Chronic Liver Disease Flashcards
Define Cirrhosis (in pathological terms)
An irreversible consequence of chronic hepatic injury w/ 3 key characteristics
- Destruction of liver cells
- Chronic inflammation –> fibrosis
- Hepatocyte regeneration –> nodules
What is the underlying pathology of fibrosis?
Due to growth factors released from inflammatory cells/Kupffer cells/hepatocytes
Inflammatory cells due to disease/response to liver cell necrosis
What is the underlying pathology of nodule formation?
Due to hepatocyte regeneration
Stellate cell activation –> myofibroblasts formed, secrete collagen –> nodules form
Nodules lack vascular/bile drainage connections
What are the common causes of cirrhosis?
Alcoholic liver disease
Non-alcoholic fatty liver disease
Chronic viral hepatitis- Hep B or C
What are the less common causes of cirrhosis?
Auto Immune hepatitis Primary biliary cirrhosis Primary sclerosing cholangitis Budd-Chiari syndrome Cystic fibrosis Drugs- methotrexate or phenytoin
What are the very rare causes of cirrhosis?
Haemochromatosis
Wilson’s
a1-antitrypsin deficiency
What is primary biliary cirrhosis?
Auto Immune destruction of intrahepatic bile cannaliculi
What are the risk factors for primary biliary cirrhosis?
40-60yr old females (90%)
IBD
How does primary biliary cirrhosis present?
Jaundice
Pruritis
Skin xanthomas
How is primary biliary cirrhosis diagnosed?
Raised anti-mitochondrial antibodies (AMA)
What are the management options for primary biliary cirrhosis?
Replacement of fat soluble vitamins
-progressive condition, 6yr average survival
What is primary sclerosing cholangitis?
Autoimmune inflammation & fibrosis around the bile ducts in the liver
-2o cirrhosis due to chronic biliary obstruction
What conditions are associated w/ primary sclerosing cholangitis?
IBD (70%)
Cholangiocarcinoma (develops in 10-15%)
What are the management options for primary sclerosing cholangitis?
No effective treatment except transplantation
What are the classic sx of chronic liver disease?
Fatigue Wt loss/anorexia Jaundice Leg swelling Bleeding/bruising Itching
What are the extrahepatic signs of chronic liver disease?
Nails - leuconychia, clubbing
Hands - palmar erythema, duyputren’s contracture, liver flap
Skin - pigmentation, spider naevi, striae
Feminization - gynaecomastia, testicular atrophy, loss of body hair
Portal HTN - caput medusae, hepatosplenomegaly, ascites
Hepatocellular failure - bruising, prolonged clotting
What are the signs of decompensated liver disease?
Encephalopathy
Ascites
Jaundice
What factors may precipitate chronic liver disease decompensating?
Alcohol binge
Variceal bleed
Hepatotoxic drugs
Portal/hepatic vein thrombosis
What is the Child-Pugh score?
Gives 1/2 yr mortality scores in chronic liver disease using
- total bilirubin
- serum albumin
- PT/INR
- ascites
- hepatic encephalopathy
What are the complications of chronic liver disease?
Hepatocellular failure
Portal HTN
Malignant change
Renal failure (hepatorenal syndrome)
What are the blood investigation of Cirrhosis?
Bloods
- FBC- thrombocytopenia
- INR- raised
- Clotting- PT raised
- LFTs normal unless decompensated
- U&Es- can have hyponatraemia and in hepatorenal syndrome will be derrangement
- AFP- hepatocellular carcinoma
- Albumin- low
Enhanced liver fibrosis
- <7.7. none to mild
- 7.7-9.8= moderate
- > 9.8= severe
Can do viral serology, autoimmune screening, copper levels, Fe studies and a1 antitrypsin levels too
What other testing (imaging) can be done for cirrhosis?
USS liver
- nodules on liver surface
- winding of arteries
- cancer
CT/MRI- look for ascites, hepatosplenomegaly
Fibroscan
-look at extent of fibrosis and liver elasticity
Endoscopy- for oesophageal varices
Liver biopsy- to confirm diagnosis
Ascitic tap if ascites
What is Wilson’s disease?
Inborn error of copper metabolism Deposition of copper in -liver -basal ganglia -cornea (Kayser-Fleischer rings)
What are the appropriate investigations in suspected Wilson’s disease?
Serum caeruloplasmin (reduced) Urinary copper (increased) Liver biopsy (increased copper)
What are the management options for Wilson’s disease?
Chelating agents (trientene, D-penicillamine)
What is Haemachromatosis?
Inherited condition of excess iron deposition leading to fibrosis & organ failure
What is the classic triad of sx in Haemachromatosis?
Bronze skin discolouration
Hepatomegaly
DM
(also hypogonadism - impotence, testicular atrophy)
What are the appropriate investigations in suspected Haemachromatosis?
Serum Fe/Ferritin (increased)
Total iron binding capacity (decreased)
Genetic testing
Liver biopsy
What are the management options for Haemachromatosis?
Vensection
-1 unit/wk
What are the common complications of cirrhosis?
Portal HTN (varices) Ascites Encephalopathy Malnutrition Hepatorenal Syndrome Hepatocellular carcinoma
How does portal HTN related to cirrhosis present?
Often asymptomatic/just hepatosplenomegaly
90% develop gastro-oesophageal varices over 10 yrs
-1/3 bleed
How does ascites related to cirrhosis present?
Mild abdo pain common
-if severe suspect spontaneous bacterial peritonitis
What are the management options for ascites?
Initial bed rest & fluid restriction/low Na diet
Spironolactone
Furosemide (if poor response)
Therapeutic paracentesis/albumin infusion (large volume)
What are the signs of spontaneous bacterial peritonitis?
Fever
Pain
Deterioration in prev well pt
How should spontaneous bacterial peritonitis be managed?
IV ceftriaxone
Prophylactic ciprofloxacin to start post infection to prevent recurrence
-also start if hepatorenal syndrome, Child Pugh score >9, fluid protein <15
What causes encephalopathy?
Accumulation of nitrogenous waste in circulation
Leads to cerebral oedema
What are the grades of encephalopathy?
Grade 1 - altered mood/behaviour, sleep disturbances
Grade 2 - increasing drowsiness/confusion
Grade 3 - stupor, incoherence, restlessness
Grade 4 - coma
What are the management options of encephalopathy?
ICU admission
20o head tilt
Oral lactulose w/ regular enemas with rifamixin
IV manitol/hyperventilation if cerebral oedema
What is hepatorenal syndrome?
Where pooling of blood due to portal HTN causes decreased blood flow to kidneys
Leads to RAAS activation and vasoconstriction of kidney arteries causing them to starve and leads to rapid deterioration in renal function
Fatal within 1 wk unless renal transplant
