Chronic Liver Disease Flashcards
What is chronic liver failure?
- liver failure may be recognised by development of coagulopathy (INR >1.5) + encephalopathy
- occur suddenly in prev healthy liver → acute liver failure
- more often it occurs on background of cirrhosis → chronic liver failure
What are symptoms of chronic liver disease?
patients may be asymptomatic or experience non-specific symptoms, particularly weakness, anorexia and fatigue, specific symptoms include:
- right hypochondrial pain due to liver distension
- abdo distension due to ascites
- ankle swelling due to fluid retention
- haematemesis + melaena from GI haemorrhage
- pruritus due to cholestasis - often an early symptom of PBC
- gynaecomastia, loss of libido + amenorrhoea due to endocrine dysfunction
- portosystemic encephalopathy
What is chronic liver disease?
- a disease process of the liver that involves progressive destruction + regeneration of liver parenchyma leading to fibrosis and cirrhosis
there are a lot
What are the potential signs of chronic liver disease?
- General: jaundice, pyrexia, hair loss, anaemia
- Compensated: palmar erythema, clubbing, dupuytren’s, xanthomas, xanthelasmas, parotid enlargement, spider naevi, gynaecomastia, hepatomegaly or smaller liver (late), splenomegaly, scratch marks, testicular atrophy, purpura + pigmented ulcers
- Decompensated: leuconychia, confusion + encephalopathy, drowsy/coma, hepatic flap, loss of proximal bulk, ascites, caput medusae, oedema
What are the infective causes of chronic liver disease?
- Hep B
- Hep C
- Hep D
What are the non-infective causes of chronic liver disease?
- autoimmune chronic hep
- alcoholic liver disease
- drugs: methyldopa, nitrofurantoin
- wilson’s disease
- a1-antitrypsin deficiency
- haemochromatosis
- primary biliary cirrhosis
- primary sclerosing cholangitis
Liver cirrhosis implies irreversible liver damage. Histologically, there is loss of normal hepatic architecture with bridging fibrosis and nodular regeneration.
What are the common causes of liver cirrhosis?
- Alcohol
- Non-alcoholic fatty liver disease
- Viral hep (B + C)
What are features of hepatic failure, as a result of cirrhosis?
- Coagulopathy
- Encephalopathy
- Hypoalbuminaemia → oedema
- Sepsis → pneumonia, septicaemia
- Spontaneous bacterial peritonitis
- Hypoglycaemia
Liver cirrhosis can also lead to portal hypertension where portal vein pressure is >15mmHg.
What are features of portal hypertension?
- Ascites
- Splenomegaly
- Portosystemic shunt incl oesophageal varices
- Caput medusae
What investigations can be done for liver cirrhosis?
- New recommended techniques include transient elastography (fibroscan) and acoustic radiation force impulse imaging
- NAFLD → enhanced liver fibrosis score
- Upper endoscopy for varices
- Liver USS every 6 months (+/- alpha-feto protein) to check for hepatocellular cancer
What is the general management of liver cirrhosis?
- good nutrition
- alcohol abstinence
- avoid NSAIDs, sedatives + opiates
- colestyramine helps pruritus (4g/12h PO, 1hr after other drugs)
- consider USS + alpha-fetoprotein every 6 months to screen for HCC
What is the treatment for ascites?
- fluid restriction (<1.5L/d) + low salt diet
-
spironolactone 100mg/24h PO
- dose increased as tolerated as it counters deranged RAAS axis
- chart daily weight + aim for weight loss of <1/2kg per day
- if response poor → add furosemide <120mg/24hr PO
How is liver transplant prioritisation calculated in the UK and what is the immediate management of it?
- Prioritisation based upon UKELD score, calc from:
- serum Na
- creatinine
- bilirubin
- INR
- Post op = 12-48hr on ITU w/ enteral feeding starting ASAP + close monitoring of LFTs
- immunosuppression examples → tacrolimus +/- mycophenolate mofetil + prednisolone
- acute rejection = pt feels unwell w/ pyrexia + tender hepatomegaly
What is hepatic encephalopathy?
- XS absorption of ammonia + glutamine from bacterial breakdown of proteins in gut → builds up and passes to brain
- XS glutamine causes osmotic imbalance → cerebral oedema
- associated w/ acute liver failure AND chronic disease
- mild cognitive impairment may precede
What are features of hepatic encephalopathy?
- confusion; altered GCS
- asterix: liver flap
- constructional apraxia (can’t draw 5 pointed star)
- triphasic slow waves on EEG
- raised ammonia level
How is hepatic encephalopathy graded?
- Irritability
- Confusion, inappropriate behaviour
- Incoherent, restless
- Coma
How is hepatic encephalopathy managed?
- treat underlying precipitating cause
- first-line → lactulose
- addition of rifaximin for secondary prophylaxis
- lactulose promotes excretion of ammonia + inc metabolism of ammonia by gut bacteria
- rifaximin modulates gut flora → reduced ammonia production
- other options → embolisation of portosystemic shunts + liver transplantation
What is primary biliary cholangitis (PBC)?
- chronic liver disorder
- middle-aged females (9:1)
- interlobular bile ducts damaged by chronic autoimmune granulomatous inflammation causing cholestasis → fibrosis, cirrhosis + portal hypertension
- often asymptomatic + diagnosed after incidental high ALP
- lethargy, sleepiness, pruritus may precede jaundice
Signs → jaundice / skin pigmentation / xanthelasma / xanthomata / hepatosplenomegaly
What do investigations for PBC show?
- ANTIMITOCHRONDRIAL ANTIBODIES (AMA)
- ALP + GGT high
- USS to exclude extrahepatic cholestasis
What is the management for PBC?
- pruritus → cholestyramine 4-8g/24h PO
- fat-soluble vitamin supplementation (ADEK)
- high-dose ursodeoxycholic acid → may improve survival + delay transplantation
- monitoring regular LFT + USS
- liver transplant → for end-stage disease or intractable pruritus
What is primary sclerosing cholangitis (PSC)?
- progressive cholestasis w/ bile duct inflammation and strictures
- associated w/ ulcerative colitis (80% of pts w/ PSC have UC)
- bile duct, gallbladder, liver + colon cancers are more common so do yearly colonoscopy + USS
- liver transplant mainstay for end-stage disease; recurrence occurs in up to 30%
- prognosis worse for those w/ IBD as 5-10% develop colon cancer post-transplant
What are the clinical features of PSC?
- cholestasis → jaundice + pruritus
- RUQ pain
- fatigue
Investigations → ERCP (diagnostic, beaded appearance), ANCA positive, onion skin on biopsy
What is non-alcoholic fatty liver disease (NAFLD)?
- most common cause of liver disease in developed world
- caused by obesity
- spectrum of disease:
- steatosis → fat in liver
- steatohepatitis → fat w/ inflammation (aka NASH)
- progressive disease may cause fibrosis + cirrhosis
What are clinical features of NAFLD?
- usually asymptomatic
- hepatomegaly
- ALT typically greater than AST
- increased echogenecity on USS
What is the investigation and treatment for NAFLD?
- incidental findings on liver USS → enhanced liver fibrosis (ELF) blood test to check for advanced fibrosis
- mainstay of Rx is lifestyle changes (weight loss) + monitoring
What is Wilson’s disease?
- rare inherited autosomal recessive disorder characterised by excessive copper deposition in tissues
- it is treatable, so screen all for cirrhosis
- onset symptoms between 10-25yrs
- children usually present with liver disease whereas first sign of disease in young adults is often neurological disease
What are the clinical features of Wilson’s disease?
- Liver → hepatitis / cirrhosis
- Neuro → basal ganglia degeneration / speech / behaviour + psych problems
- Kayser-Fleischer rings
- Renal tubular acidosis
What is the diagnosis and treatment of Wilson’s disease?
- Diagnosis → reduced serum caeruloplasmin / reduced serum copper
- Management → penicillamine (chelates copper) 1st line
