Core: Hepatobiliary Flashcards
Liver failure Aetiology
- Viruses; HAV, HBV, HCV, CMV, HSV, EBV
- POD
- Antibiotics, NSAID’s, statins.
- Acute fatty liver of pregnancy
- HELLP
- Alpha 1 antitrypsin, Wilsons, HH
- Alcohol (acute on chronic)
Liver failure Pathology
- Histology = multi-acinar necrosis
- Cerebral oedema due to break down of gut proteins to ammonia.
Acute liver failure types (timings)
1) Hyper-acute = jaundice to encephalopathy within 7 days
2) Acute = 8-28 days
3) Sub-acute = >21days yet <26 weeks.
Acute liver failure definition
- Acute liver injury + encephalopathy + deranged coagulation
Liver failure presentation
- Jaundice
- Small liver
- Encephalopathic signs - alteration in mental state, drowsiness, confusion, coma
- Fetor hepaticus
- Asterixus
- Raised ICP due to cerebral oedema.
- Bleeding and coagulopathic Sx.
- Oedema and ascites due to low albumin.
- Secondary renal failure - Hepatorenal syn.
Liver failure Ix
1) Bloods - LFT, Clotting and albumin (better markers than LFT), viral serology, Paracetamol level.
2) Imaging; CT/MRI/USS
3) Liver biopsy
4) EEG
Liver failure Rx - criteria for transfer to specialist unit
1) INR >3
2) Encephalopathy
3) Hypotensive despite fluid resus
4) Metabolic acidosis
5) PT in secs > than number of hours following POD
Liver failure Rx
1) ABCDE
2) Supportive care for encephalopathy; bowel wash out + mannitol for raised ICP + Lactulose and rifampicin.
3) Correct metabolic and electrolyte issues (including potassium and BM)
4) Correct coagulopathy w/ vit K, blood products.
5) ABx prophylaxis
6) Liver Tx is definitive.
Patient - Middle aged man w/ bronzed skin, T2DM and hepatomegaly.
- Hereditary hemochromatosis
HH Aetiology
- All types are AR inherited except Type 4 which is AD
- Type 1 = HFE gene
- Type 2 = Juvenile HJV or HAMP
- Type 3 = TfR2 gene
- Type 4 = Ferroportin
HH Pathology
- HFE interacts w/ transferrin receptor 1 which mediated intestinal iron absorption.
- Iron is absorbed exceeding binding capacity of transferrin.
- Hepcidin (made in the liver) is normally downregulated in overload, this is not the case in HH allowing for sustained overload
- Excess iron is taken up by the liver and other organs (kidneys, pancreas, brain, heart)
HH Presentation
- Triad = Bronze skin + DM + hepatomegaly
- Hypogonadism due to pituitary involvement.
- Heart failure and arrhythmia
- Arthralgia of the hands
- Cirrhosis
Reversible effects of HH
- Cardiomyopathy
- Skin pigmentation
Irreversible effects of HH
- Cirrhosis
- DM
- Hypogonadism
- Arthropathy
HH Ix
1) Bloods; serum iron is raised w/ a reduction in TIBC and transferrin saturation.
2) Genetic testing
3) LFT
4) Liver biopsy - not Dx but can help assess extent of damage.
5) MRI - reduced magnetic effect of the liver and panc due to the paramagnetic effects of ferritin and haemosiderin.
HH Rx
1) Venesection - remove excess iron to prevent tissue damage. 500ML tiwce a week until iron is normal and then 4x per year.
2) Screen relatives.
Wilson’s disease Aetiology
- AR inherited.
- Copper transporting ATPase
- chromosome 13
Wilson’s disease Pathology
- Mutation causes a failure in copper being incorporated into caeruloplasmin.
- Low serum caeruloplasmin.
- Mechanism of copper deposition is UK.
Wilson’s disease Presentation
- Often kids
- Liver problems; acute hepatitis, chronic hepatitis, cirrhosis
- Neurological defects (tremor, dysarthria, tics and dementia) personality changes
- KF rings
- Renal tubular acidosis (Fanconi syn)
Wilson’s disease Ix
1) Serum copper and caeruloplasmin - both low.
2) Urinary copper - increased
3) Liver biopsy; suggestive if high levels of copper but not diagnostic.
4) Genetic testing
Wilson’s disease Rx
1) Lifelong penicillamine 1-1.5g/day. Chelates copper.
- SE = rashes, leucopenia and renal damage
Patient - Kid presents w/ behavioural problems, deranged LFT suggesting hepatitis. You notice a tremor and unusual movement and that his speech is unusual.
Wilson’s disease
Patient - COPD + Liver disease
Alpha 1 antitrypsin.
Alpha 1 antitrypsin aetiology
- Genetics
- Gene located on chromo 14
- A1AT is an acute phase reactant.
- A1AT inhibits proteolytic enzyme neutrophil elastase.
A1AT pathology
- UK
A1AT Presentation
- Some present in childhood, often presents later.
- Liver disease (cirrhosis) + Pulmonary emphysema.
A1AT Ix
1) A1AT is low - deficiency
2) Liver biopsy - Periodic acid Schiff positive.
A1AT Rx
1) Nil
2) Manage Sx and complications.
Alcoholic liver disease aetiology
- alcohol consumption
- Ethanol is metabolised in the liver via 2 pathways.
- its metabolism results in an increase in the NAD/NADH ratio.
- This altered redox potential results in increased fatty acid synthesis and accumulation in the liver.
- ROS are released causing damage.
ALD Pathology
- Fatty liver - cells become enlarged with fat (steatosis) which resolves w/ cessation.
- Fibrosis due to alcohol transforming stellate cells to myofibroblasts.
- Alcoholic hepatitis - fatty changes + infiltration of inflammatory cells and hepatocyte necrosis.
- Alcoholic cirrhosis
ALD Presentation
- Fatty liver = often asymptomatic, or age RUQ pressure and hepatomegaly.
- Alcoholic hepatitis = Changes on biopsy and often asymptomatic
- Can produce jaundice and deranged LFT
- Or patient can become acutely unwell w/ ascites and fever due to liver necrosis
- Alcoholic cirrhosis =
- Final stage
- Patients are often well
- signs of chronic liver disease.
ALD Ix
Fatty liver
1) Elevated MCV indicates heavy drinking
2) LFT - elevated ALT,AST, GGT
3) Fibroscan
Alcoholic hepatitis
1) High WCC
2) Deranged LFT
3) Low albumin
4) Liver biopsy
ALD Rx
1) Alcohol cessation (manage effects)
2) Alcoholic hepatitis - steroids and vitamins
3) Liver Tx
Primary biliary cirrhosis aetiology
- UK
- Autoimmune
- Serum anti-mitochondrial antibodies are found.
- Mitochondrial antigen M2.
- Molecular mimicry of E.coli.
- Associated w/ Sjogren’s, scleroderma, thyroid disease, coeliac, glomerulonephritis
- Destruction of bile ducts leads to a build up of toxins in the liver.
PBC presentation
- Women 40-50yo
- Often asymptomatic and found with raised ALP or hepatomegaly
- Itching
- Fatigue
- Jaundice
- Hepatomegaly
- Xanthelasma
- RUQ discomfort.
PBC Ix
1) Mitochondrial M2 antibodies - via elisa.
2) LFT - high ALP
3) Raised serum cholesterol
4) Raised IgM
5) Liver biopsy - portal tract infiltration or lymphocytes and plasma cells.
PBC Rx
1) Ursodeoxycholic acid 10-15mg - improves bilirubin and transferase levels
2) Steroids
3) Supplement fat soluble vitamins
4) Bisphosphonates
5) Colestyramine for the itching or rifampicin or naloxone.
Primary sclerosis Cholangitis aetiology
- Chronic liver disease caused by a progressive cholestasis leading to fibrosis of bile ducts.
- Autoimmunity (ANCA)
- Associated with UC
- Risk of developing cholangiocarcinoma
PSC presentation
- Most patients are male 40yo
- Fatigue
- Itching
- RUQ pain
- Sx may remit and recur
- Jaundice
PSC Ix
1) LFT - deranged w/ increased ALP, Transferases and GGT
2) ERCP or MRCP
3) IBD Ix
PSC Rx
1) Repeated endoscopic dilatation of strictures can help ease Sx by maintaining bile outflow.
2) Ursodeoxycholic acid
4) Similar to PBC
Acute Cholecystitis Aetiology
- Infection and inflammation of the GB
- Often arising from an initial event where the GB is obstructed from emptying.
- Underlying stone disease.
- Ischaemia of the GB occurs when then GB distends.
- Infection due to E.coli, klebsiella, enterococcus and pseudomonas.
Acute cholecystitis Presentation
- Similar to biliary colic.
- Sudden onset and severe constant pain.
- RUQ and epigastric pain.
- N&V
- Fever and rigors
- Murphy’s sign
- Guarding.
Acute cholecystitis Ix
1) FBC - Raised WCC
2) Raised Inflammatory markers
3) Deranged LFT
4) USS for stones, thick GB wall and focal tenderness over the GB
Acute cholecystitis Rx
1) Cholecystectomy
2) Conservative Rx = NBM, fluids, analgesia and Abx - ceftriaxone + metronidazole.
Gallstones Aetiology
Cholesterol stones -
- Common
- Hyperlipidaemia
- Super saturation of the bile; crystallises out.
Pigment stones -
- Black = calcium bilirubinate; associated w/ haemolytic anaemia.
- Brown = calcium salts and fatty acids. Found in bile stasis and infection.
Gallstones Presentation
- Asymptomatic
- Seen incidentally
- Biliary colic - sudden onset severe RUQ pain
- Associated w/ fatty foods
- Radiation of pain to subscapular region RHS.
Gallstones Ix
1) Bloods.
2) Raised inflammatory markers
3) LFT issues
4) USS
Gallstones Rx
1) Shock dissolution
2) Cholesterol reducing meds such as statins.
3) ERCP retrieval.
Post cholecystectomy syndrome
- RUQ pain months after removal of GB
- Similar pain to biliary colic.
- Due to small bowel spasms at hepatic flexure.
Liver cirrhosis Aetiology
- Diffusely abnormal liver architecture.
- Interferes w/ hepatic blood flow.
- Alcohol, viral hepatitis, NAFLD, PBC, PSC, metabolic liver disease.
- Chronic injury
- Stellate cells transform to myofibroblasts causing fibrosis.
- Portal vein blood flow into the liver is impaired.
Liver cirrhosis presentation
- RUQ discomfort
- weakness, anorexia, fatigue
- Ascites
- Haematemesis
- Itching due to cholestasis and build up of bile salts in the blood.
- Jaundice.
- Pale stools and dark urine.
o/e
- Palmar erythema
- Xantholasmata
- Jaundice
- raised JVP
- Spider naevi
- HSM
Liver cirrhosis Ix
1) LFT
2) Albumin and PT are best indicators of hepatic function.
- Thrombocytopenia is the most sensitive and specific lad finding indicating cirrhosis in those w/ liver disease.
3) Electrolyte issues - low sodium due to oedema.
4) Fibroscan
5) USS for HCC
6) Endoscopy for varices.
Liver cirrhosis Rx
1) Manage complications of decompensated liver disease.
2) USS for HCC
3) Rx the underlying cause.
Portal HTN Aetiology
- Portal vein is formed at the union of the SMA and splenic vein.
- Blood is fed to liver from the bowel and then back to the heart from the liver via the hepatic vein.
a) Pre-hepatic = portal vein thrombosis
b) Hepatic = Distortion of the liver architecture; schistosomiasis and cirrhosis
c) Post hepatic = venous blockage beyond the liver; HF or IVCO
- As the pressure increases, other collateral vessels compensate.
- Gastro-oesophageal junction (varices), left renal vein, diaphragm, rectum.
Portal HTN presentation
- Sign of chronic liver disease.
- Haematemesis (varices)
- Malaena (upper GI bleeding)
- Ascites
- Collateral formation; dilated abdominal wall vessels, Caput medusa, haemorrhoids.
Portal HTN Ix
1) USS for portal HTN - doppler.
2) Endoscopy for Varices
3) Bloods and viral serology
4) CT/MRI
Portal HTN Rx
1) Rx portal HTN w/ BB - propranolol.
2) Endoscopic rx before bleeding; banding to stop vessels.
3) Transjugular intrahepatic portosystmic shunt - connecting hepatic vein and portal vein.
Acute bleeding;
- Banding
- Sclerotherapy
- Systemic vasoconstriction w/ terlipressin and somatostatin.
- Balloon tamponade.
Pancreatitis Aetiology
- Acute or chronic
- Acute - in a normal panc which returns to normal.
Gallstones Ethanol Trauma Steroids Mumps, malignancy Autoimmune Scorpion stings Hypercalcaemia or cholesterol ERCP Drugs - AZA, oestrogens, valproate, furosemide, metronidazole.
Pancreatitis Presentation
- Upper abdominal pain
- Epigastric
- N&V
- involvement of the retroperitoneum leads to back pain.
o/e
- Upper abdo tenderness
- Shock
- Cullens sign (peri-umbilical bruising)
- Grey-turner’s sign (flank bruising)
Pancreatitis Ix
1) Serum amylase - 3x upper limit of normal
2) Urinary amylase
3) Serum lipase
4) CRP/ESR
5) Bloods; ABG
6) CXR to exclude perforation.
7) USS for stones
8) Contrast CT 72hours after attack to assess extent of damage
9) ERRCP/MRCP
Pancreatitis Rx
1) APACHE 2 scoring
2) ABCDE
3) NG tube removal of vomit
4) Prophylactic ABx - imipenem.
5) Analgesia - tramadol.
6) TPN feeding
7) Thromboprophylaxis.
Pancreatic Ca Aetiology
- Adenocarcinoma
- Smoking
- Excess alcohol or coffee
- DM
- Chronic pancreatitis
- Genetics - PRSS-1 mutation
Pancreatic Ca Presentation
- Most are at the head of the pancreas.
- Pain + anorexia + weight loss.
- Many present late and have a long Hx of low-grade Sx.
- Painless jaundice - distal common bile duct being blocked.
- Malabsoprtion and steatorrhoea.
Pancreatic Ca Ix
1) Patients often present at a later stage w/ invasive Ca.
2) USS - mass at the head of the pancreas.
3) CT + contrast.
4) PET for mets.
5) CA19-9
Pancreatic Ca Rx
1) Whipple’s procedure - pancreatoduodenectomy + adjuvant/neo-adjuvant chemo.
2) Often palliate and manage Sx.
Alcoholic hepatitis Management
- Often nil
- Prednisolone for severe cases - Maddrey discriminant function value.
Autoimmune hepatitis
- Causes
- Presentation
- Ix
- Rx
- UK
- associated w/ other autoimmune disorders. - Chronic liver disease
- Acute hepatitis - fever, jaundice
- Amenorrhoea - Liver biopsy
- Antibody testing - Steroids and immunosuppressants; AZA.
- Liver Tx
