37 results match your criteria: "ErasmusMC - Sophia Children's Hospital[Affiliation]"

Neuroinflammatory markers at school age in preterm born children with neurodevelopmental impairments.

Brain Behav Immun Health

July 2024

Department of Neonatology, Wilhelmina Children's Hospital, University Medical Center Utrecht, Utrecht University, Utrecht, the Netherlands.

Background: Immune system activation in the neonatal period is associated with white matter injury in preterm infants. In animal studies, neonatal priming of the immune system leads to chronic activation of i.e.

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Background: Treatment-related gonadal dysfunction leading to fertility problems is a frequently encountered late effect in childhood cancer survivors (CCSs). This study evaluated reproductive outcomes and reproductive health care utilization among male CCSs compared with male siblings.

Methods: A nationwide cohort study was conducted as part of the Dutch Childhood Cancer Survivor LATER study part 1, a questionnaire and linkage study.

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Hospital-to-home transitions for children with medical complexity: part 1, a systematic review of reported outcomes.

Eur J Pediatr

September 2023

Department of Pediatrics, Amsterdam UMC location University of Amsterdam, Emma Children's Hospital, Meibergdreef 9, Amsterdam, the Netherlands.

Outcome selection to evaluate interventions to support a successful transition from hospital to home of children with medical complexity (CMC) may be difficult due to the variety in available outcomes. To support researchers in outcome selection, this systematic review aimed to summarize and categorize outcomes currently reported in publications evaluating the effectiveness of hospital-to-home transitional care interventions for CMC. We searched the following databases: Medline, Embase, Cochrane library, CINAHL, PsychInfo, and Web of Science for studies published between 1 January 2010 and 15 March 2023.

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An ESPGHAN Position Paper on the Use of Low-FODMAP Diet in Pediatric Gastroenterology.

J Pediatr Gastroenterol Nutr

September 2022

the Department of Paediatric Gastroenterology and Nutrition, Emma Children's Hospital, Amsterdam University Medical Centres, Amsterdam, The Netherlands.

Excluding oligo-, di-, monosaccharides and polyols (FODMAPs) from the diet is increasingly being used to treat children with gastrointestinal complaints. The aim of this position paper is to review the available evidence on the safety and efficacy of its use in children and provide expert guidance regarding practical aspects in case its use is considered . Members of the Gastroenterology Committee, the Nutrition Committee and the Allied Health Professionals Committee of the European Society for Pediatric Gastroenterology Hepatology and Nutrition contributed to this position paper.

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Background: Patients with Sturge-Weber syndrome type 1 (SWS1) have a port-wine birthmark (PWB) as cutaneous hallmark. Up to 35% of neonates with a high risk PWB develop SWS1. Clinical manifestations are severe and often progressive.

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Background: Currently, we cannot predict whether a pre-school child with asthma-like symptoms will have asthma at school age. Whether genetic information can help in this prediction depends on the role of genetic factors in persistence of pre-school to school-age asthma. We examined to what extent genetic and environmental factors contribute to persistence of asthma-like symptoms at ages 3 to asthma at age 7 using a bivariate genetic model for longitudinal twin data.

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Background: There are sparse real-world data on severe asthma exacerbations (SAE) in children. This multinational cohort study assessed the incidence of and risk factors for SAE and the incidence of asthma-related rehospitalization in children with asthma.

Methods: Asthma patients 5-17 years old with ≥1 year of follow-up were identified in six European electronic databases from the Netherlands, Italy, the UK, Denmark and Spain in 2008-2013.

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CRISPR-Based Adenine Editors Correct Nonsense Mutations in a Cystic Fibrosis Organoid Biobank.

Cell Stem Cell

April 2020

Hubrecht Institute, Royal Netherlands Academy of Arts and Sciences (KNAW) and University Medical Center Utrecht, 3584 CT Utrecht, the Netherlands; Oncode Institute, Hubrecht Institute, 3584 CT Utrecht, the Netherlands. Electronic address:

Adenine base editing (ABE) enables enzymatic conversion from A-T into G-C base pairs. ABE holds promise for clinical application, as it does not depend on the introduction of double-strand breaks, contrary to conventional CRISPR/Cas9-mediated genome engineering. Here, we describe a cystic fibrosis (CF) intestinal organoid biobank, representing 664 patients, of which ~20% can theoretically be repaired by ABE.

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R117H-CFTR function and response to VX-770 correlate with mRNA and protein expression in intestinal organoids.

J Cyst Fibros

September 2020

Department of Pediatric Pulmonology, Wilhelmina Children's Hospital, University Medical Center, Utrecht University, Utrecht, the Netherlands, member of ERN-LUNG; Regenerative Medicine Center Utrecht, University Medical Center Utrecht, Utrecht, the Netherlands. Electronic address:

Introduction: Variability in disease severity and CFTR modulator responses exists between patients with identical CFTR genotypes. Here, we characterized transcription, translation and function of R117H-CFTR using intestinal organoids and correlated them with in vitro responses to ivacaftor (VX-770).

Methods: Organoids were generated from individuals possessing at least one R117H-CFTR allele.

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Background: Concern over potential neurotoxicity of anesthetics has led to growing interest in prospective clinical trials using potentially less toxic anesthetic regimens, especially for prolonged anesthesia in infants. Preclinical studies suggest that dexmedetomidine may have a reduced neurotoxic profile compared to other conventional anesthetic regimens; however, coadministration with either anesthetic drugs (eg, remifentanil) and/or regional blockade is required to achieve adequate anesthesia for surgery. The feasibility of this pharmacological approach is unknown.

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The incidence of venous thromboembolism (VTE) in children is rising. Hence, there is an increasing off-label use of low-molecular-weight heparin (LMWH). There is little data about therapeutic and prophylactic LWMH dosages, and their safety and efficacy.

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Trends in paediatric inpatient antibiotic therapy in a secondary care setting.

Eur J Pediatr

August 2018

Department of Pediatrics, Franciscus Gasthuis, Kleiweg 500, 3045 PM, Rotterdam, the Netherlands.

Unlabelled: There is growing attention for antimicrobial stewardship in paediatrics. Currently, little is known about secondary care antibiotic practice. We analysed trends in time with respect to inpatient antibiotic use in a secondary paediatric care setting.

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Background:  Epidural analgesia (EDA) is used as postoperative pain treatment in children, but little is known about its efficacy after laparotomy in children. This retrospective study investigated the efficacy of postoperative EDA after laparotomy in children and the frequencies of adverse effects and complications.

Materials And Methods:  Data of all children aged 0 to 18 years undergoing laparotomy and treated with EDA in our hospital from January 2014 to December 2015 were collected.

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For the surgical treatment of Hirschsprung's disease, several surgical techniques are used to resect the distal aganglionic colon. Two frequently used techniques are the Duhamel procedure and the transanal endorectal pull-through procedure. During the '8th Pediatric Colorectal Course' in Nijmegen, November 2015, a workshop was organized to share experiences of both techniques by several experts in the field and to discuss (long term) outcomes.

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Optimal nutrition in the paediatric ICU.

Curr Opin Clin Nutr Metab Care

March 2016

ErasmusMC-Sophia Children's Hospital, Department of Paediatric Intensive Care, Dr Molewaterplein 60, 3015 GJ Rotterdam, The Netherlands.

Purpose Of Review: This article describes the current best available evidence on optimal nutrition in the paediatric intensive care based on different levels of outcome, which can be divided in surrogate and hard clinical outcome parameters.

Recent Findings: Undernutrition is associated with increased morbidity and mortality, whereas in specific cohorts of critically ill children, such as those with burn injury, obesity is associated with more complications, longer length of stay, and decreased likelihood of survival. There is a relation with adequacy of delivery of enteral nutrition and the amount of protein on length of hospital stay, neurological status, and mortality.

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The increased presence of sialylated glycans on the tumor surface has been linked to poor prognosis, yet the effects on tumor-specific T cell immunity are hardly studied. We here show that hypersialylation of B16 melanoma substantially influences tumor growth by preventing the formation of effector T cells and facilitating the presence of high regulatory T cell (Treg) frequencies. Knock-down of the sialic acid transporter created "sialic acid low" tumors, that grew slower in-vivo than hypersialylated tumors, altered the Treg/Teffector balance, favoring immunological tumor control.

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Context: Follow-up strategies after emergency department (ED) discharge, alias safety netting, is often based on the gut feeling of the attending physician.

Objective: To systematically identify evaluated safety-netting strategies after ED discharge and to describe determinants of paediatric ED revisits.

Data Sources: MEDLINE, Embase, CINAHL, Cochrane central, OvidSP, Web of Science, Google Scholar, PubMed.

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Objectives: To assess the impact of a clinical decision model for febrile children at risk for serious bacterial infections (SBI) attending the emergency department (ED).

Methods: Randomized controlled trial with 439 febrile children, aged 1 month-16 years, attending the pediatric ED of a Dutch university hospital during 2010-2012. Febrile children were randomly assigned to the intervention (clinical decision model; n = 219) or the control group (usual care; n = 220).

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Background: Clinical prediction rules (CPRs) to identify children with serious infections lack validation in low-prevalence populations, which hampers their implementation in primary care practice.

Aim: To evaluate the diagnostic value of published CPRs for febrile children in primary care.

Design And Setting: Observational cohort study among febrile children (<16 years) who consulted five GP cooperatives (GPCs) in the Netherlands.

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Juvenile myelomonocytic leukemia is a rare myeloproliferative disease in young children. While hematopoietic stem cell transplantation remains the only curative therapeutic option for most patients, children with juvenile myelomonocytic leukemia increasingly receive novel agents in phase I-II clinical trials as pre-transplant therapy or therapy for relapse after transplantation. However, response criteria or definitions of outcome for standardized evaluation of treatment effect in patients with juvenile myelomonocytic leukemia are currently lacking.

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Impact of childhood obesity on cardiac structure and function.

Curr Treat Options Cardiovasc Med

November 2014

Department of Pediatric Cardiology, ErasmusMC/Sophia Children's Hospital Rotterdam, P.O. Box 2060, 3000 CB, Rotterdam, The Netherlands,

Obesity prevalence is still on the rise worldwide in both adults and children. In adults, obesity is a significant cardiovascular risk factor associated with increased morbidity and mortality. In children and adolescents this is more controversial, but early changes in cardiovascular function have been observed with possible long-term implications.

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Vocational challenges in congenital heart disease.

Neth Heart J

May 2014

Department of Pediatrics, Division of Cardiology, Sp-2429, ErasmusMC-Sophia Children's Hospital, PO Box 2060, 3000 CB, Rotterdam, the Netherlands,

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