Specialized Centers Research Articles

القيادة الريادية مدخل لتحقيق التميز المؤسسي في الجامعات السعودية: دراسة تحليلية

The research aims to study the role of entrepreneurial leadership in achieving institutional excellence in Saudi universities, considering the rapid challenges and changes in the higher education sector. It also seeks to analyze the suitability of entrepreneurial leadership for Saudi universities and its impact on institutional performance, focusing on its key elements such as creativity, initiative, and calculated risk-taking. The study adopted a descriptive-analytical approach, analyzing previous literature on entrepreneurial leadership, examining global university experiences, and exploring entrepreneurial leadership practices in some Saudi universities. Additionally, it analyzed institutional excellence standards, including effective leadership, strategic planning, and strengthening community partnerships. The findings indicate that the implementation of entrepreneurial leadership in Saudi universities is still in its early stages. However, efforts are being made to enhance it through various initiatives and development programs. The study also identified key challenges hindering the adoption of entrepreneurial leadership, including insufficient funding, institutional resistance to change, a lack of trained leadership competencies, and unclear policies supporting entrepreneurship. The recommendations emphasize establishing specialized units within universities to support entrepreneurial leadership, developing training programs for academic leaders to enable them to adopt entrepreneurial strategies, enhancing creativity, initiative, and calculated risk-taking by establishing specialized centers within universities to support entrepreneurial ideas and finance academic and research projects focused on innovation and development, and integrating technology and artificial intelligence into entrepreneurial leadership practices to enhance institutional performance efficiency and achieve global leadership.

Congenital Lung Malformations: Outcomes after Minimally Invasive and Open Surgery in Infancy.

The management of congenital lung malformations (CLM) remains controversial. The aim of this study was to analyze the timing, approach, and outcomes of surgical treatment in children with CLM.A cohort study was conducted comparing children with CLM who underwent thoracoscopic resection with open resection via thoracotomy. All children were treated according to an institutionalized multidisciplinary coordinated treatment algorithm.Between 2002 and 2019, 68 children with CLM were treated. Thoracoscopic resection of CLM (n=44) resulted in a statistically significant longer operative time (mean 169 vs. 97 minutes, p=0.04) but shorter hospital stay (mean 7.2 vs. 16.7/12 days, p=0.01) compared to thoracotomy (n=15) or after conversion to thoracotomy (n=9). There were no major complications in either group. At a mean follow-up of 32.2 months (range 0.5-163), patients after thoracoscopic resection had statistically significantly fewer rip fusions than patients after open resection (n=0 vs. 2/2, p≤0.004) and less chest wall asymmetry than after conversion (n=0 vs. 2, p=0.004).Thoracotomy proved to be a fast and safe surgical approach in respiratory unstable CLM patients. In respiratory stable CLM patients, thoracoscopic resection was feasible in early infancy with good surgical and musculoskeletal outcomes.Thoracoscopic resection of CLM offers advantages over resection by thoracotomy in terms of surgical recovery and musculoskeletal function. Therefore, it should be offered to respiratory-stable patients in specialized centers.

The Heart Team Approach to Cardiac Arrest

Abstract Cardiac arrest is a critical emergency in cardiovascular medicine, requiring rapid, multidisciplinary interventions to enhance patient survival and neurological outcomes. This review explores the unique challenges of managing out-of-hospital and in-hospital cardiac arrest, with a focus on mechanical circulatory support and extracorporeal cardiopulmonary resuscitation for selected patients. While out-of-hospital cardiac arrest management should prioritize rapid transport to specialized centers, in-hospital cardiac arrest may allow for immediate, patient-tailored interventions. Post-cardiac arrest syndrome adds complexity, often requiring nuanced mechanical circulatory support escalation and weaning. Standardized protocols, ethical considerations, and further research are essential to refine patient selection and improve outcomes, ultimately advancing cardiac arrest care.

Streamlining Diagnosis: Reducing Time for Brain Tumor Detection in Children of an Upper-Middle-Income Country.

Delays in diagnosing pediatric brain tumors are often associated with limited awareness of the signs and symptoms by parents and healthcare professionals, as well as the absence of routine childcare follow-ups and delays in healthcare system referrals. To explore opportunities for reducing diagnostic delays, the study assessed the knowledge of pediatric brain tumor signs and symptoms, routine follow-up care for children, use of the Child Health Book (CHB), and referral intervals of a suspected case to a specialized center. This cross-sectional study collected data through interviews and virtual questionnaires. Between August and November 2023, 200 parents (pediatric and oncology departments) and 147 healthcare professionals (primary and tertiary care) participated. Except for headaches and seizures, the rates of parental recognition of warning signs were below 70%. Physicians in tertiary care demonstrated greater recognition of these warning signs than those in primary care (p=0.011). Recognition rates among nurses were below 75%. Primary and tertiary care professionals reported referral intervals >1 month in 10%-15% cases. Children routine follow-up care was reported in both levels. Over 75% of all participants reported that the CHB could be a useful tool for educating about childhood cancer. Our study provides essential insights to improve the early diagnosis of pediatric brain tumors. The findings emphasize the need to strengthen pediatric care follow-ups and use of CHB by parents and healthcare professionals to raise awareness of warning signs and symptoms, along with a flowchart for timely and accurate referrals to specialized centers.

Evaluation of the effects of Glycyrrhiza glabra and Syzygium aromaticum extracts on gene expression of Streptococcus mutans in patients with dental caries

Abstract. Al-Amili ML, Al-Jobori KM. 2025. Evaluation of the effects of Glycyrrhiza glabra and Syzygium aromaticum extracts on gene expression of Streptococcus mutans in patients with dental caries. Biodiversitas 26: 418-423. Dental caries, primarily associated with Streptococcus mutans, is among the most widespread diseases, particularly in developing regions such as Iraq. Licorice (Glycyrrhiza glabra) and clove (Syzygium aromaticum) are plants with significant economic value and antibacterial properties that potentially serve as alternatives to chemically synthesized antibiofilm agents. This study aimed to evaluate the antibacterial activity of G. glabra and S. aromaticum extracts against S. mutans and to compare their effects with those of antibiotics, mouthwashes, and toothpaste through gene expression analysis of gtfB and gtfD using RT-qPCR. In total, it did not include the methodology or provide details about the Sub-MIC because it was published separately as a second research study in another journal.100 specimens were collected from patients clinically diagnosed by dental physicians at the Hay Al-Hussein Specialized Center in Maysan City, Iraq. RNA was extracted from dentinal lesion specimens and reverse transcribed into complementary DNA (cDNA). Quantitative polymerase chain reaction (qPCR) was performed to analyze the expression of the gtfB and gtfD genes using the housekeeping gene 16S rRNA as an internal control. The analysis assessed the effects of licorice extract, clove extract, combined extracts, chlorhexidine mouthwash at sub-minimum inhibitory concentration (sub-MIC), and lacalut toothpaste on S. mutans. RT-qPCR results revealed that clove extract significantly reduced the expression of gtfB compared to other treatments, with fold changes of 0.178, 0.454, and 0.191. Licorice extract notably suppressed gtfD expression, with fold changes of 0.215, 0.390, and 0.003 for isolates 74, 80, and 46, respectively. These findings suggest that the plant extracts inhibited specific biofilm-related genes without necessarily reducing the overall bacterial growth. Therefore, these natural extracts can be developed as innovative natural anti-plaque agents.

Clinical depression and impact on patients with lysosomal diseases (LDs): Service evaluation from a specialist centre using the Beck's Depression Inventory (BDI-II)

Clinical depression and impact on patients with lysosomal diseases (LDs): Service evaluation from a specialist centre using the Beck's Depression Inventory (BDI-II)

Exploring patient experience of rehabilitation within the surgical pathway for lower limb soft tissue sarcoma in the UK: a single-centre study

PurposeThe primary treatment for localised soft tissue sarcoma (STS) is surgery. Surgery for lower limb sarcoma is associated with poorer functional outcomes than other anatomical sites. Rehabilitation is essential, yet provision is not standardised, and patient experience of current service delivery is unknown. This study therefore aimed to explore patients’ experiences of rehabilitation in the surgical pathway for lower limb STS at a United Kingdom (UK) specialist centre.MethodsA qualitative, descriptive phenomenological study was undertaken to explore patients’ rehabilitation experiences. Eight patients who had undergone lower limb STS surgery at a specialist centre were purposively sampled. Data were collected through semi-structured interviews and analysed using thematic analysis.ResultsThree main themes were identified: (1) Accessing the right services at the right time. Participants described good access to inpatient rehabilitation post-operatively but delays and challenges in accessing local services affected continuity of care. Rehabilitation gaps pre-operatively, and in facilitating return to meaningful activities, were described; (2) “Communication is key” — providing knowledge and support to navigate uncertainty. Unclear and unrealistic expectations of recovery were challenging. Communication was key to patients feeling supported and facilitating access to rehabilitation; (3) The importance of person-centred rehabilitation. Collaborative, person-centred rehabilitation optimised motivation and engagement.ConclusionParticipants experienced good access to inpatient rehabilitation post-operatively. In contrast, gaps and delays at other timepoints led to missed opportunities to support preparation for, and recovery from, surgery. A multidisciplinary approach across settings from diagnosis, to deliver person-centred rehabilitation, may improve access, expectation management and continuity of care.

Paragangliomas - Essentials for the ENT Doctor

Paragangliomas of the head and neck area should be detected early and diagnosed in their complexity at specialized centers, treated and, in the presence of paraganglioma syndrome, cared for for life. The continuing education article focuses in an abridged form on the most important basics of a complex clinical picture and deals with both sporadic head and neck paragangliomas and hereditary paraganglioma syndromes. It contains concise recommendations regarding genetic testing, the diagnosis of multicentric tumors, the multidisciplinary treatment approach and the need for lifelong monitoring.

Orthodontic Treatment of Patients With Disabilities in Norway: A Survey Among Orthodontists.

The objective of this study was to examine orthodontic treatment of patients with disabilities in Norway, as perceived by orthodontists. A digital questionnaire was sent out to the 220 registered members of the Norwegian Orthodontic Society and a response rate of 41% (n = 90) was achieved. The majority (92%) had received referrals for patients with disabilities, 53% reported treating up to 10 patients, while 37% reported treating up to 50 patients in the last 5 years. Most of the participants (64%) showed a willingness to treat more patients with disabilities, with this interest significantly and conversely linked to years in practice (p = 0.02, chi-squared test). Orthodontists desire more referrals to increase patient intake, and also financial incentives play a relevant role in this context. Regarding the preferred location for orthodontic treatment for patients with disabilities, 34% prefer treatment in specialized centers rather than the current setup in optional private practices. An association exists between this perspective and the readiness to treat additional patients (p = 0.009) CONCLUSION: Most orthodontists in Norway have treated patients with disabilities. Among the surveyed orthodontists, a notable 64% expressed a positive inclination toward treating more patients with disabilities or initiate such services. To enhance their acceptance of these patients, it seems crucial to increase referrals and provide improved financial incentives.

Survival prospects of pulmonary arterial hypertension associated with congenital heart disease: Insights from the HOPE registry.

Pulmonary arterial hypertension (PAH) is a severe complication among adult patients with congenital heart disease (ACHD). This study presents real-world data on risk stratification, pharmacotherapy and survival rates in PAH-ACHD. Data from PAH-ACHD patients were analyzed using The Hellenic Pulmonary Hypertension Registry (HOPE), spanning eight specialized centers between 2015 and 2023. Patients were categorized into low, intermediate, and high-risk groups using the ESC/ERS three-strata model to assess 1-year mortality risk. A total of 93 PAH-ACHD patients were included (median age 38.5years, 60.2% women). Most patients had an atrial septal defect (37.6%) or a ventricular septal defect (35.5%), with 11.8% presenting with complex ACHD. Eisenmenger syndrome was present in 35.5% of patients. The proportion of low-risk patients nearly doubled from first to last assessment (24.7% vs. 40.9%, p=0.001). Initially, 52.7% of patients were on PAH monotherapy, with a subsequent shift towards combination therapy (73.1%) during follow-up. Over a median follow-up of 5.9years, 29 patients (31.1%) died, with 1- and 5-year survival rates of 95.5% and 81.9%, respectively. Compared to the high-risk group, low- and intermediate-risk patients exhibited a 70% and 50% lower hazard of death, respectively. The lowest survival rates were observed in patients with Eisenmenger physiology. Survival prospects were favorable for the non-high risk patients in this nationwide cohort of PAH-ACHD patients. The observed shift towards combination therapy use may have contributed to the improvement in 1-year ESC mortality risk, underscoring the importance of timely combination therapy with PAH drugs.

Complex decision making for individual patients with penile cancer: benchmarking divergent practices in European high-volume reference centers - results from eUROGEN survey

Penile cancer (PeCa) remains a challenge due to its rarity and the lack of prospective studies, leading to treatment challenges and controversies. Guidelines offer recommendations, but discrepancies with clinical practice persist. This study analyzed treatment practices among specialists managing high-risk PeCa in European reference centers. A cross-sectional survey included 39 PeCa specialists from 13 European countries representing high-volume centers. Descriptive analysis assessed (neo)adjuvant therapy preferences, systemic regimen choices, immunotherapy use, and next-generation sequencing (NGS) integration. Variations in managing high-risk PeCa, especially in (neo)adjuvant therapy utilization, were noted among participants. The differences highlight the influence of professional backgrounds and variations in treatment approaches between participants. Systemic regimen preferences and immunotherapy utilization also varied. Limited NGS integration indicated gaps in precision medicine adoption. Limitations included sample size, self-reported data, and cross-sectional design. This study offered insights into PeCa management by specialists in high-volume European reference centers, stressing the need for evidence-based recommendations, guideline adherence, and collaboration to enhance PeCa care. Managing PeCa is complex due to its rarity and treatment controversies. This study examined practices among specialists in European reference centers, revealing treatment variations. The findings emphasize the importance of evidence-based care and collaboration in optimizing PeCa management.

Charles Huggins' Road Not Taken at the Brady Urological Institute

Introduction Twenty years before Charles Huggins became one of two urologists to win a Nobel Prize, he earned a different prestigious title: Hugh Hampton Young’s successor at the Brady Urological Institute. This article seeks to better understand Huggins’ relationship with the ‘Brady’ and why he accepted the position, only to repudiate the offer months later. Sources and Methods Archival research was conducted at the Alan Mason Chesney Medical Archives at Johns Hopkins and at the Hanna Holborn Gray Special Collections Research Center at The University of Chicago. Secondary sources were accessed as cited. Results Letters between Charles Huggins and Alfred Blalock, the Director of Surgery at Johns Hopkins Hospital (JHH), reveal that Huggins’ relationship with the Brady began earlier than previously reported. When Hugh Hampton Young retired in 1941, Blalock wanted Huggins to become the Chair, but socio-political factors interfered with his appointment. When Huggins was finally offered the position in 1945, he accepted the post but he resigned shortly thereafter. Conclusions The presented research provides insight into a turbulent period of transition at Hopkins. However, several archival gaps remain. Nevertheless, findings reveal that Huggins’ decision kept the Brady on the original trajectory set by Hugh Hampton Young. Huggins’ decision to relinquish the offered Chair position was motivated by something quite personal, his professional goals, and his sense of self.

Partington-Rochelle modified procedure of pancreatotomy with short pancreaticojejunostomy for groove pancreatitis: a technical note.

Groove pancreatitis (GP) is a chronic segmental pancreatitis which leads to altered pancreatic secretions and pancreatitis. The exact pathogenesis of GP has not been clearly identified to date but heavy smoking and chronic alcohol consumption seem to be the main factors involved. The resulting chronic pancreatitis (CP) is a debilitating disease causing abdominal pain often refractory to medical therapy, so much that the main indication for surgical treatment is intractable abdominal pain. A growing number of increasingly complex and diverse surgical operations for the treatment of this disease were reported. Operative procedures for CP are currently grouped into decompression procedures and pancreatic resections. No consensus about which one leads to the best outcomes, but every case should be tailored to specific clinical scenarios and single individuals. We report the case of a 44-year-old man with intractable abdominal pain due to GP underwent to Partington-Rochelle modified procedure. A Wirsung-jejunal side-to-side anastomosis as described by Partington-Rochelle was performed but with two variations: the first was the length of the anastomosis which was < 50mm (short anastomosis), while the second was the placement of two removable Bracci stent tube 6 Fr to drain respectively the distal and the proximal-medium part of Wirsung duct. The post-operative course was uneventful with good pain control. During follow-up no evidence of any radiologic signs of infection or fluid collections or pancreatic fistula nor recrudescent of abdominal pain. Nowadays a uniform approach to this pathology still is lacking. Although surgery can be carried out with satisfactory results in specialized centers, the specifical operation type should be tailored to every single cases.

First branchial cleft anomalies in children: long-term outcome in 16 patients.

First branchial cleft anomalies (FBCA) are rare congenital head and neck malformations, often subject to incorrect diagnosis and treatment. We present our experience with FBCA, focusing on clinical presentation, diagnosis, perioperative relation to the facial nerve, surgical approach, complications and patient satisfaction. A consecutive cohort of 16 patients undergoing surgical treatment for FBCA between 1999 and 2021 was analyzed. Demographic and clinical information was extracted from their medical records. Additionally 14 patients were interviewed by telephone to assess postoperative outcomes and patient satisfaction using a Likert scale. Sixteen patients (11 females, 5 males) were surgically treated at an age ranging from 7months to 13years. Type I and type II FBCA were evenly distributed. Lesions presented as cysts (n = 4), sinuses (n = 7) or fistulas (n = 5). The main symptoms were swelling, inflammation and discharge. Otologic symptoms were present in 31%. In 80% of patients, magnetic resonance imaging was needed to reach the correct diagnosis. Ten patients (63%) had a history of a surgical attempt in another center before definitive surgical treatment in our center. Complete excision was accomplished in all patients. Postoperative complications included limited Frey syndrome (n = 4), temporary hypertrophic scarring (n = 4), minimal paresis of the marginal mandibular branch of the facial nerve (n = 1) and limited external auditory canal stenosis (n = 1). One patient experienced a recurrence at the level of the external auditory canal, that required minor revision surgery. The average patient satisfaction score was 13.64 out of 15. Thorough clinical examination, awareness of the different entities and awareness of expert head and neck radiological advice are crucial for correct FBCA diagnosis and typing, determining the correct surgical approach. It is crucial that patients diagnosed with FBCA undergo surgery in specialized centers. Despite the challenging surgery and its potential complications, the overall patient satisfaction remains high post- treatment.

P-2317. Risk Factors Associated with post-Hematopoietic Stem Cell Transplant Mortality: Two-Year Cohort in a Public Hospital in Perú

Abstract Background In Latin America, Hematopoietic Stem Cell Transplants have experienced significant growth in recent years, both in terms of quantity and quality. Specialized hospitals and high-level medical centers have been established in several countries, including Perú, to carry out these procedures. The availability of compatible donors, the experience of the medical team, the condition of the recipient, the stage of the disease, the resources available for monitoring and surveillance of transplant complications are some of the factors involved in the success of the transplant. This study aims to describe the characteristics and factors associated with mortality in hematopoietic transplant recipients in patients post hematopoietic stem cell transplant attended at a hospital in Peru sociodemographic characteristics Methods Retrospective cohort study, conducted at a hospital in Peru between April and May 2024. We included all patients who underwent hematopoietic stem cell transplant between 2017 and 2022. We evaluated death at one hundred days of follow-up and at two years. Survival at 100 days post transplant Results We evaluated 342 medical records, median age 23 years, 53.5% were women. The most common diagnosis for the transplant was leukemia 83.6%. Mortality in the first 100 days post TPH was 8.2%, and at two years was 30.4%. In the multivariate model and at one hundred days, sepsis HRa: 24.72 (7.95 to 76.85) was associated with death and engraftment to survival HRa: 0.26 (0.07 to 0.93). Regarding mortality at two years, haploidentical transplant HRa:1.92 (1.20 to 3.08), sepsis HRa: 5.36 (2.98 to 9.63), and disease relapse HRa: 4.32 (2.80 to 6.66) were associated with death and engraftment to survival HRa: 0.12 (0.06 to 0.25). 2-year post-transplant survival Conclusion Sepsis was the primary risk factor associated with death in the first one hundred days post-transplant. At two years of follow-up, disease relapse and sepsis were the risk factors for death. Hematopoietic stem cell transplant in Peru is performed in few specialized centers and this initial report identifies the need for further interventions to reduce the risk of infection. Disclosures All Authors: No reported disclosures

Myocardial mechanics and outcomes after transcatheter aortic valve replacement. Does strain rate have added prognostic value?

Abstract Introduction Transcatheter aortic valve implantation (TAVI) has firmly become treatment option for elderly and/or high-risk patients with severe symptomatic aortic stenosis. Studies have shown that TAVI induces improvement in left ventricular (LV) systolic function, quantified using global longitudinal strain (GLS). Purpose To test if improvement in longitudinal deformation post TAVI is associated with changes in strain rate – the only non-invasive echo parameter associated with myocardial contractility. We wanted to assess impact of changes in longitudinal deformation and strain rate (SR) on clinical outcomes. Methods We studied retrospectively all consecutive patients who underwent TAVI in our Valve specialist center from 1st April 2023 to 31st March 2024. All participants underwent a transthoracic echocardiogram before and within 3 months following TAVI. We have re-measured mean trans-valvar pressure gradients and performed offline speckle tracking analysis using dedicated software (TomTec and GE EchoPAC) before and after TAVI. Data on all-cause mortality and cardiac hospitalizations were obtained from electronic medical records. Results Overall, 305 patients (mean age of 81±7.7 years) with severe aortic stenosis underwent TAVI. 255/305 patients (83%) had a successful and uncomplicated TAVI procedure, 26 (8.5%) developed complete heart block requiring permanent pacemaker insertion during the index admission, 15 (4.9%) experienced vascular access complications (pseudoaneurysm, haematoma), 5 (1.6%) developed haemopericardium requiring drainage and 5 (1.6%) had TAVI-related stroke, all patients were discharged with routine F/U in place. 247 (81%) who had complete echocardiography data were included into analysis, 45% were female. Significant reduction in trans-valvar gradient was accompanied by significant improvement in LV myocardial and endocardial GLS and average LV strain rate post-TAVI. The mean follow-up period was 246±107 days. There were 8 cases contributing to all-cause mortality and 12 cardiac hospitalizations. There was a significant difference in SR between alive and deceased patients (0.94±0.3 vs. 0.70±0.2, P=0.026). Post TAVI strain rate (HR 45.6; 95% CI 1.67 to 124.6, P&amp;lt;0.05) was the only independent predictor of all-cause mortality. Conclusion TAVI produces significant early improvement in LV mechanics. LV strain rate is an important independent predictor of all-cause mortality post TAVI. The addition of LV SR during echocardiographic assessment may be of value for the early identification of TAVI patients who are at greatest risk and who may benefit from early intensified risk factor management. KM CURVE: SR &amp; ALL CAUSE MORTALITY

Screening for sickle cell disease by point-of-care tests in Italy: pilot study on 1000 at risk children

Sickle cell disease (SCD) is a global health problem causing premature deaths and preventable severe chronic complications. A priority goal to improve outcomes both in the short and long term is the screening for early diagnosis and access to specialized care. In Italy, as in other countries, no systematic national screening program is available. A regional pilot project was developed with the aim to screen 1000 children at risk of SCD in Italy. Primary care paediatricians received point-of-care tests (POCTs) to detect abnormal haemoglobin (Hb) to be offered to children regularly followed at their own clinics. Children positive to the POCT were referred to the regional paediatric specialized centre for diagnosis confirmation and follow up. Among 1000 at risk children screened, 85 (8,5%) tested positive for an abnormal Hb. HbS trait was reported in 69 (7%) children, HbC trait in 13 (1,3%) and SCD was diagnosed in 3 (0,3% overall; 0,56% in African background) children. African family background was the most affected by sickle mutations and all children with SCD had African ancestry. Only 56/259 (22%) primary care paediatricians invited but 20/21 (95%) reception centres adhered to the pilot screening project.Conclusions: A screening program for SCD performed by the primary care paediatricians is feasible and relatively easy to organize. SCD affects mainly children with African family background and the scarce adherence of primary care paediatricians, in contrast to the high adhesion of charitable institutions, outlines the need for a mandatory screening for SCD, and improved awareness among health care providers.What is Known:• Sickle Cell Disease (SCD) is a serious global health problem that requires management in specialized centres from the first months of life.• In the absence of neonatal screening for SCD, primary health care settings represent a feasible and costeffective approach for early disease detection.What is New:• In Italy, screening for SCD performed by primary care pediatricians detected a hemoglobin variant in 8.5% children, with a disease prevalence of 0.3% in the whole population and 0.56% in children with African family background.• The poor adherence of paediatricians to the voluntary screening for SCD highlights the need for legislative interventions and training activities to ensure early diagnosis and rapid access to care for all children affected by SCD.

Plastic surgical treatment of neurofibromatosis type 1

Neurofibromatosis type1 (NF1, formerly Recklinghausen's disease) is a genetic tumor predisposition syndrome in which the mutation of atumor suppressor gene (neurofibromin) leads to the development of mostly benign neurofibromas of the skin and the central and peripheral nervous systems and malformations or tumors of other organ systems. Patients with NF1 should receive lifelong interdisciplinary care in specialized centers and important treatment decisions should be made by a regularly meeting interdisciplinary panel of experts. Plastic surgery plays an important role in the multidisciplinary management of all clinical forms of NF1-associated peripheral nerve sheath tumors, from cutaneous and subcutaneous to deep nodular and diffuse plexiform neurofibromas. Each patient requires individualized surgical planning, whereby the timing and extent of surgery are determined by the accompanying symptoms, functional and esthetic limitations, disease progression and potential malignant transformation. As any region of the body can be affected, the esthetic and reconstructive procedures required include awide range of interventions, such as eyelid surgery and facial restoration to breast shaping and nerve reconstruction or motor replacement surgery. A timely surgical intervention can have aprofoundly positive effect on the course of the disease and the quality of life of those affected and, in the case of transformation into amalignant peripheral nerve sheath tumor (MPNST), can even be lifesaving.

Management and outcomes of thoracic sarcomas - a collaboration between Orthopaedic Oncology and cardiothoracic surgery: seven-year clinical data from a tertiary referral centre

IntroductionSarcomas are rare cancers originating from mesenchymal tissues, manifesting in diverse anatomical locations, but notably in connective tissue, muscles and the skeleton. Thoracic sarcomas present a unique diagnostic and surgical challenge attributable to their rarity and pathoanatomy. Standard practice currently comprises wide surgical excision, often accompanied by adjuvant chemotherapy and/or radiotherapy. This approach necessitates a multidisciplinary team, ideally in specialised cancer centres. The Oxford Bone and Soft Tissue Tumour Service is one such centre, and routinely treats such cancers through collaboration between orthopaedic oncology and cardiothoracic surgeons, as well as members of the wider MDT. This study reports the current management and outcomes of primary thoracic sarcoma patients at the Oxford Sarcoma Service over a seven-year period.ObjectivesGiven the rarity of thoracic sarcomas, and their associated diagnostic and management complexities, our aim is to report on the treatment strategies and outcomes of primary thoracic sarcoma patients treated at the Oxford Sarcoma Service from 2017 to 2023.MethodsData pertaining to all thoracic sarcoma cases discussed in multidisciplinary meetings at the Oxford tertiary centre from 2017 to 2023 were retrieved from the local electronic database. These were analysed using appropriate statistical tests to determine significance of the various observations made.ResultsOf 113 identified cases, chondrosarcoma emerged as the most prevalent histological subtype among 22 distinct varieties. 58% of cases exhibited high-grade features. 32 sarcoma-related deaths occurred, with a mean time from diagnosis to death of 23.16 months. A notable association was observed between high-grade sarcomas and mortality (p = 0.0280). Surgical resection was performed in 77 cases, with 49% of these undergoing surgical resection alone i.e. the patient received no radio- or chemotherapy. Both surgical intervention (p < 0.0001) and clear margins (p = 0.0051) were significantly linked to improved survival. Local recurrence was noted in 28.6% of the 77 surgical cases, and predominantly in the high-grade sarcomas (81.8%). However, no statistical association was found between recurrence and margin status in our data.ConclusionOur results indicate that primary resection remains the cornerstone of thoracic sarcoma treatment, representing the single strongest independent factor for survival in treatable cases. Variability in outcomes and overall survival likely stems from factors such as histological diversity, predominance of high-grade sarcomas, and wide age range at diagnosis. Ongoing prospective database update and collaborative efforts across centres would further clarify prognoses and recommendations for specific tumours, based on observational data.

Implementing psychosocial interventions for teachers' mental health: Protocol for integrating scoping review with teachers lived experiences in LMICs.

Teachers are pivotal in shaping educational environments and student development but face significant occupational stress and high rates of mental problems. Despite the availability of various psychosocial interventions, comprehensive evidence of their effectiveness and implementation is limited for this occupational group, especially in low- and middle-income countries (LMICs). This mixed methods study aims to conduct a scoping review of characteristics, effectiveness, and implementation outcomes of psychosocial interventions for teachers' mental health and mental problems, integrating these with teachers' lived experiences to inform the implementation of mental health interventions in LMICs. The study combines a scoping review with insights from an advisory group of teaching professionals with relevant lived experiences (PWLEs). The review will focus on examining psychosocial interventions studies promoting mental health or addressing mental problems among grade 1-12 school teachers of all genders, ages and contexts, except those working in specialized settings, such as special education centers, or disaster- or crisis-stricken zones. Intervention studies exclusively focusing on physical health or job-related outcomes will be excluded. Using pre-defined search terms, quantitative and qualitative research studies, including unpublished literature, will be searched across multiple databases. Titles and abstracts of identified studies will be screened against inclusion criteria, and the potentially relevant sources will be retrieved in full. Data will be extracted using a data extraction sheet developed for the study covering variables related to participant characteristics, intervention characteristics, study context, effectiveness and implementation outcomes. A purposively selected sample of 10 PWLEs will form the study advisory group and participate in four online workshop-group discussions. The meetings will include a presentation of the scoping review findings, followed by discussions on the relevance of these intervention packages for LMICs, adaptations needed to make them acceptable among school teachers and feasible for delivery in low-resourced settings like India. The synthesis of the data will employ narrative and thematic approaches to generate actionable insights for implementing psychosocial interventions in LMICs. This study will provide comprehensive evidence on the characteristics and outcomes of psychosocial interventions for teachers' mental health and mental problems. By integrating insights from teachers with relevant lived experiences, the study will provide practical guidelines for adapting and implementing psychosocial interventions among school teachers in LMICs. Review registration number: Open Science Framework, doi.org/10.17605/OSF.IO/GF59J.