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Questions and Answers
What is a feature of hepatopulmonary syndrome?
Which of the following indicates a diagnosis of intrahepatic cholestasis of pregnancy (IHCP)?
What best defines portopulmonary hypertension?
Which symptom is commonly associated with intrahepatic cholestasis of pregnancy?
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What is a common risk factor for developing intrahepatic cholestasis of pregnancy?
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What is the recommended management for intrahepatic cholestasis of pregnancy?
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Which laboratory abnormality is expected in intrahepatic cholestasis of pregnancy?
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What common clinical feature might you observe in a patient with hepatopulmonary syndrome?
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Which of the following features is indicative of chronic portal vein thrombosis?
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What is the most common underlying condition leading to portal vein thrombosis?
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What is the primary investigation method used to assess portal vein thrombosis?
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Which treatment is indicated for acute portal vein thrombosis if performed within the first 30 days?
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Which of the following is NOT a clinical feature of chronic portal vein thrombosis?
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What potential complication may arise from thrombolysis in treating portal vein thrombosis?
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Which condition can lead to isolated splenic vein thrombosis?
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Which treatment method is effective for managing gastric varices caused by splenic vein thrombosis?
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What is a characteristic of ascitic fluid in Budd-Chiari syndrome?
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What imaging method is usually the first step in diagnosing hepatic vein occlusion?
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Which treatment approach is recommended for patients with Budd-Chiari syndrome?
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Which characteristic distinguishes sinusoidal obstruction syndrome (SOS) from Budd-Chiari syndrome?
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In the management of portal vein thrombosis, which technique is often used to relieve obstruction?
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What is the expected white blood cell count in ascitic fluid for Budd-Chiari syndrome?
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Which of the following is usually unnecessary for assessing Budd-Chiari syndrome?
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What is one of the main benefits of performing TIPS in patients with Budd-Chiari syndrome?
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Study Notes
Portal Vein Thrombosis (PVT)
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Causes of PVT:
- Up to 70% of patients with PVT have an underlying thrombotic disorder.
- Infections within the abdomen, such as acute appendicitis, acute cholecystitis or cholangitis, and pancreatitis, may lead to septic pylephlebitis and PVT.
- Chronic pancreatitis and direct trauma to the abdomen can cause isolated splenic vein thrombosis.
- In patients with cirrhosis, PVT likely results from a combination of factors: diminished portal vein blood flow, reduced protein C and S levels, and hepatocellular carcinoma.
PVT - Clinical Features
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Acute PVT:
- Abdominal pain and nausea.
- Intestinal ischemia, potentially leading to intestinal infarction, which can be fatal.
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Chronic PVT:
- Gastroesophageal varices.
- Splenomegaly.
- Thrombocytopenia.
- Variceal bleeding, usually well-tolerated without cirrhosis.
- Ascites, rare without cirrhosis.
PVT - Investigations
- Doppler ultrasonography: Useful for diagnosis.
- CT and MRI: Can identify thrombus if Doppler results are equivocal.
- Liver biochemical tests: Normal, except in patients with underlying liver disease.
- Evaluation for underlying conditions: Thorough evaluation for thrombotic disorders and hepatocellular carcinoma is important.
PVT - Treatment
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Acute PVT:
- Anticoagulation: Heparin or LMWH is indicated, potentially promoting recanalization if initiated within 30 days. It also reduces the risk of complications like bowel infarction. Long-term anticoagulation is necessary for underlying thrombotic disorders.
- Thrombolysis: Associated with a high risk of bleeding.
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Chronic PVT:
- Beta-blockers: Long-term use can reduce variceal bleeding risk.
- Band ligation of varices: Safe and effective treatment.
- Surgical portosystemic shunts: For patients where less invasive methods fail.
- Splenectomy: Effective for gastric varices caused by isolated splenic vein thrombosis.
Budd-Chiari Syndrome (BCS) - Diagnosis
- High suspicion is necessary for diagnosis due to nonspecific clinical and laboratory findings.
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Diagnostic approach:
- Color Doppler ultrasonography is the initial investigation.
- Three-phase CT or MRI is recommended if ultrasonography is inconclusive.
- Hepatic venography with inferior vena cavography confirms the diagnosis if imaging remains equivocal.
- Liver biopsy: Helpful for assessing fibrosis extent but usually unnecessary.
BCS - Treatment
- Diuretics: Useful for ascites relief but don't alter long-term outcomes.
- Anticoagulation: Heparin followed by warfarin is recommended to prevent repeat thromboses.
- Thrombolysis: Consider streptokinase followed by heparin and oral anticoagulation for recent thromboses.
- Angioplasty: Provides temporary relief for short-segment obstructions.
- Metal stent placement: Following angioplasty of a short-segment stenosis to improve long-term patency.
- TIPS (Transjugular Intrahepatic Portosystemic Shunt): Feasible for over 90% of patients despite hepatic vein occlusion.
- Liver transplantation: Considered for patients with failed minimally invasive procedures, liver failure, or partial portal vein thrombosis.
Sinusoidal Obstruction Syndrome (SOS)
- Rare condition with widespread central hepatic vein occlusion.
- Most common presentation:
- Acute form after bone marrow transplantation (BMT) or hematopoietic stem cell transplantation.
Hepatopulmonary Syndrome
- Cirrhotic patients with portal hypertension develop resistant hypoxemia due to intrapulmonary shunting through arteriovenous communications.
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Clinical features:
- Finger clubbing.
- Spider naevi.
- Cyanosis.
- Fall in SaO2 on standing.
- Resolves after liver transplantation.
Portopulmonary Hypertension
- Pulmonary hypertension in patients with portal hypertension.
- Caused by pulmonary arterial vasoconstriction and obliteration.
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Clinical presentation:
- Breathlessness.
- Fatigue.
Intrahepatic Cholestasis of Pregnancy (IHCP)
- Reversible cholestasis during pregnancy characterized by intense pruritus, elevated serum ALT and fasting bile acid levels, and spontaneous symptom relief post-delivery.
- Most common liver disease in pregnancy with prevalence ranging from 0.3% to 5.6%.
- More common in late second or third trimester but can occur in any.
- Risk factors: Advanced maternal age, history of cholestasis with oral contraceptives, and personal/family history of IHCP.
- Etiology: Multifactorial, including genetic, hormonal, and environmental factors.
IHCP - Clinical and Laboratory features
- Jaundice in 25% of patients after pruritus onset.
- Elevated serum aminotransferase levels (up to fourfold), serum bile acid levels (30-100x), and sometimes serum cholesterol/triglyceride levels.
- Fat malabsorption leading to fat-soluble vitamin deficiencies requiring supplementation.
IHCP - Diagnosis
- Clinical presentation: High bile acid levels.
- Ultrasonography: To exclude cholelithiasis.
IHCP - Treatment
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Symptom management:
- Cool sleeping environment.
- Topical alcohol and camphor menthol lotion.
- Cholestyramine.
- Ursodeoxycholic acid (UDCA) 10 to 15 mg/kg body weight.
- Early delivery: At 37 weeks is encouraged as intrauterine death is more common in the last month.
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Description
This quiz covers the causes, clinical features, and investigations related to Portal Vein Thrombosis (PVT). It explores both acute and chronic forms of PVT, their symptoms, and the underlying conditions that contribute to this serious medical issue. Understand the significance of this condition for better clinical outcomes.
