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Background: Type 1 Neurofibromatosis (NF1) is an autosomal dominant genetic disease that affects one in each 2500 individuals and results in important cognitive impairment, such as learning disorders and auditory processing disorders. In a previous study, we found that 70% of NF1 patients presented some deficit in music perception (amusia), mainly rhythmic and associated to pre-attentional electrophysiological deficits detected with Mismatch Negativity (MMN). Objectives: In the present study, we investigated how musical training may change music perception and MMN. Material and methods: Six teenagers diagnosed with NF1 aged between 12 and 16 years, 4 females and 2 males, with normal audiometric thresholds, were studied. A six-month program of musical training was proposed, directed to rhythmic learning. Assessment battery before and after training included Montreal Battery of Evaluation of Amusia (MBEA) -- short version to evaluate music processing; Gaps in Noise test to evaluate temporal auditory processing; and MMN to investigate pre attentional auditory perception. Results: All participants showed improvement in temporal auditory processing, with a reduction in mean values of gap thresholds from 10.25 to 5.41 (95% CI=1.77--7.89; p=0.006). From the six teenagers studied, only three presented detectable MMN in the pre-training evaluation. After training, all participants had detectable MMN (95% CI=0.02--0.10; p=0.049). An improvement was also observed in the mean global and specific music perception to melodic, rhythmic and musical memory, though statistically significant to the latest component (95% CI=1.62--0.04; p=0.042). Conclusions: Results obtained corroborate our hypothesis that rhythmic music training may result in improvement of temporal auditory processing, and this may be present also in the pre attentional level of auditory perception. This is a pilot study still in course. [ABSTRACT FROM AUTHOR]

This study investigated the ability of cholesterol-phosphatidylcholine liposomes loaded with chloride aluminum phthalocyanine (CL-AlClPc) to discriminate between healthy (MCF-10A) and neoplastic (MCF-7 and MDA-MB-231) breast cells for breast cancer diagnosis and treatment by photodynamic therapy (PDT) using a new drug delivery system consisting of CL-AlClPc. When PDT treatment was applied at an energy fluence of 700 mJ/cm², CL-AlClPc was more cytotoxic to neoplastic cells than to healthy breast cells because CL-AlClPc was better internalized by the tumor cells. An even higher fluorescence signal is expected for neoplastic cells during clinical treatment than for healthy cells, which will be useful for precise and targeted tumor cell detection. CL-AlClPc also facilitated better drug distribution and targeting of essential organelles inside the cells. This selectivity is critical for future in vivo diagnosis and treatment; it prevents side effects because it prioritizes tumor cells and tissues instead of healthy ones. The CL-AlClPc system designed herein had a small size (150 nm), low zeta potential (-6 mV), low polydispersity (0.16), high encapsulation rate efficiency (82.83%), and high shelf stability (12 months)., Competing Interests: Declaration of Competing Interest None., (Copyright © 2022 Elsevier B.V. All rights reserved.)

Objective: While pedicled intranasal flaps, such as the nasoseptal flap (NSF), successfully reduce postoperative CSF leaks following endoscopic endonasal approaches (EEA) to the skull base, morbidity remains prevalent. This study describes the first cadaveric description and radiographic analysis of the posterior septal nasal floor mucosal flap (PSNF) designed to reduce pedicled nasal flap morbidity. A case series is also detailed., Study Design: Cadaveric dissection, radiographic analysis, and case series., Methods: Seven cadaver specimens underwent harvest of the PSNF. PSNF flap dimensions were measured on the thirty deidentified sinus computed tomography (CT) scans. A retrospective case series was performed on the first set of patients who received the PSNF for reconstruction of a sellar base defect. Information concerning complications, symptoms, and the appearance of the flap was recorded from records of the patient's postoperative inpatient stay, 1-week postop visit, and 1-month postop visit., Results: Cadaver dissection of the PSNF flap demonstrated good arc rotation along its pedicle with ability to cover both sellar and planum defects. Cadaveric flap measurements and CT-derived measurements estimated a mean surface area of 16.7 and 18.3 cm 2 , respectively. 15 patients underwent reconstruction of a sellar or planum defect using the PSNF technique. Only one CSF leak was encountered postoperatively. Minimal crusting of the flap and donor site was seen 1 month postoperatively., Conclusion: The PSNF flap provides a sizeable pedicled region for reconstruction comparable to other pedicled nasal flaps. Our case series demonstrated good postoperative outcomes without reduced donor site morbidity., Level of Evidence: 4 Laryngoscope, 132:1561-1568, 2022., (© 2022 The American Laryngological, Rhinological and Otological Society, Inc.)

Objective: The purpose of this study was to describe a detailed investigation of craniofacial and dental characteristics in a group of Brazilian Rubinstein-Taybi syndrome (RSTS) patients., Methods and Results: Thirteen RSTS patients treated in a special care dental clinic after 10 years were studied. Panoramic radiographs were obtained from all patients, and cephalometric analysis was performed in eight patients. Five male and eight white female patients with a median age of 11.7 years were analyzed. All the RSTS patients were mouth breathers and presented malocclusion, transverse hypoplastic maxilla, nine subjects (9/13; 69.2%) had posterior crossbite, and eight (61.53%) exhibited talon cusps. Most patients presented class II skeletal pattern and were brachycephalic. Regarding systemic disorders, one patient (7.69%) reported seizure episodes during childhood, and four patients (30.76%) presented heart valve disorders. All patients presented reduced attention span, low intolerance to dental interventions, impulsiveness, and irritability., Conclusions: Since RSTS exhibits oral and skeletal changes, early dental treatment is essential for these patients. Dentists must be aware of medical problems related to heart disease and persist in conditioning techniques to obtain cooperation and avoid dental care under general anesthesia., (© 2021 Special Care Dentistry Association and Wiley Periodicals LLC.)