Gastrointestinal (3) Flashcards
What is Portal Hypertension?
Increase in the pressure within the portal vein (carries blood from the bowel to the liver)
What is the aetiology of Portal Hypertension?
- Most common cause is liver cirrhosis where the scar (fibrotic) tissue blocks the flow of blood through the liver
- Other causes include thromboembolic events, schistosomiasis and HIV related conditions
What is the epidemiology of Portal Hypertension?
Common complication of cirrhosis, can result in liver failure
What are the presenting symptoms and signs of Portal Hypertension?
- Abdominal swelling: ascites
- Haematemesis
- PR bleeding or melaena
- Signs of encephalopathy
- Caput medusae (dilated superficial abdominal veins)
Splenomegaly
What are the appropriate investigations for Portal Hypertension?
- Clinical diagnosis based on the presence of ascites or of dilated veins (caput medusae) or varices
- Others:
a. FBC: thrombocytopaenia ± anaemia, leukopaenia
b. LFTs: deranged (low albumin, raised others, pronged prothrombin time)
c. Endoscsopy (oeshgeal varices)
d. Imaging: CT scan
What is the management of Portal Hypertension?
- B-Blockers and endoscopic banding of varices if present
- Transjugular intrahepatic portosystemic shunt (TIPS): radiological procedure whereby a vascular tract is created in the liver from hepatic to portal veins allowing decompression of portal hypertension
- Ascites: Diuretics (spironolactone ± furosemide), dietary sodium restriction (2g/day), therapeutic paracentesis
- Liver transplantation is the only curative measure for cirrhosis
What are the complications of Portal Hypertension?
Complications of liver cirrhosis:
1. Jaundice
2. hepatic encephalopathy
3. Ascites, risk of SBP
4. Variceal bleeding
5. Hepatocellular carcinoma
What is Primary biliary cholangits?
- Previously referred to as primary biliary cirrhosis
- A chronic liver disorder typically seen in middle-aged females (female:male ratio of 9:1)
What is the pathophysiology of primary biliary cholangitis?
- Not fully understood although it is thought to be an autoimmune condition
- Interlobular bile ducts become damaged by a chronic inflammatory process causing progressive cholestasis which may eventually progress to cirrhosis
What is the epidemiology of primary biliary cholangitis?
Middle aged females
What are the associated conditions of primary biliary cholangitis?
- Sjogren’s syndrome (seen in up to 80% of patients)
- Rheumatoid arthritis
- Systemic sclerosis
- Thyroid disease
What are the antibodies involved in primary biliary cholangitis?
Anti-mitochondrial antibodies
What is the main presenting symptom of primary biliary cholangitis?
Itching (pruritus)
What are the early features of primary biliary cholangitis?
- May be asymptomatic (e.g. raised ALP on routine LFTs)
- Fatigue
- Pruritus
What are the clinical features of primary biliary cholangitis?
- Pruritus
- Cholestatic jaundice
- Hyperpigmentation, especially over pressure points
- RUQ pain (10%)
- Aanthelasmas, xanthomata
- Clubbing
- Hepatosplenomegaly
- May have symptoms of associated disease e.g. dry eyes and dry mouth
What is a late feature of primary biliary cholangitis?
Acute liver failure
How is primary biliary cholangitis diagnosed?
- Immunology:
a. Anti-mitochondrial antibodies (AMA) M2, 98% of patients and are highly specific
b. Smooth muscle antibodies in 30% of patients
c. Raised serum IgM - Imaging:
a. Required before diagnosis to exclude an extrahepatic biliary obstruction
b. This is typically a RUQ ultrasound or MRCP
What is the first line management of primary biliary cholangitis?
Ursodeoxycholic acid: slows disease progression and improves symptoms
What is the management of primary biliary cholangitis?
- Ursodeoxycholic acid
- For pruritus: cholestyramine
- Fat-soluble vitamin supplementation
- Liver transplantation
e.g. if bilirubin > 100 (PBC is a major indication), recurrence in graft can occur but is not usually a problem
What are the complications of primary biliary cholangitis?
- Cirrhosis → portal hypertension → ascites, variceal haemorrhage
- Osteomalacia and osteoporosis
- Significantly increased risk of hepatocellular carcinoma (20-fold increased risk)
What condition is associated with a 20x increased risk of hepatocellular cancer?
Primary biliary cholangitis
What is primary sclerosing cholangitis?
A biliary disease of unknown aetiology characterised by inflammation and fibrosis of intra and extra-hepatic bile ducts
What condition is closely associated with primary sclerosing cholangitis?
Ulcerative colitis: 4% of patients with UC have PSC, 80% of patients with PSC have UC
What other conditions are associated with primary sclerosing cholangitis?
- Crohn’s (much less common association than UC)
- HIV