221 results match your criteria: "Radboudumc Amalia Children's Hospital[Affiliation]"

Anorectal malformations.

Nat Rev Dis Primers

November 2024

Department of Paediatric Surgery, Helsinki University Central Hospital, Helsinki, Finland.

Article Synopsis
  • - Anorectal malformations (ARM) are rare congenital defects occurring in about 3.32 per 10,000 pregnancies, involving a range of issues from mild anal canal displacement to severe fusion of the anorectum, vagina, and urethra.
  • - The developmental basis of ARM stems from improper septation of the hindgut during early pregnancy, with most cases being non-syndromic and lacking clear genetic causes.
  • - Surgical correction, primarily through posterior sagittal anorectoplasty since 1982, is common, but there is a need for further improvements in surgery outcomes, prenatal detection, and understanding the psychosocial impacts on patients.
View Article and Find Full Text PDF

Unexplainable gastrointestinal complaints occasionally occur after Roux-en-Y Gastric Bypass (RYGB) surgery. We therefor investigated the impact of microbiota composition and metabolites on gastrointestinal complaints after RYGB. In the BARICO study (Bariatric surgery Rijnstate and Radboudumc neuroimaging and Cognition in Obesity), microbiota and metabolites were measured before surgery, and 6, and 24 months after surgery.

View Article and Find Full Text PDF

Background: Pediatric kidney transplantations are rarely performed, and there is limited knowledge about the diversity in current clinical practices across Europe. This study aims to explore the utility of clinical snapshot studies in identifying these disparities, establishing a foundation for future snapshot studies and standardization efforts.

Methods: A pilot clinical snapshot study was conducted, with invitations extended to all 109 pediatric kidney transplant centres in Europe.

View Article and Find Full Text PDF
Article Synopsis
  • - Anorectal malformations (ARMs) are uncommon congenital issues affecting the anus and rectum, which require complicated management; European experts are working on unified guidelines for better care.
  • - The guidelines were created based on the Dutch Quality Standard for ARMs and involved a literature review and evaluation by a panel of experts from several European countries, leading to adaptations and new recommendations.
  • - Emphasizing the need for specialized care throughout a patient's life, the guidelines outline essential diagnostic and follow-up processes for children with ARMs to ensure comprehensive management.
View Article and Find Full Text PDF

Introduction:  Being born with an anorectal malformation (ARM) can have profound and lifelong implications for patients and parents. Organization of care and communication between health care providers is an overlooked area of patient care. The European Reference Network eUROGEN for rare and complex urogenital conditions assembled a panel of experts to address these challenges and develop comprehensive guidelines for the management of ARM.

View Article and Find Full Text PDF
Article Synopsis
  • * A group of 15 experts from Europe created guidelines to help doctors and hospitals properly treat patients with ARMs based on new research and existing standards.
  • * The guidelines cover treatments, after-surgery care, toilet training, and managing issues like incontinence, emphasizing the need for a well-coordinated healthcare team.
View Article and Find Full Text PDF

Introduction:  Anorectal malformations (ARMs) are complex congenital anomalies of the anorectal region, oftentimes also affecting the genitourinary system. Although successful surgical correction can often be achieved in the neonatal period, many children will experience functional problems in the long term. The European Reference Network for rare and complex urogenital conditions (eUROGEN) assembled a panel of experts to address these challenges and develop comprehensive guidelines for the management of ARM.

View Article and Find Full Text PDF

The 274th ENMC workshop for optimizing bone strength in neuromuscular disorders (NMDs) was held on January 19-21, 2024. The group of participants included experts in the fields of bone health and neuromuscular medicine along with the patient voice. Bone strength represents a crucial aspect of the management of pediatric and adult patients with NMDs.

View Article and Find Full Text PDF

Objective: When extremely premature birth at the limits of viability is imminent, shared decision-making with parents regarding the infant's treatment is widely recommended. Aligning decisions with parental values can be challenging. So, this study aims to get insight into (1) what values parents considered important in their decision, (2) whether their decision was based on intuition and/or rational analysis and (3) parental suggestions on how to help explore and articulate values during prenatal counselling.

View Article and Find Full Text PDF

Objective: To investigate the perspectives of experienced parents regarding guidelines and personalisation for managing imminent extremely premature births (22-26 weeks gestational age (GA)) . The study examined four scenarios: no guideline, a guideline based on GA, a guideline based on GA plus other factors and a guideline based on a calculated prognosis.

Design: Nineteen semistructured qualitative interviews were conducted with Dutch parents who experienced (imminent) extremely premature births between 23+5 and 26+2 weeks of gestation.

View Article and Find Full Text PDF

Exploring the Value of an Assessment for the Professional Coaching of Residents.

J Grad Med Educ

August 2024

is a Professor, Pediatric Endocrinologist, and Program Director of Pediatric Residency Training, Department of Pediatrics, Radboud University Medical Center, Radboudumc Amalia Children's Hospital, Nijmegen, The Netherlands.

Research on how tools can support coaching of residents is lacking. We hypothesized that an electronic assessment (EA) tool presently applied in selection for residency training, which measures cognitive capacities, personality, motivational drivers, and competencies, could be a valuable tool to support coaching of residents. This study explored the value and limitations, as perceived by residents and coaches, of using the EA to facilitate a single coaching session.

View Article and Find Full Text PDF

Identification of a DNA methylation episignature for recurrent constellations of embryonic malformations.

Am J Hum Genet

August 2024

Verspeeten Clinical Genome Centre, London Health Sciences Centre, London, ON, Canada; Department of Pathology and Laboratory Medicine, Western University, London, ON, Canada. Electronic address:

The term "recurrent constellations of embryonic malformations" (RCEM) is used to describe a number of multiple malformation associations that affect three or more body structures. The causes of these disorders are currently unknown, and no diagnostic marker has been identified. Consequently, providing a definitive diagnosis in suspected individuals is challenging.

View Article and Find Full Text PDF

Impact of Microbiota and Metabolites on Intestinal Integrity and Inflammation in Severe Obesity.

Pharmaceuticals (Basel)

July 2024

Department of Medical Imaging, Anatomy, Radboud University Medical Center, Radboud Alzheimer Center, Donders Institute for Brain Cognition and Behaviour, Center for Medical Neuroscience, 6500 HB Nijmegen, The Netherlands.

Obesity is a multifactorial disease associated with low-grade inflammation. The gut is thought to be involved in obesity-related inflammation, as it is continuously exposed to antigens from food, microbiota and metabolites. However, the exact underlying mechanisms are still unknown.

View Article and Find Full Text PDF
Article Synopsis
  • * Participants were divided into three groups: clinical exposure only, continuous training, and continuous training with self-assessment, with advancements in suturing skills measured through pre- and post-tests.
  • * Results showed all groups improved overall, but the self-assessment group initially performed better, although it did not maintain that advantage after training, suggesting that reflective practices may enhance early surgical skill development.
View Article and Find Full Text PDF

Interdisciplinary assessment and treatment of paediatric drooling: two decades of experience by the Nijmegen saliva control team reflected in a stepwise algorithm.

Eur J Pediatr

September 2024

Department of Paediatric Neurology, Division of Paediatrics, Donders Institute for Brain, Cognition and Behaviour, Radboudumc Amalia Children's Hospital, Radboud University Medical Center, Geert Grooteplein Zuid 10, Nijmegen, the Netherlands.

Unlabelled: Anterior and posterior drooling are prevalent comorbidities in children with neurodevelopmental disabilities. Considering the heterogeneity of the patient population and the multifactorial aetiology of drooling, an interdisciplinary and individualised treatment approach is indispensable. However, no tool for stepwise decision-making in the treatment of paediatric drooling has been developed previously.

View Article and Find Full Text PDF

Background: The aim of this study is to evaluate whether the parameters 'time' and 'distance', measured by SurgTrac, correlate with the assessment of the same skills by blinded experts.

Methods: Basic open suturing tasks were executed by medical students. SurgTrac software measured objective parameters by tracking fingers.

View Article and Find Full Text PDF

Introduction: Proteinuria is a modifiable risk factor for chronic kidney disease (CKD) progression in children. Finerenone, a selective, non-steroidal, mineralocorticoid receptor antagonist (MRA) has been approved to treat adults with CKD associated with type 2 diabetes mellitus (T2DM) following results from the phase III clinical trials FIDELIO-DKD (NCT02540993) and FIGARO-DKD (NCT02545049). In a pre-specified pooled analysis of both studies (N = 13,026), finerenone was shown to have an acceptable safety profile and was efficacious in decreasing the risk of adverse kidney and cardiovascular outcomes and of proteinuria.

View Article and Find Full Text PDF

Nasopharyngeal microbiota in children is associated with severe asthma exacerbations.

J Allergy Clin Immunol

June 2024

Department of Paediatric Immunology and Infectious Diseases, Wilhelmina Children's Hospital/University Medical Center Utrecht, Utrecht, The Netherlands; Centre for Inflammation Research, Institute for Regeneration and Repair, University of Edinburgh, Edinburgh, United Kingdom. Electronic address:

Background: The respiratory microbiome has been associated with the etiology and disease course of asthma.

Objective: We sought to assess the nasopharyngeal microbiota in children with a severe asthma exacerbation and their associations with medication, air quality, and viral infection.

Methods: A cross-sectional study was performed among children aged 2 to 18 years admitted to the medium care unit (MCU; n = 84) or intensive care unit (ICU; n = 78) with an asthma exacerbation.

View Article and Find Full Text PDF

Background: Vesico-ureteral reflux (VUR) is a common associated urological anomaly in anorectal malformation (ARM)-patients. High-grade VUR requires antibiotic prophylaxis to prevent urinary tract infections (UTI's), renal scarring and -failure. The exact prevalence of high-grade VUR in ARM patients is unknown.

View Article and Find Full Text PDF

Purpose: Selection for postgraduate medical training is high-stakes and complex. The learning value of assessments for selection has, thus far, been underexplored, limiting their uptake as potentially meaningful learning experiences. The aim of this study was to explore the learning value residency applicants derive from an intelligence, personality, motivation and competency selection assessment and what factors influence the experienced learning value.

View Article and Find Full Text PDF

Introduction: Learning minimally invasive suturing can be challenging, creating a barrier to further implementation, especially with the development of easier methods. Nevertheless, mastering intracorporeal knot tying is crucial when alternative techniques prove inadequate. Therefore, the minimally invasive surgery (MIS) suturing skills of MIS experts are compared with a group of novices during their learning curve on a simulator.

View Article and Find Full Text PDF

Background: To deliver high-quality collaborative care, residents need to be trained across the boundaries of their medical specialty (intraprofessional learning). The current literature does not provide insights into the underlying processes that influence intraprofessional learning. The aim of this study was to gain insight into the processes that occur during intraprofessional workplace learning in residency training, by exploring everyday intraprofessional interactions experienced by residents, with the ultimate objective of improving collaborative practice.

View Article and Find Full Text PDF

Hirschsprung disease.

Nat Rev Dis Primers

October 2023

Department of Paediatric Surgery, Children's Hospital Colorado, Aurora, CO, USA.

Article Synopsis
  • * Associated syndromes include trisomy 21 and Mowat-Wilson syndrome, with genes like RET and EDNRB linked to the disease's occurrence.
  • * Diagnosis involves imaging and biopsy, and treatment usually requires surgical removal of the affected bowel, with various surgical techniques available and ongoing research aimed at improving diagnosis and patient outcomes.
View Article and Find Full Text PDF