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BackgroundCraniosynostosis is a type of skull deformity caused by premature ossification of cranial sutures in children. Given its variability and anatomical complexity, three-dimensional visualization is crucial for effective teaching and understanding. We developed a VR database with 3D models to depict these deformities and evaluated its impact on teaching efficiency, motivation, and memorability.MethodsWe included all craniosynostosis cases with preoperative CT imaging treated at our institution from 2012 to 2022. Preoperative CT scans were imported into SpectoVR using a transfer function to visualize bony structures. Measurements, sub-segmentation, and anatomical teaching were performed in a fully immersive 3D VR experience using a headset. Teaching sessions were conducted in group settings where students and medical personnel explored and discussed the 3D models together, guided by a host. Participants’ experiences were evaluated with a questionnaire assessing understanding, memorization, and motivation on a scale from 1 (poor) to 5 (outstanding).ResultsThe questionnaire showed high satisfaction scores (mean 4.49 ± 0.25). Participants (n = 17) found the VR models comprehensible and navigable (mean 4.47 ± 0.62), with intuitive operation (mean 4.35 ± 0.79). Understanding pathology (mean 4.29 ± 0.77) and surgical procedures (mean 4.63 ± 0.5) was very satisfactory. The models improved anatomical visualization (mean 4.71 ± 0.47) and teaching effectiveness (mean 4.76 ± 0.56), with participants reporting enhanced comprehension and memorization, leading to an efficient learning process.ConclusionEstablishing a 3D VR database for teaching craniosynostosis shows advantages in understanding and memorization and increases motivation for the study process, thereby allowing for more efficient learning. Future applications in patient consent and teaching in other medical areas should be explored.

Abstract Indigenous Peoples and local communities with nature-dependent livelihoods are disproportionately affected by climate change impacts, but their experience, knowledge and needs receive inadequate attention in climate research and policy. Here, we discuss three key findings of a collaborative research consortium arising from the Local Indicators of Climate Change Impacts project. First, reports of environmental change by Indigenous Peoples and local communities provide holistic, relational, placed-based, culturally-grounded and multi-causal understandings of change, largely focused on processes and elements that are relevant to local livelihoods and cultures. These reports demonstrate that the impacts of climate change intersect with and exacerbate historical effects of socioeconomic and political marginalization. Second, drawing on rich bodies of inter-generational knowledge, Indigenous Peoples and local communities have developed context-specific responses to environmental change grounded in local resources and strategies that often absorb the impacts of multiple drivers of change. Indigenous Peoples and local communities adjust in diverse ways to impacts on their livelihoods, but the adoption of responses often comes at a significant cost due to economic, political, and socio-cultural barriers operating at societal, community, household, and individual levels. Finally, divergent understandings of change challenge generalizations in research examining the human dimensions of climate change. Evidence from Indigenous and local knowledge systems is context-dependent and not always aligned with scientific evidence. Exploring divergent understandings of the concept of change derived from different knowledge systems can yield new insights which may help prioritize research and policy actions to address local needs and priorities.

Positional plagiocephaly is a deformational cranial flattening frequently treated in pediatric neurosurgical practice. Positional maneuvers and orthotic helmet therapy are preferred therapeutic options for moderate-to-severe forms. Treatment response seems to be age-dependent. Nevertheless, predictive data are vague, and cost-efficiency might be a limiting factor for treatment. The purpose of this study was to investigate the early predictive value of sonographic parameters on the efficacy of orthotic helmet therapy through the assessment of changes in skull shape and correlation of the parameters with caliper cephalometry values and with age. A consecutive cohort of 49 patients < 10 months of age, undergoing orthotic helmet therapy for positional plagiocephaly, was recruited prospectively. The authors routinely assessed the patency of the lambdoid sutures by ultrasound and the following additional skull parameters were measured: suture width, adjacent full bone thickness, adjacent cortical bone thickness and occipital angle. Caliper cephalometric values, as well as demographic and clinical data were collected. Retrospective data analysis showed an inverse relation between both cortical and full skull bone thickness and early treatment efficacy, defined by a reduction in the occipital angle. The improvement of sonographic parameters correlated with the development of cranial caliper cephalometry values. In conclusion, the sonographic assessment of skull bone thickness is a safe and cost-effective tool to predict the early efficacy of orthotic helmet therapy in positional plagiocephaly and might, therefore, help the clinician to foresee the potential evolution of the deformity.

Benign paroxysmal positional vertigo (BPPV) is the peripheral vestibular disorder that is most frequently encountered in routine neuro-otological practice. Among the three semicircular canals, the lateral semicircular canal (LSC) is the second most frequently interested in the pathological process. In most cases, LSC BPPV is attributable to a canalithiasis or cupulolithiasis mechanism. The clinical picture of LSC BPPV is that of positional nystagmus and vertigo evoked by turning the head from the supine to the side lateral position. With such a movement, a horizontal positional (and often also paroxysmal) direction-changing nystagmus is generated. Depending on whether the pathogenetic mechanism is that of canalithiasis or cupulolithiasis and depending on where the dense particles are located, LSC BPPV direction-changing positional nystagmus is geotropic or apogeotropic on both lateral sides. Due to its mechanical nature, BPPV is effectively treated by means of physical therapy. In the case of a LSC BPPV, one of the most effective therapies is the forced prolonged position (FPP), in which the patient is invited to lie for 12 h on the lateral side on which vertigo and nystagmus are less intense, to move the canaliths out from the canal (or to shift them inside of the canal from one tract to another) exploiting the force of gravity. Despite its efficacy, FPP is not always well tolerated by every patient, and it cannot be done during the diagnostic session because of its duration. The present study aimed to verify the efficacy of a different forced position, shortened forced position (SFP), with respect to the original FPP. SFP treatment would allow patients to more easily bear the forced position and physicians to control the outcome almost immediately, possibly enabling them to dismiss patients without vertigo. After 1 h of lying on the side where vertigo and nystagmus are the less intense, 38 out of 53 (71.7%) patients treated with SFP were either healed or improved. Although the outcomes are not as satisfying as those of the original FPP, SFP should be considered as a therapeutic prospect, especially by those physicians who work in collaboration with emergency departments or otherwise encounter acute patients to cure them of vertigo as soon as possible.

Introduction: Spinal astroblastoma is a rare highly malignant tumor that mainly affects children. We review the few cases described in the literature and highlight the challenges of managing this neoplasm by illustrating a case recently treated at our institutions. To our knowledge, this is the first published case of EWSR1-BEND2 fused spinal astroblastoma with long-term follow-up. Case Presentation: An 8-year-old girl was transferred from her home country to Switzerland for treatment of a recurrent intramedullary tumor of the cervical spine extending from C2-C7. The tumor was primarily diagnosed as an ependymoma of the spinal cord. Prior to her transfer to our department, the patient had undergone subtotal resection of the lesion, radiation therapy, multiple chemotherapy regimens, and biopsy of the recurrent tumor. Clinically, the patient presented with tetraparesis and had recently experienced worsening upper extremity weakness with complete loss of hand function. We performed a near total resection of the recurrent tumor. Ultra-fast Nanopore seq® based DNA methylome profiling allowed confirmation of the molecular diagnosis of a high-grade neuroepithelial tumor (HGNET-MN1) consistent with astroblastoma in less than 2 h, with subsequent molecular workup revealing a EWSR1-BEND2 fusion. After surgery, the patient gradually regained function in her hands. She was sent to a specialized pediatric rehabilitation center, and while the tumor was being followed radiologically with no adjuvant treatment planned, the patient presented with a relapse of the tumor in only 3 months. Given the acute worsening of radiating pain and sudden respiratory failure, a cervical decompression was performed. MRI of the cervical spine showed infiltration of the lower aspects of the brainstem. The patient was offered palliative comfort care. Conclusion: Spinal astroblastoma is a rare and highly aggressive tumor affecting children and young adults with a high recurrence rate and thus far not well-defined prognosis. The molecular signature of astroblastoma needs to be further characterized to establish a treatment-relevant classification and to allow a better prognostication. Currently, gross-total resection combined with radiotherapy remains the mainstay of treatment for spinal astroblastoma. [ABSTRACT FROM AUTHOR]

Introduction: The recent approval of Dupilumab has profoundly revolutionized the management of patients affected by severe and recalcitrant Chronic Rhinosinusitis with Nasal Polyps (CRSwNP). However, a review that summarizes the results of real-life studies and compares them to phase 3 studies SINUS-24 and 52 is still lacking. Materials and methods: A search of all real-life studies published from 2019 to 2023 was performed. Patients characteristics at baseline and 6 and 12 months after starting Dupilumab were extracted and compared to those from phase 3 trials: age, sex, smoking habits, comorbid asthma and aspirin-exacerbated respiratory disease (AERD), previous endoscopic sinus surgery (ESS), hematic eosinophils and total IgE, NasalAQ2 Polyps Score (NPS), smell, SNOT-22, adverse events (AEs), and response to treatment. Results: 15 papers were included with an overall number of 1658 patients. A higher rate of comorbidities and previous ESS was found in patients from real-life studies. In addition, they had worse smell and SNOT-22 at baseline compared to patients from SINUS-24 and 52. Comorbid and post-ESS patients tended to have a faster NPS and SNOT-22 improvement, although the absolute values were not clinically relevant. A more extensive surgery and a number of ESS ≥ 2 were related to worse olfactory outcomes, probably due to iatrogenic damage. No correlation was found between hematic eosinophils and outcomes. AEs were reported by 12.4% of patients and 2.2% had to discontinue dupilumab. Weight gain was an emergent AE (0.8%), probably related to the restored sense of smell and taste. Non-responders were 3.5% and they were switched to systemic steroid, ESS, or another biologic. Conclusion: Despite some differences in prescription criteria between countries, dupilumab was demonstrated to be effective even in the real-life scenario. However, emerging AEs and possible unknown long-term AEs of a likely lifelong therapy should be considered. [ABSTRACT FROM AUTHOR]

Introduction: Sinonasal intestinal-type adenocarcinomas (ITACs) are rare and aggressive tumors, closely related to professional exposure to wood dusts or leather. Here we explored the role of non-coding RNAs controlling MUC2 in liquid biopsies and tumors from ITAC patients with the aim of identifying biomarkers and molecular mechanisms to improve early diagnosis, prognosis, and therapeutic approaches for this rare cancer. Methods: MiR-34c-3p, lncRNA AF147447 and MUC2 were measured in tumors and normal mucosa, in nasal washings (NW) from the affected and non-affected nostril and in plasma from 17 ITAC patients. The Apparent Diffusion Coefficient (ADC) was also evaluated by Magnetic Resonance Imaging. Results: MiR-34c was higher in ITACs compared to the corresponding normal mucosa (p = 0.021). Differentiated tumors exhibited higher miR-34c levels (p = 0.025) and lower ADC values (p

Positional plagiocephaly is a deformational cranial flattening frequently treated in pediatric neurosurgical practice. Positional maneuvers and orthotic helmet therapy are preferred therapeutic options for moderate-to-severe forms. Treatment response seems to be age-dependent. Nevertheless, predictive data are vague, and cost-efficiency might be a limiting factor for treatment. The purpose of this study was to investigate the early predictive value of sonographic parameters on the efficacy of orthotic helmet therapy through the assessment of changes in skull shape and correlation of the parameters with caliper cephalometry values and with age. A consecutive cohort of 49 patients < 10 months of age, undergoing orthotic helmet therapy for positional plagiocephaly, was recruited prospectively. The authors routinely assessed the patency of the lambdoid sutures by ultrasound and the following additional skull parameters were measured: suture width, adjacent full bone thickness, adjacent cortical bone thickness and occipital angle. Caliper cephalometric values, as well as demographic and clinical data were collected. Retrospective data analysis showed an inverse relation between both cortical and full skull bone thickness and early treatment efficacy, defined by a reduction in the occipital angle. The improvement of sonographic parameters correlated with the development of cranial caliper cephalometry values. In conclusion, the sonographic assessment of skull bone thickness is a safe and cost-effective tool to predict the early efficacy of orthotic helmet therapy in positional plagiocephaly and might, therefore, help the clinician to foresee the potential evolution of the deformity. [ABSTRACT FROM AUTHOR]

The association of small lymphocytic lymphoma/chronic lymphocytic leukaemia (CLL) with different malignancies has been reported in the literature. Also the occurrence of a second haematological disease has been described, more frequently as a secondary event in patients receiving chemotherapeutic agents. We report a case of CLL with concurrent acute myeloid leukaemia in an untreated patient, with emphasis on the need of a detailed immunomorphological study to identify the coexistence of the two diseases in the same pathological tissue.

Summary: Stress adaptation is essential for neuronal health. While the fundamental role of mitochondria in neuronal development has been demonstrated, it is still not clear how adult neurons respond to alterations in mitochondrial function and how neurons sense, signal, and respond to dysfunction of mitochondria and their interacting organelles. Here, we show that neuron-specific, inducible in vivo ablation of the mitochondrial fission protein Drp1 causes ER stress, resulting in activation of the integrated stress response to culminate in neuronal expression of the cytokine Fgf21. Neuron-derived Fgf21 induction occurs also in murine models of tauopathy and prion disease, highlighting the potential of this cytokine as an early biomarker for latent neurodegenerative conditions. : Restelli et al. show that deletion of mitochondrial fission protein Drp1 in adult mouse neurons activates multiple stress-sensing pathways. These converge on the integrated stress response, resulting in neuron-specific expression of metabolic cytokine Fgf21. Cerebral induction of Fgf21 also occurs in mechanistically independent mouse models of protein misfolding-associated neurodegeneration. Keywords: Alzheimer’s disease, autophagy, biomarker, endoplasmic reticulum, heme, metabolism, mitochondria, neurodegeneration, tau, unfolded protein response

Vegetation in freshwater and coastal ecosystems modifies flows, retains sediment, protects banks and shorelines from erosion. Hydraulic laboratory studies with live vegetation or artificial plant mimics, or numerical models with abstracted patches, are often used to quantify the effects of vegetation on water flow and sedimentation. However, the choice of plant and patch characteristics is often not supported by field observations of patch dimensions, density or spacing between consecutive patches. The discrepancy between plants in natural conditions and in flume experiments or numerical studies may affect the relevance of these findings for natural ecosystems. In this study, we provide guidelines for building realistic vegetation patches in hydraulic studies. We collected data on four species of fully submerged freshwater aquatic macrophytes that can grow into well-defined patches. We considered three relevant levels: individual plants (inside patches), isolated patches and multiple neighbouring patches. At the plant level, we observed significant differences in biomechanical traits (Young's modulus, flexural stiffness), resulting in stem Cauchy numbers ranging from 85.25 to 325.84, and leaf Cauchy numbers from 163.81 to 2003.97. At the patch level, we found significant relationships between patch length, width and height, showing covariation among different patch characteristics. The relationships among patch dimensions differed significantly among sampling sites for three of the four species, suggesting high intraspecific variability in patch sizes. By providing a first set of guidelines for choosing correct and ecologically relevant plant characteristics, this dataset aims to improve our understanding of the complex processes occurring inside and around submerged vegetated patches. [ABSTRACT FROM AUTHOR]

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Introduction: The aim of this cohort study was to assess the outcome of single-level selective dorsal rhizotomy (SDR) in children and young adults with spastic cerebral palsy (CP) treated at our institution, focusing on patient-reported outcome measures (PROMs) and quality of life (QoL) of patients and their caregivers. Methods: We included consecutive patients undergoing SDR from 2018 to 2020 at our institution. Subjective outcome was measured through PROMs, while functional outcome was measured through baseline characteristics, operative outcome, as well as short- and long-term follow-up. Furthermore, the effect of age at the time of surgery on patient/caregiver satisfaction was analyzed. Results: Seven patients (3 female, 43%) with a median age at surgery of 11.9 years (IQR 8.7–15.5) were included. All patients had a Gross Motor Function Classification (GMFCS) score of at least IV before surgery. Five surgeries were palliative and two non-palliative. Based on PROMs, SDR showed very good QoL and health-related outcome measures for both palliative and non-palliative patients. Patient/caregiver satisfaction was higher for the early subgroup (age ≤11) than the late subgroup (age >11). Functional outcome showed reduced spasticity in both groups. Blood transfusions were never needed, while no cerebrospinal fluid leak, infection, or permanent morbidity was seen. Conclusion: Based on PROMs, SDR leads to high satisfaction and improved QoL, especially if done at an early age. Further studies with larger cohorts are necessary to underline and confirm our observations. [ABSTRACT FROM AUTHOR]

Simple Summary: Compared with other head and neck cancer types, the prognosis of recurrent early glottic cancer (rEGC) may be less dependent on the presence of regional (N) and/or distant metastases (M). The latter two are clinically infrequent due to rEGC's peculiar biology, but the currently available staging systems still rely upon these parameters. Thus, we developed a new staging system (SUD) centered on the comparison between the T stages of the recurrence and the primary tumor. Then, in our cohort of 258 patients with rEGC treated at our Institution in Florence, Italy, we verified how the SUD system performs in the prediction of the overall and disease-specific survival, compared to the other classifications already in use. (1) Background: The treatment of recurrent early glottic cancer (rEGC) remains challenging. We wanted to investigate how the oncological outcomes are affected by the initial and recurrent stages, in order to propose our newly developed Same–Up–Down (SUD) staging system. (2) Methods: In our cohort of 258 rEGC patients, we retrospectively assessed the prognostic performances of the rTNM (the TNM staging system for recurrence), CLRSS, CLRSS-2, and SUD staging systems by univariate and multivariate Cox analysis, comparing their predictive capability using Harrell's C-index. (3) Results: The SUD classification satisfactorily predicted both overall survival (p = 0.022) and second-recurrence-free survival (p = 0.024, as same + down vs. upstage) in our cohort. It also outperformed the other three systems in terms of prediction of survival, with an improvement of 1.52%, 1.18%, and 3.96% in the predictive capacity of overall survival, disease-specific survival, and second-recurrence-free survival, respectively. (4) Conclusions: The SUD staging system can efficiently predict survival in rEGC patients, whose prognosis heavily depends on both the initial and recurrent locoregional extension. [ABSTRACT FROM AUTHOR]

Background: Fatty filum terminale is a form of spinal dysraphism and a third of all patients develop symptoms such as sensory, motor, and urinary impairment. Early surgery at 6 months has the advantage that the bone density is still soft, and the patients are not ambulatory yet, promoting faster healing. Method: We present our minimal invasive surgical technique for FFT untethering. Conclusion: Due to the low complication rate and the potentially high benefit of surgery, prophylactic untethering is recommended. [ABSTRACT FROM AUTHOR]