Cirrhosis Flashcards
What are causes of cirrhosis?
Alcohol NAFLD Hep C and B PBC Autoimmune hepatitis Haemochromatosis PSC Wilson's disease Alpha 1 anti-trpsin Budd-Chiari Methotrexate
How common if NAFLD?
The commenest disease in the worls - 30% of the general population
Associated with the metabolic syndrome
Histologociallly what is NASH?
A maladaption to oxidate stress causing stearohsis
What is the 2 hit paradigm?
1st hit - excess fat accumulation
2nd hit - intrahepatic oxidative stress, lipid peroxidation , TNF-alpha, cytokine cascade
What is the metabolic syndrome?
Type 2 diabetes Obesity HDL cholesterol lower than 50 mg/dL Hypertension NASH Triglycerides over 150mg/dL
How is simple stetosis diagnosed?
US
No liver outcomes
Increased CV risk
Treatment: weight loss and exercise
How is NASH diagnosed?
Diagnosis at present by liver biopsy
Risk of progression to cirrhosis
Treatment: Weight loss and exercise, other experimental treatments
What are the different types of autoimmune liver disease?
Primary Biliary Cholangitis Auto-immune hepatitis Primary Sclerosing Cholangitis Alcohol related liver disease Drug reactions
What is the presentation of primary biliary cholangitis?
Middle ages women, usually aasymptomatic but can present with fatigue, itch without rash and exanthelasma/xanthomas
How is PBC diagnosed?
Positive AMA
Cholestatic LFTs
Liver biopsy
How is PBC treated?
Ureso deoxycholic acid
What is type 2 auto-immune hepatitis?
Children and young adults
LKM-1
Exclusive
AMA
What is type 1 auto-immune hepatitis?
Adult ANA ASMA SLA severity IgG AMA pANCA
What are the extraheaptic manifestations of type 1 auto-immune hepatits?
Autoimmune thyroditis Graves disease Chronic UC RA Pernicious anaemia Systemic sclerosis ITP SE
What is the clinical presentation of auto-immune hepatitis?
Hepatomegaly Jaundice Stigmata of chronic liver disease Splenomegaly Elevated AST and ALT Elevated PT Malaise, fatigue, lethargy, nausea, abdo pain, anoerxia
How is auto-immune hepatits diagnosed?
Elevated AST and ALT
Elevated IgG
Presence of autoimmune antibodies
Lvier biopsy
What are the differential diagnosis for auto-immune hepatitis?
Wilsons disease Alpha 1 antitrypsin deficiency Viral hepaitits Drug induced liver disease NASH PBC, PSC, autoimmune cholangitis
What does auto-immune hepatitis look like histologically?
Chronic hepatitis with marked piecemeal necoriss and lobular involvement
Numerous plasma cells
Interface hepatitis - hallmark finding
How is autoimmunne hepatitis treated?
Corticosteroids
Axathioprine
What is primary sclerosing cholangitis?
Autoimmune destrictuve disease of the large and medium sized bile ducts
How is primary sclerosing cholangitis diagnosed?
Imagine of biliary tree
Recurrent cholangitis
How is PSC trated?
Maintain bile flow, monitor for chlangiocarcinoma and colorectal cancer
What is haemocrhomatosis?
Mono-genetic autosomal recessive disease of iron overload, C282Y or H63D mutations in HFE gene
What are the complications of haemochromatosis?
Cirrhosis, cardiomyopathy, pancreatic failure, “the bronzed diabetic”
How is haemochormatosis treated?
Venesection
What is wilsons disease?
Mono-genetic autosomal recessive disesae
Loss of function or loss of protein mutations in caeruloplasmin
What is caeruloplasmin?
Copper binding protein, loss of copper regulation with massive tissue deposition of copper, especially liver and basal ganglia
What are the clinical signs of wilson’s disease?
Neurological - chorea-atheitoid movements
Hepatic - cirrhosis or sub-fulminant liver failure
Kaiser fleisher rings
How is wilson’s disease treated?
Copper chelation drugs
What is alpha 1 anti-trypsin deficiency?
Genetic, mutations in the A1At genes, multiple sites, causes variable phenotypes
Protein function lost excess tryiptic activity
What are the linical signs of alpha 1 anti-trypsin deficiency?
Lung emphysema
Liver deposition of mutant protein, cell damage
What is the treatment for alpa 1 anti-trypsin deficiency?
Supportive
What is budd-chiari?
Thrombosis of the hepatic veins, congenital webs, thrombotic tendency, protein C or S deficiency
What is the clinical picture of budd-chiari?
Acute - jaundice, tender hepatomegaly
Chronic - ascites
How is budd-chiari dianogsed?
U/S visualisation of hepatic veins
How is budd-chiari treated?
Tecanalixation or TIPS
What is methotrexate?
Drug used to treat RA and psoriasis
Dose dependent of liver toxxin as it can cause progressive fibrosis
What is cardiac cirrhosis?
Secondary to high right heart pressures (incompetent tricuspid valve, congenital, rheumatic fever, constrictive pericarditis)
How is cardiac cirrhosis diagnosed and treated?
CCF, with too much ascites or liver damage
Treatment is to treat the cardiac condition
