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Children with single-suture craniosynostosis have small but significant deficits in appearance ratings and neurodevelopment. Traditionally, these parameters are studied using a full battery of examinations, which are very time consuming. This study evaluated a convenient method to measure psychosocial parameters in this population by utilizing patient-reported outcomes measures to evaluate cognitive function and stigma. Stigma and cognitive function were measured, using the Patient-Reported Outcomes Measurement Information System and Quality of Life in Neurological Disorders questionnaires, in 59 consecutive patients at least 5 years old presenting to clinic from July of 2018 to January of 2020 with repaired single-suture craniosynostosis. Parents completed parent proxy cognitive function surveys for patients under age 8. Questionnaires were administered electronically as part of clinical care. Scores were automatically transferred to the electronic medical record and correlated with previously acquired Child Behavior Checklist results. Median time to complete the questionnaires was 57 and 49 seconds, respectively. Stigma and cognitive function were significantly correlated with the associated Child Behavior Checklist subscores (Spearman's rho, -0.384, p = 0.023; and Spearman's rho, -0.683, p = 0.001, respectively). The Patient-Reported Outcomes Measurement Information System and Quality of Life in Neurological Disorders questionnaires offer a convenient method of screening psychosocial parameters in children with single-suture craniosynostosis that otherwise would be difficult to obtain during standard visits. Short completion times and electronic scoring increase clinical utility.Diagnostic, II.

Aims Wireless capsule endoscopy (WCE) and magnetic resonance enterography (MRE) are increasingly utilized to evaluate the small bowel (SB) in Crohn’s disease (CD). The primary aims were to compare the ability of WCE and MRE to detect SB inflammation in children with newly diagnosed CD, and in the terminal ileum (TI) to compare them to ileo-colonoscopy. Secondary aims were to compare diagnostic accuracy of WCE and MRE and changes in Paris classification after each study. Methods Patients (10 to 17 years of age) requiring ileo-colonoscopy for suspected CD were invited to participate. Only patients with endoscopic/histologic evidence of CD underwent MRE and WCE. SB inflammation and extent were documented and comparative analyses performed. Results Of 38 initially recruited subjects, 20 completed the study. WCE and MRE were similarly sensitive in identifying active TI inflammation (16 [80%] versus 12 [60%]) and any SB inflammation (17 [85%] versus 16 [80%]). However, WCE detected more extensive SB disease than MRE with active inflammation throughout the SB in 15 [75%] versus 1 [5%] patient (P < 0.001). Moreover, WCE was more likely to detect proximal SB disease (jejunum and ileum) compared to MRE (85% versus 50%, P = 0.04). Overall, the Paris classification changed in 65% and 85% of patients following MRE and WCE, respectively. Conclusions WCE is as sensitive as MRE for identifying active TI inflammation, but appears more sensitive in identifying more proximal SB inflammation. In the absence of concern regarding stricturing or extra-luminal disease WCE can be considered for the evaluation of suspected SB CD.

BACKGROUND Asymptomatic children with Crohn’s disease (CD) require ongoing monitoring to ensure early recognition of a disease exacerbation. AIM In a cohort of pediatric CD patients, we aimed to assess the utility of serial fecal calprotectin measurements to detect intestinal inflammatory activity and predict disease relapse. METHODS In this prospective longitudinal cohort study, children with CD on infliximab therapy in clinical remission were included. Fecal calprotectin levels were assessed at baseline and at subsequent 2-5 visits. Clinical and biochemical disease activity were assessed using the Pediatric Crohn’s Disease Activity Index, C-reactive protein and erythrocyte sedimentation rate at baseline and at visits over the following 18 mo. RESULTS 53 children were included and eighteen patients (34%) had a clinical disease relapse during the study. Baseline fecal calprotectin levels were higher in patients that developed symptomatic relapse [median (interquartile range), relapse 723 μg/g (283-1758) vs 244 μg/g (61-627), P = 0.02]. Fecal calprotectin levels > 250 μg/g demonstrated good predictive accuracy of a clinical flare within 3 mo (area under the receiver operator curve was 0.86, 95% confidence limits 0.781 to 0.937). CONCLUSION Routine fecal calprotectin testing in children with CD in clinical remission is useful to predict relapse. Levels > 250 μg/g are a good predictor of relapse in the following 3 mo. This information is important to guide monitoring standards used in this population.

To describe the process of developing a craniosynostosis decision aid. We conducted a mixed-methods exploratory study between August 2019 and March 2020 to develop a decision aid about surgical treatment for single suture craniosynostosis. A single tertiary care academic children’s hospital. The decision aid development team consisted of surgeons, research fellows, a clinical nurse practitioner, clinical researchers with expertise in decision science, and a university-affiliated design school. Qualitative interviews (N = 5) were performed with families, clinicians (N = 2), and a helmeting orthotist to provide feedback on decision aid content, format, and usability. After cycles of revisions and iterations, 3 related decision aids were designed and approved by the marketing arm of our institution. Distinct booklets were created to enable focused discussion of treatment options for the 3 major types of single suture craniosynostosis (sagittal, metopic, unicoronal). Three decision aids representing the 3 most common forms of single suture craniosynostosis were developed. Clinicians found the decision aids could help facilitate discussions about families’ treatment preferences, goals, and concerns. We developed a customizable decision aid for single suture craniosynostosis treatment options. This tool lays the foundation for shared decision-making by assessing family preferences and providing clear, concise, and credible information regarding surgical treatment. Future research can evaluate this tool’s impact on patient–clinician discussions about families’ goals and preferences for treatment.

Autosomal recessive osteopetrosis has a variable presentation, most commonly including failure to thrive, hypocalcemia, seizures, hepatosplenomegaly, hydrocephalus, vision or hearing loss, and cytopenias. Multiple symptoms are usually seen at presentation. The variability of presentation often delays diagnosis and subsequent treatment. Here, we present a case of an infant with this condition who initially presented with triventricular hydrocephalus with Chiari I malformation. This alone is not a common presentation of this disease, and we present this case to highlight autosomal recessive osteopetrosis as a potential diagnosis in infants presenting with hydrocephalus and discuss the other associated symptoms, management, and prognosis of this condition.The patient was a full-term infant with a routine newborn period. At 6 months, the infant had macrocephaly and frontal bossing with a bulging fontanelle. She was found to have hydrocephalus with moderate ventriculomegaly involving the third and lateral ventricles with an associated Chiari 1 malformation. The infant was asymptomatic at the time. The infant was promptly referred to neurosurgery and underwent an uncomplicated ventriculoperitoneal shunt placement. Post-operative X-rays showed increased density of the skull with other bone changes suggestive of autosomal recessive osteopetrosis. Subsequent lab work and imaging studies were consistent with this condition. The diagnosis was confirmed by genetic testing, and the patient has undergone treatment with hematopoietic stem cell transplant.Hydrocephalus is a common feature of this condition, typically seen in conjunction with other systemic symptoms and laboratory findings. Our patient had a limited initial presentation of triventricular hydrocephalus with Chiari I malformation and was otherwise clinically asymptomatic. There is limited literature of such a presentation, and we highlight this case to increase awareness, as timely diagnosis of these patients is critical for treatment and future outcomes.

OBJECTIVES To evaluate quality of life (QoL) in a large cohort of pediatric patients with inflammatory bowel disease (IBD) and to identify the clinical factors that influence QoL. STUDY DESIGN This cross-sectional study analyzes a quality improvement initiative in 351 pediatric patients with IBD in British Columbia, Canada using the self-reported Pediatric Quality of Life Inventory (PedsQL) 4.0 generic scale. The questionnaire was completed at outpatient clinic and biologic infusion appointments. Statistical analysis included the t test, ANOVA, and multilinear regressions to evaluate the relationships between clinical factors and QoL. RESULTS Mean (SE) QoL scores (79.95 [0.84]) fell between previously described healthy and chronically ill populations. Disease activity was the most significant predictor of QoL, with patients in remission scoring similar (84.42 [0.87]) to well established healthy norms, and those with moderately or severely active disease having some of the lowest published PedsQL scores (63.13 [3.27]), lower than most other chronic pediatric conditions. Twenty-five patients with moderately or severely active disease at the time of survey completion had follow-up surveys identified 1 year later and had a significant improvement of both their disease activity (P

Background: Uveitis associated with juvenile idiopathic arthritis is a cause of major ocular morbidity. A substantial proportion of children are refractory to systemic methotrexate and TNF inhibitors. Our aim was to study the safety and efficacy of tocilizumab in children with juvenile idiopathic arthritis-associated uveitis refractory to both methotrexate and TNF inhibitors.Methods: This multicentre, single-arm, phase 2 trial was done following a Simon's two-stage design at seven tertiary hospital sites in the UK. Patients aged 2-18 years with active juvenile idiopathic arthritis-associated uveitis were eligible. All patients had been on a stable dose of methotrexate for at least 12 weeks and had not responded to treatment with a TNF inhibitor. Patients weighing 30 kg or more were treated with 162 mg subcutaneous tocilizumab every 2 weeks for 24 weeks, and participants weighing less than 30 kg were treated with 162 mg every 3 weeks for 24 weeks. The primary outcome was treatment response defined as a two-step decrease, or decrease to zero, from baseline in the level of inflammation (anterior chamber cells) at week 12, per the standardisation of uveitis nomenclature criteria. A phase 3 trial would be justified if more than seven patients responded to treatment. An interim analysis was planned to assess whether the trial would be stopped for futility, with futility defined as two or fewer treatment responses among ten participants. Adverse events were collected up to 30 calendar days after treatment cessation. The primary analysis was done in the intention-to-treat population and the safety analysis was done in all patients who started the treatment. This trial is registered with the International Standard Randomised Controlled Trial Number registry (ISRCTN95363507) and EU Clinical Trials Register (EudraCT 2015-001323-23).Findings: 22 participants were enrolled to the trial between Dec 3, 2015, and March 9, 2018, and 21 participants received treatment. One participant was found to be ineligible immediately after enrolment and was therefore withdrawn. Seven of 21 (median unbiased estimate of proportion 34% [95% CI 25-57]) responded to treatment (p=0·11). Safety results were consistent with the known safety profile of tocilizumab.Interpretation: The primary endpoint was not met, and thus the results do not support a phase 3 trial of tocilizumab in patients with juvenile idiopathic arthritis-associated uveitis. Importantly, data on the use of tocilizumab in clinical practice is now captured in national registries. Despite this trial not meeting the threshold required to justify a larger phase 3 trial, several patients responded to treatment; as such, tocilzumab might still be a therapeutic option in some children with uveitis refractory to anti-TNF drugs, given the absence of other treatment options.Funding: Versus Arthritis and the National Institute for Health Research Clinical Research Network: Children.

Fundamentals of nanoprecipitation process to form colloidal lignin particles (CLPs) from tetrahydrofuran (THF)-water solvent system were studied, and applied in establishment of a robust reactor design for scaled-up CLP production. Spherical lignin particles with an average diameter of 220 nm could be produced by the new reactor design. Evaporation was applied for removal of THF, concentration of the CLP dispersions, and finally for drying of the CLPs into flake like dry form. The dried CLPs could be re-dispersed in water to restore their colloidal form by applying short physical agitation. Salt triggered sedimentation of the particles was also investigated as a way for reducing the energy consumption related to water evaporation from the CLP dispersions. Aqueous thermal post-treatments were demonstrated to yield structural reinforcement of the CLP structure against solvation in various lignin solvents. In summary, the presented work pushes forward the conceptual design of large-scale CLP production, and addresses some of the foreseen technical challenges.

Surface modification of colloidal lignin particles (CLPs), which are obtained from renewable resources, is a plausible route towards novel biomaterials. Here we show that adsorption of cationic lignin onto spherical CLPs produces positively charged particles with tailored properties for the stabilization of Pickering emulsions. The threshold dosing of cationic lignin needed to achieve colloidally stable cationic dispersions was 4% relative to the dry weight of CLPs. Compared to irregular kraft lignin particles or regular CLPs, cationic CLPs stabilized a broader array of durable Pickering emulsions. This all-lignin adsorption process to prepare cationic CLPs is advantageous because it minimizes the consumption of synthetic polymers, and opens new application opportunities for structurally defined nano- and microscale lignin particles.

Waste is a complex societal problem and its management brings together various stakeholders. However, lack of sufficient information on the quantities and types of materials in the waste stream can make sustainable waste management difficult. Since waste in one sector can be valuable as a resource in another, there is a need to understand the distribution of materials within the resource stream, especially those that go to landfill. Current work is seeking to address this: whereas much material is already recycled, this is not the only management option and there remain several problematic materials and components that need to be removed from residual waste. This paper, the first of two case studies, presents a more comprehensive waste composition specification to improve the management of municipal waste. In developing the approach, waste composition specifications currently in use were reviewed and compared with the solid municipal waste collected at community recycling centres and from kerbsides. Key primary and secondary descriptors for the better management of resources arising from municipal waste were determined and the impact of these changes on the information arising from composition analysis is discussed.

Waste is intricate to manage. With greater attention now being paid to the use of waste as a resource, there is an increasing need to develop sustainable and secure management options for the most complex material resource streams. In order to achieve this, it is necessary to look to those materials, which owing to their complex structure have not traditionally been recycled, and so remain in the residual waste stream. This paper presents a methodology that has been developed to enable local authorities, or their equivalents, to assess the environmental and economic feasibility of collecting complex material streams separately, in order to improve their management of municipal solid waste. The methodology utilises available data from a number of sources, to determine the feasibility of options available. It is applied to a case study in Surrey, UK, in relation to the management of absorbent hygiene products. Currently this waste is collected as part of the residual stream, and dealt with either through landfill or energy from waste. The result of applying the new methodology suggests that the optimum solution is the separate collection of absorbent hygiene products and subsequent sterilisation and recycling.

In recent years, there has been an increased focus on sustainable, green alternatives with similar properties to conventional petroleum-based polymers. Poly(lactic acid) (PLA) is a biodegradable biopolymer which exhibits mild piezoelectric properties and has good processability which gives it potential for use in numerous existing and novel applications. The purpose of this study was to produce highly oriented and crystalline PLA electrospun fiber mats for piezoelectric applications. In order to yield a high piezoelectric constant, high crystallinity and fiber orientation are necessary. A two parallel collector set up was used to mechanically orient the fibers in the space between two copper electrodes. Voltage and feed rate were adjusted to produce smooth, oriented fibers with average diameters ranging 0.73–1.19 μm. Crystallinity and orientation were increased via hot drawing of the fiber mats and were maximized between 40 and 50 % and greater than 50 %, respectively.

This is the protocol for a review and there is no abstract. The objectives are as follows: To identify whether there are interventions, in the form of organisational changes to the way in which individuals with hypertension are cared for, that can contribute to the lowering of blood pressure and therefore are likely to reduce the adverse outcomes associated with this chronic condition.