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Questions and Answers
Which of the following is the leading cause of alcohol-related liver disease in Ireland?
Which viral hepatitis strains are predominantly associated with chronic liver disease?
What condition results from genetic deficiencies of alpha-1 antitrypsin?
Which autoimmune condition is associated with cholestasis and can cause cirrhosis?
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Which of the following medications is known to cause acute liver failure?
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Which autoimmune liver disease is primarily characterized by a T-lymphocyte-mediated attack on small intralobular bile ducts?
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Which of the following is NOT commonly associated with Primary Biliary Cholangitis?
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What symptom is typically associated with Hepatic encephalopathy?
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Which sign is NOT typically associated with chronic liver disease?
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In autoimmune hepatitis, what demographic is most commonly affected?
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Which of the following symptoms is associated with fluid accumulation due to ascites?
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What is the characteristic pathology in Primary Sclerosing Cholangitis?
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Which of the following symptoms is NOT typically present in chronic liver disease?
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Which of the following conditions can lead to chronic liver disease and cirrhosis?
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What characterizes Non-Alcoholic Fatty Liver Disease (NAFLD)?
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Which hepatitis type is associated with the highest incubation period?
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What risk factor is specifically associated with Hepatitis C?
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Among the following, which hepatitis type cannot cause chronic hepatitis?
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What is a common cause of fatty liver in individuals without excessive alcohol intake?
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Which of these statements about Hepatitis A transmission is true?
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What histological change marks the progression from Non-Alcoholic Fatty Liver to cirrhosis?
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Which hepatitis virus is known to be associated with a vaccine?
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Which population is at increased risk for Hepatitis B infection?
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What is a key characteristic of autoimmune hepatitis?
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What is the primary risk associated with long-term steroid use in autoimmune hepatitis treatment?
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Which statement about prednisolone treatment is incorrect?
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Which demographic is most commonly affected by autoimmune hepatitis?
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Which screening complication is associated with primary sclerosing cholangitis?
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How does autoimmune hepatitis typically present on a biopsy?
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What is the likelihood of treatment failure with Azathioprine in autoimmune hepatitis?
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What findings would most likely indicate cholestatic liver disease?
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What is indicated by a raised ALT/AST ratio greater than 2:1?
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Which of the following tests is NOT typically used to investigate Hepatitis C?
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Which liver function test measures synthetic liver function?
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In managing Non-Alcoholic Fatty Liver Disease, which option is essential?
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What is the priority for Hepatitis C treatment?
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What does a raised ALT/AST ratio greater than 2:1 typically indicate?
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Which of the following liver function tests is primarily a measure of liver synthetic function?
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In the diagnosis of Non-Alcoholic Fatty Liver Disease, which factor must be excluded?
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What is a critical priority in the treatment of Hepatitis C for patients with significant fibrosis or cirrhosis?
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Which investigation method is often used to demonstrate the presence of hepatic steatosis?
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Which of the following organs is NOT commonly affected by hereditary haemochromatosis?
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What is a common neurological symptom associated with Wilson's disease?
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Which genetic factor is primarily linked to hereditary haemochromatosis?
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What is the most significant risk factor for developing liver cirrhosis in Wilson's disease?
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How is hereditary haemochromatosis typically diagnosed?
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Which of the following symptoms is least associated with hereditary haemochromatosis?
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What is a potential outcome of untreated Wilson’s disease?
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What is a characteristic feature of Wilson’s disease pathology?
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What is the duration of treatment for Glecaprevir and Pibrentasvir?
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Which serological marker indicates a high infectivity level in Hepatitis B?
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What is the primary treatment option for Primary Biliary Cholangitis?
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Which of the following is a typical diagnostic feature of Primary Sclerosing Cholangitis?
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What is the target ferritin level during the maintenance phase of hereditary haemochromatosis management?
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What is a common laboratory finding in patients with Primary Biliary Cholangitis?
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What is the role of family screening in hereditary haemochromatosis?
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Which of the following medications is recommended in the treatment of Hepatitis B?
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Which condition could lead to liver cirrhosis due to excessive copper deposition?
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In the differential diagnosis for cirrhosis, which of the following is a known toxin that can cause liver damage?
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Which autoimmune condition is most directly linked to bile duct damage and can lead to cirrhosis?
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What common medication is associated with liver toxicity and is often mentioned in differential diagnoses for cirrhosis?
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Which hereditary condition primarily results from iron overload leading to liver cirrhosis?
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What is a common feature of Non-Alcoholic Fatty Liver Disease (NAFLD)?
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Which type of hepatitis cannot lead to chronic infection?
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Which of the following factors is associated with the development of Non-Alcoholic Fatty Liver Disease?
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What is a risk associated with hepatitis C infection?
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What is a common diagnostic criterion for diagnosing Non-Alcoholic Fatty Liver Disease?
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Which type of hepatitis B transmission is not classified as percutaneous?
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What is a primary feature of Wilson's disease as it relates to liver pathology?
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What is the incubation period range for Hepatitis B?
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In the context of chronic hepatitis C, what is the typical progression rate towards cirrhosis?
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Which of the following statements regarding Hepatitis E is correct?
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Study Notes
Hep E
- Hep E can very rarely have a chronic course in profoundly immunosuppressed individuals
Hereditary Haemochromatosis
- Hereditary Haemochromatosis is a cause of chronic liver disease
Wilsons disease
- Wilsons disease is a cause of chronic liver disease
Autoimmune Liver Disease
- Primary biliary cholangitis, primary sclerosing cholangitis, autoimmune hepatitis are causes of chronic liver disease
Non Alcoholic Fatty Liver Disease (NAFLD)
- High calorie intake, high fructose intake, saturated fat intake and sedentary behaviour are associated with NAFLD
- Western diet and lifestyle is associated with weight gain, obesity, and NAFLD
- NAFLD is alcohol-like liver disease in individuals who do not consume excessive alcohol
- NAFLD can progress through a histologic spectrum of liver damage including NAFLD (steatosis), steatohepatitis (NASH), and cirrhosis
- NAFLD diagnosis is one of exclusion and is made by fatty liver on imaging (ultrasound) in the absence of alcohol excess and features of the metabolic syndrome
Viral Hepatitis
- Viral hepatitis is caused by HAV, HBV, HCV, HEV
- HAV is an RNA virus with an incubation period of 15-50 days
- HBV is a DNA virus with an incubation period of 50-180 days
- HCV is an RNA virus with an incubation period of 14-84 days
- HEV is an RNA virus with an incubation period of 15-60 days
- All four viruses can cause acute hepatitis
- HBV and HCV can cause chronic hepatitis
- HBV and HCV can cause cirrhosis and primary hepatocellular carcinoma
- Vaccines are available for HAV and HBV
Hepatitis A
- Hep A is spread through close personal contact via household or sexual contact and at day-care centres
- Hep A is spread through fecal-oral contamination of food or water
- Hep A is spread through blood-borne transmission in injecting drug users (rare)
Hepatitis C Risk Factors
- Hepatitis C risk factors include:
- clotting factor treatment prior to 1987
- blood transfusion or organ transplant prior to 1992
- long-term hemodialysis
- multiple sexual partners
- injection drug use
- mass injections and traditional practices
- birth from an infected mother
Hepatitis C Disease Course
- 55-85% of individuals with acute hepatitis C develop chronic infection
- 70% of those with chronic infection develop chronic hepatitis
- 1-4% per year of those with chronic hepatitis develop cirrhosis
- 20% of those with chronic hepatitis develop hepatocellular carcinoma (HCC)
- 4-5% per year of those with cirrhosis experience decompensation
Hepatitis B Transmission
- Hepatitis B transmission routes include percutaneous and perimucosal
- Percutaneous transmission occurs through injection drug use, transfusion or transplant, occupational exposure, and parenteral practices
- Perimucosal transmission occurs through perinatal, sexual, and household transmission
- Investigate recent travel/previous residency in high-risk endemic countries such as Southeast Asia and Africa.
Autoimmune Liver Disease: Primary Biliary Cholangitis (PBC)
- PBC features a T-lymphocyte mediated attack on small intralobular bile ducts
- The continuous assault on bile duct epithelial cells leads to their gradual destruction and eventual disappearance
- PBC is associated with female gender (95%), Sjogren's syndrome, autoimmune thyroid disease, rheumatoid arthritis, celiac disease, and inflammatory bowel disease
- Approx 50% of individuals with PBC are asymptomatic at the time of diagnosis
- Symptoms of liver disease may present
Autoimmune Liver Disease: Primary Sclerosing Cholangitis (PSC)
- PSC is a chronic progressive disorder of unknown aetiology
- PSC is characterized by inflammation, fibrosis, and stricturing of medium and large ducts in the intrahepatic and/or extrahepatic biliary tree
- PSC is associated with ulcerative colitis
Autoimmune Liver Disease: Autoimmune Hepatitis (AH)
- AH is a chronic inflammatory disease of the liver
- AH is characterized by circulating autoantibodies and elevated serum globulin levels
- AH is associated with a female to male ratio of 4:1
History for Chronic Liver Disease and Cirrhosis
- Important history includes:
- alcohol use
- medication use
- drug use, especially IV drug use
- recent travel to endemic areas
- skin pigmentation
- obesity
- family history
Symptoms of Chronic Liver Disease and Cirrhosis
- Non-specific symptoms include fatigue, anorexia, and weight loss
- Decreased Glasgow Coma Scale indicates hepatic encephalopathy
- Jaundice is a symptom
- Itch (bile salt deposition in the skin) is a symptom
- Abdominal distension (ascites secondary to portal hypertension) is a symptom
- Weight gain (fluid accumulation - ascites) is a symptom
- Malaena or haematemesis (variceal bleeding) is a symptom
- Infertility (CLD leads to infertility) is a symptom
- Irregular menstrual bleeding is a symptom
Signs of Chronic Liver Disease and Cirrhosis
- General inspection:
- Decreased GCS indicates hepatic encephalopathy
- Confabulation indicates Korsakoff's
- Jaundice indicates increased bilirubin
- Grey pigmentation/tanned indicates haemochromatosis
- Arms:
- Bruising
- Scratch marks
- Tattoos
- Proximal muscle wasting
- Needle track marks
- Head and Neck:
- Scleral icterus
- Fetor hepaticus
- Conjunctival pallor
- Angular stomatitis
- Raised Jugular Venous Pressure (JVP)
- Spider naevi
- Gynaecomastia and reduced body hair
- Hands:
- Clubbing
- Palmer erythema indicates alcohol excess
- Dupuytrens contracture
- Asterixis indicates grade 2 hepatic encephalopathy
- Abdomen:
- Hepatomegaly
- Splenomegaly
- Ascites
- Caput medusa
- Other:
- Lower limb pitting oedema
- DRE -> Haemorrhoids
Differential Diagnosis for Cirrhosis
- Alcohol related liver disease
- Non-alcoholic fatty liver disease
- Viral Hepatitis - Hepatitis B, C
- Autoimmune Hepatitis
- Primary Sclerosing Cholangitis
- Primary Biliary Cholangitis
- Medication related:
- Methotrexate, Amiodarone
- Hereditary:
- Haemochromatosis, Wilson's Disease, Alpha 1 Antitrypsin Deficiency
- Toxins:
- Alcohol: leading cause in Ireland
- Paracetamol causes ACUTE liver failure
- Other drugs: Methotrexate, Amiodarone
- Deposition:
- Fat - NAFLD
- Iron - Haemochromatosis
- Wilsons - Copper
- Viral:
- Hep A, B, C, E. B and C are predominantly viral
- Autoimmune:
- AI hepatitis, PSC, PBC
- Genetic:
- Alpha 1 anti-trypsin, haemochromatosis, wilsons
Liver Blood Tests
- Liver enzymes (damage to different parts of the liver):
- ALT
- AST
- ALP
- gGT
- Liver function (the liver is doing what it should be doing):
- Bilirubin
- Albumin
- Prothrombin time
- Platelets
LFT Patterns
- Hepatocellular/parenchymal damage: raised transaminases: ALT/AST
- Cholestatic/obstructive: raised ALK PHOS/GGT
- Bilirubin can be raised in either of these patterns
- Alcoholic hepatitis: AST>ALT ratio is >2:1
- Measure of function is critical:
- Albumin, INR, APTT
Non Alcoholic Fatty Liver Disease (NAFLD) Management
- Weight loss
- Abstain from alcohol
- Immunization (Hep A, Hep B, Pneumococcal)
- Exclusion of significant alcohol consumption
- Exclusion of other causes of hepatic steatosis
- Absence of coexisting chronic liver disease
Hepatitis C Investigations
- Hepatitis C antibody test
- Hepatitis C antigen test
- Quantitative HCV RNA tests
- Genotyping
- Fibroscan
- Liver biopsy
Hepatitis C Treatment
- All treatment-naïve and treatment-experienced patients with recently acquired or chronic HCV infection must be offered treatment without delay.
- Hep C Treatment is prioritized in patients with:
- significant fibrosis or cirrhosis
- HIV/HBV coinfection
- pre/post liver transplantation
- autoantibodies can be supportive
Primary Sclerosing Cholangitis (PSC) Management
- Trial UDCA
- No proven medical therapy
- Screening for complications:
- Cholangiocarcinoma (Lifetime risk 7-15%)
Autoimmune Hepatitis
- Presents in middle-aged (or teenage) woman, a non-drinker with no viral hepatitis
- Symptoms include: fatigue, arthralgias/myalgias, oligomenorrhea, jaundice
- Liver function tests show increased ALT, AST, IgG globulins
- Positive ANA and SMA antibodies
- Interface hepatitis with lymphoplasmacytic infiltrate on biopsy
- Responds to corticosteroids
Autoimmune Hepatitis Treatment
- Prednisone is the initial treatment
- Azathioprine is used for long-term maintenance therapy
- Monitor bone protection, gastric protection, hypotension, diabetes risk, and risk of stopping steroids suddenly.
Case Presentation: Section 4
- This patient presents with symptoms of chronic liver disease and is diagnosed with autoimmune hepatitis
- Patients with AIH should be counselled about the risks and benefits of long-term steroid use.
- Long-term prednisolone use can cause complications such as osteoporosis, diabetes, hypertension, and peptic ulcer disease.
Hepatits E
- Profoundly immunosuppressed individuals can have a chronic course of Hepatitis E
Hereditary Haemochromatosis (HH)
- An inherited disorder where increased intestinal iron absorption leads to iron overload
- Most commonly caused by the C282Y variant in the HFE gene
- Not all individuals with the C282Y variant will develop iron overload
- Diagnosis requires genetic testing and monitoring iron levels in tissues
- Can affect the liver, heart, pancreas, pituitary and other organs
- Symptoms include bronze-colored skin, diabetes, joint pain, fatigue, impotence in males, cirrhosis, and abnormal ECG results
Wilson's Disease
- An inherited disorder affecting copper metabolism
- Caused by mutations in the ATP7B gene affecting a copper transporter
- Leads to copper accumulation in the liver, brain and cornea
- Can cause liver cirrhosis and neurological symptoms like difficulty speaking, Parkinsonism, ataxia, dystonia and tremor
Non-Alcoholic Fatty Liver Disease (NAFLD)
- Refers to liver disease in individuals who do not consume excessive alcohol
- Can progress from fatty liver (steatosis) to steatohepatitis and cirrhosis
- Often associated with a Western diet and lifestyle factors, including high calorie intake, high fructose intake, high saturated fat intake, and sedentary behavior
- Diagnosis involves demonstrating fatty liver on imaging and ruling out other causes like excessive alcohol consumption
Viral Hepatitis
- Hepatits A, B, C and E are categorized by the type of virus (RNA or DNA) and their incubation periods
- All can cause acute hepatitis
- Hepatitis B and C can lead to chronic hepatitis, cirrhosis and hepatocellular carcinoma
- Vaccines are available for Hepatitis A and B
Hepatitis A
- Transmission occurs through close personal contact, fecally contaminated food and water, and rarely through blood
- Can be transmitted through household or sexual contact and in day-care centers
- Food handlers and people handling raw shellfish are at risk of spreading the virus
- Injecting drug users are also at risk
Hepatitis C
- Primarily transmitted through blood
- Risk factors include blood transfusions or organ transplants before 1992, long-term hemodialysis, multiple sexual partners, injection drug use, mass injections, and birth from an infected mother
Hepatitis C Disease Course
- About 70% of people infected with Hepatitis C develop chronic infection
- Chronic hepatitis can progress to cirrhosis in 20% of cases
- Cirrhosis carries a high risk of developing hepatocellular carcinoma (HCC)
- Decompensation of cirrhosis occurs over a 4-5 year period
Hepatitis B Transmission
- Transmission can be percutaneous (through the skin), including through injection drug use, transfusions or transplants, occupational exposure, and parenteral practices
- Perimucosal transmission occurs through perinatal (mother to child), sexual, and household routes.
- Recent travel to endemic countries like Southeast Asia or Africa may increase the risk of infection
Hepatitis B Disease Course
- Most infections result in recovery
- Acute hepatitis can progress to chronic infection or fulminant (rapidly progressing) hepatitis, leading to death in some cases
Chronic Hepatitis B Infection
- Can progress to an inactive carrier state, chronic hepatitis, cirrhosis and HCC
Hereditary Haemochromatosis - Management
- Regular phlebotomy (blood removal)
- Ferritin target levels of 50-100 ug/L
- Phlebotomy performed every few months for life
- Screening of family members is essential
Primary Biliary Cholangitis
- Characterized by high levels of alkaline phosphatase and gamma-glutamyl transferase
- AST and ALT levels are usually low
- Bilirubin levels rise later in the disease course
- Cholesterol levels are elevated in 85% of cases
- Treatment involves ursodeoxycholic acid (UDCA), benzafibrate, obeticholic acid, and budesonide
Primary Sclerosing Cholangitis - Diagnosis
- Elevated alkaline phosphatase and gamma-glutamyl transferase
- History of inflammatory bowel disease
- Imaging: MRCP/ERCP is essential for diagnosis
- Characteristic findings include multifocal, short, annular strictures, resulting in a "beaded" appearance
Liver Blood Tests
- Liver enzymes (ALT, AST, ALP, GGT) indicate liver damage.
- Liver function tests (bilirubin, albumin, prothrombin time, platelets) assess the liver's ability to perform its functions
Liver Function Test Patterns
- Hepatocellular or parenchymal damage: Elevated transaminases (ALT, AST)
- Cholestatic or obstructive: Elevated alkaline phosphatase and gamma-glutamyl transferase
- Alcoholic hepatitis: AST > ALT ratio is >2:1
- Function tests (albumin, INR, APTT) are important for assessing liver function
Non-Alcoholic Fatty Liver Disease - Management
- Weight loss
- Abstinence from alcohol
- Immunization against Hepatitis A and B, and pneumococcal vaccine
- Exclusion of other causes of steatosis
- Management of co-existing chronic liver disease
Hepatitis C - Investigations
- Hepatitis C antibody test
- Hepatitis C antigen test
- Quantitative HCV RNA tests
- Genotyping
- Fibroscan
- Liver biopsy
Hepatitis C Treatment
- All treatment-naïve and treatment-experienced patients with chronic HCV infection should be offered treatment
- Treatment is prioritized in patients with significant fibrosis or cirrhosis, HIV/HBV coinfection, pre/post liver transplantation, and those with a high risk of transmitting the virus
- Antiviral therapies include glecaprevir/pibrentasvir and sofosbuvir/velpatasvir
Hepatitis B Serology
- HBsAg: Acute/Chronic infection
- Anti-HBc IgM: Acute infection
- HBeAg high infectivity
- Anti-HBe: Low infectivity
- Anti-HBs: Immunity
- Anti-HBc IgG and HBsAg: Chronic infection
- Anti-HBc IgG and anti-HBs: Resolved infection
Hepatitis B Management
- Antiviral treatment
- Prevention through vaccination
Hepatitis B Treatment
- Antiviral therapies: Pegylated Interferon, Lamivudine, Adefovir, Entecevir, Tenofovir
- Prevention: Hepatitis B vaccination is recommended for high-risk groups
Differential Diagnosis of Cirrhosis
- Alcohol related liver disease
- Non-alcoholic fatty liver disease
- Viral hepatitis (A, B, C, E)
- Autoimmune hepatitis
- Primary sclerosing cholangitis
- Primary biliary cholangitis
- Medication related (methotrexate, amiodarone)
- Hereditary (haemochromatosis, Wilson’s disease, Alpha1 antitrypsin deficiency)
- Toxins
- Paracetamol (overdose)
- Other drugs (methotrexate, amiodarone)
- Deposition
- Fat (NAFLD)
- Iron (Haemochromatosis)
- Copper (Wilson's Disease)
- Viral (Hepatitis A, B, C, E)
- Autoimmune
- AI hepatitis, PSC, PBC
- Genetic
- Alpha1 anti-trypsin, haemochromatosis, Wilson's disease.
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Description
This quiz covers various types of chronic liver diseases including Hep E, hereditary haemochromatosis, Wilson's disease, autoimmune liver disease, and non-alcoholic fatty liver disease (NAFLD). Test your understanding of the causes, progression, and implications of these conditions in this informative quiz.
