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A few cases of ocular adnexal neoformations related with MCL have been reported in the literature. We present a rare case of tumour duplicity: mantle cell lymphomas (MCL) associated with squamous cell carcinoma (SCC) localised at the level of the ocular adnexa, on left upper eye lid mass since two years of 18 mounth duration in a 57- year-old man who had previously been diagnosed with stage IV MCL for 14 months. The patient had been treated according to the R-DHAP scheme for 4 cycles, in anticipation of a possible autologous HSC transplant, which was not carried out due to a positive diagnosis at the end-of-cycle osteomedullary biopsy (BOM) check. Ophthalmological examination was performed, and afther surgical removal histological examination proved to be squamous cell carcinoma (SCC). The aim of this case report is to decode the signs, symptoms and factors associated with the formation, that appear to be a chalazion, at an early stage in order to prevent the overgrowth of the mass that could invade the surrounding tissues by infiltrating them, as well as negative aesthetic outcomes of the surgery due to the excessive size of the mass, which could compromise the patient’s quality of life. [ABSTRACT FROM AUTHOR]

X-ray emission from the gravitational wave transient GW170817 is well described as non-thermal afterglow radiation produced by a structured relativistic jet viewed off-axis. We show that the X-ray counterpart continues to be detected at 3.3 years after the merger. Such long-lasting signal is not a prediction of the earlier jet models characterized by a narrow jet core and a viewing angle ≈20 deg, and is spurring a renewed interest in the origin of the X-ray emission. We present a comprehensive analysis of the X-ray dataset aimed at clarifying existing discrepancies in the literature, and in particular the presence of an X-ray rebrightening at late times. Our analysis does not find evidence for an increase in the X-ray flux, but confirms a growing tension between the observations and the jet model. Further observations at radio and X-ray wavelengths would be critical to break the degeneracy between models. [ABSTRACT FROM AUTHOR]

Starch metabolism in guard cells plays a central role in regulating stomatal movement in response to light, elevated ambient CO2 and potentially other abiotic and biotic factors. Here, we discuss how various guard cell signal transduction pathways converge to promote rearrangements in guard cell starch metabolism for efficient stomatal responses, an essential physiological process that sustains plant productivity and stress tolerance. We suggest manipulation of guard cell starch dynamics as a previously overlooked strategy to improve stomatal behaviour under changing environmental conditions., (© The Author(s) 2024. Published by Oxford University Press on behalf of American Society of Plant Biologists.)

Fast radio bursts (FRBs) are millisecond-duration, bright (approximately Jy) extragalactic bursts, whose production mechanism is still unclear 1 . Recently, two repeating FRBs were found to have a physically associated persistent radio source of non-thermal origin 2,3 . These two FRBs have unusually large Faraday rotation measure values 2,3 , probably tracing a dense magneto-ionic medium, consistent with synchrotron radiation originating from a nebula surrounding the FRB source 4-8 . Recent theoretical arguments predict that, if the observed Faraday rotation measure mostly arises from the persistent radio source region, there should be a simple relation between the persistent radio source luminosity and the rotation measure itself 7,9 . Here we report the detection of a third, less luminous persistent radio source associated with the repeating FRB source FRB 20201124A at a distance of 413 Mpc, substantially expanding the predicted relation into the low luminosity-low Faraday rotation measure regime (<1,000 rad m -2 ). At lower values of the Faraday rotation measure, the expected radio luminosity falls below the limit-of-detection threshold for present-day radio telescopes. These findings support the idea that the persistent radio sources observed so far are generated by a nebula in the FRB environment and that FRBs with low Faraday rotation measure may not show a persistent radio source because of a weaker magneto-ionic medium. This is generally consistent with models invoking a young magnetar as the central engine of the FRB, in which the surrounding ionized nebula-or the interacting shock in a binary system-powers the persistent radio source., (© 2024. The Author(s), under exclusive licence to Springer Nature Limited.)

Nerve sheath myxoma (NSM) is a rare benign peripheral nerve sheath tumor that affects young adults. NSMs are asymptomatic, slow-growing swellings located in the upper extremities, more rarely in the lower extremities. Given the high risk of recurrence, it is recommended to perform a complete exeresis. To our knowledge, the evolution and management of NMS during pregnancy have not been described yet. We report the first case of recurrent pretibial NSM in a pregnant girl and its follow-up and outcome during and after pregnancy. NSM is difficult to diagnose clinically or using imaging. The final diagnosis remains histopathological. It is known how various types of benign and malignant skin tumors can develop or change during pregnancy. With our case, however, we documented that pregnancy does not affect the growth and evolution of NSM. Given the benign nature of the lesions and their tendency to grow slowly, during pregnancy, follow-up of NSMs can be conducted through ultrasonography and surgical treatment postponed after delivery. Our case highlights the importance of careful monitoring and individualized decision making, especially in rare scenarios such as NSM, where data on the progression of benign lesions are limited. Our case highlights the importance of a careful monitoring and a tailored treatment in rare scenarios such as NSM, where data on the progression of benign lesions are limited. Considering the benign nature of the lesions and their tendency to grow slowly, follow-up of NSMs during pregnancy can be conducted through ultrasonography, and surgical treatment can be postponed after delivery.

The Web has become an essential resource but is not yet accessible to everyone. Assistive technologies and innovative, intelligent frameworks, for example, those using conversational AI, help overcome some exclusions. However, some users still experience barriers. This paper shows how a human-centered approach can shed light on technology limitations and gaps. It reports on a three-step process (focus group, co-design, and preliminary validation) that we adopted to investigate how people with speech impairments, e.g., dysarthria, browse the Web and how barriers can be reduced. The methodology helped us identify challenges and create new solutions, i.e., patterns for Web browsing, by combining voice-based conversational AI, customized for impaired speech, with techniques for the visual augmentation of web pages. While current trends in AI research focus on more and more powerful large models, participants remarked how current conversational systems do not meet their needs, and how it is important to consider each one's specificity for a technology to be called inclusive., (© 2024. The Author(s).)

Background: Sternal cleft (SC), a rare thoracic malformation, is associated with pectus excavatum (PE) in 2.6-5% of cases. It remains unclear if these conditions are congenitally linked or if SC repair triggers PE. To investigate the potential higher frequency of PE in SC cases, we conducted a retrospective study of our SC patients., Methods: We assessed PE incidence, progression, and management in SC patients treated at our institute from 2006 to 2022. When available, we collected pre-SC repair CT scan data, calculating the Haller Index (HI) and Correction Index (CI) and compared them to a selected control group., Results: Among 8 SC patients, 7 had concomitant PE (87.5%), varying in severity. PE management ranged from observation to thoracoplasty, depending on its degree. We observed a significant pre-operative CI difference between SC and control group patients (p < 0.00001). In the last two SC repair cases, we attempted concurrent PE prevention or treatment., Conclusion: Our findings suggest an underestimated association between PE and SC in the existing literature. SC patients may exhibit a predisposition to PE from birth, which may become more apparent with growth after SC repair. Consequently, PE prevention or treatment should be considered during SC repair procedures., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Introduction: Encephalocraniocutaneous lipomatosis (ECCL) is a rare congenital syndrome with complex skin, eye, and central nervous system (CNS) symptoms. Diagnosis and treatment are challenging due to its rarity and diverse manifestations. It often involves issues like porencephalic cysts, cortical atrophy, and low-grade gliomas in the CNS, resulting in developmental delays. The spinal cord is frequently affected, leading to problems like medullary compression and radiculopathy, causing back pain and sensory/motor deficits. Surgical interventions are reserved for symptomatic cases to address hydrocephalus or alleviate spinal lipomas. This article reviews a case series to assess surgical risks and neurological outcomes., Case Series: We present a case series ECCL, focusing on the diffuse lipomatosis of the spinal cord and the intricate surgical procedures involved. A multi-stage surgical approach was adopted, with continuous neuromonitoring employed to safeguard motor pathways. We discuss clinical characteristics, imaging studies, and indications for neurosurgical interventions., Discussion: ECCL is a complex syndrome. Diagnosis is challenging and includes clinical evaluation, neuroimaging, and genetic testing. Treatment targets specific symptoms, often requiring surgery for issues like lipomas or cerebral cysts. Surgery involves laminectomies, spinal fusion, and motor pathway monitoring. Thorough follow-up is crucial due to potential CNS complications like low-grade gliomas. Hydrocephalus occurs in some cases, with endoscopic third ventriculostomy (ETV) preferred over ventriculoperitoneal shunt placement., Conclusion: Neurosurgery for ECCL is for symptomatic cases. ETV is preferred for hydrocephalus, while the treatment for lipoma is based on the presence of symptoms; the follow-up should assess growth and prevent deformities., (© 2024. The Author(s).)

Background: Diffuse neonatal hemangiomatosis (DNH) is a rare disorder typically recognized at birth or during the neonatal period. DNH involves three or more organ systems, including the central nervous system (CNS). In these cases, serious complications such as hemorrhages and obstructive hydrocephalus can develop., Case Report: We present a case of DNH with intracranial hypertension and CNS hemorrhagic lesions on the mesencephalic aqueduct, resulting in triventricular hydrocephalus, treated with endoscopic ventriculocisternostomy (ETV) and medical therapy., Discussion: DNH is a rare condition that can involve the CNS with serious complications. From a review of the literature, we highlighted only two cases of DNH with brain involvement treated surgically. We report the successful outcome of ETV, along with surgical considerations, imaging findings, and the complete resolution of cerebral and skin lesions following medical therapy., Conclusions: Medical therapy is not standardized and must be individualized. In patients who develop severe neurological symptoms such as obstructive hydrocephalus, surgery may be considered to avoid neurological sequelae., (© 2023. The Author(s).)

Starch in the stomatal guard cells is largely synthesized using carbon precursors originating from sugars imported from the leaf mesophyll. Such heterotrophic nature of guard cell starch synthesis prompted us to investigate the role of cytosolic sucrose synthases (SUS) in this pathway. Out of the six members of the Arabidopsis SUS gene family, SUS3 was the most highly expressed isoform in guard cells. The Arabidopsis sus3 mutant displayed changes in guard cell starch contents comparable to the Wild Type (WT) up until 6 h into the day. After this time point, sus3 guard cells surprisingly started to accumulate starch at very high rates, reaching the end of the day with significantly more starch than WT. Based on the phenotype of the sus3 mutant, we suggest that in guard cells, SUS3 is involved in the regulation of carbon fluxes towards starch synthesis during the second half of the day. SUS3 may be part of a previously predicted guard cell futile cycle of metabolic reactions, in which sucrose is re-synthesized from UDP-glucose to avoid excessive starch synthesis toward the end of the day. This is in contrast to typical storage organs, in which cytosolic SUS is required to produce ADP-glucose for starch synthesis.

Introduction: The prevalence of mental health disorders is rising among US service members; however, research is limited on their use of mental health care. The objective of our study was to determine whether racial and ethnic disparities exist in the use of mental health care and perceived mental health stigma among active-duty service members., Methods: We obtained data from a sample of 17,166 active-duty service members who participated in the 2018 Department of Defense Health Related Behavior Survey (HRBS). Racial and ethnic groups included Black, Hispanic, White, and other. Yes-no questions about use of mental health care and perceived mental health stigma were our outcome variables. We used multiple logistic regression to assess racial and ethnic differences in mental health care use and perceived mental health stigma by service members. Significance was set at P <.05., Results: In 2018, approximately 25.5% of service members self-reported using mental health services, and 34.2% self-reported perceived mental health stigma. Hispanic service members (AOR = 0.78) and service members in the "other" racial and ethnic group (AOR = 0.81) were less likely than their White counterparts to have used mental health care. Black (AOR = 0.68) and Hispanic (AOR = 0.86) service members were less likely than their White counterparts to self-report perceived mental health stigma., Conclusion: The 2018 HRBS showed racial and ethnic differences in mental health care use and perceived stigma among US active-duty service members. Perceived stigma was a barrier to use of mental health care among service members with a mental health condition. Culture-sensitive programs customized for different racial and ethnic groups are needed to promote mental health care and reduce perceptions of stigma associated with its use.

Introduction:  Jeune's syndrome, or asphyxiating thoracic dystrophy (ATD), is a rare autosomal recessive disorder characterized by skeletal dysplasia. Ribs are typically short and horizontal resulting-in lethal variant-in severe lung hypoplasia, progressive respiratory failure, and death. Lateral thoracic expansion (LTE) consists in staggered bilateral ribs osteotomy leading to chest expansion and lung development. Studies on LTE in ATD patients report encouraging data, but the rarity of ATD implies the lack of a standardized surgical path. The aim of this report is to present our experience with LTE, the technical modification we adopted, and patients' clinical outcome., Materials and Methods:  We retrospectively reviewed data of 11 LTE performed in 7 ATD patients with lethal variant. Information regarding pre- and postoperative clinical conditions and surgical details was collected. We adopted a single-stage or a two-stage approach based on patient clinical condition. Computed tomography (CT) scan was performed before and after surgery and lung volume was calculated., Results:  Five patients are alive, while two died in intensive care unit for other than respiratory cause (sepsis). Most patients experienced clinical improvement in terms of decreased respiratory infections rate, need for ventilation, and improved exercise tolerance. Postoperative CT scan demonstrated a median lung volume increase of 88%., Conclusion:  Mortality in ADT patients is high. However, LTE is a feasible and safe surgical approach, which could improve clinical conditions and survival rate. Survived patients showed postoperatively less oxygen requirement and improved clinical conditions., Competing Interests: None declared., (Thieme. All rights reserved.)

Context: Incidence and awareness of endocrine-related adverse events (ERAE) associated with use of immune checkpoint inhibitors (ICI) has grown with increased ICI use, yet mechanisms for ERAE prediction, surveillance, and development are not well established., Objective: We prospectively evaluated the impact of endocrine autoimmunity on ERAE development and overall survival (OS)., Methods: Adults ≥ 18 years of age prescribed ICI treatment for advanced or metastatic solid tumors and no known active/past endocrine disorders were eligible for enrollment. Thyroid, adrenal, and pancreatic antibodies as well as hormone levels were assessed prior to ICI treatment and at 8 to 9 weeks and 36 weeks after treatment for ERAE in relation to presence and changes in endocrine-specific antibodies, hormone levels, and OS., Results: Sixty patients were enrolled and ERAE were detected in 14 (23.3%), with a median onset of 52 days (IQR, 38.5-71.5) after first ICI dose. Hypothyroidism occurred in 12 (20%) patients, and 2 (3.33%) patients developed hypophysitis. Diabetes and primary adrenal insufficiency were not observed. Antibodies were detected in 14 patients (11 at baseline, 3 developed during follow-up) and their presence was significantly associated with ERAE (R2 59.3%, P < 0.001). Thyroid peroxidase antibody (20%) and thyroid-stimulating immunoglobulin (3.3%) were most common, and anti-GAD was present in 1 patient. The presence of ERAE was associated with a more favorable OS (P = 0.001)., Conclusion: Endocrine-specific autoantibodies play an important role in ERAE pathogenesis and may serve as predictive markers for early identification and treatment of ICI-induced endocrinopathies., (© The Author(s) 2022. Published by Oxford University Press on behalf of the Endocrine Society. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)