Radboud University Medical Center Research Articles

#2388 Relevance of anti-PLA2R levels in therapy decision and prediction of therapy outcome in patients with membranous nephropathy

Abstract Background and Aims Detection of anti-phospholipase A2 receptor (PLA2R) antibodies in patients with primary membranous nephropathy (MN) supports diagnosis as well as disease monitoring. An individualised therapy approach was introduced for anti-PLA2R positive MN patients at the Radboud University Medical Center. Immunosuppressive treatment (cyclophosphamide combined with steroids) was stopped when anti-PLA2R results by indirect immunofluorescence testing (IIFT) became negative. Here, we evaluated the relevance of anti-PLA2R levels for therapeutic decisions and the outcome of MN, comparing qualitative and quantitative detection methods. Method Stored serum samples were retrieved for beginning of treatment (baseline), decision point, and follow-up. Anti-PLA2R levels were determined qualitatively by IIFT as well as quantitatively by enzyme-linked immunosorbent assay (ELISA) and chemiluminescence immunoassay (ChLIA) at baseline as well as after treatment and correlated to immunological remission and persistence. Results For an evaluation of the relevance of anti-PLA2R levels at the start of therapy, serum samples of 60 patients were available. Patients sampled at the beginning of the therapy were grouped according to tertiles of anti-PLA2R levels determined by ChLIA (n[lowest tertile] = 20, n[middle tertile] = 20, n[highest tertile] = 20). Lower anti-PLA2R levels were seen in patients with relapsing disease. Higher anti-PLA2R levels were associated with more severe proteinuria. Patients in the lowest tertile of anti-PLA2R were more likely to develop immunological remission after 8 weeks of therapy. In total, 90% vs 75% vs 45% of patients in the tertiles turned anti-PLA2R negative after being treated for 8 weeks. Moreover, in this subgroup of patients who showed immunological remission after 8 weeks of therapy, only 11% of the patients in the lowest tertile needed renewed immunosuppressive therapy, while this percentage increased in the middle and highest tertiles (11% vs 53% vs 33%, p=0.033). For the assessment of anti-PLA2R levels before and after therapy, samples at baseline and week 8 of treatment were examined. At baseline, 50/50 (100%) tested positive in IIFT and ChLIA as well as 48/50 (94%) in ELISA. After 8 weeks on treatment, immunological remission based on IIFT was found in 37/51 (73%) patients. At this point, the overall agreement compared to IIFT was 92% for ChLIA and 82% for ELISA. Yet the availability of quantitative levels provided further insight into this group of patients in whom treatment was discontinued. With ChLIA, anti-PLA2R titers >5 RU/mL after 8 weeks of therapy were found in 4% of IIFT negative patients with persistent remission, vs 55% of IIFT negative patients with early relapse (p=0.006). Conclusion Individualised treatment of MN patients with cyclophosphamide and steroids has been recently introduced. In this respect, the quantitative determination of anti-PLA2R levels adds further value. Of the examined quantitative methods, ChLIA demonstrated the highest agreement with IIFT. Additional studies are needed to evaluate the impact on clinical decision making and outcome.

The Effect of Regional Anaesthesia on Free Flap Survival in Lower Extremity Reconstructions.

The effect of different modalities of anaesthesia in microvascular free flap surgery has been a topic of ongoing debate. Comparative data to study the effect of general anaesthesia and regional anaesthesia in the form of peripheral nerve blocks (PNBs) on lower extremity free flap survival is lacking to date. This study aims to elucidate the effect of regional anaesthesia on flap survival in lower extremity free flap reconstructions. A retrospective cohort study of all patients who underwent free vascularised flap reconstruction of the lower extremities between 2012 and 2021 at the Amsterdam University Medical Centre (UMC), The Netherlands, and between 2019 and 2021 at the Radboud UMC, Nijmegen, The Netherlands. In this cohort, we analysed partial and total flap failures. In this cohort, 87 patients received a total of 102 microvascular free flap reconstructions of the lower extremity. In 20.5% of these operations, patients received a supplemental PNB. Total flap failure was 23.8% in the regional anaesthesia group compared to 21% in the group with general anaesthesia only (p = 0.779). Operation time was longer for patients with regional anaesthesia (p = 0.057). Length of stay was on average 2 days shorter for patients with supplemental regional anaesthesia (p = 0.716). This is the largest cohort comparing flap survival in patients receiving general anaesthesia to general anaesthesia with a PNB in lower extremity reconstructions to date. We cannot attribute a significant beneficial or detrimental effect of regional anaesthesia to flap survival. High failure rates stress the need for future studies. Koster ITS, den Os MM, Rutten MVH, et al. The Effect of Regional Anaesthesia on Free Flap Survival in Lower Extremity Reconstructions. Strategies Trauma Limb Reconstr 2024;19(1):15-20.

The effect of combined surgery and physiotherapy on the facial asymmetry in patients with congenital muscular torticollis: a retrospective cohort study

The aim of this study was to assess the influence of surgical therapy, intraoperative evaluation, and immediate physiotherapy on the facial asymmetry of patients with congenital muscular torticollis (CMT). Patients who underwent a combination of unipolar sternocleidomastoid muscle release with perioperative physiotherapy between 2014 and 2020 in the Department of Oral and Maxillofacial Surgery, Radboud University Medical Center, Nijmegen were enrolled. Three-dimensional photographs of the face acquired 2 months preoperatively and 1.5–2 years postoperatively were assessed. Based on 43 patients, there was a significant improvement in the asymmetry indices (angles between bilateral exocanthion, bilateral cheilion, and nasion–gnathion lines): mean ± standard deviation reductions in indices (Ex–Ex)–(Ch–Ch), (Ex–Ex)–(Na–Gn), and (Ch–Ch)–(Na–Gn) of 0.88 ± 1.08° (P < 0.001), 1.04 ± 1.26° (P < 0.001), and 0.21 ± 1.06° (P = 0.024), respectively. Analysis of covariance (ANCOVA) showed that intervention at an early age (P = 0.017) and left-sided torticollis (P = 0.030) were associated with a significantly greater improvement in the facial asymmetry. This study found that the use of combined surgery and physiotherapy reduced the facial asymmetry of patients with CMT at 2 years postoperatively. Early intervention and left-sided torticollis were associated with a greater improvement in the facial asymmetry. This indicates that attention should be given to early intervention and right-sided CMT cases postoperatively.

Virtual reality to reduce periprocedural anxiety during invasive coronary angiography: rationale and design of the VR InCard trial

IntroductionPatients undergoing invasive coronary angiography (ICA) experience anxiety due to various reasons. Procedural anxiety can lead to physiological and psychological complications, compromising patient comfort and overall procedural outcomes. Benzodiazepines are...

Direct aortic cannulation, a safe alternative to femoral artery cannulation - 17 years of type A dissection surgery experience

Aim: Optimal cannulation strategy for acute type A aortic dissection (ATAAD) surgery remains debated. Recent guidelines have advocated antegrade systemic perfusion through right axillary artery (RAX) cannulation, instead of femoral artery (FA) cannulation. However, RAX cannulation can be technically challenging and time-consuming. On the other hand, direct (ascending) aorta (DA) cannulation is a swift procedure that also ensures downstream antegrade flow. In this regard, we assessed whether DA cannulation is a safe alternative to FA cannulation. Methods: Records of all patients undergoing ATAAD surgery between 2006-2022 at the Radboud University Medical Center were retrospectively reviewed. Results: In total, 281 patients underwent surgery for ATAAD during the investigated period. Three patients were excluded due to death before the start of extracorporeal circulation and four because of RAX cannulation. Of the remaining 274 patients, 53% (N = 145) received primary FA and 47% (N = 129) DA cannulation, with a success rate of 98% for both approaches. Surgical mortality (combined in-hospital and 30-day) was 9.9% (7.8% DA group vs. 11.7% FA group, P = 0.271). New permanent neurological damage was seen in 10.9% vs. 6.9% (P = 0.248), respectively. In multivariate analysis, cannulation strategies were not significantly associated with surgical mortality nor postoperative new permanent neurological damage. Conclusion: DA cannulation offers a safe and fast alternative to FA cannulation in ATAAD surgery. There were no significant differences in mortality and neurological complications. Future studies should focus on the differences between RAX and DA cannulation strategies on postoperative outcomes in ATAAD surgery.

Facilitators and barriers to the transition from outpatient clinic visits to home-based check-ups for children being treated with growth hormone: a mixed-methods study

Although the coronavirus disease 2019 (COVID-19) pandemic accelerated the adoption and expansion of telemedicine worldwide, little is known about the transition to home-based care for children. This study aims to investigate the facilitators and barriers to the transition from outpatient clinic visits to home-based check-ups (HBCU), for children being treated with growth hormone. A mixed-methods study was performed at Amalia Children’s Hospital (Radboud University Medical Centre, Nijmegen), consisting of questionnaires and semi-structured and focus group interviews. For the quantitative part, the Measurement Instrument for Determinants of Innovation (MIDI) was utilised to investigate the facilitators and barriers for the 81 participants regarding the transition to HBCU. The MIDI questionnaire is comprised of four domains: the innovation-, user-, organisation-, and the socio-political scale. Descriptive statistics were performed for analysing the questionnaires. For the qualitative part, interviews with 10 participants derived from the questionnaire and the two focus group interviews were conducted, to gain more in-depth information about the research topic, until data saturation was reached. The interviews were analysed by using the reflective thematic approach, starting with deductive coding and followed by inductive coding. Several facilitators were recognised in our study: procedural clarity, self-efficacy, convenience, patient-centred care, increased accuracy in height measurements, social support, client/patient satisfaction/cooperation, patient-centred care, the flexibility and adaptivity of HBCU, physical start-up period of HBCU, and a potential decrease in healthcare costs. However, several barriers were also noted in our study: poor compatibility with current practice, lack of consultation within the team, feeling of being less controlled by physicians, unsettledness of the organisation, an increased workload for the staff, and insufficient information communication technology (ICT) facilities.Conclusion: This study revealed that HBCU have considerable benefits for both patients and healthcare professionals, from the standpoint of innovation, user, and socio-political points of view. The identified facilitators and barriers to HBCU should be taken into account when further steps of implementing HBCU are considered.What is Known:• The Corona-Virus-Disease 2019 (COVID-19) pandemic has had an immense impact on health care worldwide. A substantial amount of the outpatient clinic visits for children treated with growth hormone was, as a result of the pandemic, transferred to online consultation. Transitioning paediatric growth hormone treatment to the home setting may be favorable for children and their parents/caregivers) as well for healthcare professionals.• Insights regarding facilitators and barriers is vital for the successful implementation and adoption of home-care technologies.What is New:• To our knowledge, we are first to report on and explicit the facilitators and barriers of the transition to home-based check-ups, via online consultation for children being treated with growth hormone.• Both children and healthcare professionals reported major facilitators and some minor barriers to the transition to home-based check-ups, illustrating their potential value. These facilitators and barriers should be considered while working towards implementation of home-based check-ups.

Surgical Treatment for Troublesome Mastoid Cavities: Canal Wall Reconstruction With Bony Obliteration Versus Subtotal Petrosectomy.

A chronically discharging modified radical mastoid cavity may require surgical intervention. We aim to explore two techniques. To compare outcomes of subtotal petrosectomy (STP) and canal wall reconstruction with bony obliteration technique (CWR-BOT). Retrospective cohort study. A tertiary referral center. All patients with a chronically discharging mastoid cavity surgically treated at the Radboud University Medical Center by STP or CWR-BOT in 2015 to 2020, excluding patients with preoperative cholesteatoma. Dry ear rate, audiometry, and rehabilitation. Healing time, number of postoperative visits, complications, cholesteatoma, and need for revision surgeries. Thirty-four (58%) patients underwent STP, and 25 (42%) CWR-BOT. A dry ear was established in 100% of patients (STP) and 87% (CWR-BOT). The air-bone gap (ABG) increased by 12 dB in STP, and decreased by 11 dB in CWR-BOT. Postoperative ABG of CWR-BOT patients was better when preoperative computed tomography imaging showed aerated middle ear aeration. ABG improvement was higher when ossicular chain reconstruction took place. Mean follow-up time was 32.5 months (STP) versus 40.5 months (CWR-BOT). Healing time was 1.2 months (STP) versus 4.1 months (CWR-BOT). The number of postoperative visits was 2.5 (STP) versus 5 (CWR-BOT). Cholesteatoma was found in 15% (STP) versus 4% (CWR-BOT) of patients. Complication rate was 18% (STP) and 24% (CWR-BOT) with a need for revision in 21% (STP) and 8% (CWR-BOT), including revisions for cholesteatoma. STP and CWR-BOT are excellent treatment options for obtaining a dry ear in patients with a chronically discharging mastoid cavity. This article outlines essential contributing factors in counseling patients when opting for one or the other. Magnetic resonance imaging with diffusion-weighted imaging follow-up should be conducted at 3 and 5 years postoperatively.

Longitudinal Course of Long Finger Flexor Shortening in Males with Duchenne Muscular Dystrophy: A Retrospective Review1.

Shortening of the long finger flexors (Flexor Digitorum Profundus, FDPs) in Duchenne Muscular Dystrophy (DMD) causes reduced hand function. Until now, longitudinal studies on the natural course of the shortening of the FDPs are lacking, which impedes recommendations on timing and evaluation of preventive measures. To investigate the longitudinal course of the FDP length during different disease stages focusing on symmetry, timing, and decline of the FDP length. A retrospective, longitudinal multicenter study was conducted in the Radboud university medical center and the Leiden university medical center. The FDP outcome was measured using goniometry and gross motor function was assessed using the Brooke score. Longitudinal mixed model analyses were used to describe the course of the FDP outcome, and to investigate symmetry in both hands. Data on 534 visits of 197 males (age ranged 4-48 years) showed that in the ambulatory stages the FDP outcome was within a normal range. The mean decline in FDP outcome is 3.5 degrees per year, the biggest decline was seen in Brooke 5 (>15 degrees per year). In Brooke 4, 41% of the FDP outcome was < 40 degrees. No significant differences were found between right and left. This study supports the consideration of preventive measures to delay shortening of the FDPs in DMD patients transitioning to a Brooke scale of 4 or higher. Besides, natural history of FDP outcome has been established, which provides a base to evaluate (preventive) interventions.

LCA comparing 3D printed splints to conventional splints for traumatic injuries

In the context of climate change, hospitals that use significant amounts of disposable products are undertaking sustainability initiatives. This can be supported by Life Cycle Analysis (LCA), in which an increase is noted in the medical domain to study and implement sustainable alternatives. However, the sustainable implementation of new technology such as 3D printing to generate personalized medical devices raises additional challenges. As common wrist injuries like a distal radius fracture, show a high prevalence (200-400 times per 100.000 persons), the department of trauma surgery at the Radboud University Medical Centre in Nijmegen, is focusing on implementing 3D printing of personalized splints. Simultaneously, a case study is initiated to assess the environmental impact of this innovation with conventional medical devices: mineral or plastic splints. This research enhances future work on sustainability implementation of the 3D printed splint treatment for wrist injuries, potentially expediting its adoption as the standard treatment. This project raises awareness about the environmental impact of medical products and treatments, while fostering collaboration for future sustainable projects in the hospital.

Arrhythmias including atrial fibrillation and congenital heart disease in Kleefstra syndrome: a possible epigenetic link.

Kleefstra syndrome (KS), often diagnosed in early childhood, is a rare genetic disorder due to haploinsufficiency of EHMT1 and is characterized by neuromuscular and intellectual developmental abnormalities. Although congenital heart disease (CHD) is common, the prevalence of arrhythmias and CHD subtypes in KS is unknown. Inspired by a novel case series of KS patients with atrial tachyarrhythmias in the USA, we evaluate the two largest known KS registries for arrhythmias and CHD: Radboudumc (50 patients) based on health record review at Radboud University Medical Center in the Netherlands and GenIDA (163 patients) based on worldwide surveys of patient families. Three KS patients (aged 17-25 years) presented with atrial tachyarrhythmias without manifest CHD. In the international KS registries, the median [interquartile range (IQR)] age was considerably younger: GenIDA/Radboudumc at 10/13.5 (12/13) years, respectively. Both registries had a 40% prevalence of cardiovascular abnormalities, the majority being CHD, including septal defects, vascular malformations, and valvular disease. Interestingly, 4 (8%) patients in the Radboudumc registry reported arrhythmias without CHD, including one atrial fibrillation (AF), two with supraventricular tachycardias, and one with non-sustained ventricular tachycardia. The GenIDA registry reported one patient with AF and another with chronic ectopic atrial tachycardia (AT). In total, atrial tachyarrhythmias were noted in six young KS patients (6/213 or 3%) with at least four (three AF and one AT) without structural heart disease. In addition to a high prevalence of CHD, evolving data reveal early-onset atrial tachyarrhythmias in young KS patients, including AF, even in the absence of structural heart disease.

Nothing about me without me: Development of a questionnaire to evaluate experiences with co-creation.

Introduction: Given the desirable move towards more personalized care management, new ways of involving patients and their caregivers in redesigning care are needed. One important tool is co-creation, which enables patients and their caregivers to play a key role in the design, development and implementation of health care innovations. Currently, health care innovations for improving care for Parkinson's Disease (PD) have been established that require the involvement of people with PD, informal and professional caregivers so that these innovations meet the needs, wishes and preferences of those who would benefit from the health care innovations. However, up to now there is no tool available that enables the evaluation of co-creation processes. Therefore, the aim of this study was to assess the core elements of co-creation and to develop a questionnaire together with people with PD, informal and professional caregivers allowing evaluation and more importantly, assessing the barriers and facilitators of co-creation.&#x0D; Aims and methods: We aimed to identify the core elements of co-creation, in order to develop a questionnaire to evaluate the co-creation process and assess the experienced barriers and facilitators of the co-creation process. We collaborated with people with PD, informal and professional caregivers who have been participating in two projects for improving health care for people with PD and their caregivers at the neurology department of the Radboud University Medical Center in Nijmegen, the Netherlands. Firstly, we used focus group discussions to identify the core elements that members of the co-creation teams deemed important for successful collaboration within co-creation groups. Secondly, we designed a questionnaire in which barriers and facilitators were assessed regarding these core elements. Thirdly, we tested the questionnaires.&#x0D; Results: Together with people with PD, informal and professional caregivers we succeed in identifying the seven core elements of co-creation: (1) Organisation, composition and communication; (2) Skills and equivalence; (3) Trust and security; (4) Feeling heard and appreciated; (5) Involvement; (6) Sustainable goals and processes; (7) Expectations and satisfaction. The questionnaire that we developed subsequently included 43 questions to evaluate the experiences of members of the co-creation teams and to assess the barriers and experiences regarding these core elements. Testing the questionnaires in existing co-creation teams provided us with important starting points for improving the co-creation process within these teams. &#x0D; Conclusion: The questionnaire we developed based on the seven core elements of co-creation that we identified in collaboration with people with PD, informal and professional caregivers provided us with important starting points for improving co-creation within existing co-creation teams. &#x0D; Future implications: Co-creation is key to improve personalized care management, but to monitor the process and evaluate satisfaction of all involved stakeholders, it is important to have an instrument that assesses the core elements of co-creation and enables further improvement of the process. We present the first step in undertaking such an evaluation, which will be followed by qualitative assessments in the form of in-depth interviews that will help to deepen our understanding on what is needed to successfully implement co-creation of health care innovations.

OP102 Towards Universal Health Coverage: Health Technology Assessment Roadmap Development In The Emirate Of Abu Dhabi Involving The Whole Ecosystem

IntroductionThe mission of the Department of Health (DoH) of Abu Dhabi in the United Arab Emirates is to provide its population with a healthy life and world leading preventive and curative services. While the DoH has regulations in place to grant market approval to new health technologies, there is a need to develop a clear overall framework for reimbursement and disinvestment decisions. Establishing a structured health technology assessment (HTA) framework is critical for informing decisions on health technologies that offer value for money, with the aim of improving equitable access to health care, financial risk protection, and, ultimately, better health outcomes.MethodsDuring 2022, the DoH collaborated with the Radboud University Medical Center to explore the feasibility of applying an evidence-informed deliberative process (EDP) HTA approach through workshops and interviews involving all stakeholders in the ecosystem, such as policy makers, principal investigators, providers, patients and public groups, product manufacturers, payers, and purchasers. A situational analysis was conducted to collect stakeholders’ views and build EDPs. Based on this analysis, a structured roadmap was developed.ResultsThe comprehensive five-year roadmap to implement a holistic HTA framework in Abu Dhabi consisted of five major elements, starting with the establishment of an appropriate HTA policy framework as a foundation. Abu Dhabi should firmly establish its HTA structure and program (in one to two years), and at the same time invest in developing and retaining HTA training capacity so that over time (within three to five years) the country can build up its own expertise to sustain the program. This needs to be accompanied by continuous awareness raising among all relevant stakeholders.ConclusionsThis roadmap is the first and most important step toward implementing a holistic HTA framework in Abu Dhabi. Technical work needs to be complemented by continuously raising awareness and involving all relevant stakeholders. Looking at the initial results and international benchmarks, HTA will significantly contribute to achieving a sustainable, high quality healthcare system.

Long-term effects of surgically corrected menstrual outflow obstruction: A case-control study.

To enhance evidence-based knowledge on long-term sequalae in patients with surgically corrected obstructing Müllerian anomalies. This long-term case-control study included patients with menstrual outflow obstruction due to congenital anomalies of the uterus or vagina, who were at least 18 years old, and for whom 2 years had elapsed since the first surgery at the start of this study. The control group consisted of women without current gynecological problems. Patients underwent a surgical correction at the Radboud University Medical Center Nijmegen between 1980 and 2013. Of 78 patients approached, 38 (49%) were included in this study. The control group consisted of 54 females. The main outcome measures were pain and health state. The following questionnaires were used: Visual Analogue Scale pain scores, European Quality of Life-5 Dimensions questionnaire (EQ-5D-3L) and the adapted Endometriosis Health Profile questionnaire (EHP-30). Patients had higher actual and maximum abdominal pain scores compared with controls (11 vs 0 [P = 0.007] and 48 vs 21 [P = 0.035], respectively). Based on the EQ-5D-3L scores, patients had more pain and discomfort (P = 0.005), more mood problems (P = 0.023), and a poorer subjective health state (P = 0.002) and self-rated health state (P = 0.031). Based on the EHP-30, patients had a significant poorer self-rated health state on four out of five subscales. In this study, following surgically corrected menstrual obstruction, patients had statistically significant higher abdominal pain scores and a poorer self-rated health state compared with controls.

Management of drug-resistant tuberculosis in Indonesia: a four-year cascade of care analysis

In Indonesia, drug resistance testing for TB largely relies on Xpert MTB/RIF, and it is unknown what proportion of drug-resistant (DR) TB is adequately diagnosed and treated. We conducted a cascade of care analysis on a cohort of presumptive rifampicin-resistant (RR) TB patients registered in 2015-2018 in a tertiary hospital in Indonesia. Estimated incidences of (presumptive) DR-TB cases were assumption-based using global reports. Data on diagnosis and consecutive cascades steps, including their timing were collected from national electronic registers, and medical records. We described a secondary cascade for patients receiving treatment not supported by phenotypic drug susceptibility testing (pDST). Factors associated with delay and loss between diagnosis and treatment were identified using logistic regression. Less than a third of estimated incident TB cases at risk of DR-TB were identified as presumptive DR-TB case and tested, and 9.8% (982/10,065) of estimated true DR-TB cases was diagnosed. Of those diagnosed, only 45.1% (443/982) had treatment regimens supported by pDST results, but this did not significantly influence treatment outcomes. Only 25.5% (250/982) of diagnosed patients completed all steps of the cascade including successful treatment. Delays between diagnosis and treatment were substantial, and more common among those referred from a primary healthcare facility, and among those who were employed, living outside of Bandung, and reporting engagement with the private sector. The DR-TB care cascade in this urban setting in Indonesia is characterized by substantial attrition and delays. Strategies to increase access to DR-TB diagnosis accompanied by optimisation of clinical care could substantially improve outcomes and reduce onward transmission. Radboud university medical center and University of Otago.

Gender-aware Parkinson’s care: a design-based study of patient perspectives on gender norms and gender-sensitive care

Gender dimensions are progressively recognised as a relevant social determinant of health in people with Parkinson's disease (PD). However, little is known about the impact of gender norms and stereotypes on illness experiences of men and women with PD and what they consider important focal points for gender-sensitive PD care. We conducted two equity-centred design (ECD) sessions on December 7, 2022 and December 8, 2022, at the Radboud University Medical Centre in the Netherlands. This participatory multi-method approach includes patients in the research and design process and was used to explore the impact of gender norms and stereotypes in illness experiences and generate patient-driven recommendations for gender-aware Parkinson's care. Quantitative survey data and design-based data were descriptively analysed, and qualitative focus group discussions were thematically analysed. This study included thirteen men and fifteen women with PD in the Netherlands. All participants were of Dutch descent, with a median age of 65.5 years and a median clinical disease duration of 4.2 years. The gendered stereotype that "people with PD are old men" affected both men's and women's perception of living with the disease and the perceptions of their social environment. Men described masculine stereotypes related to physical strength and provider roles, while women expressed those related to feminine physical appearance and caregiver roles, influencing their illness experiences. For some, these norms influenced personal behaviours, while for others, they affected experiences through societal attitudes. Our findings suggested that several gender norms and stereotypes influence the illness experiences of men and women with PD, manifesting at ideological, interpersonal, and internalised levels. Some participants internalised these norms, affecting their coping behaviours, while others encountered them in broader ideological contexts that shaped societal attitudes and interpersonal relationships. To advance gender sensitive PD care, it's essential to explore the impact of gender roles and norms, especially regarding how they might impede coping strategies, care access and utilisation for individuals of diverse gender identities. The Gatsby Foundation and co-funded by the PPP Allowance by Health∼Holland. Travel reimbursements for participants were made available through a Parkinson's Foundation grant (PF-FBS-2026).

Influence of miniscrew-assisted rapid palatal expansion (MARPE) on the interdental papilla height of maxillary central incisors

ObjectivesTo evaluate the influence of miniscrew-assisted rapid palatal expansion (MARPE) on the interdental papilla height of maxillary central incisors.Materials and methodsPatients who completed MARPE treatment at the Radboud University Medical Center between 2018 and 2021 were included in this retrospective study. The papilla height between the maxillary central incisors was evaluated on frontal intraoral photographs taken before expansion (T0) and 1.5 years after MARPE treatment (T1) using the Jemt classification. The difference in Jemt score at T0 and T1 was the primary outcome variable. In addition, gender, age, Angle classification, MARPE duration, midpalatal suture maturation stage, maximal central diastema (MCD) immediately after expansion, crown width to length ratio (W/L), pretreatment overlap of maxillary central incisors, and the distance between the approximal contact point of the central incisors and the bone crest (CP-B) were also record.ResultsTwenty-two patients were included (2 men, 20 women, mean age 27.3 ± 8.8 years) and 4 patients (18%) showed a significant reduction in the Jemt score following MARPE (p = 0.04), indicating papilla recession. Interdental papilla recession was significantly associated with the increase of CP-B (p = 0.02), smaller W/L (p < 0.01), overlapping of maxillary central incisors (p < 0.01), and smaller MCD immediately after expansion (p = 0.02).ConclusionsOne and a half years after MARPE, 18% of patients exhibited mild recession of papilla height of the maxillary central incisors. Overlapping and smaller W/L of maxillary central incisors were prognostic factors for interdental papilla recession.Clinical relevanceClinicians have to be aware of and inform the patients about the occurrence of papilla recession following MARPE.

Exploring professionals' views regarding prenatal counselling in congenital diaphragmatic hernia.

Congenital diaphragmatic hernia (CDH) is a congenital malformation in which the diaphragm and lungs are underdeveloped, leading to cardiorespiratory and other problems. This study aimed to explore professionals' views regarding prenatal counselling in CDH. A qualitative study was performed among healthcare professionals involved in the care of CDH patients in Radboud university medical center Amalia Children's Hospital. Semi-structured interviews were conducted until saturation was achieved. Transcripts were qualitatively analysed to gain insight into professionals' views regarding counselling. Eighteen professionals with various backgrounds were included. The professionals agreed that the first counselling session should be soon after diagnosis and additional sessions should be offered. Concerning counselling content, participants considered explanation of the diagnosis, prognosis, short- and long-term consequences, treatment options and practical aspects important. As for decision-making about possible termination of pregnancy, all professionals emphasised the importance of the parental role, but the preferred parental involvement varied. Regarding practical aspects, preferred counsellors were a neonatologist, obstetrician, paediatric surgeon and/or medical social worker. Participants emphasised that the counselling should be adjusted to parents' needs. This study gained insight into professionals' views regarding the timeline, content, decision-making process, and practical aspects of prenatal counselling in CDH.

Comparison and predictors of implantable cardioverter-defibrillator therapy for primary and secondary prevention.

Implantable cardioverter-defibrillators (ICDs) are effective in detecting and treating ventricular arrhythmias. Studies on ICD therapy for different indications (primary and secondary prevention) and possible predictors of ICD therapy are limited. In this study, the incidence and type of ICD therapy were related to the indication and the underlying cardiac pathology. Asingle-centre, retrospective and observational study was performed of 482patients who underwent ICD implantation for primary (53.3%) or secondary prevention (46.7%) between 2015 and 2020 at the Radboud University Medical Centre. During amedian follow-up of 2.4years (interquartile range 0.2-3.9), the occurrence of appropriate ICD therapy for primary versus secondary prevention was 9.7% and 27.6%, respectively (p < 0.001). Time to appropriate ICD therapy was significantly shorter in the secondary prevention group (p < 0.001). No difference in ICD therapy was seen for different underlying aetiologies. In the majority of cases (70%) ICD therapy was given for ventricular tachycardia (VT). The occurrence of adverse events (16.3% vs 17.3%, p = 0.772), hospitalisation for cardiovascular reasons (29.2% vs 35.1%, p = 0.559) and all-cause mortality (12.5% vs 11.6%, p = 0.763) were similar in both groups. Male gender (3.53, 95% confidence interval (CI) (1.003, 12.403), p = 0.049) and secondary prevention indication (4.90, 95% CI (1.495, 16.066), p = 0.009) were predictors of appropriate ICD therapy. The risk associated with appropriate ICD therapy is higher in secondary prevention patients, who have their first therapy within ashorter time frame after device implantation. Rates of complications, hospitalisation and all-cause mortality are comparable. Future treatment options should target the prevention of ICD therapy, mainly by preventing the recurrence of VT.

Effect of PCSK9 Inhibition on the Plasma Proteome: A SPIRE SubStudy.

HomeArteriosclerosis, Thrombosis, and Vascular BiologyAhead of PrintEffect of PCSK9 Inhibition on the Plasma Proteome: A SPIRE SubStudy Open AccessResearch ArticlePDF/EPUBAboutView PDFSections ToolsAdd to favoritesDownload citationsTrack citationsPermissions ShareShare onFacebookTwitterLinked InMendeleyReddit Jump toOpen AccessResearch ArticlePDF/EPUBEffect of PCSK9 Inhibition on the Plasma Proteome: A SPIRE SubStudy Jordan M. Kraaijenhof, Tjerk S.J. Opstal, Jan H. Cornel, Dipender Gill, G. Kees Hovingh, Alberico L. Catapano, Wolfgang Koenig, Paul M Ridker, Erik S.G. Stroes and Nick S. Nurmohamed Jordan M. KraaijenhofJordan M. Kraaijenhof https://orcid.org/0000-0001-7566-6403 Department of Vascular Medicine, Amsterdam University Medical Centers, University of Amsterdam, The Netherlands (J.M.K., G.K.H., E.S.G.S., N.S.N.). Search for more papers by this author , Tjerk S.J. OpstalTjerk S.J. Opstal https://orcid.org/0000-0003-3654-0327 Department of Cardiology, Radboud University Medical Centre, Nijmegen, the Netherlands (T.S.J.O., J.H.C.). Department of Cardiology, Northwest Clinics, Alkmaar, the Netherlands (T.S.J.O., J.H.C.). Search for more papers by this author , Jan H. CornelJan H. Cornel https://orcid.org/0000-0002-1006-2112 Department of Cardiology, Radboud University Medical Centre, Nijmegen, the Netherlands (T.S.J.O., J.H.C.). Department of Cardiology, Northwest Clinics, Alkmaar, the Netherlands (T.S.J.O., J.H.C.). Search for more papers by this author , Dipender GillDipender Gill https://orcid.org/0000-0001-7312-7078 Department of Epidemiology and Biostatistics, School of Public Health, Imperial College London, United Kingdom (D.G.). Search for more papers by this author , G. Kees HovinghG. Kees Hovingh https://orcid.org/0000-0002-8145-1676 Department of Vascular Medicine, Amsterdam University Medical Centers, University of Amsterdam, The Netherlands (J.M.K., G.K.H., E.S.G.S., N.S.N.). Search for more papers by this author , Alberico L. CatapanoAlberico L. Catapano https://orcid.org/0000-0002-7593-2094 Department of Pharmacological and Biomolecular Sciences, University of Milan, Milano, Italy (A.L.C.). IRCCS Multimedica, Milano, Italy (A.L.C.). Search for more papers by this author , Wolfgang KoenigWolfgang Koenig https://orcid.org/0000-0002-2064-9603 Deutsches Herzzentrum München, Technische Universität München, Munich, Germany (W.K.). German Centre for Cardiovascular Research (DZHK e.V.), partner site Munich Heart Alliance, Germany (W.K). Institute of Epidemiology and Medical Biometry, University of Ulm, Germany (W.K.). Search for more papers by this author , Paul M RidkerPaul M Ridker https://orcid.org/0000-0003-1249-4522 Divisions of Preventive Medicine and Cardiovascular Diseases, Brigham and Women’s Hospital, Harvard Medical School, Boston, MA (P.M.R.). Search for more papers by this author , Erik S.G. StroesErik S.G. Stroes https://orcid.org/0000-0001-9555-6260 Department of Vascular Medicine, Amsterdam University Medical Centers, University of Amsterdam, The Netherlands (J.M.K., G.K.H., E.S.G.S., N.S.N.). Search for more papers by this author and Nick S. NurmohamedNick S. Nurmohamed Correspondence to: Nick S. Nurmohamed, MD, Departments of Vascular Medicine and Cardiology, Amsterdam UMC, University of Amsterdam, Meibergdreef 9, 1105 AZ Amsterdam, The Netherlands. Email E-mail Address: [email protected] https://orcid.org/0000-0001-9045-6009 Department of Vascular Medicine, Amsterdam University Medical Centers, University of Amsterdam, The Netherlands (J.M.K., G.K.H., E.S.G.S., N.S.N.). Department of Cardiology, Amsterdam University Medical Centers, Vrije Universiteit Amsterdam, The Netherlands (N.S.N.). Search for more papers by this author Originally published15 Jun 2023https://doi.org/10.1161/ATVBAHA.123.319272Arteriosclerosis, Thrombosis, and Vascular Biology. 2023;0FootnotesFor Sources of Funding and Disclosures, see page XXX.Correspondence to: Nick S. Nurmohamed, MD, Departments of Vascular Medicine and Cardiology, Amsterdam UMC, University of Amsterdam, Meibergdreef 9, 1105 AZ Amsterdam, The Netherlands. Email n.s.[email protected]nl Previous Back to top Next FiguresReferencesRelatedDetails Advertisement Article InformationMetrics © 2023 The Authors. Arteriosclerosis, Thrombosis, and Vascular Biology is published on behalf of the American Heart Association, Inc., by Wolters Kluwer Health, Inc. This is an open access article under the terms of the Creative Commons Attribution License, which permits use, distribution, and reproduction in any medium, provided that the original work is properly cited.https://doi.org/10.1161/ATVBAHA.123.319272PMID: 37317854 Originally publishedJune 15, 2023 Keywordsinflammationproteomicslipid-lowering therapyphenotypecardiovascular diseasePDF download Advertisement SubjectsAtherosclerosisDiagnostic TestingLipids and CholesterolSecondary Prevention

Hypertrophic neuropathy: a possible cause of pain in children with Noonan syndrome and related disorders

This study is aimed at describing the findings of high-resolution nerve ultrasound in children with Noonan syndrome (NS) and related disorders experiencing pain in their legs. This retrospective cohort study was conducted in the NS expert center of the Radboud University Medical Center in the Netherlands. Patients were eligible if they were younger than 18 years, clinically and genetically diagnosed with NS or a NS related disorder, and experienced pain in their legs. Anamneses and physical examination were performed in all children. In addition, high-resolution nerve ultrasound was used to assess nerve hypertrophy and, if needed, complemented spinal magnetic resonance imaging was performed. Over a period of 6 months, four children, three with NS and one child with NS with multiple lentigines, who experienced pain of their legs were eligible for inclusion. Muscle weakness was found in two of them. High-resolution nerve ultrasound showed (localized) hypertrophic neuropathy in all patients. One child underwent additional spinal magnetic resonance imaging, which showed profound thickening of the nerve roots and plexus. Conclusion: In the four children included with a NS and related disorders, pain was concomitant with nerve hypertrophy, which suggests an association between these two findings. The use of high-resolution nerve ultrasound and spinal magnetic resonance imaging might result in better understanding of the nature of this pain and the possible association to nerve hypertrophy in patients with NS and related disorders.What is Known:• Children with Noonan syndrome and related disorders may report pain in their legs, which is often interpreted as growing pain.• Some adults with Noonan syndrome and related disorders have hypertrophic neuropathy as a possible cause of neuropathic pain.What is New:• This is the first study using high-resolution nerve ultrasound in children with Noonan syndrome and related disorders experiencing pain in their legs.• Hypertrophic neuropathy was diagnosed as possible cause of pain in four children with Noonan syndrome and related disorders.