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Background: Approximately 28% of the patients with cystic fibrosis are affected by cholelythiasis. More than 40% of them have a symptomatic disease, which would mandate cholecystectomy., Aim: The aim of this study was to review surgical and respiratory outcomes and quality of life scores of cystic fibrosis patients undergoing laparoscopic cholecystectomy for symptomatic cholelythiasis to verify the hypothesis that cholecystectomy is a low-risk operation by laparoscopy, not affecting unfavorably respiratory function and quality of life., Patients and Methods: Study group was consisted of 9 patients with a mean age of 24.8±8.1 years (range, 15 to 38 y), 2 male and 7 female patients, with cystic fibrosis and symptomatic cholelithiasis. Three patients also presented common bile duct stones. All the patients underwent perioperative Positive End-Expiratory Pressure mask sessions and aggressive antibiotic regimens. At the middle of the antibiotics regimen period, a standard laparoscopic cholecystectomy was performed. In the 3 cases with common duct lithiasis, the so-called "rendezvous" technique was carried out. Preoperatively, intraoperatively, and postoperatively, respiratory function was strictly monitored by the evaluation of SO2 and of the forced expiratory volume in 1 second (FEV1). Preoperatively and 6 months after laparoscopic cholecystectomy the Gastro Intestinal Quality of Life Index was evaluated on all patients., Results: All the operations were completed laparoscopically. No mortality was observed. The intraoperative mean SO2 was 89.0%±5.6% (range, 80% to 95%), versus 82.8%±8.5% (range, 66% to 91%) at the extubation (P=0.006). Intraoperative respiratory functions were stable in 6 patients. In 3 patients, a severe bronchospasm occurred determining marked desaturation. Preoperative mean FEV1 was 70.5%±7.0% (range, 55% to 75%) versus 61.8%±13.2% (range, 39% to 80%) 48 hours after the operation (P=0.132). The 3 patients, who experienced intraoperatively severe bronchospasm, reported a 48 hours postoperative FEV1 under 60%. All the patients showed disappearance of postprandial colicky pain and vomiting. Preoperative mean total Gastro Intestinal Quality of Life Index score was 105.2±13.6 versus 117.8±10 at 6-month follow-up (P=0.015)., Conclusions: On the basis of a proper surgical timing and adequate preoperative physiokinesis therapy, laparoscopic cholecystectomy is a safe and indicated procedure in patients with cystic fibrosis and symptomatic cholelithiasis and it is able to significantly improve the quality of life. Quality of life of these patients it not worsened while symptoms and risks of biliary gallstones are removed.