004) and had lower heart rates (p = .01) throughout the intraoperative period compared with patients who did not receive dexmedetomidine. The combination of dexmedetomidine and ketamine may provide effective and safe IM sedation prior to the induction of GA.Nicolaides-Baraitser syndrome (NCBRS) is a rare congenital genetic disorder characterized by distinctive facial features similar to Treacher Collins syndrome (TCS). We report the first case of successful nasal fiberoptic intubation in a patient with NCBRS with micrognathia and limited mouth opening due to trismus. A 9-year-old girl with NCBRS and dental caries was scheduled to undergo general anesthesia for a dental extraction. Initial attempts at oral intubation using a video laryngoscope were unsuccessful. However, subsequent attempts at nasal intubation using a flexible fiberoptic scope were successful. This report highlights that patients with NCBRS may present with difficult airways to manage and intubate.A 36-year-old man underwent direct laryngoscopy with routine general anesthesia for a knee procedure. Several days later, he experienced pain involving an ulceration along the medial aspect of the right mandible in the floor of the mouth. This evolved to a painful bony mass, and subsequently, a bony sequestrum was spontaneously shed. The initially misdiagnosed pathologic process occurred several more times on both sides of the mouth. A computed tomography scan eventually revealed large bilateral mandibular tori, a feature that likely predisposed the patient to this course of events. Pain in the floor of the mouth after airway manipulation should be carefully evaluated and the possibility of osteonecrosis considered.Preformed cuffed oral endotracheal tubes are widely used to intubate children undergoing oral surgery. To evaluate the efficacy and safety of oral Ring-Adair-Elwyn (RAE) Microcuff® pediatric endotracheal tubes, we retrospectively investigated the endotracheal tube exchange rate and associated complications in Japanese children younger than 2 years of age undergoing cheiloplasty or palatoplasty. The exchange rate was 3.5%, and although unplanned extubations occurred in 2 patients, no severe complications were observed. Our results suggest that oral RAE Microcuff® tubes are effective and safe for intubating Japanese children younger than 2 years of age, with a low tube exchange rate and minor complications.ZUMA-3 is a phase 1/2 study evaluating KTE-X19, an autologous anti-CD19 chimeric antigen receptor (CAR) T-cell therapy, in adult relapsed/refractory (R/R) B-ALL. We report the phase 1 results. Following fludarabine/cyclophosphamide lymphodepletion, patients received a single infusion of KTE-X19 at 2, 1, or 0.5×106 cells/kg. The rate of dose-limiting toxicities (DLTs) within 28 days following KTE-X19 infusion was the primary endpoint. KTE-X19 was manufactured for 54 enrolled patients and administered to 45 (median age 46 years [range, 18-77]). No DLTs occurred in the DLT-evaluable cohort. Grade ≥3 cytokine release syndrome (CRS) and neurologic events (NE) occurred in 31% and 38% of patients, respectively. To optimize the benefit-risk ratio, revised adverse event (AE) management for CRS and NE (earlier steroid use for NE and tocilizumab only for CRS) was evaluated at 1×106 cells/kg KTE-X19. In the 9 patients treated under revised AE management, 33% had grade 3 CRS and 11% had grade 3 NE, with no grade 4/5 NE. The overall complete remission rate correlated with CAR T-cell expansion and was 83% in patients treated with 1×106 cells/kg and 69% in all patients. Minimal residual disease was undetectable in all responding patients. At 22.1 months (range, 7.1-36.1) median follow-up, the median duration of remission was 17.6 months (95% CI, 5.8-17.6) in patients treated with 1×106 cells/kg and 14.5 months (95% CI, 5.8-18.1) in all patients. KTE-X19 treatment provided a high response rate and tolerable safety in adults with R/R B-ALL. Phase 2 is ongoing at 1×106 cells/kg with revised AE management.Primary immunodeficiencies in the costimulatory molecule CD27 and its ligand, CD70, predispose for pathologies of uncontrolled Epstein-Barr virus (EBV) infection in nearly all affected patients. We demonstrate that both depletion of CD27+ cells and antibody blocking of CD27 interaction with CD70 cause uncontrolled EBV infection in mice with reconstituted human immune system components. While overall CD8+ T-cell expansion and composition are unaltered after antibody blocking of CD27, only some EBV-specific CD8+ T-cell responses, exemplified by early lytic EBV antigen BMLF1-specific CD8+ T cells, are inhibited in their proliferation and killing of EBV-transformed B cells. This suggests that CD27 is not required for all CD8+ T-cell expansions and cytotoxicity but is required for a subset of CD8+ T-cell responses that protect us from EBV pathology.Bortezomib, lenalidomide, dexamethasone plus transplant is a standard of care for eligible Multiple Myeloma patients. As responses can deepen with time, regimens with longer and more potent induction/consolidation phases are needed. In this phase II study, patients received eight 28-day cycles of carfilzomib (K) 20/36mg/m2 (D1-2,8-9,15-16), lenalidomide (R) 25 mg (D1-21), and dexamethasone (d) 20 mg (D1-2,8-9,15-16,22-23). All patients proceeded to transplant after 4 cycles and received 1-year lenalidomide maintenance (10 mg, D1-21). The primary objective was stringent complete response (sCR) at the completion of consolidation. Overall, 48 patients were screened and 46 enrolled; 21% had adverse cytogenetics. Among 42 evaluable patients after consolidation, 26 were in sCR (61.9%), 27 in ≥CR (64.3%) 92.6% had undetectable Minimal Residual Disease by flow cytometry (≥2.5 x10-5) and 63.0% by next generation sequencing (10-6). Median time to CR was 10.6 months. By MFC and NGS, 69.0% and 66.7% patients, respectively had undetectable MRD at some point. Nedometinib nmr With a median follow-up of 60.5 months, 21 patients progressed and 10 died (7 from MM). Median PFS was 56.4 months. There was no KRd related death. Four patients discontinued the program due to toxicities; 56 serious AEs were reported in 31 patients including 8 cardiovascular events (2 heart failures, 5 pulmonary embolisms or deep vein thrombosis). Common grade 3/4 AEs were hematological (74%) and infectious (22%). In conclusion, 8 cycles of KRd produce fast and deep responses in transplant eligible NDMM patients. Safety profile is acceptable but cardiovascular AEs should be closely monitored.