Browsing by Subject "Mouth"

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    Avoiding allogenic blood transfusions in endoscopic angiofibroma surgery
    (2016) Wasl, Hisham; McGuire, Jessica; Lubbe, Darlene
    BackgroundSurgical approaches for many tumours are often limited by blood loss, exposure and risk to vital anatomical structures. Therefore, the standard of care for certain skull base tumours has become endoscopic transnasal resection. Other surgical disciplines often use cell salvage techniques, but review of the otolaryngology literature revealed very few case reports. This study investigated the value and safety of salvage-type autologous blood transfusion during the endoscopic resection of juvenile nasopharyngeal angiofibromas (JNA).MethodsJNA is a rare vascular nasal tumour and the study extended over a 3-year period to obtain adequate patient numbers. All patients undergoing endoscopic resection during this period were included in the population sample. Ten patients with JNA were identified and underwent embolization prior to the endoscopic resection. In all cases the intraoperative blood salvage apparatus was used. Close post-operative monitoring was performed.ResultsHomologous blood transfusion could be avoided in all cases. Postoperative monitoring revealed transient bacteraemia in two cases where the leukocyte filter was not used, but no evidence of septicaemia.ConclusionsPerioperative cell saver and autologous blood transfusion in endonasal JNA surgery is safe. Homologous blood transfusion can be avoided by using this technique. The use of cell salvage allows for single stage surgery without the need to abandon surgery due to excessive blood loss and its future use is promising.
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    Open Access
    Long-term use of Everolimus in patients with Tuberous Sclerosis Complex: final results from the EXIST-1 study
    (Public Library of Science, 2016) Franz, David N; Belousova, Elena; Sparagana, Steven; Bebin, E Martina; Frost, Michael D; Kuperman, Rachel; Witt, Olaf; Kohrman, Michael H; Flamini, J Robert; Wu, Joyce Y; Curatolo, Paolo; De Vries, Petrus J; Berkowitz, Noah; Niolat, Julie; Jóźwiak, Sergiusz
    BACKGROUND: Everolimus, a mammalian target of rapamycin (mTOR) inhibitor, has demonstrated efficacy in treating subependymal giant cell astrocytomas (SEGAs) and other manifestations of tuberous sclerosis complex (TSC). However, long-term use of mTOR inhibitors might be necessary. This analysis explored long-term efficacy and safety of everolimus from the conclusion of the EXIST-1 study (NCT00789828). Methods and FINDINGS: EXIST-1 was an international, prospective, double-blind, placebo-controlled phase 3 trial examining everolimus in patients with new or growing TSC-related SEGA. After a double-blind core phase, all remaining patients could receive everolimus in a long-term, open-label extension. Everolimus was initiated at a dose (4.5 mg/m 2 /day) titrated to a target blood trough of 5-15 ng/mL. SEGA response rate (primary end point) was defined as the proportion of patients achieving confirmed ≥50% reduction in the sum volume of target SEGA lesions from baseline in the absence of worsening nontarget SEGA lesions, new target SEGA lesions, and new or worsening hydrocephalus. Of 111 patients (median age, 9.5 years) who received ≥1 dose of everolimus (median duration, 47.1 months), 57.7% (95% confidence interval [CI], 47.9-67.0) achieved SEGA response. Of 41 patients with target renal angiomyolipomas at baseline, 30 (73.2%) achieved renal angiomyolipoma response. In 105 patients with ≥1 skin lesion at baseline, skin lesion response rate was 58.1%. Incidence of adverse events (AEs) was comparable with that of previous reports, and occurrence of emergent AEs generally decreased over time. The most common AEs (≥30% incidence) suspected to be treatment-related were stomatitis (43.2%) and mouth ulceration (32.4%). CONCLUSIONS: Everolimus use led to sustained reduction in tumor volume, and new responses were observed for SEGA and renal angiomyolipoma from the blinded core phase of the study. These findings support the hypothesis that everolimus can safely reverse multisystem manifestations of TSC in a significant proportion of patients. Trial Registration ClinicalTrials.gov NCT00789828