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27 Cards in this Set

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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

9

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

4

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

4

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

11

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

5

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%

Aetiology of SBP

Ascitic PMN >250mm3, culture


- aerobic gram negative: e. coli, klebsiella


- aerobic gram positives: strep

3

Treatment of SBP

- tazocin/cefotaxime, until sensitivities are known


- ciprofloxacin prevents high recurrence

2

Definition of hepatorenal syndrome

The development of renal failure in those with advanced liver disease


- IV albumin, terlipressin

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

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

4

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

Definition of hereditary haemochromatosis

An inherited multisystem disorder resulting from abnormal iron metabolism that causes increased intestinal iron absorption and deposition in multiple organs

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

5

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

4

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

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

7

Management of Wilson's disease

- diet: avoid high copper foods (liver, chocolate, nuts)


- penicillamine: Cu chelator, lifelong


- liver transplant


- screen siblings

4

Definition of primary biliary cirrhosis (PBC)

Cirrhosis arising from bile duct destruction due to chronic granulomatous inflammation (may have autoimmune component)

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)

7

Associated diseases in PBC

- thyroid disease


- RA, Sjogren's, scleroderma


- coeliac disease


- membranous glomerulonephritis

4

Treatment of PBC

- cholestyramine: pruritis


- codeine phosphate: diarrhoea


- bisphosphonates: osteoporosis


- ADEK vitamins


- liver transplant



5

Definition of primary sclerosis cholangitis (PSC)

Inflammation, fibrosis & strictures form in the intra- & extrahepatic ducts leading to chronic biliary obstruction and cirrhosis

Clinical features of PSC

- may be asymptomatic
- jaundice: pale stools, dark urine
- pruritis
- abdominal pain
- hepatosplenomegaly

5

Complications of PSC

- bacterial cholangitis


- cholangiocarcinoma


- colorectal cancer

3

Associated diseases in PSC

- UC: 3% have PSC, while >80 with PSC have UC/Crohn's


- Crohn's


- autoimmune hepatitis


-HIV

4

Treatment of PSC

- same as PBC


- ABx for cholangitis


- endoscopic stenting for dominant strictures


- cancer screening


- liver transplant: recurrence in 30%

5