Liver et al Flashcards
Main 4 points in acute liver failure
Hepatic encephalopahty
Jaundice
Coagulopathy
With no evidence of serious prior liver disease.
Time scale and classification of acute liver disease
Hyperacute = within 7days Acute = 8-29 days Subacute = 4 to 12 weeks
Also Fulminant = within 2 weeks and subfulminant within 2-12weeks.
Risk factors for acute liver failure
Chronic alcohol use
Poor nutrition
Older age (over 40)
Narcotic use
Causes of acute liver failure
Paracetamol toxicity Viral hepatitis - HBV and HAV Budd-Chiari syndrome CMV, EBV Autoimmune liver disease Wilson's disease Alpha-1 antitrypsin deficiency.
S+S of acute liver failure
Jaundice
Symptoms of hepatic encephalopathy - altered mood, sleep disturbance, slurred speech, confused.
Abdo pain
Nausea and vomiting
O/E:
Signs of hepatic encephalopathy - apraxia, liver flap, stupor.
Jaundice
Hepatomegaly
How to test for apraxia
Copy a 5 point star
Show me how to brush your teeth, use a hammer, comb your hair etc etc
Ix for acute liver failure
Laboratory: FBC U+E LFT Clotting and synthetic liver function BM Paracetamol levels Viral hepatitis antibodies Copper studies, alpha1-anti trypsin levels etc etc Blood culture, urine culture, ascitic tap mc+s
Imaging: Abdo USS CT/MRI of liver CT head Doppler USS of liver and portal vein.
Management of acute liver failure
In ITU 🌈
A-E including urinary catheter, NG tube, frequent BMs.
Treat cause.
Treating complications of acute liver failure
1) Cerebal oedema
2) Ascites
3) Hypoglycaemia
4) Encephalopathy
1) 20% Mannitol IV
2) Fluid and salt restriction, diuretic e.g spironolactone.
3) 10% glucose IV
4) Lactulose + Rifaximin.
List 5 hepatotoxic drugs
Pyrazinamide, Isoniazid (TB) Paracetamol Methotrexate Azothioprine Infliximab
Liver function test:
Which are markers of hepatocellular damage?
Which are markers of the liver’s synthetic function
Damage = ALT, AST, ALP, gamma-GT.
Synthetic liver function = PT, Albumin and bilirubin.
Interpreting ALT and ALP
ALT = mostly found within hepatocytes so if serum levels high = injury to hepatocytes. ALP = high concentrations in liver, bile duct and bone tissue, if serum levels high = cholestasis.
Therefore:
a >10X rise in ALT and a <3X rise in ALP = hepatocellular injury.
a >3X rise in ALP and a <10X rise in ALT = cholestasis.
And can have a mixed picture too.
List some functions of the liver
Bile production - stored in gallbladder.
Metabolise fat soluble vitamins e.g. vitamin D hydroxylation.
Drug metabolism using CYP450 enzymes.
Conjugation and elimination bilrubin
Synthesis of plasma proteins e.g. albumin
Synthesis of clotting factors
Gluconeogenesis
Pathophysiology of cirrhosis
Fibrosis and loss of normal liver architecture to abnormal, regenerative nodules.
Causes of cirrhosis
Chronic HBV and HCV Non-alcohol fatty liver diease Chronic alcohol Alpha1-antitrypsin deficiency Wilson's disease Budd-Chiari syndrome Drugs = methotrexate Primary biliary cholangitis
Presentation of liver cirrhosis
Abdo distension Jaundice Itch Easy bruising Poor memory Fatigue and weakness Peripheral oedema
O/E: LOTS!!!
Hands = Leuconychia (white nails), clubbing, palmar erythema, spider naevi, Dupuyren’s contracture.
Face = telangiectasia, xanthelasma, yellowing of sclera in eyes (jaundice)
Abdo = gynaecomastia, hepatomegaly, visible collateral vessel sin the abdo wall. Ascites + shifting dullness and fluid thrill on percussion.
Other = muscle wasting, loss of pubic hair, testicular atrophy on men.
Investigating cirrhosis and expected results
Laboratory: LFT - raised liver enzymes. Gamma-GT - raised. Albumin - low PT and INR - high FBC Ascites tap mc+s Find cause - viral hepatitis antibodies serology, ferritin, alpha1-antitrypsin levels.
Imaging: Abdo US + duplex Abdo CT/MRI Liver biopsy Transient elastography
What will you see in the histology of a liver biopsy in cirrhosis
Loss of hepatic architecture.
Bridging fibrosis.
Nodular regeneration.
Classification of liver cirrhosis
Child-Pugh-Turcotte Score.
Based on: (BAAIN mnemonic) Bilirubin Ascities Albumin low INR high Encephalopathy
Class = level of cirrhosis and determines life expectancy.
End-stage liver disease score
Model of End-Stage Liver Disease score uses bilirubin, sodium, creatinine and INR/PT to assess liver disease.
Used in liver transplant list.
Management of cirrhosis
Lifestyle advice = avoid alcohol, exercise to avoid muscle wasting, good nutrition, avoid hepatotoxic drugs (NSAIDs).
Management of ascites = fluid restriction, low salt diet, spironolactone +/- furesomide, daily weight.
Liver transplant list appropriateness.
Fluid restriction level in ascites and target weight loss with spironolactone
Max 1.5L per day.
Aim for up to 0.5g/day of weight loss.
Complications of cirrhosis
Hepaticellular carcinoma Coagulopathy Encephalopathy Sepsis Spontaneous bacterial peritonitis Hypoglycaemia Oesophageal varicies (and GI haemorrhage)
Spontaneous bacterial peritonitis
Ascites + sudden deterioration.
Commonly E.coli, Klebsiella
Rx = Cefotaxime or Tazosin
Can give prophylactic Ciprofloxacin.
Portal triad and another thing which helps bile flow
Portal vein
Hepatic artery
Bile duct
Found in a corner of each liver lobular.
Bile canaliculi.
Decompensated cirrhosis
Acute deterioration in a patient with chronic liver disease.
Precipitated by: infection (Spont bac peritonitis), high alcohol intake, drugs (NSAIDS, opiates), portal vein thrombus.
S+S: jaundice, increasing ascites, hepatic encephalopathy, AKI, oesophageal varices haemorrhage/GI bleed.
Ix: Vital signs, FBC, U+E, LFT, coagulation, blood glucose, blood cultures, ascitic tap MC+S, urine dipstick, CXR and AXR. Alcohol intake history.
Rx: A to E assessment, treat cause (ABx, FFP/transfusion, lactulose + Rifaxmin)
Grading of hepatic encephalopathy
West-Haven criteria
Compound involved in hepatic encephalopathy
Serum ammonia build up causing glutamine excess in brain which draws fluid in = cerebral oedema.
Name 3 metabolic liver diseases and how are they all inherited
Hereditary haemochromatosis, Chr 6
Wilson’s disease, Chr 13
Alpha-1 antitrypsin deficiency, Chr 14
All autosomal recessive 🧬
Pathophysiology of hereditary haemochromatosis
Autosomal recessive.
Inherited on chromosome 6, HFE gene.
Lack of hormone HEPCIDIN which regulates iron levels.
Increased iron absorption from intestine.
Deposits of iron in liver, heart, skin, joints and pancreas.
More common in males as females loose blood and hence iron during menses.
S+S of hereditary haemochromatosis
Early in life = asymptomatic, tiredness, arthralgia, low libido.
Mid-life = slate-grey tinged skin pigmentations, arthralgia, DM, fatigue and weakness, hepatomegaly, signs of cirrhosis and chronic liver disease, cardiac arrythmias.
Where is the arthralgia common in HH
2nd and 3rd metacarpophalangeal in hands.
Investigating hereditary haemochromatosis
Iron studies:
Serum transferrin saturation = >45% (specific marker).
Serum ferritin = raised.
Serim TIBC = low/normal
LFT HFE gene testing. MRI Liver biopsy + Perl's stain = iron loading and fibrosis. Liver FibroScan
Ix extra-hepatic manifestations: echocardiogram, BM/HbA1c/OGTT.
Management of hereditary haemochromatosis
Weekly venesection - target ferritin <50mcg/L.
Monitor for DM and arrhythmias.
Consider for transplant.
Pathophysiology of Wilson’s disease
Autosomal recessive.
Mutation in chromosome 13.
Copper retention in liver and basal ganglia due to impaired excretion in the liver.
Presentation of Wilson’s disease
Early in life = Liver disease signs (hepatitis, cirrhosis).
Young adults = CNS signs (tremor, dysarthria, dystonia, dementia, ataxia).
Psych = depression, change in libido, personality change, poor memory.
O/E:
Kayser-Fleischer rings from copper in iris.
Blue nails
Hypermobile joints
Grey tinged skin
Hepatomegaly and signs of cirrhosis (spider naevi, jaundice, ascites, bruising).
Investigating Wilson’s disease
Copper studies:
24hr urinary copper excretion = high
Serum copper = low
Serum caeruloplasmin = low
LFT = raised enzymes. Hepatic biopsy = high copper levels. Slit-lamp exam = Kayser-Fleischer rings. Head MRI = degeneration of basal ganglia. Genetic testing for Wilson's disease
Management of Wilson’s disease
Lifestyle advice = avoid copper rich foods (chocolate, mushrooms, liver meat). Avoid alcohol and hepatotoxic drugs.
Chelation agent to bind to copper and excrete in urine = penicillamine (oral)
Prevent copper absorption = zinc compounds.
Liver transplantation appropriateness.
Screen family!
Drug for chelation of copper in Wilson’s disease
Penicillamine
S/Es = rash, nausea, anaemia, haematuria etc etc.
Pathophysiology of alpha1-antitrypsin deficiency
Autosome recessive.
Chromosome 14, SERPINA1 gene.
Loss of protease inhibitor involved in inflammatory cascade causing serpinopathy.
Loss of neutrophil elastase enzyme action.
S+S of alpha1-antitrypsin deficiency
Mostly males.
Symptoms fo COPD in under 40yrs e.g. productive cough, SOBOE, wheeze.
Symptoms of cirrhosis e.g. ascites, jaundice, confusion, hepatomegaly, cholestasis.
Investigating alpha1-antitrypsin deficiency
Serum alpha1-antitrypsin levels = low –> genetic testing/phenotyping.
Spirometry = obstructive pattern.
CXR
LFTs - raised enzymes.
Liver biopsy = diastase-resistant globules, +ve for Periodic acid Schiff
Management of alpha1-antitrypsin deficiency
Lifestyle advice - smoking cessation, annual influenza vaccine.
Treat as for COPD
Monitor LFT, screen for HCC.
Drug to help pruritus in liver disease
Colestyramine.
Pathophysiology behind autoimmune hepatitis
Autoantibodies against hepatocyte surface antigen cause inflammation and necrosis.
T-cell mediated attack.
Associated with HLA-DR3
Common patients with autoimmune hepatitis
Females. Not usually that old, under 40yrs.
Presentation of autoimmune hepatitis
Acute = 40% present acutely.
Fever, jaundice, abdo pain.
Subacute = gradual onset jaundice, pruritus, hepatomegaly, abdo discomfort, signs of cirrhosis (confusion, ascites)
CFx of other autoimmune diseases e.g. arthralgia, bowel dysfunction (UC).
Investigating autoimmune hepatitis
LFT - raised transaminases, ALT/AST >1.5X.
FBC - anaemia, low WCC, low platelets esp if splenic dysfunction too.
Criteria:
Serum protein electrophoresis for autoantibodies - ANA, ASMA, LKM1.
Hypergammaglobulinaemia esp IgG
Liver biopsy - mononuclear infiltrates of portal and periportal areas, piecemeal necrosis, fibrosis, cirrhosis.
What is seen in liver biopsy of autoimmune hepatitis
Mononuclear infiltrates in portal and periportal areas = interface hepatitis.
Piecemeal necrosis
Fibrosis.
Management of autoimmune hepatitis
1) Prednisolone - high dose then reduce after 1 month for maintenance.
2) If corticosteroids CI can use Azathioprine or if not responsive to mono therapy use in combo.
Liver transplant
Definition of portal hypertension
Increase in portal venous pressure due to increase in hepatic vascular resistance and hepatic blood flow. Commonly caused by chronic end-stage liver disease.
The raised pressure opens up venous collaterals to connect the portal and system circulation.
Clinically significant if pressure gradient is >10mmHg.
Causes of portal HTN
Prehepatic - before portal vein in liver:
- Congenital atresia
- Portal venous thrombus
- Extrinsic compression by tumour.
Hepatic:
- Cirrhosis
- Chronic hepatitis
- Sarcoid granulomata.
Post-hepatic - block in hepatic vein:
- Budd-Chiari syndrome
- Constrictive pericarditis
- Right heart failure
Sites for portal-systemic circulation collaterals
Gastro-oesophageal junction = oesophageal varices.
Anterior abdo wall = visible as caput medusae around umbilical area.
Ano-rectal junction
Retroperitoneal viscera.
Effects of portal HTN on systemic circulation
Produce hyperdynamic circulation Bounding pulse Hypotension Increases cardiac output Salt and water retention = hypervolaemia and hypernatraemia.
Presentation of portal HTN
Presents as chronic liver disease mostly - jaundice, alcohol consumption, ascites, hepatomelgay, splenomegaly, hepatic encephalopathy, visible collaterals on abdo wall, spider naevi, gynaecomastia, liver flap, oesophageal varicies bleed.
Vital signs = bounding pulse, low BP but warm peripheries.
Investigating portal HTN
Abdo USS. Doppler US of portal vessels. Spiral CT Endoscopy for oesophageal varices. hepatic venous pressure gradient (>10mmHg)
Bloods:
LFT, U+E, FBC, clotting profile, PT.
Find cause - iron studies, copper studies, hepatitis serology etc etc
Management of portal HTN
Reduce BP = non-selective beta blockers (propranolol), nitrates.
Surgical = transjugular intrahepatic bypass shunt.
Treat or manage cause - diuretics, salt and fluid restriction,
Hepatopulmonary syndrome
Complication of portal HTN
Hepatic dysfunction, hyperaemia, extreme vasodilation.
Common cause of haemoperitoneum
Trauma + HCC
Common cancers in the liver
Most common are due to metastasis from breast, bronchus and GI tract.
Primary liver tumours = hepatocellullar carcinomas.
Risk factors/Causes for hepatocellular carcinoma
MALE Hepatitis B and C virus. Heavy, chronic alcohol consumption. Metabolic syndrome and obesity. Non-alcohol fatty liver disease.
S+S of HCC
Fatigue Anorexia RUQ pain Loss of weight Bruit over liver area on auscultation.
S+S of cirrhosis e.g.
Itch, bleeding oesophageal varices, ascites, jaundice, hepatomegaly,collateral veins on chest, liver flap.
Ix for HCC
CT = DIAGNOSTIC, then MRI and BIOPSY.
Lab:
FBC, LFT, U+E, PT, viral hepatitis serology, alpha fetoprotein.
Screening via ultrasound scans.
Management of HCC
Resection of tumour.
Ablation therapy
Liver transplant list.
Advanced/untreated = Lenvatinib or Sorafenib.
Not great prognosis, 6months from diagnosis.
Prevention measures of HCC
High-risk group annual ultrasound scan screening and monitor AFP (HBV patients).
HBV vaccine programme.
Needle exchanges, safer sex education.
Another name for Budd-Chiari Syndrome
Hepatic venous outflow obstruction - obstruction anywhere from inferior vena cava to small hepatic veins.
Triad in Budd-Chiari
Abdo pain, ascites and hepatomegaly (quite rapid onset).
Causes of hepatic venous outflow obstruction/ B-C syndrome
Vascular - right heart failure, constricitve pericarditis, protein C and protein D deficiency and other thrombophilic disoders.
Infective - syphilis, amoebic abscess
Trauma
Autoimmune - SLE, Sjogren’s, IBD
Iatrogenic - COCP
Neoplastic - HCC, Wilm’s tumour
Congenital - congenital anatomical abnormality in vena cava.
Presentation of hepatic venous outflow obstruction
Sudden abdo pain
Ascites
Hepatomegaly
AKI
rare = jaundice.
Ix and Mx of hepatic venous outflow obstruction
Doppler USS
Other = LFT, PT, ascitic fluid mc+s, CT or MRI of liver, US of hepatic vessels.
Liver biopsy.
Treat cause.
Pathophyisology behind ascites
Decreased oncotic pressure due to hypoalbuminaemia and increased hydrostatic pressure form portal hypertension
3 stages of alcohol liver disease
Steatosis (fatty), mainly triglycerides.
Hepatitis (inflammation and necrosis)
Cirrhosis (regenerative nodules, fibrosis, loss of normal architecture)
Investigating alcohol liver disease
LFT Bilirubin Albumin gamma-GT PT FBC, U+E.
Hepatic USS
Liver biopsy - depends on extent of disease on what you will see. Steatosis –>. hepatitis –> cirrhosis signs.
Treatment of alcohol liver disease
Lifestyle = REDUCE alcohol consumption. Avoid smoking, reduce weight, nutritional advice.
Pharma = corticosteroids e.g prednisolone.
Assess appropriate for transplant?
Other causes/RFx of non alcohol fatty liver disease
Metabolic syndrome/Obesity T2DM PCOS OSA Hypothyroidism Drugs e.g. methotrexate, corticosteroids
Signs and symptoms of alcohol and non alcohol related fatty liver disease (steatosis/steatohepatitis)
Asymptomatic Fatigue Right upper quadrant pain. Hepatomegaly Persistent elevation in LFTs!
S+S of cirrhosis:
Pruritus, jaundice, nausea, bruising, gynaecomastia, spider naevi, ascites etc etc
LFT in steatosis
ALT x3 and are higher than AST levels.
Although AST:ALT ratio is <1
LFT in acute alcohol hepatitis
AST:ALT ratio >2
Management of non alcohol related fatty liver disease
LIFESTYLE: avoid alcohol, loose weight, control co-morbidities e.g. DM, HTN.
Secondary care Rx = vitamin E
Causes of liver abscesses
Pyogenic = bacteria e.g. E.coli, Klebsiella. Complications of appendicitis, cholangitis, biliary stones. Amoebic = complication of amoebiasis - Entamoeba histolytica.
RFx = DM, immunocompromised, local malignancies, visit to amoebiasis area.
S+S of liver abscess
Fever Fatigue Nausea and vomiting RUQ tenderness Hepatomegaly
Ix for liver abscess
FBC LFT Synthetic function - albumin and PT Blood cultures CRP and ESR Serology for E.histolytica.
Abdo USS –> CT.
Guided needle aspiration for mc+s
Mx of liver abscess
Drainage
ABx - Tazosin
Acute pancreatitis causes
Causes: Gall stones Ethanol/alcohol Trauma Surgery Malignancy Autoimmune conditions e.g. SLE Scorpion venom Hyperlipidaemia, Hypercalcaemia Post-endoscopic retrograde cholangiopancreatography. EMV, mumps and other infections Drugs e.g. thiazide diuretics
Complications of acute pancreatitis
Haemorrhagic pancreatitis
Necrosis of pancreas and decline in pancreatic function and infected pancreatic necrosis.
Pseudocyst (disruption in pancreatic ducts lead to collections of pancreatic fluid).
Pancreatic abscess
Fistula e.g. to abdo cavity causing ascites, pericardial cavity causing effusions.
Presentation of acute pancreatitis
Severe, sudden onset epigastric pain, radiating to back. (intensity and location of pain not correlate with severity)
Worse on movement and alleviated by fetal position.
Nausea and vomiting
Anorexia
O/E: tachycardia Hypotension Abdo tenderness in upper right quadrant, rebound and guarding. Abdo distension. Cullen's sign Grey-Turners sign
Cullen’s sign
and
Grey-Turners sign
Present in haemorrhage pancreatitis.
Cullens = bluish discolouration around umbilicus.
Grey-Turner’s = bluish bruising-like discolouration on flanks.
Ix and Mx for acute pancreatitis
Resusciation - oxygen, fluids, analgesia, assess feeding ability/NG tube, Abx e.g. Ciprofloxacin.
Ix to prioritise - Lipase, amylase, haematocrit (sign of necrosis), ABG. Transabdo USS in resus bay, CT of abdomen.
Analgesia - not morphine as can spasm Sphincter of Oddi, use buprenorphine ± intravenous (IV) benzodiazepines.
Criteria for acute pancreatitis prognosis
Glasgow criteria
Uses info from first 48hrs of admission to predict prognosis. Score greater than 2 = severe pancreatitis likely.
Parameters = PANCREAS PaO2 Age (>55) Neutrophils/WCC Calcium Renal function (urea) Enzymes (Lactate dehydroengase) Albumin Sugar (blood glucose)
Criteria for alcohol induced pancreatitis
Ranson score
Causes of chronic pancreatitis
ALCOHOL Smoking Autoimmune e.g. Sjogren's, primary biliary cirrhosis. Drugs - oestrogen, thiazide diuretics. Tropical Idiopathic
S+S of chronic pancreatitis
Recurrent, chronic upper epigastric pain. Can radiate to back. Described as deep, severe. Alleviated on sitting forward.
Nausea
Anorexia
Hx of alcohol misuse
Hx of recurrent acute pancreatitis.
Exocrine dysfunction = weight loss (malabsorption), statorrhoea.
Endocrine dysfunction = DM and impaired glucose regulation.
O/E:
Jaundice
Abdo distension
2 pathological mechanisms in chronic pancreatitis
Endocrine dysfunction from islets of langerhans, failure to produce insulin.
Exocrine dysfunction from acini cells, failure to produce digestive enzymes.
Ix chronic pancreatitis
Lab: FBC Faecal elastase U+E Creatinine Amylase Calcium HbA1c LFT
Imaging:
Abdominal USS
CT scan
Management of chronic pancreatitis
Lifestyle advice - avoid smoking and alcohol.
Diet advice - low fat, high protein, high calorie diet
Analgesia - paracetamol, ibuprofen.
Nutritional - replace pancreatic enzymes with Lipase, Creon. Supplement fat soluble vitamins.
Screen for DM
Screen for osteoporosis (malabsorption complication)
Dietary and nutritional support in chronic pancreatitis
Low fat, high protein, high calorie diet.
Supplement fat soluble vitamins
Pancreatic enzyme supplements.
Main complication of chronic pancreatitis
Diabetes mellitus.
Functions of pancreas
Endocrine - Islets of Langerhans produce insulin
Exocrine - acinar cells produce pancreatic fluid
Pancreatic fluid = Lipase, amylase, proteases, bicarbonate and water etc etc
Histology of pancreatic cancers
Most cancers are exocrine tumours - adenocarcinomas esp infiltrating ductal adenocarcinoma of head/neck/uncinate.
Endocrine cancers = pancreatic neuroendocrine tumours (PET).
Risk factors for pancreatic exocrine tumours
Smoking FHx Obesity Chronic pancreatitis Peutz-Jegher's syndrome
Presentation of pancreatic exocrine tumours
Epigastric pain, radiating to back. Easing on sitting forward.
Back pain.
Painless, progressive, obstructive jaundice
Weight loss
Anorexia
Steatorrhoea and malabsorption
O/E:
Palpable gallbladder = Courvoisier’s sign.
Courvoisier’s sign
Palpable gallbladder with painless jaundice.
Cause unlikely to be gallstones therefore Ix for neoplasm.
Investigating pancreatic cancer
Lab:
FBC, LFT, glucose.
TUMOUR MARKER = CA19-9.
Imaging:
Pancreatic protocol CT scan or PET-CT
Endoscopic US with tissue sampling –> Biopsy histology and cytology.
Management of pancreatic cancer
Nutritional support - pancreatin
Resection - Whipple’s procedure
Chemotherapy
Pallative care
Example of a pancreatic neuroendocrine tumour
Insulinoma
Vipoma
Biliary colic
Right upper quadrant pain, may radiate to intra-scapular region.
Impact of a stone in cystic duct or ampulla of water.
Acute cholecystitis
RUQ pain + fever/raised WCC
Distension of the gallbladder causing ischaemia.
+ve Murphy’s sign.
Ascending cholangitis
MEDICAL EMERGENCY!!
Stasis of bile due to obstruction leads to infection (E.coli, Klebsiella).
RUQ pain + fever/raised WCC + Jaundice = CHARCOT’S TRIAD
What is Charcot’s triad and when does it occur
RUQ pain + fever/raised WCC + jaundice.
In ascending cholangitis (Infection of bile duct)
Types of gallstones
Cholesterol stones (most common)
Pigment - black or brown.
Mixed
What is in bile?
Cholesterol, bile pigments (broken down Hb), phospholipids.
Cholelithiasis
Presence of solid stones in gallbladder
Presentation of gallstones
Most are asymptomatic.
Biliary colic (most common presentation) - post-prandial, nausea.
Acute cholecystitis
Ascending cholangitis = jaundice.
Who gets gallstones // Risk factors
Family history, fat, around 40, females.
Obesity, DM,
Investigating gallstones
FBC, LFT, lipase and amylase.
Abdominal USS shows thickened gallbladder wall, dilated CBD.
ERCP
MRCP (magnetic resonance cholangiopancreatography).
Managing gallstones
Watch and wait.
Analgesia - pethidine
Laparoscopic cholecystectomy +/- bile duct clearance.
Murphy’s sign - what is it and when is it seen?
2 fingers over RUQ and patient inspires = pain and pt will stop breathing in. Repeat in left upper quad and no pain.
Seen in acute cholecystitis.
Ix, Mx and complications for ascending cholangitis
ABC (if all there make diagnosis if only A+ B or C suspected it)
A = systemic inflammation (fever, elevated WCC or CRP)
B = Cholestasis (Jaundice abnormal LFTs)
C = Imaging KUB XR/USS or CT or MRCP (biliary dilation, evidence of gallstones)
Other Ix = U+E, amylase, blood cultures.
Mx = Resuscitation. Broad spec ABx (metronidazole + ceftriaxone), emergency biliary drainage.
Cx = sepsis/septic shock, AKI, liver abscess, liver failure.
What conditions are associated with primary sclerosis cholangitis
Inflammatory bowel disease (UC and Crohn’s)
Hepato-biliary malignancies e.g. HCC
Colorectal cancer.
Pathophysiology behind primary sclerosis cholangitis
Chronic, cholestatic liver disease due to inflammation and fibrosis of intrahepatic and extra hepatic bile ducts.
Leads to bile duct stricture formation.
Progressive disorder which can cause development of cirrhosis, portal hypertension and hepatic decompensation.
Presentation of primary sclerosis cholangitis
Hx of IBD
RUQ pain
Jaundice
Weight loss, fatigue.
O/E:
Hepatomegaly
Jaundice (yellowing of sclera)
Ix for primary sclerosis cholangitis
LFT - high ALP, ALT. Immunoglobulins - high IgG Autoantibodies e.g. p-ANCA Imaging: Liver USS, MRCP - shows multifocal strictures and dilations of bile ducts. Biopsy to stage.
Mx of primary sclerosis cholangitis
Lifestyle advice = avoid alcohol.
Supplementation for fat soluble vitamins
Pruritus = Colestyramine.
Bile acid analogue = Ursodeoxycholic acid, improve LFT not life expectancy.
Annual colonoscopy for colorectal cancer.
Liver transplant
Another name for primary biliary cirrhosis
Primary biliary cholangitis
Pathophysiology behind biliary cholangitis
Chronic autoimmune granulomatous inflammation damages interlobular bile ducts (canals of Hering) leading to cholestasis, fibrosis, cirrhosis and portal hypertension.
Which autoantibody is most highly associated with primary biliary cholangitis
Antimitochondrial antibodies (AMA)
Sterotypical patient presenting with primary biliary cholangitis
50yr old woman who’s sister has got similar symptoms.
S+S of primary biliary cholangitis
Fatigue
Pruritus
RUQ pain
O/E:
jaundice
Xanthelasma
hepatomegaly
Most common autoimmune conditions associated with primary biliary cholangitis
Sjorgren’s syndrome - dry eyes, dry mouth.
Thyroid disease
Criteria to diagnose primary biliary cholangitis
Biochemical evidence fo cholestasis (raised ALP).
AMA +ve
Biopsy shows evidence of interlobular bile duct destruction.
Ix for primary biliary cholangitis
Biochemical: LFT - raised ALP, raised gamma-GT Autoantibodies - AMA + Thyroid function test Immunoglobulins - raised IgM Cholesterol raised In later disease - raised bilirubin
Imaging
USS to exclude other causes of cholestasis. MRCP/cholangiography.
Biopsy
Mx for primary biliary cholangitis
Pruritus = cholestyramine
Bile acid analogue = Ursodeoxycholic acid
Immunosuppression = Prednisolone
Curative = transplant
Differences between primary sclerosis cholangitis and primary biliary cholangitis
PSC = male, sclerosis, intrahepatic and extrahepatic ducts, associated with p-ANCA autoantibodies, common in UC and colorectal cancer patients.
PBC = female, interlobular hepatic bile duct involvement, associated with AMA autoantibodies, common in Sjorgren’s and thyroid disease patients.
Name a cancer of the biliary tree 🌲 and list some facts about it.
Cholangiocarcinoma / Klatskin’s tumour:
- Ductal adenocarcinomas
- Associated with liver flukes so more common in SE Asia.
- Jaundice, RUQ pain, pruritus, hepatomegaly, weight loss, cholestasis (raised ALP).
- CA19-9 and CEA raised.
- Rx = resection.
Where is bile made and where is stored
Made in liver, stored and concetrated in gall bladder.
What is the poo and wee like in cholestasis?
Dark urine, pale stools - conjugated hyperbilirubinaemia.
Conjugated bilirubin can’t be excreted in faeces so reflexed back into systemic circulation and excreted in urine.
Blood test results in cholestasis
Raised ALP (x3) Raised serum bilirubin
Main symptoms of cholestasis and their treatment
Jaundice
Dark urine, pale stools
Pruritus - cholestyramine
2 types of causes for jaundice
Unconjugated hyperbilirubinaemia - unconjugated bilirubin is water insoluble. Caused by: over production from haemolysis, poor hepatic uptake from ischaemic hepatitis, Gilbert’s syndrome.
Conjugated hyperbilirubinaemia - conjugated bilirubin is soluble so dark urine. Caused by: hepatocellular dysfunction from cirrhosis, haemachromatosis, hepatitis, Budd-Chiari and cholestasis from PBC, PSC, pancreatic cancer.
What type of bilirubin is found in urine and faeces
Urine = urobilinogen Faeces = stercobilinogen.
Describe hepatorenal syndrome
- Renal failure with severe liver disease.
- Poor prognosis.
- Renal vasoconstriction, portal hypertension.
- Diagnosis must exclude cause of renal failure.
- Mx = terlipression, dialysis, TIPS
Causes of ascites
Cirrhosis and liver disease
Heart failure
Nephrotic syndrome
Ovarian malignancy
2 signs O/E for ascites
Fluid thrill
Shifting dullness
Complications of ascites
Spontaneous bacterial peritonitis
Hepatorenal syndrome
Why would someone with chronic liver disease get prophylactic ABx
For spontaneous bacterial peritonitis - which has a high mortality rate.
CFx, common organisms and Mx for SBP
CFx = abdo pain, fever, vomiting. O/E: guarding, rebound tenderness, pain on palpation diffusely over abdo.
Ix = paracentesis (cloudy appearance, high neutrophils, gram-staining and culture..
Mx = ABx e.g. ceftriaxone
Common organisms = E.coli, S.aureus, Klebsiella.
Ix for Budd-Chiari
Doppler USS
Biopsy of steatohepatitis
Fat inclusions and cells are swollen
if alcohol related = mallory bodies
Complications of HBV
Chronic = HCC, Cirrhosis. Acute = Fulminant hepatic necrosis - increasing INR, need transplant!
Autoimmune hepatitis and associated antibodies
Type 1 = most common. ANA (antinuclear antibody) +ve, ASM (anti smooth muscle) +ve.
Type 2 = anti-LKM1, rapidly progressive.
Type 3 = anti-SLA
Differentials for acute pancreatitis
Abdo aorta aneurysm PUD Intestinal obstruction Cholangitis Mesenteric ischaemia
What is TIPS
Transjugular intrahepatic portosystemic shunt.
Shunt form hepatic to portal vein to relieve pressure.
Surgical/endoscopic interventions for a variceal upper GI bleed
band ligation
balloon tamponade
sclerotherapy
injection with adhesives/glue
Criteria for HCC and components of score
CLIP (Cancer of the liver Italian programme)
Scores for Child-Pugh stage, Tumour morphology, Extent/presence of portal vein thrombosis, Serum alpha fetoprotein.
