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13.01.16 Liver teaching

Gilbert's syndrome
  • Most common hereditary cause of increased bilirubin
    • Found in up to 5% of the population
  • Major characteristic is jaundice, caused by hyperbilirubinemia
  • Caused by reduced activity glucuronyltransferase, which conjugates bilirubin (and a few other lipophilic molecules)
  • Can manifest in stressful/fasting conditions

Autoimmune liver disease
  • Diseases:
    • Autoimmune hepatitis
    • Primary biliary cirrhosis
    • Primary sclerosing cholangitis
  • Characteristics:
    • Chronic diseases
    • Long natural course
    • Symptoms when present can be challenging to manage
  • Location:
    • Autoimmune hepatitis is a parenchymal disease
    • Primary biliary cirrhosis and primary sclerosing cholangitis are biliary diseases
  • Management:
    • Primary biliary cirrhosis: Ursodeoxycholic acid
    • Autoimmune hepatitis: Prednisolone and azathioprine
    • Primary sclerosing cholangitis: Lacks effective medical interventions; transplantation when indicated is highly successful



Budd-Chiari
  • CRUCIAL TO EXCLUDE!
    • e.g. Young pt with abdo. pain an jaundice..
  • Presents with classical triad:
    • Abdominal pain
    • Ascites
    • Hepatomegaly
  • Syndrome can be fulminant, acute, chronic, or asymptomatic
  • Causes
    • The cause cannot be found in about half of the patients
    • Primary (75%): thrombosis of the hepatic vein
      • Polycythemia vera
      • Pregnancy
      • Post partum state
      • Use of oral contraceptive
      • Paroxysmal nocturnal hemoglobinuria
      • Hepatocellular carcinoma
    • Secondary (25%): compression of the hepatic vein by an outside structure (e.g. a tumor)

Decompensated chronic liver disease
  • Primary cause:
    • Alcohol
    • NAFLD
      • Diabetes
      • Obesity
      • Dyslipidaemia
      • Metabolic syndrome
    • HepC or B
    • Autoimmune hepatitis
    • Drugs
      • MTX
      • Amiodarone
    • Alpha1 antitrypsin deficiency
    • Wilson's
    • Hereditary haeochromatosis
    • Biliary disease
      • PSC
      • PBC
  • Examination
    • Jaundice
    • Encephalopathy
    • Clubbing
    • Hair loss
    • Gynaecomastia
    • Palmar erythaema
    • Testicular atrophy
    • Caput medusa
    • Spider naevae
    • Dupuytren's
    • Ascites
  • Causes of decompensation
    • Alcohol
    • Secondary insult to liver (e.g. viral)
    • Drugs
    • Infection elsewhere
    • Reduced blood supply
    • Ammonia
      • High-protein feed
    • End-stage (spontaneous)
  • Features of decompensation:
    • Portal hypertension
      • Variceal bleed etc
    • Bruising/coagulopathy
    • Ascites
    • Encephalopathy
    • Jaundice
    • Malnutrition
    • Hepatoma



The Model for End-Stage Liver Disease (MELD) Score
  • For assessing the severity of chronic liver disease
  • MELD = 3.78[Ln serum bilirubin (mg/dL)] + 11.2[Ln INR] + 9.57[Ln serum creatinine (mg/dL)] + 6.43
  • 3 month mortality:
    • 40 or more — 71.3% mortality
    • 30–39 — 52.6% mortality
    • 20–29 — 19.6% mortality
    • 10–19 — 6.0% mortality
    • <9 — 1.9% mortality

Child-Pugh score
  • Mnemonic
    • A: Ascites
    • B: Bilirubin
    • C: Clotting
    • D: Decreased albumin
    • E: Encephalopathy
  • Used to assess the prognosis of chronic liver disease, mainly cirrhosis
Measure 1 point 2 points 3 points
Total bilirubin, μmol/l (mg/dl) <34 (<2) 34-50 (2-3) >50 (>3)
Serum albumin, g/l >35 28-35 <28
PT INR <1.7 1.71-2.30 > 2.30
Ascites None Mild Moderate to Severe
Hepatic encephalopathy None Grade I-II (or suppressed with medication) Grade III-IV (or refractory)
  • Child-Pugh class A to C:
Points Class One year survival Two year survival
5-6 A 100% 85%
7-9 B 81% 57%
10-15 C 45% 35%



A little bit of IBD too
  • Acute UC
    • IV corticosteroids
    • 5-ASA
    • Cyclosporin / infliximab if refractory
    • Surgical opinion early - but only 10% need surgery
    • Daily AXR
      • Perforation
      • Dilatation
    • Don't forget stool cultures!
      • Increased risk of infection in IBD
  • Amoebic colitis looks a lot like Crohn's
    • Be careful before you start steroids!
  • Pseudomembranous colitis
    • Characterized by diarrhea, abdominal pain, and fever
    • Often caused by C. diff
    • Pseudomenranous exudate on walls of colon
  • Topical treatment in UC
    • Suppositories for anus/rectum
    • Enema can treat up to splenic flexure
  • Truelove and Witts criteria:
Variable Mild disease Severe disease Fulminant disease
Stools/d <4 >6 >10
Blood in stool Intermittent Frequent Continuous
Temperature Normal >37.5 >37.5
Pulse Normal >90 >90
Hemoglobin Normal <75% of normal Transfusion required
ESR <30 >30 >30
Radiologic features ---- Air

Edematous wall

Thumbprinting

Dilatation
Clinical signs ---- Abd. tenderness Abd. distention and tenderness


Notes
  • Hepatitis incubation
    • A/E short - 2 weeks
    • B long - Weeks-months
  • Alcohol => Raised GGT + bili, but normal-ish AST/ALT
  • A good drug history is essential in liver disease
    • May be delayed effect (weeks/months)
    • NSAIDs, paracetamol, anti-TB, antibiotics....
  • Antimitochondrial antibody + cholestasis = Primary biliary cirrhosis
  • Benign intrahepatic cholestasis in pregnancy
  • Fatty liver
    • Present in 5-10% of general population
    • Causes only a slight increase in ALT/AST
  • Ischaemic hepatitis / Shock liver
    • => High ALT/AST
  • Babies are CMV carriers
    • Nappy changing etc
  • PSC => Cholangiocarcinoma (=> monitoring)
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