Impact of Presence, Level, and Closure of a Stoma on Growth in Young Children: A Retrospective Cohort Study.

Introduction:  A stoma will cause nutrients loss which could result in impaired growth. Impaired growth can negatively impact long-term development. This study aims to evaluate: (1) the effect of stomas on growth comparing small bowel stoma versus colostomy and (2) if early closure (within 6 weeks), proximal small bowel stoma (within 50 cm of Treitz), major small bowel resection (≥ 30 cm), or adequate sodium supplementation (urinary level ≤ 30 mmol/L) influences growth.

Stability of a two-dimensional biomorphoelastic model for post-burn contraction.

We consider the stability analysis of a two-dimensional model for post-burn contraction. The model is based on morphoelasticity for permanent deformations and combined with a chemical-biological model that incorporates cellular densities, collagen density, and the concentration of chemoattractants. We formulate stability conditions depending on the decay rate of signaling molecules for both the continuous partial differential equations-based problem and the (semi-)discrete representation.

Mutations in Neurobeachin-like 2 do not impact Weibel-Palade body biogenesis and von Willebrand factor secretion in gray platelet syndrome Endothelial Colony Forming Cells.

Background: Patients with gray platelet syndrome (GPS) and Neurobeachin-like 2 (NBEAL2) deficiency produce platelets lacking alpha-granules (AGs) and present with lifelong bleeding symptoms. AGs are lysosome-related organelles and store the hemostatic protein von Willebrand factor (VWF) and the transmembrane protein P-selectin. Weibel-Palade bodies (WPBs) are lysosome-related organelles of endothelial cells and also store VWF and P-selectin.

The impact of necrotizing soft tissue infections on the lives of survivors: a qualitative study.

Purpose: Necrotizing soft tissue infections (NSTI) are potentially lethal infections marked by local tissue destruction and systemic sepsis, which require aggressive treatment. Survivors often face a long recovery trajectory. This study was initiated to increase understanding of the long-term impact of NSTI on health related quality of life (HRQoL), and how care may be improved.

Two-year outcomes following a randomised platelet transfusion trial in preterm infants.

Objective: Assess mortality and neurodevelopmental outcomes at 2 years of corrected age in children who participated in the PlaNeT-2/MATISSE (Platelets for Neonatal Transfusion - 2/Management of Thrombocytopenia in Special Subgroup) study, which reported that a higher platelet transfusion threshold was associated with significantly increased mortality or major bleeding compared to a lower one.

Design: Randomised clinical trial, enrolling from June 2011 to August 2017. Follow-up was complete by January 2020.

The Complexity of the Post-Burn Immune Response: An Overview of the Associated Local and Systemic Complications.

Burn injury induces a complex inflammatory response, both locally and systemically, and is not yet completely unravelled and understood. In order to enable the development of accurate treatment options, it is of paramount importance to fully understand post-burn immunology. Research in the last decades describes insights into the prolonged and excessive inflammatory response that could exist after both severe and milder burn trauma and that this response differs from that of none-burn acute trauma.

Personalised azithromycin+metronidazole (PAZAZ), in combination with standard induction therapy, to achieve a faecal microbiome community structure and metagenome changes associated with sustained remission in paediatric Crohn's disease (CD): protocol of a pilot study.

Introduction: Early relapse in Crohn's disease (CD) is associated with a more severe disease course. The microbiome plays a crucial role, yet strategies targeting the microbiome are underrepresented in current guidelines. We hypothesise that early manipulation of the microbiome will improve clinical response to standard-of-care (SOC) induction therapy in patients with a relapse-associated microbiome profile.

Childhood cancer and hematological disorders negatively affect spermatogonial quantity at diagnosis: a retrospective study of a male fertility preservation cohort.

Study Question: What is the impact of cancer or hematological disorders on germ cells in pediatric male patients?

Summary Answer: Spermatogonial quantity is reduced in testes of prepubertal boys diagnosed with cancer or severe hematological disorder compared to healthy controls and this reduction is disease and age dependent: patients with central nervous system cancer (CNS tumors) and hematological disorders, as well as boys <7 years are the most affected.

What Is Known Already: Fertility preservation in pediatric male patients is considered based on the gonadotoxicity of selected treatments. Although treatment effects on germ cells have been extensively investigated, limited data are available on the effect of the disease on the prepubertal male gonad.

Comparing the SARS-CoV-2-specific antibody response in human milk after homologous and heterologous booster vaccinations.

Human milk contains SARS-CoV-2-specific antibodies after COVID-19 vaccination. These milk antibodies decrease several months post-vaccination. Whether booster immunization restores human milk antibody levels, potentially offering prolonged passive immunity for the infant, remains unknown.

Hematologically important mutations: Leukocyte adhesion deficiency (second update).

Leukocyte adhesion deficiency (LAD) is an immunodeficiency caused by defects in the adhesion of leukocytes (especially neutrophils) to the blood vessel wall. As a result, patients with LAD suffer from severe bacterial infections and impaired wound healing, accompanied by neutrophilia. In LAD-I, characterized directly after birth by delayed separation of the umbilical cord, mutations are found in ITGB2, the gene that encodes the β subunit (CD18) of the β integrins.

Longitudinal SARS-CoV-2 humoral response in MS patients with and without SARS-CoV-2 infection prior to vaccination.

Introduction: During the COVID-19 pandemic, certain disease modifying therapies (DMTs) used in multiple sclerosis (MS), such as anti-CD20 therapies, have been associated with decreased humoral responses after SARS-CoV-2 vaccination. Hybrid immunity, referring to immunity after both vaccination and SARS-CoV-2 infection might increase humoral responses.

Methods: This was a substudy of two prospective cohort studies on SARS-CoV-2 antibodies after SARS-CoV-2 infection and vaccination.

Efficacy and Safety of Alirocumab in Children and Adolescents With Homozygous Familial Hypercholesterolemia: Phase 3, Multinational Open-Label Study.

Background: Despite progress in treating homozygous familial hypercholesterolemia, most patients do not achieve low-density lipoprotein cholesterol (LDL-C) targets. This study examined efficacy and safety of the PCSK9 (proprotein convertase subtilisin/kexin type 9) inhibitor, alirocumab, in pediatric patients (aged 8-17 years) with inadequately controlled homozygous familial hypercholesterolemia.

Methods: In this open-label, single-arm, multinational, Phase 3 study, patients (n=18) received alirocumab 75 mg or 150 mg (bodyweight <50 kg/≥50 kg) every 2 weeks as an adjunct to background treatment.

[A new technique for inguinal hernia repair in children: laparoscopic percutaneous inguinal ring suturing].

Inguinal hernia repair is one of the most frequently performed operations in the pediatric population and laparoscopic hernia repair is currently increasingly performed in Dutch academic and non-academic hospitals. The laparoscopic PIRS-technique is invented by Prof. Dr.

Sensitivity of a two-dimensional biomorphoelastic model for post-burn contraction.

We consider a two-dimensional biomorphoelastic model describing post-burn scar contraction. This model describes skin displacement and the development of the effective Eulerian strain in the tissue. Besides these mechanical components, signaling molecules, fibroblasts, myofibroblasts, and collagen also play a significant role in the model.

Barriers and perceived benefits of early mobilisation programmes in Dutch paediatric intensive care units.

Background: Early mobilisation of critically ill adults has been proven effective and is safe and feasible for critically ill children. However, barriers and perceived benefits of paediatric intensive care unit (PICU) staff involvement in mobilising critically ill children are largely unknown.

Aim: To explore the barriers and perceived benefits regarding early mobilisation of critically ill children as perceived by PICU staff.

Medical decision-making competence regarding puberty suppression: perceptions of transgender adolescents, their parents and clinicians.

According to international transgender care guidelines, transgender adolescents should have medical decision-making competence (MDC) to start puberty suppression (PS) and halt endogenous pubertal development. However, MDC is a debated concept in adolescent transgender care and little is known about the transgender adolescents', their parents', and clinicians' perspectives on this. Increasing our understanding of these perspectives can improve transgender adolescent care.

Reliability of the Sexual Knowledge Picture Instrument: a potential diagnostic instrument for sexual abuse in young children.

Objective: To determine the intra-rater and inter-rater reliability of the Sexual Knowledge Picture Instrument (SKPI), a potential diagnostic instrument for young suspected victims of sexual abuse containing three scoring forms, that is, verbal responses, non-verbal reactions and red flags.

Design: Video-recorded SKPI interviews with children with and without suspicion of child sexual abuse were observed and scored by two trained, independent raters. The second rater repeated the assessment 6 weeks after initial rating to evaluate for intra-rater reliability.

Intestinal Ultrasound Is Accurate to Determine Endoscopic Response and Remission in Patients With Moderate to Severe Ulcerative Colitis: A Longitudinal Prospective Cohort Study.

Background & Aims: Intestinal ultrasound (IUS) is noninvasive, cost-effective, and accurate to determine disease activity in ulcerative colitis (UC). In this study, we prospectively evaluated IUS for treatment response in a longitudinal cohort by using endoscopy and histology as gold standards.

Methods: Consecutive patients with moderate to severe UC (endoscopic Mayo score [EMS] ≥2) starting tofacitinib treatment were included.

The disease-specific clinical trial network for primary ciliary dyskinesia: PCD-CTN.

Primary ciliary dyskinesia (PCD) is a rare genetic disorder characterised by impaired mucociliary clearance leading to irreversible lung damage. In contrast to other rare lung diseases like cystic fibrosis (CF), there are only few clinical trials and limited evidence-based treatments. Management is mainly based on expert opinions and treatment is challenging due to a wide range of clinical manifestations and disease severity.

Preterm Birth is Associated with Lower Academic Attainment at Age 12 Years: A Matched Cohort Study by Linkage of Population-Based Datasets.

Objective: To compare academic attainment at age 12 years in preterm children born below 30 weeks of gestation with matched term-born peers, using standardized, nationwide and well-validated school tests.

Study Design: This population-based, national cohort study was performed by linking perinatal data from the nationwide Netherlands Perinatal Registry with educational outcome data from Statistics Netherlands and included 4677 surviving preterm children born at 25-29 weeks of gestational age and 366 561 controls born at 40 weeks of gestational age in 2000-2007. First, special education participation rate was calculated.

Long-term outcomes following antenatal exposure to low-dose aspirin: study protocol for the 4-year follow-up of the APRIL randomised controlled trial.

Introduction: The use of low-dose aspirin by pregnant women to prevent preterm pre-eclampsia is gradually increasing. The administration of aspirin during pregnancy improves perinatal outcome, which could translate into improved child outcome in the long term. However, antenatal exposure to aspirin could have adverse effects on child development that may manifest later in life.

Prevalence and Risk Factors for Hyposalivation and Xerostomia in Childhood Cancer Survivors Following Different Treatment Modalities-A Dutch Childhood Cancer Survivor Study Late Effects 2 Clinical Study (DCCSS LATER 2).

Background: Limited data are available on the risk factors of salivary gland dysfunction in long-term childhood cancer survivors (CCS). The objective of this cross-sectional study, part of the multidisciplinary multicenter Dutch CCS Study Late Effects 2 (DCCSS LATER 2), was to assess the prevalence of and risk factors for hyposalivation and xerostomia in CCS. Methods: From February 2016 until March 2020, 292 CCS were included.