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DEFINITION
 rapid development of severe acute liver injury
with impaired function and encephalopathy
in previously normal liver or well compensated
liver disease
 encephalopathy within 8 wks previous
 healthy liver

 encephalopathy within 2 wks of developing
 jaundice with previous underlying liver
 dysfunction
 FHF > 8 wks - 6 months
 cerebral edema is common in FHF and rare
  in sub FHF
 renal failure and portal hypertension are
  more frequently with sub FHF
 Reverse altered mental and neuromotor function
 Associated acute or chronic liver disease
cause
acetaminophen over dose       39
in determine                  17
idiosyncratic drug reaction   13
viral hepatitis A, B          12

                                   -
Hepatitis viruses

 hepatitis A most common acute viral hepatitis but
  rare for acute infection to progress to ALF

 Hepatitis B most common viral cause ALF
Toxins
Vascular

 portal vein thrombosis
 Budd-Chiari syndrome (hepatic vein thrombosis)
 veno-occlusive disease
 ischemic hepatitis
Metabolic

 Wilson's disease
 acute fatty liver of pregnancy
 Reye's syndrome
Miscellaneous

 malignant infiltration of the liver,
 heat stroke
 sepsis
 autoimmune hepatitis.
Acute liver failure
 nonspecific symptoms
 malaise
 nausea
 Jaundice
 Ecchymoses
 Etc…..
Hepatic encephalopathy
Ages 10–60 years
 Handwriting and hand coordination deteriorate in
  stages 1 and 2
 Asterixis prominent in stage 2
 Reflexes symmetrically hyperactive in stage 3
 Mental and neurologic signs change rapidly (over 6–12
  hours)
Age >60
 Signs of underlying liver disease diminish (25%)
 Confusion more prominent
 Precipitating GI hemorrhage or infection less often
  identified
 Remains in stage 1 or 2 for many days
 Progression slower
Age <10
 Signs of underlying liver disease prominent; usually
  FHF or extremely advanced cirrhosis
 Progression through the stages very rapid, often 6–12
  hours
 Wilson disease can imitate HE
Hepatic encephalopathy




 http://emedicine.medscape.com/article/177354-clinical#showall
 Hepatic encephalopathy
 Cerebral edema
 Sepsis
 Renal failure
 Circulatory dysfunction
 Coagulopathy
 Gastrointestinal bleeding
 Pulmonary complication
 Metabolic disturbance  metabolic acidosis,
 hypoglycemia, hypophosphatemia
The clinical setting and findings diagnosis in 80% of
the cases.
The treatment response often confirms the diagnosis
 Hematology
   CBC, coagulation
 Biochemistry, blood gas
   blood glucose, BUN, creatinine, electrolyte, LFT
 Blood gas
 Virological markers
   Hepatitis profile (A, B, C, delta)
 Microbiology
   Hemoculture
   Sputum / urine culture
 Electroencephalogram (EEG)
 Specific treatment     Liver transplant Stage 3 and 4
 Complication treatment
   Hepatic encephalopathy
   Cerebral edema
   Sepsis
   Renal failure
   Circulatory dysfunction
   Coagulopathy
   Gastrointestinal bleeding
   Pulmonary complication
   Metabolic disturbance
Hepatic encephalopathy (HE)
   ammonia within the gut lumen
 Concept ammonia
          precipitating factors              IGSCALP

 Restrict Protein diet = 40-70 g/day
 non-absorbable disaccharides = lactulose
 antibiotic = rifaximin, neomycin, metronidazole,
 vancomycin
First Line
 50% Lactulose syrup 30–60 mL PO 4 times daily when ≥3
  bowel movements occur daily.
 Lactulose enema 300 mL plus 700 mL tap water

 If worsening or no improvement in 2 days, add antibiotics:
 Rifaximin: 400 mg 3 times a day
 Neomycin: 1–2 g per day divided q6–8h, if renal status is
  good
 Metronidazole and vancomycin are alternative antibiotics.
 Antacids as needed

Second Line
 Flumazenil (benzodiazepine antagonist)
Cerebral edema
 astrocyte edema
 IICP and brainstem herniation, most common causes
  of death
 classic signs IICP include Cushing's triad and
 Neurologic manifestations hypertonic, hyperreflexia,
  and altered pupillary responses
Treatment cerebral edema
      Control ICP <   mmHg and CPP >        mmHg
   Env. with minimal sensory stimulation
   elevate head position
   prevent overhydration
   if ICP >   mmHg       Hyperventilation
    PCO2 <      mmHg if no response use
    hyperosmotic agents
   manitol 0.5 - 1 g/kg
   If no response use pentobarbitone 3-5 mg/kg IV
1. Liver transplant
    Liver recovery
        2.1 Grade      encephalopathy(50)
               Grade I-II recovery 65-70
               Grade III           40-50
               Grade IV            < 20
             Age

          cause FHF       acetaminophen >
idiosyncratic drug reactions > Wilson’s disease
                             Acute liver failure.


 http://emedicine.medscape.com/article/186101-
  overview#showall
 http://www.sciencedirect.com/science/article/pii/S135
  7272502003965
 Eric Goldberg, Sanjiv Chopra. Acute liver failure:
  Definition and etiology. Uptodate . Mar 2010
 Eric Goldberg, Sanjiv Chopra. Acute liver failure:
  Prognosis and management. Dec 2010
 I = infection
 G = GI bleeding
 S = sedation
 C = constipation
 A = alkalosis
 L = low K
 P = protein high

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Fulminant hepatic failure (fhf)

  • 1.
  • 3.  rapid development of severe acute liver injury with impaired function and encephalopathy in previously normal liver or well compensated liver disease
  • 4.  encephalopathy within 8 wks previous healthy liver  encephalopathy within 2 wks of developing jaundice with previous underlying liver dysfunction
  • 5.  FHF > 8 wks - 6 months
  • 6.  cerebral edema is common in FHF and rare in sub FHF  renal failure and portal hypertension are more frequently with sub FHF
  • 7.  Reverse altered mental and neuromotor function  Associated acute or chronic liver disease
  • 8.
  • 9.
  • 10.
  • 11. cause acetaminophen over dose 39 in determine 17 idiosyncratic drug reaction 13 viral hepatitis A, B 12 -
  • 12. Hepatitis viruses  hepatitis A most common acute viral hepatitis but rare for acute infection to progress to ALF  Hepatitis B most common viral cause ALF
  • 14. Vascular  portal vein thrombosis  Budd-Chiari syndrome (hepatic vein thrombosis)  veno-occlusive disease  ischemic hepatitis
  • 15. Metabolic  Wilson's disease  acute fatty liver of pregnancy  Reye's syndrome
  • 16. Miscellaneous  malignant infiltration of the liver,  heat stroke  sepsis  autoimmune hepatitis.
  • 17. Acute liver failure  nonspecific symptoms  malaise  nausea  Jaundice  Ecchymoses  Etc…..
  • 18. Hepatic encephalopathy Ages 10–60 years  Handwriting and hand coordination deteriorate in stages 1 and 2  Asterixis prominent in stage 2  Reflexes symmetrically hyperactive in stage 3  Mental and neurologic signs change rapidly (over 6–12 hours)
  • 19. Age >60  Signs of underlying liver disease diminish (25%)  Confusion more prominent  Precipitating GI hemorrhage or infection less often identified  Remains in stage 1 or 2 for many days  Progression slower
  • 20. Age <10  Signs of underlying liver disease prominent; usually FHF or extremely advanced cirrhosis  Progression through the stages very rapid, often 6–12 hours  Wilson disease can imitate HE
  • 22.  Hepatic encephalopathy  Cerebral edema  Sepsis  Renal failure  Circulatory dysfunction  Coagulopathy  Gastrointestinal bleeding  Pulmonary complication  Metabolic disturbance metabolic acidosis, hypoglycemia, hypophosphatemia
  • 23. The clinical setting and findings diagnosis in 80% of the cases. The treatment response often confirms the diagnosis
  • 24.  Hematology  CBC, coagulation  Biochemistry, blood gas  blood glucose, BUN, creatinine, electrolyte, LFT  Blood gas  Virological markers  Hepatitis profile (A, B, C, delta)  Microbiology  Hemoculture  Sputum / urine culture  Electroencephalogram (EEG)
  • 25.  Specific treatment Liver transplant Stage 3 and 4  Complication treatment  Hepatic encephalopathy  Cerebral edema  Sepsis  Renal failure  Circulatory dysfunction  Coagulopathy  Gastrointestinal bleeding  Pulmonary complication  Metabolic disturbance
  • 26. Hepatic encephalopathy (HE)  ammonia within the gut lumen  Concept ammonia precipitating factors IGSCALP  Restrict Protein diet = 40-70 g/day  non-absorbable disaccharides = lactulose  antibiotic = rifaximin, neomycin, metronidazole, vancomycin
  • 27. First Line  50% Lactulose syrup 30–60 mL PO 4 times daily when ≥3 bowel movements occur daily.  Lactulose enema 300 mL plus 700 mL tap water  If worsening or no improvement in 2 days, add antibiotics:  Rifaximin: 400 mg 3 times a day  Neomycin: 1–2 g per day divided q6–8h, if renal status is good  Metronidazole and vancomycin are alternative antibiotics.  Antacids as needed Second Line  Flumazenil (benzodiazepine antagonist)
  • 28. Cerebral edema  astrocyte edema  IICP and brainstem herniation, most common causes of death  classic signs IICP include Cushing's triad and  Neurologic manifestations hypertonic, hyperreflexia, and altered pupillary responses
  • 29. Treatment cerebral edema Control ICP < mmHg and CPP > mmHg  Env. with minimal sensory stimulation  elevate head position  prevent overhydration  if ICP > mmHg Hyperventilation PCO2 < mmHg if no response use hyperosmotic agents  manitol 0.5 - 1 g/kg  If no response use pentobarbitone 3-5 mg/kg IV
  • 30. 1. Liver transplant Liver recovery 2.1 Grade encephalopathy(50) Grade I-II recovery 65-70 Grade III 40-50 Grade IV < 20 Age cause FHF acetaminophen > idiosyncratic drug reactions > Wilson’s disease
  • 31. Acute liver failure.  http://emedicine.medscape.com/article/186101- overview#showall  http://www.sciencedirect.com/science/article/pii/S135 7272502003965  Eric Goldberg, Sanjiv Chopra. Acute liver failure: Definition and etiology. Uptodate . Mar 2010  Eric Goldberg, Sanjiv Chopra. Acute liver failure: Prognosis and management. Dec 2010
  • 32.  I = infection  G = GI bleeding  S = sedation  C = constipation  A = alkalosis  L = low K  P = protein high