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

Background: Inherited metabolic diseases (IMDs) may have considerable implications for patients and their families. Despite their individual rarity, covering a spectrum of over 1800 distinct diseases, the diseases collectively exert a significant impact, with often lifelong disabilities. The United for Metabolic Diseases consortium was established to catalyze research with translation into the best possible care.

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Objectives: Juvenile idiopathic arthritis (JIA) is the most common type of arthritis among children. It can cause joint pain and permanent physical damage, which affects mobility and daily activities. The EQ-5D-Y-3L self-report version has been validated in JIA, but the validity of EQ-5D-Y-5L remains unknown.

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Objective: The OMERACT Juvenile Idiopathic Arthritis (JIA) Working Group (WG) aimed to reach agreement on a consensus-based definition and description of the core domain related to patient perception of overall well-being and disease activity.

Methods: A committee of patient research partners, clinicians, methodologists, and researchers drafted working definitions and descriptions. The WG conducted two iterative electronic stakeholder surveys to obtain consensus on domain description, definition, and the distinction between patient perception of overall well-being and disease activity.

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Article Synopsis
  • Overweight and obesity are prevalent issues among childhood cancer survivors, affecting nearly half of those studied, with higher rates among brain tumor survivors and older women.
  • The study assessed various measures of overweight, including body mass index (BMI), waist circumference, and fat percentage, revealing that traditional BMI metrics might underestimate the problem, particularly in survivors who underwent certain treatments.
  • Key factors linked to overweight included being overweight at diagnosis, receiving cranial radiotherapy, and having growth hormone deficiency, with dual-energy X-ray absorptiometry identifying an additional 30% at risk.
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Objectives: Care for JIA patients has been transformed in the biologics era; however, biologics carry important (although rare) risks and are costly. Flares after biological withdrawal are seen frequently, yet there is little clinical guidance to identify which patients in clinical remission can safely have their biologic discontinued (by stopping or tapering). We examined what characteristics of the child or their context are important to pediatric rheumatologists when making the decision to discuss withdrawal of biologics.

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Article Synopsis
  • The study explores how various tumor segmentation strategies in diffusion-weighted MRI affect the apparent diffusion coefficient (ADC) in pediatric rhabdomyosarcoma patients.
  • A review of 76 articles identified common segmentation strategies, with findings showing significant variation in methods used to define regions of interest, volume, and areas excluded during the process.
  • Results indicated that excluding certain tumor areas led to lower ADC measurements, emphasizing the need for standardized segmentation methods in future MRI studies to improve treatment response monitoring.
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Factors associated with care- and health-related quality of life of caregivers of children with juvenile idiopathic arthritis.

Pediatr Rheumatol Online J

July 2022

Department of Community Health Sciences, Cumming School of Medicine, University of Calgary, Room 3C56, Health Research Innovation Centre, 3280 Hospital Drive NW, Calgary, AB, T2N 4Z6, Canada.

Objective: This study investigates the relationship of child, caregiver, and caring context measurements with the care-related quality of life (CRQoL) and health-related quality of life (HRQoL) of caregivers of children with juvenile idiopathic arthritis (JIA).

Methods: We performed a cross-sectional analysis of baseline data on caregivers of children with JIA from Canada and the Netherlands collected for the "Canada-Netherlands Personalized Medicine Network in Childhood Arthritis and Rheumatic Diseases" study from June 2019 to September 2021. We used the CRQoL questionnaire (CarerQoL), adult EQ-5D-5L, and proxy-reported Youth 5-Level version of EuroQoL (EQ-5D-5L-Y) to assess caregiver CRQoL, caregiver HRQoL, and child HRQoL, respectively.

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Background: Few studies have examined pediatric rheumatologists' approaches to treatment decision making for biologic therapy for patients with juvenile idiopathic arthritis (JIA). This study presents the qualitative research undertaken to support the development of a Best-Worst Scaling (BWS) survey for tapering in JIA. The study objectives were to (1) describe the treatment decision-making process of pediatric rheumatologists to initiate and taper biologics; and (2) select attributes for a BWS survey.

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Purpose: Arterio-ureteral fistula (AUF) is an uncommon diagnosis, but potentially lethal. Although the number of reports has increased over the past two decades, the true incidence and contemporary urologists' experience and approach in clinical practice remains unknown. This research is conducted to provide insight in the incidence of AUF in The Netherlands, and the applied diagnostic tests and therapeutic approaches in modern practice.

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Purpose: Arterio-ureteral fistula (AUF) is an uncommon diagnosis, but increasingly reported and potentially lethal. This systematic review comprehensively presents risk factors, pathophysiology, location and clinical presentation of AUF aiming to increase clinical awareness of this rare but life-threatening condition, and to put this entity into a contemporary perspective with modern diagnostic tools and treatment strategies.

Materials And Methods: This review was performed according to the PRISMA (Preferred Reporting Items for a Systematic Review and Meta-Analysis of Individual Participant Data) guidelines.

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A Retrospective Analysis of Female Müllerian Duct Anomalies in Association With Congenital Renal Abnormalities.

J Pediatr Adolesc Gynecol

October 2021

Department of Pediatric Urology, Wilhelmina Children's Hospital UMC Utrecht, Utrecht, the Netherlands; Department of Pediatric Surgery, Division of Pediatric Urology and Andrology, I.M. Sechenov First Moscow State Medical University, Moscow, Russia.

Study Objective: Müllerian (paramesonephric) duct anomalies (MDAs) are associated with several coexisting congenital abnormalities, including renal abnormalities. Although congenital renal abnormalities may remain asymptomatic, the consequences should not be underestimated. In both the literature and clinical practice, it remains necessary to improve awareness of the co-occurrence of different congenital renal abnormalities in women with MDAs.

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Objectives: Complete deficiency of alternative pathway (AP) complement factors, explained by homozygous mutations, is a well-known risk factor for invasive bacterial infections; however, this is less obvious for heterozygous mutations. We describe two siblings with a heterozygous NM_001928.3(CFD):c.

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Introduction: The adolescent years represent a key period for the development of musculoskeletal complaints (MSC) and the differences between boys and girls. We evaluated the prevalence and course of MSC and factors associated with MSC while growing up from age 11 to age 20.

Methods: Questionnaire-based data at age 11 (n = 2,638), age 14 (n = 2,517), age 17 (n = 2,094) and at age 20 (n = 2,206) from the ongoing Prevention and Incidence of Asthma and Mite Allergy (PIAMA) birth cohort were analyzed.

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The striking difference between men and women in headache prevalence is suggested to develop in adolescence. Although headaches are common and affect quality of life and daily functioning, the evidence needed to develop effective counselling and preventive approaches is still limited. Using data collected at age 11, 14, 17, and 20 years in the Dutch Prevention and Incidence of Asthma and Mite Allergy birth cohort study (n = 3064 with ≥ 1 questionnaire), we assessed headache prevalence and incidence in girls and boys and explored associations with early life, environmental, lifestyle, health, and psychosocial factors.

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Introduction: Enuresis is a common problem in children. One treatment option is a wetting alarm that provides an alarm when incontinence occurs. A drawback of this approach is that the child is still awakened by wet sheets.

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SENS-U: clinical evaluation of a full-bladder notification - a pilot study.

J Pediatr Urol

August 2019

Department of Pediatric Urology, Wilhelmina Children's Hospital UMC Utrecht, PO Box 85090, 3508 AB Utrecht, the Netherlands; Department of Pediatric Urology and Andrology, Sechenov Moscow State Medical University, Moscow, Russia.

Introduction: Urinary incontinence is one of the most commonly treated disorders in children at school age. Recently, a new, wearable bladder sensor became available, the SENS-U Bladder Sensor. The SENS-U is a small, wireless ultrasonic sensor, which continuously monitors the bladder filling and provides a personalized notification when it is time to go to the toilet.

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Article Synopsis
  • The SENS-U Bladder Sensor is a wearable device designed to help children who experience urinary incontinence by notifying them when they need to use the restroom.
  • In a study involving 30 children aged 6-12, the SENS-U successfully detected bladder fullness in 90% of cases during urodynamic tests, with a strong correlation between bladder size and infused volume.
  • The study suggests that the SENS-U could improve bladder awareness in children, and future research may explore its effectiveness in incontinence training.
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The translation of basic insight in immunological mechanisms underlying inflammation into clinical practice of inflammatory diseases is still challenging. Here we describe how-through continuous dialogue between bench and bedside-immunological knowledge translates into tangible clinical use in a complex inflammatory disease, juvenile idiopathic arthritis (JIA). Systemic JIA (sJIA) is an autoinflammatory disease, leading to the very successful use of IL-1 antagonists.

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Objective: To improve the efficacy of newborn screening (NBS) for very long chain acyl-CoA dehydrogenase deficiency (VLCADD).

Patients And Methods: Data on all dried blood spots collected by the Dutch NBS from October 2007 to 2010 (742.728) were included.

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Mesenchymal stromal cells for treatment of arthritis.

Best Pract Res Clin Rheumatol

August 2014

Pediatric Immunology, Wilhelmina Children's Hospital/UMC Utrecht, Netherlands. Electronic address:

Patients with refractory inflammatory arthritis can still respond favourable to autologous haematopoietic stem cell transplantation. However, this treatment has a high morbidity and even 5% mortality. Mesenchymal stromal cells (MSC), a subset of the non-haematopoietic stromal cells obtained from bone marrow, were found to have a strong immunosuppressive effect.

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