A 13-year patient journey of infant giant clival chordoma: case report and literature review.

Chordomas are rare malignant bone tumours that develop from the ectopic remnants of the embryonic notochord. In contrast to adults, the majority in children under 16 present intra-cranially (63%). In 2006, we reported the youngest case of a large clival chordoma, a 15-week old baby, the second case to present without skull base involvement and the fourth case of chordoma in a patient with tuberous sclerosis (TS) Kombogiorgas (Childs Nerv Syst 22(10):1369-1374, 2006). In this report, we provide an update on this patient's journey through a range of therapeutic options and summarize an update of the literature, since 2006, for this patient group.

Orthognathic Surgery in Goldenhar Syndrome With a Rare Course of the IAN.

Although there are multiple variations of accessory foramina described in the literature, to our knowledge there is only 1 report of an isolated mandibular foramen and inferior alveolar neurovascular bundle that courses through the lateral ramus, particularly in those with branchial arch syndromes. Goldenhar syndrome, of the oculo-auriculo-vertebral spectrum, is a rare congenital condition, which most characteristically presents with hemifacial microsomia. Depending on the severity of hemifacial microsomia, there are predictable treatment modalities to correct the consequent facial asymmetry. We report on a patient with Goldenhar syndrome who was found to have a unique course of the inferior alveolar nerve during orthognathic surgery work-up and treatment.

Outcome measures in solid organ donor management research: a systematic review.

AIM: We systematically reviewed published outcome measures across randomised controlled trials (RCTs) of donor management interventions. METHODS: The systematic review was conducted in accordance with recommendations by the Cochrane Handbook and Preferred Reporting Items for Systematic Reviews and Meta-Analyses statement. We searched MEDLINE, Embase, CENTRAL, Web of Science, and trial databases from 1980 to February 2021 for RCTs of donor management interventions. RESULTS: Twenty-two RCTs (n=3432 donors) were included in our analysis. Fourteen RCTs (63.6%) reported a primary outcome relating to a single organ only. Eight RCTs primarily focused on aspects of donor optimisation in critical care. Thyroid hormones and methylprednisolone were the most commonly evaluated interventions (five and four studies, respectively). Only two studies, focusing on single organs (e.g. kidney), evaluated outcomes relating to other organs. The majority of studies evaluated physiological or biomarker-related outcomes. No study evaluated recipient health-related quality of life. Only one study sought consent from potential organ recipients. CONCLUSIONS: The majority of RCTs evaluating donor management interventions only assessed single-organ outcomes or effects on donor stability in critical care. There is a need for an evaluation of patient-centred recipient outcomes and standardisation and reporting of outcome measures for future donor management RCTs.

Brief, effective experience to increase first-year medical students' nutrition awareness.

Background: Wellness is an important concept for medical students to learn, both for their own health and for their patients. Since nutrition is an essential part of one's wellness that can positively or negatively impact one's health, it is important for medical students to learn approaches to nutritional wellness. Studies have shown that physicians' nutrition attitudes and clinical practices are positively correlated with their dietary practices.Objective: Here, we describe a brief nutrition-based education experience for first-year students offered at the start of the medical school curriculum that is designed to increase their nutrition awareness.Design: The nutrition experience involved five components: 1) having students complete three 24-hour food recalls; 2) comparing their recalls to nutrient standards; 3) emphasizing strategies that include simple, nutritionally sound food choices and preparation; 4) surveying students on their implementation of personal healthy nutritional strategies; and 5) requesting future recommendations for modifying the educational experience.Results: Most students' diets did not meet the recommended dietary levels for several nutrients, and these deficiencies corresponded to specific food group inadequacies. Forty percent of the students responded to a three-month follow-up survey. Of these students, 46% implemented one of the presented strategies to improve their food intake. Most changes included the addition or deletion of a particular food. Seventy-three percent recommended repeating the program in the future.Conclusions: We demonstrate that a brief 2.5-hour nutrition wellness experience can increase nutrition awareness and promote dietary change in incoming medical students. Many felt that the experience was valuable and recommended offering a similar experience to future classes.

Optimisation of the organ donor and effects on transplanted organs: a narrative review on current practice and future directions.

Mortality remains high for patients on the waiting list for organ transplantation. A marked imbalance between the number of available organs and recipients that need to be transplanted persists. Organs from deceased donors are often declined due to perceived and actual suboptimal quality. Adequate donor management offers an opportunity to reduce organ injury and maximise the number of organs than can be offered in order to respect the donor's altruistic gift. The cornerstones of management include: correction of hypovolaemia; maintenance of organ perfusion; prompt treatment of diabetes insipidus; corticosteroid therapy; and lung protective ventilation. The interventions used to deliver these goals are largely based on pathophysiological rationale or extrapolations from general critical care patients. There is currently insufficient high-quality evidence that has assessed whether any interventions in the donor after brain death may actually improve immediate post-transplant function and long-term graft survival or recipient survival after transplantation. Improvements in our understanding of the underlying mechanisms following brain death, in particular the role of immunological and metabolic changes in donors, offer promising future therapeutic opportunities to increase organ utilisation. Establishing a UK donor management research programme involves consideration of ethical, logistical and legal issues that will benefit transplanted patients while respecting the wishes of donors and their families.

Toward a Unifying Model of Self-Regulation: A Developmental Approach.

The ability to self-regulate is key to healthy, competent functioning. The breadth of evidence supporting the importance of self-regulation is matched by such a diversity of terms, concepts, measures, and levels of analysis that the National Institutes of Health called for progress toward a unifying model. In this article, we review a lineage of conceptual models and suggest a path toward a more unifying model of self-regulation that encompasses both the dynamics of moment-to-moment changes and age-related change. Drawing from these models, we define self-regulation as the influence of the recruitment of executive processes on prepotent responses. We define these terms, locating self-regulation in the dynamic relations between prepotent responses and executive processes, and offer a theoretical-mathematical approach to testing this model.

Perception of Cry Characteristics in 1-Month-Old Infants Later Diagnosed with Autism Spectrum Disorder.

This study investigates parental perceptions of cries of 1-month-old infants later diagnosed with autism spectrum disorder (ASD) and non-ASD controls. Parents of children with and without ASD listened to cry recordings of infants later diagnosed with ASD and comparison infants and rated them on cry perception scales. Parents completed the Broad Autism Phenotype Questionnaire (BAPQ) to assess the potential relations between traits associated with autism and cry perception. Across parents, ASD infant cries were rated as more distressed, less typical, and reflecting greater pain, with no significant differences between parent groups. Parents of children with ASD scored higher on the BAPQ compared to parents of children without ASD. Follow up analyses explored the relations between BAPQ score and cry ratings.

Burden of cytomegalovirus disease in allogeneic hematopoietic cell transplant recipients: a national, matched cohort study in an inpatient setting.

PURPOSE OF THE STUDY: No studies have compared the risk of mortality or graft-versus-host disease, in an inpatient setting in France, in allogeneic hematopoietic cell transplant recipients who develop cytomegalovirus disease with those who do not. This study assessed the impact of cytomegalovirus disease on clinical outcomes and healthcare resource utilization in allogeneic hematopoietic cell transplant recipients using the French Programme de Médicalisation des Systèmes d'Information database. PATIENTS AND METHODS: Recipients who had undergone allogeneic hematopoietic cell transplant in French hospitals between 2008 and 2011 were included in this retrospective, matched cohort study. Those with cytomegalovirus disease were each matched with two allogeneic hematopoietic cell transplant recipients without cytomegalovirus disease according to demographic and clinical characteristics. Probabilities of in-hospital mortality, graft rejection and/or graft-versus-host disease, and healthcare resource utilization were compared up to 12 months after cytomegalovirus disease diagnosis. RESULTS: Overall, 4884 transplant recipients were enrolled, of which 194 had cytomegalovirus disease. Of these, 165 recipients with cytomegalovirus disease were matched to 330 without cytomegalovirus disease (1:2 ratio). The development of cytomegalovirus disease was associated with a significantly higher risk of in-hospital mortality (relative risk = 1.7, p = 0.0005) and higher cumulative number of inpatient days (p < 0.0001), but was not associated with a significantly higher risk of graft rejection and/or graft-versus-host disease or healthcare costs. CONCLUSIONS: Due to the increased risk of in-hospital mortality and higher cumulative number of inpatient days in allogeneic hematopoietic cell transplant recipients with cytomegalovirus disease versus those without, new strategies to prevent and manage cytomegalovirus disease are warranted.

Variability in distribution and use of tuberculosis diagnostic tests in Kenya: a cross-sectional survey.

BACKGROUND: Globally, 40% of all tuberculosis (TB) cases, 65% paediatric cases and 75% multi-drug resistant TB (MDR-TB) cases are missed due to underreporting and/or under diagnosis. A recent Kenyan TB prevalence survey found that a significant number of TB cases are being missed here. Understanding spatial distribution and patterns of use of TB diagnostic tests as per the guidelines could potentially help improve TB case detection by identifying diagnostic gaps. METHODS: We used 2015 Kenya National TB programme data to map TB case notification rates (CNR) in different counties, linked with their capacity to perform diagnostic tests (chest x-rays, smear microscopy, Xpert MTB/RIF®, culture and line probe assay). We then ran hierarchical regression models for adults and children to specifically establish determinants of use of Xpert® (as per Kenyan guidelines) with county and facility as random effects. RESULTS: In 2015, 82,313 TB cases were notified and 7.8% were children. The median CNR/100,000 amongst 0-14yr olds was 37.2 (IQR 20.6, 41.0) and 267.4 (IQR 202.6, 338.1) for ≥15yr olds respectively. 4.8% of child TB cases and 12.2% of adult TB cases had an Xpert® test done, with gaps in guideline adherence. There were 2,072 microscopy sites (mean microscopy density 4.46/100,000); 129 Xpert® sites (mean 0.31/100,000); two TB culture laboratories and 304 chest X-ray facilities (mean 0.74/100,000) with variability in spatial distribution across the 47 counties. Retreatment cases (i.e. failures, relapses/recurrences, defaulters) had the highest odds of getting an Xpert® test compared to new/transfer-in patients (AOR 7.81, 95% CI 7.33-8.33). Children had reduced odds of getting an Xpert® (AOR 0.41, CI 0.36-0.47). HIV-positive individuals had nearly twice the odds of getting an Xpert® test (AOR 1.82, CI 1.73-1.92). Private sector and higher-level hospitals had a tendency towards lower odds of use of Xpert®. CONCLUSIONS: We noted under-use and gaps in guideline adherence for Xpert® especially in children. The under-use despite considerable investment undermines cost-effectiveness of Xpert®. Further research is needed to develop strategies enhancing use of diagnostics, including innovations to improve access (e.g. specimen referral) and overcoming local barriers to adoption of guidelines and technologies.

Collaboration between doctors and nurses in children's cancer care: insights from a European project.

PURPOSE: It has long been recognised that effective cancer care is not possible without multi-professional team working. Collaboration and multi-professional working however are known to be less than straightforward. This project aimed to use a collaborative approach to explore and facilitate professional groups to work together more effectively in the field of children's cancer care. METHOD: Based on an earlier project in Italy, a three-year seminar series was organised involving both a doctor and nurse from 15 paediatric haematology/oncology units across Europe. Participants had to be able to speak English and commit to participate in annual seminars as well as the development and implementation of a local project to enhance doctor-nurse collaboration in their own unit. Appreciative Inquiry was the methodological approach used to address organisational as well as interpersonal change. RESULTS: Fifteen doctor-nurse teams were initially selected from a range of different countries, and 10 completed the project. Key outcomes reported include implementation and successful completion of projects, publication of the results achieved, participant satisfaction with improvements in collaboration. Feedback from participants would suggest that change had been implemented and possibly sustained. CONCLUSIONS: Active involvement and group support were required for success. More formal relationships needed to be activated with participating centres to guarantee support for those involved in implementing lasting change. A web-based resource to allow other programmes and centres to use the resources developed has been made available. The same approach, we believe, could be used to improve multi-professional working in the care of other childhood illnesses.

Oxygen is an essential medicine: a call for international action.

Hypoxaemia is commonly associated with mortality in developing countries, yet feasible and cost-effective ways to address hypoxaemia receive little or no attention in current global health strategies. Oxygen treatment has been used in medicine for almost 100 years, but in developing countries most seriously ill newborns, children and adults do not have access to oxygen or the simple test that can detect hypoxaemia. Improving access to oxygen and pulse oximetry has demonstrated a reduction in mortality from childhood pneumonia by up to 35% in high-burden child pneumonia settings. The cost-effectiveness of an oxygen systems strategy compares favourably with other higher profile child survival interventions, such as new vaccines. In addition to its use in treating acute respiratory illness, oxygen treatment is required for the optimal management of many other conditions in adults and children, and is essential for safe surgery, anaesthesia and obstetric care. Oxygen concentrators provide the most consistent and least expensive source of oxygen in health facilities where power supplies are reliable. Oxygen concentrators are sustainable in developing country settings if a systematic approach involving nurses, doctors, technicians and administrators is adopted. Improving oxygen systems is an entry point for improving the quality of care. For these broad reasons, and for its vital importance in reducing deaths due to lung disease in 2010: Year of the Lung, oxygen deserves a higher priority on the global health agenda.

Policy interventions that attract nurses to rural areas: a multicountry discrete choice experiment.

OBJECTIVE: To evaluate the relative effectiveness of different policies in attracting nurses to rural areas in Kenya, South Africa and Thailand using data from a discrete choice experiment (DCE). METHODS: A labelled DCE was designed to model the relative effectiveness of both financial and non-financial strategies designed to attract nurses to rural areas. Data were collected from over 300 graduating nursing students in each country. Mixed logit models were used for analysis and to predict the uptake of rural posts under different incentive combinations. FINDINGS: Nurses' preferences for different human resource policy interventions varied significantly between the three countries. In Kenya and South Africa, better educational opportunities or rural allowances would be most effective in increasing the uptake of rural posts, while in Thailand better health insurance coverage would have the greatest impact. CONCLUSION: DCEs can be designed to help policy-makers choose more effective interventions to address staff shortages in rural areas. Intervention packages tailored to local conditions are more likely to be effective than standardized global approaches.

Overexpression of hns in the plant growth-promoting bacterium Enterobacter cloacae UW5 increases root colonization.

AIMS: Plant growth-promoting rhizobacteria (PGPR) introduced into soil often do not compete effectively with indigenous micro-organisms for plant colonization. The aim of this study was to identify novel genes that are important for root colonization by the PGPR Enterobacter cloacae UW5. METHODS AND RESULTS: A library of transposon mutants of Ent. cloacae UW5 was screened for mutants with altered ability to colonize canola roots using a thermal asymmetric interlaced (TAIL)-PCR-based approach. A PCR fragment from one mutant was reproducibly amplified at greater levels from genomic DNA extracted from mutant pools recovered from seedling roots 6 days after seed inoculation compared to that from the cognate inoculum cultures. Competition assays confirmed that the purified mutant designated Ent. cloacae J28 outcompetes the wild-type strain on roots but not in liquid cultures. In Ent. cloacae J28, the transposon is inserted upstream of the hns gene. Quantitative RT-PCR showed that transposon insertion increased expression of hns on roots. CONCLUSIONS: These results indicate that increased expression of hns in Ent. cloacae enhances competitive colonization of roots. SIGNIFICANCE AND IMPACT OF THE STUDY: A better understanding of the genes involved in plant colonization will contribute to the development of PGPR that can compete more effectively in agricultural soils.

Estimation of renal function and its potential impact on carboplatin dosing in children with cancer.

Renal function-based carboplatin dosing is used routinely in paediatric oncology clinical practice. It is important that accurate assessments of renal function are carried out consistently across clinical centres, a view supported by recently published British Nuclear Medicine Society (BNMS) guidelines for measuring glomerular filtration rate (GFR). These guidelines recommend the use of a radioisotope method for GFR determination, with between two and five blood samples taken starting 2 h after radioisotope injection and application of the Brochner-Mortensen (BM) correction factor. To study the likely impact of these guidelines, we have investigated current practices of measuring GFR in all 21 Children's Cancer and Leukaemia Group (CCLG) paediatric oncology centres in the United Kingdom. This information was used to evaluate the potential impact on renal function-based carboplatin dosing using raw 51Cr-EDTA clearance data from 337 GFR tests carried out in children with cancer. A questionnaire survey revealed that between two and four samples were taken after isotope administration, with BM and Chantler corrections used in 38% (8/21) and 28% (6/21) of centres, respectively. A change from Chantler to BM correction, based on the BNMS guidelines, would result in a > 10% decrease in carboplatin dose in at least 15% of patients and a > 25% decrease in 2% of patients. A greater proportion of patients would have an alteration in carboplatin dose when centres not using any correction factor implement the BM correction. The increase in estimated 51Cr-EDTA half-life observed by omitting the I h sample decreases carboplatin dose by > 10% in 23-52% of patients and by > 25% in 3% of patients. This study highlights current variations in renal function measurement between clinical centres and the potential impact on carboplatin dosing. A standard methodology for estimating GFR should be followed to achieve uniform dosing in children with cancer.

Heat transfer coefficient: Medivance Arctic Sun Temperature Management System vs. water immersion.

BACKGROUND AND OBJECTIVE: To improve heat transfer, the Medivance Arctic Sun Temperature Management System (Medivance, Inc., Louisville, CO, USA) features an adhesive, water-conditioned, highly conductive hydrogel pad for intimate skin contact. This study measured and compared the heat transfer coefficient (h), i.e. heat transfer efficiency, of this pad (hPAD), in a heated model and in nine volunteers' thighs; and of 10 degrees C water (hWATER) in 33 head-out immersions by 11 volunteers. METHODS: Volunteer studies had ethical approval and written informed consent. Calibrated heat flux transducers measured heat flux (W m-2). Temperature gradient (DeltaT) was measured between skin and pad or water temperatures. Temperature gradient was changed through the pad's water temperature controller or by skin cooling on immersion. RESULTS: The heat transfer coefficient is the slope of W m-2/DeltaT: its unit is W m-2 degrees C-1. Average with (95% CI) was: model, hPAD = 110.4 (107.8-113.1), R2 = 0.99, n = 45; volunteers, hPAD = 109.8 (95.5-124.1), R2 = 0.83, n = 51; and water immersion, hWATER = 107.1 (98.1-116), R2 = 0.86, n = 94. CONCLUSION: The heat transfer coefficient for the pad was the same in the model and volunteers, and equivalent to hWATER. Therefore, for the same DeltaT and heat transfer area, the Arctic Sun's heat transfer rate would equal water immersion. This has important implications for body cooling/rewarming rates.

Evaluation of assessment instruments used on children presenting with altered level of consciousness.

OBJECTIVE: To compare current care with a proposed gold standard (formal coma scale use) for assessment of children presenting with altered consciousness. DESIGN: A prospective study. SETTING: Kenyatta National Hospital, Nairobi, Kenya. SUBJECTS: One hundred and seventy children consecutively admitted to the hospital. MAIN OUTCOME MEASURES: Proportion of children assessed using a formal coma scale (gold standard) while presenting with altered consciousness. RESULTS: Findings revealed that only 8% of children were assessed using the formal coma scale at admission. The level of neurological deficit was described using general and potentially misleading descriptive terms rather than formal coma scales. CONCLUSION: The use of a formal coma scale and relevant simple investigations were seldom used. The quality of care of children admitted to the hospital with altered consciousness standard would benefit from defining and implementing management guidelines (protocols).

Outcome of children with posterior fossa medulloblastoma: a single institution experience over the decade 1994-2003.

AIM: While the impact of radiotherapy in the management of medulloblastoma was recognised, the introduction of chemotherapy was investigated in clinical trials and shown to confer an additional advantage. We reviewed the outcome of a series of consecutive patients to assess the impact in a population-based clinical establishment. MATERIALS AND METHODS: A series of 38 children treated for medulloblastoma at Birmingham Children's Hospital between 1994 and 2003 was analysed. The effect of surgery, radiotherapy, chemotherapy and metastasis on survival was analysed. RESULTS: The overall 5-year survival rate was 61.4% for the 36 patients who had resective surgery, while 2 patients had biopsy only and died within a few months. There was no operative mortality. The incidence of hydrocephalus needing permanent shunting was higher in the first 3 years of life (p = 0.007, chi-square). The 5-year survival rate of patients with total and sub-total excision of medulloblastoma was 61.1% and 61.8%, respectively. The 5-year survival rate of patients older than 3 years was 73.4% and for patients under 3 years was 36.3% (p = 0.007, log rank). Metastases at presentation did not influence survival. All deaths occurred in the first 32 months. CONCLUSION: The contribution of chemotherapy in the improvement of the overall survival appears more evident in children younger than 3 years or presenting with metastases. The absence of significant difference in survival between patients with total or sub-total excision of medulloblastoma supports the view that total excision of medulloblastoma can be avoided when the risk for potential intra-operative damage and consequent neurological deficits is high.

Infantile clivus chordoma without clivus involvement: case report and review of the literature.

BACKGROUND: We present a giant clival chordoma with disseminated disease but without involvement of the clivus. To our knowledge, this is the youngest child and only the second case, presenting without base of skull involvement, in paediatric literature and the fourth reported case of a chordoma in a patient with tuberous sclerosis. DISCUSSION: We discuss the subtle presentation, difficulties in diagnosis and management and also review the literature.