Center is an important indicator for choice of invasive therapy in polycystic liver disease

Summary Polycystic liver disease (PLD) is a rare genetic disorder with progressive cyst growth as the primary phenotype. Therapy consists of volume reduction through invasive surgical or radiological procedures. To understand the process of treatment decision, our aim was to identify factors that in...

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Veröffentlicht in:Transplant international 2017-01, Vol.30 (1), p.76-82
Hauptverfasser: D'Agnolo, Hedwig M. A., Kievit, Wietske, Munster, Kim N., Laan, Jouke J. H., Nevens, Frederik, Drenth, Joost P. H.
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container_issue 1
container_start_page 76
container_title Transplant international
container_volume 30
creator D'Agnolo, Hedwig M. A.
Kievit, Wietske
Munster, Kim N.
Laan, Jouke J. H.
Nevens, Frederik
Drenth, Joost P. H.
description Summary Polycystic liver disease (PLD) is a rare genetic disorder with progressive cyst growth as the primary phenotype. Therapy consists of volume reduction through invasive surgical or radiological procedures. To understand the process of treatment decision, our aim was to identify factors that increased the likelihood of treatment. We performed a cross‐sectional study using an international population of patients with PLD. We collected data on the following therapies: liver transplantation, resection, fenestration, and aspiration sclerotherapy. Data on the potential determinants, sex, center, autosomal dominant polycystic kidney disease (ADPKD), autosomal dominant polycystic liver disease (ADPLD), age at diagnosis, symptoms, and phenotype, were included. We corrected for follow‐up time. We included 578 patients in our study, and 35% underwent invasive therapy. Multivariate regression analysis showed that number of symptoms and age at diagnosis of PLD increased the likelihood of treatment (respectively, RR: 1.4, P < 0.001 and RR = 1.4, P = 0.03). The choice for liver transplantation or aspiration sclerotherapy was center dependent (RR: 0.7, P < 0.001 and RR: 1.1, P = 0.03, respectively). The results of our international cross‐sectional study suggest that a higher number of symptoms and every 10 years of PLD diagnosis increase the risk to undergo treatment by 40%. The choice to elect a particular modality is center dependent.
doi_str_mv 10.1111/tri.12875
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A. ; Kievit, Wietske ; Munster, Kim N. ; Laan, Jouke J. H. ; Nevens, Frederik ; Drenth, Joost P. H.</creator><creatorcontrib>D'Agnolo, Hedwig M. A. ; Kievit, Wietske ; Munster, Kim N. ; Laan, Jouke J. H. ; Nevens, Frederik ; Drenth, Joost P. H.</creatorcontrib><description>Summary Polycystic liver disease (PLD) is a rare genetic disorder with progressive cyst growth as the primary phenotype. Therapy consists of volume reduction through invasive surgical or radiological procedures. To understand the process of treatment decision, our aim was to identify factors that increased the likelihood of treatment. We performed a cross‐sectional study using an international population of patients with PLD. We collected data on the following therapies: liver transplantation, resection, fenestration, and aspiration sclerotherapy. 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subjects Adult
Age Factors
autosomal dominant polycystic kidney disease
autosomal dominant polycystic liver disease
Cross-Sectional Studies
Cysts - surgery
Female
Follow-Up Studies
Genes, Dominant
Hospitals - classification
Humans
Liver Diseases - surgery
Liver Transplantation
Male
Middle Aged
Multivariate Analysis
Phenotype
Polycystic Kidney, Autosomal Dominant - surgery
polycystic liver disease
Registries
Retrospective Studies
Sclerotherapy - methods
surgery
treatment
Treatment Outcome
title Center is an important indicator for choice of invasive therapy in polycystic liver disease
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