58 results match your criteria: "Wilhelmina Children's Hospital Utrecht[Affiliation]"

Complex health challenges require professionals to operate across disciplines and to better connect with society. Here, we showcase a community-engaged and challenge-based educational model in which undergraduate students conduct transdisciplinary research on authentic complex biomedical problems. This concept reinforces translational medicine, human capital, and exemplifies synergy between education, research, healthcare, and society.

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Brain MRI Injury Patterns across Gestational Age among Preterm Infants with Perinatal Asphyxia.

Neonatology

October 2024

Department of Neonatology, Wilhelmina Children's Hospital Utrecht and Utrecht Brain Center, University Medical Center Utrecht, Utrecht University, Utrecht, The Netherlands.

Introduction: Brain injury patterns of preterm infants with perinatal asphyxia (PA) are underreported. We aimed to explore brain magnetic resonance imaging (MRI) findings and associated neurodevelopmental outcomes in these newborns.

Methods: Retrospective multicenter study included infants with gestational age (GA) 24.

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Background: Children treated for a malignancy are at risk to develop serious illness from a COVID-19 infection. Pegylated asparaginase (PEG-asparaginase) is used in the treatment of acute lymphoblastic leukemia. Allergy to this drug is common and both asparaginase and polyethylene glycol (PEG) are identified as possible antigens.

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Objectives: Wearable alarm systems are frequently used tools added to urotherapy for children with both daytime and nighttime urinary incontinence. For functional daytime incontinence (DUI) specifically, the effect of alarm interventions has not been systematically reviewed. This study systematically evaluates, summarizes, reviews, and analyzes existing evidence about the effect of wearable alarm systems in urotherapy for children with functional DUI.

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Objective: To explore and compare the contents and scores of the Assessment of Life Habits (Life-H) with the Utrecht Scale for Evaluation of Rehabilitation-Participation (USER-Participation) in adolescents with cerebral palsy.

Design: Youth versions of both instruments were used for (1) content comparison and (2) analyses of relations between both instruments, based on cross-sectional data.

Setting: Clinic.

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Background: Lower urinary tract dysfunction or functional urinary incontinence is a common condition with a prevalence up to 21% between 6 and 8 year-old children. It is associated with an impaired quality of life, lower self-esteem, and social stigmatization. Urotherapy is the first treatment of choice for functional daytime urinary incontinence (DUI) in children.

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Background Plasma biomarkers may aid in the detection of anthracycline-related cardiomyopathy (ACMP). However, the currently available biomarkers have limited diagnostic value in long-term childhood cancer survivors. This study sought to identify diagnostic plasma biomarkers for ACMP in childhood cancer survivors.

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Systemic juvenile idiopathic arthritis (sJIA, also called Still's disease) is a rare childhood auto-inflammatory disease with significant morbidity. This case report illustrates the clinical course and highlights diagnostic challenges. FDG-PET/CT imaging may be beneficial in the diagnostic process for some cases, in order to achieve rapid diagnosis and early treatment.

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Urinary Tract Infections (UTIs) are among the most frequently occurring infections in the hospital. Urinalysis and urine culture are the main tools used for diagnosis. Whereas urinalysis is sufficiently sensitive for detecting UTI, it has a relatively low specificity, leading to unnecessary treatment with antibiotics and the risk of increasing antibiotic resistance.

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Background The long-term burden of cardiovascular disease after repair of coarctation of the aorta (CoA) has not been elucidated. We aimed to determine the incidence of and risk factors for cardiovascular events in adult patients with repaired CoA. Additionally, mortality rates were compared between adults with repaired CoA and the general population.

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Synthetic MRI of Preterm Infants at Term-Equivalent Age: Evaluation of Diagnostic Image Quality and Automated Brain Volume Segmentation.

AJNR Am J Neuroradiol

May 2020

From the Department of Radiology (T.V., M.N., N.W., G.-J.A., S.R., J.d.M., H.R.), Vrije Universiteit Brussels, Universitair Ziekenhuis Brussels, Brussels, Belgium.

Background And Purpose: Neonatal MR imaging brain volume measurements can be used as biomarkers for long-term neurodevelopmental outcome, but quantitative volumetric MR imaging data are not usually available during routine radiologic evaluation. In the current study, the feasibility of automated quantitative brain volumetry and image reconstruction via synthetic MR imaging in very preterm infants was investigated.

Materials And Methods: Conventional and synthetic T1WIs and T2WIs from 111 very preterm infants were acquired at term-equivalent age.

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Contrast-enhanced ultrasonography (US) has become an important supplementary tool in many clinical applications in children. Contrast-enhanced voiding urosonography and intravenous US contrast agents have proved useful in routine clinical practice. Other applications of intracavitary contrast-enhanced US, particularly in children, have not been widely investigated but could serve as a practical and radiation-free problem-solver in several clinical settings.

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NGLY1 encodes the enzyme N-glycanase that is involved in the degradation of glycoproteins as part of the endoplasmatic reticulum-associated degradation pathway. Variants in this gene have been described to cause a multisystem disease characterized by neuromotor impairment, neuropathy, intellectual disability, and dysmorphic features. Here, we describe four patients with pathogenic variants in NGLY1.

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Distinct interferon signatures and cytokine patterns define additional systemic autoinflammatory diseases.

J Clin Invest

April 2020

Translational Autoinflammatory Diseases Section (TADS), NIAID/NIH, Bethesda, Maryland, USA.

BACKGROUNDUndifferentiated systemic autoinflammatory diseases (USAIDs) present diagnostic and therapeutic challenges. Chronic interferon (IFN) signaling and cytokine dysregulation may identify diseases with available targeted treatments.METHODSSixty-six consecutively referred USAID patients underwent underwent screening for the presence of an interferon signature using a standardized type-I IFN-response-gene score (IRG-S), cytokine profiling, and genetic evaluation by next-generation sequencing.

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Instruments Measuring Physical Activity in Individuals Who Use a Wheelchair: A Systematic Review of Measurement Properties.

Arch Phys Med Rehabil

March 2020

Research Group Lifestyle and Health, Hogeschool Utrecht, University of Applied Sciences, Utrecht; Shared Utrecht Pediatric Exercise Laboratory, Utrecht; Netherlands Institute for Health Services Research, Utrecht, the Netherlands.

Objective: To systematically review the evidence evaluating validity or reliability of self-reported and device-based instruments, to measure physical activity (PA) in individuals who use a wheelchair, and to make recommendations for the selection of PA outcomes tools.

Data Sources: PubMed, Embase, and CINAHL were systematically searched.

Study Selection: Studies reporting measurement properties of instruments to assess PA in individuals who use a wheelchair.

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The above article was published online with incorrect author name. The right spelling should be Damjana Kljucevsek instead of Damjana Kjucevsek. The correct name is presented here.

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Very early onset inflammatory bowel disease (VEO-IBD) is defined as disease presenting before the age of 6. These children require a tailored imaging approach because conventional imaging studies can be difficult to perform at such a young age. Unlike inflammatory bowel disease in older children and adults, colonic disease predominates in VEO-IBD, and small-bowel disease is rare.

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Interstitial lung disease (ILD) is highly prevalent in collagen vascular diseases and reduction of ILD is an important therapeutic target. To that end, reliable quantification of pulmonary disease severity is of great significance. This study systematically reviewed the literature on automated computed tomography (CT) quantification methods for assessing ILD in collagen vascular diseases.

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The triad of micrognathia, glossoptosis, and concomitant airway obstruction defined as "Robin sequence" (RS) is caused by oropharyngeal developmental events constrained by a reduced stomadeal space. This sequence of abnormal embryonic development also results in an anatomical configuration that might predispose the fetus to a cleft palate. RS is heterogeneous and many different etiologies have been described including syndromic, RS-plus, and isolated forms.

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Introduction: A tubularized conduit from an open 2-cm vascularized ileal segment is a frequently used technique to create a continent catheterizable channel in cases of an inappropriate or absent appendix. In the long term, many patients experience catheterization problems with the classic ileal segment tube, and even more with spiral or double-segment tubes.

Objective: The objective of this paper was to introduce an ileocystoplasty modification combined with a long ileal segment tube that has better support by surrounding tissue than other lengthy ileal segment tubes.

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A specific type of acute brain injury can occur during birth, presenting on ultrasound examination with focal, unilateral, or asymmetrical change in the core of the superior frontal gyri. Ultrasound inspection of the superior gyri near the convexity of the frontal lobe is warranted following mechanically difficult delivery.

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Objective: 3-Methylglutaconic aciduria, dystonia-deafness, hepatopathy, encephalopathy, Leigh-like syndrome (MEGDHEL) syndrome is caused by biallelic variants in SERAC1.

Methods: This multicenter study addressed the course of disease for each organ system. Metabolic, neuroradiological, and genetic findings are reported.

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Objective: To compare long-term results of 3 different techniques used to create continent catheterizable channels (CCCs) in children in a single institution.

Patients And Methods: Data were retrospectively collected from 112 children who had a CCC (appendicovesicostomy [APV], tubularized bladder flap [TBF] or Monti) created between December 1995 and December 2013. Primary outcome was revision-free survival.

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