U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Hepatic encephalopathy

MedGen UID:
5513
Concept ID:
C0019151
Disease or Syndrome
Synonym: Hepatoencephalopathy
SNOMED CT: Hepatic encephalopathy (13920009); Hepatocerebral encephalopathy (13920009); Portal systemic encephalopathy (449902003); Gaustad's syndrome (449902003); Transient hepatargy syndrome (449902003); HE - Hepatic encephalopathy (13920009)
 
HPO: HP:0002480
Monarch Initiative: MONDO:0001711

Definition

Central nervous system dysfunction in association with liver failure and characterized clinically (depending on degree of severity) by lethargy, confusion, nystagmus, decorticate posturing, spasticity, and bilateral Babinski reflexes. [from HPO]

Term Hierarchy

Conditions with this feature

Patent ductus venosus
MedGen UID:
91033
Concept ID:
C0344688
Congenital Abnormality
A congenital defect of the vasculature such that there is a shunt (by-pass) of blood directly from the portal vein to the vena cava (i.e., the blood from the portal vein is not filtered through the liver).
Infantile liver failure syndrome 2
MedGen UID:
815981
Concept ID:
C3809651
Disease or Syndrome
Infantile liver failure syndrome-2 (ILFS2) is an autosomal recessive disorder characterized by recurrent episodes of acute liver failure during intercurrent febrile illness. Patients first present in infancy or early childhood, and there is complete recovery between episodes with conservative treatment (summary by Haack et al., 2015). For a discussion of genetic heterogeneity of infantile liver failure syndrome, see ILFS1 (615438).
Infantile liver failure syndrome 3
MedGen UID:
1684678
Concept ID:
C5231437
Disease or Syndrome
Infantile liver failure syndrome-3 is an autosomal recessive disorder characterized by recurrent episodes of acute liver failure during intercurrent febrile illness. Patients first present in infancy or early childhood, and there usually is complete recovery between episodes with conservative treatment. Affected individuals also have skeletal anomalies of the vertebral bodies and femoral heads (summary by Cousin et al., 2019). For a discussion of genetic heterogeneity of infantile liver failure syndrome, see ILFS1 (615438).
X-linked lymphoproliferative disease due to SH2D1A deficiency
MedGen UID:
1770239
Concept ID:
C5399825
Disease or Syndrome
X-linked lymphoproliferative disease (XLP) has two recognizable subtypes, XLP1 and XLP2. XLP1 is characterized predominantly by one of three commonly recognized phenotypes: Inappropriate immune response to Epstein-Barr virus (EBV) infection leading to hemophagocytic lymphohistiocytosis (HLH) or severe mononucleosis. Dysgammaglobulinemia. Lymphoproliferative disease (malignant lymphoma). XLP2 is most often characterized by HLH (often associated with EBV), dysgammaglobulinemia, and inflammatory bowel disease. HLH resulting from EBV infection is associated with an unregulated and exaggerated immune response with widespread proliferation of cytotoxic T cells, EBV-infected B cells, and macrophages. Dysgammaglobulinemia is typically hypogammaglobulinemia of one or more immunoglobulin subclasses. The malignant lymphomas are typically B-cell lymphomas, non-Hodgkin type, often extranodal, and in particular involving the intestine.
Immunodeficiency 91 and hyperinflammation
MedGen UID:
1794283
Concept ID:
C5562073
Disease or Syndrome
Immunodeficiency-91 and hyperinflammation (IMD91) is an autosomal recessive complex immunologic disorder characterized by both immunodeficiency and recurrent infections, often to viruses or mycobacteria, as well as by hyperinflammation with systemic involvement. Affected individuals present in infancy with variable features, including fever, infection, thrombocytopenia, renal or hepatic dysfunction, recurrent infections, or seizures. Most patients eventually develop hepatic or renal failure, compromised neurologic function, lymphadenopathy or hepatosplenomegaly, and multiorgan failure resulting in death. More variable features may include intermittent monocytosis, features of hemophagocytic lymphohistiocytosis (HLH), and serologic evidence of hyperinflammation. The disorder is thought to result from dysregulation of the interferon response to viral stimulation in the innate immune system (summary by Le Voyer et al., 2021; Vavassori et al., 2021).
Phosphoenolpyruvate carboxykinase deficiency, cytosolic
MedGen UID:
1801754
Concept ID:
C5574905
Disease or Syndrome
Cytosolic phosphoenolpyruvate carboxykinase deficiency causes a defect in gluconeogenesis that results in a 'biochemical signature' of fasting hypoglycemia with high tricarboxylic acid cycle intermediate excretion, particularly of fumarate. Other biochemical anomalies that may be seen during metabolic crisis include ketonuria, dicarboxylic aciduria, and urea cycle dysfunction (Vieira et al., 2017). See PCKDM (261650) for a discussion of mitochondrial PCK (PEPCK2; 614095) deficiency.
Hepatorenocardiac degenerative fibrosis
MedGen UID:
1808950
Concept ID:
C5676996
Disease or Syndrome
Hepatorenocardiac degenerative fibrosis (HRCDF) is a primarily fibrotic disease affecting the liver, kidney, and heart, with considerable variability in disease onset and expression. Affected individuals develop degenerative hepatic fibrosis in childhood or early adulthood, with variable later onset of fibrocystic kidney disease and hypertrophic cardiomyopathy (Devane et al., 2022).
Liver disease, severe congenital
MedGen UID:
1823968
Concept ID:
C5774195
Disease or Syndrome
Severe congenital liver disease (SCOLIV) is an autosomal recessive disorder characterized by the onset of progressive hepatic dysfunction usually in the first years of life. Affected individuals show feeding difficulties with failure to thrive and features such as jaundice, hepatomegaly, and abdominal distension. Laboratory workup is consistent with hepatic insufficiency and may also show coagulation defects, anemia, or metabolic disturbances. Cirrhosis and hypernodularity are commonly observed on liver biopsy. Many patients die of liver failure in early childhood (Moreno Traspas et al., 2022).

Professional guidelines

PubMed

Tapper EB, Parikh ND
JAMA 2023 May 9;329(18):1589-1602. doi: 10.1001/jama.2023.5997. PMID: 37159031Free PMC Article
Cook AM, Morgan Jones G, Hawryluk GWJ, Mailloux P, McLaughlin D, Papangelou A, Samuel S, Tokumaru S, Venkatasubramanian C, Zacko C, Zimmermann LL, Hirsch K, Shutter L
Neurocrit Care 2020 Jun;32(3):647-666. doi: 10.1007/s12028-020-00959-7. PMID: 32227294Free PMC Article
Smith A, Baumgartner K, Bositis C
Am Fam Physician 2019 Dec 15;100(12):759-770. PMID: 31845776

Recent clinical studies

Etiology

Zacharias HD, Kamel F, Tan J, Kimer N, Gluud LL, Morgan MY
Cochrane Database Syst Rev 2023 Jul 19;7(7):CD011585. doi: 10.1002/14651858.CD011585.pub2. PMID: 37467180Free PMC Article
D'Amico G, Bernardi M, Angeli P
J Hepatol 2022 Jan;76(1):202-207. Epub 2021 Jun 23 doi: 10.1016/j.jhep.2021.06.018. PMID: 34157322
Moran S, López-Sánchez M, Milke-García MDP, Rodríguez-Leal G
World J Gastroenterol 2021 Jun 14;27(22):3050-3063. doi: 10.3748/wjg.v27.i22.3050. PMID: 34168407Free PMC Article
Elsaid MI, Rustgi VK
Clin Liver Dis 2020 May;24(2):157-174. Epub 2020 Mar 2 doi: 10.1016/j.cld.2020.01.001. PMID: 32245524
Bass NM, Mullen KD, Sanyal A, Poordad F, Neff G, Leevy CB, Sigal S, Sheikh MY, Beavers K, Frederick T, Teperman L, Hillebrand D, Huang S, Merchant K, Shaw A, Bortey E, Forbes WP
N Engl J Med 2010 Mar 25;362(12):1071-81. doi: 10.1056/NEJMoa0907893. PMID: 20335583

Diagnosis

European Association for the Study of the Liver. Electronic address: easloffice@easloffice.eu; European Association for the Study of the Liver
J Hepatol 2022 Sep;77(3):807-824. Epub 2022 Jun 17 doi: 10.1016/j.jhep.2022.06.001. PMID: 35724930
Rudler M, Weiss N, Bouzbib C, Thabut D
Clin Liver Dis 2021 May;25(2):393-417. Epub 2021 Mar 11 doi: 10.1016/j.cld.2021.01.008. PMID: 33838857
Rose CF, Amodio P, Bajaj JS, Dhiman RK, Montagnese S, Taylor-Robinson SD, Vilstrup H, Jalan R
J Hepatol 2020 Dec;73(6):1526-1547. Epub 2020 Oct 21 doi: 10.1016/j.jhep.2020.07.013. PMID: 33097308
Weissenborn K
Drugs 2019 Feb;79(Suppl 1):5-9. doi: 10.1007/s40265-018-1018-z. PMID: 30706420Free PMC Article
Patidar KR, Bajaj JS
Clin Gastroenterol Hepatol 2015 Nov;13(12):2048-61. Epub 2015 Jul 9 doi: 10.1016/j.cgh.2015.06.039. PMID: 26164219Free PMC Article

Therapy

Zacharias HD, Kamel F, Tan J, Kimer N, Gluud LL, Morgan MY
Cochrane Database Syst Rev 2023 Jul 19;7(7):CD011585. doi: 10.1002/14651858.CD011585.pub2. PMID: 37467180Free PMC Article
Tapper EB, Parikh ND
JAMA 2023 May 9;329(18):1589-1602. doi: 10.1001/jama.2023.5997. PMID: 37159031Free PMC Article
Won SM, Oh KK, Gupta H, Ganesan R, Sharma SP, Jeong JJ, Yoon SJ, Jeong MK, Min BH, Hyun JY, Park HJ, Eom JA, Lee SB, Cha MG, Kwon GH, Choi MR, Kim DJ, Suk KT
Int J Mol Sci 2022 Aug 12;23(16) doi: 10.3390/ijms23168999. PMID: 36012266Free PMC Article
Caraceni P, Vargas V, Solà E, Alessandria C, de Wit K, Trebicka J, Angeli P, Mookerjee RP, Durand F, Pose E, Krag A, Bajaj JS, Beuers U, Ginès P; Liverhope Consortium
Hepatology 2021 Sep;74(3):1660-1673. Epub 2021 Jun 7 doi: 10.1002/hep.31708. PMID: 33421158Free PMC Article
Ooijevaar RE, Terveer EM, Verspaget HW, Kuijper EJ, Keller JJ
Annu Rev Med 2019 Jan 27;70:335-351. Epub 2018 Nov 7 doi: 10.1146/annurev-med-111717-122956. PMID: 30403550

Prognosis

Perez Ruiz de Garibay A, Kortgen A, Leonhardt J, Zipprich A, Bauer M
Crit Care 2022 Sep 26;26(1):289. doi: 10.1186/s13054-022-04163-1. PMID: 36163253Free PMC Article
Rudler M, Weiss N, Bouzbib C, Thabut D
Clin Liver Dis 2021 May;25(2):393-417. Epub 2021 Mar 11 doi: 10.1016/j.cld.2021.01.008. PMID: 33838857
Trebicka J, Fernandez J, Papp M, Caraceni P, Laleman W, Gambino C, Giovo I, Uschner FE, Jimenez C, Mookerjee R, Gustot T, Albillos A, Bañares R, Janicko M, Steib C, Reiberger T, Acevedo J, Gatti P, Bernal W, Zeuzem S, Zipprich A, Piano S, Berg T, Bruns T, Bendtsen F, Coenraad M, Merli M, Stauber R, Zoller H, Ramos JP, Solè C, Soriano G, de Gottardi A, Gronbaek H, Saliba F, Trautwein C, Özdogan OC, Francque S, Ryder S, Nahon P, Romero-Gomez M, Van Vlierberghe H, Francoz C, Manns M, Garcia E, Tufoni M, Amoros A, Pavesi M, Sanchez C, Curto A, Pitarch C, Putignano A, Moreno E, Shawcross D, Aguilar F, Clària J, Ponzo P, Jansen C, Vitalis Z, Zaccherini G, Balogh B, Vargas V, Montagnese S, Alessandria C, Bernardi M, Ginès P, Jalan R, Moreau R, Angeli P, Arroyo V; PREDICT STUDY group of the EASL-CLIF Consortium
J Hepatol 2020 Oct;73(4):842-854. Epub 2020 Jul 13 doi: 10.1016/j.jhep.2020.06.013. PMID: 32673741
Krishnarao A, Gordon FD
Clin Liver Dis 2020 May;24(2):219-229. Epub 2020 Mar 2 doi: 10.1016/j.cld.2020.01.004. PMID: 32245529
Elsaid MI, Rustgi VK
Clin Liver Dis 2020 May;24(2):157-174. Epub 2020 Mar 2 doi: 10.1016/j.cld.2020.01.001. PMID: 32245524

Clinical prediction guides

Ballester MP, Tranah TH, Balcar L, Fiorillo A, Ampuero J, Kerbert AJC, Thomsen KL, Escudero MD, Mandorfer M, Reiberger T, Shawcross DL, Romero-Gómez M, Montoliu C, Carbonell-Asins JA, Jalan R
J Hepatol 2023 Oct;79(4):967-976. Epub 2023 Jun 3 doi: 10.1016/j.jhep.2023.05.022. PMID: 37277075
Br VK, Sarin SK
Clin Mol Hepatol 2023 Jul;29(3):670-689. Epub 2023 Mar 20 doi: 10.3350/cmh.2022.0103. PMID: 36938601Free PMC Article
Gairing SJ, Schleicher EM, Galle PR, Labenz C
Hepatol Commun 2023 Apr 1;7(4) Epub 2023 Mar 17 doi: 10.1097/HC9.0000000000000096. PMID: 36930868Free PMC Article
Bunchorntavakul C, Reddy KR
Clin Liver Dis 2017 Nov;21(4):769-792. Epub 2017 Jul 29 doi: 10.1016/j.cld.2017.06.002. PMID: 28987262
Sweigart JR, Bradley B, Grigorian AY
J Hosp Med 2016 Aug;11(8):591-4. Epub 2016 Mar 7 doi: 10.1002/jhm.2579. PMID: 26949923

Recent systematic reviews

Liang A, Brar S, Almaghrabi M, Khan MQ, Qumosani K, Teriaky A
Medicine (Baltimore) 2023 Sep 22;102(38):e35266. doi: 10.1097/MD.0000000000035266. PMID: 37746955Free PMC Article
Zacharias HD, Kamel F, Tan J, Kimer N, Gluud LL, Morgan MY
Cochrane Database Syst Rev 2023 Jul 19;7(7):CD011585. doi: 10.1002/14651858.CD011585.pub2. PMID: 37467180Free PMC Article
Bai Z, Wang L, Wang R, Zou M, Méndez-Sánchez N, Romeiro FG, Cheng G, Qi X
Hepatol Int 2022 Dec;16(6):1468-1483. Epub 2022 Sep 1 doi: 10.1007/s12072-022-10374-z. PMID: 36048318
Fu J, Gao Y, Shi L
PLoS One 2022;17(4):e0267647. Epub 2022 Apr 26 doi: 10.1371/journal.pone.0267647. PMID: 35471992Free PMC Article
Hoilat GJ, Ayas MF, Hoilat JN, Abu-Zaid A, Durer C, Durer S, Adhami T, John S
BMJ Open Gastroenterol 2021 May;8(1) doi: 10.1136/bmjgast-2021-000648. PMID: 34006606Free PMC Article

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.
    • Bookshelf
      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Recent activity

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...