BACKGROUND:Polycystic liver disease (PLD) is a phenotypical expression of autosomal dominant polycystic kidney disease and isolated polycystic liver disease. Somatostatin analogues, such as lanreotide, reduce polycystic liver volume. AIM: To establish long-term outcome and safety of lanreotide. METHODS: This was an open-label, observational extension study of a 6-month, randomised, placebo-controlled trial with lanreotide (120 mg/month) in PLD. The length of total treatment was 12 months. Primary endpoint was relative change in liver volume, as determined by CT-volumetry after 12 months of treatment. We offered patients a CT scan 6 months after stopping lanreotide. RESULTS: A total of 41/54 (76%) patients participated in the extension study. Liver volume decreased by 4% (IQR -8% to -1%) after 12 months of treatment. The greatest effect was observed during the first 6 months of treatment (decrease of 4% (IQR -6% to -1%)). Liver volume remained unchanged during the following 6 months. We found that liver volume increased by 4% (IQR 0-6%) 6 months after end of treatment (n = 22). CONCLUSIONS:Lanreotide reduces liver volume within the first 6 months of treatment and the beneficial effect is maintained in the following 6 months. Stopping results in recurrence of polycystic liver growth. This suggests that continuous use of lanreotide is needed to maintain its effect.
RCT Entities:
BACKGROUND:Polycystic liver disease (PLD) is a phenotypical expression of autosomal dominant polycystic kidney disease and isolated polycystic liver disease. Somatostatin analogues, such as lanreotide, reduce polycystic liver volume. AIM: To establish long-term outcome and safety of lanreotide. METHODS: This was an open-label, observational extension study of a 6-month, randomised, placebo-controlled trial with lanreotide (120 mg/month) in PLD. The length of total treatment was 12 months. Primary endpoint was relative change in liver volume, as determined by CT-volumetry after 12 months of treatment. We offered patients a CT scan 6 months after stopping lanreotide. RESULTS: A total of 41/54 (76%) patients participated in the extension study. Liver volume decreased by 4% (IQR -8% to -1%) after 12 months of treatment. The greatest effect was observed during the first 6 months of treatment (decrease of 4% (IQR -6% to -1%)). Liver volume remained unchanged during the following 6 months. We found that liver volume increased by 4% (IQR 0-6%) 6 months after end of treatment (n = 22). CONCLUSIONS: Lanreotide reduces liver volume within the first 6 months of treatment and the beneficial effect is maintained in the following 6 months. Stopping results in recurrence of polycystic liver growth. This suggests that continuous use of lanreotide is needed to maintain its effect.
Authors: Patricia Munoz-Garrido; José J G Marin; María J Perugorria; Aura D Urribarri; Oihane Erice; Elena Sáez; Miriam Úriz; Sarai Sarvide; Ainhoa Portu; Axel R Concepcion; Marta R Romero; María J Monte; Álvaro Santos-Laso; Elizabeth Hijona; Raúl Jimenez-Agüero; Marco Marzioni; Ulrich Beuers; Tatyana V Masyuk; Nicholas F LaRusso; Jesús Prieto; Luis Bujanda; Joost P H Drenth; Jesús M Banales Journal: J Hepatol Date: 2015-06-01 Impact factor: 25.083
Authors: Maria J Perugorria; Tatyana V Masyuk; Jose J Marin; Marco Marzioni; Luis Bujanda; Nicholas F LaRusso; Jesus M Banales Journal: Nat Rev Gastroenterol Hepatol Date: 2014-09-30 Impact factor: 46.802
Authors: Myrte K Neijenhuis; Tom J G Gevers; Marie C Hogan; Patrick S Kamath; Titus F M Wijnands; Ralf C P M van den Ouweland; Marie E Edwards; Jeff A Sloan; Wietske Kievit; Joost P H Drenth Journal: Hepatology Date: 2016-04-15 Impact factor: 17.425