Abstract

AMPUERO, Javier et al. Polycystic liver in the adult (PLA) in Spain: analysis of a structured survey analysing the experience and attitude of gastroenterologists in Spain. Rev. esp. enferm. dig. [online]. 2014, vol.106, n.4, pp.263-275. ISSN 1130-0108.

Background: Polycystic liver in the adult (PLA) is a rare disease characterized by chronic liver enlargement. Objective: To analyse gastroenterologists' involvement in, experience with, and attitude toward diagnosing, monitoring, and treating patients with PLA in Spain. Methods: Each of seven study coordinators contacted 15 specialists in their geographic area about participating in the study via an online structured survey. Results: Of the 105 clinics contacted, 88 completed the questionnaire, with a mean of 3 patients being followed per practice, although 6 clinics were following more than 20 patients with PLA. Patients were being followed mainly by the Department of Hepatology (81 %) and/or the Department of Gastroenterology (33 %). The majority of patients were diagnosed (98 %) and monitored (97 %) using liver ultrasound. When diagnosed, 76 % of patients were under 50 years of age, females predominating. The primary treatment objective for the patients was symptomatic management. Pharmacotherapy was prescribed by 28 % of physicians: Somatostatin analogues, primarily, followed by mTOR inhibitors. One-third of the clinics indicated that they had patients who had undergone liver transplant and/or surgery. Conclusions: Ultrasound is the diagnosing and monitoring method of choice. Among the clinics using pharmacotherapy for symptomatic management, somatostatin analogues were the drugs of choice. These clinics' infrequent use of invasive procedures suggests that they perceive the various invasive techniques as not very effective.

Keywords : Multiple hepatic cysts; Polycystic kidney disease; Polycystic liver disease; Somatostatin analogues; mTOR inhibitors; Liver transplant; Fenestration via laparotomy or laparoscopy; Autosomal-dominant polycistic liver-disease (PCLD); Autosomal-dominant polycistic kidney disease (PCKD).

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