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)