Hepatic failure: Difference between revisions
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*[[Acute liver failure]] (Hyperacute and subacute) is a rare but serious clinical syndrome characterized by the sudden loss of hepatic function in a person without evidence of preexisting liver disease (Exceptions to this definition include Wilson's disease, reactivation of hepatitis B virus infection, and autoimmune hepatitis)<ref name="pmid22675278">{{cite journal |vauthors=Patton H, Misel M, Gish RG |title=Acute liver failure in adults: an evidence-based management protocol for clinicians |journal=Gastroenterol Hepatol (N Y) |volume=8 |issue=3 |pages=161–212 |date=March 2012 |pmid=22675278 |pmc=3365519 |doi= |url=}}</ref> | *[[Acute liver failure]] (Hyperacute and subacute) is a rare but serious clinical syndrome characterized by the sudden loss of hepatic function in a person without evidence of preexisting liver disease (Exceptions to this definition include Wilson's disease, reactivation of hepatitis B virus infection, and autoimmune hepatitis)<ref name="pmid22675278">{{cite journal |vauthors=Patton H, Misel M, Gish RG |title=Acute liver failure in adults: an evidence-based management protocol for clinicians |journal=Gastroenterol Hepatol (N Y) |volume=8 |issue=3 |pages=161–212 |date=March 2012 |pmid=22675278 |pmc=3365519 |doi= |url=}}</ref> | ||
*Neurological complications, Abnormal hemostasis, and bleeding complications, Multiorgan failure,infection.<ref name="pmid27540338">{{cite journal |vauthors=Munoz SJ |title=Complications of Acute Liver Failure |journal=Gastroenterol Hepatol (N Y) |volume=10 |issue=10 |pages=665–8 |date=October 2014 |pmid=27540338 |pmc=4988224 |doi= |url=}}</ref> | |||
==Prognosis== | ==Prognosis== |
Revision as of 05:38, 31 July 2020
Hepatic failure | |
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Synonyms and keywords: Liver failure; fulminating hepatic failure
Overview
Liver failure is the inability of the liver to perform its normal synthetic and metabolic function as part of normal physiology.
Historical Perspective
- The original definition of Acute liver failure by Trey and Davidson was in 1959.[1]
- In the late 1980s and early 1990s, more terminologies of Acute liver failure proposed in.[2][3]
- Term of Acute-on-chronic Liver failure suggested by Jalan and willimas in 2002.[4]
Classification
Three forms are recognized:
- Acute liver failure - development of hepatic encephalopathy (confusion, stupor and coma) and decreased production of proteins (such as albumin and blood clotting proteins) within four weeks of the first symptoms (such as jaundice) of a liver problem. "Hyperacute" liver failure is said to be present if this interval is 7 days or less, while "subacute" liver failure is said to be present if the interval is 5-12 weeks.[3]
- Chronic liver failure - usually occurs in the context of cirrhosis, itself potentially the result of many possible causes, such as excessive alcohol intake, hepatitis B or C, autoimmune, hereditary and metabolic causes (such as iron or copper overload or non-alcoholic fatty liver disease).Encephalopathy develops after 6 months.
- Acute on Chronic Liver Failure - when acute hepatic decompensation observed in patients with preexisting chronic liver disease characterized by one or more extrahepatic organ failures with a significantly increased risk of death.[5]
Pathophysiology
- The pathophysiology depends on the etiology of the Acute liver failure.
- Most cases of Acute liver failure (except acute fatty liver of pregnancy and Reye syndrome) will have massive hepatocyte necrosis and/or apoptosis leading to liver failure. Hepatocyte necrosis occurs due to ATP depletion causing cellular swelling and cell membrane disruption.
- The pathophysiology of cerebral edema and hepatic encephalopathy is seen in Acute Liver Failure is multi-factorial and includes altered blood-brain barrier secondary to inflammatory mediators leading to microglial activation, accumulation of glutamine secondary to ammonia crossing the BBB and subsequent oxidative stress leading to depletion of adenosine triphosphate (ATP) and guanosine triphosphate (GTP). This ultimately leads to astrocyte swelling and cerebral edema.
- Chronic liver failure is the result of Cirrhosis which is is an advanced stage of liver fibrosis that is accompanied by distortion of the hepatic vasculature.[6]
Causes
Etiology of Acute Liver disease
Viruses
- Hepatitis A virus
- Hepatitis B virus ± delta virus
- Hepatitis E virus
- Herpes simplex virus
- Varicella-zoster virus
- Cytomegalovirus
- Epstein-Barr virus
- Human herpesvirus-6
- Adenovirus, Coxsackie B virus,
- hemorrhagic fever virus
Drugs
- Beractant
- Carfilzomib
- Caspofungin acetate
- Cefadroxil
- Ceftazidime
- Chlordiazepoxide
- Dactinomycin
- diclofenac (patch)
- Didanosine
- Diflunisal
- Efavirenz
- Fulvestrant
- gadoxetate
- Gemcitabine
- Indinavir
- Interferon gamma
- Ixabepilone
- Meropenem
- Micafungin sodium
- Nitisinone
- Oxaprozin
- Oxazepam
- Sorafenib
- Sulfasalazine
- Trovafloxacin mesylate
- Zafirlukast
Metabolic Disease
Toxins
Cardiovascular disease
Autoimmune diseases
Malignant infiltration
Differential Diagnosis
Autoimmune hepatitis must be differentiated from other diseases that cause jaundice, nausea and vomiting, abdominal pain, arthragia, and hepatomegaly such as Hepatitis A,B,C, E, drug induced hepatitis, CMV hepatitis, EBV hepatitis, alcoholic hepatitis, Primary biliary cirrhosis and Primary sclerosing cholangitis.[7][8][9][10][11][12][13][14][15]
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Epidemiology and Demographics
- Acute liver failure affects approximately 2,000–3,000 Americans each year.[16]
- Chronic liver disease and cirrhosis are the 12(th) leading cause of death in the United States.[17]
- Liver transplantation is the second most common solid organ transplantation, yet less than 10% of global transplantation needs are met at current rates.
Natural History, Complications and Prognosis
- Acute liver failure (Hyperacute and subacute) is a rare but serious clinical syndrome characterized by the sudden loss of hepatic function in a person without evidence of preexisting liver disease (Exceptions to this definition include Wilson's disease, reactivation of hepatitis B virus infection, and autoimmune hepatitis)[16]
- Neurological complications, Abnormal hemostasis, and bleeding complications, Multiorgan failure,infection.[18]
Prognosis
The King's College Criteria (KCC) may be used.[16]
Diagnosis
The following evaluation is recommended to help determine the etiology of liver failure. Determination of etiology assists in directing therapy and estimating prognosis:
History and Symptoms
- Obtain a detailed medical history from the patient and/or family, including the first onset of the symptom(s); all medications used over the last 6 months, including prescription medications, over-the-counter agents, herbal supplements, wild mushrooms, or other alternatives/complementary therapies;
- Obtain a detailed history of current and prior substance use; current or prior depression (including assessment of suicidality), anxiety, psychosis, or
other mental illness; viral prodrome; and recent travel.
Physical Examination
- Complete physical examination should be performed.
- Assessment of mental status, the neurologic examination, and the fundoscopic examination in patients with Hepatic Encephalopathy of stage 2 or greater.
Laboratory Findings
- laboratory tests are recommended for establishing an etiology and determining the prognosis of Acute liver failure:
- Hepatic panel
- Complete blood count with differential
- Prothrombin time/international normalized ratio
- Metabolic panel
- Magnesium level
- Phosphorus level
- Amylase and lipase levels
- Toxicology screen (including acetaminophen and salicylate levels)
- Factor V level
- a-fetoprotein level
- Arterial lactate level
- Arterial blood gas level
- Arterial ammonia level (in patients with stage 2 or greater hepatic encephalopathy)
- Viral hepatitis serologies: Hepatitis B surface antigen (hepatitis delta immunoglobulin (Ig)G if the surface antigen is positive), Hepatitis B core IgM, Hepatitis A IgM, Hepatitis E IgG (if positive, ribonucleic acid testing should be ordered in select patients), Hepatitis C antibody
- HIV antibody
- Blood and urine cultures
- Urinalysis
- Blood type
- Serum pregnancy test (for patients with childbearing potential)
- Thromboelastogram
- Diagnostic transjugular liver biopsy
Imaging
- Abdominal ultrasound with Doppler to confirm portal and hepatic vein patency
- Non-contrast computed tomography (CT) scan of the head for patients with Hepatic encephalopathy
Treatment
Effective medical Therapies for specific causes of liver failure and hepatic encephalopathy.[16]
- Acetaminophen intoxication Oral NAC: 140 mg/kg loading dose, then 70 mg/kg every 4 hours until discontinued by hepatology or transplantation surgery attending physician
IV NAC: 150 mg/kg loading dose, then 50 mg/kg IV over 4 hours, then 100 mg/kg IV over 16 hours as a continuous infusion until discontinued by hepatology or transplantation surgery attending physician
- Amanita phalloides(mushroom intoxication) Charcoal: via NGT every 4 hours alternating with silymarin, Penicillin G: 1 g/kg/day IV and NAC (Dosing as for acetaminophen
overdose.),Silymarin: 300 mg PO/NGT every 12 hours,Legalon-SIL: 5 mg/kg/day IV (given in 4 divided doses) or 5 mg/kg IV loading dose followed by 20 mg/kg/day via continuous infusion
- Herpes simplex virus infectionAcyclovir: 10 mg/kg IV every 8 hours (using IBW) adjusted for kidney function
- Cytomegalovirus infection Ganciclovir: 5 mg/kg IV every 12 hours (using IBW) adjusted for kidney function
- Autoimmune hepatitis Methylprednisolone: 60 mg/day IV Kessler WR, et al73
- Hepatitis B virus infection Entecavir (taken on an empty stomach) or tenofovir at standard renal adjusted doses
- Acute fatty liver of pregnancy /HELLP Delivery of the fetus
Treatment of Hepatic Encephalopathy
Liver transplantation: One of the most important, yet difficult, aspects of care for patients with Acute Liver failure is the determination of the need for urgent liver transplantation. We recommend early and rapid evaluation for transplantation candidacy.
Contraindicated medications
Severe hepatic failure is considered an absolute contraindication to the use of the following medications:
- Carvedilol
- Conjugated estrogens/bazedoxifene
- Diclofenamide
- Dronedarone
- Nebivolol
- Rosuvastatin
- Simvastatin
- Spironolactone
- Sulfamethoxazole/Trimethoprim
- Doxorubicin Hydrochloride
- Tipranavir
The ALFSG index is a newer option that may be more accurate.[19]
References
- ↑ Riordan SM, Williams R (May 2008). "Perspectives on liver failure: past and future". Semin. Liver Dis. 28 (2): 137–41. doi:10.1055/s-2008-1073113. PMID 18452113.
- ↑ Bernuau J, Rueff B, Benhamou JP (May 1986). "Fulminant and subfulminant liver failure: definitions and causes". Semin. Liver Dis. 6 (2): 97–106. doi:10.1055/s-2008-1040593. PMID 3529410.
- ↑ 3.0 3.1 O'Grady JG, Schalm SW, Williams R (July 1993). "Acute liver failure: redefining the syndromes". Lancet. 342 (8866): 273–5. doi:10.1016/0140-6736(93)91818-7. PMID 8101303.
- ↑ Jalan R, Williams R (2002). "Acute-on-chronic liver failure: pathophysiological basis of therapeutic options". Blood Purif. 20 (3): 252–61. doi:10.1159/000047017. PMID 11867872.
- ↑ Moreau R, Jalan R, Gines P, Pavesi M, Angeli P, Cordoba J, Durand F, Gustot T, Saliba F, Domenicali M, Gerbes A, Wendon J, Alessandria C, Laleman W, Zeuzem S, Trebicka J, Bernardi M, Arroyo V (June 2013). "Acute-on-chronic liver failure is a distinct syndrome that develops in patients with acute decompensation of cirrhosis". Gastroenterology. 144 (7): 1426–37, 1437.e1–9. doi:10.1053/j.gastro.2013.02.042. PMID 23474284.
- ↑ Schuppan D, Afdhal NH (March 2008). "Liver cirrhosis". Lancet. 371 (9615): 838–51. doi:10.1016/S0140-6736(08)60383-9. PMC 2271178. PMID 18328931.
- ↑ Selmi C, Bowlus CL, Gershwin ME, Coppel RL (2011). "Primary biliary cirrhosis". Lancet. 377 (9777): 1600–9. doi:10.1016/S0140-6736(10)61965-4. PMID 21529926.
- ↑ Lindor KD, Gershwin ME, Poupon R, Kaplan M, Bergasa NV, Heathcote EJ (2009). "Primary biliary cirrhosis". Hepatology. 50 (1): 291–308. doi:10.1002/hep.22906. PMID 19554543.
- ↑ Koff RS (1998). "Hepatitis A". Lancet. 351 (9116): 1643–9. doi:10.1016/S0140-6736(98)01304-X. PMID 9620732.
- ↑ Ciocca M (2000). "Clinical course and consequences of hepatitis A infection". Vaccine. 18 Suppl 1: S71–4. PMID 10683554.
- ↑ Fargo MV, Grogan SP, Saguil A (2017). "Evaluation of Jaundice in Adults". Am Fam Physician. 95 (3): 164–168. PMID 28145671.
- ↑ Leevy CB, Koneru B, Klein KM (1997). "Recurrent familial prolonged intrahepatic cholestasis of pregnancy associated with chronic liver disease". Gastroenterology. 113 (3): 966–72. PMID 9287990.
- ↑ Hov JR, Boberg KM, Karlsen TH (2008). "Autoantibodies in primary sclerosing cholangitis". World J. Gastroenterol. 14 (24): 3781–91. PMC 2721433. PMID 18609700.
- ↑ Bond LR, Hatty SR, Horn ME, Dick M, Meire HB, Bellingham AJ (1987). "Gall stones in sickle cell disease in the United Kingdom". Br Med J (Clin Res Ed). 295 (6592): 234–6. PMC 1247079. PMID 3115390.
- ↑ Malakouti M, Kataria A, Ali SK, Schenker S (2017). "Elevated Liver Enzymes in Asymptomatic Patients - What Should I Do?". J Clin Transl Hepatol. 5 (4): 394–403. doi:10.14218/JCTH.2017.00027. PMC 5719197. PMID 29226106.
- ↑ 16.0 16.1 16.2 16.3 Patton H, Misel M, Gish RG (March 2012). "Acute liver failure in adults: an evidence-based management protocol for clinicians". Gastroenterol Hepatol (N Y). 8 (3): 161–212. PMC 3365519. PMID 22675278.
- ↑ Asrani SK, Larson JJ, Yawn B, Therneau TM, Kim WR (August 2013). "Underestimation of liver-related mortality in the United States". Gastroenterology. 145 (2): 375–82.e1–2. doi:10.1053/j.gastro.2013.04.005. PMC 3890240. PMID 23583430.
- ↑ Munoz SJ (October 2014). "Complications of Acute Liver Failure". Gastroenterol Hepatol (N Y). 10 (10): 665–8. PMC 4988224. PMID 27540338.
- ↑ Rutherford A, King LY, Hynan LS, Vedvyas C, Lin W, Lee WM; et al. (2012). "Development of an accurate index for predicting outcomes of patients with acute liver failure". Gastroenterology. 143 (5): 1237–43. doi:10.1053/j.gastro.2012.07.113. PMC 3480539. PMID 22885329.