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Sachi Parikh scp21@ic.ac.uk Based off slides from Georgina Hagger Biliary and Liver MedEd Y3 Written Exam Lectures 2023SESSION STRUCTURE Aetiology History Presentation Investigations Management = Gold Standard = High YieldSESSION CONTENT Conditions: Presentations: • Gallstones and biliary colic • Ascites • Acute cholangitis • Change in stool colour • Primary sclerosing cholangitis • Jaundice • Cholecystitis • Melaena • Pruritus • Autoimmune hepatitis • Abdominal mass • Non-alcoholic steatohepatitis • Nausea and vomiting/diarrhoea and • Alcoholic hepatitis constipation • Liver failure • Organomegaly • Cirrhosis • Acute abdominal pain • Ascites • Chronic abdominal pain • Haemochromatosis • Wilson’s disease • Primary biliary cirrhosis/cholangitis • Liver abscess/cyst SBA 1 A 55-year-old woman presents to the ED with a 2-hour history of abdominal pain. She describes this as a colicky pain in the right upper quadrant accompanied by nausea. The pain started after eating lunch. On examination, there are no signs of fever or jaundice. Blood results reveal ALT, AST, ALP and GGT are all within normal range. Amylase and lipase are also within normal range. Given the most likely diagnosis, what is the definitive management for this patient? a) Analgesia b) Do nothing c) Elective laparoscopic cholecystectomy d) Emergency laporoscopic cholecystectomy e) ERCP SBA 1 A 55-year-old woman presents to the ED with a 2-hour history of abdominal pain. She describes this as a colicky pain in the right upper quadrant accompanied by nausea. The pain started after eating lunch. On examination, there are no signs of fever or jaundice. Blood results reveal ALT, AST, ALP and GGT are all within normal range. Amylase and lipase are also within normal range. Given the most likely diagnosis, what is the definitive management for this patient? a) Analgesia b) Do nothing c) Elective laparoscopic cholecystectomy d) Emergency laporoscopic cholecystectomy e) ERCPGallstone Disease Cholelithiasis = gallstone disease Gallstones form due to a supersaturation of bile. 3 main types: - Cholesterol - Pigment - Mixed Risk factors 5 Fs: Fat (obesity) Female Fertile Forty Family history (+ pregnancy, oral contraceptives, haemolytic anaemia, malabsorption)Biliary Colic Gallbladder neck transiently blocked by gallstonesBiliary Colic History Presentation • Right upper quadrant pain • Right upper quadrant Differentials: ➢ Sudden,dull,colicky or epigastric GORD ➢ Typically lasts longer than 30 mins tenderness PUD Pancreatitis ➢ May be precipitated by fatty foods IBD ➢ Pain responds well to analgesia • Nausea/vomiting are commonBiliary Colic Investigations Management • Bloods: • Analgesia ➢ FBC (ie. WCC) and CRP – should be • Lifestyle advice (eg.low-fat diet,exercise) normal as there should beno signs of • Offer elective laparoscopic cholecystectomy inflammation ➢ LFTs – should show normal liver enzymes (AST, ALT,ALP, GGT,bilirubin) ➢ Amylase and lipase – should show normal levels; done to rule out pancreatitis • Abdominal USS – gallstones or sludge present SBA 2 The patient returns to the ED 5 weeks later before her scheduled surgery with severe abdominal pain. The pain is now constant, and she reports fevers. On examination the patient reports pain on inspiration when the abdomen is palpated in RUQ. There are no signs of jaundice. Given the most likely diagnosis, what is the first-line investigation to confirm the diagnosis? a) Abdominal USS b) CT abdo/pelvis c) ERCP d) MRCP e) MRI SBA 2 The patient returns to the ED 5 weeks later before her scheduled surgery with severe abdominal pain. The pain is now constant, and she reports fevers. On examination the patient reports pain on inspiration when the abdomen is palpated in RUQ. There are no signs of jaundice. Given the most likely diagnosis, what is the first-line investigation to confirm the diagnosis? a) Abdominal USS b) CT abdo/pelvis c) ERCP d) MRCP e) MRIAcute Cholecystitis Acute cholecystitis = acute gallbladder inflammation Risk Factors: • Gallstones • Previous episode of biliary pain • Severe illness • Total parenteral nutrition • DiabetesAcute Cholecystitis History • Constant right upper quadrant or epigastric pain • Fever • Lethargy Presentation • Right upper quadrant or epigastric tenderness • Positive Murphy’s sign • If perforated: signs of sepsis/guardingAcute Cholecystitis Investigations • Bloods: ➢ FBC – ↑WCC ➢ ↑CRP ➢ LFTs – AST and ALT, (↑)ALP and bilirubin ➢ amylase and lipase • 1 line = abdominalUSS ➢ Presence of gallstones,pericholecystic fluid,distended gallbladder, thickened gallbladder wall • MRCP ➢ If bile duct dilated but no CBD stones visualisedAcute Cholecystitis Management Complications • Analgesia and • Mirizzi syndrome antibiotics +/- fluid resuscitation • Gallbladder empyema Definitive treatment: • Chronic cholecystitis • 1 line: early laparoscopic • Cholecystoduodenal cholecystectomy fistula nd • 2 line: percutaneous cholecystostomy SBA 3 A 65-year-old man presents to the ED with severe right upper quadrant pain. He reports feeling feverish and quite itchy. On examination, he has yellowing of the sclera, a temperature of 39°C and a blood pressure of 85/60 mmHg. Given the most likely diagnosis, which of the following symptoms/signs indicates a higher severity? a) Fever b) Hypotension c) Jaundice d) Pruritus e) RUQ pain SBA 3 A 65-year-old man presents to the ED with severe right upper quadrant pain. He reports feeling feverish and quite itchy. On examination, he has yellowing of the sclera, a temperature of 39°C and ablood pressure of 85/60 mmHg. Given the most likely diagnosis, which of the following symptoms/signs indicates a higher severity? a) Fever b) Hypotension c) Jaundice d) Pruritus e) RUQ painAcute Cholangitis Acute cholangitis = infection of the biliary tree Most commonly caused by obstruction Risk factors: • >50years of age • Cholelithiasis history • Benign stricture • Malignant stricture • Post-procedure injury • History of primary sclerosing cholangitis – contributes to ¼ of casesAcute Cholangitis History Presentation • Diffuse right upper quadrant pain • Right upper quadrant • Risk factors (recent biliary tract instrumentation, gallstones) tenderness • Pale stools, pruritus • Jaundice • Fever • Tachycardia • Signs of severe disease: hypotension, mental status changes, signs of sepsisAcute Cholangitis Investigations Acutehepatocellular ChronichepatocellularCholestasis damage damage ALT ↑↑ Normal or ↑ Normal or ↑ • Bloods: ➢ FBC – ↑WCC ALP Normal or ↑ Normal or ↑ ↑↑ GGT Normal or ↑ Normal or ↑ ↑↑ ➢ ↑CRP ➢ LFTs – ↑ALP, GGT and bilirubin Bilirubin ↑ or ↑↑ Normal or ↑ ↑↑ ➢ Blood cultures ➢ U&Es - ↑urea in severe illness • ERCP – both diagnostic and therapeutic • Abdominal USS ➢ Showing bile duct dilatation >6mm in diameter, may also show gallstones • MRCP (not required for diagnosis)Acute Cholangitis Management • Obtain IV access – give fluids,take bloods,take cultures • Initiate broad spectrum empirical antibiotics (ie. Tazocin (tazobactam/piperacillin)) Definitive management is biliary decompression with: ERCP complications: • 1 line – ERCP +/- sphincterotomy and stenting • Repeated cholangitis – ifinadequate biliary drainage • 2 line – percutaneous transhepatic • Pancreatitis cholangiography • Bleeding • Perforation SBA 4 A 45-year-old male presents to his GP with a 3-month history of fatigue. He has also noticed itchiness and abdominal discomfort. On examination, he has scleral icterus. He has a past medical history significant for ulcerative colitis. Blood tests reveal elevated bilirubin, ALP and GGT. Tests forauto-antibodies reveal ANCA and ANA positive but AMA negative. What is the most likely diagnosis? a) Autoimmune hepatitis b) Haemochromatosis c) Primary biliary cholangitis d) Primary sclerosing cholangitis e) Wilson’s disease SBA 4 A 45-year-old male presents to his GP with a3-month history of fatigue. He has also noticed itchiness and abdominal discomfort. On examination, he has scleral icterus. He has a past medical history significant forulcerative colitis. Blood tests reveal elevated bilirubin, ALP and GGT. Tests forauto-antibodies reveal ANCA and ANA positivebut AMA negative. What is the most likely diagnosis? a) Autoimmune hepatitis b) Haemochromatosis c) Primary biliary cholangitis d) Primary sclerosing cholangitis e) Wilson’s diseasePrimary Sclerosing Cholangitis Primary sclerosing cholangitis = chronic cholestatic liver condition involving intrahepatic and/or extrahepatic bile duct inflammation and stricture formation Aetiology = unknown Risk factors: • Male sex (2:1 male:female ratio) • IBD • Genetic predispositionPrimary Sclerosing Cholangitis History Presentation • Pruritus • Jaundice • Non-specific RUQ or • Features suggestive of portal hypertension: splenomegaly, epigastric pain ascites, encephalopathy • Fatigue • Fever Differentials: • Secondary sclerosing cholangitis • Weight loss • Immunoglobulin G4 cholangitis • Autoimmune hepatitis • Primary biliary cholangitisPrimary Sclerosing Cholangitis Investigations • Abdominal ultrasound scan – initial investigation • MRCP – preferred to ERCP due to fewer risks • Bloods: ➢ Multifocal intrahepatic and/or extrahepatic strictures ➢ LFTs and dilations ❖ ↑ALP, GGT and bilirubin ➢ Can have a dominant biliary stricture ❖ Mild-to-moderate ↑ in AST & ALT ➢ In patients with severe disease: • ERCP – if MRCPnon-diagnostic ❖ ↓Albumin ❖ FBC: thrombocytopenia, anaemia, leukopenia ❖ ↑PT/INR • IgM elevated, IgG normalor elevated • Auto-antibodies (none specific to PSC) • ANCA, ANA, anti-SMA – can all be +ve • Antimitochondrial antibody (AMA) is - vePrimary Sclerosing Cholangitis Management Complications Early stage: • Liver failure/cirrhosis • Observation and lifestyle changes • Cholangiocarcinoma • Monitor for complications • Bacterial cholangitis • Pruritus relief (eg. Cholestyramine, • Fat-soluble vitamin deficiencies rifampin) (A, D, Eand K) • Colorectal cancer End-stage: • Liver transplantation SBA 5 A 55-year-old male presents to his GP with a 6-month history of fatigue and malaise. He has also experienced abdominal discomfort. He has a past medical history significant for Type 2 diabetes mellitus and hypercholesterolaemia. On average, he drinks two units of alcohol a week. On examination, he has a BMI of 31 kg/m . Liver function tests reveal elevated ALT and AST. Given the most likely diagnosis, what is the gold-standard test for confirmation of diagnosis? a) Abdominal USS b) CT abdomen/pelvis c) Liver biopsy d) MRI abdomen e) OGD SBA 5 A 55-year-old male presents to his GP with a 6-month history of fatigue and malaise. He has also experienced abdominal discomfort. He has a past medical history significant for Type 2 diabetes mellitus and hypercholesterolaemia. On average, he drinks two units of alcohol a week. On examination, he has a BMI of 31 kg/m . Liver function tests reveal elevated ALT and AST. Given the most likely diagnosis, what is the gold-standard test for confirmation of diagnosis? a) Abdominal USS b) CT abdomen/pelvis c) Liver biopsy d) MRI abdomen e) OGD Non-alcoholic Steatohepatitis (NASH)/ Metabolic dysfunction-Associated Steatohepatitis (MASH) NASH/MASH – stage of non- alcoholic fatty liver disease/metabolic dysfunction- associated steatotic liver disease Risk factors: • Obesity • Insulin resistance or diabetes • Dyslipidaemia • Hypertension • Metabolic syndrome • Rapid weight loss • Medications • TPNNAFLD/NASH -> MASLD/MASH History Presentation • Presence of risk factors • Truncal obesity (ie. more fatin abdomen, • ABSENCE of significant alcohol rather than limbs) use • Hepatosplenomegaly • Fatigue and malaise • Signs of chronic liver disease (eg. jaundice, • Right upper quadrant altered mental status, ascites) – if discomfort presenting atadvanced stageNAFLD/NASH -> MASLD/MASH • Liver ultrasound – showing abnormal echotexture Investigations Bloods: • Elastography – can identify fibrosis or cirrhosis • LFTs ➢ ↑ AST, ALT • Liver biopsy – gold-standard ➢ ALP and GGT can be ↑ ➢ Steatosis, inflammation, hepatocyte ballooning, degeneration, fibrosis ➢ Bilirubin ↑ in decompensated cirrhosis • Lipids – ↑ total cholesterol, LDL and triglyceride BUT ↓ HDL • Autoimmune markersand autoantibodies • Viral serology – to rule out viral hepatitis • Metabolic screening including glucoseNAFLD/NASH -> MASLD/MASH Management Without end-stage liver disease: • Diet and exercise • Pioglitazone • Vitamin E • Weight loss pharmacotherapy • Roux-en-Y gastric bypass With end-stage liver disease: • Liver transplantation nd • 2 line = transjugular intrahepatic portosystemic shunt (TIPS) SBA 6 A 67-year-old woman presents to the ED complaining of abdominal pain. In the last few days she has also noticed yellowing of her skin and felt feverish. She reports an alcohol history of 20 units per week for the last 20 years. Given the most likely diagnosis, which of the following signs/symptoms indicates the severe disease? a) Abdominal pain b) Ascites c) Coagulopathy d) Fever e) Varices SBA 6 A 67-year-old woman presents to the ED complaining of abdominal pain. In the last few days she has also noticed yellowing of her skin and felt feverish. She reports an alcohol history of 20 units per week for the last 20 years. Given the most likely diagnosis, which of the following signs/symptoms indicates the severe disease? a) Abdominal pain b) Ascites c) Coagulopathy d) Fever e) VaricesAlcoholic Hepatitis Alcoholic hepatitis = stage of alcoholic liver disease Risk factors: • Prolonged and heavy alcohol consumption • Hepatitis C • Female sex • Cigarette smoking • Obesity • Age >65 yearsAlcoholic Hepatitis History Presentation • Malaise • Increased temperature, pulse rate and respirations • Anorexia • Tender hepatomegaly • Right upper • Jaundice (recent onset) quadrant • Bleeding abdominal • Ascites pain Signs of severe hepatitis: • Diarrhoea and • Jaundice vomiting • Encephalopathy • CoagulopathyAlcoholic Hepatitis Investigations Management • Bloods: • Alcohol abstinence and alcohol withdrawal management if needed (eg. ➢ LFTs chlordiazepoxide) ❖↑ AST , ALT+ AST:ALT ratio >2 • Optimise nutrition and give vitamins (eg. ❖↑ bilirubin vitamin K and thiamine (ie. IV pabrinex if needed)) ➢ ↑ INR/prothrombin time • Infections screen ➢ FBC: ↑ WCC, ↓ platelets • Liver biopsy – would confirm If severe: diagnosis but rarely necessary • Steroids – prednisoloneCirrhosis Most common causes: Cirrhosis = pathological end-stage of • Chronic Hep B and C any chronic liver disease • Alcohol-related liver disease • NAFLD Risk factors: • Alcohol misuse Compensated Decompensated • IV drug use cirrhosis cirrhosis • Unprotected intercourse • Obesity • Country of birthCirrhosisCirrhosis Investigations • Abdominal USS, CT and MRI – can detect signs of advanced cirrhosis and portal hypertension • Bloods: • Elastography – can identify fibrosis or cirrhosis ➢ LFTs • Liver biopsy – most sensitive and specific ❖ ↑ AST, ALT (ALT>AST in all aetiologies but alcohol), ↑ GGT ❖ Bilirubin ↑ in decompensated cirrhosis ➢ ↓ albumin ➢ ↓ platelets ➢ ↑ INR ➢ ↓ Na + • Investigate underlying causeCirrhosis Management • Treatment of underlying cause and prevention ofsuperimposed hepatic insult • Monitor for complications e.g. ascites, varices and encephalopathy Decompensated disease: • Liver transplantation nd • 2 line = transjugular intrahepatic portosystemic shunt Cirrhosis Complications Ascites Portosystemic varices Hepatic encephalopathy Pathological collection of fluid in peritoneal Portal hypertension can result in Build up of ammonia in circulation where it cavity portosystemic varices including oesophageal passes to the brain Present with abdominal distension + shifting varices Astrocytes convert to glutamine which dullness builds up in the brain leading to cerebral Ruptured oesophageal varices: oedema Management: Present as an acute upper GI bleed • Sodium restriction Management Presents with altered mentalstatus, • Diuretic therapy – spironolactone (1 st • ABCDE approach behaviour change, liver flap and eventually line) • Give IV terlipressin and broad-spectrum coma antibiotics Spontaneous bacterial peritonitis = further • Endoscopic banding Management complication • Prophylaxis: propranolol • Oral lactulose +/- rifaximin • TIPSS if resistant varices • Avoid sedativesAcute liver failure Acute liverfailure = rare disease defined by jaundice, coagulopathy, and hepatic encephalopathy in patients with no priorliver disease Risk factors: • Chronic alcohol misuse • Poor nutritional status/fasting • Female sex Causes: • Pregnancy • Paracetamol overdose • Drug-induced liver injury • Chronic hep B • Acute hepatitisB • Chronic pain and narcotic use • Herbal and dietary supplement • Autoimmune hepatitis hepatotoxicity • Ischaemic hepatitis • Acute hepatitisAAcute liver failure History Presentation • Presence of risk factors • Jaundice • Hepatotoxic medication use e.g. • Signs of hepatic encephalopathy paracetamol, herbal supplement • Hepatomegaly (acute viral) • No history of chronic liver disease • Absence of stigmata of chronic liver • Abdominal pain disease • Malaise • Signs of cerebral oedema • Nausea and vomiting • Depression or suicidal ideationAcute liver failure Investigations Management • Bloods: • Intensive care management • LFTs • Mandatory after development of • ↑ AST, ALT, ALP and bilirubin encephalopathy (requires neurology • ↑ INR (>1.5) status monitoring) • U&Es • ABG and lactate • Monitoring of glucose,electrolytes and cultures • Paracetamol levels • Assessment for liver transplantation • Viral serology • Autoimmune hepatitis markers • Aetiology specific treatment (eg. acetylcysteine for paracetamol overdose) SBA 7 A 45-year-old woman presentsto her GP complaining of a change to her skin pigmentation. She has noticed over the last 2months a yellowingof her skin. She has also felt more tired than usual and noticed some pain in the joints in her hands. She has a past medical history significant for Graves’ disease. LFTs show raised AST, ALT,bilirubin, ALPand GGT. Given the most likely diagnosis, which autoantibody is theleast likely to be positive? a) Anti-LKM antibodies b) Anti-mitochondrial antibodies c) Anti-nuclear antibodies d) Anti-smooth muscle antibodies e) Anti-soluble liver antigens or liver/pancreas antibodies SBA 7 A 45-year-old woman presentsto her GPcomplaining of a change to her skin pigmentation. She has noticed over the last 2 months a yellowing of her skin. She has also felt more tired than usual and noticed some pain in the jointsin her hands. She has a past medical history significant for Graves’disease. LFTs show raised AST,ALT, bilirubin, ALP and GGT. Given the most likely diagnosis, which autoantibody is the leastlikely to bepositive? a) Anti-LKM antibodies b) Anti-mitochondrial antibodies c) Anti-nuclear antibodies d) Anti-smooth muscle antibodies e) Anti-soluble liver antigens or liver/pancreas antibodiesAutoimmune hepatitis Autoimmune hepatitis = chronic inflammatory disease characterized by presence of auto-antibodies, inflammatory changes on histology and response to immunosuppressive treatment Risk factors: • Female sex • Genetic predisposition • Immune dysregulationAutoimmune hepatitis History Presentation • Presence of risk factors • Hepatomegaly • Fatigue • Jaundice • Malaise • Signs of advanced chronic liver disease • Anorexia • Abdominal discomfort • Arthralgia (small joints) • Nausea • FeverAutoimmune hepatitis Investigations • Abdominal USS – check for extra-hepatic • Bloods: biliary obstruction and complications of chronic liver disease • LFTs • ↑ AST, ALT, ALP, GGT and bilirubin • Liver biopsy – essential forestablishing diagnosis and evaluating severity • ↓ albumin • Portal mononuclear and plasma cell infiltrate • ↑ IgG • ↑ PT • Autoantibodies: • Type 1: ANA, SMAs, anti-SLA/LP, anti- ASGP-R • Type 2: anti-LKM-1, anti-SLA/LPAutoimmune hepatitis Management Acute severe disease Active disease • High dose corticosteroid e.g. prednisolone, If treatment expected to be <6 months methylprednisolone • Corticosteroid monotherapy • If not improvingemergency liver If treatment expected to be >6 months transplant evaluation • Corticosteroid + immunosuppressant (e.g. azathioprine) If ongoing with decompensated disease then evaluate for liver transplant (+ high-dose corticosteroid monotherapy) SBA 8 A 70-year-old woman presentsto her GP complaining of increased itchiness. She has also felt more tired in recent months and has noticed her eyes and mouth are quite dry.On examination, these is smooth-edged, non-tender hepatomegaly. She has a past medical history significant for rheumatoid arthritis. LFTs show a raised ALPand GGT. Autoantibody tests are positive for anti-mitochondrial antibody. Given the most likely diagnosis, which of the following treatments would prolong survival? a) Analgesia b) Colestyramine c) Methylprednisolone d) Rifampin e) Ursodeoxycholic acid SBA 8 A 70-year-old woman presentsto her GPcomplaining of increased itchiness. She has also felt more tired in recent months and has noticed her eyes and mouth are quite dry. On examination, these is smooth-edged, non-tender hepatomegaly. She has a past medical history significant for rheumatoid arthritis. LFTs show a raised ALP and GGT. Autoantibody testsare positive for anti-mitochondrial antibody. Given the most likely diagnosis, which of the following treatments would prolong survival? a) Analgesia b) Colestyramine c) Methylprednisolone d) Rifampin e) Ursodeoxycholic acidPrimary biliary cholangitis PBC = chronic inflammatory disease of small intrahepatic bile ducts Risk factors: • Female sex (F:M ratio = 10:1) • Genetic predisposition • Immune dysregulationPrimary biliary cholangitis History Presentation • Presence of risk factors • Hepatomegaly (smooth-edged, non- • PMHx and FHx of autoimmune disease tender, slight enlargement) • PMHx of hypercholesterolaemia • Signs of advanced chronic liver disease • Pruritus • Fatigue • Dry eyes and mouth • Abdominal discomfort • Sleep disturbancePrimary biliary cholangitis Investigations Management • Bloods: • Bile acid analogue – ursodeoxycholic • LFTs acid (+ obeticholic acid if • ↑ ALP and GGT unresponsive) • ↑ bilirubin (in advanced disease) • If pruritic: bile acid sequestrant e.g. • ⟷ AST and ALT colestyramine • Lifestyle support • Anti-mitochondrial antibodies • If end-stage liver disease or refractory • Abdominal USS – exclude obstructive duct pruritus – liver transplant lesionsPBC vs PSC SBA 9 A 20-year-old man is brought to the ED by his father. The father reportsthat over recent weeks the patient has been behavingoddly and seems low in mood.In the last few days, he has developed difficulties with walking and has slurred speech. On examination,the patient has a tremor and dysdiadochokinesia. In addition,he has scleral icterus. Further examination of his eyes with a slit-lamp reveals gold-brown pigment surroundingthe cornea. Given the most likely diagnosis, what isthe inheritance pattern of this condition? a) Autosomal dominant b) Autosomal recessive c) Mitochondrial d) X-linked dominant e) X-linked recessive SBA 9 A 20-year-old man is brought to the ED by his father. The father reportsthat over recent weeks the patient has been behavingoddly and seems low in mood. In the last few days, he has developed difficulties with walking and has slurred speech. On examination,the patient has a tremor and dysdiadochokinesia. In addition,he has scleral icterus. Further examination of his eyes with a slit-lamp reveals gold-brown pigment surroundingthe cornea. Given the most likely diagnosis, what isthe inheritance pattern of this condition? a) Autosomal dominant b) Autosomalrecessive c) Mitochondrial d) X-linked dominant e) X-linked recessiveWilson’s disease Wilson’s= autosomal recessive disorder of copper accumulation Risk factors: • ATP7B gene mutation • FHx of Wilson’sWilson’s diseaseWilson’s disease Investigations Management • Bloods: • Oral chelation therapy e.g. • LFTs - ↑ AST + ALT penicillamine • ↓ serum caeruloplasmin • Zinc • ↑ 24hr urinary copper • Dietary restriction of copper • Slit lamp examination – Kasyer-Fleischer rings • If deteriorating hepatic failure – liver • Liver biopsy – copper measurement transplant • MRI brain • DNA testingfor ATP7B mutationHaemochromatosis Haemochromatosis = autosomal recessive disorder of dysregulated dietary iron absorption and macrophage iron release Risk factors: • Middle age • Male sex • White ancestry • FHx • Supplemental iron Complications: Cirrhosis Hepatocellular carcinoma Arthropathy Diabetes Heart diseaseHaemochromatosis History Presentation • Presence of risk factors • Hepatomegaly • Fatigue • Skin pigmentation (bronzingof skin) • Weakness • Congestive heart failure • Lethargy • Arthralgias • PMHx - diabetes mellitus • Impotence in males and loss of libidoHaemochromatosis Investigations Management • Bloods: • Stage 0 and 1 – observation and • ↑ serum transferrin saturation (>45%) • ↑ serum ferritin follow-up, lifestyle changes,Hep A and • ↑ AST/ALT B vaccination • ↑ fastingglucose • Stages 2, 3 and 4 • ↓ testosterone, LH and FSH • 1 line = phlebotomy/venesection • (+ lifestyle + vaccinations) • Liver biopsy – measure of liver iron • 2 line = iron chelation therapy content and damage (MRI is non-invasive e.g. deferasirox alternative) • EchocardiogramLiver abscess: Summary slide Aetiology: Examination: Investigations: Amoebic abscess: FBC - ↑ WCC Cause = Entamoeba histolytica Typically, from RUQ tenderness LFTs - ↑ ALP (+ALT, bilirubin) polymicrobial bacterial +/- hepatomegaly Similar presentationtoother infection Blood and fluid cultures abscesses but Hx of recent travel Causes = cholecystitis, to endemic region cholangitis, diverticulitis, USS – poorly-defined lesions with appendicitis, septicaemia hyper- and hypo-echoic areas Blood andstool samples will have E. histolytica antibodies CT w/ contrast – similar to USS Management = antibiotics alone History: Management: Complications: ✶ Fever ✶ Abscess rupture ✶ Fluid resuscitation ✶ Rigors ✶ Antibiotic therapy ✶ Image-guided aspiration of abscess (+/- catheter ✶ Abdominal pain drainage) + bloating, nausea, + treat underlying cause anorexiaLiver cysts: Summary slide Aetiology: Examination: Investigations: Hepatomegaly LFTs – typically normal Simple cysts Polycystic liver disease (≥20 cysts, Differentials: ≥1cm in size) USS – shows cysts Cystic neoplasms Hydatid cysts CT w/ contrast if cystic neoplasm suspected History: Management: Complications: ✶ Polycystic liver disease can result in ✶ Normally ✶ Simple cysts – most require nointerventionjust monitoring, cirrhosis and portal hypertension asymptomatic symptomatic US-guided aspiration or laparoscopic de-roofing ✶ Pain on increased cyst ✶ Polycystic liver disease – asymptomatic disease can just be monitored, US-guided aspiration may provide pain relief but size laparoscopic de-roofingpreferredfor symptomatic Canalso experience ✶ Cystic neoplasms – aspiration or biopsy avoided to prevent nausea and early peritoneal seedingof malignancy satiety and anorexia ✶ Liver lobe resectiontreatment of choice w/ diagnosis confirmation onFeedback