Two-dimensional (2D) morphologic measurements can quantify the severity of liver disease in children with autosomal recessive polycystic kidney disease (ARPKD)

2021 ◽  
Author(s):  
Adarsh Ghosh ◽  
Suraj D. Serai ◽  
Shyam Sunder B. Venkatakrishna ◽  
Mohini Dutt ◽  
Erum A. Hartung
Keyword(s):  
Liver Disease ◽  
Kidney Disease ◽  
Polycystic Kidney Disease ◽  
Autosomal Recessive ◽  
Two Dimensional ◽  
Polycystic Kidney

scholarly journals Ultrasound Elastography to Quantify Liver Disease Severity in Autosomal Recessive Polycystic Kidney Disease

2019 ◽  
Vol 209 ◽  
pp. 107-115.e5 ◽  
Author(s):  
Erum A. Hartung ◽  
Jessica Wen ◽  
Laura Poznick ◽  
Susan L. Furth ◽  
Kassa Darge
Keyword(s):  
Liver Disease ◽  
Kidney Disease ◽  
Polycystic Kidney Disease ◽  
Disease Severity ◽  
Autosomal Recessive ◽  
Ultrasound Elastography ◽  
Polycystic Kidney

Liver Disease in Autosomal Recessive Polycystic Kidney Disease

2014 ◽  
Vol 59 (2) ◽  
pp. 190-196 ◽  
Author(s):  
Topi T. Luoto ◽  
Mikko P. Pakarinen ◽  
Timo Jahnukainen ◽  
Hannu Jalanko
Keyword(s):  
Liver Disease ◽  
Kidney Disease ◽  
Polycystic Kidney Disease ◽  
Autosomal Recessive ◽  
Polycystic Kidney

scholarly journals PPAR-γ agonist ameliorates kidney and liver disease in an orthologous rat model of human autosomal recessive polycystic kidney disease

2011 ◽  
Vol 300 (2) ◽  
pp. F465-F474 ◽  
Author(s):  
Daisuke Yoshihara ◽  
Hiroki Kurahashi ◽  
Miwa Morita ◽  
Masanori Kugita ◽  
Yoshiyuki Hiki ◽  
...  
Keyword(s):  
Cell Proliferation ◽  
Body Weight ◽  
Liver Disease ◽  
Kidney Disease ◽  
Polycystic Kidney Disease ◽  
Autosomal Recessive ◽  
Interstitial Fibrosis ◽  
Polycystic Kidney ◽  
Ppar Γ ◽  
Cystic Area

In autosomal recessive polycystic kidney disease (ARPKD), progressive enlargement of fluid-filled cysts is due to aberrant proliferation of tubule epithelial cells and transepithelial fluid secretion leading to extensive nephron loss and interstitial fibrosis. Congenital hepatic fibrosis associated with biliary cysts/dilatations is the most common extrarenal manifestation in ARPKD and can lead to massive liver enlargement. Peroxisome proliferator-activated receptor γ (PPAR-γ), a member of the ligand-dependent nuclear receptor superfamily, is expressed in a variety of tissues, including the kidneys and liver, and plays important roles in cell proliferation, fibrosis, and inflammation. In the current study, we determined that pioglitazone (PIO), a PPAR-γ agonist, decreases polycystic kidney and liver disease progression in the polycystic kidney rat, an orthologous model of human ARPKD. Daily treatment with 10 mg/kg PIO for 16 wk decreased kidney weight (% of body weight), renal cystic area, serum urea nitrogen, and the number of Ki67-, pERK1/2-, and pS6-positive cells in the kidney. There was also a decrease in liver weight (% of body weight), liver cystic area, fibrotic index, and the number of Ki67-, pERK1/2-, pERK5-, and TGF-β-positive cells in the liver. Taken together, these data suggest that PIO inhibits the progression of polycystic kidney and liver disease in a model of human ARPKD by inhibiting cell proliferation and fibrosis. These findings suggest that PPAR-γ agonists may have therapeutic value in the treatment of the renal and hepatic manifestations of ARPKD.

Liver disease in autosomal recessive polycystic kidney disease

2005 ◽  
Vol 9 (5) ◽  
pp. 634-639 ◽  
Author(s):  
Benjamin L. Shneider ◽  
Margret S. Magid
Keyword(s):  
Liver Disease ◽  
Kidney Disease ◽  
Polycystic Kidney Disease ◽  
Autosomal Recessive ◽  
Polycystic Kidney

scholarly journals Telmisartan Ameliorates Fibrocystic Liver Disease in an Orthologous Rat Model of Human Autosomal Recessive Polycystic Kidney Disease

PLoS ONE ◽  
2013 ◽  
Vol 8 (12) ◽  
pp. e81480 ◽  
Author(s):  
Daisuke Yoshihara ◽  
Masanori Kugita ◽  
Mai Sasaki ◽  
Shigeo Horie ◽  
Koichi Nakanishi ◽  
...  
Keyword(s):  
Liver Disease ◽  
Kidney Disease ◽  
Polycystic Kidney Disease ◽  
Rat Model ◽  
Autosomal Recessive ◽  
Polycystic Kidney

scholarly journals Prospective evaluation of kidney and liver disease in autosomal recessive polycystic kidney disease-congenital hepatic fibrosis

2020 ◽  
Vol 131 (1-2) ◽  
pp. 267-276
Author(s):  
Nehna Abdul Majeed ◽  
Esperanza Font-Montgomery ◽  
Linda Lukose ◽  
Joy Bryant ◽  
Peter Veppumthara ◽  
...  
Keyword(s):  
Liver Disease ◽  
Kidney Disease ◽  
Polycystic Kidney Disease ◽  
Hepatic Fibrosis ◽  
Autosomal Recessive ◽  
Congenital Hepatic Fibrosis ◽  
Prospective Evaluation ◽  
Polycystic Kidney

scholarly journals Initial evaluation of hepaticT1relaxation time as an imaging marker of liver disease associated with autosomal recessive polycystic kidney disease (ARPKD)

2015 ◽  
Vol 29 (1) ◽  
pp. 84-89 ◽  
Author(s):  
Ying Gao ◽  
Bernadette O. Erokwu ◽  
David A. DeSantis ◽  
Colleen M. Croniger ◽  
Rebecca M. Schur ◽  
...  
Keyword(s):  
Liver Disease ◽  
Kidney Disease ◽  
Polycystic Kidney Disease ◽  
Autosomal Recessive ◽  
Initial Evaluation ◽  
Polycystic Kidney

Can progression of autosomal dominant or autosomal recessive polycystic kidney disease be prevented?

2001 ◽  
Vol 21 (5) ◽  
pp. 430-440 ◽  
Author(s):  
Ira D. Davis ◽  
Katherine MacRae Dell ◽  
William E. Sweeney ◽  
Ellis D. Avner
Keyword(s):  
Kidney Disease ◽  
Polycystic Kidney Disease ◽  
Autosomal Recessive ◽  
Autosomal Dominant ◽  
Polycystic Kidney
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