Variation in follow-up for children born very preterm in Europe.

BACKGROUND: Children born very preterm (<32 weeks of gestation) face high risks of neurodevelopmental and health difficulties compared with children born at term. Follow-up after discharge from the neonatal intensive care unit is essential to ensure early detection and intervention, but data on policy approaches are sparse. METHODS: We investigated the characteristics of follow-up policy and programmes in 11 European countries from 2011 to 2022 using healthcare informant questionnaires and the published/grey literature. We further explored how one aspect of follow-up, its recommended duration, may be reflected in the percent of parents reporting that their children are receiving follow-up services at 5 years of age in these countries using data from an area-based cohort of very preterm births in 2011/12 (N = 3635). RESULTS: Between 2011/12 and 22, the number of countries with follow-up policies or programmes increased from 6 to 11. The policies and programmes were heterogeneous in eligibility criteria, duration and content. In countries that recommended longer follow-up, parent-reported follow-up rates at 5 years of age were higher, especially among the highest risk children, born <28 weeks' gestation or with birthweight <1000 g: between 42.1% and 70.1%, vs. <20% in most countries without recommendations. CONCLUSIONS: Large variations exist in follow-up policies and programmes for children born very preterm in Europe; differences in recommended duration translate into cross-country disparities in reported follow-up at 5 years of age.

Prediction of movement difficulties at 5 years from parent report at 2 years in children born extremely preterm.

AIM: To assess the predictive validity of parent-reported gross motor impairment (GMI) at age 2 years to detect significant movement difficulties at age 5 years in children born extremely preterm. METHOD: Data were from 556 children (270 males, 286 females) born at less than 28 weeks' gestation in 2011 to 2012 in 10 European countries. Parent report of moderate/severe GMI was defined as walking unsteadily or unable to walk unassisted at 2 years corrected age. Examiners assessed significant movement difficulties (score ≤ 5th centile on the Movement Assessment Battery for Children, Second Edition) and diagnoses of cerebral palsy (CP) were collected by parent report at 5 years chronological age. RESULTS: At 2 years, 66 (11.9%) children had moderate/severe GMI. At 5 years, 212 (38.1%) had significant movement difficulties. Parent reports of GMI at age 2 years accurately classified CP at age 5 years in 91.0% to 93.2% of children. Classification of moderate/severe GMI at age 2 years had high specificity (96.2%; 95% confidence interval 93.6-98.0) and positive predictive value (80.3%; 68.7-89.1) for significant movement difficulties at age 5 years. However, 74.5% of children with significant movement difficulties at 5 years were not identified with moderate/severe GMI at age 2 years, resulting in low sensitivity (25.1%; 19.4-31.5). INTERPRETATION: This questionnaire may be used to identify children born extremely preterm who at age 2 years have a diagnosis of CP or movement difficulties that are likely to have a significant impact on their functional outcomes at age 5 years.

Developmental motor problems and health-related quality of life in 5-year-old children born extremely preterm: A European cohort study.

AIM: To measure the association between cerebral palsy (CP) and non-CP-related movement difficulties and health-related quality of life (HRQoL) among 5-year-old children born extremely preterm (<28 weeks gestational age). METHOD: We included 5-year-old children from a multi-country, population-based cohort of children born extremely preterm in 2011 to 2012 in 11 European countries (n = 1021). Children without CP were classified using the Movement Assessment Battery for Children, Second Edition as having significant movement difficulties (≤5th centile of standardized norms) or being at risk of movement difficulties (6th-15th centile). Parents reported on a clinical CP diagnosis and HRQoL using the Pediatric Quality of Life Inventory. Associations were assessed using linear and quantile regressions. RESULTS: Compared to children without movement difficulties, children at risk of movement difficulties, with significant movement difficulties, and CP had lower adjusted HRQoL total scores (ß [95% confidence interval] = -5.0 [-7.7 to -2.3], -9.1 [-12.0 to -6.1], and - 26.1 [-31.0 to -21.2]). Quantile regression analyses showed similar decreases in HRQoL for all children with CP, whereas for children with non-CP-related movement difficulties, reductions in HRQoL were more pronounced at lower centiles. INTERPRETATION: CP and non-CP-related movement difficulties were associated with lower HRQoL, even for children with less severe difficulties. Heterogeneous associations for non-CP-related movement difficulties raise questions for research about mitigating and protective factors.

Postnatal growth restriction and neurodevelopment at 5 years of age: a European extremely preterm birth cohort study.

OBJECTIVE: To investigate whether extrauterine growth restriction (EUGR) during the neonatal hospitalisation by sex among extremely preterm (EPT) infants is associated with cerebral palsy (CP) and cognitive and motor abilities at 5 years of age. STUDY DESIGN: Population-based cohort of births <28 weeks of gestation with data from obstetric and neonatal records and parental questionnaires and clinical assessments at 5 years of age. SETTING: 11 European countries. PATIENTS: 957 EPT infants born in 2011-2012. MAIN OUTCOMES: EUGR at discharge from the neonatal unit was defined as (1) the difference between Z-scores at birth and discharge with <-2 SD as severe, -2 to -1 SD as moderate using Fenton's growth charts (Fenton) and (2) average weight-gain velocity using Patel's formula in grams (g) per kilogram per day (Patel) with <11.2 g (first quartile) as severe, 11.2-12.5 g (median) as moderate. Five-year outcomes were: a CP diagnosis, intelligence quotient (IQ) using the Wechsler Preschool and Primary Scales of Intelligence tests and motor function using the Movement Assessment Battery for Children, second edition. RESULTS: 40.1% and 33.9% children were classified as having moderate and severe EUGR, respectively, by Fenton and 23.8% and 26.3% by Patel. Among children without CP, those with severe EUGR had lower IQ than children without EUGR (-3.9 points, 95% Confidence Interval (CI)=-7.2 to -0.6 for Fenton and -5.0 points, 95% CI=-8.2 to -1.8 for Patel), with no interaction by sex. No significant associations were observed between motor function and CP. CONCLUSIONS: Severe EUGR among EPT infants was associated with decreased IQ at 5 years of age.

Effect of systemic hydrocortisone in ventilated preterm infants on parent-reported behavioural outcomes at 2 years' corrected age: follow-up of a randomised clinical trial.

OBJECTIVE: To report the parent-reported behavioural outcomes of infants included in the Systemic Hydrocortisone To Prevent Bronchopulmonary Dysplasia in preterm infants study at 2 years' corrected age (CA). DESIGN: Randomised placebo-controlled trial. SETTING: Dutch and Belgian neonatal intensive care units. PATIENTS: Infants born <30 weeks' gestation and/or birth weight <1250 g, and ventilator dependent in the second week of life. INTERVENTION: Infants were randomly assigned to a 22-day course of systemic hydrocortisone (cumulative dose 72.5 mg/kg; n=182) or placebo (n=190). MAIN OUTCOME MEASURES: Parent-reported behavioural outcomes at 2 years' CA assessed with the Child Behavior Checklist (CBCL 1½-5). RESULTS: Parents completed the CBCL of 183 (70% (183/262)) infants (hydrocortisone group, n=96; placebo group, n=87). Multiple imputation was used to account for missing data. Infants with critically elevated T-scores (>55) were found in 22.9%, 19.1% and 29.4% of infants for total, internalising and externalising problems, respectively; these scores were not significantly different between groups (mean difference -1.52 (95% CI -4.00 to 0.96), -2.40 (95% CI -4.99 to 0.20) and -0.81 (95% CI -3.40 to 1.77), respectively). In the subscales, we found a significantly lower T-score for anxiety problems in the hydrocortisone group (mean difference -1.26, 95% CI -2.41 to -0.12). CONCLUSION: This study found high rates of behaviour problems at 2 years' CA following very preterm birth, but these problems were not associated with hydrocortisone treatment initiated between 7 and 14 days after birth in ventilated preterm infants. TRIAL REGISTRATION NUMBER: NTR2768; EudraCT 2010-023777-19.

Short-term pulmonary and systemic effects of hydrocortisone initiated 7-14 days after birth in ventilated very preterm infants: a secondary analysis of a randomised controlled trial.

OBJECTIVE: Observational studies in preterm infants suggest that systemic hydrocortisone improves pulmonary condition but may also lead to systemic adverse effects. We report the short-term pulmonary and systemic effects of hydrocortisone initiated in the second week. DESIGN: Randomised placebo-controlled trial. SETTING: Dutch and Belgian neonatal intensive care units. PATIENTS: Infants born <30 weeks' gestation and/or birth weight <1250 g, and ventilator dependent in the second week of life. INTERVENTION: Infants were randomly assigned to a 22-day course of systemic hydrocortisone (cumulative dose 72.5 mg/kg; n=182) or placebo (n=190). MAIN OUTCOME MEASURES: Data on extubation, ventilator settings, glucose levels, and blood pressure were recorded daily and analysed during the first 7 days of treatment using linear mixed-effects models. RESULTS: Infants in the hydrocortisone group (24.3%) failed extubation less often compared with placebo (38.6%, crude risk difference: -14.3% (95% CI: -23.4% to -4.8%)). The estimated difference in daily rate of change between hydrocortisone and placebo was -0.42 cmH2O (95% CI: -0.48 to -0.36) for mean airway pressure, -0.02 (95% CI: -0.02 to -0.01) for fraction of inspired oxygen, -0.37 (95% CI: -0.44 to -0.30) for respiratory index, 0.14 mmol/L (95% CI: 0.08 to 0.21) for blood glucose levels and 0.83 mm Hg (95% CI: 0.58 to 1.09) for mean blood pressure. CONCLUSIONS: Systemic hydrocortisone initiated between 7 and 14 days after birth in ventilated preterm infants improves pulmonary condition, thereby facilitating weaning and extubation from invasive ventilation. The effects of hydrocortisone on blood glucose levels and blood pressure were mild and of limited clinical relevance. TRIAL REGISTRATION NUMBER: Netherlands Trial Register (NTR2768; https://www.trialregister.nl/trial/2640) and European Union Clinical Trials Register (EudraCT, 2010-023777-19).

Glomerular filtration rate in critically ill neonates and children: creatinine-based estimations versus iohexol-based measurements.

BACKGROUND: Acute kidney injury (AKI) and augmented renal clearance (ARC), both alterations of the glomerular filtration rate (GFR), are prevalent in critically ill children and neonates. AKI and ARC prevalence estimates are based on estimation of GFR (eGFR) using serum creatinine (SCr), which is known to be inaccurate. We aimed to test our hypothesis that AKI prevalence will be higher and ARC prevalence will be lower in critically ill children when using iohexol-based measured GFR (mGFR), rather than using eGFR. Additionally, we aimed to investigate the performance of different SCr-based eGFR methods. METHODS: In this single-center prospective study, critically ill term-born neonates and children were included. mGFR was calculated using a plasma disappearance curve after parenteral administration of iohexol. AKI diagnosis was based on the KDIGO criteria, SCr-based eGFR, and creatinine clearance (CrCL). Differences between eGFR and mGFR were determined using Wilcoxon signed-rank tests and by calculating bias and accuracy (percentage of eGFR values within 30% of mGFR values). RESULTS: One hundred five children, including 43 neonates, were included. AKI prevalence was higher based on mGFR (48%), than with KDIGO or eGFR (11-40%). ARC prevalence was lower with mGFR (24%) compared to eGFR (38-51%). eGFR equations significantly overestimated mGFR (60-71 versus 41 ml/min/1.73 m2, p < 0.001-0.002). Accuracy was highest with eGFR equations based on age- and sex-dependent equations (up to 59%). CONCLUSION: Iohexol-based AKI prevalence was higher and ARC prevalence lower compared to standard SCr-based eGFR methods. Age- and sex-dependent equations for eGFR (eGFR-Smeets for neonates and eGFR-Pierce for children) best approached measured GFR and should preferably be used to optimize diagnosis of AKI and ARC in this population. A higher resolution version of the Graphical abstract is available as Supplementary information.

Mode of Delivery and Incidence of Bronchopulmonary Dysplasia: Results from the Population-Based EPICE Cohort.

INTRODUCTION: Bronchopulmonary dysplasia (BPD) represents a tremendous disease burden following preterm birth. The strong association between compromised gas exchange after birth and BPD demands particular focus on the perinatal period. The mode of delivery can impact on lung fluid clearance and microbial colonization, but its impact on BPD and potential trade-off effects between death and BPD are not established. METHODS: A total of 7,435 live births (24+0 to 31+6 weeks postmenstrual age) in 19 regions of 11 European countries were included. Principal outcomes were death and BPD at 36 weeks. We estimated unadjusted and adjusted associations with mode of delivery using multilevel logistic regression to account for clustering within units and regions. Sensitivity analyses examined effects, taking into consideration regional variations in C-section rates. RESULTS: Compared to vaginal delivery, delivery by C-section was not associated with the incidence of BPD (OR 0.92, 95% CI: 0.68-1.25) or the composite outcome of death or BPD (OR 0.94, 95% CI: 0.74-1.19) after adjustment for perinatal and neonatal risk factors in the total cohort and in pregnancies for whom a vaginal delivery could be considered. Sensitivity analyses among singletons, infants in cephalic presentation, and infants of ≥26+0 weeks of gestation did not alter the results for BPD, severe BPD, and death or BPD, even in regions with a high C-section rate. CONCLUSIONS: In our population-based cohort study, the mode of delivery was not associated with the incidence of BPD. The intention to reduce BPD does not justify a C-section in pregnancies where a vaginal delivery can be considered.

Movement Difficulties at Age Five Among Extremely Preterm Infants.

BACKGROUND AND OBJECTIVES: Children born extremely preterm (EPT), <28 weeks' gestational age, face higher risks of movement difficulties than their term-born peers. Studies report varying prevalence estimates and prognostic factors identifying children who could benefit from early intervention are inconsistent. This study investigated the prevalence of movement difficulties in children born EPT and associated risk factors. METHODS: Data come from a population-based EPT birth cohort in 2011 and 2012 in 11 European countries. Children without cerebral palsy were assessed at 5 years of age (N = 772) with the Movement Assessment Battery for Children-Second Edition, which classifies movement difficulties as none (>15th percentile), at risk (6th-15th percentile) and significant (≤5th percentile). Associations with sociodemographic, perinatal, and neonatal characteristics collected from obstetric and neonatal medical records and parental questionnaires were estimated using multinomial logistic regression. RESULTS: We found 23.2% (n = 179) of children were at risk for movement difficulties and 31.7% (n = 244) had significant movement difficulties. Lower gestational age, severe brain lesions, and receipt of postnatal corticosteroids were associated with significant movement difficulties, whereas male sex and bronchopulmonary dysplasia were associated with being at risk and having significant movement difficulties. Children with younger, primiparous, less educated, and non-European-born mothers were more likely to have significant movement difficulties. Differences in prevalence between countries remained after population case-mix adjustments. CONCLUSIONS: This study confirms a high prevalence of movement difficulties among EPT children without cerebral palsy, which are associated with perinatal and neonatal risk factors as well as sociodemographic characteristics and country.

Motor-related health care for 5-year-old children born extremely preterm with movement impairments.

AIM: To (1) determine the proportion of 5-year-old children born extremely preterm (EPT) with movement difficulties including cerebral palsy (CP) and the proportion of these children receiving motor-related health care (MRHC), and (2) describe factors associated with receiving MRHC. METHOD: Children born before 28 weeks' gestation in 2011 to 2012 in 11 European countries were assessed with the Movement Assessment Battery for Children, Second Edition (MABC-2) at 5 years of age. Information on family characteristics, child health including CP diagnosis, and health care use were collected using parent-report questionnaires. MRHC was defined as visits in the previous year with health care providers (physical and occupational therapists) specialized in assessing/treating motor problems. We analysed receipt of MRHC and associated factors among children at risk of movement difficulties (MABC-2 score 6th-15th centiles), with significant movement difficulties (SMD; ≤5th centile) or with CP. RESULTS: Of 807 children assessed at 5 years 7 months (SD 4 months; 4 years 7 months-7 years 1 month), 412 were males (51.1%), 170 (21.1%) were at risk of movement difficulties, 201 (24.9%) had SMD, and 92 (11.4%) had CP. Those who received MRHC comprised 89.1% of children with CP, 42.8% with SMD, and 25.9% at risk of movement difficulties. MRHC for children with SMD varied from 23.3% to 66.7% between countries. Children were more likely to receive MRHC if they had other developmental problems or socioemotional, conduct, or attention difficulties. INTERPRETATION: Efforts are needed to increase MRHC for 5-year-old children born EPT with movement difficulties. WHAT THIS PAPER ADDS: Children born extremely preterm without cerebral palsy frequently experienced motor difficulties. Most of these children were not receiving motor-related health care (MRHC). Large geographical differences throughout Europe were observed in receipt of MRHC. Socioemotional problems were related to MRHC use.

Electronic Decision Support in the Delivery Room Using Augmented Reality to Improve Newborn Life Support Guideline Adherence: A Randomized Controlled Pilot Study.

INTRODUCTION: The Newborn Life Support (NLS) guideline aims to provide healthcare professionals a consistent approach during neonatal resuscitation. Adherence to this and analogous guidelines has repetitively been proven to be difficult.This study evaluates adherence to guideline using a novel augmented reality (Microsoft HoloLens) electronic decision support tool during standardized simulated neonatal resuscitation compared with subjects working from memory alone. METHODS: In this randomized controlled pilot study, 18 professionals responsible for neonatal resuscitation were randomized to the intervention group and 11 to the control group. Demographic characteristics were similar between both groups. A standardized neonatal resuscitation scenario was performed, which was recorded and later assessed for adherence to the NLS algorithm by 2 independent reviewers. Secondary outcomes were error classification in case of algorithm deviation and time to the execution or completion of critical steps in the algorithm to determine delay. RESULTS: Median (interquartile range) scores of a theoretical maximum of 40 in the intervention group were 34 (32.5-35.5) versus 29 (27-33) in the control group ( P = 0.004). Errors of commission were committed less frequently with the electronic decision support tool 2 (1-2.5) compared with 4 (2-4) in the control group ( P = 0.029). Analysis of time to initiation or completion of key steps in the NLS algorithm showed no significant differences between both groups. CONCLUSIONS: Healthcare professionals using an electronic decision support tool showed improved adherence to the NLS guideline during simulated neonatal resuscitation.

Neonatal pulmonary hypertension after severe early-onset fetal growth restriction: post hoc reflections on the Dutch STRIDER study.

The aim was to reflect on the unexpected finding of persistent pulmonary hypertension of the neonate (PPHN) and pulmonary hypertension in infants born within the Dutch STRIDER trial, its definition and possible pathophysiological mechanisms. The trial randomly assigned pregnant women with severe early-onset fetal growth restriction to sildenafil 25 mg three times a day versus placebo. Sildenafil use did not reduce perinatal mortality and morbidity, but did result in a higher rate of neonatal pulmonary hypertension (PH). The current paper reflects on the used definition, prevalence, and possible pathophysiology of the data on pulmonary hypertension. Twenty infants were diagnosed with pulmonary hypertension (12% of 163 live born infants). Of these, 16 infants had PPHN shortly after birth, and four had pulmonary hypertension associated with sepsis or bronchopulmonary dysplasia. Four infants with PPHN in the early neonatal period subsequently developed pulmonary hypertension associated with bronchopulmonary dysplasia in later life. Infants with pulmonary hypertension were at lower gestational age at delivery, had a lower birth weight and a higher rate of neonatal co-morbidity. The infants in the sildenafil group showed a significant increase in pulmonary hypertension compared to the placebo group (relative risk 3.67; 95% confidence interval 1.28 to 10.51, P = 0.02). CONCLUSION: Pulmonary hypertension occurred more frequent among infants of mothers allocated to antenatal sildenafil compared with placebo. A possible pathophysiological mechanism could be a "rebound" vasoconstriction after cessation of sildenafil. Additional studies and data are necessary to understand the mechanism of action. WHAT IS KNOWN: • In the Dutch STRIDER trial, persistent pulmonary hypertension in the neonate (PPHN) was more frequent among infants after antenatal sildenafil exposure versus placebo. WHAT IS NEW: • The current analysis focuses on the distinction between PPHN and pulmonary hypertension associated with sepsis or bronchopulmonary dysplasia and on timing of diagnosis and aims to identify the infants at risk for developing pulmonary hypertension. • The diagnosis pulmonary hypertension is complex, especially in infants born after severe early-onset fetal growth restriction. The research field could benefit from an unambiguous consensus definition and standardized screening in infants at risk is proposed.

Digital decision aid for prenatal counseling in imminent extreme premature labor: development and pilot testing.

BACKGROUND: In case of extreme premature delivery at 24 weeks of gestation, both early intensive care and palliative comfort care for the neonate are considered treatment options. Prenatal counseling, preferably using shared decision making, is needed to agree on the treatment option in case labor progresses. This article described the development of a digital decision aid (DA) to support pregnant women, partners and clinicians in prenatal counseling for imminent extreme premature labor. METHODS: This DA is developed following the International Patient Decision Aid Standards. The Dutch treatment guideline and the Dutch recommendations for prenatal counseling in extreme prematurity were used as basis. Development of the first prototype was done by expert clinicians and patients, further improvements were done after alpha testing with involved clinicians, patients and other experts (n = 12), and beta testing with non-involved clinicians and patients (n = 15). RESULTS: The final version includes information, probabilities and figures depending on users' preferences. Furthermore, it elicits patient values and provides guidance to aid parents and professionals in making a decision for either early intensive care or palliative comfort care in threatening extreme premature delivery. CONCLUSION: A decision aid was developed to support prenatal counseling regarding the decision on early intensive care versus palliative comfort care in case of extreme premature delivery at 24 weeks gestation. It was well accepted by parents and healthcare professionals. Our multimedia, digital DA is openly available online to support prenatal counseling and personalized, shared decision-making in imminent extreme premature labor.

Validation of an Instrument for Real-Time Assessment of Neonatal Intubation Skills: A Randomized Controlled Simulation Study.

OBJECTIVE: This study aimed to evaluate the construct validity and reliability of real-time assessment of a previously developed neonatal intubation scoring instrument (NISI). STUDY DESIGN: We performed a randomized controlled simulation study at a simulation-based research and training facility. Twenty-four clinicians experienced in neonatal intubation ("experts") and 11 medical students ("novices") performed two identical elective intubations on a neonatal patient simulator. Subjects were randomly assigned to either the intervention group, receiving predefined feedback between the two intubations, or the control group, receiving no feedback. Using the previously developed NISI, all intubations were assessed, both in real time and remotely on video. Construct validity was evaluated by (1) comparing the intubation performances, expressed as percentage scores, with and without feedback, and (2) correlating the intubation performances with the subjects' level of experience. The intrarater reliability, expressed as intraclass correlation coefficient (ICC), of real-time assessment compared with video-based assessment was determined. RESULTS: The intervention group contained 18 subjects, the control group 17. Background characteristics and baseline intubation scores were comparable in both groups. The median (IQR) change in percentage scores between the first and second intubation was significantly different between the intervention and control group (11.6% [4.7-22.8%] vs. 1.4% [0.0-5.7%], respectively; p = 0.013). The 95% CI for this 10.2% difference was 2.2 to 21.4%. The subjects' experience level correlated significantly with their percentage scores (Spearman's R = 0.70; p <0.01). ICC's were 0.95 (95% CI: 0.89-0.97) and 0.94 (95% CI: 0.89-0.97) for the first and second intubation, respectively. CONCLUSION: Our NISI has construct validity and is reliable for real-time assessment. KEY POINTS: · Our neonatal intubation scoring instrument has construct validity.. · Our instrument can be reliably employed to assess neonatal intubation skills directly in real time.. · It is suitable for formative assessment, i.e., providing direct feedback during procedural training..

High Healthcare Use at Age 5 Years in a European Cohort of Children Born Very Preterm.

OBJECTIVES: To describe parent-reported healthcare service use at age 5 years in children born very preterm and investigate whether perinatal and social factors and the use of very preterm follow-up services are associated with high service use. STUDY DESIGN: We used data from an area-based cohort of births at <32 weeks of gestation from 11 European countries, collected from birth records and parental questionnaires at 5 years of age. Using the published literature, we defined high use of outpatient/inpatient care (≥4 sick visits to general practitioners, pediatricians, or nurses, ≥3 emergency room visits, or ≥1 overnight hospitalization) and specialist care (≥2 different specialists or ≥3 visits). We also categorized countries as having either a high or a low rate of children using very preterm follow-up services at age 5 years. RESULTS: Overall, 43% of children had high outpatient/inpatient care use and 48% had high specialist care use during the previous year. Perinatal factors were associated with high outpatient/inpatient and specialist care use, with a more significant association with specialist services. Associations with intermediate parental educational level and unemployment were stronger for outpatient/inpatient services. Living in a country with higher rates of very preterm follow-up service use was associated with lower use of outpatient/inpatient services. CONCLUSIONS: Children born very preterm had high healthcare service use at age 5 years, with different patterns for outpatient/inpatient and specialist care by perinatal and social factors. Longer follow-up of children born very preterm may improve care coordination and help avoid undesirable health service use.

Adherence to the ABCDE approach in relation to the method of instruction: a randomized controlled simulation study.

BACKGROUND: The Airway, Breathing, Circulation, Disability, and Exposure (ABCDE) approach is widely recommended and taught in many resuscitation courses. This study assessed the adherence to the ABCDE algorithm and whether this was affected by the instruction method used to teach this approach. METHODS: Randomized controlled trial in which simulation was used as investigational method. Between June 2017 and January 2018, neonatal healthcare providers routinely participated in simulated neonatal advanced life support (NALS) scenarios, using a high-fidelity manikin. They were randomly assigned to a video-based instruction (intervention group) or a conventional lecture (control group) as the method of instruction. One blinded researcher evaluated the adherence to the ABCDE approach on video with an assessment tool specifically designed and tested for this study. The primary outcomes were: 1) the overall adherence and 2) the between-group difference in individual adherence to the ABCDE approach, both expressed as a percentage score. Secondary outcomes were: 1) the scores of each profession category (nurses, neonatal ward clinicians, fellows/neonatologists) and 2) the scores for the separate domains (A, B, C, D, and E) of the algorithm. RESULTS: Seventy-two participants were assessed. Overall mean (SD) percentage score (i.e. overall adherence) was 31.5% (19.0). The video-based instruction group (28 participants) adhered better to the ABCDE approach than the lecture group (44 participants), with mean (SD) scores of 38.8% (18.7) and 27.8% (18.2), respectively (p = 0.026). The difference in adherence between both groups could mainly be attributed to differences in the adherence to domain B (p = 0.023) and C (p = 0.007). Neonatal ward clinicians (39.9% (18.2)) showed better adherence than nurses (25.0% (15.2)), independent of the study group (p = 0.010). CONCLUSIONS: Overall adherence to the ABCDE algorithm was rather low. Video-based instruction resulted in better adherence to the ABCDE approach during NALS training than lecturing. TRIAL REGISTRATION: ISRCTN registry, trial ID ISRCTN95998973 , retrospectively registered on October 13th, 2020.

Training practices in neonatal and paediatric life support: A survey among healthcare professionals working in paediatrics.

AIM: To evaluate neonatal and paediatric life support training practices across Europe. METHODS: We conducted a descriptive study. Paediatric residents, general paediatricians, and subspecialists were surveyed to assess how paediatric basic and advanced life support (PBLS/PALS) and neonatal life support (NLS) are practically arranged and utilised throughout Europe. A mini-Delphi approach was used for survey development. Eligible professionals in general and university hospitals received a web link to the survey. RESULTS: 498 respondents from 16 countries were included. A large majority of responses came from the Netherlands (n = 393) and Belgium (n = 42). Therefore, analysis was based on these responses. PBLS was more frequently offered than PALS and NLS, though not to all professionals caring for children. For PBLS, PALS, and NLS, official recertification varied between 35-75%. Approximately 80-90% had read the latest guidelines, at least partially. Sixty to seventy percent felt capable of instant PALS, 75-90% considered themselves able to perform PBLS and NLS instantly. Not reading the guidelines and less confidence about instant resuscitation seemed to occur more often in the lower and higher age/experience groups compared to the intermediate age/experience groups. A quarter of the respondents <30 years did not feel prepared for instant PALS. General paediatricians appeared to feel most capable of instant resuscitation. General and university hospitals had rather similar training practices and facilities. Manikins were predominantly low-fidelity, especially in general hospitals. Barriers to course participation were high costs, lack of time, the non-compulsory status, remote location, and unavailability of courses. CONCLUSION: Although most paediatric professionals receive life support training, guideline reading, recertification, training utilisation, and resuscitation preparedness require improvement. Barriers to course participation should be addressed.

Fast detection of FOXF1 variants in patients with alveolar capillary dysplasia with misalignment of pulmonary veins using targeted sequencing.

BACKGROUND: Alveolar capillary dysplasia with misalignment of pulmonary veins (ACD/MPV) is a lethal congenital lung disorder associated with heterozygous variants in the FOXF1 gene or its regulatory region. Patients with ACD/MPV unnecessarily undergo invasive and expensive treatments while awaiting a diagnosis. The aim of this study was to reduce the time to diagnose ACD/MPV by developing a targeted next-generation sequencing (NGS) panel that detects FOXF1 variants. METHODS: A FOXF1-targeted NGS panel was developed for detection of mutations and large genomic alterations and used for retrospective testing of ACD/MPV patients and controls. Results were confirmed with Sanger sequencing and SNP array analysis. RESULTS: Each amplicon of the FOXF1-targeted NGS panel was efficiently sequenced using DNA isolated from blood or cell lines of 15 ACD/MPV patients and 8 controls. Moreover, testing of ACD/MPV patients revealed six novel and six previously described pathogenic or likely pathogenic FOXF1 alterations. CONCLUSION: We successfully designed a fast and reliable targeted genetic test to detect variants in the FOXF1 gene and its regulatory region in one run. This relatively noninvasive test potentially prevents unnecessary suffering for patients and reduces the use of futile and expensive treatments like extra-corporeal membrane oxygenation. IMPACT: FOXF1-targeted NGS potentially prevents ACD/MPV patients from unnecessary suffering and expensive treatments. FOXF1-targeted NGS potentially reduces the number of misdiagnosis in ACD/MPV patients. Retrospective testing of ACD/MPV patients using FOXF1-targeted NGS revealed six novel pathogenic or likely pathogenic variants.

Validation of an Assessment Instrument for Pediatric Basic Life Support.

OBJECTIVES: To develop a valid and reliable instrument for the assessment of pediatric basic life support (PBLS). METHODS: An assessment instrument for PBLS was developed, based on 3 existing scoring systems and the European Resuscitation Council PBLS guideline. We tested if experienced PBLS instructors performed better than medical students on a standard PBLS examination on a low-fidelity pediatric manikin (construct validity). To pass the examination, 15 penalty points or less were required. The examinations were videotaped. One researcher assessed all videos once, and approximately half of them twice (intrarater reliability). A second researcher independently assessed part of the videos (interrater reliability). The time needed to assess 1 examination was determined. RESULTS: Face and content validity were established, because PBLS experts reached consensus on the instrument and because the instrument incorporated all items of the European Resuscitation Council algorithm. Of the 157 medical students that were scored, 98 (62.4%) passed the examination. Fourteen PBLS instructors were scored; all passed (100%). Pass rate (62.4% vs 100%) and median penalty points (15 [interquartile range, 10-22.5] vs 7.5 [interquartile range, 1.25-10]) were significantly different between students and instructors (P = 0.005 and <0.001, respectively). Reassessment demonstrated a κ for intrarater reliability of 0.62 (95% confidence interval, 0.45-0.81) (substantial agreement); κ for interrater reliability was 0.51 (95% confidence interval, 0.09-0.93) (moderate agreement). It took approximately 3 minutes to assess 1 videotaped examination. CONCLUSIONS: Our instrument for the (video-based) assessment of PBLS is valid and sufficiently reliable. It is also designed to be practical, time-efficient, and applicable in various settings, including resource limited.