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General Medicine > Hepatology > Flashcards

Hepatology Flashcards

1
Q

Abnormalities in which LFTs indicate hepatocellular disease?

A

Alanine aminotransferase (ALT)
Aspartate aminotransferase (AST)

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2
Q

Abnormalities in which LFTs indicate cholestatic disease?

A

Alkaline phosphatase (ALP)
Gamma glutamyl transferase (GGT)

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3
Q

Abnormalities in which LFTs indicate a problem with synthetic function?

A

Albumin
Bilirubin
PT (INR)

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4
Q

Why is there a relative deficiency of ALT compared to AST in alcoholic liver disease

A

ALT is pyridoxine dependent and alcoholic patients are pyridoxine deplete

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5
Q

Where are ALT and AST found in the body?

A

ALT is liver specific
AST is found in the liver, myocardium, skeletal muscle, kidneys, brain, pancreas, erythrocytes

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6
Q

What do elevated ALP indicate

A

ALP is found in liver, bone, placenta and thus raised levels can indicate cholestatic liver disease, accelerated growth in puberty, Pagets disease, osteoblastic metastases e.g., prostate cancer, pregnancy

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7
Q

What do elevated GGT levels indicate

A

GGT is specific to the liver so can help confirm that a raised ALP is due to liver disease
Also indicates alcohol abuse or use of certain medications

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8
Q

What does an elevated PT suggest

A

Prolonged prothrombin time/INR –> increased risk of bleeding
- Lack of liver synthetic function (vitamin K dependent clotting factors)
- Vitamin K deficiency (malnutrition, malabsorption 2˚ to pancreatic insufficiency/biliary disease)
- Vitamin K inhibition due to anticoagulation e.g., warfarin

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9
Q

How to differentiate between vitamin K deficiency or liver disease as a cause of prolonged PT

A

Reversal of coagulopathy with 10mg of vitamin K suggests that PT elongation is not due to synthetic problem
Other synthetic tests will be abnormal in liver disease and pt is often hypoglycaemic

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10
Q

Risk factors for viral hepatitis

A

IVDU
Sexual behaviour esp. MSM
Migration from high prevalence area
Blood transfusion prior to routine screening/in country without routine screening

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11
Q

What simple features on exam may suggest alcoholism

A

Dupuytren’s contracture
Partotidomegaly

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12
Q

What does acanthosis nigricans suggest

A

Insulin resistance
Consider metabolic syndrome, fatty liver, T2DM, PCOS

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13
Q

An AST/ALT ratio above 2 suggests

A

Alcoholic liver disease

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14
Q

Transaminases of > 1000 U/L (very high) suggest

A

Acute viral pancreatitis
Paracetamol toxicity
Ischaemic hepatopathy
Acute biliary obstruction
Medications/drug induced liver injury (DLI)

Transaminases > 500 are practically never due to alcohol alone - alternative cause should be sought

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15
Q

Symptoms suggesting chronic liver disease

A

May be no symptoms
Abdominal/RUQ pain, anorexia, nausea, weight loss
Malaise, fatigue, myalgia, fever
Pruritus, dark urine, pale stools, jaundice
Bleeding

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16
Q

Signs suggestive of chronic liver disease

A

Hepatocellular dysfunction: spider naevi, palmar erythema, leukonychia, gynaecomastia, testicular atrophy, body hair loss, hepatomegaly
Portal HTN: ascites, peripheral oedema, hepatomegaly, splenomegaly, caput medusae, petechiae, variceal bleeding
Poor synthetic function: ecchymoses, peripheral oedema
End stage liver disease: progressive severe fatigue, encephalopathy (asterixis, fetor coma)

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17
Q

Constant RUQ (or epigastric) pain suggests

A

Liver disease e.g., hepatitis

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18
Q

Severe, episodic RUQ (or epigastric) pain suggests

A

Biliary disease

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19
Q

How does celiac disease affect LFTs

A

Moderate ALT and ALP elevation wth normal bilirubin

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20
Q

Explain bilirubin metabolism

A

Haem from RBCs is degraded by MOs –> biliverdin –> biliverdin reductase –> UCB –> binds albumin and moves to hepatocytes –> conjugated in 2 step process in ER by addition of a glucuronic acid molecule in each step catalysed by UDP-glucuronosyl transferase –> CB is then secreted into bile into SI –> converted by intestinal bacteria to urobilinogen 80% of which is excreted in faeces as stercobilin and 20% is not –> 10% of the remaining urobilinogen is exreted as urobilin by kidneys and 90% returns to liver via enterohepatic circulation

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21
Q

3 phases of bilirubin metabolism

A

Uptake
Conjugation
Excretion

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22
Q

When does jaundice occur

A

Normal levels of bilirubin = ~ 25umol/L (conjugated up to 5umol/L)
Jaundice occurs ~ 50umol/L

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23
Q

Common causes of hepatitis

A

HAV, HBV, HCV
Alcohol liver disease
NAFLD
Ischaemic hepatopathy
Drug induced hepatitis
Autoimmune hepatitis
Haemochromatosis, a1at deficiency, Wilsons disease

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24
Q

Common causes of cholestasis

A

Primary sclerosing cholangitis
Primary biliary cholangitis/cirrhosis
Common duct obstruction e.g., tumour, stone (choledocolithiasis), stricture
Infiltration e.g., maligancy, amyloid, sarcoid
Drug induced cholestasis

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25
Q

Assessment of jaundice/hyperbilirubinaenia

A

Clinical picture e.g., pruritus, pale stool, dark urine suggests intrahepatic/posthepatic cholestasis; hepatomegaly, RUQ pain, nausea, risk factors for hepatitis, fever suggests viral hepatitis etc.

FBC (leukocytosis may suggest infection e.g., cholangitis, anaemia may suggest haemolysis, abnormal mononuclear cells suggest EBV –> perform monospot, leucopaenia often occurs in viral hepatitis)
LFTs (hepatitis -> high AST and ALT vs extrahepatic obstruction –> high ALP and GGT)
- perform fractionation of bilirubin to get proportion of direct and indirect bilirubin
Viral markers (HAV, HBV, HCV, CMV, EBV, HIV)

USS to assess size of bile ducts (dilated in extrahepatic obstruction), level of obstruction, cause of obstruction
- can further Ix obstruction with MRCP or FNA for biopsy and pathological Dx

Consider autoantibodies e.g., AMA for PBC

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26
Q

Broad causes of jaundice

A

Haemolysis (excess of UCB)
Failure of hepatic uptake (e.g., rifampin)
Impaired conjugation e.g., impaired glucuronyl transferase action in Crigler-Najjar syndrome
Impaired bilirubin excretion e.g., drug cholestasis, cirrhosis
Extrahepatic obstruction

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27
Q

Classification of jaundice and causes

A

Prehepatic (UCB)
- Haemolysis: G6PD deficiency, spherocytosis, SCD, blood transfusions (increased risk of pigment gallstones, dark urine due to increased urobilinogen excretion)
- Ineffective erythropoiesis: thalassaemia, pernicious anaemia
- Impaired conjugation: physiologic jaundice of newborn (transient low UGT activity), Gilbert syndrome (low UGT activity but normal liver function, rise in bilirubin with fasting/stress/sickness), Crigler-Najjar syndrome (absent UGT; kernicterus, fatal)
Intrahepatic (UCB 2nd and CB 1st)
- Non-obstructive biliary disease: hepatitis, cirrhosis (inflammation disrupts hepatocytes and small bile ductules), congestive hepatopathy, PBC, CF (dark urine due to increased urine bilirubin), benign recurrent intrahepatic cholestasis
- Obstructive intrahepatic cholestasis (biliary obstruction in liver in bile canaliculi): hepatocellular carcinoma/tumours of liver, intrahepatic gallstones, PSC
Extrahepatic cholestasis/obstructive (obstruction of ducts bw liver and duodenum; CB)
- Choledochilthiasis, inflamamtion e.g., PSC, malformation of biliary tract, pancreatic or bile duct carcinoma, bile duct stricture

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28
Q

Causes of post-operative jaundice

A

Hypotension, infection, drugs e.g., halothane, cardiac failure, haemolysis, haematoma resorption, bile duct injury/retained gallstone, renal failure, TPN

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29
Q

What kind of virus is HAV

A

RNA picornavirus

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30
Q

How is HAV transmitted

A

Faecal-oral route (ingestion of contaminated food or water e.g., shellfish), saliva and rarely via bloodborne products or sexual contact

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31
Q

What is the incubation period of HAV

A

Short: 2-3 weeks

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32
Q

Clinical features of HAV

A

Viraemia/prodrome (1-2 weeks): non-specifically unwell, nausea, anorexia, vomiting, malaise, fever, RUQ pain, tender hepatomegaly –> many recover at this stage and remain anicteric

Icteric infection (2 weeks): jaundice, pale stool dark urine, hepatomegaly +/- splenomegaly but viraemia Sx improve

Illness resolves in 3-6 weeks

Rarely severe disease esp. elderly: acute hepatitis, encephalopathy, death

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33
Q

Liver biochemistry in HAV

A

LFTs
- prodrome: raised ALT, AST (can remain high for weeks –> ~ 6mo)
- icteric: elevated bilirubin, AST, ALT
Serology
- anti-HAV IgM = acute infection
UEC (AKI has been reported in HAV - check)

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34
Q

How is HAV managed?

A

Notifiable infection and passive and active immunisation of exposed contacts
Supportive Mx

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35
Q

Prevention of HAV

A

Good hygiene
Killed by boiling water for 10 minutes
Active immunisation = inactivated HAV vaccine given to people traveling to endemic areas, pts with chronic liver disease, those with haemophilia, workers in frequent contact with hepatitis
Passive immunisation = normal human Ig used if recent (< 2 weeks) exposure to HAV (HAV vaccine also given)

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36
Q

What kind of virus is HBV

A

DNA hepadna virus

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37
Q

How is HBV transmitted

A

Percutaneous (blood, blood products, saliva), sexual, vertical

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38
Q

Incubation period HBV

A

Long: 1-5mo

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39
Q

Risk factors for HBV

A

Multiple sexual partners
MSM
IVDU
Unscreened blood transfusion
Unsterile tattoo/piercing
Born in high prevalence area

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40
Q

Investigations for HBV

A

Screening:
- HBsAg (active or chronic infection)
- Anti-HBc: if acute HBV suspected Anti-HBc IgM can be requested (acute hepatitis B if high titre, chronic hepatitis B if low titre, IgG suggests past exposure
- Anti-HBs (immunity)
If positive full viral profile and investigations performed
- FBC, LFT, INR, AFP
- Liver USS
- HBeAg (high infectivity/high level infection, persistence implies continued infectious state/development of chronicity)
- Anti-HBe (seroconversion)
- HBV DNA (implies viral replication –> levels indicate response to antiviral Tx)
- APRI/fibroscan to assess for cirrhosis
- HAV, HCV, HDV, HIV

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41
Q

Describe the phases of chronic HBV infection

A

Immune tolerance (HBeAg positive chronic hepatitis)
- HBeAg +, HBV DNA high, ALT normal (no Tx required)
Immune clearance (HBeAg positive chronic hepatitis)
- HBeAg +, HBV DNA high, ALT high (Tx required)
Immune control (HBeAg negative chronic hepatitis)
- Anti-HBe +, HBV DNA low, ALT normal (no Tx required)
Immune escape (HBeAg negative chronic hepatitis)
- AntiHBe +, HBV DNA high, ALT high (Tx required)

Or immunotolerance (no Tx), HBeAg + chronic hepatitis (Tx), HBeAg - chronic hepatitis (Tx), inactive carrier state (no Tx)

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42
Q

Prevention of HBV vertical transmission

A

Transmission from a HBeAg + mother to child at parturition is 85%
Vaccination and administration of HBIG to neonate reduces this by 95% but in extremely high viral loads this may be attenuated so in this case the mother should receive tenofovir in the last trimester of pregnancy in addition to neonatal vaccination and HBIG

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43
Q

Prevention of HBV horizontal transmission

A

Reducing risk of exchange of body fluids
High risk groups should be vaccinated
- MSM
- Adults with multiple sexual partners
- IVDU
- Haemodialysis patients
- Healthcare workers
- Household members of carriers

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44
Q

Which phases of HBV infection can lead to HCC and cirrhosis

A

Immune clearance and immune-escape/reactivation
Given risk of reactivation in silent carrier state there must be lifelong monitoring

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45
Q

What does reduction in eAg and seroconversion each year imply

A

Resolution into silent carrier stare or emergence of core promotor/pre-core mutants responsible for severe and progressive hepatitis (monitor ALT, HBV DNA +/- liver biopsy)

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46
Q

What is the HBV serology and histology in an immunotolerant individual

A

HBsAg +
HBeAg +
Anti-HBc -
Anti-HBs -
Anti-Hbe -
HBV DNA high
ALT normal
Histology normal

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47
Q

What is the HBV serology and histology in a pt with HBeAg + chronic hepatitis B

A

HBsAg +
HBeAg +
Anti-HBc -
Anti-HBs -
Anti-Hbe -
HBV DNA high
ALT high
Histology hepatitis

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48
Q

What is the HBV serology and histology in a pt with HBeAg - chronic hepatitis B

A

HBsAg +
HBeAg -
Anti-HBc -
Anti-HBs -
Anti-Hbe +
HBV DNA mildly elevated
ALT high
Histology hepatitis

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49
Q

What is the HBV serology and histology in an inactive carrier

A

HBsAg +
HBeAg -
Anti-HBc -/+
Anti-HBs -
Anti-Hbe +
HBV DNA low
ALT normal
Histology normal

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50
Q

What is the HBV serology and histology in a resolved infection

A

HBsAg -
HBeAg -
Anti-HBc +
Anti-HBs +
Anti-Hbe +
HBV DNA 0
ALT normal
Histology normal

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51
Q

What is the HBV serology and histology in a vaccinated pt

A

HBsAg -
HBeAg -
Anti-HBc -
Anti-HBs +
Anti-Hbe -
HBV DNA 0
ALT normal
Histology normal

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52
Q

How does HBV lead to HCC in absence of cirrhosis

A

Priming of tumour oncogenes and inactivation of TSGs

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53
Q

What monitoring is required in a HBeAg positive chronic infection (immune tolerance)

A

6monthly LFTs
6-12monthly HBeAg and antiHBe
12 monthly HBV DNA, liver fibrosis assessment
Periodic review of household contacts and sexual partners

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54
Q

What monitoring is required in a HBeAg negative chronic infection (immune control)

A

6 monthly LFTs
12 monthly HBV DNA liver fibrosis assessment

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55
Q

What is management for chronic HBV

A

Entecavir or tenofovir (nucleoside and nucleodtide analogues) or pegylated interferon in women wishing to become pregnant
Notification
Screening of household contacts and administration of HBIG and HBV vaccine

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56
Q

What monitoring is required for pts on HBV treatment (HbeAg - or + chronic hepatitis)

A

Regular LFTs, UECs, HBV DNA, serum phosphate if on tenofovir
If HBe+: HbeAg, antiHbe
If HBV DNA undetectable: HBsAg, antiHBs
If cirrhotic: FBC, INR
HCC surveillance with 6monthly USS and AFP in certain groups (ATSI, Asian, HDV coinfection, FHx etc)

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57
Q

What kind of virus is HCV

A

RNA flavivirus

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58
Q

How is HCV transmitted

A

Percutaneous (blood/blood products e.g., IVDU, unsterile tattoos/piercings, unscreened blood transfusion, needestick injury; saliva), rarely sexual, rarely vertical

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59
Q

HBV acute infection clinical presentation

A

Varies
- serum-sickness like syndrome (rash, myalgia, fever, arthralgia)
- subclinical hepatitis
- symptomatic hepatitis: fever, rash, myalgia, arthralgia, fatigue, anorexia, N, V, RUQ pain, jaundice

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60
Q

HBV chronic infection clinical presentation

A

Infection persisting for > 6 mo
- asymptomatic/non-specific Sx
- hepatic failure
- acute hepatitis Sx

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61
Q

Risk factors for HCV infection

A

IVDU
Blood transfusion without screening
Healthcare workers
Unsterile tattoos
High prevalence areas (Asia, Middle East)

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62
Q

What is cirrhosis?

A

Irreversible end-point of longstanding liver damage and advanced stage of fibrous tissue accumulation characterised by disruption of normal hepatic parenchyma and diffuse remodelling into perenchymal nodules (regenerated hepatocytes) surrounded by fibrous bands plus microvascular distortion and a variable degree of portosystemic shunting

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63
Q

Causes of cirrhosis

A

Chronic HBV, HCV
NAFLD
Alcohol liver disease
Autoimmune

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63
Q

MCCs of cirrhosis

A

Chronic HBV, HCV
NAFLD
Alcoholic liver disease
(but anything causing chronic liver damage e.g., PBC, PSC, autoimmune hepatitis, wilson disease, a1a1 deficiency, Budd-Chiari, congestive hepatopathy with cardiac cirrhosis etc.)

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64
Q

Pathophysiology of cirrhosis

A

Degeneration and necrosis of hepatocytes –> Kupffer cells activated and destroy hepatocytes and release inflammatory cytokines e.g., TGF-B and PDGF –> activation of normally quiescent stellate cells (beneath endothelial cells lining sinusoids) –> stellate cells produce collagen –> fibrotic bands and regenerative nodules replace parenchyma –> fibrotic tissue compresses hepatic sinusoids and venules –> increased portal vein hydrostatic pressure –> intrasinusoidal HTN and dysfunctional sinusoids –> loss of liver Fx/impaired substrate exchange

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65
Q

Clinical effects of cirrhosis

A

Portal HTN
- ascites (due to increased HS and reduced oncotic pressure)
- congestive splenomegaly and hypersplenis –> thrombocytopaenia and haemolytic anaemia
- portosystemic shunting (oesophageal and gastric varices, haemorrhoids, caput medusae)
- hepatorenal syndrome
Decreased detoxification
- palmar erythema, spider naevi, testicular atrophy, gynaecomastia, loss of male pattern body hair distribution (excess oestrogen)
- hepatic encephalopathy, asterixis, coma (increased serum ammonia crossing BBB)
- jaundice and pruritus (decreased bilirubin metabolism and no albumin to bind to)
Decreased protein synthesis
- hypoalbuminaemia –> oedema
- coagulopathy (decreased 2, 7, 9, 10)
Reduced vitamin D hydroxylation

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66
Q

What is the Child-Pugh score and what are it’s components

A

A prognostic grading score to assess the severity of cirrhosis
A: hypoalbuminaemia
B: hyperbilirubinaemia
C: coagulopathy/prolonged INR
D: distension/ascites
E: encephalopathy

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67
Q

What are signs of decompensation in cirrhosis

A

Jaundice
Hepatic fetor
Asterixis and impaired mentation

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68
Q

What is the life expectancy for different Child-Pugh classes

A

Child‑Pughclass A: almost normal
Child‑Pughclass B:one-yearsurvival rate ofapprox. 80%
Child‑Pughclass C:one-yearsurvival rate ofapprox. 45%
Score > 7 is candidate for transplant

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69
Q

Causes of hepatic decompensation in cirrhosis

A

Infection e.g., UTI, cellulitis, SBP, pneumonia, bacteraemia, HAV/HBV/HCV/HDV/CMV/EBV
Metabolic e.g., renal failure, hyponatraemia, uraemia, volume depletion (variceal bleed)
Drugs: alchol, paracetamol, diuretics, opiates
Malignancy: HCC, cholangiocarcinoma, met
Vascular: portal vein thrombosis, Budd-chiari syndrome

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70
Q

Complications of cirrhosis

A

Ascites
Portal HTN, shunting and variceal bleeding
Encephalopathy
Hepatopulmonary syndrome, portopulmonary syndrome, cirrhotic cardiomyopathy
HCC

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71
Q

Tx of cirrhosis

A

Tx underlying cause
Alcohol cessation always
Cessation of offending medication
Viral hepatitis Tx if relevant
Mx of autoimmune liver disease/Wilsons disease/haemochromayosis etc.

High energy and high protein diet
Moderate exercise
Correction of nutritional deficiencies esp. vit D and thiamine

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72
Q

Why does ascites occur with liver cirrhosis

A

Reduced synthesis of albumin = reduced capillary oncotic pressure –> fluid moves out of vessels
Hepatic resistance to blood flow and increased capillary HS pressure -> fluid moves out of vessels
Hepatic resistance to blood flow, splanchnic vasodilation and portosystemic shunt formation –> release of systemic vasodilators –> RAAS –> increased fluid retention (secondary to sodium)

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73
Q

When new-onset ascites in cirrhotic pt is detected what Ix should be done?

A

Paracentesis for
- measurement of serum ascites to albumin gradient (SAAG): if > 11g/L ascites is almost always due to portal HTN
- MC&S
- cytology
- protein
- LDH

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74
Q

How is ascites in cirrhosis Mx

A

No added sodium diet
Diuretics e.g., spironolactone 100mg + furosemide 40mg
Regular weight and serum biochemistry checks
Refractory
- Large volume paracentesis
- Transjugular intrahepatic portosystemic shunting
- Liver transplant

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75
Q

Complications of diuretic use in cirrhosis

A

Hepatic encephalopathy
Renal impairment
Hypotension
Electrolyte imbalance

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76
Q

Explain how a large volume paracentesis is performed in cirrhotic ascites

A

Can be done outpatient
For every L of ascites removed give IV 8g albumin to prevent orthostatic hypotension and renal failure from fluid shifts

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77
Q

What are the risks of TIPS

A

Hepatic encephalopathy
Shunt-induced congestive cardiac failure (pt must have echo to ensure LEF > 60% before TIPS)

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78
Q

What is hepatorenal syndrome?

A

A marker of decompensated cirrhosis requiring the presence of ascites and characterised by rising creatinine in absence of other causes of renal failure

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79
Q

Compare type 1 and type 2 HRS

A
  1. rapidly progressive reduction of renal Fx (doubling of creatinine in < 2 weeks) characterised by acute kidney injury (median survival 2 weeks)
  2. moderate renal failure with steady/slowly progressive course characterised by refractory ascites (median survival 4-6mo)
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80
Q

MC precipitants of HRS

A

bacterial infection esp. SBP, GI bleed, paracentesis

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81
Q

Management of hepatorenal syndrome

A

Exclude renal parenchymal disease/obstruction/other causes
Septic screen including paracentesis

Discontinue diuretics
Volume expansion
Tx infection/precipitant
Avoid nephrotoxins

Vasoconstrictors: splanchnic and systemic vasoconstriction allows for improved renal perfusion
- e.g., terlipressin (ADH analogue) or midodrine (noradrenaline) with somatostatin/octreotide
Albumin infusion with terlipressin improves efficacy

Liver transplant must be considered

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82
Q

How does HRS occur

A

Hepatic resistance to blood flow and portosystemic shunting –> systemic vasodilation –> RAAS & sympathetic NS activation –> sodium and water retention –> progressive renal vasoconstriction

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83
Q

What is the risk of reversing hyponatraemia too quickly

A

Osmotic demyelination syndrom

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84
Q

How does hyponatraemia occur in cirrhosis

A

Impaired free water excretion from increased ADH

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85
Q

What is spontaneous bacterial peritonitis

A

Infection of ascitic fluid in absence of any focal intra-abdominal, surgically treatable source of infection

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86
Q

Clinical presentation of sponatenous bacterial peritonitis in cirrhosis

A

MC: deteriorating liver Fx, encephalopathy, renal failure, or shock
Less common: abdo pain, peritonism
Possibly asymptomatic

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87
Q

When should a diagnostic tap/paracentesis looking for SBP need to be performed?

A

At first episode of ascites and every subsequent presentation in which person with ascites has deteriorated

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88
Q

How is spontaneous bacterial peritonitis Dx

A

Ascitic fluid analysis: 1 of
- High neutrophil count

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88
Q

How is spontaneous bacterial peritonitis Dx

A

Ascitic fluid analysis: 1 of
- High neutrophil count

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88
Q

How is spontaneous bacterial peritonitis Dx

A

Ascitic fluid analysis: 1 of
- High neutrophil count

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88
Q

How is spontaneous bacterial peritonitis Dx

A

Ascitic fluid analysis: 1 of
- High neutrophil count

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88
Q

How is spontaneous bacterial peritonitis Dx

A

Ascitic fluid analysis: 1 of
- High neutrophil count

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88
Q

How is spontaneous bacterial peritonitis Dx

A

Ascitic fluid analysis: 1 of
- High neutrophil count
- High WCC
- Positive culture

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89
Q

What organisms are most commonly implicated in SBP

A

GNRs esp. escherichia coli
Streptococci esp. enterococcus

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90
Q

When is a secondary cause of peritonitis e.g., bowel perforation more likely than SBP

A

Polymicrobial culture
Localising peritoneal signs

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91
Q

Treatment of spontaneous bacterial peritonitis

A

Empirical: IV ceftriaxone 2g daily or cefotaxime 2g 8 hourly
If kidney impairment or high risk of HRS give albumin infusion
If pt not improving repeat paracentesis after 2 days and if neutrophil count has not dropped to 25% of original suspect resistant organism and change antibiotics

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91
Q

Prevention of spontaneous bacterial peritonitis in patients with cirrhosis

A

Secondary antibiotic prophylaxis after first episode: trimethoprim + sulfamethoxazole 160 + 800mg PO daily
Primary prophylaxis is only recommended in high risk patients: cirrhosis, ascites, low ascites fluid protein concentration and either impaired kidney function or liver failure score 9+

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91
Q

When is variceal bleeding a risk

A

When portal HTN is > 12mmHg and portosystemic shunts have formed but cannot facilitate the high pressure flow

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91
Q

How does portal HTNive gastropathy tend to present

A

IDA rather than obvious GI haemorrhage

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91
Q

What is endoscopic feature that suggests high risk of variceal bleed

A

Red-wale sign = red streak over varix suggesting recent bleeding
Bleeding
Fibrin clot

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91
Q

What is primary prophylaxis for variceal bleeding

A

Pts with large varices should be given beta-blockers e.g., propranalol (or carvedilol)
Endoscopic prophylaxis may also be attempted (band-ligation) in high risk varices

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91
Q

When should patients with liver disease have an upper endoscopy

A

All patients with definite/suspected cirrhosis to look for oesophageal varices
Repeat every 2 years if normal
Repeat in 1 year if low risk varices
Give propranolol and/or endoscopic band ligation if high risk varices

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91
Q

Management of variceal bleeding

A

Resuscitation and supportive care
Blood transfusion
Manage coagulopathy and thrombocytopaenia
Terlipressin/somatostatin analogue e.g., ocretotide
Prophylactic ABx e.g., ceftriaxone
Endoscopy –> band ligation, clipping, balloon tamponade
TIPS
Mx alcohol withdrawal and comorbid conditions
Propranolol

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91
Q

MC complications after variceal haemorrhage

A

Sepsis
AKI
Hepatic encephalopathy

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92
Q

Management of portal HTNive gastropathy

A

Propranolol –> TIPS if propranolol ineffective

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93
Q

What is hepatic encephalopathy and how does it occur

A

The neurological and psychiatric dysfunction/deterioration in mental status and cognitive function that occurs in individuals with significant hepatic dysfunction and portosystemic shunts due to inadequate elimination of metabolic products and accumulation of neurotoxic metabolites like ammonia that can cross BBB.

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94
Q

Precipitants of hepatic encephalopathy

A

Volume depletion (often from diuretics –> correct with IV albumin and stop diuretics)
Infection (treat with ABx)
Renal failure and electrolyte imbalance (correct)
Alcohol (cease and Mx withdrawal)
Sedatives (cease)
TIPS (lactulose or rifaximin)
Hepatocellular carcinoma
Protein load including GI bleed (endoscopy and Mx of bleed)
Constipation (lactulose)

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95
Q

How do lactulose and rifaximin work in hepatic encephalopathy

A

Lactulose is converted to lactic acid by intestinal flora and as the gut is acidified ammonia is converted to ammonium which is excreted in faeces
Rifaximin reduces the number of ammonia producing bacteria in gut

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96
Q

Clinical features of hepatic encephalopathy

A

Fatigue, lethargy, apathy
Altered consciousness: mild confusion –> stupor –> coma
Disorientation, irritability
Memory loss
Impaired sleeping pattern
Inappropriate behaviour
Slurred speech
Asterixis

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97
Q

Mx of acute hepatic encephalopathy

A

Mx of underlying precipitants and stopping drugs that may worsen encephalopathy (sedatives, opioids, PPIs)
Intubate if coma
Antibiotics if indicated
Nutritional support if required
Lactulose PO (or NG/PR) with goal of 2-3 semi-formed stools/day

98
Q

Prevention of recurrent hepatic encephalopathy or treatment of chronic hepatic encephalopathy

A

Lactulose 3-4 x daily (movicol or osmolax = macrogol if not tolerated)
Add rifaximin BD if repeated episodes despite Tx
Portal venous phase CT in chronic hepatic encephalopathy –> if large shunt present consider angiographic occlusion

99
Q

Should dietary protein be restricted in hepatic encephalopathy

A

No - does not offer benefit and risks malnutrition

100
Q

What is portopulmonary HTN and how does it affect MCly

A

Pulmonary arterial HTN in presence of portal HTN once other secondary causes have been excluded
6% of pts with cirrhosis; most at risk = females with autoimmune hepatitis

101
Q

Dx and Mx of portopulmonary HTN

A

Dx: echo –> confirmed by RH catheterisation
Mx: vasodilators e.g., PGs, PDE5inhibitors, endothelin antagonists e.g., bosentan

102
Q

What is hepatopulmonary syndrome and how is it Dx?

A

Increased pulmonary perfusion due to pulmonary vascular dilatation without increase in ventilation –> hypoxaemia presenting as dyspnoea on exertion/when upright

Dx = demonstration of intrapulmonary vascular shunting with echo while injecting microbubbles into peripheral vein which should not normally flow into pulmonary vasculature but will due to shunting

Mx = liver transplant

103
Q

What is cirrhotic cardiomyopathy

A

Systolic and diastolic dysfunction and QT prolongation in cirrhotic patients contributing to SCD, HRS, post TIPS cardiac failure and poorer perioperative performance in liver transplantation

104
Q

Define acute liver failure

A

Rapid deterioration of hepatocellular function characterised by encephalopathy/cerebral oedema and altered mentation and coagulopathy occurring within 26 weeks of initial liver injury in absence of pre-existing (or well-compensated) liver disease

105
Q

Define fulminant liver failure

A

Variant of acute liver failure as a result of massive hepatic necrosis and MCly associated with drugs/toxins esp. paracetamol in which encephalopathy develops within 8 weeks of onset of symptoms

106
Q

MCCs of acute liver injury

A

Drug induced liver injury esp. acetaminophen overdose
Mushroom poisoning
Viral hepatitis
Rarer: Wilson’s, Budd-Chiari, autoimmune hepatitis, ischaemic hepatopathy

107
Q

Management of acute liver injury

A

Supportive
Tx of RICP e.g., mannitol
Liver transplantation often required
Tx of specific aetiology: N-acetyl cysteine for paracetamol overdose, NAC and penicillin G for mushroom poisoning, nucleoside analogue for HBV, prednisolone for autoimmune hepatitis

108
Q

Indications for liver transplantation

A

End-stage cirrhosis
Fulminant hepatic failure
Primary sclerosing cholangitis
Budd-Chiari syndrome
Caroli disease

109
Q

Does infection occur in viral hepatitis pts who receive transplants

A

HCV: yes but prognosis still same as others
HBV: yes and graft survival reduced by ~20% but risk reduced by prophylactic HBIG and pre-treatment with nucleoside analogue

110
Q

Contraindications to liver transplant

A

Absolute: active sepsis outside biliary tract, metastatic hepatobiliary malignancy, HIV infection, advanced cardiopulmonary disease
Relative: older age (> 60), biliary infection, unreformed alcoholism, blocked portal vein, previous major upper abdominal surgery, major renal impairment, other medical disease e.g., poorly controlled DM

111
Q

Laboratory testing for Acute Liver Failure

A

LFTs
PT/INR

UEC, CMP, glucose
FBC, group and hold
ABG and arterial lactate
Amylase and lipase (exclude pancreatitis as a complication of ALF)
Ammonia

Paracetamol level
Urine toxicology screen

Viral hepatitis serologies: Anti-HAV IgM, HBsAg, antiHBc IgM, anti-HCV, HCV RNA, anti-HEV, HSV1 IgM, VZV
HIV1, HIV2 (implications for transplantation)

Ceruloplasmin level (pts < 40yo without other possible cause)
Iron studies (for haemochromatosis)
Autoimmune: ANA, ASMA, quantitative immunoglobulins

Pregnancy test

CXR (aspiration pneumonia in hepatic encephalopathy pt, pylmonary oedema etc)
Abdo USS with Doppler (Budd-Chiari syndrome, hepatomegaly, splenomegaly, hepatic surface nodularity)

112
Q

What are USS Doppler findings in Budd-Chiari syndrome

A

Hepatic vessel thrombosis
Loss of hepatic venous signal
Reverse flow in portal vein

113
Q

Causes of very high transaminases

A

Drug induced liver injury
Ischaemic hepatopathy
Acute viral hepatitis
Acute biliary obstruction

114
Q

What is a possible cause of abnormal LFTs and arrhythmia

A

Amiodarone toxicity

115
Q

How does the OCP affect the liver

A

Cause/stimulate hepatic adenoma growth (and v rarely HCC)
Cause focal nodular hyperplasia and peliosis hepatitis
Cholesterol gallstones
Budd-chiari syndrome

116
Q

How does acetaminophen overdose cause acute liver disease

A

At therapeutic doses most acetaminophen is metabolised to pharmacologically inactive glucuronide but ~5% is converted via CYP450 pathway to toxic NAPQI which is hepatotoxic. Glutathione inactivates NAPQI but its reserves are depleted when high paracetamol doses are consumed resulting in accumulation of NAPQI causing severe hepatic necrosis, fulminant liver failure and death without transplantation.

Additional risk factors include alcohol binge + acetaminophen (alcohol depletes glutathione and activates CYP450 system resulting in more NAPQI) and malnutrition (glutathione deficiency)

117
Q

Immediate liver transplant complications

A

Surgical:
- vascular and biliary breakdown or occlusion
- infections/collections
Primary non-function
Sepsis
Rejection

118
Q

Medium term liver transplant complications

A

Biliary strictures:
- anastamotic
- ischaemic
- hepatic artery stenosis/thrombosis –> ischaemic stricture
Rejection
Infection (viral e.g., CMV)
Recurrence of underlying disease (e.g., HCV)

119
Q

Long term complications of liver transplant

A

Metabolic (often from immunosuppressive drugs) –> weight gain, cholesterol, DM, HTN, renal impairment
Cancer –> skin, post-transplant lymphoproliferative disorder, solid
Infections
Chronic rejection/non-compliance
Recurrence –> HCV/HBV/PSC/PBC/AIH/alcohol

120
Q

Examples of drugs causing acute hepatitis

A

Halothane, phenytoin, chlorothiazide

121
Q

Examples of drugs causing cholestasis

A

HS reaction: phenothiazines, sulfonamide, erythromycin
Dose related: OCP, anabolic steroids

122
Q

Examples of drugs causing fatty liver

A

Methotrexate, tetracycline, amiodarone, itamin A, corticosteroids

123
Q

Cytotoxic liver drugs

A

Acetaminophen

124
Q

Drugs causing peliosis hepatitis (large blood filled cavities)

A

OCP, corticosteroids, tamoxifen

125
Q

What LFTs suggest alcoholic liver disease

A

AST/ALT ratio < 2 and very high GGT
Dx confirmed with liver biopsy

126
Q

How is acute alcoholic hepatitis Tx

A

Alcohol withdrawal
Supportive care
Nutritional supplementation
If severe –> prednisolone 40mg PO daily for 7 days
Acetaminophen should not be given for pain relief

127
Q

Pathophysiology of alcoholic liver disease (steatosis –> hepatitis)

A

Ethanol degraded in liver to acetylCoA by alcohol dehydrogenase –> NADH excess –> NDAH drives formation of G3P and fatty acids- -> increased TG synthesis in liver and accompanying inflammation –> steatohepatitis –> chronic inflammation leads to fibrosis and cirrosis

128
Q

GI complications of alcoholism

A

Chronic liver disease (alcoholic steatosis, hepatitis, cirrhosis)
Hepatomegaly due to fatty liver and chronic liver disease
Watery diarrhoea from alcohol or steatorrhea due to chronic alcoholic pancreatitis
Pancreatitis (chronic and acute attacks)
Acute gastritis (erosions)
Parotitis/parotidomegaly

129
Q

CVS complications of alcoholism

A

HTN, cardiomyopathy, arrhythmias

130
Q

Nervous system complications of alcoholism

A

Wernicke encephalopathy and Korsakoff syndrome 2˚ to thiamine deficiency
Pellagra 2˚ niacin (B3) deficiency
Withdrawal: tremor, seizures, hallucinations, delirium tremens
Dementia (cerebral atrophy and corpus callosum demyelination)
Cerebellar degeneration
Central pontine myelonosis –> pseudobulbar palsy, spastic quadriplegia
Autonomic neuropathy
Proximal myopathy
Acute intoxication

131
Q

Haematological complications of alcoholism

A

Megaloblastic anaemia (dietary B9 deficiency, rarely B12 deficiency in chronic pancreatitis with failure to cleave B12-R-protein complex)
IDA (bleeding erosions/portal HTN)
Aplastic anaemia (direct toxic effect on bone marrow)
Thrombocytopaenia (bone marrow suppression, hypersplenism)

132
Q

Metabolic abnormalities associated with alcoholism

A

Acidosis (lactic, ketoacidosis)
Hypoglycaemia
Hypocalcaemia
Hypomagnesaemia
Hypertriglyceridaemia
Hyperuricaemia

133
Q

What is Budd Chiari syndrome

A

Condition of hepatic vein/outflow obstruction leading to congestion of the liver with subsequent hepatocyte damage and progressive liver failure, associated with hepatomegaly, ascites, and abdominal discomfort.

134
Q

What causes Budd Chiari syndrome

A

Hypercoagulable state –> thrombosis
MC = thrombotic occlusion of hepatic vein 2˚ to chronic myeloproliferative neoplasm e.g., polycythaemia vera
Other causes of hypercoagulability e.g., paraneoplastic thrombocytosis, pregnancy and postpartum period, clotting disorders (antithrombin III deficieny e.g., prothrombin mutation, protein C deficiency, protein S deficiency; antiphospholipid syndrome), paroxysmal noctural haemoglobinuria, chronic inflammatory diseases, and SEs of medication e.g., oestrogen can cause thrombotic occlusion also
Obstruction may also be a result of invasion/compression of hepatic veins by HCC, renal carcinoma, chronic infections (TB, amebiasis, syphilis etc.)

135
Q

Buddy-Chiari syndrome clinical presentation

A

Abdominal pain
Tender hepatomgealy
Ascites and abdominal distension
Jaundice
Varices

Dependent on rate of thrombus formation
Fulminant: severe necrosis with jaundice and encephalopathy within 8 weeks
Acute: collaterals have not developed so pain, necrosis, ascites dominate
Subacute: collaterals have developed so hepatic necrosis is minimal: unexplained varices/splenomegaly
Chronic: decompensated cirrhosis

136
Q

How is Budd-Chiari syndrome diagnosed

A

Bloods: FBC, LFTs (elevated AST, ALT, ALP, bilirubin, decreased albumin), UEC, coags (prolonged PT in fulminant), thrombophilia screening, JAK2 mutation
Flow cytometry for CD55- and CD59-deficient blood cells (presence suggests paroxysmal nocturnal haemoglobinuria)
Doppler USS (altereations in hepatic and/pr caval beins in form of thrombosis, stenosis, fibrotic cord etc.)
CT and MRI abdo may be useful also and if suspicion remains high but tests are negative do hepatic venography

137
Q

Budd-Chiari syndrome Tx

A

Thrombolysis or radiological intervetion/angioplasty or surgical shunting or urgent liver transplant if fulminant

Anticoagulation (enoxaparin/heparin and warfarin)
- continue lifelong

Mx ascites with diuretics, sodium restriction and, if required, large volume paracentesis
Mx complications of cirrhosis

138
Q

MCC of chronic liver disease in developed world

A

Non-alcoholic fatty liver disease and non-alcoholic steatohepatitis

139
Q

Clinical features of NASH

A

Metabolic syndrome
Asymptomatic
Possible dull RUQ discomfort
Hepatomegaly
Signs of cirrhosis and portal HTN possible

140
Q

How is NASH Dx

A

Usually clinically
Abnormal transaminases in setting of obesity/DM/hyperlipidaemia etc.
Other causes of liver disease excluded (alcohol, medications, viruses, iron overload)
Liver USS may be used to help confirm Dx but does not rule out NAFLD if negative
Assessment of fibrosis and cirrhosis

141
Q

MCC of morbidity and mortality in NAFLD

A

CVD and malignancy

142
Q

What is NALFD

A

Presence of hepatic steatosis (fatty liver) in individuals who do not consume large quantities of alcohol and who do not have another cause of secondary hepatic fat accumulation (e.g., HCV, Wilson Disease, medications

Progresses from steatosis and fat accumulation in hepatocytes to inflammation (steatohepatitis) and fibrosis as a result of lipid peroxidation and inflammation (enhanced by insulin resistance)

143
Q

Tx of NAFLD

A

Aims = weight loss, control of blood glucose insulin resistance, lipid levels

  • Low GI diet and low fat diet
  • Exercise
  • 10% reduction in body weight over 6mo
  • No alcohol intake
  • Manage cardiovascular risk factors
  • 6 monthly HCC surveillance
  • Routine immunisations +/- HAV, HBV
144
Q

Causes other than obesity/metabolic syndrome of hepatic steatosis

A
  1. Protein calorie malnutrition or starvation
  2. TPM
  3. Drugs e.g., OCP, corticosteroids, methotrexate, amiodarone, tamoxifen, CCBs, tetracycline
  4. Lipodystrophy
  5. Acute fatty liver of pregnancy
  6. IBD
145
Q

What is Wilson’s Disease

A

Rare AR disease of copper excess secondary to mutation in ATP7B copper transport gene

146
Q

When does Wilson disease present

A

Young adult life usually up to age 35

147
Q

How does Wilson’s disease present

A

Liver disease: variable (cirrhosis/acute liver failure)
Neurological disease: parkinsonism, tremor, rigidity, dysarthria, dystonia, psychiatric disease (depression, irritability, psychosis), cognitive impairment
Kayser-Fleisher rings (copper accumulation in cornea –> green-brown rings in periphery of iris)

148
Q

Diagnosis of Wilson’s disease

A

Combination testing:
- slit lamp exam for K-F rings
- serum ceruloplasmin (reduced; acute phase reactant and can be falsely elevated in hepatitis or depressed in cirrhosis with impaired synthetic Fc hence combo test)
- 24 hour urinary copper (excess)
Liver biopsy for definitive diagnosis

149
Q

Wilsons disease Tx

A

Penicillamine (+ pyridoxine) –> promotes urinary copper excretion and inhibits GI copper absorption
Or trientine = copper chelator
Fulminant and curative = transplant

150
Q

How does alpha 1 antitrypsin deficiency affect liver

A

AR disease involving abnormally folded a1at –> accumulates in liver –> cirrhosis
Dx with electropheresis and Tx with transplant
Pts have emphysema

151
Q

What gene is affected in hereditary haemochromatosis

A

HFE

152
Q

What is the cause of aqcuired haemochromatosis

A

Multiple blood transfusions or secondary erythropoesis e.g., thalassaemia or sideroblastic anaemia

153
Q

Clinical presentation of haemochromatosis

A

Incidental finding on iron studies
Men more likely to present with clinical features as women are protected by menses until menopause
Advanced: hepatomegaly, DM, pigmentation (bronze diabetes), arthropathy, dilated cardiomyopathy, erectile dysfunction (2˚ pituitary iron deposition)

154
Q

Screening for haemochromatosis

A

Fasting transferrin saturation (increased)
Ferritin (increased)
- can be rasied as an acute phase reactant including in hepatitis/other inflammatory liver disease
- look for progressive rise rather than fluctuating level

155
Q

How is haemochromatosis confirmed

A

Genetic testing
MRI if very high ferritin
consider liver biopsy and chemical iron staining to determin hepatic iron index if uncertain

156
Q

Treatment of haemochromatosis

A

Avoidance of excess alcohol
Regular venesection (arthropathy and endocrine changes do not respond - irreparable damage)
6monthly HCC screening if cirrhosis

157
Q

What is autoimmune hepatitis

A

Idiopathic necroinflammatory liver disease characterised by presence of autoantibodies, elevated globulins, and interface hepatitis on liver biopsy

158
Q

How does autoimmune hepatitis present

A

Common = fatigue and arthralgia
Weight loss
Upper abdominal pain
Hepatomegaly
Advanced = jaundice, RUQ pain, splenomegaly, ascites

159
Q

How is autoimmune hepatitis Dx

A

Positive autoantibodies: ANA, anti-smooth muscle antibody (ASMA) in type 1 AIH, anti liver-kidney microsomal type 1 antibody (anti-LKMA1) in type 2 AIH
Elevated IgG
Typical histology on liver biopsy: interface hepatitis with prominent plasma cell infiltrate
Absence of viral hepatitis/other causes of hepatitis

160
Q

What is the typical patient group affected by autoimmune hepatitis

A

Females
Bimodal age: 10-20yo and 45-75yo
Other autoimmune disease

161
Q

Tx of autoimmune hepatitis

A

Induction: prednisolone 40-60mg PO daily
Maintenance: azathiopurine 25-50mg PO daily

162
Q

What is primary biliary cholangitis/cirrhosis

A

Chronic progressive autoimmune liver disease characterised by destruction of intralobular bile ducts

163
Q

Who does PBC typically affect

A

Middle age women
Autoimmune history

164
Q

Who does PSC typically affect

A

Young men with UC

165
Q

What is primary sclerosing cholangitis

A

Progressive chronic inflammation of intrahepatic and extrahepatic bile ducts

166
Q

How does PSC present

A

Cholestasis: jaundice, pruritus, pale stool dark, urine
Possible acute cholangitis: fever, RUQ pain, jaundice
Hepatosplenomegaly

167
Q

How does PBC present

A

Fatigue
Cholestasis: jaundice, pruritus!!!! (marked generalised pruritus), pale stool, dark urine
Hyperpigmentation
Xanthomas and xanthelasma (defective hepatic conversion of cholesterol)
Hepatosplenomegaly

168
Q

How do serological markers differ between PSC and PBC

A

PSC: pANCA+ (80%)
PBC: AMA+ (90%)

169
Q

How do MRCP findings differ between PSC and PBC

A

PSC: Irregular and beaded ducts
PBC: Pruned ducts

170
Q

Complications of PSC

A

Portal HTN, liver failure, bile duct carcinoma

171
Q

Complications of PBC

A

Osteomalacia (deficiency of fat soluble vitamins)
Steatorrhea (bile acid deficiency, associated pancreatic insufficiency or celiac disease)
Portal HTN, liver failure

172
Q

Diagnosis of PBC

A

LFTs: Raised ALP and GGT
Hypercholesterolaemia
AMA+, ANA+, Elevated IgM
Abdo USS to screen for mechanical bile duct obstruction
MRCP to confirm intrahepatic bile duct obstruction

173
Q

Diagnosis of PSC

A

FBC
LFTs: Raised ALP and GGT, normal or high conjugated bilirubin
Lipid profile: High total cholesterol
Serology: pANCA, ANA, possible IgG or IgM elevation
Abdo USS to screen for bile duct abnormality
MRCP to confirm multifocal intrahepatic and/or extrahepatic structures and dilatations
ERCP if MRCP is non-diagnostic or therapeutic intervention anticipated (brush cytology for malignancy, dilation of strictures, extraction of stones etc.)

174
Q

Treatment of PSC

A

ERCP with biliary stenting may relieve cholestatic Sx if dominant stricture
Liver transplant
Surveillance for varices and HCC once cirrhotic

175
Q

PBC Tx

A

Ursodeoxycholic acid 15mg/kg/day
Liver transplant
Surveillance for varices and HCC once cirrhotic
Replacement vitamin D

176
Q

What is cholelithiasis

A

The presence of gallstones within the gallbladder

177
Q

What types of gallstones are there

A

Cholesterol stones (MC)
Pigment stones (calcium bilrubinate)

178
Q

What causes cholesterol gallstones

A

Cholesterol supersaturation in bile relative to bile salts and phospholipids, crystallisation promoting factors within bile, and gallbladder stasis/reduced motility

179
Q

Risk factors for cholesterol gallstones

A

Increasing age (>40)
F > M (oestrogen increases cholesterol in bile)
FHx
Multiparity or pregnancy (progesterone in pregnancy also causes biliary stasis)
Obesity +/- metabolic syndrime
Rapid weight loss (elevated leptin in rapid weight loss increases cholesterol secretion into bile)
High cholesterol diet
OCP, fibrates, HRT
Ileal disease or resection (CD, CF; reduced bile acid reabsorption)
Cirrhosis
Spinal cord injury
DM
Liver cirrhosis
TPN (biliary stasis)

180
Q

Risk factors for pigment stones

A

Hyperbilirubinaemia 2˚ haemolytic anaemia e.g., G6PD deficiency, spherocytosis, SCD, thalassaemia, prosthetic valves, malaria, hypersplenism, foot trauma in long distance runners
Ileal resection/disease
TPN
Alcoholic cirrhosis

181
Q

What is the cause of mixed/brown pigment stones

A

Bacterial infections and parasites in biliary tree causing release of B-glucuronidase by damaged hepatocytes and bacteria resulting in precipitation of calcium carbonate, cholesterol and calcium bilirubinate

182
Q

How does cholelithiasis present

A

Mostly asymptomatic but may present as biliary colic if stone impacts in cystic duct

Biliary colic:
- dull constant RUQ/epigastric pain (sudden onset, severe, crescendo)
- < 6hours
- esp. postprandial/post fatty meal (CCK –> gallbladder contraction –> stone forced into cystic duct)
- pain may radiate into R shoulder/subscapular region (increased intraluminal pressure irritates phrenic nerve)
- nausea, vomiting, early satiety, bloating, dyspepsia
- most common mid-evening

183
Q

What is cholecystitis

A

Inflammation of gallbladder secondary to obstruction of gallbladder emptying

184
Q

Causes of cholecystitis

A

Acute calculous cholecystitis: cystic duct obstruction by gallstone –> distension and inflammation of gallbladder (progressive distension may compromise vascular supply)
+/- Possible secondary infection
Acalculous cholecystitis: in critically ill patients with conditions predisposing to bile stasis and reduced GB perfusion e.g., MOF, severe trauma/burns, surgery, sepsis, TPN, prolonged fasting

185
Q

Presentation of acute cholecystitis

A

RUQ pain typically more severe and prolonged (>6hrs) than biliary colic
Postprandial
Radiation to R scapula
+ Murphy sign = sudden pause in inspiration upon deep palpation of RUQ due to pain
Guarding and peritoneal signs
Fever, malaise, anorexia, N, V

186
Q

Investigation of suspected acute cholecystitis

A

Abdo USS = single most useful Ix for Dx of gallstone-related disease

FBC (mild leukocytosis)
CRP (raised)
LFTs (serum bilirubin, ALP, and aminotransferases may be mildly elevated in cholecystitis even without obstruction –> more elevated suggests obstruction)

187
Q

What findings are seen on abdominal USS in cholecystitis

A

Gallstones in gallbladder esp obstructing neck/cystic duct: stones cast an acoustic shadow
Thickening and oedema of gallbladder wall
Distended gallbladder
Focal tenderness while using probe over gallbladder wall
Possible fat stranding

188
Q

DDx for biliary colic

A

Spasm of hepatic flexure in IBS
Carcinoma of R colon
Atypical PUD, renal colic and pancreatitis

189
Q

DDx acute cholecystitis

A

Acute pancreatitis
Perforated peptic ulceration
Intrahepatic abscess
Basal pneumonia
MI

190
Q

Mx of symptomatic gallstones

A

Cholecystectomy
- Admitted with gallstone complications –> perform during admission
- Pain alone –> elective procedure but minimise wait time due to risk of recurrance/complications
Only performed if symptomatic and not for incidental asymptomatic stones

191
Q

Benefits of laparoscopic cholecystectomy

A

Post-op pain minimised
Short period of ileus
Early mobilisation
Day-surgery and cost benefits

192
Q

When is laparoscopic cholecystectomy CI and what is the alternative

A

Open cholecystectomy

Extensive previous upper abdo surgery (adhesions and difficulty IDing anatomy), ongoing bile duct obstruction, portal HTN

193
Q

Mx of acute cholecystitis

A

Initial Mx is conservative: NPO, IV fluids, opiate analgesia, IV antibiotics (IV gentamicin and amoxicillin/ampicillin 2g 6hourly)
Delay cholecystectomy for a few days to allow Sx to settle

If increasing pain and fever/failure of response to conservative mMx consider gangrene/empyema of GB

194
Q

Common bacteria assoc. with acute cholecystitis

A

Escherichia coli
Bacteroides fragilis
Klebsiella spp.
Enterococcus spp.
Pseudomonas spp.

195
Q

What should be done if empyema/gangrene of GB suspected

A

Urgent transabdominal USS or CT
Surgical intervention (or temporary radiologically placed gallbladder drain for empyema esp. if unfit for surgery)

196
Q

Complications of cholecystectomy

A

Biliary leak from cystic duct or gallbladder bed
Injury to bile duct –> bile duct stricture and 2˚ biliary liver injury
Injury to extrahepatic biliary tree (more likely with variant duct anatomy)
.2% mortality
Post-cholecystectomy syndrome: RUQ pain continuing probably due to functional bowel disorder/hepatic flexure spasm or rarely retained stones in CBD or Sphincter of Oddi dysfunction

197
Q

Describe non-surgical Mx of symptomatic gallbladder disease

A

Stone dissolution: pure or near-pure cholesterol stones can be solubilised by increasing bile salt content of bile with longterm ursodeoxycholic acid +/- cholesterol lowering drugs e.g., statins, ezetemibe
Shock-wave lithotripsy: Radiologically or USS directed shock wave to fragment stones (requires patent cystic duct so the fragments can pass) –> high recurrence rate

198
Q

What is ascending cholangitis

A

An ascending bacterial infection of biliary tract facilitated by bile stasis

199
Q

What is the cause of ascending cholangitis

A

Bile stasis MCly due to stone in CBD (or stricture/fluke infection/ERCP/indwelling stent)
Bacteria: E. coli, klebsiella, S. faecalis, enterobacter, bacteroides

200
Q

Presentation of ascending cholangitis

A

CHARCOT TRIAD
Abdominal pain (mostly RUQ)
High fever
Jaundice

REYNOLDS PENTAD
Hypotension
Mental status changes

Tachycardia, features of sepsis, septic shock and MOD

201
Q

Investigations for ascending cholangitis

A

FBC (elevated neutrophils)
CRP and ESR (raised)
LFTs (raised bilirubin, ALP, GGT; mildly elevated AST, ALT)
Lipase (check for stone-related pancreatitis)
PT (may be prolonged if bile duct obstruction has been sustained over several days due to decreased vitK absorption)

Transabdominal USS –> dilatation of intrahepatic biliary radicles
- stones in distal CBD are poorly visualised and often missed
MRCP –> stones in CBD and dilated duct
CT –> exclusion of other CBD obstruction causes (head of pancreas carcinoma)

ERCP+/- endoscopic USS: good visualisation of small calculi in non-dilated ducts and for therapeutic use (stone removal)

202
Q

Management of acute cholangitis

A

High morbidity and mortality

IV antibiotics and urgent bile duct drainage

203
Q

How is bile duct drainage achieved

A

Via ERCP and sphincterotomy –> stones removed by balloon or basket catheters

In severely ill pt a stent can be inserted into bile duct to maintain bile drainage without need to remove stones (minimising procedure time)

Alternative = radiologically placed percutaneous biliary drain

204
Q

Complications of gallstones

A

Acute cholecystitis
Acute cholangitis
Gallstone pancreatitis
Gallstone ileus (biliary enteric fistula as gallstone erodes through GB wall –> moves into intestine and lodges at ileocaecal junction)
Chronic cholecystitis

205
Q

How is acalculous cholecystitis treated

A

IV gentamicin, metronidazole 500mg 12 hourly and amoxicillin/ampicillin 2g 6 hourly
Percutaneous gallbladder drainage or urgent cholecystectomy

206
Q

What is porcelain gallbladder

A

Calcification of gallbladder wall seen on XR or noncontrast CT due to chronic cholecystitis –> increases risk of gallbladder cancer so Tx with laparoscopic cholecystectomy even if asymptomatic

207
Q

How is HCV Dx

A

Acute HCV: LFTs (possible ALT elevation), anti-HCV EIA followed by HCV RNA if positive to confirm HCV viraemia
Chronic HCV:

208
Q

How is acute HCV Tx

A

Pegylated interferon

209
Q

Progression of chronic HCV

A

Asymptomatic
Chronic hepatitis and cirrhosis in 20 years (25%)
Insidious fatigue and raised transaminases

210
Q

Extrahepatic manifestations of HCV

A

Mixed cryoglobulinaemia: presents with palpable purpura
Porphyria cutanea tarda
Polyarthralgia and polyarthritis
Membranoproliferative glomerulonephritis
Lichen planus
Hashimoto’s thyroiditis
B cell lymphoma

211
Q

Mx of HCV

A

General:
HAV and HBV vaccine
Mx chronic liver disease if present
Advise against dangerous alcohol consumption
Aim for healthy bodyweight
Regular HCC surveillance if cirrhotic (6mo USS +/-AFP)

Specific: Antivirals e.g., velpatasvir + sofosbuvir (with or without cirrhosis) or glecaprevir+pibrentasvir (without cirrhosis or renal failure)

212
Q

What is required for HDV infection and what is the result

A

HDV is an RNA virus that only causes infection with HBsAg positivity and thus is prevented with HBV vaccination/immunity

Superinfection –> severe disease and death

213
Q

When should HEV be considered

A

Traveler from low-income countries with acute hepatitis but +ve serology for HAV and HBV

214
Q

What predisposes to fatal fulminant EBV hepatitis

A

T3 of pregnancy

215
Q

In acute hepatitis, in addition to hepatitis viruses what other viruses should be test for

A

CMV IgM –> suggests likely cause of hep
EBV IgM –> suggests likely cause of hep
anti-HIV –> risk of opportunistic hepatobiliary infections
Toxoplasmosis serology –> can cause hep and present with adenopathy and hepatosplenomegaly
Q fever serology –> Q fever can cause hep and pneumonia in combo

216
Q

Histological assessment of liver fibrosis

A

Biopsy histology:

F0, F1 = minimal fibrosis
F2, F3 = advanced fibrosis
F4 = cirrhosis

217
Q

Non invasive assessment of liver fibrosis

A

Serum biomarkers:
APRI: AST to platelet ratio
- APRI < 1 makes cirrhosis unlikely and < 0.7 reduces likelihood of significant fibrosis
Fibrotest: uses a2macrogloubulin, haptoglobin, gammaglobulin, apoplipoprotein a1, GGT and total bilirubin
NFS = NAFLD score using age, BMI, transaminases, platelets and albumin

Elastography uses USS and MRI to measure stiffness of liver correlating with amount of fibrosis
e.g., Fibroscan or MR elastography

218
Q

Causes of acute pancreatitis

A

MC = chronic alcohol abuse, gallstones, ERCP
Steroids/Cushing syndrome, thiazides, OCP, tetracycline, azathiopurine, furosemide, sodium valproate
Mycoplasma, mumps, infectious mononucleosis, molluscum, contagiosum
Autoimmune (IgG4)
Idiopathic
Congenital: CF (CFTR), cationic trypsinogen (PRSSI), SPINK, pancreas divisum (presence of dorsal and ventral ducts which are not fully developed –> impaired drainage and backpressure –> enzymatic activation), MENII
Hypercalcaemia, hypertriglyceridaemia
Trauma (handlebars of bike into epigastrium)
Obstruction of pancreatic duct by cancer or roundworm
Penetrating peptic ulcer
Hypothermia
Scorpion sting
Hyperparathyroidism (w/erosion of terminal phalanges)

219
Q

Symptoms of acute pancreatitis

A

Severe abdominal pain lasting several hours
- epigastric and LUQ radiation to back
- rapid onset (over ~20mins)
N, V (MC = retching; stomach lies anterior and is irritated –> repeated small volume enuresis), anorexia

220
Q

Signs of acute pancreatitis

A

Can become febrile but often not at first
Tender epigastrium
Distension
Signs of retroperitoneal bleeding:
- Cullen’s sign: bruising discolouration around umbilicus indicating haemoperitonum
- Turner’s sign: bruising discolouration of flanks (retroperitoneal blood dissecting along tissue planes)
Hypotension, tachypnoea, hypoxaemia

221
Q

Investigations for acute pancreatitis

A

FBC and differential (leukocytosis; hct > 44% indicates poorer prog)
CRP (elevated)
Lipase (elevated; does not correlate with severity - pancreatic destruction –> lower lipase)
- NB. serum amylase (>3 x normal suggests pancreatitis) falls after 2-3 days while lipase remains elevated for 7-14 days
LFTs (determine whether gallstones are cause)
UEC
Ca (hypercalcaemia can be a cause; hypocalcaemia can be a result and prognostic marker)
Consider TGs

Imaging is not always needed:
Transabdominal USS
- confirms/excludes gallstones
- pancreatic inflammation, peripancreatic stranding, calcifications, fluid collections
AXR
- sentinel loop: 1 isolated loop of SB in epigastrium
- cut-off sign: transverse colon just disappears
- gallstones (if radio-opaque but not common)
Abdominal CT can confirm Dx:
- enlargement of pancreas
- irregular contour
- obliteration of the peri-pancreatic fat
- necrosis
- pseudocysts

222
Q

Other than pancreatitis, why might lipase me raised

A

Anything that irritates retroperitoneum/pancreas

Perforated peptic ulcer (usually posterior perforation into lesser sac)
Perforation of gallbladder
Ruptured AAA
Ruptured ectopic pregnancy
Mesenteric infarction
Afferent loop obstruction (may also cause pancreatitis)

223
Q

Pathophysiology of acute gallstone pancreatitis

A

Bilio-pancreatic reflux (secondary to stone obstruction) –> backpressure initiates autodigestion of pancreas

224
Q

Pathophysiology of acute EToH pancreatitis

A

Acetaldehyde increases acinar cell permeability –> premature activation on wrong side of BM –> autodigestion initiated

225
Q

What scoring system is used to predict severity of acute pancreatitis

A

Ranson criteria
Measure at admission
- Age > 55
- WCC > 16
- BGL > 11.1
- LDH > 350
- AST > 250
Measure at 48 hours
- Hct >/= 10% fall
- Urea >/= 1.8mmol/L rise
- Ca < 2mmol/L
- pO2 < 60mmHg
- Base defecit > 4
- Fluid sequestration > 6L
APACHE
SIRS
Other risk factors include obesity, age, comorbidities

226
Q

Define the criteria for Systemic Inflammatory Response Syndrome

A

2 or more of following:
Temperature > 38.5˚C or < 35˚C
HR > 90bpm
RR > 20bpm or PACO2 < 32mmHg
WCC > 12 or < 4 (or > 10% immature band forms)

227
Q

What are some death markers in pancreatitis

A

Death markers: MODS, elevated creatinine, pleural effusion (ARDS), early non-enhanced CT scan, necrosis

228
Q

Management of acute pancreatitis

A

Resuscitation:
- supplemental O2 via non- rebreather
- PO/IV fluids as tolerated
- Early ID of patients with dropping PO2 –> ventilation
Conservative Mx
- Early enteral nutrition (NGT/NJT)
- No antibiotics unless severe with pancreatic necrosis
- Tx underlying cause e.g., laparoscopic cholecystectomy if gallstones at index admission if mild or within 3 months

229
Q

MCC deaths in acute pancreatitis

A

Early: sepsis
Late: haemorrhage or continuing sepsis

230
Q

Intra-abdominal complications of acute pancreatitis

A

Necrotising pancreatitis
Pancreatic abscess
Pancreatic pseudocyst
SMA/splenic vein/portal vein thrombosis (pancreas wraps around vessels and inflammation causes changes in vessel wall)
Duodenal necrosis (shared blood supply by pancreaticoduodenal arteries)
Splenic artery inflammatory aneurysm

231
Q

Systemic complications of acute pancreatitis

A

Hypovolaemic shock
Septic shock
ARDS
Coagulopathy
Ileus
Jaundice
Hyperglycaemia
Hypocalcaemia
Pancreatic encephalopathy

232
Q

Longterm complications of pancreatitis

A

Chronic pancreatitis
DM (extensive pancreatic tail damage where most islets are)
Malabsorption (pancreatic exocrine insufficiency)
Neuropathy
Opiate addiction

233
Q

Consequences of necrotising pancreatitis

A

50% will have organ failure
50% will have resolution as it evolves into walled off pancreas necrosis
Necrosis can become infected –> consider if patient deteriorates or fails to improve after a week

234
Q

Clinical features of necrotising pancreatitis

A

Intractable pain (due to raised intra-parenchymal pressures within walled off pancreatic necrosis), N, V
Inability to maintain adequate weight
LGF

235
Q

Why is early enteral nutrition important in necrotising pancreatiis

A

Breakdown of gut-mucosal barrier in the absence of enteral nutrition results in translocation of bacteria into necrosed pancreatic tissue

236
Q

What factors are predictive of pancreatic necrosis

A

High Ranson score
High CRP
Areas on CT that don’t perfuse

237
Q

How can infection of pancreatic necrosis be confirmed

A

Gas on imaging
CT-guided FNA

238
Q

Tx of necrotising pancreatiis

A

IV ABx and supportive Mx
Usually conservative Mx to allow body to wall it off
But can do open debridement

239
Q

How does pancreatic abscess present and how is it Dx

A

Fever and shock 4-6 weeks after acute pancreatitis
CT-guided biopsy with MC&S

240
Q

How is pancreatic abscess Tx

A

Immediate surgical debridement, drainage and antibiotics

241
Q

What is a pancreatic pseudocyst

A

Fluid collection rich in pancreatic enzymes into lesser sac/ surrounded by granulation tissue and fibrin

242
Q

Clinical features of pancreatic pseudocyst

A

Persisting pain, tender epigastric mass, elevated lipase

243
Q

DDx of pancreatic pseudocyst

A

cystadenoma, cystadenocarcinoma, IPMT

244
Q

Complications of pancreatic pseudocyst

A

Infection (stasis of fluid)
Rupture –> pancreatic ascites
Haemorrhage presenting as sudden increase in pain due to pressure increase and compression of structures
Obstruction of biliary tree/GIT

245
Q

Mx of pancreatic pseudocyst

A

Expectant in most pts but if >5cm lasting > 6mo it will need Tx

  • Endoscopic: endoscopic cystogastrostomy in which cyst is drained into stomach
  • Radiological: percutaneous catheter-drainage with trans-gastric stents
  • Surgical gastrostomy or Roux-en-Y cystojejunostimy
246
Q

What is chronic pancreatitis

A

Syndrome of progressive, irreversible and destructive inflammatory changes in the pancreas resulting in permanent structural damage and leading to impairment of exocrine and endocrine functions

247
Q

Serum amylase and lipase in chronic pancreatitis

A

Normal/low

248
Q

Causes of chronic pancreatitis

A

MC = chronic alcohol ingestion
Idiopathic
Hereditary: SPINK1, cationic trypsinogen gene (PRSS1)
Cystic Fibrosis
Pancreatic divisum
Autoimmune
Tumour
Hyperparaythroidism

249
Q

Clinical presentation of chronic pancreatitis

A

Initially asymptomatic

Recurrent abdominal pain referred to left or right or radiating to back
If diaphragm involved may have pleuritic pain radiating to shoulder

Progressive exocrine dysfunction –> steatorrhea and diarrhoea
Endocrine dysfunction –> DM

Classic triad = recurrent abdominal pain witn pancreatic calcification, DM, and steatorrhea

250
Q

Investigations for chronic pancreatitis

A

INITIAL
CT abdomen (or MRI) to confirm presence of calcified pancreas
Endoscopic ultrasonography (EUS) can confirm Dx if CT is normal –> EUS guided FNA is required to differentiate from pancreatic cancer
secretin-enhanced MRCP can show abnormal exocrine function
ECRP can precipitate pancreatitis but can be helpful if CBD/pancreatic stones are present and require removal

FURTHER
Genetic testing
ESR, ANA, IgG4
Faecal elastase (low)
Faecal fat (high)
- pt consumes 100g of fat for 3 days
Steatocrit (like faceal fat but not over 72 hours)
Fasting BSL/GTT, HbA1C

Biopsy (inflammation, fibrosis, loss of acini) is confirmatory but poorly tolerated and rarely done

251
Q

What is the prognosis and causes of death in chronic pancreatitis

A

Prognosis: 50% die within 20yrs
Life expectancy reduced 15yrs
Causes of death: respiratory cancers (usually pts are smokers), malnutrition, diabetes complications, suicide, crime, opiate addiction etc

252
Q

Complications of chronic pancreatitis

A

Exocrine dysfunction –> steatorrhea, fat malabsorption, vit ADEK B12 deficiency
Endocrine dysfunction (> 80% pancreas destroyed)
Pseudocysts (2˚ to ductal disruptions)
Dudodenal and bile duct obstruction
Pancreatic ascites and pleural effusion (2˚ to disruption of pancreatic duct and fistula formatuon bw chest/abdomen or rupture of pseudocyst with tracking of pancreatic fluid into abdo cavity)
Splenic vein thrombosis 2˚ splenic vein inflammation
Pseudoaneurysm of vessels close to pancreas

253
Q

Treatment of chronic pancreatiti

A

Management of exocrine and endocrine dysfunction
- Replacement of pancreatic enzymes by oral pancreatic enzymes (lipase with each meal)
- PPI to prevent gastric acid from breaking down enzymes
- Mx of DM (insulin)
- Chronic pain Mx (consider endoscopic therapy or nerve block)

254
Q

Autoimmune pancreatitis type 1 is associated with

A

IgG4, M > F, involvement of other organs (kidneys, LNs, retroperitoneum)

255
Q

Autoimmune pancreatitis type 2 is associated with

A

IBD

256
Q

Tx of autoimmune pancreatitis

A

Corticosteroids

257
Q

Presentation of autoimmune pancreatitis

A

Obstructive jaundice

258
Q

Name benign pancreatic cysts

A

Serous cystadenoma
Pseudocyst

259
Q

Name potentially malignant pancreatic cysts

A

Intraductal papillary mucinous neoplasms: main duct (high risk), branch duct (low-moderate risk)

Mucinous cystic neoplasms (females, mdoerate risk)

Solid pseudopapillary neoplasms (moderate - high risk)

260
Q

How should potentially malignant pancreatic cysts be investigated

A

MRCP - if uncertain but lesion is worrying consider endoscopic USS
Refer main duct IPMN, MCN or SPN for consideration of resection

261
Q

Causes of hepatomegaly

A

Apparent
1. Low lying diaphragm
2. Riedel’s lobe
3. Early cirrhosis
Inflammation
1. Hepatitis (HAV, HBV, HCV, EBV, CMV, HSV, autoimmune etc.)
2. Schistosomiasis
3. Hepatic abscess (pyogenic or amoebic)
Cysts
1. Hydatid cyst
2. Polycystic liver
Metabolic
1. Fatty liver
2. Glycogen storage disease
3. Amyloid deposition/sarcoidosis
4. Wilsons disease
5. Hereditary haemochromatosis
Haematological
1. Leukaemia
2. Lymphoma
3. Myeloproliferative disorders
4. Thalassaemia/sickle cell disease/malaria and other haemolytic anaemias
Tumours
1. Primary or secondary tumours
Venous congestion
1. Right heart failure
2. Constrictive pericarditis
3. Hepatic vein occlusion/thrombosis
Biliary obstruction esp. extrahepatic

262
Q

Investigations for hepatomegaly

A
  • FBC + blood film, UEC, LFTs, CRP/ESR, coags
  • USS abdomen
  • Additional tests based on likely cause:
    o IgM HAV, HBsAg, IgM anti-HBc, anti-HCV, anti-HEV
    o CMV, EBV, HSV
    o ANA, ASMA, LKM, AMA, Igs
    o Ferritin
    o Plasma copper, urine copper, caeruloplasmin
    o Alpha-fetoprotein
    o MRCP etc.

General Medicine (3 decks)

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