423 results match your criteria: "University Medical Center-Sophia Children's Hospital[Affiliation]"

Article Synopsis
  • Growth hormone (GH) treatment is generally safe, but there have been concerns regarding its long-term mortality risk in adults.
  • A study involving data from 676 clinics assessed the long-term safety of GH by examining adverse drug reactions (ADRs) and serious adverse events (SAEs) in pediatric patients categorized into low, intermediate, and high-risk groups.
  • Results showed no increased mortality risk or higher incidence of adverse events linked to GH dosage across any risk category, indicating that GH treatment remains safe over time.
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Background: In prehospital care, the Helicopter Emergency Medical Service (HEMS) can be dispatched for critically injured or ill children. However, little detail is known about dispatches for children, in terms of the incidence of prehospital interventions and overall mortality. The primary objective of this study is to provide an overview of pediatric patient characteristics and incidence of interventions.

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Objective: Leukocyte telomere length (LTL) is one of the markers of biological aging as shortening occurs over time. Shorter LTL has been associated with adiposity and a higher risk of cardiovascular diseases. The objective was to assess LTL and LTL shortening during the first 2 years of life in healthy, term-born infants and to associate LTL shortening with potential stressors and body composition.

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Background:  The pharmacokinetic (PK) properties of extended half-life (EHL) factor VIII (FVIII) concentrates differ, leading to variation in the optimal dosing regimen for the individual patient. The aim of this study was to establish these PK differences for various EHL FVIII concentrates by in silico simulations.

Methods:  FVIII level over time profiles of rFVIII-SC, BAY 81-8973, rFVIII-Fc, BAX 855, BAY 94-9027, and standard half-life (SHL) rFVIII concentrates were simulated for 1,000 severe hemophilia A patients during steady-state dosing of 40 IU/kg every 72 hours or dosing as advised in the summary of product characteristics (SmPC).

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Back to base pairs: What is the genetic risk for red bloodcell alloimmunization?

Blood Rev

July 2021

Amsterdam UMC, University of Amsterdam, Emma Children's Hospital, Pediatric Hematology, Amsterdam, the Netherlands; Leiden University Medical Center, Department of Clinical Epidemiology, Leiden, the Netherlands. Electronic address:

Red blood cell (RBC) alloimmunization is a serious complication of blood transfusions, challenging selection of compatible units for future transfusions. Genetic characteristics may be associated with the risk of RBC alloimmunization and may therefore serve to identify high-risk patients. The aim of this systematic review was to summarize the available evidence on genetic risk factors for RBC alloimmunization.

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Association of inflammatory biomarkers with subsequent clinical course in suspected late onset sepsis in preterm neonates.

Crit Care

January 2021

Department of Pediatrics, Division Neonatology, Erasmus Medical Center, Erasmus MC, University Medical Center-Sophia Children's Hospital, Research Neonatology (Sk-4246), PO Box 2060, 300 CB, Rotterdam, The Netherlands.

Background: Sepsis is a major health issue in preterm infants. Biomarkers are used to diagnose and monitor patients with sepsis, but C-reactive protein (CRP) is proven not predictive at onset of late onset neonatal sepsis (LONS) diagnosis. The aim of this study was to evaluate the association of interleukin-6(IL-6), procalcitonin (PCT) and CRP with subsequent sepsis severity and mortality in preterm infants suspected of late onset neonatal sepsis.

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Coronavirus entry is mediated by the spike protein that binds the receptor and mediates fusion after cleavage by host proteases. The proteases that mediate entry differ between cell lines, and it is currently unclear which proteases are relevant in vivo. A remarkable feature of the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) spike is the presence of a multibasic cleavage site (MBCS), which is absent in the SARS-CoV spike.

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Article Synopsis
  • Identifying monogenic IBD patients is essential for tailored management, and the position statement provides recommendations for using genomics in this evaluation across different age groups.
  • A systematic review by pediatric IBD specialists led to recommendations for next-generation DNA sequencing in routine clinical practice, but routine genetic testing is advised only for specific cases based on age of onset and family history.
  • A diagnostic algorithm with a gene panel of 75 genes has been developed to assist clinicians, emphasizing the importance of genetic testing before procedures like hematopoietic stem cell transplantation.
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Longitudinal human milk macronutrients, body composition and infant appetite during early life.

Clin Nutr

May 2021

Department of Pediatrics, Subdivision of Endocrinology, Erasmus University Medical Center - Sophia Children's Hospital, Rotterdam, the Netherlands; Dutch Growth Research Foundation, Rotterdam, the Netherlands.

Background & Aims: Breastfeeding is the gold standard infant feeding. Data on macronutrients in relation to longitudinal body composition and appetite are very scarce. The aim of this study was to investigate longitudinal human milk macronutrients at 1 and 3 months in association with body composition and appetite during early life in healthy, term-born infants.

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Background/purpose: Coagulation complications are frequent, unwanted occurrences in extracorporeal membrane oxygenation (ECMO) treatment, possibly influenced by the pump in the ECMO-circuit. We hypothesized that fewer complications would occur with a smaller, heparin-coated ECMO system with a centrifugal pump (CP) than with one with a roller pump (RP) and that after conversion, complication rates would decrease over time.

Methods: This single-center, retrospective chart study included all first neonatal and pediatric ECMO runs between 2009 and 2015.

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Aims: Under- and, especially, overdosing of replacement therapy in haemophilia A patients may be prevented by application of other morphometric variables than body weight (BW) to dose factor VIII (FVIII) concentrates. Therefore, we aimed to investigate which morphometric variables best describe interindividual variability (IIV) of FVIII concentrate pharmacokinetic (PK) parameters.

Methods: PK profiling was performed by measuring 3 FVIII levels after a standardized dose of 50 IU kg FVIII concentrate.

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Objective: Emergency medical service (EMS) is responsible for prehospital care encompassing all ages, irrespective of injury cause or medical condition, which includes peripartum emergencies. When patients require care more advanced than the level provided by the national EMS protocol, an EMS physician-staffed Dutch helicopter emergency medical service (HEMS) may be dispatched. In the Netherlands in 2016, there were 21.

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Esophageal atresia (EA) and tracheoesophageal fistula (TEF) are relatively frequently occurring foregut malformations. EA/TEF is thought to have a strong genetic component. Not much is known regarding the biological processes disturbed or which cell type is affected in patients.

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3D alveolar in vitro model based on epithelialized biomimetically curved culture membranes.

Biomaterials

January 2021

MERLN Institute for Technology-Inspired Regenerative Medicine, Maastricht University, Universiteitssingel 40, 6229 ER, Maastricht, the Netherlands. Electronic address:

There is increasing evidence that surface curvature at a near-cell-scale influences cell behaviour. Epithelial or endothelial cells lining small acinar or tubular body lumens, as those of the alveoli or blood vessels, experience such highly curved surfaces. In contrast, the most commonly used culture substrates for in vitro modelling of these human tissue barriers, ion track-etched membranes, offer only flat surfaces.

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Background: Latinx immigrants are exposed to multiple stressors before, during, and after migration. However, most past research has assumed the effects of these stressors are uniform across Latinx groups despite considerable within-group variation. The purpose of this study was to (a) assess the moderating effects of several risk and protective factors on the association between cumulative lifetime adversity and depression among U.

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Objectives: To compare the pharmacokinetics and pharmacodynamics of IV midazolam after cardiac surgery between children with and without Down syndrome.

Design: Prospective, single-center observational trial.

Setting: PICU in a university-affiliated pediatric teaching hospital.

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Intelligence, educational attainment, and brain structure in those at familial high-risk for schizophrenia or bipolar disorder.

Hum Brain Mapp

January 2022

Department of Psychiatry, University Medical Center Utrecht Brain Center, University Medical Center Utrecht, Utrecht University, Utrecht, Netherlands.

Article Synopsis
  • First-degree relatives of schizophrenia patients (SZ-FDRs) exhibit similar brain and cognitive abnormalities as patients, while those related to bipolar disorder (BD-FDRs) show larger brain volume but inconsistent cognitive patterns.
  • A meta-analysis involving nearly 6,000 participants found that SZ-FDRs have a thinner cortex and lower IQ scores compared to controls, whereas BD-FDRs show a larger cortical surface area with only slight IQ reductions.
  • Both relative groups achieved similar educational levels, indicating that structural brain differences are likely connected to the predisposition for schizophrenia or bipolar disorder rather than general cognitive impairments.
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Current monitoring techniques in neonates lack sensitivity for hypoxia at cellular level. The recent introduction of the non-invasive Cellular Oxygen METabolism (COMET) monitor enables measuring mitochondrial oxygen tension (mitoPO), based on oxygen-dependent quenching of delayed fluorescence of 5-aminolevulinic acid (ALA)-enhanced protoporphyrin IX. The aim is to determine the feasibility and safety of non-invasive mitoPO monitoring in surgical newborns.

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Objectives: Advances in genetic technologies provide opportunities for patient care and ethical challenges. Clinical care of patients with rare Mendelian disorders is often at the forefront of those developments. Whereas in classical polygenic inflammatory bowel disease (IBD), the predictive value of genetic variants is very low, predictive prenatal genetic diagnosis can inform families at high risk of severe genetic disorders.

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Rationale: Severe acute asthma (SAA) can be fatal, but is often preventable. We previously observed in a retrospective cohort study, a three-fold increase in SAA paediatric intensive care (PICU) admissions between 2003 and 2013 in the Netherlands, with a significant increase during those years of numbers of children without treatment of inhaled corticosteroids (ICS).

Objectives: To determine whether steroid-naïve children are at higher risk of PICU admission among those hospitalised for SAA.

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Quality of life after esophageal replacement in children.

J Pediatr Surg

February 2021

Department of Pediatric Surgery, Wilhelmina Children's Hospital, University Medical Center Utrecht, P.O. Box 85090, 3508, AB, Utrecht, The Netherlands.

Purpose: Assessing quality of life (QoL) after esophageal replacement (ER) for long gap esophageal atresia (LGEA).

Methods: All patients after ER for LGEA with gastric pull-up (GPU n = 9) or jejunum interposition (JI n = 14) at the University Medical Center Groningen and Utrecht (1985-2007) were included. QoL was assessed with 1) gastrointestinal-related QoL using the Gastrointestinal Quality of Life Index (GIQLI)), 2) general QoL (Child Health questionnaire CHF87-BREF (children)/World Health Organization questionnaire WHOQOL-BREF (adults)), and 3) health-related QoL (HRQoL) (TNO AZL TACQoL/TAAQoL).

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Bipolar disorder (BD) is a heterogeneous disorder that contains neurodevelopmental differences. Defining homogeneous subgroups of BD patients by using age at onset (AAO) as a specifier may promote the classification of biomarkers. This study compares peripheral BDNF levels between pediatric and adult BD patients to investigate the associations between BDNF levels, AAO, and illness duration.

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The aim of this study is to develop a tool that is aligned with patients' and health professionals' needs to address sexual health in the context of anorectal malformations and Hirschsprung's disease. A multiphased participatory action-research was conducted. First, an inventory of needs was made through interviews (11 patients, 11 professionals), three online focus groups (4 patients, 20 professionals), and a questionnaire (38 patients).

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Exploring copy number variants in deceased fetuses and neonates with abnormal vertebral patterns and cervical ribs.

Birth Defects Res

November 2020

Department of Obstetrics and Gynecology, Division of Obstetrics and Fetal Medicine, Erasmus MC, University Medical Center Rotterdam, Rotterdam, The Netherlands.

Background: Cervical patterning abnormalities are rare in the general population, but one variant, cervical ribs, is particularly common in deceased fetuses and neonates. The discrepancy between the incidence in the general population and early mortality is likely due to indirect selection against cervical ribs. The cause for the co-occurrence of cervical ribs and adverse outcome remains unidentified.

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