Abstract: Background: Hereditary angioedema is a rare autosomal dominantly inherited immunodeficiency disorder characterized by potentially life-threatening angioedema attacks. Objective: We aimed to investigate the clinical and genetic features of a family with angioedema attacks. Methods: The medical history, clinical features and C1-INH gene mutation of a Turkish family were investigated and outcomes of long-term treatments were described. Results: Five members had experienced recurrent swellings on the face and extremities triggered by trauma. They were all misdiagnosed as familial Mediterranean fever (FMF) depending on frequent abdominal pain and were on colchicine therapy for a long time. They had low C4 and C1-INH protein concentrations and functions. A mutation (c.1247T>A) in C1-INH gene was detected. They were diagnosed as having hereditary angioedema with C1-INH deficiency (C1-INH hereditary angioedema) for the first time. Three of them benefited from danazol treatment without any significant adverse events and one received weekly C1 esterase replacement treatment instead of danazol since she had a medical history of thromboembolic stroke. Study limitations: Small sample size of participants. Conclusion: Patients with C1-INH hereditary angioedema may be misdiagnosed as having familial Mediterranean fever in regions where the disorder is endemic. Medical history, suspicion of hereditary angioedema and laboratory evaluations of patients and their family members lead the correct diagnoses of hereditary angioedema. Danazol and C1 replacement treatments provide significant reduction in hereditary angioedema attacks.

ABSTRACT Purpose To determine whether using different intraperitoneal insufflation pressures for transperitoneal laparoscopic urologic surgeries decreases postoperative pain. Materials and Methods 76 patients who underwent transperitoneal laparoscopic upper urinary tract surgery at different insufflation pressures were allocated into the following groups: 10mmHg (group I, n=24), 12mmHg (group II, n=25) and 14mmHg (group III, n=27). These patients were compared according to age, gender, body mass index (BMI), type and duration of surgery, intraoperative bleeding volume, postoperative pain score and length of hospital stay. A visual analog scale (VAS) was used for postoperative pain. Results Demographic characteristics, mean age, gender, BMI and type of surgeries were statistically similar among the groups. The mean operation time was higher in group I than group II and group III but this was not statistically significant (P=0.810). The mean intraoperative bleeding volume was significantly higher in group I compared with group II and group III (P=0.030 and P=0.006). The mean length of postoperative hospital stays was statistically similar among the groups (P=0.849). The mean VAS score at 6h was significantly reduced in group I compared with group III (P=0.011). At 12h, the mean VAS score was significantly reduced in group I compared with group II and group III (P=0.009 and P<0.001). There was no significant difference in the mean VAS scores at 24h among three groups (P=0.920). Conclusion Lower insufflation pressures are associated with lower postoperative pain scores in the early postoperative period.

ABSTRACT Purpose Postoperative urinary retention (POUR) is one of the most common complications after surgical procedures under spinal anaesthesia. Recent studies have shown the beneficial effects of alpha-adrenergic blockers in preventing POUR. The aim of this prospective study was to investigate and compare the prophylactic effects of tamsulosin and alfuzosin on POUR after urologic surgical procedures under spinal anaesthesia. Materials and Methods A total of 180 males who underwent elective urologic surgery were included in this study. The patients were randomly allocated into three Groups. The Group I received placebo. Patients in Group II were given 0.4mg of tamsulosin orally 14 and 2 hours before surgery. Patients in Group III were given 10mg of alfuzosin ER orally 10 and 2 hours before surgery. All patients were closely followed for 24 hours postoperatively and their episodes of urinary retentions were recorded. Results There were 60 patients in each Group. Their mean age was 35.95±15.16 years. Fifteen patients in Group I (25%), 3 patients in Group II (5%) and 4 patients in Group III (6.7%) required catheterization because of urinary retention. In tamsulosin group and alfuzosin group, there were a significantly lower proportion of patients with POUR compared with the placebo Group (p=0.002 and p=0.006). The beneficial effects of tamsulosin and alfuzosin on POUR were similar between both Groups (p=0.697). Conclusion This study suggests that the use of prophylactic tamsulosin or alfuzosin can reduce the incidence of urinary retention and the need for catheterization after urologic surgical procedures under spinal anaesthesia.