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

• • Child-Pugh class A to C:

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:

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)