1406 POLYCYSTIC LIVER DISEASE PATIENTS HAVE LOW QUALITY OF LIFE WHICH IS ASSOCIATED WITH CONCURRENT ABDOMINAL SYMPTOMS

2013 ◽  
Vol 58 ◽  
pp. S564
Author(s):  
T.F. Wijnands ◽  
T.J. Gevers ◽  
W. Kievit ◽  
J.P. Drenth
2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Carlo Garofalo ◽  
Ivana Capuano ◽  
Luigi Pennino ◽  
Ilaria De Gregorio ◽  
Eleonora Riccio ◽  
...  

AbstractA clear evidence on the benefits of somatostatin analogues (SA) on liver outcome in patients affected by polycystic liver disease is still lacking. We performed a meta-analysis of RCTs and a trial sequential analysis (TSA) evaluating the effects of SA in adult patients with polycystic liver disease on change in liver volume. As secondary outcome, we evaluated the effects on quality of life as measured by SF36-questionnaire. Six RCTs were selected with an overall sample size of 332 adult patients with polycystic liver disease (mean age: 46 years). Mean liver volume at baseline was 3289 ml in SA group and 3089 ml in placebo group. Overall, unstandardized mean difference in liver volume was − 176 ml (95%CI, − 406, 54; p < 0.133). Heterogeneity was low (I2:0%, p < 0.992). However, we performed a moderator analysis and we found that a higher eGFR significantly correlates to a more pronounced effect of SA on liver volume reduction (p = 0.036). Cumulative Z-curve in TSA did not reach either significance and futility boundaries or required information size. Three RCTs have evaluated Quality of life parameters measured by SF36-QOL questionnaire for a total of 124 patients; no significant difference was found on the effect of SA on QOL parameters when compared with placebo. The present meta-analysis revealed a potential effect of SA on reduction of liver volume and quality of life parameters, but results did not reach a statistical significance. These data could be explained by the need of further studies, as demonstrated through TSA, to reach an adequate sample size to confirm the beneficial outcomes of SAs treatment.


2016 ◽  
Vol 98 (1) ◽  
pp. 18-23 ◽  
Author(s):  
S Khan ◽  
A Dennison ◽  
G Garcea

Introduction Somatostatin analogues and rapamycin inhibitors are two classes of drugs available for the management of polycystic liver disease but their overall impact is not clearly established. This article systematically reviews the literature on the medical management of polycystic liver disease. The outcomes assessed include reduction in liver volume and the impact on quality of life. Methods The English language literature published between 1966 and August 2014 was reviewed from a MEDLINE®, PubMed, Embase™ and Cochrane Library search. Search terms included ‘polycystic’, ‘liver’, ‘sirolimus’, ‘everolimus’, ‘PCLD’, ‘somatostatin’, ‘octreotide’, ‘lanreotide’ and ‘rapamycin’. Both randomised trials and controlled studies were included. References of the articles retrieved were also searched to identify any further eligible publications. The studies included were appraised using the Jadad score. Results Seven studies were included in the final review. Five studies, of which three were randomised trials, investigated the role of somatostatin analogues and the results showed a mean reduction in liver volume ranging from 2.9% at six months to 4.95 ±6.77% at one year. Only one randomised study examined the influence of rapamycin inhibitors. This trial compared dual therapy with everolimus and octreotide versus octreotide monotherapy. Liver volume reduced by 3.5% and 3.8% in the control and intervention groups respectively but no statistical difference was found between the two groups (p=0.73). Two randomised trials investigating somatostatin analogues assessed quality of life using SF-36®. Only one subdomain score improved in one of the trials while two subdomain scores improved in the other with somatostatin analogue therapy. Conclusions Somatostatin analogues significantly reduce liver volumes after six months of therapy but have only a modest improvement on quality of life. Rapamycin inhibitors do not confer any additional advantage.


2014 ◽  
Vol 34 (10) ◽  
pp. 1578-1583 ◽  
Author(s):  
Titus F. M. Wijnands ◽  
Myrte K. Neijenhuis ◽  
Wietske Kievit ◽  
Frederik Nevens ◽  
Marie C. Hogan ◽  
...  

2017 ◽  
Vol 6 (1) ◽  
pp. 81-88 ◽  
Author(s):  
Myrte K Neijenhuis ◽  
Wietske Kievit ◽  
Stef MH Verheesen ◽  
Hedwig M D’Agnolo ◽  
Tom JG Gevers ◽  
...  

Background Symptoms in polycystic liver disease (PLD) are thought to be caused by compression of organs and structures by the enlarged liver. Aim The aim of this article is to assess the impact of liver volume on symptoms and quality of life (QoL) in PLD. Methods We included PLD patients from two prospective studies that used the PLD-questionnaire (PLD-Q) for symptom assessment. QoL was assessed through SF-36, summarized in a physical (PCS) and mental (MCS) component score. Liver volume was correlated with PLD-Q total scores. Patients were classified based on height-corrected liver volume in mild (<1600 ml), moderate (1600–3200 ml), and severe (>3200 ml) disease. PLD-Q and QoL (PCS and MCS) scores were compared across disease stages. Results We included 82 of 131 patients from the original studies (disease stages; mild n = 26, moderate n = 33, and severe n = 23). Patients with larger liver volume reported higher symptom burden ( r = 0.516, p < 0.001). Symptom scores increased with disease progression, except for abdominal pain ( p = 0.088). PCS decreased with advancing disease ( p < 0.001), in contrast to MCS ( p = 0.055). Moderate ( p = 0.007) and severe ( p < 0.001) PLD patients had lower PCS scores than the general population. Conclusion PLD with larger liver volume is more likely to be symptomatic and is associated with lower QoL.


Surgery ◽  
2020 ◽  
Vol 168 (1) ◽  
pp. 25-32
Author(s):  
Lucas H.P. Bernts ◽  
Myrte K. Neijenhuis ◽  
Marie E. Edwards ◽  
Jeff A. Sloan ◽  
Jenna Fischer ◽  
...  

2006 ◽  
Vol 12 (8) ◽  
pp. 1268-1277 ◽  
Author(s):  
Gabriele I. Kirchner ◽  
Kinan Rifai ◽  
Tobias Cantz ◽  
Bjoern Nashan ◽  
Christoph Terkamp ◽  
...  

2019 ◽  
Vol 70 (1) ◽  
pp. e555-e556
Author(s):  
Lucas H.P. Bernts ◽  
Myrte Neijenhuis ◽  
Marie E. Edwards ◽  
Rory L. Smoot ◽  
David M. Nagorney ◽  
...  

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