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Chronic active hepatitis

MedGen UID:
105441
Concept ID:
C0520463
Disease or Syndrome
Synonyms: Chronic Active Hepatitis; Hepatitis, Chronic Active
SNOMED CT: Chronic active hepatitis (197284004); CAH - Chronic active hepatitis (197284004); CAH - Chronic aggressive hepatitis (197284004)
 
HPO: HP:0200120

Definition

Chronic hepatitis associated with recurrent clinical exacerbations, extrahepatic manifestations, and progression to cirrhosis. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVChronic active hepatitis

Conditions with this feature

Polyglandular autoimmune syndrome, type 1
MedGen UID:
39125
Concept ID:
C0085859
Disease or Syndrome
Autoimmune polyglandular syndrome type I is characterized by the presence of 2 of 3 major clinical symptoms: Addison disease, and/or hypoparathyroidism, and/or chronic mucocutaneous candidiasis (Neufeld et al., 1981).
Alstrom syndrome
MedGen UID:
78675
Concept ID:
C0268425
Disease or Syndrome
Alström syndrome is characterized by cone-rod dystrophy, obesity, progressive bilateral sensorineural hearing impairment, acute infantile-onset cardiomyopathy and/or adolescent- or adult-onset restrictive cardiomyopathy, insulin resistance / type 2 diabetes mellitus (T2DM), non-alcoholic fatty liver disease (NAFLD), and chronic progressive kidney disease. Cone-rod dystrophy presents as progressive visual impairment, photophobia, and nystagmus usually starting between birth and age 15 months. Many individuals lose all perception of light by the end of the second decade, but a minority retain the ability to read large print into the third decade. Children usually have normal birth weight but develop truncal obesity during their first year. Sensorineural hearing loss presents in the first decade in as many as 70% of individuals and may progress to the severe or moderately severe range (40-70 db) by the end of the first to second decade. Insulin resistance is typically accompanied by the skin changes of acanthosis nigricans, and proceeds to T2DM in the majority by the third decade. Nearly all demonstrate hypertriglyceridemia. Other findings can include endocrine abnormalities (hypothyroidism, hypogonadotropic hypogonadism in males, and hyperandrogenism in females), urologic dysfunction / detrusor instability, progressive decrease in renal function, and hepatic disease (ranging from elevated transaminases to steatohepatitis/NAFLD). Approximately 20% of affected individuals have delay in early developmental milestones, most commonly in gross and fine motor skills. About 30% have a learning disability. Cognitive impairment (IQ <70) is very rare. Wide clinical variability is observed among affected individuals, even within the same family.
Familial cirrhosis
MedGen UID:
350049
Concept ID:
C1861556
Disease or Syndrome
Complement component 4b deficiency
MedGen UID:
482271
Concept ID:
C3280641
Disease or Syndrome
A reduced level of the complement component C4b in circulation.

Recent clinical studies

Etiology

Chlilek A, Roger C, Muller L, Carles MJ, Stephan R, Laureillard D, Lavigne JP, Lefrant JY, Sotto A
BMC Infect Dis 2019 Jul 17;19(1):636. doi: 10.1186/s12879-019-4278-7. PMID: 31315560Free PMC Article
Pourhassan A, Fouladi DF, Samani SM, Asl SM
Biol Trace Elem Res 2015 Oct;167(2):187-93. Epub 2015 Mar 21 doi: 10.1007/s12011-015-0309-4. PMID: 25795173
El-Kannishy G, Arafa M, Abdelaal I, Elarman M, El-Mahdy R
Saudi J Gastroenterol 2012 Nov-Dec;18(6):375-9. doi: 10.4103/1319-3767.103429. PMID: 23150023Free PMC Article
Arasli M, Ustundag Y, Delikanli B, Harmandar F, Buyukuysal C
Hepatogastroenterology 2012 May;59(115):851-7. doi: 10.5754/hge11580. PMID: 22193343
Ge J, Wang K, Meng QH, Qi ZX, Meng FL, Fan YC
J Clin Immunol 2010 Jan;30(1):60-7. Epub 2009 Sep 10 doi: 10.1007/s10875-009-9328-2. PMID: 19756987

Diagnosis

Chlilek A, Roger C, Muller L, Carles MJ, Stephan R, Laureillard D, Lavigne JP, Lefrant JY, Sotto A
BMC Infect Dis 2019 Jul 17;19(1):636. doi: 10.1186/s12879-019-4278-7. PMID: 31315560Free PMC Article
Manfredi R, Dentale N, Calza L
Int J STD AIDS 2012 Mar;23(3):e48-50. doi: 10.1258/ijsa.2009.009333. PMID: 22581898
Arasli M, Ustundag Y, Delikanli B, Harmandar F, Buyukuysal C
Hepatogastroenterology 2012 May;59(115):851-7. doi: 10.5754/hge11580. PMID: 22193343
Fung J, Lai CL, Chan SC, But D, Seto WK, Cheng C, Wong DK, Lo CM, Fan ST, Yuen MF
Am J Gastroenterol 2010 May;105(5):1116-22. Epub 2009 Nov 17 doi: 10.1038/ajg.2009.665. PMID: 19920809
Palumbo E, Scotto G, Faleo G, Cibelli DC, Angarano G
Braz J Infect Dis 2007 Jun;11(3):311-3. doi: 10.1590/s1413-86702007000300002. PMID: 17684630

Therapy

Meng JP, Luo P, Bai Y, Cui F
J Int Med Res 2020 Jan;48(1):300060519884157. doi: 10.1177/0300060519884157. PMID: 31939340Free PMC Article
Chlilek A, Roger C, Muller L, Carles MJ, Stephan R, Laureillard D, Lavigne JP, Lefrant JY, Sotto A
BMC Infect Dis 2019 Jul 17;19(1):636. doi: 10.1186/s12879-019-4278-7. PMID: 31315560Free PMC Article
Tagliamento M, Grossi F, Paolino S, Rijavec E, Genova C, Rossi G, Biello F, De Maria A
Immunotherapy 2019 Jul;11(10):873-879. Epub 2019 Jun 3 doi: 10.2217/imt-2019-0025. PMID: 31156006
Pourhassan A, Fouladi DF, Samani SM, Asl SM
Biol Trace Elem Res 2015 Oct;167(2):187-93. Epub 2015 Mar 21 doi: 10.1007/s12011-015-0309-4. PMID: 25795173
Cui F, Meng J, Luo P, Chen P
BMC Infect Dis 2014 Feb 1;14:55. doi: 10.1186/1471-2334-14-55. PMID: 24484458Free PMC Article

Prognosis

El-Kannishy G, Arafa M, Abdelaal I, Elarman M, El-Mahdy R
Saudi J Gastroenterol 2012 Nov-Dec;18(6):375-9. doi: 10.4103/1319-3767.103429. PMID: 23150023Free PMC Article
Arasli M, Ustundag Y, Delikanli B, Harmandar F, Buyukuysal C
Hepatogastroenterology 2012 May;59(115):851-7. doi: 10.5754/hge11580. PMID: 22193343
Fung J, Lai CL, Chan SC, But D, Seto WK, Cheng C, Wong DK, Lo CM, Fan ST, Yuen MF
Am J Gastroenterol 2010 May;105(5):1116-22. Epub 2009 Nov 17 doi: 10.1038/ajg.2009.665. PMID: 19920809
Jang JW, Lee YC, Kim MS, Lee SY, Bae SH, Choi JY, Yoon SK
J Viral Hepat 2007 Mar;14(3):169-75. doi: 10.1111/j.1365-2893.2006.00788.x. PMID: 17305882
Scotto G, Palumbo E, Fazio V, Cibelli DC, Saracino A, Angarano G
Infez Med 2006 Sep;14(3):145-51. PMID: 17127828

Clinical prediction guides

El-Kannishy G, Arafa M, Abdelaal I, Elarman M, El-Mahdy R
Saudi J Gastroenterol 2012 Nov-Dec;18(6):375-9. doi: 10.4103/1319-3767.103429. PMID: 23150023Free PMC Article
Manfredi R, Dentale N, Calza L
Int J STD AIDS 2012 Mar;23(3):e48-50. doi: 10.1258/ijsa.2009.009333. PMID: 22581898
Soylu A, Kiliçkesmez O, Poturoğlu S, Dolapçioğlu C, Serez K, Sevindir I, Yaşar N, Akyildiz M, Kumbasar B
Diagn Interv Radiol 2010 Sep;16(3):204-8. Epub 2010 Jul 25 doi: 10.4261/1305-3825.DIR.2810-09.1. PMID: 20658448
Fung J, Lai CL, Chan SC, But D, Seto WK, Cheng C, Wong DK, Lo CM, Fan ST, Yuen MF
Am J Gastroenterol 2010 May;105(5):1116-22. Epub 2009 Nov 17 doi: 10.1038/ajg.2009.665. PMID: 19920809
Hiramatsu S, Maruyama T, Ito H, Shimoda S, Kaji Y, Harada M
Int Heart J 2005 Nov;46(6):1033-40. doi: 10.1536/ihj.46.1033. PMID: 16394599

Recent systematic reviews

Dean T, Dewey A, Bara A, Lasserson TJ, Walters EH
Cochrane Database Syst Rev 2004;(1):CD003270. doi: 10.1002/14651858.CD003270.pub2. PMID: 14974011

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