Screening, diagnosis and follow-up of Brugada syndrome in children: a Dutch expert consensus statement.

Brugada syndrome (BrS) is a rare inherited arrhythmia syndrome. Affected children may experience life-threatening symptoms, mainly during fever. The percentage of SCN5A variant carriers in children is higher than in adults.

Thymus and activation-regulated chemokine (TARC) as treatment response marker for paediatric Hodgkin lymphoma: A pilot study.

Classical Hodgkin lymphoma (cHL) is characterised by malignant Hodgkin Reed-Sternberg cells located in an inflammatory microenvironment. Blood biomarkers result from active cross-talk between malignant and non-malignant cells. One promising biomarker in adult patients with cHL is thymus and activation-regulated chemokine (TARC).

Nutritional follow-up in children after discharge: Organisation in a tertiary care centre.

Background: Aiming to improve the standard of care for patients with a nutritional intervention, we evaluated how nutritional follow-up (FU) is organised at discharge and after 6 months.

Methods: From 16 November 2020 until 20 December 2020, we retrospectively included patients admitted for > 1 day to the general ward of a tertiary hospital. Medical charts were reviewed for demographics, anthropometric measurements and nutritional interventions (e.

Haploinsufficiency Impacts Gastrointestinal Function and Leads to Pediatric Intestinal Pseudo-obstruction.

Pediatric Intestinal Pseudo-obstruction (PIPO) is a congenital enteric disorder characterized by severe gastrointestinal (GI) dysmotility, without mechanical obstruction. Although several genes have been described to cause this disease, most patients do not receive a genetic diagnosis. Here, we aim to identify the genetic cause of PIPO in a patient diagnosed with severe intestinal dysmotility shortly after birth.

Rescue Paracetamol in Postoperative Pain Management in Extremely Low Birth Weight Neonates Following Abdominal Surgery: A Single Unit Retrospective Study.

Background: Intravenous paracetamol added to morphine reduces postoperative morphine consumption in (near)term neonates. However, there are only sparse data on intravenous paracetamol as multimodal strategy in extremely low birth weight (ELBW) neonates.

Objectives: This study aims to assess the effects of rescue intravenous paracetamol on postoperative pain management (≤48 h postoperatively) in relation to both analgesic efficacy (validated pain assessment, drug consumption, adequate rescue medication) and safety (hypotension and bradycardia).

Evaluation of exclusive enteral nutrition and corticosteroid induction treatment in new-onset moderate-to-severe luminal paediatric Crohn's disease.

To induce remission in luminal paediatric Crohn's disease (CD), the ESPGHAN/ECCO guideline recommends treatment with exclusive enteral nutrition (EEN) or oral corticosteroids. In newly diagnosed moderate-to-severe paediatric CD patients, we determined the proportion of patients in which EEN or corticosteroids induced remission and maintained remission on azathioprine monotherapy. We included patients from the "TISKids" study assigned to the conventional treatment arm.

Nutritional intake and gastro-intestinal symptoms in critically ill COVID-19 patients.

Background & Aims: Critically ill COVID-19 patients seem hypermetabolic and difficult to feed enterally, due to gastro-intestinal (GI) symptoms such as high gastric residual volumes (GRV) and diarrhea. Our aim was to describe the association of nutritional intake and GI symptoms during first 14 days of ICU admission.

Methods: Observational study including critically ill adult COVID-19 patients.

First genome-wide association study of esophageal atresia identifies three genetic risk loci at , //, and .

Esophageal atresia with or without tracheoesophageal fistula (EA/TEF) is the most common congenital malformation of the upper digestive tract. This study represents the first genome-wide association study (GWAS) to identify risk loci for EA/TEF. We used a European case-control sample comprising 764 EA/TEF patients and 5,778 controls and observed genome-wide significant associations at three loci.

The Safety of Intralesional Steroid Injections in Young Children and Their Effectiveness in Anastomotic Esophageal Strictures-A Meta-Analysis and Systematic Review.

Objective: Intralesional steroid injections (ISI) are a widely used technique for various pediatric indications and represent a possible adjuvant treatment for anastomotic esophageal strictures. Yet, no consensus has been reached neither on their safety in the pediatric population or their effectiveness in esophageal atresia patients. This systematic review aimed to assess the safety of ISI in young children through a meta-analysis and to summarize the current knowledge on the effectiveness of ISI in anastomotic esophageal strictures.

Study Protocol of the Exercise Study: Unraveling Limitations for Physical Activity in Children With Chronic Diseases in Order to Target Them With Tailored Interventions-A Randomized Cross Over Trial.

Physical activity is associated with many physiological and psychological health benefits across the lifespan. Children with a chronic disease often have lower levels of daily physical activity, and a decreased exercise capacity compared to healthy peers. In order to learn more about limitations for physical activity, we investigate children with four different chronic diseases: children with a Fontan circulation, children with Broncho Pulmonary Dysplasia (BPD), Pompe disease and inflammatory bowel disease (IBD).

Three-dimensional printing in paediatric orthopaedic surgery.

Three-dimensional (3D) printing is a rapidly evolving and promising field to improve outcomes of orthopaedic surgery. The use of patient-specific 3D-printed models is specifically interesting in paediatric orthopaedic surgery, as limb deformity corrections often require an individual 3D treatment. In this editorial, various operative applications of 3D printing in paediatric orthopaedic surgery are discussed.

Clinical validation of digital biomarkers for paediatric patients with asthma and cystic fibrosis: potential for clinical trials and clinical care.

Background: Digital biomarkers are a promising novel method to capture clinical data in a home setting. However, clinical validation prior to implementation is of vital importance. The aim of this study was to clinically validate physical activity, heart rate, sleep and forced expiratory volume in 1 s (FEV) as digital biomarkers measured by a smartwatch and portable spirometer in children with asthma and cystic fibrosis (CF).

-related lung disease in people with cystic fibrosis: can imaging help us to diagnose disease?

In people with cystic fibrosis (PwCF), viscous sputum and dysfunction of the mucociliary escalator leads to early and chronic infections. The prevalence of in sputum is high in PwCF and the contribution of to the progression of structural lung disease has been reported. However, overall, relatively little is known about the contribution of to CF lung disease.

The Incidence and Characteristics of Venous Thromboembolisms in Paediatric-Onset Inflammatory Bowel Disease: A Prospective International Cohort Study Based on the PIBD-SETQuality Safety Registry.

Background And Aims: Guidelines regarding thromboprophylaxis for venous thromboembolisms [VTEs] in children with inflammatory bowel disease [IBD] are based on limited paediatric evidence. We aimed to prospectively assess the incidence of VTEs in paediatric-onset IBD [PIBD], characterize PIBD patients with a VTE and identify potential IBD-related risk factors.

Methods: From October 2016 to September 2020, paediatric gastroenterologists prospectively replied to the international Safety Registry, monthly indicating whether they had observed a VTE case in a patient <19 years with IBD.

Longitudinal Health Status and Quality of Life After Esophageal Atresia Repair.

Objectives: To longitudinally evaluate self-reported and proxy-reported health status (HS) and quality of life (QoL) of school-aged children born with esophageal atresia (EA).

Methods: We obtained Pediatric Quality of Life Inventory (HS) and DUX-25 (QoL) questionnaires from children born with EA between 1999 and 2011 at 8 and/or 12 years old. Children completed self-reports during neuropsychological assessments in a prospective longitudinal follow-up program.

Clinical signs, interventions, and treatment course of three different treatment protocols in patients with Crouzon syndrome with acanthosis nigricans.

Objective: Crouzon syndrome with acanthosis nigricans (CAN) is a rare and clinically complex subtype of Crouzon syndrome. At three craniofacial centers, this multicenter study was undertaken to assess clinical signs in relation to the required interventions and treatment course in patients with CAN.

Methods: A retrospective cohort study of CAN was performed to obtain information about the clinical treatment course of these patients.

Hospital Presentation for Self-Harm in Youth as a Risk Marker for Later Psychotic and Bipolar Disorders: A Cohort Study of 59 476 Finns.

Expanding clinical strategies to identify high risk groups for psychotic and bipolar disorders is a research priority. Considering that individuals diagnosed with psychotic and bipolar disorder are at high risk of self-harm, we hypothesised the reverse order relationship would also be true (ie, self-harm would predict psychotic/bipolar disorder). Specifically, we hypothesised that hospital presentation for self-harm would be a marker of high risk for subsequent development of psychotic/bipolar disorder and sought to test this hypothesis in a large population sample.

A review of different resuscitation platforms during delayed cord clamping.

There is a large body of evidence demonstrating that delaying clamping of the umbilical cord provides benefits for term and preterm infants. These benefits include reductions in mortality in preterm infants and improved developmental scores at 4 years of age in term infants. However, non-breathing or non-vigorous infants at birth are excluded due to the perceived need for immediate resuscitation.

ERS International Congress 2020: highlights from the Paediatric Assembly.

In this review, the Paediatric Assembly of the European Respiratory Society (ERS) presents a summary of the highlights and most relevant findings in the field of paediatric respiratory medicine presented at the virtual ERS International Congress 2020. Early Career Members of the ERS and Chairs of the different Groups comprising the Paediatric Assembly discuss a selection of the presented research. These cover a wide range of research areas, including respiratory physiology and sleep, asthma and allergy, cystic fibrosis, respiratory infection and immunology, neonatology and intensive care, epidemiology, bronchology and lung and airway development.

Supplementation of vitamins, trace elements and electrolytes in the PEPaNIC Randomised Controlled Trial: Composition and preparation of the prescription.

Background And Aims: Following the results of the paediatric early versus late parenteral nutrition in critical illness (PEPaNIC) multicentre, randomised, controlled trial, the new ESPGHAN/ESPEN/ESPR/CSPEN and ESPNIC guidelines recommend to consider withholding parenteral macronutrients for 1 week, while providing micronutrients, in critically ill children if enteral nutrition is insufficient. Critically ill children are suspected to be vulnerable to micronutrient deficiencies due to inadequate enteral nutrition, increased body's demands and excessive losses. Hitherto, micronutrient requirements in PICU are estimated based on recommended daily intakes for healthy children and expert opinion.

Methadone effectively attenuates acute and long-term consequences of neonatal repetitive procedural pain in a rat model.

Background: Painful procedures in early life cause acute pain and can alter pain processing at a spinal level lasting into adulthood. Current methods of analgesia seem unable to prevent both acute and long-term hypersensitivity associated with neonatal pain. The current study aims to prevent acute and long-term hypersensitivity associated with neonatal procedural pain using methadone analgesia in rat pups.

Structure and Function of the Vocal Cords after Airway Reconstruction on Magnetic Resonance Imaging.

Objectives/hypothesis: Dysphonia is a common problem at long-term follow-up after airway surgery for laryngotracheal stenosis (LTS) with major impact on quality of life. Dysphonia after LTS can be caused by scar tissue from initial stenosis along with anatomical alterations after surgery. There is need for a modality to noninvasively image structure and function of the reconstructed upper airways including the vocal cords to assess voice outcome and possible treatment after LTS.