Universität zu Köln

Janssens, Peter ORCID: 0000-0002-0981-8621, Weydert, Caroline ORCID: 0000-0001-6598-4395, De Rechter, Stephanie, Wissing, Karl Martin, Liebau, Max Christoph ORCID: 0000-0003-0494-9080 and Mekahli, Djalila ORCID: 0000-0003-0954-6088 (2018). Expanding the role of vasopressin antagonism in polycystic kidney diseases: From adults to children? Pediatr. Nephrol., 33 (3). S. 395 - 409. NEW YORK: SPRINGER. ISSN 1432-198X

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Abstract

Polycystic kidney disease (PKD) encompasses a group of genetic disorders that are common causes of renal failure. The two classic forms of PKD are autosomal recessive polycystic kidney disease (ARPKD) and autosomal dominant polycystic kidney disease (ADPKD). Despite their clinical differences, ARPKD and ADPKD share many similarities. Altered intracellular Ca2+ and increased cyclic adenosine monophosphate (cAMP) concentrations have repetitively been described as central anomalies that may alter signaling pathways leading to cyst formation. The vasopressin V2 receptor (V2R) antagonist tolvaptan lowers cAMP in cystic tissues and slows renal cystic progression and kidney function decline when given over 3 years in adult ADPKD patients. Tolvaptan is currently approved for the treatment of rapidly progressive disease in adult ADPKD patients. On the occasion of the recent initiation of a clinical trial with tolvaptan in pediatric ADPKD patients, we aim to describe the most important aspects in the literature regarding the AVP-cAMP axis and the clinical use of tolvaptan in PKD.

Item Type:	Journal Article
Creators:	Creators Email ORCID ORCID Put Code Janssens, Peter UNSPECIFIED orcid.org/0000-0002-0981-8621 UNSPECIFIED Weydert, Caroline UNSPECIFIED orcid.org/0000-0001-6598-4395 UNSPECIFIED De Rechter, Stephanie UNSPECIFIED UNSPECIFIED UNSPECIFIED Wissing, Karl Martin UNSPECIFIED UNSPECIFIED UNSPECIFIED Liebau, Max Christoph UNSPECIFIED orcid.org/0000-0003-0494-9080 UNSPECIFIED Mekahli, Djalila UNSPECIFIED orcid.org/0000-0003-0954-6088 UNSPECIFIED
URN:	urn:nbn:de:hbz:38-193628
DOI:	10.1007/s00467-017-3672-x
Journal or Publication Title:	Pediatr. Nephrol.
Volume:	33
Number:	3
Page Range:	S. 395 - 409
Date:	2018
Publisher:	SPRINGER
Place of Publication:	NEW YORK
ISSN:	1432-198X
Language:	English
Faculty:	Unspecified
Divisions:	Unspecified
Subjects:	no entry
Uncontrolled Keywords:	Keywords Language V2 RECEPTOR ANTAGONIST; PLANAR CELL POLARITY; RENAL CONCENTRATING CAPACITY; AUTOSOMAL-DOMINANT; SURROGATE MARKER; PRIMARY CILIA; CYST GROWTH; ARGININE-VASOPRESSIN; SIGNALING PATHWAYS; PKHD1 MUTATIONS Multiple languages Pediatrics; Urology & Nephrology Multiple languages
Refereed:	Yes
URI:	http://kups.ub.uni-koeln.de/id/eprint/19362