Cirrhosis

Cirrhosis Definitions Characteristic distortion of the normal liver architecture due to excessive fibrosis Compensated cirrhosis: stable phase Clinically significant portal HTN may exit Decompensated cirrhosis: Presence of complications such as ascites, hepatic encephalopathy, variceal haemorrhage or nonobstructive jaundice Causes Common Others Primary biliary cholangitis Secondary biliary cholangitis Autoimmune hepatitis Alcohol Hereditary haemochromatosis Most common Drugs e.g. methotrexate Hepatitis B, C and D Cystic fibrosis NAFLD Wilison’s disease Veno occlusive disease Alpha 1 antitrypsin deficiency Idiopathic Clinical Features Fatigue Appetite loss Weight loss Icterus Jaundice Edema Ascites Easy bruising Hepatic encephalopathy: Confusion, drowsiness, personality changes, slurred speech Hormonal: hair loss, loss of sexual function, irregular periods, Dark urine Pale stools Palmar erythema Investigations LFTs First line Sr albumin and PT -> best indicators of liver function Elevation of ALT and AST elevation Slightly ALP elevation Biochemistry Cr more than 130 -> poor prognostic marker Ultrasound Initial imaging modality Size changes Fatty changes and fibrosis -> diffusely increased echogenicity Marginal nodularity Distortion of arterial vascular architecture CT scan Hepatosplenomegaly Dilated collaterals Arterial phase contrast enhanced scans To detect HCC MRI scan Useful for tumor detection For both benign and malignant Transient elastography Used to avoid biopsy Difficult in obese patients Endoscopy For detection and management of varices and poral hypertensive gastroscopy Varices can be managed with banding or injection sclerotherapy Liver biopsy Gold standard for confirming type and severity Findings: Nodules Fibrosis Architectural distortion Vascular changes Classification of histological types Type Description Uniformly small nodules -> <3 mm Micronodular Thick and regular fibrous bands Large Variable sized Macronodular 3mm to 5 mm Broader, irregular fibrous bands Image Mixed Features of both Management Compensated cirrhosis Goal ->prevent decompensation Non-selective beta blockers -> carvedilol (better as it has an additional vasodilator activity) or propranolol Useful when there is portal hypertension -> otherwise not useful Ascites Sodium restriction Adequate protein and energy intake Ascites -> satiety -> poor nutrition Diuretics Spironolactone at 50 to 100 mg orally daily -> if inadequate increase by 100 mg daily every 4 to 7 days for a maximum of 400mg If can’t tolerate then -> amilrodone Id nonresponsive to either of above -> add furosemide Large volume paracentesis Draining 5 to 10 L over 1 to 6 hours Albumin is recommended when undergoing It Albumin 20% 8g (40 mL) IV for each litre of ascitic fluid drained Trans jugular intrahepatic portosystemic shunts for refractory cases Liver transplant for refractory cases Coagulopathies Correction with blood products before low bleeding risk procedures e.g. paracentesis is not recommended Poral vein thrombosis can happen in up to 25% of patients Also give vitamin K to help correct clotting factors When decompensated: Unfractionated and low molecular weight heparin Warfarin should not be used as when there is a liver dysfunction it is hard to interpret INR values Direct-acting oral anticoagulants should not be used in Child–Pugh class C cirrhosis. In Child–Pugh class B cirrhosis, apixaban and dabigatran can be used with caution under specialist supervision. Rivaroxaban is not recommended in Child–Pugh class B cirrhosis because safety data are lacking. Gastro-oesophageal varices Endoscopic surveillance is not recommended if: Are non-selective beta blocker H ave a platelet count of more than 150 × 10 9 /L and a liver stiffness of less than 20 kPa Endoscopic surveillance every 2 to 3 years when cirrhosis is compensated and no varices are present Primary prevention: Non-selective beta blockers carvedilol or propranolol orally daily Variceal band ligation Recommended for: Imminent risk of rupture Contraindication or intolerance to beta blockers Non-adherent to beta blockers Heart rate reduction to achieved with beta blockers Should be repeated every 2 to 6 weeks until varices are obliterated Management of bleeding varices Manage at high dependency unit Resuscitation to restore and maintain hemodynamic stability Blood transfusion -> avoid over transfusion -> increases portal pressure Aim for Hb of 70 to 80 Emergency endoscopy within 12 hours Confirm source/cause of bleeding Oesophageal varices -> endoscopic ligation Gastric varices -> cyanoacrylate Reduce portal HTN when there is upper GI bleeding via terlipressin or octreotide Intravenous injection followed by continuous transfusion Are to be continued after endoscopy Secondary prevention Risk of bleeding within next 12 months after initial bleeding episode is 60% Endoscopic band ligation and beta blocker therapy Most effective therapy Pre-emptive placement of a trans jugular intrahepatic portosystemic shunt (TIPS) within 72 hours of variceal haemorrhage can reduce mortality and may be considered in selected patients who are at high risk of rebleeding after endoscopic therapy. Hepatic Encephalopathy Changes in cognition, personality, emotions, sleep disturbances and distortions Ask patient to draw a 5 pointed star May be overt or covert Covert: More common Subtle dysfunction that is hard to detect on routine examination E.g. impaired concentration, decision making, sleep disturbances Diagnosis can be made using specialised psychometric tests Overt: Reproducible physical signs consistent with more global and obvious neurological impairment E.g. flapping tremor, disorientation, drowsiness and coma Assess for fitness to drive Severe and acute Aims to reduce absorption of toxic amines by decreasing colonic bacterial levels Lactulose at 30 ml orally -> hourly to 2 hourly to induce rapid laxative effect then reduce to 3 to 4 times a day -> aim for two to three semisoft stools a day lactulose increases gas production when used at high doses -> monitor for bowel distension if unconscious and cannot swallow -> can use an NGT recurrent and chronic lactulose at 30 ml 3 to 4 times a day if cannot tolerate -> use macrogol 3350 orally e.g. Movicol or Osmolax if persistent despite wither -> use rifaximin 550 mg orally twice daily protein restriction is not recommended Hepatocellular carcinoma Common complication if cirrhosis regardless of aetiology Biopsy is not required for diagnosis MDT management Surgery may be offered if cirrhosis is compensated and do not have portal hypertension Non-surgical therapies -> for patients who are not suitable for surgery Supportive care is only appropriate when patients have decompensated cirrhosis or advanced HCC Hepatorenal syndrome Needs exclusion of other causes of renal injury Prevention: Monitor diuretic use Give IV albumin if undergoing large volume paracentesis Prophylactic antibiotics Avoid nephrotoxic drugs Management if it happens: Aims to: Correct hypovolemia Treat infection of present Stop contributory drugs -> e.g. nephrotoxins, vasodilators, beta blockers, diuretics Give albumin IV If kidney function does not improve Terlipressin + albumin Liver transplant If not referred until late and severe liver failure -> may not be suitable Consider when: Refractory ascites Episode of spontaneous bacterial peritonitis Recurrent or chronic hepatic encephalopathy Small HCC Hepatopulmonary syndrome Mode of end stage liver disease score of more than 13. Nutrition, exercise and bone health Malnutrition is common Protein requirements double 1.2 to1.5 g/kg of body weight Nutritional screening is recommended for all patients Obesity is hard to manage Reduced salt diet – limit to 2.5g or less If unable to meet nutritional requirements Oral nutritional supplements are recommended Osteoporosis and bone loss happen in 30% of patients DXA should be performed every 2 years Prognosis Can be assessed using the Child-Pugh Score using: Total bilirubin Serum albumin INR Degree of ascites Compensated: good prognosis if cause is treated Decomposition: high short-term mortality Degree of encephalopathy Complications Higher risk of perioperative surgical and anaesthetic complications Higher mortality rate Portal vein hypertension Ascites Most common Varices Splenomegaly Jaundice Coagulopathy Hepatic encephalopathy Malnutrition and muscle wasting -> sarcopenia Hepatorenal syndrome Osteoporosis Hepatopulmonary syndrome Increase cancer risk Esp HCC Cirrhotic cardiopulmonary