Universität zu Köln

Mueller, Roman-Ulrich, Haas, Christian S. and Sayer, John A. ORCID: 0000-0003-1881-3782 (2018). Practical approaches to the management of autosomal dominant polycystic kidney disease patients in the era of tolvaptan. Clin. Kidney J., 11 (1). S. 62 - 70. OXFORD: OXFORD UNIV PRESS. ISSN 2048-8513

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Abstract

Background: Autosomal dominant polycystic kidney disease (ADPKD) is the most common inherited kidney disease worldwide. The renal phenotype is characterized by progressive cystic enlargement of the kidneys leading to a decline in renal function, hypertension and often end-stage renal disease (ESRD). Supportive care with blood pressure control and management of pain, urinary infections and renal stone disease has, until recently, been the mainstay of treatment. With the recent approval of tolvaptan for use in ADPKD, the disease progression may now be targeted specifically. Algorithms that guide treatment initiation have been proposed but a more pragmatic and patient-individualized approach is often needed to make decisions regarding therapy. It is highly important to identify ADPKD patients with rapidly progressive disease who are likely to benefit most from this treatment and avoid treatment in patients that are unlikely to reach ESRD. Methods and Results: Here we present a series of cases of ADPKD patients in whom therapy with tolvaptan has been considered and report the rationale for the treatment decisions based on available lifestyle, clinical, biochemical, radiological and genetic data. Conclusions: These cases provide a discussion for the use of tolvaptan in ADPKD within the nephrology clinic and allow insights into the practicalities of using this therapy outside of clinical trials.

Item Type:	Journal Article
Creators:	Creators Email ORCID ORCID Put Code Mueller, Roman-Ulrich UNSPECIFIED UNSPECIFIED UNSPECIFIED Haas, Christian S. UNSPECIFIED UNSPECIFIED UNSPECIFIED Sayer, John A. UNSPECIFIED orcid.org/0000-0003-1881-3782 UNSPECIFIED
URN:	urn:nbn:de:hbz:38-197094
DOI:	10.1093/ckj/sfx071
Journal or Publication Title:	Clin. Kidney J.
Volume:	11
Number:	1
Page Range:	S. 62 - 70
Date:	2018
Publisher:	OXFORD UNIV PRESS
Place of Publication:	OXFORD
ISSN:	2048-8513
Language:	English
Faculty:	Unspecified
Divisions:	Unspecified
Subjects:	no entry
Uncontrolled Keywords:	Keywords Language RENAL REPLACEMENT THERAPY; VOLUME PROGRESSION; MUTATED GENE; PREDICTORS; DIAGNOSIS; OUTCOMES Multiple languages Urology & Nephrology Multiple languages
Refereed:	Yes
URI:	http://kups.ub.uni-koeln.de/id/eprint/19709