Voices of experience: insights from Dutch parents on periviability guidelines and personalisation.

Objective: To investigate the perspectives of experienced parents regarding guidelines and personalisation for managing imminent extremely premature births (22-26 weeks gestational age (GA)) . The study examined four scenarios: no guideline, a guideline based on GA, a guideline based on GA plus other factors and a guideline based on a calculated prognosis.

Design: Nineteen semistructured qualitative interviews were conducted with Dutch parents who experienced (imminent) extremely premature births between 23+5 and 26+2 weeks of gestation.

Medication adherence to CFTR modulators in patients with cystic fibrosis: a systematic review.

Background: In the last decade, a fundamental shift in the treatment of cystic fibrosis (CF) took place due to the introduction of CF transmembrane conductance regulator (CFTR) modulators. Adequate medication adherence is a prerequisite for their effectiveness, but little is known about adherence to CFTR modulators. We aimed to assess the extent of medication adherence to CFTR modulators in patients with CF and assess which characteristics are associated with adherence.

Predictive validity of the Standardized Infant NeuroDevelopmental Assessment (SINDA) to identify 4-5 year-old children at risk of developmental delay in a low-risk sample.

Background: Early detection of developmental problems is important as it allows for early intervention. Previous studies, in high-risk infants, found high predictive values of atypical scores on the Standardized Infant NeuroDevelopmental Assessment (SINDA) for later neurodevelopmental disorders (i.e.

Physical performance before and after hematopoietic stem cell transplantation in pediatric patients: a potential role for prehabilitation.

The trajectory of hematopoietic stem cell transplantation (HSCT) is often accompanied by physically disabling complications that impair physical performance of pediatric patients. However, knowledge about when impairments in physical performance arise and the factors contributing to these impairments is limited. Therefore, we conducted a retrospective analysis of physical performance 100 days post-HSCT in patients aged 3-18 years.

Potential, Pitfalls, and Future Directions for Remote Monitoring of Chronic Respiratory Diseases: Multicenter Mixed Methods Study in Routine Cystic Fibrosis Care.

Background: The current literature inadequately addresses the extent to which remote monitoring should be integrated into care models for chronic respiratory diseases (CRDs).

Objective: This study examined a remote monitoring program (RMP) in cystic fibrosis (CF) by exploring experiences, future perspectives, and use behavior over 3 years, with the aim of developing future directions for remote monitoring in CRDs.

Methods: This was a mixed methods, multicenter, observational study in 5 Dutch CF centers following a sequential explanatory design.

Shining light on chest shielding.

Impact: Key message: Chest shielding during phototherapy does not reduce the incidence of symptomatic PDA in preterm infants and does not affect phototherapy's efficacy. This study adds to the existing literature by providing a double-blinded RCT confirming previous inconsistent findings on the impact of chest shielding on PDA incidence. However, phototherapy may exert cardiovascular changes, including vasodilatation.

ECTOPIC trial: The efficacy of flEcainide Compared To metOprolol in reducing Premature ventrIcular Contractions: A randomized open-label crossover study in pediatric patients.

Background: Frequent premature ventricular contractions (PVCs) in children are usually considered benign. Symptoms and left ventricular dysfunction are indications for treatment with antiarrhythmic drugs.

Objective: This study aimed to evaluate the efficacy of flecainide vs metoprolol in reducing PVCs in children.

Explorative study on the value of hepcidin in predicting iron non-responsiveness in paediatric inflammatory bowel disease.

Background: In a considerable proportion of anaemic children with inflammatory bowel disease (IBD), haemoglobin (Hb) does not normalise after iron therapy. We evaluated the added value of novel iron markers (hepcidin and soluble transferrin receptor [sTfR]) as compared to traditional iron markers (ferritin and transferrin saturation [TSAT]) to determine the best strategy for the prediction of non-responsiveness to iron suppletion.

Methods: In this secondary analysis of prospectively collected data, we measured iron markers in anaemic children (Hb Z-score < -2.

Bilirubin measurements in neonates: uniform neonatal treatment can only be achieved by improved standardization.

Measurement of total bilirubin (TBil) concentration in serum is the gold standard approach for diagnosing neonatal unconjugated hyperbilirubinemia. It is of utmost importance that the measured TBil concentration is sufficiently accurate to prevent under treatment, unnecessary escalation of care, or overtreatment. However, it is widely recognized that TBil measurements urgently require improvement in neonatal clinical chemistry.

Cerebrovascular Autoregulation in Preterm Infants Using Heart Rate or Blood Pressure: A Pilot Study.

Background: Cerebrovascular autoregulation (CAR) is often impaired in preterm infants but requires invasive mean arterial blood pressure (MABP) measurements for continuous assessment. We aimed to assess whether using heart rate (HR) results in different CAR assessment compared with using MABP.

Methods: We compared CAR (moving window correlation-coefficient with cerebral oxygenation saturation (rSO)), and percentage of time with impaired CAR (%timeCARi) calculated by either HR (TOHRx, tissue oxygenation heart rate reactivity index) or MABP (COx, cerebral oximetry index) during the first 72 h after birth, and its association with short-term cerebral injury.

Evaluation of the Newborn Screening Pilot for Sickle Cell Disease in Suriname Using the Non-Adoption, Abandonment, Scale-Up, Spread, and Sustainability (NASSS) Framework.

The early detection of sickle cell disease (SCD) is vital to reduce mortality among affected children. Suriname currently lacks a newborn screening programme (NSP) for SCD. We performed a pilot programme to evaluate the scalability of such an initiative.

Healthcare costs related to respiratory syncytial virus in paediatric intensive care units in the Netherlands: a nationwide prospective observational study (the BRICK study).

Background: The implementation of the approved respiratory syncytial virus (RSV) preventive interventions in immunisation programmes is advancing rapidly. Insight into healthcare costs of RSV-related paediatric intensive care unit (PICU) admissions is lacking, but of great importance to evaluate the impact of implementation. Therefore, this study aimed to determine the total annual RSV-related paediatric intensive care healthcare costs in the Netherlands.

Short and long-term acceptability and efficacy of extended-release cornstarch in the hepatic glycogen storage diseases: results from the Glyde study.

Background: Hypoglycaemia is the primary manifestation of all the hepatic types of glycogen storage disease (GSD). In 2008, Glycosade, an extended-release waxy maize cornstarch, was reported as an alternative to uncooked cornstarch (UCCS) which could prolong the duration of fasting in the GSD population. To date, there has been minimal published experience in (a) young children, (b) the ketotic forms of GSD, and (c) with daytime dosing.

Asthma Inception: Epidemiologic Risk Factors and Natural History Across the Life Course.

Asthma is a descriptive label for an obstructive inflammatory disease in the lower airways manifesting with symptoms including breathlessness, cough, difficulty in breathing, and wheezing. From a clinician's point of view, asthma symptoms can commence at any age, although most patients with asthma-regardless of their age of onset-seem to have had some form of airway problems during childhood. Asthma inception and related pathophysiologic processes are therefore very likely to occur early in life, further evidenced by recent lung physiologic and mechanistic research.

Screening and surveillance of hepatocellular carcinoma by serum des-gamma-carboxy prothrombin in patients with glycogen storage disease type Ia.

No sensitive tumor marker for hepatocellular carcinoma (HCC) is available for patients with glycogen storage disease type Ia (GSDIa), in whom alpha-fetoprotein and carcino-embryonic antigen levels often remain normal. We describe increased levels of the HCC tumor marker des-gamma-carboxy prothrombin (DCP) in GSDIa patients with HCC. In one case DCP levels normalized after liver transplantation.

Improving paediatric movement disorders care: Insights on rating scales utilization and clinical practice.

Aim: This exploratory study evaluates rating scale usage by experts from the European Reference Network for Rare Neurological Diseases (ERN-RND) for paediatric MD, considering factors like diagnosis, intellectual disability, age, and transition to adult care. The aim is to propose a preliminary framework for consistent application.

Methods: A multicentre survey among 25 ERN-RND experts from 10 European countries examined rating scale usage in paediatric MD, categorizing MD into acute, non-progressive, and neurodegenerative types.

Executive Summary: The Pediatric Extracorporeal Membrane Oxygenation Anticoagulation CollaborativE (PEACE) Consensus Conference.

Objectives: To present recommendations and consensus statements with supporting literature for the clinical management of neonates and children supported with extracorporeal membrane oxygenation (ECMO) from the Pediatric ECMO Anticoagulation CollaborativE (PEACE) consensus conference.

Data Sources: Systematic review was performed using PubMed, Embase, and Cochrane Library (CENTRAL) databases from January 1988 to May 2021, followed by serial meetings of international, interprofessional experts in the management ECMO for critically ill children.

Study Selection: The management of ECMO anticoagulation for critically ill children.