scholarly journals Congenital Hepatic Fibrosis and Autosomal Recessive Polycystic Kidney Disease

2012 ◽  
Vol 54 (5) ◽  
pp. 580-587 ◽  
Author(s):  
Arvind Srinath ◽  
Benjamin L. Shneider
Keyword(s):  
Kidney Disease ◽  
Polycystic Kidney Disease ◽  
Hepatic Fibrosis ◽  
Autosomal Recessive ◽  
Congenital Hepatic Fibrosis ◽  
Polycystic Kidney

scholarly journals PKHD1 sequence variations in 78 children and adults with autosomal recessive polycystic kidney disease and congenital hepatic fibrosis

2010 ◽  
Vol 99 (2) ◽  
pp. 160-173 ◽  
Author(s):  
Meral Gunay-Aygun ◽  
Maya Tuchman ◽  
Esperanza Font-Montgomery ◽  
Linda Lukose ◽  
Hailey Edwards ◽  
...  
Keyword(s):  
Kidney Disease ◽  
Polycystic Kidney Disease ◽  
Hepatic Fibrosis ◽  
Autosomal Recessive ◽  
Congenital Hepatic Fibrosis ◽  
Polycystic Kidney ◽  
Sequence Variations

scholarly journals Autosomal recessive polycystic kidney disease and congenital hepatic fibrosis (ARPKD/CHF)

2008 ◽  
Vol 39 (2) ◽  
pp. 100-111 ◽  
Author(s):  
Baris Turkbey ◽  
Iclal Ocak ◽  
Kailash Daryanani ◽  
Esperanza Font-Montgomery ◽  
Linda Lukose ◽  
...  
Keyword(s):  
Kidney Disease ◽  
Polycystic Kidney Disease ◽  
Hepatic Fibrosis ◽  
Autosomal Recessive ◽  
Congenital Hepatic Fibrosis ◽  
Polycystic Kidney

scholarly journals Evidence for a “Pathogenic Triumvirate” in Congenital Hepatic Fibrosis in Autosomal Recessive Polycystic Kidney Disease

2016 ◽  
Vol 2016 ◽  
pp. 1-10 ◽  
Author(s):  
Lu Jiang ◽  
Pingping Fang ◽  
James L. Weemhoff ◽  
Udayan Apte ◽  
Michele T. Pritchard
Keyword(s):  
Kidney Disease ◽  
Polycystic Kidney Disease ◽  
Hepatic Fibrosis ◽  
Cyst Wall ◽  
Autosomal Recessive ◽  
Fluid Secretion ◽  
Congenital Hepatic Fibrosis ◽  
Therapeutic Approaches ◽  
Polycystic Kidney ◽  
Cyst Growth

Autosomal recessive polycystic kidney disease (ARPKD) is a severe monogenic disorder that occurs due to mutations in thePKHD1gene. Congenital hepatic fibrosis (CHF) associated with ARPKD is characterized by the presence of hepatic cysts derived from dilated bile ducts and a robust, pericystic fibrosis. Cyst growth, due to cyst wall epithelial cell hyperproliferation and fluid secretion, is thought to be the driving force behind disease progression. Liver fibrosis is a wound healing response in which collagen accumulates in the liver due to an imbalance between extracellular matrix synthesis and degradation. Whereas both hyperproliferation and pericystic fibrosis are hallmarks of CHF/ARPKD, whether or not these two processes influence one another remains unclear. Additionally, recent studies demonstrate that inflammation is a common feature of CHF/ARPKD. Therefore, we propose a “pathogenic triumvirate” consisting of hyperproliferation of cyst wall growth, pericystic fibrosis, and inflammation which drives CHF/ARPKD progression. This review will summarize what is known regarding the mechanisms of cyst growth, fibrosis, and inflammation in CHF/ARPKD. Further, we will discuss the potential advantage of identifying a core pathogenic feature in CHF/ARPKD to aid in the development of novel therapeutic approaches. If a core pathogenic feature does not exist, then developing multimodality therapeutic approaches to target each member of the “pathogenic triumvirate” individually may be a better strategy to manage this debilitating disease.

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

Morbidity from Congenital Hepatic Fibrosis after Renal Transplantation for Autosomal Recessive Polycystic Kidney Disease

2002 ◽  
Vol 2 (4) ◽  
pp. 360-365 ◽  
Author(s):  
Khalid Khan ◽  
Sara Jane Schwarzenberg ◽  
Harvey L. Sharp ◽  
Arthur J. Matas ◽  
Blanche M. Chavers
Keyword(s):  
Renal Transplantation ◽  
Kidney Disease ◽  
Polycystic Kidney Disease ◽  
Hepatic Fibrosis ◽  
Autosomal Recessive ◽  
Congenital Hepatic Fibrosis ◽  
Polycystic Kidney

Variable expression of autosomal recessive polycystic kidney disease and congenital hepatic fibrosis within a family

1988 ◽  
Vol 29 (3) ◽  
pp. 639-647 ◽  
Author(s):  
Bernard S. Kaplan ◽  
Paige Kaplan ◽  
Jean-Pierre de Chadarevian ◽  
Sigrid Jequier ◽  
Sean O'Regan ◽  
...  
Keyword(s):  
Kidney Disease ◽  
Polycystic Kidney Disease ◽  
Hepatic Fibrosis ◽  
Autosomal Recessive ◽  
Congenital Hepatic Fibrosis ◽  
Polycystic Kidney ◽  
Variable Expression
Sign in / Sign up

Export Citation Format

Share Document