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27 Cards in this Set
- Front
- Back
- 3rd side (hint)
Causes of cirrhosis |
- alcohol abuse - chronic hepatitis C (or B) - NAFLD/NASH (more severe) - genetic: Wilson's disease, alpha-1 antitrypsin deficiency (supportive), hereditary haemochromastosis, cystic fibrosis - infection: hepatitis, CMV, EBV - autoimmune: autoimmune hepatitis, primary biliary cirrhosis, primary sclerosing cholangitis - drugs: methotrexate, amiodarone, methyldopa, isoniazid - neoplasm: HCC, mets - vascular: Budd-Chiari malformation, RHF, constrictive pericarditis |
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Clinical features of chronic liver disease |
- hands: clubbing, leukonychia (decreased albumin), Terry's nails, palmar erythema, Duputren's contracture
- face: conjunctival pallor, xanthelasma (PBC), parotid enlargement - trunk: spider naevi, gynaecomastia, loss of 2o sexual hair - abdomen: striae, hepatomegaly (may be small in late disease), splenomegaly, caput medusae, testicular atrophy |
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Complications of chronic liver disease |
- decompensation (hepatic failure): jaundice, encephalopathy, oedema & ascites (hypoalbuminaemia), bruising (coagulopathy, vitamin K, platelets, FFP, blood), hypoglycaemia - spontaneous bacterial peritonitis - portal HTN: SAVE, splenomegaly, ascites (fluid & salt restrict, spirinolactone, furesemide, ascitic tap, daily weight, TIPPS), varices (OGD screening & banding), encephalopathy (lactulose, enema, rifaximin, avoid sedatives) - increased risk of HCC |
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Identifying the cause of cirrhosis |
- alcohol: increased MCV, increased GGT - NASH: hyperlipidaemia, increased glucose - infection: hepatitis, CMV & EBV serology - hereditary haemochromatosis: ferritin, iron, decreased TIBC - Wilson's disease: decreased copper & caeruloplasmin, liver biopsy - alpha-1 antitrypsin: levels decreased in serum - autoimmune hepatitis: smooth muscle antigen (SMA), anti-liver kidney microsomal, anti-nuclear antibodies (anti-LKM), IgG - PBC: antimitochondrial antibodies (ANA), IgM - PSC: antineutrophil cytoplasmic antibody (ANCA), ANA - HCC: alpha-feto protein (AFP), US, liver biopsy - SBP: ascitic tap with >250mm3 of PMN |
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Classification of cirrhosis |
Child-Pugh grading predicts risk of bleeding, mortality & need for treatment - albumin - bilirubin - clotting - distension: ascites - encephalopathy * >8 is significant risk of variceal bleeding |
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Definition of spontaneous bacterial peritonitis (SBP) |
An acute bacterial infection of ascitic fluid in those with cirrhosis in which no source is generally identified
- complicated by hepatorenal syndrome in 30% |
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Aetiology of SBP |
Ascitic PMN >250mm3, culture - aerobic gram negative: e. coli, klebsiella - aerobic gram positives: strep |
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Treatment of SBP |
- tazocin/cefotaxime, until sensitivities are known - ciprofloxacin prevents high recurrence |
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Definition of hepatorenal syndrome |
The development of renal failure in those with advanced liver disease - IV albumin, terlipressin |
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Managing the causes of chronic liver disease |
- general: good nutrition, alcohol abstinence (baclofen for cravings), cholestyramine for pruritis), screen for varices & HCC - HCV: interferon-alpha - PNC: ursodeoxycholic acid - Wilson's disease: penicillinamine |
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Definition of metabolic syndrome (NAFLD/NASH) |
Central obesity (increased waist circumference) & 2 of: - increased triglycerides - decreased HDL - HTN - hyperglycaemia: DM, impaired glucose tolerance/fasting glucose |
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Definition of Budd-Chiari malformation |
Obstruction of the hepatic vein which leads to ischaemia & hepatocyte damage causing liver failure/insidious cirrhosis - hypercoagulable states: myeloproliferative disorders - local tumour: HCC - congenital |
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Definition of hereditary haemochromatosis |
An inherited multisystem disorder resulting from abnormal iron metabolism that causes increased intestinal iron absorption and deposition in multiple organs |
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Clinical features of hereditary haemochromatosis |
Iron MEALS - myocardial: dilated cardiomyopathy, arrhythmias - endocrine: DM (pancreas), hypogonadism/infertility (pituitary), hypocalcaemia/osteoporosis (parathyroid gland) - arthritis: 2nd/3rd MCP, knees, shoulders - liver: chronic liver disease, cirrhosis, HCC, hepatomegaly - skin: slate grey discolouration |
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Treatment of hereditary haemochromatosis |
- iron removal: venesection (HCT <0.5), desferrioxamine is 2nd line - general: monitor DM, low iron diet - screening: serum ferritin & genotype - transplant: in cirrhosis |
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Definition of Wilson's disease |
Mutation of copper transporting ATPase that impairs hepatocyte incorporation of copper into caeruloplasmin & excretion into bile which leads to accumulation of copper in the liver and other organs |
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Clinical features of Wilson's disease |
CLANKAH - cornea: Kayser-Fleisher rings (70%) - liver disease - arthritis: chondrocalcinosis, osteoporosis - neurology: parkinsonism, spasticity, dysarthria, dysphagia, ataxia, depression, dementia, psychosis - kidney: Fanconi syndrome (excess excretion, osteomalacia) - abortions - haemolytic anaemia: Coombs negative |
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Management of Wilson's disease |
- diet: avoid high copper foods (liver, chocolate, nuts) - penicillamine: Cu chelator, lifelong - liver transplant - screen siblings |
4 |
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Definition of primary biliary cirrhosis (PBC) |
Cirrhosis arising from bile duct destruction due to chronic granulomatous inflammation (may have autoimmune component) |
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Clinical features of PBC |
PPBBCCS - pruritis & fatigue - pigmentation of the face - bones: osteoporosis, osteomalacia - big organs: hepatosplenomegaly - cirrhosis & coagulopathy - cholesterol: xanthelasma, xanthoma - steatorrhoea *may be asymptomatic, jaundice is a late sign(survical <2yrs from this point) |
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Associated diseases in PBC |
- thyroid disease - RA, Sjogren's, scleroderma - coeliac disease - membranous glomerulonephritis |
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Treatment of PBC |
- cholestyramine: pruritis - codeine phosphate: diarrhoea - bisphosphonates: osteoporosis - ADEK vitamins - liver transplant |
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Definition of primary sclerosis cholangitis (PSC) |
Inflammation, fibrosis & strictures form in the intra- & extrahepatic ducts leading to chronic biliary obstruction and cirrhosis |
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Clinical features of PSC |
- may be asymptomatic
- jaundice: pale stools, dark urine - pruritis - abdominal pain - hepatosplenomegaly |
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Complications of PSC |
- bacterial cholangitis - cholangiocarcinoma - colorectal cancer |
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Associated diseases in PSC |
- UC: 3% have PSC, while >80 with PSC have UC/Crohn's - Crohn's - autoimmune hepatitis -HIV |
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Treatment of PSC |
- same as PBC - ABx for cholangitis - endoscopic stenting for dominant strictures - cancer screening - liver transplant: recurrence in 30% |
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