Common Liver diseases in the Emergency Department

1. Dr. A. Barai
MBBS, MRCS, MSc (Critical Care)
Registrar in Emergency Medicine
LIVER EMERGENCIES

2. • Hepatobiliary system emergencies, although not that
common in the ED, can be life threatening conditions
that demand early intervention.
• Complications of the conditions can be the main
reason for ED presentation.
• Main focus is for early recognition and treatment.

3. ACUTE LIVER FAILURE

4. • Incidence:
– 2000 cases/year in USA
– 200-300 transplants
• Duration of symptoms
– Median 6 days (0-74)
• Jaundice to encephalopathy:
– Median 2 days (0-61)
• Disposition:
– 93% in 3 weeks.
Acute Liver Failure Group: Ostapowicz et al, Ann Int Med 2002Acute Liver Failure Group: Ostapowicz et al, Ann Int Med 2002

5. Definitions
• Liver failure:
Failure of hepatic synthetic and metabolic function.
• Fulminant hepatic failure:
Acute liver failure with encephalopathy (within 8
weeks) with a previously normal liver.
• Acute liver failure:
Liver failure + encephalopathy within 26 weeks of
illness
• Chronic liver failure:
Liver failure without encephalopathy
• Acute on chronic liver failure:
Liver failure with the development of encephalopathy

6. • Hyperacute liver failure:
– Presents within 7 days of onset.
– 36% survival with medical management alone
(single most common cause in UK and USA is
Paracetamol poisoning).
• Subacute liver failure:
– Presents from 29-72 days,
– Less likely to get cerebral oedema, but more likely
to have ascites.
– Poorer 14% survival.
Acute liver failure

7. • Sleep disturbance
• Asterixis
• Hyperreflexic
• Can be hemiplegic
Hepatic encephalopathy

9. • Acute on Chronic Liver Failure:
– Acute deterioration in liver function over days to weeks in
patients with pre-existing chronic liver disease (CLD).
– Poor prognosis from underlying cirrhosis and end-stage
liver disease (ESLD) with portal hypertension, ascites and
multi-organ failure.
– Much more common than ALF.
– Features include jaundice, coagulopathy, encephalopathy
(precipitated by sepsis including spontaneous bacterial
peritonitis, or GI bleed, alcohol, constipation,
hypokalaemia, and drugs including NSAIDs and sedatives),
hepatorenal syndrome and hepatopulmonary syndrome.

10. • Viral hepatitis
• Drug induced hepatitis:
• Other causes
Aetiology

12. • Multi-organ failure
• Encephalopathy
– cerebral edema
– CNS ammonia
• Infection
• Coagulopathy
• Hypoglycemia
Complications

13. • Bloods: INR, LFT, FBC, UEC
• USS
• CXR
Investigations

14. • General supportive:
– Hospitalize if INR >1.5;
– IPPV for Grade 3 or 4 coma or respiratory failure,
– Invasive monitoring including ICP monitor (ICP <
25 mmHg) +/- jugular bulb O2
– Infusion 5-10% dextrose (watch for
hyponatraemia),
– Fluids and vasopressor/ noradrenalin therapy.
– GI bleeding prophylaxis.
Management

15. • Specific to complications:
– Encephalopathy with cerebral oedema. Correct
avoidable factors (hypoxia, sepsis, hyperthermia,
hemorrhage, hypokalaemia, benzodiazepines),
– Monitor ICP early. Give mannitol 0.5 g/kg if ICP ≥
25 mmHg, or hypertonic saline 7.5% boluses 2.0
mL/kg.
– Lactulose and neomycin appear not to work, and
have complications such as aspiration and
nephrotoxicity, respectively.
Management

16. • Infection. Daily surveillance for bacterial (S.aureus,
S.pneumoniae and E.coli) and fungal (Candida) infections,
including primary peritonitis. Empiric and or prophylactic
broad-spectrum antibiotics + antifungals given.
• Haemodynamic failure including acute oliguric renal failure.
Epoprostenol (PGI2), angiotensin, vasopressors, NOS
antagonists.
• Coagulopathy. Vit K 10 mg IV; FFP / platelets for active
bleeding; recombinant Factor VIIa (rFVIIa) with FFP – use
declining + many contraindications.
• N. acetylcysteine: IV for paracetamol poisoning, even if
ingested 48-72 hours before.
• Orthotopic liver transplantation (OLT).
• Liver support systems. ‘Bridging support’ to transplantation,
but no convincing outcome efficacy data yet
Management

17. • Resuscitation:
A – Intubated if unresponsive from encephalopathy
(RSI to prevent aspiration)
B – May have respiratory failure from pleural
effusions and may have aspirated requiring
mechanical ventilation
C – Fluid maintenance, often have a hyperdynamic
circulation, vasoactive medication
D – Monitoring for intra-cranial hypertension: ICP
bolt, mannitol, propofol, thiopentone, moderate
hypothermia (32-33 C), hypertonic saline
• Once stabilized early consultation with Liver
Transplant Centre
• Vigilant monitoring for infection (bacterial, fungal)
Management of Fulminant HF

18. Specific treatment

19. • Encephalopathy:
— Lactulose -> increases ammonia elimination
— Metronidazole -> alter gut flora to decrease
ammonia production
— Flumazenil (controversial)
• Coagulopathy:
– Only treat with FFP if bleeding or prior to procedures
– FVIIa safe and effective
– NAC: continue until encephalopathy resolves
• TIPS procedure (Transjugular Intrahepatic Portosystemic Shunt)
• Short-term extracorporeal hepatic support MARS
(Molecular Adsorbent Recirculation System)
Specific treatment

20. Specific treatment: TIPS

21. Specific treatment: MARS

22. Liver transplant

24. • Paracetamol induced fulminant hepatic failure
pH < 7.3 or INR > 6.5 (PT > 100s)
+
Cr > 300micromol/L
+
grade III or IV encephalopathy
• Non-Paracetamol induced fulminant hepatic failure
INR > 6.5 (PT > 100s) or any 3 of the following variables:
(1) age < 10 or > 40 yrs
(2) aetiology – non A, non B hepatitis, halothane hepatitis,
idiosyncratic drug reactions
(3) duration of jaundice before encephalopathy > 7 days
(4) INR > 3.5 (PT > 50s)
(5) bilirubin > 300micromol/L
King’s College Criteria

25. Ostapowicz et al, Ann Int Med 2002 (US Acute Liver Failure Study Group)
308 ALF patients
136 (44%)
Listed for Transplant
30
Died on list
17
Removed from list
89 (65%)
Transplanted
14
Dead
75
Alive
10
Alive
7
Dead

26. SPONTANEOUS BACTERIAL
PERITONITIS

28. • Spontaneous bacterial peritonitis (SBP) is
defined as an ascitic fluid infection without an
evident intra-abdominal surgically treatable
source.
Definitions

29. • Spontaneous bacterial peritonitis occurs in both
children and adults
• It’s well-known and ominous complication in patients
with cirrhosis.
• Poor long-term prognosis.

30. • In patients with ascites, the frequency may be as
high as 18%.
• This number has grown from 8% over the past 2
decades, most likely secondary to an increased
awareness of spontaneous bacterial peritonitis and a
lowered threshold to perform diagnostic
paracentesis.
• No race predilection is known for spontaneous
bacterial peritonitis.
• In patients with ascites, both sexes are affected
equally.
Epidemiology

31. • Traditionally, 75% of spontaneous bacterial
peritonitis infections have been caused by aerobic
gram-negative organisms (50% of these being
Escherichia coli).
• The remainder has been due to aerobic gram-
positive organisms (19% streptococcal species).
Aetiology

32. Cholongitas E, Papatheodoridis GV, Lahanas A, Xanthaki A, Kontou-Kastellanou C, Archimandritis AJ. Increasing
frequency of Gram-positive bacteria in spontaneous bacterial peritonitis. Liver Int. Feb 2005;25(1):57-61
• Some data suggest that the percentage of gram-
positive infections may be increasing.
• One study cites a 34.2% incidence of Streptococci,
ranking in second position after Enterobacteriaceae.
• Viridans group streptococci (VBS) accounted for
73.8% of these streptococcal isolates.
• Anaerobic organisms are rare because of the high
oxygen tension of ascitic fluid.
• A single organism is noted in 92% of cases, and 8% of
cases are polymicrobial.

33. • The mortality rate ranges from 40-70% in adult
patients with cirrhosis.
• Rates are lower in children with nephrosis.
• Patients with concurrent renal insufficiency have
been shown to be at a higher risk of mortality.
• Mortality may be decreasing among all subgroups of
patients because of advances in its diagnosis and
treatment.
Prognosis

34. • Fever and chills occur in as many as 80% of patients.
• Abdominal pain or discomfort is found in as many as
70% of patients.
• Other signs and symptoms may include the
following:
– Worsening or unexplained encephalopathy
– Diarrhea
– Ascites that does not improve following administration of
diuretic medication
– Worsening or new-onset renal failure
– Paralytic Ileus
Clinical presentation

35. • Bloods: CRP, UEC, FBC
• Blood culture: Positive in 33% cases
• Urine culture
• Chest xray
• Abdominal xray
• Diagnostic paracentesis
• Ultrasound scans
• CT abdomen
Investigations

36. American Association for the Study of Liver Diseases
(AASLD) guidelines: depending on peritoneal fluid PMN counts
• PMN counts of 250 cells/µL or greater in a
community-acquired setting (in the absence of
recent beta-lactam antibiotic exposure) should
receive empiric antibiotic therapy (eg, an intravenous
third-generation cephalosporin, preferably
cefotaxime 2 g every 8 hours).
• PMN counts of 250 cells/µL or more in a nosocomial
setting or patients who have recently received beta-
lactam antibiotics should receive empiric antibiotic
therapy based on local susceptibility testing of
bacteria.
Management

37. – Alernatively, Ofloxacin 400mg BD
– Contraindications:
• Prior exposure to quinolones
• Vomiting
• Shock
• Grade II (or higher) hepatic encephalopathy
• Serum creatinine greater than 3 mg/dL
Management

38. • PMN count greater than 500 cells/µL should
universally be admitted and treated for spontaneous
bacterial peritonitis, regardless of peritoneal fluid
Gram stain result. Antibiotics should be initiated as
soon as possible.
Management

39. • PMN count below 250 cells/µL, management
depends upon the results of ascitic fluid cultures.
– All symptomatic patients should be admitted.
– Patients whose culture results are positive should be
treated for spontaneous bacterial peritonitis.
– A select subset of patients who are completely
asymptomatic yet have positive culture results may be
managed without treatment but must undergo a follow-up
paracentesis within 24-48 hours.
Management

40. • All symptomatic patients with a peritoneal fluid PMN
count of 250-500 cells/µL should be admitted and
treated for spontaneous bacterial peritonitis.
Management

42. BUDD CHIARI SYNDROME

43. • Budd-Chiari syndrome is an uncommon condition
induced by thrombotic or nonthrombotic obstruction
of hepatic venous outflow and characterized by
hepatomegaly, ascites, and abdominal pain.
• Incidence: 1 in a million
• The prognosis is poor in patients with Budd-Chiari
syndrome who remain untreated, with death
resulting from progressive liver failure in 3 months to
3 years from the time of diagnosis

44. • Hematologic disorders
• Inherited thrombotic diathesis
• Pregnancy and postpartum[11]
• Oral contraceptives
• Chronic infections
• Chronic inflammatory diseases
• Tumors
• Congenital membranous obstruction
• Hepatic venous stenosis
• Hypoplasia of the suprahepatic veins
• Postsurgical obstruction
• Posttraumatic obstruction
• Total parenteral nutrition (TPN): Budd-Chiari syndrome has been
reported as a complication of TPN via an IVC catheter in a neonate
Aetiology

45. • Polycythemia rubra vera
• Paroxysmal nocturnal hemoglobinuria
• Unspecified myeloproliferative disorder
• Antiphospholipid antibody syndrome
• Essential thrombocytosis
Haematological disrorders

46. • Protein C deficiency
• Protein S deficiency
• Antithrombin III deficiency
• Factor V Leiden deficiency
Coagulopathy

47. Chronic infections
• Hydatid cysts
• Aspergillosis
• Amebic abscess
• Syphilis
• Tuberculosis
Chronic inflammatory diseases
• Behçet disease
• Inflammatory bowel disease
• Sarcoidosis
• Systemic lupus erythematosus
• Sjögren syndrome
• Mixed connective-tissue disease
Infections and inflammations

48. • Ascitic fluid provides useful clues to the diagnosis, including
the following
• Patients usually have high protein concentrations (>2 g/dL);
this may not be present in persons with the acute form of
Budd-Chiari syndrome
• The white blood cell (WBC) count is usually less than 500/µL
• The serum ascites–albumin gradient is usually less than 1.1
(except in the acute forms of the disease)
• Routine biochemical test results are usually nonspecific in
Budd-Chiari syndrome, although mild elevations in serum
aminotransferase and alkaline phosphatase levels are present
in 25-50% of patients.
Investigations

49. • Ultrasound scan
• CT scan
• MRI scan
• Venography
Imaging techniques

50. Ultrasound scan

51. CT scan

52. Venography

53. • Medical therapy can be instituted for short-
term, symptomatic benefit, the use of such
treatment alone is associated with a high 2-
year mortality rate (80-85%).
• Diuretics,
• Anticoagulants, and
• Thrombolytics
Management

54. • Anticoagulant therapy:
– Enoxaperine
– Warfarin: INR 2-3
• Thrombolytic therapy:
– Urokinase
– Alteplase
• Interventional radiology
• Variceal treatment
Management

55. • Paracentesis
• Portal decompression: Decompression of the hepatic
vasculature should be offered if portal hypertension
is the cause of the symptoms. Either surgery or a
transjugular intrahepatic portosystemic shunt (TIPS)
procedure can be performed
• Liver transplantation
Management

56. • Jalan R, Williams R, Bernuau J. Paracetamol: are therapeutic doses entirely
safe? Lancet 2006; 368: 2195-6. (Editorial)
• Stravitz R, Kramer A, Davern T et al. Intensive care of patients with acute
liver failure: Recommendations of the US Acute Liver Failure Study Group.
Crit Care Med 2007;35:2498-2508.
• O’Grady JG. Acute liver failure. Postgrad Med J 2005; 81:148-54.
• Lai W, Murphy N. Management of acute liver failure. Cont Educ Anaes, Crit
Care & Pain 2004; 4: 40-43.
• Cadogan, M. Acute liver failure: lecture notes. Life in the fats lane. 2015.
[Online]. URL: http://lifeinthefastlane.com/aftb-lecture-notes-liver-failure/
• Macnaughtan J, Thomas H. Liver failure at the front door. Clinical Medicine
2010;10:73-8.
• Bailey C, Hern H. Hepatic failure: An evidence-based approach in
the emergency department. Emergency Medicine Practice 2010;12(4):1-24.
• Roy, PK. and Anand, BS. Budd Chiari syndrome. Medscape. 2015 [Online].
URL: http://emedicine.medscape.com/article/184430-overview.
References

57. Thank you.