Fulminant hepatic failure (FHF)

DEFINITION

rapid development of severe acute liver injury

with impaired function and encephalopathy in previously normal liver or well compensated liver disease

Fulminant hepatic failure (FHF)

encephalopathy within 8 wks previous healthy liver

encephalopathy within 2 wks of developing jaundice with previous underlying liver dysfunction

แบ่�งเป็�น 2 ระยะ

FHF > 8 wks - 6 months

Subfulminant hepatic failure (sub FHF)

cerebral edema is common in FHF and rare in sub FHF

renal failure and portal hypertension are more frequently with sub FHF

FHF VS sub FHF

Reverse altered mental and neuromotor function

Associated acute or chronic liver disease

Hepatic Encephalopathy

Pathophysiology

causes

cause พบร้�อยละacetaminophen over dose in determineidiosyncratic drug reaction viral hepatitis A, B

39171312

The Acute liver Failure Study Group

พ.ศ.2541- 2544

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

causes

Toxins

causes

Vascular

portal vein thrombosisBudd-Chiari syndrome (hepatic vein

thrombosis)veno-occlusive diseaseischemic hepatitis

causes

Metabolic

Wilson's diseaseacute fatty liver of pregnancyReye's syndrome

causes

Miscellaneous

malignant infiltration of the liver,heat strokesepsisautoimmune hepatitis.

causes

Acute liver failurenonspecific symptomsmalaise nauseaJaundiceEcchymosesEtc…..

Sign and symptom

Hepatic encephalopathyAges 10–60 yearsHandwriting and hand coordination

deteriorate in stages 1 and 2Asterixis prominent in stage 2Reflexes symmetrically hyperactive in stage 3Mental and neurologic signs change rapidly

(over 6–12 hours)

Sign and symptom

Age >60Signs of underlying liver disease diminish

(25%)Confusion more prominentPrecipitating GI hemorrhage or infection less

often identifiedRemains in stage 1 or 2 for many daysProgression slower

Sign and symptom

Age <10Signs of underlying liver disease prominent;

usually FHF or extremely advanced cirrhosisProgression through the stages very rapid,

often 6–12 hoursWilson disease can imitate HE

Sign and symptom

Hepatic encephalopathy

Sign and symptom

http://emedicine.medscape.com/article/177354-clinical#showall

Hepatic encephalopathyCerebral edemaSepsisRenal failureCirculatory dysfunctionCoagulopathyGastrointestinal bleedingPulmonary complicationMetabolic disturbance เช่�น metabolic acidosis,

hypoglycemia, hypophosphatemia

FHF complication

Diagnosis

The clinical setting and findings diagnosis in 80% of the cases.The treatment response often confirms the diagnosis

HematologyCBC, coagulation

Biochemistry, blood gasblood glucose, BUN, creatinine, electrolyte, LFT

Blood gasVirological markers

Hepatitis profile (A, B, C, delta)Microbiology

HemocultureSputum / urine culture

Electroencephalogram (EEG)

Lab investigation

Specific treatment Liver transplant Stage 3 and 4

Complication treatmentHepatic encephalopathyCerebral edemaSepsisRenal failureCirculatory dysfunctionCoagulopathyGastrointestinal bleedingPulmonary complicationMetabolic disturbance

Treatment

Hepatic encephalopathy (HE) ammonia within the gut lumenConcept ammonia precipitating factors IGSCALP

Restrict Protein diet = 40-70 g/daynon-absorbable disaccharides = lactuloseantibiotic = rifaximin, neomycin,

metronidazole, vancomycin

Complication treatment

First Line50% 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

goodMetronidazole and vancomycin are alternative antibiotics.Antacids as needed

Second LineFlumazenil (benzodiazepine antagonist)

Cerebral edemaastrocyte edemaIICP and brainstem herniation, most common

causes of death classic signs IICP include Cushing's triad and Neurologic manifestations hypertonic,

hyperreflexia, and altered pupillary responses

Complication treatment

Treatment cerebral edemaControl ICP < 20 mmHg and CPP > 50

mmHgEnv. with minimal sensory stimulationelevate head position prevent overhydrationif ICP > 20 mmHg Hyperventilation

PCO2 < 25 mmHg if no response use hyperosmotic agents

manitol 0.5 - 1 g/kgIf no response use pentobarbitone 3-5

mg/kg IV

Complication treatment

1. Liver transplant 2. Liver recovery

2.1 Grade ของ encephalopathy(50)Grade I-II recovery 65-70Grade III 40-50Grade IV < 20

2.2 Age ถ้�าอาย�น�อยกว่�า 10 ป็� หร�อมากกว่�า 40 ป็�โอกาสฟื้�� นคื�นก!น�อยลง

2.3 cause FHF เช่�น acetaminophen >idiosyncratic drug reactions > Wilson’s disease

prognosis

รศ.พญ. ว่$ฒนา ส�ข&ไพศาลเจร)ญ. Acute liver failure. ภาคืว่)ช่าอาย�รศาสตร, คืณะแพทยศาสตร, มหาว่)ทยาล$ย

ขอนแก�นhttp://

emedicine.medscape.com/article/186101-overview#showall

http://www.sciencedirect.com/science/article/pii/S1357272502003965

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 = infectionG = GI bleedingS = sedationC = constipationA = alkalosisL = low KP = protein high

IGSCALP