International Cross-sectional Study Research Articles

Nurse educator education in six European countries: a descriptive study / Ausbildung von Pflegepädagog/-innen in sechs europäischen Ländern – eine deskriptive Studie

Abstract Objectives Information on nurse educator education is scarce. The present study thus aims to provide an overview of the requirements for and standards of nurse educator education in six European countries in order to enable further reflection and promote discourse on the topic. Methods: A descriptive international cross-sectional comparative study was conducted across six European countries. Data were collected via an online questionnaire completed by experts in nurse educator education or in organisations with specialist knowledge about nurse educator preparation (n = 11). The data were analysed, compared, and condensed. Results The participating countries differ in terms of their educational requirements and the working environments they provide for nurse educators. Formal nurse educator education is available in three of the six countries. Furthermore, in the countries with formal education for nurse educators, differences exist in terms of the regulation and implementation of the education. The educational requirements of nurse educators in higher education are mostly not specific to nursing and are instead general requirements for professional (tertiary) or higher education. Conclusions The analysis reveals heterogeneous regulations, standards, and pathways in the education of nurse educators in the six different counties included in the study. Despite some formal and content-related similarities, no uniform picture of the educational requirements, programmes, or standards was identified. The results of this international comparative research confirm the need for further research that examines whether the education of nurse educators meets the global need both for skilled nurses and for more Europe-wide collaboration in nurse educator education.

Prevalence, predictors and types of unpleasant and adverse effects of meditation in regular meditators: international cross-sectional study

BackgroundMeditation is commonly implemented in psychological therapies since the ‘third wave’ of cognitive–behavioural therapy has increased the focus on mindfulness-based interventions. Although extensive research literature demonstrates its benefits, little is known about potential adverse effects.AimsThe aim of this study is to report the prevalence, type and severity of particularly unpleasant meditation-related experiences in the largest cross-sectional study on this topic to date, with 1370 regular meditators.MethodThe participants were asked whether they ever encountered particularly unpleasant experiences as a result of their meditation experience. For the first time, the type and severity of those experiences were assessed and the association with several predictors, such as pre-existing mental disorders, were explored via logistic and linear regression.ResultsSimilar to previous studies, 22% of participants (95% CI 20–24) reported having encountered unpleasant meditation-related experiences, and 13% of participants (95% CI 3–5) reported experiences that were categorised as adverse. Those were mostly of affective, somatic and cognitive nature. Unpleasant meditation-related experiences were more likely to occur in participants with pre-existing mental illnesses (P= 0.000, 95% CI 1.25–2.12).ConclusionsThis study demonstrates that unpleasant meditation-related experiences are prevalent among meditators and, to a relevant extent, severe enough to warrant further scientific inquiry. Longitudinal studies are needed to examine whether the unpleasant meditation-related experiences are merely negative and thus should be avoided, or are an inherent part of the contemplative path.

Physical and psychological correlates of self and body compassion in women with polycystic ovary syndrome.

This international cross-sectional study examined the level and potential correlates of self and body compassion in women (n = 227) with polycystic ovary syndrome (PCOS). Participants completed an online survey examining quality of life, psychological health, body image and self and body compassion. Low levels of self and body compassion were found, with depression and body image concern identified as negative correlates of self and body compassion. Physical health was identified as a positive correlate of body compassion. The findings identify the important relationship between body image, psychological health and self and body compassion in women with PCOS.

Urinary Incontinence in Competitive Women Powerlifters: A Cross-Sectional Survey

BackgroundUrinary incontinence (UI) can negatively affect a woman’s quality of life, participation in sport and athletic performance. The objectives of this study were to determine the prevalence of UI in competitive women powerlifters; identify possible risk factors and activities likely to provoke UI; and establish self-care practices.MethodsThis international cross-sectional study was conducted using an online survey completed by 480 competitive women powerlifters aged between 20 and 71 years. The Incontinence Severity Index (ISI) was used to determine the severity of UI.ResultsWe found that 43.9% of women had experienced UI within the three months prior to this study. The deadlift was the most likely, and the bench-press the least likely exercise to provoke UI. ISI scores were positively correlated with parity (τ = 0.227, p < 0.001), age (τ = 0.179, p < 0.001), competition total (τ = 0.105, p = 0.002) and body mass index score (τ = 0.089, p = 0.009). There was no significant correlation between ISI and years strength training (τ = − 0.052, p = 0.147) or years powerlifting (τ = 0.041, p = 0.275). There was a negative correlation between ISI score with having a pelvic floor assessment (η = 0.197), and the ability to correctly perform pelvic floor exercises (η = 0.172).ConclusionThe prevalence of UI in this cohort was at the upper limit experienced by women in the general population. Women who had undergone a pelvic floor examination or were confident in correctly performing pelvic floor exercises experienced less severe UI.

Age and gender aspects of the association of generalized obesity with development of fatal and nonfatal cardiovascular events (results of the prospective cohort study “Interepepid”)

Background. Currently, obesity has become one of the most important medical and social problems in the world due to its high prevalence. Numerous literature data indicate that high BMI is associated with increased mortality from cardiovascular diseases.The aim: to study the relationship between overweight and generalized obesity with the development of fatal and non-fatal cardiovascular events among residents of the Chui region of the Kyrgyz Republic, taking into account gender and age.Material and methods. This study was carried out as part of the international crosssectional epidemiological study “Interepid”. The total follow-up period was 7 years. Life status at the end of the follow-up period was monitored in 1096 respondents out of 1341 initial cohorts (response – 82.1 %). The endpoints were cases of fatal and non-fatal cardiovascular events. The frequency of events was assessed using methods of analysis of survival (Cox proportional hazard regression model, parametric survival models, survival by the Kaplan – Meier method).Results. A total of 181 cases of fatal and non-fatal cardiovascular events (16.44 %) were recorded during the follow-up. In persons with normal body weight, the incidence of cardiovascular complications was 10 %. In overweight individuals, the value of this indicator increased and reached 17.99 % (p˂ 0.001), and in individuals with obesity – 24.5 % (p˂ 0.0001). The increase in the frequency of events with the increase in body mass index did not depend on the gender of the respondents but was typical only for people under 60 years of age. For elderly patients, there was no relationship between BMI and the development of cardiovascular complications. In contrast, the relative risk had an insignificant tendency to decrease by 2.6 % for every 1 kg/m2 increase in body mass index (RR – 0.974; 95% CI: 0.924–1.025).Conclusion. The increase of body mass index above 25 kg/m2 is an independent risk factor for the development of fatal and non-fatal cardiovascular events in the analyzed cohort of the Chui region residents, regardless of gender, but only in persons under 60 years of age.

The Impact of the COVID-19 Pandemic on Resident Physicians Well-Being in the Surgical and Primary Care Specialties in the United States and Canada.

Purpose: The COVID-19 pandemic disrupted the professional, social, and spiritual activities of resident physicians around the world, impacting wellness and personal relationships. Moreover, social distancing caused significant limitations or shutdown of places of worship, including churches, synagogues, mosques, etc. Our goal was to survey resident physicians in primary care and surgical subspecialties in the United States (U.S.) and Canada and to examine the effect of the COVID-19 pandemic on their well-being.Methods: An international cross-sectional study was performed in November 2020, using an anonymous survey of programs in the U.S. and Canada, containing 20 questions to assess the impact of the pandemic on resident participation in social and spiritual activities and the effects on their wellness, and personal relationships. The emails with survey links attached were sent to individual program coordinators from accredited residency training programs in the United States and Canada. This consisted of programs accredited by the American Osteopathic Association (AOA), The Royal College of Physicians and Surgeons of Canada (RCPSC), and the Accreditation Council of Graduate Medical Education (ACGME). The survey was evenly divided among surgical programs (General Surgery, Neurological Surgery, Orthopedic Surgery, Urological Surgery, and Integrated Surgical Residency Programs such as Plastic Surgery, Cardiothoracic Surgery, Pediatric Surgery, and Vascular Surgery) as well as primary care programs (Internal Medicine and Family Medicine).Results: A total of 196 residents, 60 primary care residents, and 136 surgery residents participated in the study. Ninety-six participants (49%) were female, and 98 of the participants (50%) were male, with the remainder two residents identifying as “Other.” Of the primary care residents, the majority (39, 65%) were female. Conversely, the majority (77, 57%) of surgery residents were male.Conclusion: The COVID-19 pandemic has affected the social lives, relationships, and spiritual well-being of both surgical and primary care resident physicians. However, primary care residents reported significantly greater engagement in personal relationships and were more likely to express feelings of mental and physical exhaustion, prohibiting social attendance.

Abstract 9007: Higher Level of Chronic Illness Management is Associated With Greater Trust in the Heart Transplant Team

Trust is associated with better patient outcomes, such as better medication adherence and improved patient satisfaction. However, the association between trust and level of chronic illness management (CIM) has not yet been addressed. We assessed the hypothesis that level of CIM will be associated with trust in the HTx team. Additionally, we explored potential meso-level moderators of this relationship (time spent on patient visits, number of visits, multidisciplinarity, CIM competencies, and CIM preparedness of the healthcare team). A secondary analysis was conducted using the international cross-sectional BRIGHT study, which included 1397 HTx recipients and 100 HTx clinicians from 36 HTx centers across 11 countries. Level of CIM was measured using the Patient Assessment of Chronic Illness Care (PACIC – patients’ report) and the CIMI BRIGHT (clinicians’ reports). Patients’ trust in the HTx team was measured using an adapted Wake Forest Trust scale. Multiple logistic regression was used to test the hypothesis. 1397 patients (mean age 53.6 13.2 years; 72.7% male; 85.9% white; average time post HTx 3.4 1.4 years) and 100 HTx clinicians (mean age 46.2 10.2 years; 87% female; 85% nurses; mean time worked in HTx center 10.0 7.5 years) were included. Even after controlling for patient’s age, gender, race, education, employment, years post HTx, health literacy, language congruency, and comorbidities, level of CIM as assessed by both patients and clinicians was significantly associated with trust in the HTx team (odds ratio (OR)=1.85, p&lt;.001; OR=1.35, p=.012, respectively). Patients from centers with higher levels of CIM were more likely to trust their HTx team. Lastly, only ‘ time spent on patient visits ’ significantly moderated the relationship between level of CIM (PACIC and CIMI) and trust (OR=1.61, p=.037; OR=1.56, p=.048, respectively), wherein the impact of time in fostering trust towards the HTx team was greater when level of CIM was high. In conclusion, level of CIM (assessed from patients’ and clinicians’ perspectives) was positively associated with trust. Our findings also suggest that allotting more time for patients during follow-up visits is a relevant factor to consider to further optimize the HTx care ecosystem.

Nursing education in the pandemic: A cross-sectional international study

BackgroundThe COVID-19 pandemic has raised the need for distance learning in nursing education. The necessary precautions have been taken in nursing schools involving the application of various restrictions, including the suspension of face-to-face classes and the closure of educational institutions, and this has had a profound effect on nursing educators and nursing students alike. ObjectivesThe study seeks to answer the following questions:1)What are the characteristics of distance nursing education during the pandemic?2)What are the nursing students' views on online education during the pandemic?3)What difficulties have been experienced by nursing educators during the pandemic?4)What are the views and suggestions of nursing educators in regards to nursing education during the pandemic? Designdescriptive, cross-sectional, multicentered and international study. SettingsAn online survey was completed by 30 nursing educators working in establishments listed among the top 60 highest-ranked nursing schools in the world. Participantsnursing educators in undergraduate nursing programs. MethodAn internet-based survey comprising open-ended and multiple choices questions was disseminated to 60 nursing schools on the 2020 QS World University Ranking list. ResultsSurvey responses were received from 30 nursing schools in 30 countries. Since the announcement of the pandemic, the structure of distance education in nursing has taken different forms from one country to another, and nursing educators and students alike have encountered a diversity of problems during this process. The findings of the present study reveal that 65% of the nursing educators thought that they had been caught unprepared for the COVID-19 outbreak, 44% thought that the nursing program outcomes had been achieved through distance education, and 48% encountered Internet-related problems. ConclusionWe believe that the present study will (i) aid in the decisions of nursing educators considering a transition to distance education, provide suggestions to those that have already made such a transition or inspire those seeking to improve the effectiveness of practice in obligatory cases, (ii) serve as a guide for educational institutions, and (iii) contribute to the taking of precautions to counter potential problems.

PAID-PL-The Polish Version of the Problem Areas in Diabetes Scale: Perfect Reliability and a One-Factor Structure.

PurposeThe aim of this study was to assess the structure and validate the Polish version of the Problem Areas in Diabetes (PAID) scale, as the current translations of the original English version significantly vary in their psychometric properties.Patients and MethodsTwo hundred and sixteen consecutive Polish outpatients were invited to participate in this international cross-sectional study on depression in diabetes. The research was based on the demographic and clinical characteristics of the study population, including the level of glycated hemoglobin (HbA1c) and scores obtained in the Polish versions of the following questionnaires: PAID, World Health Organization-Five Well-Being Index (WHO-5), Patient Health Questionnaire 9 (PHQ-9). The psychiatric diagnosis was conducted with the use of Mini-International Neuropsychiatric Interview (M.I.N.I.).ResultsExploratory factor analyses yielded a 1-factor structure that included all 20 items. The internal consistency of the Polish version of PAID was high (Cronbach α = 0.97). There were significant positive correlation between PAID and PHQ-9 and a negative correlation between PAID and WHO-5. We also observed a negative association between PAID scores and age and a positive correlation between PAID and HbA1c levels. Patients with depression reported significantly higher PAID scores as compared with those without depressive symptoms.ConclusionThe Polish version of PAID has a one-factor structure and is a reliable, valid outcome measure for Polish outpatients with type 2 diabetes and it may constitute a useful instrument for screening for psychologic issues in diabetic patients during their appointments at the diabetes clinic.

Evaluation of Research Opportunities and Limitations for Plastic Surgery Residents: An International Survey.

Conducting research during specialty training provides an opportunity to develop critical thinking and leadership skills along with a better understanding of the scientific literature. However, trainees often find it difficult to undertake research, in the context of labor-intensive surgical training. The aim of this study is to evaluate the research output and limitations of plastic surgery residents in different countries. An international cross-sectional study involving plastic surgery trainees and recent postgraduates from Brazil, Chile, Germany, and the United Kingdom was conducted. A survey inquiring into academic productivity, limitations to conducting research, and working-hours patterns was distributed among eligible participants. From September to December 2019, 106 surveys were retrieved. Most respondents declared having participated in at least 1 project that resulted in a presentation or publication during their training (90.6% in national presentations, 68% international presentations, 67% in national publications, and 66% international publications). Having completed a previous research fellowship was associated with a statistically higher academic output (P < 0.05). Seventy-nine percent of respondents felt that their participation in research activities would have been greater if limiting factors had been addressed, including lack of time (72.5%) and insufficient supervision and mentoring (55%). Optimizing plastic surgery trainees' participation in scientific research is beneficial both for residents and their mentors. Research fellowships can provide an opportunity for academically oriented trainees to further develop their research skills. Protected time and adequate mentoring can help not only to increase residents' research output, but also to recruit the next generation of academic plastic surgeons.

Geographic variation in management of patients with placenta accreta spectrum: An international survey of experts (GPASS).

To describe global geographic variations in the diagnosis and management of placenta accreta spectrum (PAS). An international cross-sectional study was conducted among PAS experts practicing at medical institutions in member states of the United Nations. Survey questions focused on diagnostic evaluation and management strategies for PAS. A total of 134 centers participated. Participating centers represented each of the United Nations' designated regions. Of those, 118 (88%) reported practicing in a medium-volume or high-volume center. First-trimester PAS screen was reported in 35 (26.1%) centers. Respondents consistently implement guideline-supported care practices, including utilization of ultrasound as the primary diagnostic modality (134, 100%) and implementation of multidisciplinary care teams (115, 85.8%). Less than 10% of respondents reported routinely managing PAS without hysterectomy; these centers were predominantly located in Europe and Africa. Antepartum management and availability of mental health support for PAS patients varied widely. Worldwide, there is a strong adherence to PAS care guidelines; however, regional variations do exist. Comparing variations in care to outcomes will provide insight into the clinically significant practice variability.

Pain Assessment and Management in Pediatric Intensive Care Units Around the World, an International, Multicenter Study.

The adequate assessment and management of pain remains a challenging task in the Pediatric Intensive Care Unit (PICU). Our goal is to describe how pain is assessed and managed in PICUs around the world and to examine how human and material resources impact achievement of this goal. An international multicenter cross-sectional observational study was designed with the participation of 34 PICUs located in urban, suburban, and rural areas of 18 countries. We evaluated how PICUs around the world assessed and managed pain according to the Initiative for Pediatric Palliative Care recommendations, and how human and material resources impacted achievement of this goal. Data was collected for this study from 2016 to 2018 using questionnaires completed by medical doctors and nurses. In this paper, we focus on the indicators related to how pain is managed and assessed. The average achievement of the goal of pain relief across all centers was 72.2% (SD: 21.1). We found a statistically significant trend of more effective pain management scores, routine assessment, proper documentation, and involvement of pain management experts by increasing country income. While there are efforts being made worldwide to improve the knowledge in pain assessment and management, there is a lack of resources to do so appropriately in low-middle-income countries. There is a mismatch between the existing guidelines and policies, which are mainly designed in high income countries, and the resources available in lower resourced environments.

Psychological Factors, Leisure Activities, and Satisfaction during the COVID-19 Pandemic: A Cross-Sectional Study in Eleven Spanish-Speaking Countries.

The COVID-19 pandemic has affected the lives of millions of people worldwide. This study aimed to analyze the effects of several psychological factors (self-esteem, self-control, and emotional stability) over lifestyle-related variables (time spent on leisure activities) and the levels of satisfaction (family, friends, work, and leisure satisfaction) experienced during the COVID-19 outbreak. Data for this article were retrieved as part of a cross-sectional international study conducted in eleven Spanish-speaking countries between March and September 2020. The analyses were conducted using the responses of 9500 persons (65.95% women, 34.05% men). Structural equation modeling was used to test the direct and indirect effects of the psychological variables on satisfaction variables mediated by the time engaged in leisure activities. Our model indicated that psychological factors significantly predicted the amount of time spent in leisure activities and satisfaction. Overall, results indicate that self-esteem is a relevant psychological factor to consider in the development of psychological interventions directed at promoting healthy lifestyles. Nevertheless, further research is needed to validate the direction of the associations found in this study.

Primary palliative care integrated model in paediatric ICU: an international cross-sectional study

ObjectivesNumbers are rising of chronically and critically ill, technology-dependent children, who are admitted to paediatric intensive care units (PICUs). An integrated model of care (IMOC), that combines paediatric critical care...

The Determinants of Living with Long-Term Conditions: An International Cross-Sectional Study.

It is essential that healthcare and social professionals understand the daily lives of people with chronic diseases, and the variables that influence them. The aim of this study was to identify the determinants influencing the process of living with long-term conditions. To investigate this, an observational, international, cross-sectional study was carried out. A consecutive sample of 1788 Spanish-speaking population living with chronic obstructive pulmonary disease, chronic heart failure and type 2 diabetes mellitus were included. Descriptive statistics and multiple linear regression models were performed. The linear regression models identified that social support (β = 0.39, p < 0.001) and the satisfaction with life (β = 0.37, p < 0.001) were the main determinants in the process of living with a long-term condition (49% of the variance). Age (β = −0.08, p = 0.01) and disease duration (β = 0.07, p = 0.01) were determinants only in the chronic heart failure subgroup, and country was significant in the chronic obstructive pulmonary disease subgroup (β = −0.15, p = 0.002). Satisfaction with life and social support were key determinants influencing the process of living with long-term conditions. As such, those aspects should be included in the design of interventions focused on the achievement of a positive living in people with long-term conditions.

Cannabinoid use and effects in patients with epidermolysis bullosa: an international cross-sectional survey study

BackgroundEpidermolysis bullosa (EB) patient anecdotes and case reports indicate that cannabinoid-based medicines (CBMs) may alleviate pain and pruritus and improve wound healing. CBM use has not been characterized in the EB patient population.ObjectivesTo evaluate CBM use among EB patients, including CBM types, effects on symptoms (e.g., pain and pruritus), disease process (e.g., blistering, wounds, and inflammation), well-being (e.g., sleep, appetite) and concomitant medications.MethodsEnglish-speaking EB patients or caregivers completed an online international, anonymous, cross-sectional survey regarding CBM use. Respondents reported the types of CBMs, subsequent effects including perceived EB symptom alteration, changes in medication use, and side effects.ResultsSeventy-one EB patients from five continents reported using or having used CBMs to treat their EB. Missing question responses ranged between 0 (0%) and 33 (46%). Most used more than one CBM preparation (mean: 2.4 ± 1.5) and route of administration (mean: 2.1 ± 1.1). Topical and ingested were the most common routes. Pain and pruritus were reported retrospectively to decrease by 3 points (scale: 0–10; p < 0.001 for both) after CBM use. Most reported that CBM use improved their overall EB symptoms (95%), pain (94%), pruritus (91%) and wound healing (81%). Most participants (79%) reported decreased use of pain medications. The most common side-effect was dry mouth (44%).ConclusionsCBMs improve the perception of pain, pruritus, wound healing, and well-being in EB patients and reduced concomitant medication use. Nevertheless, a direct relation between the use of CBMs and reduction of the above-mentioned symptoms cannot be proven by these data. Therefore, future controlled studies using pharmaceutically standardised CBM preparations in EB are warranted to delineate the risks and benefits of CBMs.

Assessing the knowledge of endometriosis diagnostic tools in a large, international lay population: an online survey.

To assess the general population's knowledge regarding the utility and availability of tools to diagnosis endometriosis, with a focus on ultrasound. An international cross-sectional online survey study was performed between August and October 2019. 5301 respondents, representing 73 countries. In all, 23 questions survey focused on knowledge of endometriosis diagnosis distributed globally via patient- and community-endometriosis groups using social media. Descriptive data of the knowledge of diagnostic tools for diagnosing endometriosis, including details about diagnosis using ultrasound. In all, 84.0% of respondents had been previously diagnosed with endometriosis, 71.5% of whom had been diagnosed at the time of surgery. Ultrasound and MRI were the methods of diagnosis in 6.5% and 1.8%, respectively. A total of 91.8%, 28.8% and 16.6% of respondents believed surgery, ultrasound and MRI could diagnose endometriosis, respectively (more than one answer allowed). In those diagnosed by surgery, 21.7% knew about ultrasound as a diagnosis method, whereas in those diagnosed non-surgically, 51.5% knew (P < 0.001). In all, 14.7%, 31.1% and 18.2% stated superficial, ovarian and deep endometriosis could be diagnosed with ultrasound (32.9% stated they did not know which phenotypes of endometriosis could be diagnosed). Lastly, 58.4% of respondents do not believe they could access an advanced ultrasound in their region. There is a limited appreciation for the role of non-surgical diagnostic tests for endometriosis among lay respondents to this survey. International survey shows limited awareness of lay respondents about non-surgical endometriosis diagnostic tools.

Medical student knowledge and critical appraisal of machine learning: a multicentre international cross-sectional study.

To utilise effectively tools that employ machine learning (ML) in clinical practice medical students and doctors will require a degree of understanding of ML models. To evaluate current levels of understanding, a formative examination and survey was conducted across three centres in Australia, New Zealand and the United States. Of the 245 individuals who participated in the study (response rate=45.4%), the majority had difficulty with identifying weaknesses in model performance analysis. Further studies examining educational interventions addressing such ML topics are warranted.

Women doctors in female urology: current status and implications for future workforce.

To objectively determine the percentage of female trainees and consultants who are interested in their career being focussed on female urology (FU) in order to facilitate the improved planning for the future of this sub-specialty. This was an international cross-sectional study spanning 1year, from December 2018 to December 2019. An anonymous, voluntary survey was generated using the online survey generator Survey monkey® . The survey was sent to urology consultants and trainees who were female from Australia, New Zealand, and Canada. The total response rate to the survey was 61%. Up to 50% of female consultants and trainees selected a career in FU due to their gender, but up to 75% of respondents were also interested in FU of their own accord. Common concerns held by a majority of respondents included both the medical community's and the public's lack of awareness of FU as a component of urological expertise. Despite these concerns, most of the trainees were not concerned regarding their future work opportunities in FU, and many had intentions to pursue a fellowship in FU. Female urology is an increasingly popular sub-specialisation of urology, given the steady increase in the intake of female trainees. Similar trends were identified internationally. Urology training in this area will need to continue to increase the community's and the primary health care referrer's awareness in order to ensure the continued success and growth of the sub-specialty.

International retrospective natural history study of LMNA-related congenital muscular dystrophy Short Title: LMNA-CMD natural history

Muscular dystrophies due to heterozygous pathogenic variants in LMNA gene cover a broad spectrum of clinical presentations and severity with an age of onset ranging from the neonatal period to adulthood. The natural history of these conditions is not well defined, particularly in patients with congenital or early onset who arguably present with the highest disease burden. Thus the definition of natural history endpoints along with clinically revelant outcome measures is essential to establishing both clinical care planning and clinical trial readiness for this patient group. We designed a large international cross-sectional retrospective natural history study of patients with genetically proven muscle laminopathy who presented with symptoms before two years of age intending to identify and characterize an optimal clinical trial cohort with pertinent motor, cardiac and respiratory endpoints. Quantitative statistics were used to evaluate associations between LMNA variants and distinct clinical events. The study included 151 patients (Median age at symptom onset 0.9 years, range: 0.0-2.0). Age of onset and age of death were significantly lower in patients who never aquired independent ambulation compared to patients who achieved independent ambulation. Most of the patients acquired independent ambulation (n = 101, 66.9%), and subsequently lost this ability (n = 86; 85%). The age of ambulation acquisition (Median: 1.2 years, range: 0.8-4.0) and age of ambulation loss (Median: 7 years, range 1.2-38.0) were significantly associated with the age of the first respiratory interventions and the first cardiac symptoms. Respiratory and gastrointestinal interventions occurred during first decade while cardiac interventions occurred later. Genotype-phenotype analysis showed that the most common mutation, p.Arg249Trp (20%), was significantly associated with a more severe disease course. This retrospective natural history study of early onset LMNA-related muscular dystrophy confirms the progressive nature of the disorder, initially involving motor symptoms prior to onset of other symptoms (respiratory, orthopedic, cardiac, and gastrointestinal). The study also identifies subgroups of patients with a range of long-term outcomes. Ambulatory status was an important mean of stratification along with the presence or absence of the p.Arg249Trp mutation. These categorizations will be important for future clinical trial cohorts. Finally, this study furthers our understanding of the progression of early onset LMNA-related muscular dystrophy and provides important insights into the anticipatory care needs of LMNA-related respiratory and cardiac manifestations.