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Hepatic necrosis

MedGen UID:
57487
Concept ID:
C0151798
Disease or Syndrome
Synonym: Liver necrosis
SNOMED CT: Hepatic necrosis (87248009); Liver necrosis (87248009)
 
HPO: HP:0002605

Definition

The presence of cell death (necrosis) affecting the liver. [from HPO]

Conditions with this feature

Deficiency of 3-hydroxyacyl-CoA dehydrogenase
MedGen UID:
266222
Concept ID:
C1291230
Disease or Syndrome
3-hydroxyacyl-CoA dehydrogenase deficiency is an inherited condition that prevents the body from converting certain fats to energy, particularly during prolonged periods without food (fasting).\n\nInitial signs and symptoms of this disorder typically occur during infancy or early childhood and can include poor appetite, vomiting, diarrhea, and lack of energy (lethargy). Affected individuals can also have muscle weakness (hypotonia), liver problems, low blood glucose (hypoglycemia), and abnormally high levels of insulin (hyperinsulinism). Insulin controls the amount of glucose that moves from the blood into cells for conversion to energy. Individuals with 3-hydroxyacyl-CoA dehydrogenase deficiency are also at risk for complications such as seizures, life-threatening heart and breathing problems, coma, and sudden death. This condition may explain some cases of sudden infant death syndrome (SIDS), which is defined as unexplained death in babies younger than 1 year.\n\nProblems related to 3-hydroxyacyl-CoA dehydrogenase deficiency can be triggered by periods of fasting or by illnesses such as viral infections. This disorder is sometimes mistaken for Reye syndrome, a severe disorder that may develop in children while they appear to be recovering from viral infections such as chicken pox or flu. Most cases of Reye syndrome are associated with the use of aspirin during these viral infections.
Dyskeratosis congenita, autosomal dominant 1
MedGen UID:
1645250
Concept ID:
C4551974
Disease or Syndrome
Dyskeratosis congenita and related telomere biology disorders (DC/TBD) are caused by impaired telomere maintenance resulting in short or very short telomeres. The phenotypic spectrum of telomere biology disorders is broad and includes individuals with classic dyskeratosis congenita (DC) as well as those with very short telomeres and an isolated physical finding. Classic DC is characterized by a triad of dysplastic nails, lacy reticular pigmentation of the upper chest and/or neck, and oral leukoplakia, although this may not be present in all individuals. People with DC/TBD are at increased risk for progressive bone marrow failure (BMF), myelodysplastic syndrome or acute myelogenous leukemia, solid tumors (usually squamous cell carcinoma of the head/neck or anogenital cancer), and pulmonary fibrosis. Other findings can include eye abnormalities (epiphora, blepharitis, sparse eyelashes, ectropion, entropion, trichiasis), taurodontism, liver disease, gastrointestinal telangiectasias, and avascular necrosis of the hips or shoulders. Although most persons with DC/TBD have normal psychomotor development and normal neurologic function, significant developmental delay is present in both forms; additional findings include cerebellar hypoplasia (Hoyeraal Hreidarsson syndrome) and bilateral exudative retinopathy and intracranial calcifications (Revesz syndrome and Coats plus syndrome). Onset and progression of manifestations of DC/TBD vary: at the mild end of the spectrum are those who have only minimal physical findings with normal bone marrow function, and at the severe end are those who have the diagnostic triad and early-onset BMF.

Professional guidelines

PubMed

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
Chang ML, Liaw YF
J Hepatol 2014 Dec;61(6):1407-17. Epub 2014 Aug 29 doi: 10.1016/j.jhep.2014.08.033. PMID: 25178562
Williams S, Chan AK
Semin Fetal Neonatal Med 2011 Dec;16(6):329-39. Epub 2011 Sep 16 doi: 10.1016/j.siny.2011.08.005. PMID: 21925985

Recent clinical studies

Etiology

Chuang YY, Huang YC
J Microbiol Immunol Infect 2019 Dec;52(6):851-857. Epub 2019 Sep 30 doi: 10.1016/j.jmii.2019.08.018. PMID: 31607572
Goel A, Aggarwal P
Natl Med J India 2007 Jul-Aug;20(4):182-91. PMID: 18085124
Stedman C
Semin Liver Dis 2002;22(2):195-206. doi: 10.1055/s-2002-30104. PMID: 12016550
Kelly DA
Indian J Pediatr 1999;66(1 Suppl):S104-9. PMID: 11132456
Forrest JA, Clements JA, Prescott LF
Clin Pharmacokinet 1982 Mar-Apr;7(2):93-107. doi: 10.2165/00003088-198207020-00001. PMID: 7039926

Diagnosis

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
Chuang YY, Huang YC
J Microbiol Immunol Infect 2019 Dec;52(6):851-857. Epub 2019 Sep 30 doi: 10.1016/j.jmii.2019.08.018. PMID: 31607572
Dusse LM, Alpoim PN, Silva JT, Rios DR, Brandão AH, Cabral AC
Clin Chim Acta 2015 Dec 7;451(Pt B):117-20. Epub 2015 Oct 23 doi: 10.1016/j.cca.2015.10.024. PMID: 26525965
Goel A, Aggarwal P
Natl Med J India 2007 Jul-Aug;20(4):182-91. PMID: 18085124
Stedman C
Semin Liver Dis 2002;22(2):195-206. doi: 10.1055/s-2002-30104. PMID: 12016550

Therapy

Starkey Lewis P, Campana L, Aleksieva N, Cartwright JA, Mackinnon A, O'Duibhir E, Kendall T, Vermeren M, Thomson A, Gadd V, Dwyer B, Aird R, Man TY, Rossi AG, Forrester L, Park BK, Forbes SJ
J Hepatol 2020 Aug;73(2):349-360. Epub 2020 Mar 11 doi: 10.1016/j.jhep.2020.02.031. PMID: 32169610Free PMC Article
Chuang YY, Huang YC
J Microbiol Immunol Infect 2019 Dec;52(6):851-857. Epub 2019 Sep 30 doi: 10.1016/j.jmii.2019.08.018. PMID: 31607572
Chang ML, Liaw YF
J Hepatol 2014 Dec;61(6):1407-17. Epub 2014 Aug 29 doi: 10.1016/j.jhep.2014.08.033. PMID: 25178562
Stedman C
Semin Liver Dis 2002;22(2):195-206. doi: 10.1055/s-2002-30104. PMID: 12016550
Forrest JA, Clements JA, Prescott LF
Clin Pharmacokinet 1982 Mar-Apr;7(2):93-107. doi: 10.2165/00003088-198207020-00001. PMID: 7039926

Prognosis

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
Gasmi B, Kleiner DE
Clin Liver Dis 2020 Feb;24(1):61-74. Epub 2019 Oct 31 doi: 10.1016/j.cld.2019.09.004. PMID: 31753251Free PMC Article
Kleiner DE
Liver Int 2018 Feb;38(2):198-209. Epub 2017 Sep 19 doi: 10.1111/liv.13584. PMID: 28865179
Chang ML, Liaw YF
J Hepatol 2014 Dec;61(6):1407-17. Epub 2014 Aug 29 doi: 10.1016/j.jhep.2014.08.033. PMID: 25178562
Kelly DA
Indian J Pediatr 1999;66(1 Suppl):S104-9. PMID: 11132456

Clinical prediction guides

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
Li Y, Xiao SY
J Med Virol 2020 Sep;92(9):1491-1494. Epub 2020 May 13 doi: 10.1002/jmv.25973. PMID: 32369204
Kleiner DE
Liver Int 2018 Feb;38(2):198-209. Epub 2017 Sep 19 doi: 10.1111/liv.13584. PMID: 28865179
Chang ML, Liaw YF
J Hepatol 2014 Dec;61(6):1407-17. Epub 2014 Aug 29 doi: 10.1016/j.jhep.2014.08.033. PMID: 25178562
Davis M
Int Clin Psychopharmacol 1991 Summer;6(2):97-103. doi: 10.1097/00004850-199100620-00004. PMID: 1960384

Recent systematic reviews

Segalini E, Morello A, Leati G, Di Saverio S, Aseni P
Updates Surg 2022 Oct;74(5):1511-1519. Epub 2022 Sep 4 doi: 10.1007/s13304-022-01372-9. PMID: 36059024Free PMC Article
Nacif LS, Fernandes MR, Waisberg DR, Pinheiro RS, Rocha-Santos V, Galvão F, Andraus W, Carneiro-D'Albuquerque L
Clinics (Sao Paulo) 2022 Jan-Dec;77:100042. Epub 2022 Apr 26 doi: 10.1016/j.clinsp.2022.100042. PMID: 35870265Free PMC Article
Barrios Sanjuanelo A, Abelló Munarriz C, Cardona-Arias JA
J Pediatr Surg 2021 Apr;56(4):678-685. Epub 2020 Aug 29 doi: 10.1016/j.jpedsurg.2020.08.019. PMID: 32981659
Green CS, Bulger EM, Kwan SW
J Trauma Acute Care Surg 2016 Mar;80(3):529-37. doi: 10.1097/TA.0000000000000942. PMID: 26670113Free PMC Article
Abdualmjid RJ, Sergi C
J Pharm Pharm Sci 2013;16(3):376-404. doi: 10.18433/j36g6x. PMID: 24021288

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