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Evidence for a “Pathogenic Triumvirate” in Congenital Hepatic Fibrosis in Autosomal Recessive Polycystic Kidney Disease
Joint Authors
Apte, Udayan
Pritchard, Michele
Jiang, Lu
Fang, Pingping
Weemhoff, James L.
Source
Issue
Vol. 2016, Issue 2016 (31 Dec. 2016), pp.1-10, 10 p.
Publisher
Hindawi Publishing Corporation
Publication Date
2016-11-07
Country of Publication
Egypt
No. of Pages
10
Main Subjects
Abstract EN
Autosomal recessive polycystic kidney disease (ARPKD) is a severe monogenic disorder that occurs due to mutations in the PKHD1 gene.
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.
American Psychological Association (APA)
Jiang, Lu& Fang, Pingping& Weemhoff, James L.& Apte, Udayan& Pritchard, Michele. 2016. Evidence for a “Pathogenic Triumvirate” in Congenital Hepatic Fibrosis in Autosomal Recessive Polycystic Kidney Disease. BioMed Research International،Vol. 2016, no. 2016, pp.1-10.
https://search.emarefa.net/detail/BIM-1097942
Modern Language Association (MLA)
Jiang, Lu…[et al.]. Evidence for a “Pathogenic Triumvirate” in Congenital Hepatic Fibrosis in Autosomal Recessive Polycystic Kidney Disease. BioMed Research International No. 2016 (2016), pp.1-10.
https://search.emarefa.net/detail/BIM-1097942
American Medical Association (AMA)
Jiang, Lu& Fang, Pingping& Weemhoff, James L.& Apte, Udayan& Pritchard, Michele. Evidence for a “Pathogenic Triumvirate” in Congenital Hepatic Fibrosis in Autosomal Recessive Polycystic Kidney Disease. BioMed Research International. 2016. Vol. 2016, no. 2016, pp.1-10.
https://search.emarefa.net/detail/BIM-1097942
Data Type
Journal Articles
Language
English
Notes
Includes bibliographical references
Record ID
BIM-1097942