Polycystic liver: clinical characteristics of patients with isolated polycystic liver disease compared with patients with polycystic liver and autosomal dominant polycystic kidney disease

Hoevenaren, Inge A.; Wester, Ruth; Schrier, Robert W.; McFann, Kim; Doctor, R. Brian; Drenth, Joost P.H.; Everson, Gregory T.

doi:10.1111/j.1478-3231.2007.01595.x

Cited by 104 publications

(111 citation statements)

References 16 publications

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“…2 The elevated AP and gGT levels probably reflect activation of cholangiocytes. 9,[23][24][25][26] Serum transaminases are normal or only mildly elevated. 2 Bilirubin is rarely elevated but in advanced cases jaundice may arise due to compression of the common bile duct secondary to a strategically located cyst.…”

Section: Laboratory Findingsmentioning

confidence: 99%

Medical and surgical treatment options for polycystic liver disease1

et al. 2010

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Section: Laboratory Findingsmentioning

confidence: 99%

Medical and surgical treatment options for polycystic liver disease1

et al. 2010

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“…44,45 At present, it is not known whether there is a constitutive inhibition of mTOR pathway in patients affected by PRKCSH mutations. Furthermore, the effect of mTOR inhibition is not limited to autophagy induction.…”

Section: Methodsmentioning

confidence: 99%

Deficiency of hepatocystin induces autophagy through an mTOR-dependent pathway

Yang

Zhao

et al. 2011

Autophagy

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“…All PCLD liver cysts arise from progressive dilatation of abnormal ductules that display the same epithelium and structures as functioning cholangiocytes (22,23), but patients with iPCLD tend to have larger sized cysts (24). The annual growth of PCLD livers is in the range of 0.9-3.2% of the initial hepatic volume and the speed becomes faster and faster (20).…”

Section: Discussionmentioning

confidence: 99%

Overexpression of Aquaporin 1 on cysts of patients with polycystic liver disease

Li¹,

Shi²,

Zhao³

et al. 2015

Rev Esp Enferm Dig

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Background and objective: Polycystic liver disease (PCLD) represents a group of genetic disorders that include autosomal dominant polycystic kidney disease (ADPKD) and isolated polycystic liver disease (iPCLD). There is currently no definitive treatment except for liver transplantation. The aim of this study was to assess the expression level of aquaporin 1 (AQP1) on the PCLD cysts with different sizes and provide the potential therapeutic target.Methods: We collected 3 normal bile ducts, and recruited 8 patients with simple liver cyst disease, 24 patients with ADPKD, and 17 patients with iPCLD. AQP1 expression in different types of cyst walls and in normal bile ducts was detected using real time quantitative PCR, western blot and immunofluorescence staining. We also compared AQP1 expression levels in cysts of different sizes. Besides, ionic concentrations, pH and osmolality of cyst fluid were analyzed.Results: The results showed that AQP1 expression in PCLD cysts was significantly higher than that in simple liver cysts and the normal bile ducts. In addition, a comparable increasing trend was found in cysts of smaller sizes to cysts of larger sizes. pH values, the sodium and chloride concentrations were higher in cyst fluid than that in the serum.Conclusions: AQP1 was overexpressed in cystic cholangiocytes. A tendency of increased AQP1 protein expression in correlation with the cyst size was also found. These observations offered a direction into the molecular mechanisms of cyst expansion and maybe provide new treatment strategies to reduce fluid secretion into liver cysts.

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Polycystic liver: clinical characteristics of patients with isolated polycystic liver disease compared with patients with polycystic liver and autosomal dominant polycystic kidney disease

Abstract: Although PCLD in patients with PCLD is characterized by larger and greater number of hepatic cysts, the clinical course is relatively benign compared with ADPKD.

Cited by 104 publications

References 16 publications

Medical and surgical treatment options for polycystic liver disease1

Medical and surgical treatment options for polycystic liver disease1

Deficiency of hepatocystin induces autophagy through an mTOR-dependent pathway

Overexpression of Aquaporin 1 on cysts of patients with polycystic liver disease

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