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Cirrhosis, cryptogenic

MedGen UID:
82760
Concept ID:
C0267809
Disease or Syndrome
Synonym: Cirrhosis, Cryptogenic
SNOMED CT: Cryptogenic cirrhosis (89580002); Idiopathic cirrhosis (89580002)
 
OMIM®: 148070; 215600

Definition

Cryptogenic cirrhosis is a condition that impairs liver function. People with this condition develop irreversible liver disease caused by scarring of the liver (cirrhosis), typically in mid- to late adulthood.

The liver is a part of the digestive system that helps break down food, store energy, and remove waste products, including toxins. Minor damage to the liver can be repaired by the body. However, severe or long-term damage can lead to the replacement of normal liver tissue with scar tissue.

In the early stages of cryptogenic cirrhosis, people often have no symptoms because the liver has enough normal tissue to function. Signs and symptoms become apparent as more of the liver is replaced by scar tissue. Affected individuals can experience fatigue, weakness, loss of appetite, weight loss, nausea, swelling (edema), enlarged blood vessels, and yellowing of the skin and whites of the eyes (jaundice).

People with cryptogenic cirrhosis may develop high blood pressure in the vein that supplies blood to the liver (portal hypertension). Cryptogenic cirrhosis can lead to type 2 diabetes, although the mechanism is unclear. Some people with cryptogenic cirrhosis develop cancer of the liver (hepatocellular cancer). [from MedlinePlus Genetics]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVCirrhosis, cryptogenic

Professional guidelines

PubMed

Alswat KA, Fallatah HI, Al-Judaibi B, Elsiesy HA, Al-Hamoudi WK, Qutub AN, Alturaify N, Al-Osaimi A
Saudi Med J 2019 Jun;40(6):531-540. doi: 10.15537/smj.2019.6.23980. PMID: 31219486Free PMC Article
Margini C, Dufour JF
Liver Int 2016 Mar;36(3):317-24. Epub 2015 Dec 25 doi: 10.1111/liv.13031. PMID: 26601627
Perlmutter DH
Clin Liver Dis 2004 Nov;8(4):839-59, viii-ix. doi: 10.1016/j.cld.2004.06.001. PMID: 15464658

Recent clinical studies

Etiology

Yoosuf S, Singh P, Khaitan A, Strand TA, Ahuja V, Makharia GK
Am J Gastroenterol 2023 May 1;118(5):820-832. Epub 2022 Dec 23 doi: 10.14309/ajg.0000000000002123. PMID: 36599134
Idilman R, Aydogan M, Oruncu MB, Kartal A, Elhan AH, Ellik Z, Gumussoy M, Er R, Ozercan M, Duman S, Toruner M, Cinar K, Soykan I, Beyler AR, Ozkan H
Dig Dis 2021;39(4):358-365. Epub 2020 Nov 3 doi: 10.1159/000512746. PMID: 33142287
Ben-Chetrit E, Yazici H
Clin Exp Rheumatol 2017 Nov-Dec;35 Suppl 108(6):108-112. Epub 2017 Jun 9 PMID: 28598780
Mohammed OK, Mahadeva S
J Gastroenterol Hepatol 2015 Sep;30(9):1423-8. doi: 10.1111/jgh.12978. PMID: 25867030
Marchesini G, Moscatiello S, Di Domizio S, Forlani G
J Clin Endocrinol Metab 2008 Nov;93(11 Suppl 1):S74-80. doi: 10.1210/jc.2008-1399. PMID: 18987273

Diagnosis

Horlenko OM, Kossey GB, Pushkarenko OA, Prylypko LB
Wiad Lek 2022;75(10):2536-2540. doi: 10.36740/WLek202210141. PMID: 36472294
Lai J, Wang HL, Zhang X, Wang H, Liu X
Arch Pathol Lab Med 2022 Aug 1;146(8):940-946. doi: 10.5858/arpa.2021-0339-RA. PMID: 34871361
Nalbantoglu I, Jain D
Semin Diagn Pathol 2019 Nov;36(6):389-394. Epub 2019 Jul 24 doi: 10.1053/j.semdp.2019.07.003. PMID: 31395291
Lonardo A, Targher G
Int J Mol Sci 2017 Sep 12;18(9) doi: 10.3390/ijms18091955. PMID: 28895919Free PMC Article
Ben-Chetrit E, Yazici H
Clin Exp Rheumatol 2017 Nov-Dec;35 Suppl 108(6):108-112. Epub 2017 Jun 9 PMID: 28598780

Therapy

Fukushima M, Miyaaki H, Sasaki R, Haraguchi M, Miuma S, Hara T, Soyama A, Hidaka M, Eguchi S, Nakao K
Intern Med 2023 May 15;62(10):1415-1423. Epub 2022 Sep 28 doi: 10.2169/internalmedicine.0514-22. PMID: 36171128Free PMC Article
Horlenko OM, Kossey GB, Pushkarenko OA, Prylypko LB
Wiad Lek 2022;75(10):2536-2540. doi: 10.36740/WLek202210141. PMID: 36472294
Eshraghian A, Nikeghbalian S, Kazemi K, Shamsaeefar A, Geramizadeh B, Malek-Hosseini SA
HPB (Oxford) 2020 Apr;22(4):521-528. Epub 2019 Aug 17 doi: 10.1016/j.hpb.2019.07.015. PMID: 31431413
Younossi Z, Stepanova M, Sanyal AJ, Harrison SA, Ratziu V, Abdelmalek MF, Diehl AM, Caldwell S, Shiffman ML, Schall RA, McColgan B, Subramanian GM, Myers RP, Muir A, Afdhal NH, Bosch J, Goodman Z
J Hepatol 2018 Dec;69(6):1365-1370. Epub 2018 Aug 23 doi: 10.1016/j.jhep.2018.08.013. PMID: 30144554
Campos MA, Lascano J
Ther Adv Respir Dis 2014 Oct;8(5):150-61. Epub 2014 Jul 9 doi: 10.1177/1753465814542243. PMID: 25013223

Prognosis

Fukushima M, Miyaaki H, Sasaki R, Haraguchi M, Miuma S, Hara T, Soyama A, Hidaka M, Eguchi S, Nakao K
Intern Med 2023 May 15;62(10):1415-1423. Epub 2022 Sep 28 doi: 10.2169/internalmedicine.0514-22. PMID: 36171128Free PMC Article
Köse E, Çağatay E, Yaraş T, Kısa PT, Güler S, Gülten ZA, Akarsu M, Oktay Y, Kayalı HA, Arslan N
Turk J Med Sci 2022 Aug;52(4):1075-1084. Epub 2022 Aug 10 doi: 10.55730/1300-0144.5410. PMID: 36326406Free PMC Article
Younossi Z, Stepanova M, Sanyal AJ, Harrison SA, Ratziu V, Abdelmalek MF, Diehl AM, Caldwell S, Shiffman ML, Schall RA, McColgan B, Subramanian GM, Myers RP, Muir A, Afdhal NH, Bosch J, Goodman Z
J Hepatol 2018 Dec;69(6):1365-1370. Epub 2018 Aug 23 doi: 10.1016/j.jhep.2018.08.013. PMID: 30144554
Rinaldi L, Nascimbeni F, Giordano M, Masetti C, Guerrera B, Amelia A, Fascione MC, Ballestri S, Romagnoli D, Zampino R, Nevola R, Baldelli E, Iuliano N, Rosato V, Lonardo A, Adinolfi LE
World J Gastroenterol 2017 Feb 28;23(8):1458-1468. doi: 10.3748/wjg.v23.i8.1458. PMID: 28293093Free PMC Article
Mohammed OK, Mahadeva S
J Gastroenterol Hepatol 2015 Sep;30(9):1423-8. doi: 10.1111/jgh.12978. PMID: 25867030

Clinical prediction guides

Horlenko OM, Kossey GB, Pushkarenko OA, Prylypko LB
Wiad Lek 2022;75(10):2536-2540. doi: 10.36740/WLek202210141. PMID: 36472294
Köse E, Çağatay E, Yaraş T, Kısa PT, Güler S, Gülten ZA, Akarsu M, Oktay Y, Kayalı HA, Arslan N
Turk J Med Sci 2022 Aug;52(4):1075-1084. Epub 2022 Aug 10 doi: 10.55730/1300-0144.5410. PMID: 36326406Free PMC Article
Nalbantoglu I, Jain D
Semin Diagn Pathol 2019 Nov;36(6):389-394. Epub 2019 Jul 24 doi: 10.1053/j.semdp.2019.07.003. PMID: 31395291
Rinaldi L, Nascimbeni F, Giordano M, Masetti C, Guerrera B, Amelia A, Fascione MC, Ballestri S, Romagnoli D, Zampino R, Nevola R, Baldelli E, Iuliano N, Rosato V, Lonardo A, Adinolfi LE
World J Gastroenterol 2017 Feb 28;23(8):1458-1468. doi: 10.3748/wjg.v23.i8.1458. PMID: 28293093Free PMC Article
Marchesini G, Moscatiello S, Di Domizio S, Forlani G
J Clin Endocrinol Metab 2008 Nov;93(11 Suppl 1):S74-80. doi: 10.1210/jc.2008-1399. PMID: 18987273

Recent systematic reviews

Yoosuf S, Singh P, Khaitan A, Strand TA, Ahuja V, Makharia GK
Am J Gastroenterol 2023 May 1;118(5):820-832. Epub 2022 Dec 23 doi: 10.14309/ajg.0000000000002123. PMID: 36599134
Losurdo G, Castellaneta A, Rendina M, Carparelli S, Leandro G, Di Leo A
Aliment Pharmacol Ther 2018 Mar;47(6):704-714. Epub 2018 Jan 22 doi: 10.1111/apt.14521. PMID: 29359341
Vajro P, Paolella G, Maggiore G, Giordano G
J Pediatr Gastroenterol Nutr 2013 Jun;56(6):663-70. doi: 10.1097/MPG.0b013e31828dc5c5. PMID: 23434875

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