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)