Wilhelmina Children's Hospital

Mucopolysaccharidosis type I is a rare metabolic disorder characterized by the accumulation of glycosaminoglycans, leading to musculoskeletal disorders such as carpal tunnel syndrome. This retrospective cohort study was performed to determine the prevalence and timing of the development of carpal tunnel syndrome, assess the efficacy of surgery on recurrence and the effect of haematopoietic stem cell transplantation in resolving accumulated degradation products. Thirty-three mucopolysaccharidosis type I patients, born between 2001 and 2023, were included in this study. They received annual screening and treatment at our specialist hospital. Patient demographics, clinical symptoms of carpal tunnel syndrome pre- and post-carpal tunnel release and histopathological sections of the flexor retinaculum were collected. Regression analyses were conducted to assess cumulative risks and determine hazard ratios and survival curves were plotted. Twenty-seven of 33 mucopolysaccharidosis type I patients, with a median age at the latest follow-up of 13 years (IQR 7.7 to 18.3), were diagnosed with carpal tunnel syndrome on electrophysiological tests or ultrasound at a median age of 3.6 years (IQR 2.8 to 5.2). Only a few patients exhibited clinical symptoms. Twenty-one patients underwent carpal tunnel release and 13 patients experienced recurrence. The highest risk of developing carpal tunnel syndrome was within the first 6 years of life. Eight of the 11 patients accumulated degradation products within lysosomes despite successful haematopoietic stem cell transplantation. In this study, 27 mucopolysaccharidosis type I patients were diagnosed with carpal tunnel syndrome, showing a high-risk window from 0 to 6 years of age. Recurrence of carpal tunnel syndrome after carpal tunnel release surgery was common, occurring in 61.9% of patients. III.

Implementation of newly approved RSV preventive options is advancing rapidly. Therefore, we examined pregnant women's preferences regarding these RSV preventive options to optimise their implementation in immunisation programmes. A mixed method study was conducted including a cross-sectional survey (March 2022 - March 2023) and a qualitative study (January-February 2024) at 15 antenatal clinics and the Wilhelmina Children's Hospital in the Netherlands. The questionnaire was created using the theoretical 5Cs framework about vaccine hesitancy. Utilizing a 10-point rating scale, the importance of several factors influencing preference for maternal or infant immunisation were evaluated and participants were asked which option they would prefer. To further explore factors that influence vaccine decision-making focus groups were performed and analysed using inductive thematic analysis. A total of 412 women completed the questionnaire and 12 women participated in three focus groups. Majority of women (77.4%, 319/412) expressed a positive attitude to obtain either RSV preventive option. When asked for a definite decision, almost 90% of women preferred maternal vaccination. The most important influencing factors were protection against RSV disease and the safety of immunisation for the child before (i.e. in utero) and after birth. During the focus groups, advice of healthcare professionals was considered an important factor in decision making and all women referred to the balance between vaccine safety, prevention of the disease and duration of protection. Women would change their choice to the other option in case the other option has a greater protection against RSV. We found that the majority of women have a positive attitude toward receiving either a maternal vaccine or infant immunisation. Therefore, it is likely that Dutch pregnant women would be positive to receive either RSV preventive option, provided they receive accurate and comprehensive information that specifically addresses the factors they consider most important.

Shareable abstractSustainable development of neonatal and paediatric lung organoid research requires effective regulatory and collaborative frameworks. Ethically compliant tissue sources should be prioritised; robust ethical governance on sensitive sources is essential.https://bit.ly/4b9qeLf

Neonatal encephalopathy (NE)-including hypoxic-ischemic encephalopathy (HIE)-is associated with significant morbidity and mortality worldwide. While data registries provide hypothesis-generating data, aid in quality improvement, and track management/outcomes over time, we recently demonstrated that only 4 of 1,281 (0.3%) variables were collected by all 22 international NE/HIE registries: birth weight, gestational age, and 1- and 5-minute Apgar scores. In response, our team set out to develop a set of common inpatient data elements to enable future harmonization of NE/HIE registry data. Using a modified Delphi method, a panel of 14 international NE experts voted to separate the 1,281 variables from our previous study into "core elements," "supplemental elements," or those that "do not need to be collected." Based on the anonymous survey results, we created draft lists of core and supplemental common data elements (CDEs). These lists were further revised through group consensus meetings and external feedback from global registry leaders and attendants at 2 international conferences. The final data forms include 164 core elements and 225 supplemental elements stratified into 11 domains: demographics; pregnancy, labor, and delivery; delivery room; transport; acid-base; therapeutic hypothermia; neuromonitoring and seizures; neuroimaging; laboratory values (other than acid-base); hospital course; and discharge. The CDEs are the first of several important steps to further standardize research and reporting of NE/HIE investigations to perform more efficient and powerful clinical research in the field moving forward. Future studies need to establish a clinical and research definition of HIE and develop CDEs for collecting long-term, family-centered follow-up data postdischarge.

( Eur J Anaesthesiol . 2025;42:332-339. doi: 10.1097/EJA.0000000000002127.) This observational study aimed to evaluate the impact of introducing intrathecal morphine (ITM) on the postoperative recovery quality of mothers undergoing cesarean delivery (CD). Ensuring optimal recovery for mothers after CD is crucial, as it enhances their ability to care for their newborns and supports their overall physical, emotional, and psychological well-being. While ITM has been shown to effectively reduce postoperative pain, its broader adoption has been limited.

The microbiological diagnosis of severe pneumonia in intensive care unit (ICU) patients remains challenging. We investigated whether amplicon-based sequencing (ABS) could provide additional value in this setting. Bacterial 16S rRNA coupled with fungal internal transcribed spacer (ITS) ABS was compared with standard of care (SOC) comprising cultures, PCR and galactomannan on bronchoalveolar lavages of immunosuppressed and immunocompetent ICU patients presenting severe pneumonia (n = 24) and noninfected lung-transplanted patients (n = 12). The ABS-matched SOC results were complete for 20/36 (55.5%) samples. Regarding 16S rRNA, ten samples showed partial agreement, whereas five others were fully discordant. Discordances were observed mainly for gram-negative organisms, either for quantification (Enterobacteria) or identification (Enterobacter sp., Haemophilus sp.). Only 1 of the 36 ITS results were discordant with the SOC results. The ABS has proven to be more valuable in immunosuppressed patients. Despite limitations in bacterial identification, pathogens not reported by SOC were detected in 8 samples (coinfections, bacterial superinfections from viral pneumonia). ITS identification was reliable, and semiquantification helped distinguish P. jirovecii infection and colonization. The correlation between galactomannan and ITS was good. While 16S rRNA sequencing is interesting for severe pneumonia of undefined aetiology in IS patients, ITS could provide additional diagnostic value for severe fungal pneumonia.

ObjectiveCancer during pregnancy poses significant physical and psychological challenges for expectant mothers and their partners. While elevated stress and anxiety levels in pregnancy are well-documented, the specific parental psychological impact of cancer during pregnancy remains unexplored, and could be different for patients and partners. This study represents the first full prospective analysis, complementing earlier retrospective research by analyzing self-reported perspectives and coping strategies among both pregnant cancer patients and their partners.MethodsParticipants were prospectively recruited within the International Network on Cancer, Infertility, and Pregnancy (INCIP) across The Netherlands, Belgium, USA and Italy. Participants completed the Cancer and Pregnancy Questionnaire (CPQ), developed by our research team, and a shortened version of the Cognitive Emotion Regulation Questionnaire (CERQ). Differences in concerns and coping strategies were analyzed using Wilcoxon Signed-Rank tests, Cliff's Delta for effect sizes, and Spearman's correlation analysis.ResultsBetween 2009 and 2024, 220 patients and 189 partners participated. Compared to partners, patients reported significantly greater concerns about their child's health and the implications of the disease, treatment, and upcoming delivery. Patients exhibited higher use of both adaptive (e.g., Positive Reappraisal, Refocusing) and maladaptive (e.g., Self-blame, Rumination) coping strategies, and a stronger desire to continue the pregnancy. Satisfaction with medical care was similar between groups. Maternal age at diagnosis was linked to maladaptive strategies (Catastrophizing and Blaming Others), while higher gestational age at diagnosis was associated with both adaptive (Positive Refocusing and Positive Reappraisal), and the maladaptive coping strategy Catastrophizing.ConclusionsThis study provides valuable insights into the psychological challenges experienced by pregnant cancer patients and their partners, emphasizing the need for personalized care that includes psychological support from the start.

Radiotherapy (RT) is essential in pediatric cancer treatment and often requires complete immobility. In younger or noncompliant children, this is typically achieved through sedation or general anesthesia (GA), which raises concerns about acute complications and potential long-term neurodevelopmental effects. Despite widespread use, standardized anesthesia protocols for pediatric RT are lacking. To support the development of practice recommendations, the SIOPE Working Group on Pediatric Anesthesia in Radiation Therapy conducted a systematic literature review. Studies from Medline and Embase were reviewed (March-September 2024) according to PRISMA guidelines, focusing on sedation and GA in pediatric RT. Thirty-nine studies were included, mostly retrospective and of low to moderate quality. Considerable heterogeneity was observed in anesthetic techniques, staffing, and monitoring. Propofol-based sedation was most frequently reported, with favorable safety when delivered by experienced pediatric anesthetists. Complication rates varied widely and were often poorly defined. Additional concerns included long-term neurocognitive impact, vascular access, and procedural burden, especially in resource-limited settings. Evidence supports the safe use of sedation/GA in pediatric RT, but current literature is limited and inconsistent. Standardized protocols and prospective studies are urgently needed to better define safety, long-term outcomes, and staffing requirements.