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A European guideline on Robin Sequence was developed within the European Reference Network for rare and/ or complex craniofacial anomalies and ear, nose, and throat disorders. The guideline provides an overview of optimal care provisions for patients with Robin Sequence and recommendations for the improvement of care.

A European guideline on Robin Sequence was developed within the European Reference Network for rare and/or complex craniofacial anomalies and ear, nose, and throat disorders. The guideline provides an overview of optimal care provisions for patients with Robin Sequence and recommendations for the improvement of care.

Purpose: The purpose of this study is to evaluate all potential factors associated with laryngeal injury after endotracheal intubation in the pediatric population. Methods: A systematic literature search was conducted in Medline, Embase, Cochrane, web of science and Google scholar up to 20th of March 2023. We included all unique articles focusing on factors possibly associated with intubation-injury in pediatric patients. Two independent reviewers determined which articles were relevant by coming to a consensus, quality of evidence was rated using GRADE criteria. All articles were critically appraised according to the PRISMA guidelines. The articles were categorized in four outcome measures: post-extubation stridor, post-extubation upper airway obstruction (UAO) necessitating treatment, laryngeal injury found at laryngoscopy and a diagnosed laryngotracheal stenosis (LTS). Results: A total of 24 articles with a total of 15.520 patients were included. The incidence of post-extubation stridor varied between 1.0 and 30.3%, of post-extubation UAO necessitating treatment between 1.2 and 39.6%, of laryngeal injury found at laryngoscopy between 34.9 to 97.0% and of a diagnosed LTS between 0 and 11.1%. Although the literature is limited and quality of evidence very low, the level of sedation and gastro-esophageal reflux are the only confirmed associated factors with post-extubation laryngeal injury. The relation with age, weight, gender, duration of intubation, multiple intubations, traumatic intubation, tube size, absence of air leak and infection remain unresolved. The remaining factors are not associated with intubation injury. Conclusion: We clarify the role of the potential factors associated with laryngeal injury after endotracheal intubation in the pediatric population.

Background & aims: Critically ill children are at risk of micronutrient deficiencies, which might lead to poor clinical outcomes. However, the interpretation of micronutrient concentrations in plasma is complicated due to age-dependent and critical illness-dependent changes. Certain red blood cell (RBC) concentrations might reflect the overall body status more reliably than plasma levels in the presence of systemic inflammatory response. This study longitudinally examined micronutrient concentrations in both plasma and RBC in critically ill children. Methods: This secondary analysis of the PEPaNIC RCT investigated the impact of early versus late initiation of parenteral macronutrient supplementation in critically ill children. All children received micronutrients when EN was insufficient (<80 % energy requirements). Blood samples were obtained on days 1, 3, 5 and 7 of Paediatric Intensive Care Unit (PICU) admission. Inductively coupled plasma mass spectrometry was used to measure zinc, selenium, and copper in plasma and selenium, copper, and magnesium in RBCs. Plasma magnesium was measured with colorimetric detection. Micronutrient concentrations were compared with age-specific reference values in healthy children and expressed using Z-scores. Changes in micronutrient concentrations over time were examined using the Friedman and post hoc Wilcoxon signed-rank tests. Results:For 67 critically ill children, median (Q1; Q3) age 9.5 (5.5; 13.2) years, PIM3 score −2.3 (−3.1; −0.8), samples were available at various time points during their PICU stay. For 22 patients, longitudinal samples were available. On day 1, the median plasma Z-score for zinc was −5.2 (−5.2; −2.9), copper −1.6 (−2.9; −0.2), selenium −2.6 (−3.8; −1.0), magnesium −0.2 (−1.6; 1.3), and median RBC Z-score for copper was 0.5 (−0.1; 1.3), selenium −0.3 (−1.1; 0.7), magnesium 0.2 (−0.4; 1.3). In the longitudinal analysis, plasma zinc was sign

Neonates and infants surviving critical illness show impaired growth during critical illness and are at risk for later neuropsychological impairments. Early identification of individuals most at risk is needed to provide tailored long-term follow-up and care. The research question is whether early growth during hospitalization is associated with growth and neuropsychological outcomes in neonates and infants after pediatric intensive care unit admission (PICU). This is a secondary analysis of the PEPaNIC trial. Weight measurements upon PICU admission, at PICU discharge, at hospital discharge, at 2- and 4-year follow-up, and of different subgroups were compared using (paired) t-tests. Multiple linear regression analyses were performed to investigate the association between early growth in weight measures and neuropsychological outcomes at 4-year follow-up. One hundred twenty-one infants were included, and median age upon admission was 21 days. Growth in weight per week was less than the age-appropriate norm, resulting in a decrease in weight-for-age Z-score during hospitalization. Weight is normalized at 2- and 4-year follow-up. Weight gain in kilograms per week and change in weight Z-score were not associated with neurodevelopmental outcome measures at 4-year follow-up. Lower weight-for-age Z-score at PICU admission and at hospital discharge was associated only with lower weight and height Z-scores at 4-year follow-up. Conclusion: Growth in weight during hospital stay of young survivors of critical illness is impaired. Worse early growth in weight is associated with lower weight and height but not with neuropsychological outcomes at 4-year follow-up. (Table presented.)

In deze uitgave wordt voor het vierde jaar op rij de balans opgemaakt over hoe het gesteld is met amfibieën, reptielen en vissen in Nederland. Veel soorten hebben het onverminderd moeilijk. Van specialistische soorten, zoals de kwabaal, weten we dat ze ernstig onder druk staan en zonder herstelmaatregelen dreigen te verdwijnen uit Nederland. In deze balans is aandacht besteed aan het onderzoek naar grootschalig natuurherstel voor geschikte leefomstandigheden ten gunste van deze mysterieuze vis.

This report documents key insights arising from a strategic expert consultation and learning event organisedi n the Netherlands from 19-23 September 2022. During the event experiences, good practices and emerging lessons stemming from the implementation of FNS-REPRO, various Horn of Africa NUFFIC programmes and other initiatives on building food systems resilience, as well as key insights from other experts and practitioners, were shared and elaborated upon

Deze RAVON-balans toont hoe het onze amfibieën, reptielen en vissen vergaat en geeft soms ook verklaringen van waargenomen trends. Het is heel duidelijk dat veel soorten het nog altijd moeilijk hebben, maar er zijn gelukkig ook winnaars en lichtpuntjes.

Background & aims: Pediatric intensive care nutrition is a growing research field on the intersection of three large research domains: Pediatrics, Critical Care Medicine and Nutrition & Dietetics. This study, using Research Intelligence tools, such as bibliometric network visualization software, was designed to map the developments in research topics and collaboration in the field over the past thirty years, and discuss how these developments align with recent recommendations and guidelines of the active expert groups in the field. Methods: We searched the Web of Science Core Collection for relevant full articles, reviews, letters and proceedings papers. To describe the research field a search strategy was iteratively designed based on the combinations of relevant key words. The articles the were found were processed using software designed for bibliometric network mapping. Filters were applied to select only the most relevant articles for the field. Results: The resulting visualizations show the network of researchers active in the field of pediatric intensive care nutrition, and the collaborations between them. Using the most frequently used key terms a map was created to show the most prominent research areas within the field, and the development of attention for these topics over time. Conclusions: The network analyses show a research field that is gaining momentum, with several cores of research activity in different institutions. Some research groups collaborate on specific topics within the field, while others seem to be more isolated. The analyses uncover the potential for future collaborations and emerging topics of attention in the different areas of research in the field. The results are compared to recent recommendations for research priorities by active networks in the field. We discuss similarities and discrepancies.

Background & aims: Hypophosphatemia during critical illness has been associated with adverse outcome. The reintroduction of enteral or parenteral nutrition, leading to refeeding hypophosphatemia (RFH), has been presented as potential risk factor. We investigated the occurrence of early RFH, its association with clinical outcome, and the impact of early parenteral nutrition (PN) on the development of early RFH in pediatric critical illness. Methods: This is a secondary analysis of the PEPaNIC randomized controlled trial (N = 1440), which showed that withholding supplemental parenteral nutrition (PN) for 1 week (late-PN) in the pediatric intensive care unit (PICU) accelerated recovery and reduced new infections compared to early-PN (<24 h). Patients with renal replacement therapy or unavailable phosphate concentrations were excluded from this analysis. Early RFH was defined as serum/plasma phosphate <0.65 mmol/L and a drop of >0.16 mmol/L within 3 days of admission to the PICU. The association between baseline characteristics and early RFH, and the association of early RFH with clinical outcome were investigated using logistic and linear regression models, both uncorrected and corrected for possible confounders. To examine the impact of nutritional intake on phosphate concentrations, structural nested mean models with propensity score and censoring models were used. Results: A total of 1247 patients were eligible (618 early-PN, 629 late-PN). Early RFH occurred in 40 patients (3%) in total, significantly more in the early-PN group (n = 31, within-group occurrence 5%) than in the late-PN-group (n = 9, within-group occurrence 1%, p < 0.001). Patients who were older (OR 1.14 (95% CI 1.08; 1.21) per year added, p < 0.001) and who had a higher Pediatric Risk of Mortality (PIM3) score had a higher risk of developing early RFH (OR 1.36 (95% CI 1.15; 1.59) per unit added, p < 0.001), whereas patients in the late-PN group had a lower risk of early RFH (

Given the high prevalence of OSA in children with syndromic and complex craniosynostosis (SCC) and the consequences of untreated OSA, it is important to assess their nutritional status and growth. Yet, literature regarding growth in children with SCC remain scarce. Therefore, this study aimed to (1) illustrate the growth pattern in SCC, (2) determine the impact of OSA on this growth pattern, and (3) evaluate the effect of surgical treatment of OSA on growth over time. A retrospective study was performed in children with SCC, who were treated at the Dutch Craniofacial Center (Rotterdam, Netherlands). Growth variables (height, weight, weight-for-age standard-deviation-score (SDS), weight-for-height SDS, and height-for-age SDS) and degree of OSA (obstructive apnea-hypopnea index) were assessed. Of the 153 children with SCC, 38 (25%) were acutely malnourished at some point during follow-up, of whom 21 had disease-related acute malnutrition. Children with moderate-severe OSA had significant lower weightfor-height SDS compared to children without OSA (p= 0.0063). Growth parameters (weight-for-age SDS, weight-for-height SDS, height-for-age SDS) in children with SCC without OSA were not impaired as they did not differ from the normal healthy population, with exception of the patients with Saethre-Chotzen syndrome (SCS) who had a significantly lower SDS for height-for-age.Conclusion: Children with SCC have a substantial chance of developing acute malnutrition at some point during growth. Additionally, in children with moderate-severe OSA, a significant lower SDS for weight-for-height is present, indicating the importance of assessing the weight and growth pattern in children who are clinically suspected for OSA.

Patients with mandibular hypoplasia and upper airway obstruction are at an increased risk of feeding and swallowing difficulties. Little has been described regarding these outcomes following mandibular distraction. The aim of this study was to evaluate the effect of mandibular distraction on feeding and swallowing function. A retrospective study was performed on 22 patients with non-isolated mandibular hypoplasia and severe upper airway obstruction treated with mandibular distraction. Median age at first mandibular distraction was 3.1 years (interquartile range 2.3–6.0 years) and the median follow-up time was 3.5 years (interquartile range 2.0–9.4 years). Prior to mandibular distraction, feeding difficulties were present in 18 patients. Swallowing difficulties were present in 20 patients, all of whom had problems in the oral phase of swallowing, while 11 patients had additional problems in the pharyngeal phase. Following mandibular distraction, at the time of follow-up, feeding difficulties persisted in 13 patients. Swallowing difficulties in the oral phase remained present in all 20 patients, while pharyngeal phase problems persisted in seven patients. In conclusion, feeding and swallowing difficulties are highly prevalent in non-isolated patients and often persist following mandibular distraction. Moreover, these can be the reason that decannulation cannot be accomplished. Hence, awareness and close follow-up by a specialized speech therapist is of paramount importance.

This report provides insights and lessons generated from different (internal and external) sources and critically reflected upon during a sensemaking workshop in South-Sudan in June 2022. This workshop is part of a series of sensemaking workshops, that aim to support evidence-based and adaptive programming for the Food and Nutrition Security Resilience Program (FNS-REPRO) in Somaliland, Sudan and South Sudan. This program aims to contribute to food and nutrition security and resilient livelihoods in protracted crises, by paying attention to natural resource management (NRM), value chain development (fodder in Somaliland,gum Arabic in Sudan, and seed in South Sudan), and production and consumption of nutritious food. Key partners in the program are FAO and WUR

Background & aims: The Remote Malnutrition Application (R-MAPP) was developed during the COVID-19 pandemic to provide support for health care professionals (HCPs) working in the community to complete remote nutritional assessments, and provide practical guidance for nutritional care. The aim of this study was to modify the R-MAPP into a version suitable for children, Pediatric Remote Malnutrition Application (Pedi-R-MAPP), and provide a structured approach to completing a nutrition focused assessment as part of a technology enabled care service (TECS) consultation. Methods: A ten-step process was completed: 1) permission to modify adult R-MAPP, 2) literature search to inform the Pedi-R-MAPP content, 3) Pedi-R-MAPP draft, 4) international survey of HCP practice using TECS, 5) nutrition experts invited to participate in a modified Delphi process, 6) first stakeholder meeting to agree purpose/draft of the tool, 7) round-one online survey, 8) statements with consensus removed from survey, 9) round-two online survey for statements with no consensus and 10) second stakeholder meeting with finalisation of the Pedi-R-MAPP nutrition awareness tool. Results: The international survey completed by 463 HCPs, 55% paediatricians, 38% dietitians, 7% nurses/others. When HCPs were asked to look back over the last 12 months, dietitians (n = 110) reported that 5.7 ± 10.6 out of every 10 appointments were completed in person; compared to paediatricians (n = 182) who reported 7.5 ± 7.0 out of every 10 appointments to be in person (p < 0.0001), with the remainder completed as TECS consultations. Overall, 74 articles were identified and used to develop the Pedi-R-MAPP which included colour-coded advice using a traffic light system; green, amber, red and purple. Eighteen participants agreed to participate in the Delphi consensus and completed both rounds of the modified Delphi survey. Agreement was reached at the first meeting on the purpose and draft sections of the proposed tool

Study objectives: Unobtrusive monitoring of sleep and sleep disorders in children presents challenges. We investigated the possibility of using Ultra-Wide band (UWB) radar to measure sleep in children. Methods: Thirty-two children scheduled to undergo a clinical polysomnography participated; their ages ranged from 2 months to 14 years. During the polysomnography, the children's body movements and breathing rate were measured by an UWB-radar. A total of 38 features were calculated from the motion signals and breathing rate obtained from the raw radar signals. Adaptive boosting was used as machine learning classifier to estimate sleep stages, with polysomnography as gold standard method for comparison. Results: Data of all participants combined, this study achieved a Cohen's Kappa coefficient of 0.67 and an overall accuracy of 89.8% for wake and sleep classification, a Kappa of 0.47 and an accuracy of 72.9% for wake, rapid-eye-movement (REM) sleep, and non-REM sleep classification, and a Kappa of 0.43 and an accuracy of 58.0% for wake, REM sleep, light sleep and deep sleep classification. Conclusion: Although the current performance is not sufficient for clinical use yet, UWB radar is a promising method for non-contact sleep analysis in children., Mechanical, Maritime and Materials Engineering, Bio-Electronics

There are reports of children COVID-19 or COVID-19 like symptoms with hyperinflammatory multisystem syndrome, ARDS, gastrointestinal and atypical Kawasaki disease presenting to PICU worldwide temporally associated with COVID-19, for which there are important nutrition support considerations. As a result, the European Society of Pediatric and Neonatal Intensive Care – Metabolism, Endocrine and Nutrition group (ESPNIC-MEN) and paediatric nutritionists working in PICUs are being consulted regarding nutrition management of critically ill children with COVID-19 or COVID-19 like symptoms. Therefore, the aim of this short report is to provide a summary of nutrition support recommendations for critically ill children with COVID-19. They are based on the ESPNIC-MEN section recommendations published in January 2020 and surviving sepsis recommendations from February 2020.

Background & aims: In the absence of methodologically sound randomized controlled trials (RCTs), current recommendations for timing and amount of enteral nutrition (EN) in critically ill children are based on observational studies. These studies have associated achievement of a higher EN intake in critically ill children with improved outcome. Inherent to the observational design of these underlying studies, thorough insight in possible confounding factors to correct for is essential. We evaluated the associations between EN intake and 1) patient and daily clinical characteristics and 2) clinical outcomes adjusted for these patient and clinical characteristics during the first week of critical illness with a multivariable mixed model. Methods: This secondary analysis of the multicentre PEPaNIC RCT investigated a subgroup of critically ill children with daily prospectively recorded gastrointestinal symptoms and EN intake during the first week with multivariable analyses using two-part mixed effect models, including multiple testing corrections using Holm's method. These models combined a mixed-effects logistic regression for the dichotomous outcome EN versus no EN, and a linear mixed-effects model for the patients who received any EN intake. EN intake per patient was expressed as mean daily EN as % of predicted resting energy expenditure (% of EN/REE). Model 1 included 40 fixed effect baseline patient characteristics, and daily parameters of illness severity, feeding, medication and gastrointestinal symptoms. Model 2 included these patient and daily variables as well as clinical outcomes. Results: Complete data were available for 690 children. EN was provided in 503 (73%) patients with a start after a median of 2 (IQR 2–3) days and a median % of EN/REE of 38.8 (IQR 14.1–79.5) over the first week. Multivariable mixed model analyses including all patients showed that admission after gastrointestinal surgery (−49%EN/REE; p = 0.002), gastric feeding (−31% EN/REE

Background & aims: Different metabolic phases can be distinguished in critical illness, which influences nutritional treatment. Achieving optimal nutritional treatment during these phases in critically ill patients is challenging. COVID-19 patients seem particularly difficult to feed due to gastrointestinal problems. Our aim was to describe measured resting energy expenditure (mREE) and feeding practices and tolerance during the acute and late phases of critical illness in COVID-19 patients. Methods: Observational study including critically ill mechanically ventilated adult COVID-19 patients. Indirect calorimetry (Q-NRG+, Cosmed) was used to determine mREE during the acute (day 0–7) and late phase (>day 7) of critical illness. Data on nutritional intake, feeding tolerance and urinary nitrogen loss were collected simultaneously. A paired sample t-test was performed for mREE in both phases. Results: We enrolled 21 patients with a median age of 59 years [44–66], 67% male and median BMI of 31.5 kg/m2 [25.7–37.8]. Patients were predominantly fed with EN in both phases. No significant difference in mREE was observed between phases (p = 0.529). Sixty-five percent of the patients were hypermetabolic in both phases. Median delivery of energy as percentage of mREE was higher in the late phase (94%) compared to the acute phase (70%) (p = 0.001). Urinary nitrogen losses were significant higher in the late phase (p = 0.003). Conclusion: In both the acute and late phase, the majority of the patients were hypermetabolic and fed enterally. In the acute phase patients were fed hypocaloric whereas in the late phase this was almost normocaloric, conform ESPEN guidelines. No significant difference in mREE was observed between phases. Hypermetabolism in both phases in conjunction with an increasing loss of urinary nitrogen may indicate that COVID-19 patients remain in a prolonged acute, catabolic phase.

Study objectives: Unobtrusive monitoring of sleep and sleep disorders in children presents challenges. We investigated the possibility of using Ultra-Wide band (UWB) radar to measure sleep in children. Methods: Thirty-two children scheduled to undergo a clinical polysomnography participated; their ages ranged from 2 months to 14 years. During the polysomnography, the children's body movements and breathing rate were measured by an UWB-radar. A total of 38 features were calculated from the motion signals and breathing rate obtained from the raw radar signals. Adaptive boosting was used as machine learning classifier to estimate sleep stages, with polysomnography as gold standard method for comparison. Results: Data of all participants combined, this study achieved a Cohen's Kappa coefficient of 0.67 and an overall accuracy of 89.8% for wake and sleep classification, a Kappa of 0.47 and an accuracy of 72.9% for wake, rapid-eye-movement (REM) sleep, and non-REM sleep classification, and a Kappa of 0.43 and an accuracy of 58.0% for wake, REM sleep, light sleep and deep sleep classification. Conclusion: Although the current performance is not sufficient for clinical use yet, UWB radar is a promising method for non-contact sleep analysis in children.

Objective: Indications for tracheostomy have changed over the last decades and clinical outcome varies depending on the indication for tracheostomy. By gaining more insight in the characteristics and outcome of the tracheostomized pediatric population, clinical care can be improved and a better individual prognosis can be given. Therefore, we studied the outcome of our pediatric tracheostomy population in relation to the primary indication over the last 16 years. Methods: We retrospectively included children younger than 18 years of age with a tracheostomy tube in the Erasmus Medical Center, Sophia children's hospital. The primary indication for tracheostomy, gender, age at tracheostomy, age at decannulation, comorbidity, mortality, closure of a persisting tracheocutaneous fistula after decannulation, surgery prior to decannulation and the use of polysomnography were recorded and analyzed. Results: Our research group consisted of 225 children. Reasons for a tracheostomy were first divided in two major diagnostic groups: 1) airway obstruction group (subgroups: laryngotracheal obstruction and craniofacial anomalies) and 2) pulmonary support group (subgroups: cardio-pulmonary diseases and neurological diseases). Children in the airway obstruction group were younger when receiving a tracheostomy (3.0 months vs. 31.0 months, p < 0.05), they were tracheostomy dependent for a longer time (median 21.5 months vs. 2.0 months, p < 0.05) and they required surgery more often (74.5% vs. 8.3%, p < 0.05) than the children in the pulmonary support group. The decannulation rate of children with a laryngotracheal obstruction is high (74.8%), but low in all other subgroups (craniofacial anomalies; 38.5%, cardio-pulmonary diseases; 34.6% and neurological diseases; 52.9%). Significantly more children (36.7%) died in the pulmonary support group due to underlying comorbidity, mainly in the cardio-pulmonary diseases subgroup. Surgery for a persisting tracheocutaneous fistula was

U heeft de allereerste RAVON-balans in handen. Hierin geven we aan hoe het is gesteld met amfibieën, reptielen en vissen in Nederland. Gaan ze vooruit, achteruit en waarom dan wel?