Fígado policístico - relato de caso / Polycystic liver - case report

Vanessa Frajblat, Maria Eduarda Ragghiante Ferreira, Viviane Lozano Espasandin

Abstract


Polycystic liver disease (PLD) is a rare genetic disorder characterized by mutations in genes encoding for proteins involved in the transport of fluid and growth of epithelial cells in the liver. There are two distinct forms of PLD; in isolation and in association with polycystic kidney disease (PKD) (1). The severity of the disease in patients with PKD is highly variable, meanwhile  the presence of liver cysts is the most important clinical feature of polycystic liver disease (2,3). There are currently several options of treatment available, including drug terapy and surgical optopns (1,4).

 

 


Keywords


Polycystic liver, Polycystic liver disease.

References


Kothadia JP, Kreitman K, Shah JM. Polycystic Liver Disease. In: StatPearls. Treasure Island (FL): StatPearls Publishing; May 19, 2021.

Cnossen WR, Drenth JP. Polycystic liver disease: an overview of pathogenesis, clinical manifestations and management. Orphanet J Rare Dis. 2014 May 1;9:69. doi: 10.1186/1750-1172-9-69. PMID: 24886261; PMCID: PMC4030533.

Cornec-Le Gall E, Torres VE, Harris PC. Genetic Complexity of Autosomal Dominant Polycystic Kidney and Liver Diseases. J Am Soc Nephrol. 2018 Jan;29(1):13-23. doi: 10.1681/ASN.2017050483. Epub 2017 Oct 16. PMID: 29038287; PMCID: PMC5748917.

van Aerts RMM, van de Laarschot LFM, Banales JM, Drenth JPH. Clinical management of polycystic liver disease. J Hepatol. 2018 Apr;68(4):827-837. doi: 10.1016/j.jhep.2017.11.024. Epub 2017 Nov 24. PMID: 29175241.

Zhang ZY, Wang ZM, Huang Y. Polycystic liver disease: Classification, diagnosis, treatment process, and clinical management. World J Hepatol. 2020;12(3):72-83. doi:10.4254/wjh.v12.i3.72

Onori P, Franchitto A, Mancinelli R, et al. Polycystic liver diseases. Dig Liver Dis. 2010;42(4):261-271. doi:10.1016/j.dld.2010.01.006

Temmerman F, Missiaen L, Bammens B, et al. Systematic review: the pathophysiology and management of polycystic liver disease. Aliment Pharmacol Ther. 2011;34(7):702-713. doi:10.1111/j.1365-2036.2011.04783.x

van Aerts RMM, Kievit W, D'Agnolo HMA, Blijdorp CJ, Casteleijn NF, Dekker SEI, de Fijter JW, van Gastel M, Gevers TJ, van de Laarschot LFM, Lantinga MA, Losekoot M, Meijer E, Messchendorp AL, Neijenhuis MK, Pena MJ, Peters DJM, Salih M, Soonawala D, Spithoven EM, Visser FW, Wetzels JF, Zietse R, Gansevoort RT, Drenth JPH; DIPAK-1 Investigators. Lanreotide Reduces Liver Growth In Patients With Autosomal Dominant Polycystic Liver and Kidney Disease. Gastroenterology. 2019 Aug;157(2):481-491.e7. doi: 10.1053/j.gastro.2019.04.018. Epub 2019 Apr 22. PMID: 31022403.

Aussilhou B, Dokmak S, Dondero F, et al. Treatment of polycystic liver disease. Update on the management. J Visc Surg. 2018;155(6):471-481. doi:10.1016/j.jviscsurg.2018.07.004

Gevers TJ, Drenth JP. Somatostatin analogues for treatment of polycystic liver disease. Curr Opin Gastroenterol. 2011;27(3):294-300. doi:10.1097/MOG.0b013e328343433f

Gevers TJ, Drenth JP. Diagnosis and management of polycystic liver disease. Nat Rev Gastroenterol Hepatol. 2013;10(2):101-108. doi:10.1038/nrgastro.2012.254




DOI: https://doi.org/10.34117/bjdv7n6-424

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