Liver and friends Flashcards
Types of liver injury? Presentation?
Acute–> liver failure/ recovery
Chronic–> cirrhosis–> liver failure: varices, hepatoma or recovery
Acute= malaise, nausea, anorexia, jaundice
Rarer= confusion, bleeding, liver pain, hypoglycaemia
Chronic= ascites, oedema, haematemesis, malaise, anorexia, wasting, bruising, itching, hepatomegaly, abnormal LFTs
rarer= confusion, jaundice
What serum liver function tests are there? Jaundice types and known as?
Serum bilirubin, albumin, prothrombin time
Serum liver enzymes= alkaline phosphatase, gamma-GT, hepatocellular: transaminases (AST, ALT)
Unconjugated= ‘pre-hepatic’, Gilberts, haemolysis
Conjugated= ‘cholestatic,’
liver disease- ‘hepatic,’
bile duct obstruction ‘post hepatic’
Urine, stools, itching and LFTs in pre-hepatic/ hepatic and post-hepatic jaundice?
Pre-hepatic- urine= normal, stools= normal, no itching, LFTs normal
Hepatic/ post-hepatic- urine= dark, stools= may be pale, itching= maybe, liver tests= abnormal
Features of gallstones? Risk factors? Presentation?
Most form in gallbladder, 1/3 women over 60
70% cholesterol, 30% pigment +/- calcium
Risk factors= female, fat, fertile, most= asymptomatic
Cholecystitis= inflame of gall bladder, cholangitis= inflammation of bile duct
Management of gallstones? Bile duct stones? Other stone types, features and causes? Factors for stones being symptomatic?
Laporoscopic cholecystectomy- removal of gall bladder
Bile acid dissolution therapy= <1/3 success
ERCP w/ sphincterotomy and removal, crushing, stent placement, surgery for larger stones
Pigment stones- small friable and irregular, causes= haemolysis
Cholesterol stones- large, often solitary, causes= female, age, obesity
Mixed- faceted (calcium salts, pigment and cholesterol)
Smoking, parity
What is unconjugated hyperbilirubinaemia? Causes x4?
Pre-hepatic–> haemolytic jaundice–> normal LFT
Overproduction- haemolysis
Impaired hepatic uptake- drugs, right HF
Impaired conjugation: Gilberts syndrome- decreased UDP glucoronyl transferase enzyme, Crigler-Najjar disease- enzyme mutation
Physiological neonatal jaundice- fetal Hb replaced with adult Hb, excrete bilirubin as quickly as an adult
What is conjugated hyperbilirubinaemia? What is cholestatic jaundice? Divided into what?
Increased in conjugated bilirubin- water soluble, dark urine, pale stools
Impaired/ obstructed bile flow- associated with itching and abnormal LFT
Intra-hepatic cholestatis- viruses, pregnancy, cirrhosis, alcohol, drugs–> hepatocellular swelling/ abnormalities of bile excretion
Extra-hepatic cholestasis- bile duct stones, Mirrizi’s syndrome, carcinoma of bile duct or pancreas or PSC–> bile outflow obstruction
Symptoms of jaundice?
Biliary pain, rigors, abdomen swelling, weight loss, past history of biliary disease/ intervention/ malignancy/ heart failure/ blood products, drug history, social history, look for hepatomegaly, splenomegaly or ascites
Investigations for jaundice?
Liver enzymes- very high AST/ ALT suggests liver disease
Biliary obstruction- 90%= dilated intrahepatic bile ducts on US
CT, MRCP, ERCP
Urine- bilirubin is absent in pre-hepatic causes, obstructive= urobilinogen absent
Haematology- FBC, clotting, film, reticulocyte count, Coombs’ test and haptoglobin for haemolysis, malaria parasites
Chem- U&E, LFT, ALT, AST, alk phos, gamma-GT, total protein, albumin, AST>1000 probably viral hepatitis
Micro- blood and other cultures, leptospirosis, hep A, B, C serology
US to see if bile ducts dilated, gallstones, hepatic metastases, pancreatic mass —> treat cause
What is acute cholecystitis? Causes?
Inflammation of the gallbladder
Blockage of cystic duct w/ gallstones (cholelithiasis)–> build-up of bile in gallbladder–> right upper abdominal pain
Conc bile, pressure and bacterial infection- irritates gallbladder wall–> inflammation
May cause continuous epigastric or RUQ pain, vomiting, fever, local peritonism
Main difference from biliary colic= inflammatory component- local peritonism, fever, WCC increase
If moves to CBD- obstructive jaundice and cholangitis may occur
Signs of acute cholecystitis? Tests? Tx?
Murphy’s sign- 2 fingers over RUQ, pt breathes in, causes pain and arrest of inspiration, only +ve if same test in LUQ does not cause pain
Phlegmon may be palpable- RUQ mass of inflamed adherent momentum and bowel
WCC increase, US- thick walled, shrunken GB, peri cholecystic fluid, stones, CBD dilated, plain AXR
Nil by mouth, pain relief, IVI- cefuroxime IV
Laparoscopic cholecystectomy
Open surgery if GB perforation
Elderly/ high risk= ERCP, cholecystectomy
Acalculous= cholecystostomy, give IV amoxicillin and clavulinic acid and IV fluids
Symptoms of chronic cholecystitis? Tests and Tx? If symptoms persist post-op consider what?
Chronic inflammation +/- colic, flatulent dyspepsia, vague abdominal discomfort, distension, nausea, flatulence, fat intolerance
US to image stones and CBD diameter, MRCP to find CBD stones
Cholecystectomy- dilated CBD with stones= ERCP and sphincterotomy before surgery
Hiatus hernia, IBS, peptic ulcer, relapsing pancreatitis, tumour
What is biliary colic used to describe? Gallstones are symptomatic with what? Rx and Tx?
A type of pain related to the gallbladder that occurs when a gallstone transiently obstructs the cystic duct and the gallbladder contracts
With cystic duct obstruction/ passed into CBD, RUQ pain +/- jaundice
Rx= analgesia, rehydrate, NBM, elective laparoscopic cholecystectomy
Diff diagnosis of biliary colic? Other presentations?
Overlap as part of spectrum of gallstone disease, urinalysis, CXR and ECG help exclude other diseases
Obstructive jaundice with CBD stones- LFT worsening, ERCP with sphincterotomy and biliary trawl, then cholecystectomy may be needed, or open surgery
Ascending cholangitis
Gallstone ileus
Pancreatitis
Mucocoele/ empyema- obstructed GB filled with mucus
Silent stones
Mirizzi’s syndrome- stone in GB presses on bile duct causing jaundice
Gallbladder perforation- rare due to dual blood supply
Causes of drug induced liver injury? 3 types?
Acute hepatitis, acute liver failure, paracetamol, idiosynchratic
Hepatocellular= ALT>2 ULN, ALT/ Alk phos> 5
Cholestatic= Alk phos>2 ULN or ratio <2
Mixed= ratio >2, but <5
Direct toxicity and idiosyncratic DILI
Approach to drug induced liver injury?
What did you start recently- contact GP, time course= critical- onset usually 1-12 weeks of starting, earlier= unusual, may be several weeks after stopping
Resolution- 90% within 3 months of stopping, 5-10% prolonged
Inadvertent re-challenge, seen in 6%
Drugs known to cause jaundice? Not involved?
Antibiotics- augmenting, flucloxacillin, erythromycin, septrin, TB drugs
CNS drugs- chlorpromazine, carbamazepine valproate, paroxetine, immunosuppressants, analgesics/ musculoskeletal, PPIs, dietary supplements
Low dose aspirin, NSAIDs other than diclofenac, Beta blockers, HRT, ACEi, thiazides, CCBs
What is given to counteract paracetamol induced fulminant hepatic failure? Supportive to correct what? Poor prognosis indicators?
N acetyl cysteine (NAC)
Coagulation defects, fluid electrolyte and acid base balance, renal failure, hypoglycaemia, encephalopathy
Late presentation, acidosis<7.3 pH, prothrombin time> 70 seconds, serum creatinine> 300micromol/ l, consider liver transplant- 80% mortality otherwise
What does alcoholic liver disease encompass? Chronic consumption results in what? Still functions after what % hepatocytes dead?
Liver manifestations of alcohol overcomsumption, including fatty liver, alcoholic hepatitis and chronic hepatitis w/ liver fibrosis or cirrhosis
Secretion of pro-inflammatory cytokines (tif-alpha, IL6 and IL8), oxidative stress, lipid per oxidation and acetylaldehyde toxicity–> inflammation, apoptosis and eventually fibrosis of liver cells
75%
Changes way liver metabolises/ produces what? Acute alcohol injury causes what and is mediated by what? Injured liver cells may accumulate what? Fat accumulation is known as what within hepatocytes? Large/ small droplet known as what? Associated with and mediated by what?
Fat
Hepatocyte ballooning, by neutrophils
Cytoskeletal protein which appears irregular and red on an H&E stain- Mallory’s hyalin
Steatosis- macrovesicular/ micro vesicular- acute/ chronic liver injury, by lymphocytes
Alcohol and other drugs have most affect on cells with lowest what? Damage results in what? % heavy alcohol drinkers getting serious ALD, % stop and % reduce drinking?
On cells with lowest oxygen and blood supply (zone 3)- fibrosis, with pericellular fibrosis in zone 3 been more common in alcohol-related injury than fibrosis of portal tracts
10-20%, 25% and 25%
Fatty liver can lead to what? S+S of alcoholic hepatitis? Tests?
Alcoholic hepatitis/ cirrhosis + infection–> acute decompensation
Malaise, TPR increase, anorexia, D&V, tender hepatomegaly+/- jaundice, bleeding, ascites
Blood–> WCC increase, platelets decreased, INR increase, AST increase, MCV increase, urea increase
Signs of severe hepatitis? Most need what? Screen for what? Stop what? What vitamin given?
Jaundice, encephalopathy or coagulopathy
Hospitalising; urinary catheter and CVP monitoring may be needed
Infections +/- ascitic fluid tap, treat for SBP
Alcohol consumption- chlordiazepoxide ORAL for withdrawal symptoms
Vit K IV for 3 days, thiamine PO
In alcoholic hepatitis, optimise what? Don’t use low what diets? Daily what tests? Prednisolone for 5d tapered over 3 weeks if Maddrey score above what with what? CI? What else? Prognosis?
Nutrition- use ideal body weight for calculations
Low-protein diets even if encephalopathy
Daily weight, LFT, U&E, INR, Na+ decrease= common, water restriction may make worse
>31 and encephalopathy
Liver transplant
Mild episodes hardly affect mortality, severe= 50% at 30d, 1 yr after admission= 40% dead
What is biliary sepsis/ ascending cholangitis? Gallstone risk factors? Diagnosis based on Charcots triad?
Obstruction of the common bile duct–> bile stasis–> invasion of bacteria from the duodenum e.g. e.coli–> obstructive jaundice
Jaundice, RUQ pain, fever
What is cirrhosis? Signs of this?
Where liver does not function properly due to long-term damage, comes on slowly over months/ years, early on= often no symptoms
Scar tissue replaces normal parenchyma= irreversible
None (just increase LFT)/ decompensated end-stage liver disease
Chronic liver disease- Leukonychia= white nails, Terrys nails- white proximally but distal 1/3 reddened by telangiectasis, clubbing, palmar erythema, hyperdynamic circulation; Dupuytren’s contracture; spider naevi, Xanthelasma, Gynaecomastia, atrophic testes, loss of body hair, parotid enlargement, hepatomegaly
Complications of cirrhosis?
Hepatic failure- coagulopathy (decreased factors 2, 7, 9 and 10 causes increased INR), encephalopathy, hypoalbumineria, sepsis, SBP, hypoglycaemia
Portal hypertension- ascites, splenomegaly, portosystemic shunt including oesophageal varices, caput medusae- enlarged superficial periumbilical veins
HCC- increased risk
Tests for cirrhosis?
Blood= LFT- abnormal/ increased AST, ALT, increased alk phos, increased gamma GT, loss of synthetic function, decreased albumin +/- increased PT/ INR, decreased WCC and platelets= hypersplenism
Liver US- small liver/ hepatomegaly, splenomegaly, focal liver lesion(s), hepatic vein thrombus, reversed flow in portal vein or ascites
MRI= caudate lobe size increased, smaller islands of regenerating nodules
Ascitic tap- fluid sent for urgent MC&S, neutrophils> 250/mm3= SBP
Liver biopsy- clinical diagnosis confirmed
Management of liver cirrhosis?
Good nutrition= vital, alcohol abstinence, avoid NSAIDs, sedatives and opiates, colestyramine helps pruritus, US+/- alpha-fetoprotein every 3-6 months to screen for HCC
PBC= high-dose ursodeoxycholic acid may normalise LFT, penicillamine for Wilson’s disease
Ascites= bed rest, fluid restriction, low-salt diet, PO spironolactone, chart daily weight for weight loss, add furosemide if needs be, therapeutic paracentesis with concomitant albumin infusion may be tried
SBP= cefotaxime/ tazocin for 5d, prophylaxis for high risk
Renal failure
Prognosis and poor prognostic indicators in cirrhosis? Survival from liver transplantation?
5yr survival= 50%, encephalopathy, serum Na+<110mmol/L, serum albumin< 25g/L; increased INR
Only definitive Tx, increases 5yr survival from 20% in end-stage disease–> 70%
Types of liver failure? What is it? What is fulminant liver failure?
Acute hepatic failure- occur suddenly in previously healthy liver, often from decompensation of chronic liver disease= acute-on-chronic hepatic failure
Clinical syndrome resulting from massive necrosis of liver cells leading to severe impairment of liver function
Hyperacute= encephalopathy within 7d of onset of jaundice, acute= within 8-28 days, subacute= within 5-26 weeks
Decreasing risk of cerebral oedema as onset of encephalopathy is increasingly delayed
What is acute liver failure? Causes of chronic liver failure?
Rapid development of hepatocellular dysfunction- coagulopathy and mental state changes specifically in patient without known liver disease
Alcohol, non-alcoholic steatohepatitis (NASH), viral hep- B,C, immune (autoimmune hep, PBC, sclerosing cholangitis), metabolic- haemochromatosis, Wilsons, alpha 1 antitrypsin deficiency, vascular- Budd-Chiari
Investigations for liver failure? Why do these pts ‘go off’? Signs?
Viral serology- hep B surface antigen, hep C antibody, immunology- autoantibodies, coeliac antibodies, Its, biochem- iron, copper studies: caeruloplasmin, 24 hr urine copper, USS/CT/MRI
Constipation, drugs, GI bleed, infection, hypo-naetraemia, kalaemia, glycaemia, alcohol withdrawal, other
Jaundice, hepatic encephalopathy- unable to remove toxins, fetter hepaticus- smells like pear drops, asterisks, constructional apraxia- cannot copy 5-pointed stat,
Tests for liver failure?
FBC, U&E, LFT, clotting, glucose, paracetamol level, hepatitis, CMV and EBV serology, ferritin, alpha-1-antitrypsin, caeruloplasmin, autoantibodies
Blood culture, urine culture, ascitic tap for MC&S of ascites
CXR, abode US, Doppler studies of portal vein
EEG, evoked potentials have limited role
Management of liver failure?
Beware sepsis, hypoglycaemia, GI varices and encephalopathy
20 degrees uptilit in ITU, protect airway with intubation and insert NG tube to avoid aspiration and remove any blood from stomach
Insert urinary and central venous catheters to help assess fluid status
Montior T degree respirations, pulse, BP, pupils, urine output hourly, daily weights
Check FBC, U&E, LFT and INR daily
10% glucose IV, 1L/12h to avoid hypoglycaemia, blood glucose every 1-4h
Treat the cause
Dietary help
Seizures= lorazepam
Haemodialysis, avoid sedatives
PPI
Liver failure Tx complications? Worse prognosis indicators?
Cerebral oedema- 20% mannitol IV ITU
Ascites= restrict fluid, diuretics, weigh daily
Bleeding- vitamin IV for 3 days, platelets, FFP
Infection= ceftriaxone 1-2g/24h IV, not gentamicin
50ml of 50% glucose for hypoglycaemia
Avoid sedatives, head-tilt 20 degrees,, lactulose and regular enemas
Grade 3-4 encephalopathy, age> 40 years, albumin<30g/L, increased INR, drug-induced liver failure, late-onset hepatic failure= worse than fulminant
Renal failure in liver disease due to what? Coma in liver disease due to what? End-stage liver disease represented by what? Risk in long-standing cirrhosis?
Drugs, infection, GI bleeding, myoglobinuria, renal tract obstruction
Hepatic encephalopathy, infection, GI bleed, constipation, hypokalaemia, drugs, hyponatraemia/ hypoglycaemia, intracranial event
Cirrhosis- residual nodules remaining small due to micro nodular can regenerate–> macronodular cirrhosis- replications errors–> neoplasia
Hepatocellular carcinoma
Other consequences of liver dysfunction? Drugs prescribing in liver disease?
Malnutrition, coagulopathy, endocrine changes, vit K deficiency, hypoglycaemia
Analgesia- sensitive to opiaites, sedation- short-acting benzodiazepines, diuretics- excess weight loss, hyponatraemia, hyperkalaemia, renal failure
Antihypertensives- can often stop, avoid ACE inhibitors, aminoglycosides- avoid
Tx of liver disease consequences?
Malnutrition= naso-gastric feeding, variceal bleeding= endoscopic banding, propanolol, terlipressin, encephalopathy= lactulose, ascites/ oedema= salt/ fluid restriction, diuretics, paracentesis Infections= antibiotics
What is viral hepatitis? Causes and symptoms of acute hepatitis? Of chronic hepatitis? Complications?
Acute<6m since onset, chronic>6m since onset
Infectious(viral- Hep A-E, herpes viruses (EBV, CMV, VZV,) non-viral= leptospirosis, toxoplasmosis, coxiella or non infectious- alcohol, drugs, toxins, pregnancy, autoimmune, hereditary metabolic
Can be asymptomatic, malaise, myalgia, GI upset, abdo pain, +/- jaundice, tender hepatomegaly, raised AST, ALT +/- bilirubin
Can be asymptomatic, signs of chronic liver disease, decompensated= jaundice, ascites, low albumin, coagulopathy, encephalopathy
Hepatocellular carcinoma, portal hypertension
What is and transmission of hep A? Incubation period? Management?
Most common, RNA, worldwide, faeco-oral transmission via contaminated food/ water (shellfish, travellers, food handlers)
Short incubation period–> fever, malaise, anorexia, nausea, arthralgia then jaundice, splenomegaly and adenopathy appearing (2-3 weeks,) acute hep only, usually self-limiting, 100% immunity post infection
Supportive, monitor liver function, management of close contacts (HBIG given to within 14 days contact,) primary prevention= vaccine (travellers, work exposure, prevention of secondary cases, lifestyle risk, other liver disease)
What is and transmission of hep E? Presents? Tx?
Small RNA virus, faeco-oral transmission, also zoonotic- pig reservoir, self limiting acute hep, mortality increased in pregnant women
Often asymptomatic, can cause chronic in immunocompromised, serology similar to hep A, HEV RNA= detect chronic infection, vaccine being developed
Supportive, ribavirin, prevention- food hygiene, animal husbandry, vaccine being developed
What is and transmission of hep B? Incubation period? Natural history?
Hepadnavirus-DNA virus, transmission= blood-borne, vertical, highly infectious
>90% clear acute infection, those–> chronic HBV
1-6m, S&S similar to HAV but arthralgia and urticarial (red rash on skin) more common
Immune tolerance phase= high viral replication, highly infective, ALT normal
Immune clearance phase= liver inflammation with high ALT and HBV DNA levels
Inactive HBV carrier phase- HBV DNA levels low, ALT normal and no liver inflammation, HBeAg absent
Reactivation phase- ALT and HBV DNA levels persistently increased–> inflammation on liver biopsy and fibrosis develops
Management of HBV? Tx?
Supportive, monitor liver function, management of contacts w/vaccine and HBIG asap, follow-up HbsAg @6m, primary prevention
Pegylated interferon-alpha-2a SC- immune response, SEs= flu-like, thyroid issues, anaemia, mood disturbance
or nucleotide analogues, tenofovir, entecavir ORAL- inhibits viral replication, high barrier to resistance w/ minimal SEs, need renal monitoring
What is and transmission of hep D? Chronic hep B and D–> what? What is seen with chronic HBV? % response to prolonged course of pegylated interferon-alpha?
Defective RNA virus- need presence of HBV, blood and bodily fluids transmission
With HBV–> increased severity of acute infection
Increased rate of fibrosis progression, Hep D= usually dominant (HBV DNA low, HDV RNA high)
Super-infection(secondary acute hepatitis, increased fulminant risk, increased progression of liver disease)
30%
What is and transmission of hep C? HCV testing? Risk factors for progression?
Flavivirus (RNA), genotypes 1-6, transmission= blood borne
Look for HCV antibody if you have infection exposure, median of 90 days detection, false negatives if acute infection/ immunocompromised
Look for HCV RNA if have current infection or acute/ chronic infection diagnosis
Male, older, higher viral load, alcohol, HIV, HBV
Tx and prevention of hep C?
Pegylated interferon and ribavirin (ORAL)- sustained virological response w/ undetectable HCV RNA for 6m post end of tx–> cure
New direct acting antivirals- protease inhibitors- teleprevir, simeprevir, declatasvir, ledipasvir, sofobusvir
No vaccine, previous infection NOT immunity, screening blood products, lifestyle modification, universal precautions handling bodily fluids
What is autoimmune hepatitis? Classification is by what? Presents and epidemiology?
Inflammatory liver disease- suppressor T-cell defects with autoantibodies directed against hepatocyte surface antigens
By autoantibodies
Young/ middle-aged women, up to 40%= acute, signs of AI disease- fever, malaise, urticarial rash, poly arthritis, pleurisy, pulm infiltration or glomerulonephritis
Remainder= gradual jaundice or asymptomatic and diagnosed with chronic liver failure, amenorrhea= common, tends to attenuate pregnancy
Complications and tests for AI hepatitis? Diagnosis?
Those with cirrhosis/ drug therapy
Serum bilirubin, AST, ALT and alk phos increased, hypergammaglobulinaemia, +ve autoantibodies, anaemia, WCC decreased and platelets decreased indicated hypersplenism
Liver biopsy- mononuclear infiltrate of lymphocytes and plasma cells in portal and periportal areas –> apoptosis, necrosis, chronic disease–> cirrhosis= worst
MRCP- excludes PSC if alk phos disproportionately increased
Based in IgG levels, autoantibodies, histology, some overlap e.g. PSC, PBC and chronic viral hepatitis
Management and prognosis of AI hepatitis?
Immunosuppressant therapy- prednisolone PO for 1 month, decreased by 5mg a month, corticosteroids sometimes stopped after 2 years, azathioprine to maintain remission
Liver transplantation- for decompensated cirrhosis or failure to medical therapy, 10 yr survival= 75%
Doesn’t matter if symptomatic/ asymptomatic, cirrhosis reduces, overlap- AIH-PBC is worse than AIH-AIC
What is primary biliary cirrhosis? What is it marked by? What is cholestasis? Can lead to what?
Autoimmune disease of the liver
By slow progressive destruction of the small bile ducts of the liver by chronic autoimmune granulomatous inflammation- intralobular ducts and Canals of Herine affected early in disease
Ducts damaged- bile and other toxins build up in the liver, damages liver over time
Scarring, fibrosis, portal HTN and cirrhosis
The disease of PBC is what? Staging is done by what system?What is stage 1? Close up view of bile duct branch shows what?
Patchy- all stages may co-exist in same liver, disease may not be detected on initial biopsy
Ludwig system- stage 1= florid duct lesion- dense mixed inflammatory infiltrate, rich in lymphocytes, present within portal tract, centred on bile duct
Epithelium is disrupted and damaged by lymphocytes, destroys bile duct branch eventually- portal tract has hepatic artery and portal branches but no bile duct= ductopaenia
Primary biliary cirrhosis presents as what? Associated diseases? Cause? What are hallmark of PBC? Risk increased by?
Often asymptomatic, alkaline phos increased on routine LFT, lethargy, dry eyes, joint pain, vatical bleeding, liver failure, sleepiness, pruritus
Sjogrens, thyroiditis, scleroderma, CRST, rheumatoid arthritis, lung and coeliac disease
Unknown environmental triggers and genetic predisposition–> loss of immune tolerance to self-mitochondrial proteins
Antimitochondrial antibodies- AMA
+ve family history, many UTIs, smoking, past pregnancy, other AI diseases, increased use of nail polish/ hair dye, typical age= 50 years
Signs and complications of PBC? Tests?
Jaundice, skin pigmentation, Xanthelasma- yellow plaques in eye, xanthomata; hepatosplenomegaly
Those of cirrhosis, osteoporosis common, malabsorption of fat-soluble vitamins due to cholestasis and decrease bilirubin in gut lumen–> osteomalacia and coagulopathy; HCC
Blood- increased alk phos, increased gamma GT, mildly increased AST and ALT, late= increased bilirubin decreased albumin, increased prothrombin time, AMA M2 subtype +ve, Igs increased, TSH and cholesterol increased or normal
US exclude extra hepatic cholestasis
Biopsy not usually needed- look for granulomas around bile ducts +/- cirrhosis
Tx of primary biliary cirrhosis? Prognosis?
Symptomatic- pruritus- colestyramine PO, naltrexone and rifampicin may help, diarrhoea= codeine phosphate e.g. PO, osteoporosis prevention
Fat-soluble vitamin prophylaxis- A D and K, high dose UDCA–> improved enzymes, bilirubin
Regular LFT,US, AFP
Liver trans for end-stage= bili> 100micromol/L
Once jaundice develops, survival< 2 years without transplantation
What is primary sclerosing cholangitis? Can lead to what? Characterised by what histologically?
Disease of bile ducts that causes inflammation and obliterative fibrosis of bile ducts and/ or outside of liver
Impedes flow of bile to intestines–> cirrhosis, LF, strictures +/- gallstones
Early stages= peri-ductal oedema, ‘‘onion ring,’’ lymphocytes, lesions in irregular fashion along ducts, secondary cholangitis with neutrophils within bile ducts may be present