European Consensus Research Articles

Lomitapide for the treatment of paediatric patients with homozygous familial hypercholesterolaemia (APH-19): results from the efficacy phase of an open-label, multicentre, phase 3 study

Homozygous familial hypercholesterolaemia (HoFH) is a rare inherited disorder characterised by extremely high concentrations of LDL cholesterol, leading to early-onset atherosclerosis. Lomitapide is an orally administered microsomal triglyceride transfer protein (MTP) inhibitor that effectively lowers LDL cholesterol and is approved for adults with HoFH. We aimed to investigate the efficacy and safety of lomitapide in paediatric patients with HoFH receiving standard-of-care lipid-lowering therapy. APH-19 is an open-label, single-arm, phase 3 trial performed at 12 study centres in Germany, Israel, Italy, Saudi Arabia, Spain, and Tunisia. A 6-week run-in period was followed by a 24-week efficacy phase and an 80-week safety phase. Patients aged 5-17 years, on stable lipid-lowering therapy, with HoFH diagnosed using the criteria from the 2014 European Atherosclerosis Society Consensus Panel on HoFH were titrated to maximum tolerated doses of oral lomitapide, starting at 2 mg (patients aged 5-15 years) or 5 mg (patients aged 16-17 years). The primary endpoint was the percentage change from baseline to week 24 in LDL cholesterol, which was assessed in patients who had received at least one dose of lomitapide, and who had a baseline and at least one post-baseline measurement. The secondary outcomes were the percentage change from baseline at week 24 in total cholesterol, non-HDL cholesterol, VLDL cholesterol, apolipoprotein B, triglycerides, and lipoprotein(a). Safety was assessed in patients who received at least one dose of study drug. This study is registered with ClinicalTrials.gov, NCT04681170. Between Dec 20, 2020, and Oct 16, 2022, 43 patients were included and treated (24 [56%] were female and 19 [44%] were male, and median age was 10·7 years [7·0-14·0]). Mean change from baseline in LDL cholesterol at week 24 was -53·5% (95% CI -61·6 to -45·4, p<0·0001). Mean percentage reductions were observed at week 24 for non-HDL cholesterol (-53·9%, 95% CI -61·7 to -46·1, p<0·0001), total cholesterol (-50·0%, 95% CI -57·6 to -42·4, p<0·0001), VLDL cholesterol (-50·2%, -59·1 to -41·2, p<0·0001), apolipoprotein B (-52·4%, -60·3 to -44·5, p<0·0001), triglycerides was -49·9% (-58·8 to -41·0, p<0·0001), and lipoprotein(a) (-11·3%, -32·9 to 10·3 [in 21 patients with measurements in mg/dL]; -23·6%, -38·2 to -9·0 [in 22 patients with measurements in nmol/L]; p=0·0070 combined). Adverse events were mostly mild, and gastrointestinal and hepatic in nature. Adverse events of special interest were reported for five (12%) patients (gastrointestinal in two patients and hepatic in three). One serious treatment-emergent adverse event was reported (also classed as an adverse event of special interest): an increase in hepatic enzymes, resulting in two dose interruptions, two dose reductions, and a repeated dose escalation. Lomitapide provided a significant, clinically meaningful LDL cholesterol reduction and has the potential to be an efficient, LDL receptor-independent option for paediatric patients with HoFH. Amryt Pharmaceuticals.

Insights on Chinese and European consensus for USgHIFU treatment of pancreatic cancer

Insights on Chinese and European consensus for USgHIFU treatment of pancreatic cancer

Risk and determinants of sarcopenia in people with diabetes: a case–control study from Qatar Biobank cohort

BackgroundDiabetes is associated with impairments in muscle mass and quality increasing the risk of sarcopenia. Thus, this study aimed to investigate the odds of sarcopenia and its associated risk factors among Qatari adults (> 18 years), while exploring the modulating effects of health and lifestyle factors.MethodsUsing a case–control design, data from 767 participants (481 cases with diabetes and 286 controls without diabetes) was collected from Qatar Biobank (QBB). Sociodemographic, lifestyle factors including dietary intake, anthropometric and biochemical measures were analyzed. Handgrip strength, Dual X-ray absorptiometry (DXA), and Bio-impedance were used to assess muscle strength, muscle mass and muscle quality, respectively. The risk of sarcopenia was estimated using the European consensus on definition and diagnosis of sarcopenia.ResultsCases with diabetes were older (55 vs. 36 years; P < 0.001), had higher BMI (31.6 vs. 28.3 kg/m2; P < 0.001), lower cardiorespiratory fitness (50.0% “Moderate” fitness for cases, 62.9% “High” fitness for controls), and consumed less total (59.0 vs. 64.0; P = 0.004) and animal protein (39.0 vs. 42.0; P = 0.001), compared to controls based on a computed score. Participants with diabetes also had lower appendicular lean mass/BMI, handgrip strength, and higher probability of sarcopenia/probable sarcopenia (P < 0.005). Adjusted multiple logistic regression revealed that elevated cardiorespiratory fitness (β = 0.299, 95%CI:0.12–0.74) and blood triglycerides (β = 1.475, 95% CI: 1.024–2.124), as well as being a female (β = 0.086, 95%CI: 0.026–0.288) and having higher BMI (β = 0.908, 95%CI: 0.852–0.967) and ALM/BMI (β = 0.000, 95% CI: 0.000–0.007) are independent predictors (p < 0.05) of sarcopenia risk.ConclusionsThis study highlights the intricate relationship between diabetes and sarcopenia, revealing modifiable risk factors. Individuals with diabetes were found to have a higher likelihood of sarcopenia, which was associated with lower fitness levels and higher blood triglycerides. Protective factors against sarcopenia included being female and having higher BMI and ALM/BMI ratios.

Сеульський та Європейський консенсуси з питань розвитку: чи можлива спільна платформа по Україні?

In the context of the tasks of the post-war recovery of Ukraine’s economy and social sphere, a comparative analysis has been carried out of the conceptual foundations of international development assistance provided by the G20 countries, in particular the newly industrialized countries, within the framework of the Seoul Consensus on Development, announced at the G-20 Seoul Summit (2010), and the European Union and its member states within the framework of the new European Consensus on Development (2017). It is shown that the former focuses on growth primarily through investment in infrastructure and solving long-term structural problems of development, and the latter on increasing well-being and fighting poverty. The Seoul Consensus, despite the participation of Western donors, including the EU, in its formulation, largely reflects the principles and messages of newly emerging donors representing the leading new industrial states. The Seoul Consensus is currently a unique strategic compromise on international development aid not only between the West represented by its leading world powers and the European Union, on the one hand, and the largest new industrial states of the modern world - on the other, but also between the Beijing Consensus and the Mumbai Consensus - alternative strategies of socio-economic modernization and sustainable economic growth put forward by China and India, respectively, in their active competition for leadership among the countries of the Global South. The change in the geopolitical and geo-economic landscape has forced the EU to review the criteria for the distribution and prioritization of development aid in order to be able to compete and cooperate with new donors such as China, South Korea, Saudi Arabia, Turkey, etc. The new European consensus emphasizes the instrumentalization and securitization of development, with less attention to democracy and human rights. This shift objectively narrows the gap between traditional and new donors of international development assistance. The author substantiates the prospect of further analysis of the convergent potential of the above-mentioned consensuses in the direction of developing based on the unique geopolitical and geo-economic positioning of Ukraine in the modern world, the "Kyiv Consensus on Development" as a joint conceptual, strategic and political platform of the international donor community for the post-war reconstruction of Ukraine.

Does an Inpatient Rehabilitation Admission Via the Community Prevent Hospital Readmissions in Older Adults? An Integrated Care Initiative

Abstract Background Rehabilitation interventions are essential in supporting frail older adult’s recovery following periods of decompensation and preventing future hospitalizations. Previously patients (age &amp;gt; 65 years) could only access inpatient rehabilitation via an inpatient stay in an acute hospital. Within the ICT (Integrated Care Team) in Dublin North West (DNW) a community rehabilitation pathway to two inpatient rehabilitation units exists. We retrospectively evaluated patients that were admitted to rehabilitation units via this pathway and measured their 90-day readmission rate to their local acute hospital. Methods 17 referrals were received from three pathways: Primary Care, the Holly Day clinic and the Frailty Intervention Team (FITT). Clinical frailty score’s (CFS), Falls risk (as per the world falls guidelines) and sarcopenia status as per the European Consensus on the definition of sarcopenia (EWGSOP) were recorded prior to admission. Readmissions within 90 days were collected from the hospital electronic database. Results CFS scores ranged from 5 to 7. All patients were identified as a high risk of falls and sarcopenic. The 90-day readmission rate was 11%. 5.5% of these were due to falls and the other 5.5% was due to the exacerbation of a pre-existing condition. All had CFS score of &amp;gt;6. Conclusion Readmission to acute care is not common in our patient cohort and shows the involvement of the ICT Geriatrician and Physiotherapist in identifying and referring frail older adults for inpatient rehab is successful. Ongoing development of interagency pathways and further referrals is needed to further progress this pathway and measure its efficacy in preventing readmission to an acute hospital. A 90 day and 180 day follow up and falls risk outcome measure pre and post admission would further demonstrate the success of this initiative and help measure the efficacy of this initiative in reducing the risk of preventable hospitalizations in older adults.

PROs for RARE: protocol for development of a core patient reported outcome set for individuals with genetic intellectual disability

IntroductionRare genetic neurodevelopmental disorders and intellectual disability (ID), collectively called genetic ID (GID), can profoundly impact daily functioning and overall well-being of affected individuals. To improve our understanding of the impact of GID and advancing both care and research, measuring relevant patient reported outcomes (PROs) is crucial. Currently, various PROs are measured for GID. Given the shared comorbidities across disorders, we aim to develop a generic core PRO set for children and adults with GID.Methods and resultsDeveloping the generic core PRO set entails the following steps: 1) providing an overview of potentially relevant PROs by scoping reviews and qualitative research; 2) integrating and conceptualizing these PROs (i.e., describing the content of the PROs in detail) into a pilot generic core PRO set; and 3) prioritizing relevant PROs by a European Delphi survey and consensus meetings.ConclusionsThis protocol presents the steps for developing a generic core PRO set for children and adults with GID. The next step involves selecting suitable patient reported outcome measures (PROMs) to adequately measure these PROs: the generic core PROM set. This generic core PROM set needs validation in the GID population, and eventually implementation in care and research, facilitating the aggregation and analysis of PRO data and guaranteeing continuous integration of the patient perspective in both care and research.

Comparison of the effect of surfactant administration during nasal continuous positive airway pressure with that of nasal continuous positive airway pressure alone on respiratory distress syndrome

Background. The American Academy of Pediatrics states that prophylactic surfactant is no longer advised when continuous positive airway pressure (CPAP) is frequently used to stabilize preterm newborns and where the rate of prenatal corticosteroid is high. On the other hand, the European Consensus Guidelines on the Management of Respiratory Distress Syndrome (RDS) urge giving prophylactic surfactants to infants with RDS as early as possible. In this study, we aim to investigate the appropriate guidelines to be adopted in our neonatal intensive care unit about selective or elective INSURE (intubation-surfactant-extubation) method. Materials and methods. This is a clinical trial in which a total number of 120 neonate patients were diagnosed with RDS and had gestational age of 30–34 weeks. Two neonatal intensive care units have been included in the study: group A (n = 87) was treated with CPAP plus INSURE and group B (n = 33) was treated with CPAP only. The primary outcome we searched for is the fate of the patients. The secondary outcomes were length of hospital stay, CPAP management, oxygen therapy, and RDS complications. Results. Group B show a shorter length of hospital stay and CPAP treatment; however, this difference was not statistically significant. On the other hand, oxygen therapy duration was significantly shorter in group B than in group A. Both groups show very close rates of RDS complications (32 vs 33 %). Regarding the primary outcomes, group A had a better survival rate in comparison to group B (88.5 vs 78.8 %); however, this difference was not significant, with P-value of 0.173. Conclusions. In neonates with RDS, the routine INSURE method followed by CPAP gives no more superiority in outcomes (both primary, i.e. survival rate, and secondary, i.e. complications) than CPAP alone.

Blackmailing and Identity Profiling? The Behaviour of Populist Radical Right Governments in EU Development Policy

EU development policy has in recent years become more contested and politicised. One key factor driving this trend is the increasing influence of populist radical right parties (PRRPs). Previous studies have focused on PRRPs as opposition parties. This contribution breaks new ground by exploring the behaviour of PRRP-led governments in EU development policy. More specifically, the article analyses how and to what extent this behaviour is characterised by “unpolitics,” an approach to undermining EU policymaking. We define “cross-policy blackmailing” and “identity profiling” as two potential strategies of unpolitics in EU development policy and probe these in two case studies. The first case concerns the New European Consensus on Development and the second is the EU’s positioning in the negotiations and signing of the Samoa Agreement. In the first case, we find that PRRP-led governments fundamentally rejected the decision-making rules as well as the norms on migration, gender, and sexual and reproductive health rights, using a strategy of identity profiling. In the second case, PRRP-led governments significantly stalled the conclusion of the agreement by combining cross-policy blackmailing and identity profiling. Based on this analysis, we generalise on the scope conditions of unpolitics in EU development policy.

Exploring the role of calf circumference as a predisposing factor for intra-hospital delirium: investigating potential gender differences: revealing potential gender variances

BackgroundMalnutrition and sarcopenia significantly increase the risk of intra-hospital delirium, particularly among older adults. Given the potential correlation between calf circumference (CC) and these conditions, CC emerges as a promising predisposing factor for delirium. This study aims to investigate the independent association between delirium and anthropometric parameters, focusing on evaluating CC’s predictive capacity for intra-hospital delirium risk. Additionally, it aims to compare CC’s predictive performance with the widely used Mini Nutritional Assessment (MNA), while also considering potential gender disparities.MethodsThis is a retrospective study which enrolled patients aged ≥ 65 years from September 2021 to March 2022 at the Padova Hospital (Italy). Physical characteristics, intra-hospital delirium incidence, and body composition were assessed. Sarcopenia was diagnosed using the 2019 European Consensus criteria.ResultsAmong 207 subjects, delirium affected 19% of patients. CC showed a significant association with intra-hospital delirium among the analyzed anthropometric parameters. ROC curves indicated that CC’s predictive capacity for delirium onset was comparable to MNA (p = 0.98), particularly in women. In a multivariable logistic regression model, female gender and higher cognitive and CC scores emerged as protective factors against delirium onset, with each unit increase in CC associated with a 24% reduction in the odds of delirium. Conversely, sarcopenia did not significantly influence delirium onset.ConclusionsCC shows promise as a predisposing factor for intra-hospital delirium, similar to MNA, albeit with significant gender differences. CC could serve as a valuable tool for assessing delirium risk among female patients. Further validation of these findings is necessary through larger-scale studies.

Cardiologists' perspective on termination of pacemaker therapy-an anonymous survey among cardiologists in Germany.

The patient's right to refuse pacemaker therapy is mentioned in the relevant European consensus statement but additional information is only available on deactivation of implantable cardioverter deactivator and not on other cardiac implantable electronic devices such as pacemakers. Therefore, we were interested in opinions, concerns and attitudes of cardiologists, who are the primary contact persons for such requests, since the number of patients asking for withdrawal of pacemaker therapy is likely to increase leaving cardiologists and healthcare professionals with a difficult medical but also ethical problem. An anonymous questionnaire was sent to all German cardiology departments (N = 288). 48% of cardiology departments responded by sending back 247 completed questionnaires. Most participating cardiologists were experienced when considering the duration of their professional activity. Almost all of the respondents regularly perform check-ups of pacemakers. The majority of cardiologists answering our questionnaire were prepared to deactivate a pacemaker upon patients' request, and have done so. In pacemaker dependency, however, the willingness to withdraw decreases, even if death is imminent, for fear of causing distressing symptoms, sense of being responsible for patients possible immediate death, or fear of legal consequences. The survey could clearly show that uncertainties remain among cardiologists dealing with a patient's wish for withdrawal, especially in cases of pacemaker dependency. We suggest that official statements of cardiologic societies in Europe are issued to clarify ethical, legal and practical aspects of pacemaker withdrawal. Registered in the German Clinical Trials Register (DRKS00026168) on 30.08.2021.

Impact of bone marrow fibrosis on outcomes of allogeneic hematopoietic stem cell transplantation in acute myeloid leukemia.

Bone marrow fibrosis (BMF) of unknown etiology was common in hematological malignancies, but its prognostic value for acute myeloid leukemia (AML) is unclear. We interrogated data from 532 newly diagnosed subjects with AML receiving allogeneic hematological stem cell transplantation to evaluate the prognostic impact of BMF on transplant outcomes. Using the European consensus on the grading of BMF at diagnosis, 255 (48%) subjects were BMF-0, 209 (39%), BMF-1 and 68 (13%), BMF-2-3. Subjects with BMF-2-3 had poor overall survival (P < 0.001), disease-free survival (P < 0.001) and a higher incidence of relapse (CIR, P < 0.001). Multi-variable analyses in subjects achieving pre-transplant complete remission showed BMF-2-3 was an independent risk factor for CIR (Hazard Ratio [HR] = 2.17, (95% CI, 1.11, 4,24); P = 0.02). Furthermore, BMF-2-3 group showed delayed neutrophil and platelet engraftment and delayed B cell recovery post-transplantation. These findings demonstrate the significance of BMF in transplant outcomes and attract more attention to AML with BMF.

Assessment and monitoring of lung disease in patients with severe alpha 1 antitrypsin deficiency: a european delphi consensus of the EARCO group

BackgroundCurrently, there is conflicting information and guidance on the effective management of Alpha 1 Antitrypsin Deficiency (AATD). Establishing a consensus of assessment and disease management specific to AATD is important for achieving a standardized treatment pathway and for improving patient outcomes. Here, we aim to utilize the Delphi method to establish a European consensus for the assessment and management of patients with severe AATD.MethodsTwo rounds of a Delphi survey were completed online by members of the European Alpha-1 Research Collaboration (EARCO). Respondents were asked to indicate their agreement with proposed statements for patients with no respiratory symptoms, stable respiratory disease, and worsening respiratory disease using a Likert scale of 1–7. Levels of agreement between respondents were calculated using a weighted average.ResultsRound 1 of the Delphi survey was sent to 103 members of EARCO and 38/103 (36.9%) pulmonologists from across 15 countries completed all 109 questions. Round 2 was sent to all who completed Round 1 and 36/38 (94.7%) completed all 79 questions. Responses regarding spirometry, body plethysmography, high-resolution computed tomography, and the initiation of augmentation therapy showed little variability among physicians, but there was discordance among other aspects, such as the use of low-dose computed tomography in both a research setting and routine clinical care.ConclusionsThese results provide expert opinions for the assessment and monitoring of patients with severe AATD, which could be used to provide updated recommendations and standardized treatment pathways for patients across Europe.

Dynamic analysis of the relationship between systemic lupus erythematosus disease activity and psychosocial support.

Systemic lupus erythematosus (SLE) is a multi-system autoimmune disease that significantly affects both the physical and mental health of patients. Psychosocial support systems play a crucial role in managing chronic diseases, yet their specific impact on the disease activity of SLE patients remains unclear. This study aims to explore the dynamic relationship between disease activity in SLE patients and various types of psychosocial support systems. We conducted a retrospective longitudinal observational study, including 150 SLE patients who received treatment at our hospital from January 2022 to January 2023. Emotional support, tangible support, social interaction support, and informational support were assessed using the revised Social Support Rating Scale. Disease activity was quantified using the European Consensus Lupus Activity Measurement. The relationship between psychosocial support and disease activity was analyzed using Spearman's rank correlation coefficient and multiple linear regression models, with Bootstrap resampling employed to test the robustness of the results. We found a significant negative correlation between psychosocial support and SLE disease activity, with emotional support, social interaction support, and informational support showing stronger negative correlations. Multiple regression analysis revealed that the inhibitory effects of emotional support, social interaction support, and informational support on disease activity increased over time. Although the impact of tangible support was not statistically significant, it gradually became more apparent over time. Our findings indicate a significant negative correlation between psychosocial support and SLE disease activity, particularly with emotional support, social interaction support, and informational support. Over time, the impact of tangible support also becomes evident. These findings provide important references for the comprehensive treatment and management of SLE patients. However, due to the observational nature of the study, the causality of this relationship requires further exploration.

A European Consensus on the Consistent Use of the Term "Keratinocyte Cancer".

Keratinocyte-derived skin cancers comprise basal cell carcinoma, squamous cell carcinoma, its precursor actinic keratosis, and Bowen's disease. Historically, this group of neoplasms has been subsumed under the term non-melanoma skin cancer. However, the term non-melanoma skin cancer can be misleading and lacks precision. Therefore, more precise and reasonable terminology, valuing the relevance of keratinocyte-derived cancer, appears pertinent to meet its clinical and scientific significance. A group of experienced dermato-oncologists initiated a consensus approach to promote the use of the term "keratinocyte cancer" instead of "non-melanoma skin cancer" when referring to carcinomas and their precursors that are derived from keratinocytes. The vote among members of the consensus group indicated unanimous agreement on the consistent use of the term "keratinocyte cancer" instead of "non-melanoma skin cancer". International delegates also voted in favour of the revised terminology. The more precise and, by means of etiopathogenesis, correct term "keratinocyte cancer" should be consistently used for malignancies originated from keratinocytes. This is expected to have a positive impact on patient-physician communication and gives better justice to this important group of keratinocyte-derived cancers.

The association between HIV-related stigma, HIV knowledge and HIV late presenters among people living with HIV (PLHIV) attending public primary care clinic settings in Selangor.

HIV late presenters were defined as individuals presenting with a CD4 count below 350 cells/μL or with an AIDS-defining event, according to the European Late Presenter Consensus working group. Early diagnosis and treatment of HIV have proven beneficial for people living with HIV (PLHIV), reducing the burden on healthcare systems, and contributing to ending the HIV/AIDS epidemic. However, in Malaysia, over 50% of newly diagnosed HIV patients present late, leading to increased morbidity and premature mortality. This study aims to determine the prevalence of late HIV presenters and its association with HIV-related stigma and HIV knowledge among PLHIV attending public primary care clinics in Selangor. A cross-sectional study was conducted at selected public health clinics in Selangor, involving PLHIV aged 18 years and older, who were diagnosed since 2019. HIV-related stigma was measured using the Malay version of Berger's HIV Stigma Scale, and HIV knowledge was assessed using the Malay version of Brief HIV-KQ-18. Univariate and multivariate logistic regression analyses were performed to identify factors associated with late HIV presentation. A total of 400 participants were included in the study, with 60.0% (n = 240, 95% CI: 55.0-65.0) classified as late presenters. The participants had a mean age of 30.29 (±7.77) years. The risk factors for late presenters were high levels of HIV-related stigma (aOR = 1.049, 95% CI: 1.034-1.063, p-value <0.001), low levels of HIV knowledge (aOR = 0.709, 95% CI: 0.646-0.778, p-value <0.001), tertiary education background (aOR = 15.962, 95% CI: 1.898-134.235, p-value = 0.011), and being single (aOR = 3.582, 95% CI: 1.393-9.208, p-value = 0.008). This study highlights the association between high levels of HIV-related stigma, low levels of HIV knowledge, and late HIV presentation. Interventions targeting stigma reduction and HIV education can promote early testing and prompt access to care, improving health outcomes for PLHIV.

Acute generalized exanthematous pustulosis: European expert consensus for diagnosis and management.

Acute generalized exanthematous pustulosis (AGEP) is a rare, usually drug-induced, acute pustular rash. Despite the lack of strong data supporting the effectiveness of topical or systemic corticosteroids in this drug reaction, they are widely used. More generally, there is no consensus on the diagnostic modalities and the management of patients with AGEP. We aimed to provide European expert recommendations for the diagnosis and management or patients with AGEP. Members of the ToxiTEN group of the European Reference Network (ERN)-skin, all dermatologists and/or allergologists with expertise in drug reactions, elaborated these recommendations based on their own experience and on a review of the literature. Recommendations were separated into the following categories: professionals involved, assessment of the diagnosis of AGEP, management of the patient and allergological work-up after the acute phase. Consensus was obtained among experts for the list of professionals involved for the diagnosis and management of AGEP, including the minimum diagnostic work-up, the setting of management, the treatments, the modalities and the timing of allergological work-up and follow-up. European experts in drug allergies propose herein consensus on the diagnosis and management of patients with AGEP. A multidisciplinary approach is warranted, including dermatologists, allergologists and pharmacovigilance services.

Home Birth from a Comparative Legal Perspective

The reproductive rights of women touch upon healthcare provided during childbirth, which involve freedom of choice of the place of childbirth. Thus, the issue of home birth is of interest among the legislators, which may be interpreted as part of an ”obligation” posed by international legal documents to ensure that respectful care is provided to women during the whole time of pregnancy. However, currently, there is no evident European consensus on how home birth shall be regulated thus, the legal approach is different among European states. The decision-making of the European Court on Human Rights has influenced domestic legislation, especially in Hungary (Ternovszky v. Hungary case), however, there was a completely different outcome in the Czech Republic in a factually very similar case (Dubská and Krejzová v. Czech Republic). Accurately, what is the woman’s role during childbirth? One model suggests that she is the passive side, and the physician has the main role in successfully conducting the birth. The other model follows the ideal, that childbirth is centred around the woman, and her competency in her body and mind makes her the one in control. Nevertheless, alternative birthing methods are not newfangled in some countries such as the Netherlands, where traditional midwife-supervised home birth can be carried out if a woman chooses so, unlike in Slovakia where this freedom of choice is not evident and discouraged by the legislators. This study tries to shed light on why legislation on this issue is so different in these European states.

Development of an ontology for laparoscopic transabdominal adrenalectomy via a comprehensive modified Delphi survey and its validation on a multicentric pilot data set for surgical training and future video analysis with machine learning algorithms.

Lay Summary Surgical technique is essential to ensure safe minimally invasive adrenalectomy. Due to the relative rarity of adrenal surgery, it is challenging to ensure adequate exposure in surgical training. Surgical video analysis supports auto-evaluation, expert assessment and could be a target for automatization. The developed ontology was validated by a European expert consensus and is applicable across the surgical techniques encountered in all participating centres, with an exemplary demonstration in bi-centric recordings. Standardization of adrenalectomy video analysis may foster surgical training and enable machine learning training for automated safety alerts.

2024 update: European consensus statement on gene therapy for spinal muscular atrophy

Spinal muscular atrophy (SMA) is one of the most common genetic diseases and was, until recently, a leading genetic cause of infant mortality. Three disease-modifying treatments have dramatically changed the disease trajectories and outcome for severely affected infants (SMA type 1), especially when initiated in the presymptomatic phase. One of these treatments is the adeno-associated viral vector 9 (AAV9) based gene therapy onasemnogene abeparvovec (Zolgensma®), which is delivered systemically and has been approved by the European Medicine Agency for SMA patients with up to three copies of the SMN2 gene or with the clinical presentation of SMA type 1. While this broad indication provides flexibility in patient selection, it also raises concerns about the risk-benefit ratio for patients with limited or no evidence supporting treatment.In 2020, we convened a European neuromuscular expert working group to support the rational use of onasemnogene abeparvovec, employing a modified Delphi methodology. After three years, we have assembled a similar yet larger group of European experts who assessed the emerging evidence of onasemnogene abeparvovec's role in treating older and heavier SMA patients, integrating insights from recent clinical trials and real-world evidence. This effort resulted in 12 consensus statements, with strong consensus achieved on 9 and consensus on the remaining 3, reflecting the evolving role of onasemnogene abeparvovec in treating SMA.

Margin of Appreciation in Interpreting Freedom of Religion: A Critical Appraisal on Role of European Court of Human Rights

This intricate analysis delves into the discernible inconsistencies characterizing the European Court of Human Rights’ (ECtHR) application of the margin of appreciation (MoA) doctrine concerning Article 9 of the European Convention on Human Rights. The evident absence of explicit guidelines delineating the parameters of the margin, exemplified by the Kokkinakis case, results in a perplexing and contradictory implementation of this legal tool. The Court’s vacillation in standards of review, notably illustrated in Sahin’s and Lautsi’s cases, underscores a jurisprudential ambivalence in matters of religious expression. Moreover, the Court’s oscillation in calibrating the margin, as observed in S.A.S v. France and Eweida and Others v. UK, raises valid concerns about judicial impartiality and potential predilections. The reliance on the absence of European consensus, as seen in cases like Dahlab v. Switzerland and Lautsi v. Italy, introduces an element of interpretive subjectivity that may undermine the robustness of human rights imperatives. In light of these complexities, this analysis calls for a more lucid, principled and judicious application of the MoA doctrine by the ECtHR to ensure the consistent protection of fundamental human rights within the European legal framework.