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Objectives: To identify variables predicting interfractional anatomical variations measured with cone-beam CT (CBCT) throughout abdominal paediatric radiotherapy, and to assess the potential of surface-guided radiotherapy (SGRT) to monitor these changes. Methods: Metrics of variation in gastrointestinal (GI) gas volume and separation of the body contour and abdominal wall were calculated from 21 planning CTs and 77 weekly CBCTs for 21 abdominal neuroblastoma patients (median 4 years, range: 2 – 19 years). Age, sex, feeding tubes, and general anaesthesia (GA) were explored as predictive variables for anatomical variation. Furthermore, GI gas variation was correlated with changes in body and abdominal wall separation, as well as simulated SGRT metrics of translational and rotational corrections between CT/CBCT. Results: GI gas volumes varied 74 ± 54 ml across all scans, while body and abdominal wall separation varied 2.0 ± 0.7 mm and 4.1 ± 1.5 mm from planning, respectively. Patients < 3.5 years (p = 0.04) and treated under GA (p < 0.01) experienced greater GI gas variation; GA was the strongest predictor in multivariate analysis (p < 0.01). Absence of feeding tubes was linked to greater body contour variation (p = 0.03). GI gas variation correlated with body (R = 0.53) and abdominal wall (R = 0.63) changes. The strongest correlations with SGRT metrics were found for anterior-posterior translation (R = 0.65) and rotation of the left-right axis (R = −0.36). Conclusions: Young age, GA, and absence of feeding tubes were linked to stronger interfractional anatomical variation and are likely indicative of patients benefiting from adaptive/robust planning pathways. Our data suggest a role for SGRT to inform the need for CBCT at each treatment fraction in this patient group. Advances in knowledge: This is the first study to suggest the potential role of SGRT for the management of internal interfractional anatomical variation in paediatric abdominal radiotherapy.

本案例為一名56歲女性因反覆眩暈及耳鳴，經檢查後初步診斷為良性陣發性姿勢性眩暈（Benign Paroxysmal Positional Vertigo, BPPV），接受右耳耳石復位術（Canalith Reposition Procedure），然而，隨著病程進展，接續出現關鍵性的前額眉心處的頭痛及視力模糊的症狀，藉由詳細地病史詢問、身體評估、及相關的檢驗及影像檢查，採用排除及納入相關診斷的鑑別過程，最後確診為蝶鞍空洞症，在詳細地病情解釋後，病人接受經鼻腔內視鏡蝶竇蝶鞍減壓併自體脂肪填充及鼻中隔皮瓣重建手術。不幸地，術後產生腦脊髓液鼻漏的併發症，在及時地重新評估、鑑別診斷及處置，有效的控制併發症避免神經功能惡化，最後病人順利出院，因此引發筆者撰寫動機，期望分享此病例照護經驗，提醒同仁臨床照護病人時需要時刻保持警覺，確保病人回復健康。 The case in this study is a 56-year-old woman who was initially diagnosed with benign paroxysmal postural dizziness because of repeated vertigo and tinnitus after serial examinations. She underwent reduction of otolith of right ear (Canalith Reposition Procedure). However, her symptoms remained and subsequent symptoms of frontal headache and blurred vision occurred. Although symptoms such as vertigo, tinnitus, and headache are common in Meniere’s disease, benign paroxysmal postural vertigo (BPPV), cerebral infarction, etc. eventually we revised the diagnosis to the Empty Sella Syndrome through detailed inquiry of medical history, physical assessment, and image examinations.After well-addressed explanation of the clinical condition, the patient received endoscopic transnasal transsphenoidal approach and packing sella with autologous fat, reconstruction by cartilage and nasal septal flap. Unfortunately, the sequel of cerebrospinal fluid rhinorrhea occurred after the operation. By promptly reassessment, differential diagnosis with proper treatment, the complication was effectively controlled to avoid neurological deterioration, and the patient was discharged without further complication. I sincerely share the experience of this specific case with my colleagues. Keeping vigilant in clinical care of patients to ensure they could return to health. &nbsp

Radiation may cause long-term splenic dysfunction, risking potentially fatal late sepsis. We aimed to review this complication's magnitude in paediatric radiotherapy and gauge the level of awareness of the spleen as an organ at risk.Clinical trial protocols and radiotherapy guidelines, patient/parent information sheets, and professional guidance documents were reviewed to assess the perceived risk of radiotherapy-related splenic dysfunction. Paediatric oncologists and paediatric radiation oncologists across Europe were surveyed to estimate the level of understanding of this risk and to ascertain current practice. Spleen doses received in practice were examined. A systematic review of relevant publications was undertaken.The risk is not mentioned in most clinical trials, patient information leaflets, or professional guidance documents. When mentioned, a threshold dose of 40 Gy is cited. The survey showed only limited awareness. More than half of patients assessed received spleen doses in excess of 10 Gy. The systematic review identified one paper reporting a relative mortality risk of 5.5 with spleen doses in the 10-20 Gy range.The risk of mortality from overwhelming infection is poorly recognised. We therefore recommend routine delineation of the spleen. Protocols and guidelines should give a spleen dose objective as low as reasonably achievable, ideally mean10 Gy without compromise to target volumes. Revised evidence-based guidelines and continuing professional development activities should inform oncologists. Patient/parent information should mention the risk and the dose received be communicated to colleagues. Antibiotic prophylaxis and/or (re)vaccination should be considered if the mean spleen dose is ≥10 Gy.

Introduction Despite the highly prevailing rate of sensory over-responsivity, problem behaviors, and anxiety among emerging adults with autism spectrum disorder (ASD), little is known about the nature of their experiences. While previous studies have indicated potential relationships among these conditions, their connections still need to be clarified. This study aimed to examine the relationships between sensory over-responsivity, problem behaviors, and anxiety. Methods Fifty-seven emerging adults with ASD (Mage = 21.4, SD = 2.5; males = 80.7%) were recruited. The Adult Sensory Profile - Chinese version, Problem Behavior Scale of Scales of Independent Behavior - Revised, and Generalized Anxiety Disorder-7 - Chinese version (GAD-7) were used. Results The percentage of emerging adults with ASD who scored more than most people in the two quadrants were sensation sensitivity = 33.3% and sensation avoiding = 26.3%. Approximately 66.7%, 27.5%, and 50.9% of participants had internalizing behaviors, externalizing behaviors, and asocial behaviors, respectively. Approximately, 64.9% of the participants scored on or above the cut-off score of 6 on the GAD-7. Sensation sensitivity and sensation avoiding were significantly correlated to problem behaviors; and anxiety was a mediating variable that accounts for the relationships between sensory over-responsivity and problem behaviors. Conclusion These results indicated that emerging adults with ASD showing problem behaviors might also report more exaggerated responses toward sensory inputs and experience more anxiety symptoms. The results indicate that sensory over-responsivity, problem behaviors, and anxiety may have common underlying mechanisms. Findings can be useful to understand the impacts of such difficulties for emerging adults with ASD.

Proton beam therapy (PBT) is now well established for the treatment of certain pediatric brain tumors. The intrinsic properties of PBT are known to reduce long-term negative effects of photon radiotherapy (PRT). To better understand the intracranial effects of PBT, we analyzed the longitudinal imaging changes in a cohort of children with brain tumors treated by PBT with clinical and radiotherapy dose correlations. Retrospective imaging review of 46 patients from our hospital with brain tumors treated by PBT. The imaging findings were correlated with clinical and dose parameters. Imaging changes were assessed by reviewing serial magnetic resonance imaging (MRI) scans following PBT over a follow-up period ranging from 1 month to 7 years. Imaging changes were observed in 23 patients undergoing PBT and categorized as pseudoprogression (10 patients, 43%), white matter changes (6 patients, 23%), parenchymal atrophy (6 patients, 23%), and cerebral large vessel arteriopathy (5 patients, 25%). Three patients had more than one type of imaging change. Clinical symptoms attributable to PBT were observed in 13 (28%) patients. In accordance with published literature, we found evidence of varied intracranial imaging changes in pediatric brain tumor patients treated with PBT. There was a higher incidence (10%) of large vessel cerebral arteriopathy in our cohort than previously described in the literature. Twenty-eight percent of patients had clinical sequelae as a result of these changes, particularly in the large vessel arteriopathy subgroup, arguing the need for angiographic and perfusion surveillance to pre-empt any morbidities and offer potential neuro-protection.

INTRODUCTION: Whilst most patients with PLGG will survive, varying morbidities derived from patient,tumour & treatment characteristics can afflict life-long disabling functional impairments. No PLGG studies have evaluated potential prognostic factors for important functional outcomes. METHODS: We performed retrospective analysis of all children diagnosed with PLGG at GOSH 1980-2021.Review of medical notes recorded patient demographics,tumour characteristics & treatment data.Functional outcomes included endocrine,educational,visual (in OPG),auditory & physical function.Multivariate regression analysis(p

The health status and health behaviors among support staff providing daily support for people with intellectual and developmental disabilities (IDD-SS) in community-based organizations (CBOs) have not been systematically studied. This study examined the health impact of IDD-SS workers who participated in a HealthMatters Program: Train-the-Trainer Certified Instructor Workshop followed by implementing a 12-week HealthMatters Program for people with intellectual and developmental disabilities (IDD) that they care for as part of their employment. A total of 48 IDD-SS were enrolled into either an intervention ( n = 28) or control group ( n = 20). IDD-SS in the intervention group received an 8-hour HealthMatters Program: Train-the-Trainer Workshop immediately prior to teaching a 12-week HealthMatters Program for people with IDD. Assessments were conducted with IDD-SS before and after completing the 12-week HealthMatters Program to evaluate whether IDD-SS experienced any benefit of the training and teaching the program on their own health and health behaviors. Relative to the control group, the IDD-SS in the intervention group showed significant improvements in social/environmental supports for nutrition ( F = 4.92, p = .032), exercise outcome expectations ( F = 6.58, p = .014), nutrition outcome expectations ( F = 8.87, p = .005), fruit and vegetable intake ( F = 13.62, p = .001), knowledge of fruit and vegetable intake recommendations ( F = 11.25, p = .002), and stages of change for eating fruits and vegetables ( F = 6.86, p = .012). Results demonstrated that IDD-SS benefited from the health education programming. Findings support the need to develop programs and organizational policies for health promotion activities for direct care staff.

The purposes of this study were to develop a new measure, the Facilitators and Barriers to Physical Activity Scale for People with Mental Illness (FBPAS-MI) and to validate the psychometrics. This study included two phases. In Phase I, a literature review and five focus groups were conducted to develop an item bank. Then, expert consultation and cognitive interviews were used for content validity. In Phase II, 147 participants with severe mental illness were recruited from 10 community-based psychiatric rehabilitation centers in Taiwan to examine the psychometrics. Principal axis factoring method with direct oblique rotation was used to identify the underlying constructs of FBPAS-MI. Concurrent validity, internal consistency and test-retest reliability were examined. The results revealed five domains in the facilitator subscale (15 items) and two domains in the barrier subscale (11 items). Both subscales showed satisfactory internal consistency (all Cronbach’s α >0.80); the concurrent validity and test-retest reliability were established. This study confirmed that the FBPAS-MI is a valid and reliable scale to understand perceived facilitators and barriers to physical activity for severe mental illness. The FBPAS-MI can provide useful information in the development of individualized health promotion program for this population.Implications for RehabilitationThe Facilitators and Barriers to Physical Activity Scale for People with Mental Illness (i.e., FBPAS-MI) is a valid and reliable assessment to examine key factors affecting physical activity participation among people with severe mental illness.Psychiatric rehabilitation practitioners could use the FBPAS-MI to help develop and tailor individualized physical activity promotion programs based on identified facilitators and barriers to activity. The Facilitators and Barriers to Physical Activity Scale for People with Mental Illness (i.e., FBPAS-MI) is a valid and reliable assessment to examine key factors affecting physical activity participation among people with severe mental illness. Psychiatric rehabilitation practitioners could use the FBPAS-MI to help develop and tailor individualized physical activity promotion programs based on identified facilitators and barriers to activity.

Background Sleep disruption is pervasive in people with schizophrenia, but few studies have explored their sleep experiences. This study aims to identify factors relevant to sleep problems and explore coping methods used by community-dwelling people with schizophrenia. Methods Eighteen participants with schizophrenia were recruited from three mental health centers in Taiwan. They completed a semi-structured interview and the Pittsburgh Sleep Quality Index (PSQI) assessment. The Person-Environment-Occupation model offered a framework to assess factors related to sleep. Thematic analysis was used for the qualitative data analysis. Results Factors related to sleep were classified under person, environment, and occupation domains. The person domain included three subthemes: psychiatric symptoms, unpleasant emotions, and frustration about sleep. The environment domain included three subthemes: sensory intrusions from the environment, quality of bedding, and roommates. The occupation domain included sleep interruption and sleep preparation. There were notable discrepancies in sleep quality between the participants’ narratives and their PSQI global scores. Regarding coping methods for poor sleep, sleep medication was the primary strategy while some participants also used other strategies, such as modifying the environment, adjusting routines, or engaging in activities that improve sleep quality. Conclusions Psychiatric symptoms and nightmares were identified as unique sleep disruptions in people with schizophrenia, and poor economic status was also found to impact their sleep. The sleep quality of people with schizophrenia tends to be poor, as identified by the PSQI, even though they may have positive perceptions of their sleep quality. Our participants appeared to prefer to take hypnotics to address their sleep problems, which may be due to limited knowledge about alternatives. Mental health professionals are encouraged to receive training in the application of non-pharmacological approaches to support their clients’ issues related to sleep.

Additional file 1.

Aims To establish an infrastructure for sustainable, comprehensive data collection and systematic outcomes evaluation for UK patients receiving proton beam therapy (PBT). Materials and methods A Proton Outcomes Working Group was formed in 2014 to develop a national minimum dataset for PBT patients and to define a clinically integrated informatics solution for data collection. The Christie Proton Beam Therapy Centre formed its Proton Clinical Outcomes Unit in 2018 to collect, curate and analyse outcomes data prospectively for UK-treated patients and retrospectively for UK patients referred abroad for PBT since 2008 via the Proton Overseas Programme (POP). Results A single electronic form (eForm) was developed to capture the agreed data, using a data tree approach including conditional logic: data items are requested once, further questions depend on previous answers and are sensitive to tumour site and patient pathway time point. Relevant data automatically populate other forms, saving time, prompting completeness of clinical assessments and ensuring data consistency. Completed eForm data populate the electronic patient record and generate individualised outputs, including consultation letters, treatment summary and surveillance plans, based on organs at risk irradiated, age and sex. All data regarding POP-treated patients are verified and migrated into the system, ensuring that patient data, whether overseas or UK treated, are consistently recorded. The eForm utilises a ‘user friendly’ web portal interface, the Clinical Web Portal, including clickable tables and infographics. Data items are coded to a universally recognised standard comparable with other data systems. Patient-reported outcomes are also integrated, highlighting significant toxicities and prompting a response. Outcomes data can be correlated with dosimetric DICOM data to support radiation dose modelling. Conclusion Outcomes data from both POP-treated and The Christie-treated patients support long-term care, allow evaluation of PBT efficacy and safety, assist future selection of PBT patients and support hypothesis generation for future clinical trials.

Neurons use multiple modes of endocytosis, including clathrin-mediated endocytosis (CME) and activity-dependent bulk endocytosis (ADBE), during mild and intense neuronal activity, respectively, to maintain stable neurotransmission. While molecular players modulating CME are well characterized, factors regulating ADBE and mechanisms coordinating CME and ADBE activations remain poorly understood. Here we report that Minibrain/DYRK1A (Mnb), a kinase mutated in autism and up-regulated in Down’s syndrome, plays a novel role in suppressing ADBE. We demonstrate that Mnb, together with calcineurin, delicately coordinates CME and ADBE by controlling the phosphoinositol phosphatase activity of synaptojanin (Synj) during varying synaptic demands. Functional domain analyses reveal that Synj’s 5′-phosphoinositol phosphatase activity suppresses ADBE, while SAC1 activity is required for efficient ADBE. Consequently, Parkinson’s disease mutation in Synj’s SAC1 domain impairs ADBE. These data identify Mnb and Synj as novel regulators of ADBE and further indicate that CME and ADBE are differentially governed by Synj’s dual phosphatase domains.

Chapter 8 discusses lymphoid and haematological malignancy and related conditions in children and young people. Radiotherapy is less frequently used in haematological malignancy and allied disorders than it once was. In Hodgkin lymphoma, better risk stratification, more effective systemic therapy and the use of 18F-fluoro-deoxyglucose positron emission tomography (18F-FDG-PET) imaging to assess response to chemotherapy have facilitated a safe reduction in the indications for radiotherapy. Nonetheless, radiotherapy remains an important component of multimodality therapy in patients with advanced or poorly responding disease. In acute leukaemia, radiotherapy is no longer used as part of first-line treatment schedules. Instead, it is reserved for refractory and relapsed disease. Total body irradiation remains valuable as part of conditioning prior to haemopoietic stem cell transplantation. Cranial and testicular radiotherapy is helpful for selected patients who relapse in these sanctuary sites. Radiotherapy is also indicated infrequently in some unusual and benign haematological conditions.