may progress to cirrhosis.
What is the clinical presentation of alcoholic liver disease?
Asymptomatic, hepatomegaly, (nausea, vomiting and diarrhoea can occur due to alcohol consumption)
What are the investigations of alcoholic liver disease?
o FBC: macrocytic anaemia, elevated WCC (in alcoholic hepatitis)
o LFTs: elevated aspartate transaminase and alanine aminotransferase (indicate liver damage), elevated bilirubin and gamma glutamyl transferase
o Liver biopsy:
oAlcoholic fatty liver – macrovesicular droplets of triglycerides
oAlcoholic hepatitis – ballooned hepatocytes that contain eosinophilic material called Mallory bodies, surrounded by neutrophils
oAlcoholic liver cirrhosis – destruction of liver architecture and fibrosis, regenerating nodules, Mallory bodies also present
What is the treatment of alcoholic liver disease?
Alcohol abstinence (withdrawal treated with diazepam), IV thiamine to prevent encephalopathy, liver transplantation, N-acetyl-cysteine (paracetamol OD)
How is the units of alcohol calculated?
(Strength (ABV) × Volume (ml) )/1000
What is the maximum recommended upper limit for alcohol intake?
14 units
What is the definition of non-alcoholic liver disease?
Macrovesicular hepatic steatosis in those who do not consume alcohol in amounts generally considered harmful to the liver
What is the clinical presentation of non-alcoholic liver disease?
Asymptomatic, hepatomegaly
What are the investigations of non-alcoholic liver disease?
o LFTs: mild increase in serum aminotransferases (AST and ALT) and gamma glutamyl transferase
o Liver ultrasound: steatosis in the absence of other injurious cases
o Liver biopsy: hepatocytes contain macrovesicular droplets of triglycerides
What is the treatment of non-alcoholic liver disease?
Lifestyle modifications
What is the definition of cirrhosis?
Fibrosis and conversion of normal liver architecture to structurally abnormal nodules known as regenerative nodules
What is the aetiology of cirrhosis?
Chronic liver diseases
What is the clinical presentation of cirrhosis?
Abdominal distention, jaundice and pruritis, haematemesis and melena, leukonychia, spider naevi, clubbing, palmar erythema, xanthelasma (yellow fat deposits under skin usually around eyelids), loss of body hair, hepatomegaly, bruising, ankle oedema, ascites
What are the investigations of cirrhosis?
o LFTs: elevated aspartate aminotransferase, alanine aminotransferase, alkaline phosphatase, and gamma-glutamyl transferase
o Ultrasound: liver surface nodularity, small liver
o Liver biopsy: architectural distortion of the liver parenchyma with formation of regenerative nodules
What is the treatment of cirrhosis?
Lifestyle modifications, liver transplant
What is the definition of hepatitis A?
Small, unenveloped RNA virus which is spread by the faecal-oral route and is associated with acute, self-limiting inflammation of the liver
What is the clinical presentation of hepatitis A?
Fever, malaise, nausea and vomiting, jaundice, hepatomegaly, right upper quadrant pain, clay-coloured stools
What are the investigations of hepatitis A?
o LFTs: elevated aspartate aminotransferase and alanine aminotransferase
o Anti-HAV immunoglobulins: elevated IgM in acute infection and IgG following infection
What is the treatment of hepatitis A?
Vaccination, supportive therapy
What is the definition of hepatitis B?
Enveloped dsDNA virus which is transmitted through blood products and bodily fluids and is associated with acute and chronic inflammation of the liver
What is the clinical presentation of hepatitis B?
Asymptomatic, fever, malaise, nausea and vomiting, anorexia right upper quadrant pain, clay-coloured stools, arthralgia, fatigue, headache, distaste for cigarettes, urticaria, hepatomegaly
What are the investigations of hepatitis B?
o FBC: microcytic anaemia (aplastic anaemia)
o LFTs: elevate aspartate aminotransferase, alanine aminotransferase, alkaline phosphatase, and bilirubin
o HBV serology:
oHBV core antibodies – positive in previous and chronic infections
oHBV surface antibodies – positive in vaccinated and previous infections
oHBV surface antigen – positive in chronic infections and up to 6 months post-acute infection
What is the treatment of hepatitis B?
Blood screening, safe sex, vaccination, supportive therapy, nucleoside analogues (tenofovir)
What is the definition of hepatitis C?
Enveloped RNA flavivirus which is transmitted through blood products and bodily fluids and is associated with acute and chronic inflammation of the liver
What is the clinical presentation of hepatitis C?
Asymptomatic, malaise, fatigue, intermittent right upper quadrant pain
What are the investigations of hepatitis C?
HCV serology:
oHCV antibodies – positive (can be negative in acute infection and in immunocompromised patients)
oHCV RNA – positive
What is the treatment of hepatitis C?
Pegylated interferon alpha and oral ribavirin, or a combination of directly acting anti-viral drugs, supportive therapy
What is the definition of hepatitis D?
Incomplete RNA virus enclosed in a shell of Hepatitis B surface antigen which is transmitted through blood products and bodily fluids and is associated with acute and chronic inflammation of the liver
What is the clinical presentation of hepatitis D?
Asymptomatic, fever, malaise, nausea and vomiting, anorexia right upper quadrant pain, clay-coloured stools, arthralgia, fatigue, headache, distaste for cigarettes, urticaria, hepatomegaly
What are the investigations of hepatitis D?
o HBV serology
o Anti-HDV antibodies – positive
What is the treatment of hepatitis D?
HBV vaccination, pegylated interferon-alpha 2A, liver transplant
What is the definition of hepatitis E?
Small, unenveloped RNA virus which is spread by the faecal-oral route and is associated with acute, self-limiting inflammation of the liver
What is the clinical presentation of hepatitis E?
Asymptomatic, fever, malaise, nausea and vomiting, anorexia right upper quadrant pain, clay-coloured stools, arthralgia, fatigue, headache, distaste for cigarettes, urticaria, hepatomegaly
What are the investigations of hepatitis E?
o LFTs: elevated aspartate aminotransferase and alanine aminotransferase
o Anti-HEV immunoglobulins: elevated IgM in acute infection and IgG following infection
What is the treatment of hepatitis E?
Supportive therapy
What is the definition of autoimmune hepatitis?
Chronic inflammatory disease of the liver of unknown aetiology
What is the clinical presentation of autoimmune hepatitis?
Asymptomatic, fatigue, malaise, anorexia, abdominal discomfort, hepatomegaly, jaundice
What are the investigations of autoimmune hepatitis?
o Anti-smooth muscle antibodies (ASMA) and anti-nuclear antibodies (ANA): positive in type I AIH
o Liver biopsy: mononuclear infiltrate of portal and periportal areas, and piecemeal necrosis with or without fibrosis
What is the treatment of autoimmune hepatitis?
Steroids, liver transplant
What is the definition of haemochromatosis?
Dysregulated dietary iron absorption and increased iron release from macrophages
What is the aetiology of haemochromatosis?
Autosomal recessive condition which occurs due to mutations in the haemochromatosis gene on the short arm of chromosome 6
What is the pathophysiology of haemochromatosis?
Hepatic hepcidin gene expression is inappropriately low, which permits ongoing duodenal absorption of iron despite replete iron stores
What is the clinical presentation of haemochromatosis?
Asymptomatic, fatigue, arthralgia, weakness, hepatomegaly, erectile dysfunction, decreased libido, skin bronzing, diabetes mellitus, cirrhosis, impotence, arrhythmia
What are the investigations of haemochromatosis?
o Serum transferrin saturation: increased
o Serum ferritin: increased
o Haemochromatosis gene genotyping
o Liver biopsy: iron content elevated
What is the treatment of haemochromatosis?
Venesection, iron chelation therapy (desferroxamine)
What is the definition of Wilson’s disease?
Autosomal-recessive disease of copper accumulation and toxicity caused by a defect in an enzyme involved in the biliary excretion of excess copper
What is the clinical presentation of Wilson’s disease?
Hepatitis, cirrhosis, fulminant liver failure, tremor, dysarthria, involuntary movements, dysphagia, dyskinesia, dementia, depression, Kayser-Fleischer ring, blue lunule, arthritis, hypermobile joints, grey skin
What are the investigations of Wilson’s disease?
o FBC: haemolytic anaemia o Serum copper and caeruloplasmin: low o Urinary copper: elevated o Slit-lamp exam: Kayser-Fleischer ring o Liver biopsy: increased hepatic copper, hepatitis, and cirrhosis
What is the treatment of Wilson’s disease?
Penicillamine (copper chelation), liver transplantation
What is the definition of alpha 1 anti-trypsin deficiency?
Autosomal codominant genetic disorder. Allele mutations cause ineffective activity of alpha-1 antitrypsin (ATT) the enzyme responsible for neutralising neutrophil elastase and preventing inflammatory tissue damage in the lungs
What is the clinical presentation of alpha 1 anti-trypsin deficiency?
Jaundice, bruising, spider naevi, palmar erythema, hepatomegaly, ascites, leukonychia, confusion, asterixis (flapping tremor), cachexia, dyspnoea, cough, wheeze, ankle swelling
What are the investigations of alpha 1 anti-trypsin deficiency?
o Pulmonary function testing: significantly reduced FEV1, FVC, and FEV1/FVC; increased TLC; impaired CO-diffusing capacity
o Serum ATT: low
o Genotyping: characteristic AAT alleles
o Liver biopsy: Periodic Acid Schiff positive, with diastase resistant globules
What is the treatment of alpha 1 anti-trypsin deficiency?
Supportive therapy
What is the definition of ascites?
Effusion and accumulation of serous fluid in the abdominal cavity
What is the aetiology of ascites?
Cirrhosis, malignancy, heart failure, TB, pancreatitis
What is the clinical presentation of ascites?
Abdominal distention, nausea, anorexia, constipation, flank fullness, fluid thrill, shifting dullness
What is the treatment of ascites?
Treat underlying cause, diuretics (spironolactone), salt and fluid restriction
What is the definition of peritonitis?
Inflammation of the peritoneum
What is the aetiology of peritonitis?
Appendicitis, ectopic pregnancy, TB, obstruction, ulcer
What is the clinical presentation of peritonitis?
Abdominal pain, diarrhoea, swelling, shock, tenderness and rebound pain, abdominal rigidity
What are the investigations of peritonitis?
Ascitic tap: elevated WCC
What is the treatment of peritonitis?
Broad spectrum antibiotics, fluid resuscitation, surgery (wash-out)
