BACKGROUND: The French healthcare system has been affected by the COVID-19 pandemic in 2020, including cancer care.
METHODS: In order to evaluate the impact of this pandemic on cancer incidence, the Isere Departmental Cancer Registry compared the actual 2020 incidence of melanoma, breast, colorectal, prostate and lung cancers with the expected 2020 incidence based on data collected by the Registry between 2015 and 2019, taking into account periods of lockdown and reopening. When available, cancer stages and/or prognostic scores were recorded.
RESULTS: During the period of initial confinement, a 54%, 50% and 36,8% drop in incidence was observed for breast, prostate and colorectal cancer respectively. Although their annual incidence remained stable, a worsening trend emerged as a decline in the number of low stages/scores at diagnosis in favour of higher stages/scores towards the end of 2020. In contrast, a significant 17,8% drop was observed in annual incidence of melanoma, particularly for Breslow scores < 1 (-27,4%). However, this trend was noticeable before the lockdown, as well as the 14% reduction in the incidence of lung cancer in women, but not in men.
CONCLUSIONS: The incidence of certain cancers was caught up over the year but the COVID-19 pandemic seems to be associated with a change in their severity at diagnosis throughout 2020. The downward trends in female lung cancer and melanoma incidence point to complex underlying phenomena. Further analysis is still needed to assess the global impact of the COVID-19 pandemic on cancer incidence.

BACKGROUND: In Slovakia, a mandatory national universal pediatric total cholesterol (TC) screening program is in place to identify cases of familial hypercholesterolemia (FH). However, the program\'s effectiveness has not been systematically assessed.
OBJECTIVE: This study aimed to estimate the prevalence of FH among parents of children that had elevated TC levels identified during screening.
METHODS: This prospective, non-interventional, observational study enrolled parents of 11-year-old children who underwent TC screening in 23 selected pediatric outpatient clinics between 2017 and 2018. FH was diagnosed using the Dutch Lipid Clinic Network (DLCN) criteria and targeted next-generation sequencing. The primary objective was to estimate the proportion of children with a TC level of >188 mg/dL (>4.85 mmol/L) who had a parent with a confirmed diagnosis of FH.
RESULTS: A total of 112 parents of 56 children with an elevated TC level were enrolled. Five children (8.9%) had a parent in whom FH was genetically confirmed. Without genetic analysis, all five parents would only be diagnosed with \"possible FH\" by DLCN criteria. Of parents, 83.9% (n = 94/112) had an LDL-C level of >116 mg/dL (>3 mmol/L), but only 5.3% (n = 5/94) received lipid-lowering therapy. Among the five parents with genetically confirmed FH, all had an LDL-C level >116 mg/dL (>3 mmol/L), with a mean (±SD) of 191 (±24) mg/dL (4.94 [±0.61] mmol/L). Only two of these parents received lipid-lowering therapy.
CONCLUSIONS: The present study demonstrates the significance of mandatory universal pediatric TC screening in identifying families with FH and other at-risk families in need of lipid-lowering therapy.

UNASSIGNED: Thyroid cancer is recognized as the predominant form of endocrine cancer. The likelihood of cancer recurrence and the development of distant metastases varies depending on the cancer\'s pathology and stage. Iran currently lacks country-specific data on thyroid cancer, which can potentially result in clinicians deviating from the optimal treatment. The primary objectives of establishing such a registry are to determine the incidence, identify risk factors, and evaluate treatment outcomes for thyroid cancer within the Iranian population. Ultimately, the overarching goal of this protocol study is to reduce mortality and morbidity rates among thyroid cancer patients by implementing appropriate interventions based on the findings derived from this registration system.
UNASSIGNED: The study will enroll all individuals aged 18 years and older who have received a diagnosis of primary thyroid carcinoma based on pathology criteria. Data will be collected from various thyroid clinic centers. The participating centers include the Endocrinology Clinic at Shariati Hospital, the Thyroid Clinic in the Nuclear Medicine Center at Shariati Hospital, as well as pathology and nuclear medicine centers in Kerman and Bushehr. Patient records comprise information on outpatient visits to the clinic.
UNASSIGNED: The registry aims to enhance treatment approaches and follow-up protocols while serving as a foundation for conducting clinical, epidemiological, and basic science studies based on robust evidence-based data.

UNASSIGNED: Osteoporotic fractures can result in significant health complications and an increased risk of death. Registry studies could provide better treatment options and improve patient outcomes by providing useful information about the disease. The present study describes the protocol for an osteoporosis registry in Iran.
UNASSIGNED: This registry is a prospective multicenter cohort study recruiting patients with osteoporosis from Iran. The inclusion criteria of the study are individuals diagnosed with primary or secondary osteoporosis according to the diagnostic criteria of the study; patients will be identified and recruited from outpatient clinics in this registry. All patients diagnosed with primary or secondary osteoporosis are the target population of the study. Our expected sample size is 1000 participants and the study will continue for at least 2 years. The measurements of the Iranian Osteoporosis Registry include four parts: (i) variables measured by the specific questionnaires package, (ii) bone mineral density (BMD, (iii) clinical examination, and (iv) lab data. The final questionnaire package includes \"demographics information\", \"socioeconomic status\", \"lifestyle\", \"reproductive health\", \"medical history and medication\", \"Osteoporosis diagnosis gap\", \"Osteoporosis adherence and treatment gap\", \"fracture history and fall risk assessment\", \"FRAX ® tool \", \"hospitalization and death outcomes\", \"low back pain\", \"hospitalization history\", \"attitude toward osteoporosis\", \"osteoporosis awareness\", \"osteoporosis related-performance\", \"quality of life (Iranian version of SF12 questionnaire )\", and \"food frequency questionnaire (FFQ)\". Clinical examination of this registry includes anthropometric measurements (including height, weight, body mass index (BMI), waist circumference, hip circumference, and right wrist circumference), and blood pressure. The baseline questionnaires will be filled out right after patients are diagnosed with osteoporosis and then osteoporotic patients will be followed up regularly on a yearly basis. In the follow-up visit, variables that may have changed over time are updated. The main outcomes include registration of fall, fracture, hospitalization, medication adherence, and death. An online web-based user-friendly software is also developed for data collection. Data analysis will be conducted with the collaboration of data-mining experts and epidemiologists at the end of each follow-up.
UNASSIGNED: The Iran Osteoporosis Registry will be a valuable source of information regarding osteoporosis outcomes (i.e. fractures, hospitalizations, adherence, and death at the national level), and its results will be very beneficial and practical for policy makers in the field of musculoskeletal diseases.

BACKGROUND: The pathogenesis and clinical profiles of patients with pulmonary hypertension (PH) associated with interstitial lung disease (ILD-PH) are poorly understood. Whether and to what extent pulmonary arterial hypertension (PAH)-specific therapy improves hemodynamic and outcome in ILD-PH are also unknown.
OBJECTIVE: This study aims to clarify the characteristics, clinical course and response to PAH-specific therapy of ILD and/or PH by enrolling three unique subsets: PAH, ILD-PH, and ILD.
METHODS: The proposed study is a retrospective and prospective, multi-centre, observational cohort study of patients treated at any of three university hospitals in the Hokkaido region of Japan who have any one of the following: PAH; ILD-PH with or without PAH features; or ILD without PH. We aim to enrol 250 patients in total. For the retrospective observation period, data obtained after 1 January 2010, will be analysed, and the prospective observation period will be 1 year. We will compare the clinical data of patients with ILD-PH with those of patients with PAH and those of patients with ILD without PH in the real-world clinical setting. In addition, within the cohort of patients with ILD-PH, we will explore the subset with \"ILD-PH with PAH features\" and compare the response to PAH-specific therapy with that of PAH. The primary outcome will be the change in pulmonary vascular resistance from first treatment to follow-up in patients with PAH and ILD-PH with PAH features (excluding ILD-PH without PAH feature and ILD-no-PH for the primary outcome). The exploratory outcomes will include analyses of PH-associated biomarkers, right ventricular function and patient-reported outcomes.
RESULTS: This is a protocol article and the results will be presented after data collection is completed.
CONCLUSIONS: The POPLAR study will provide data that help better understand the pathophysiology of ILD-PH and improve the quality of life and outcome of patients with PH and/or ILD.
BACKGROUND: Japan Registry of Clinical Trials: jRCT1010230018.

OBJECTIVE: Ki-67 is recommended by international/national guidelines for risk stratification in early breast cancer (EBC), particularly for defining \"intermediate risk,\" despite inter-laboratory/inter-observer variability and cutoff uncertainty. We investigated Ki-67 (> 10%- < 40%, determined locally) as a prognostic marker for intermediate/high risk in EBC, pN0-1 patients.
METHODS: This prospective, non-interventional, real-world study included females ≥ 18 years, with pN0/pN1mi/pN1, HR+ , HER2-negative EBC, and locally determined Ki-67 ranging 10%-40%. The primary outcome was changes in treatment recommendations after disclosing the Oncotype DX Breast Recurrence Score®(RS) assay result.
RESULTS: The analysis included 567 patients (median age, 57 [range, 29-83] years; 70%/1%/29%/ with pN0/pN1mi/pN1 disease; 81% and 19% with RS results 0-25 and 26-100, respectively). The correlations between local and central Ki-67, local Ki-67, and the RS, and central Ki-67 and the RS results were weak (r = 0.35, r = 0.3, and r = 0.46, respectively), and discrepancies were noted in both directions (e.g., local Ki-67 was lower or higher than central Ki-67). After disclosing the RS, treatment recommendations changed for 190 patients (34%). Changes were observed in pN0 and pN1mi/pN1 patients and in patients with centrally determined Ki-67 ≤ 10% and > 10%. Treatment changes were aligned with RS results (adding chemotherapy for patients with higher RS results, omitting it for lower RS results), and their net result was 8% reduction in adjuvant chemotherapy use (from 32% pre-RS results to 24% post-RS results).
CONCLUSIONS: The Oncotype DX® assay is a tool for individualizing treatments that adds to classic treatment decision factors. The RS result and Ki-67 are not interchangeable, and Ki-67, as well as nodal status, should not be used as gatekeepers for testing eligibility, to avoid under and overtreatment.

BACKGROUND: Chronic thromboembolic pulmonary hypertension (CTEPH) is a progressive pulmonary vascular disorder with substantial morbidity and mortality, also a disease underdiagnosed and undertreated. It is potentially curable by pulmonary endarterectomy (PEA) in patients with surgically accessible thrombi. Balloon pulmonary angioplasty (BPA) and targeted medical therapy are options for patients with distal lesions or persistent/recurrent pulmonary hypertension after PEA. There is an urgent need to increase the awareness of CTEPH. Qualified CTEPH centers are still quite limited. Baseline characteristics, management pattern and clinical outcome of CTEPH in China needs to be reported.
METHODS: The CHinese reAl-world study to iNvestigate the manaGEment pattern and outcomes of chronic thromboembolic pulmonary hypertension (CHANGE) study is designed to provide the multimodality treatment pattern and clinical outcomes of CTEPH in China. Consecutive patients who are ≥ 14 year-old and diagnosed with CTEPH are enrolled. The diagnosis of CTEPH is confirmed in right heart catheterization and imaging examinations. The multimodality therapeutic strategy, which consists of PEA, BPA and targeted medical therapy, is made by a multidisciplinary team. The blood sample and tissue from PEA are stored in the central biobank for further research. The patients receive regular follow-up every 3 or 6 months for at least 3 years. The primary outcomes include all-cause mortality and changes in functional and hemodynamic parameters from baseline. The secondary outcomes include the proportion of patients experiencing lung transplantation, the proportion of patients experiencing heart and lung transplantation, and changes in health-related quality of life. Up to 31 December 2023, the study has enrolled 1500 eligible patients from 18 expert centers.
CONCLUSIONS: As a real-world study, the CHANGE study is expected to increase our understanding of CTEPH, and to fill the gap between guidelines and the clinical practice in the diagnosis, assessment and treatment of patients with CTEPH. REGISTRATION NUMBER IN CLINICALTRIALS.GOV: NCT05311072.

BACKGROUND: Aneurysmal subarachnoid haemorrhage (aSAH) is a life-threatening disease with high mortality and morbidity. Patients with aSAH in Sweden are cared for at one of six neuro intensive care units (NICU) or at a general intensive care unit (ICU).This study aimed to describe the incidence, length of stay, time in ventilator and mortality for these patients.
METHODS: This is a retrospective, descriptive study of patients with aSAH, registered in the Swedish Intensive care Registry between 2017 and 2019. The cohort was divided in sub-cohorts (NICU and general ICU) and regions. Mortality was analysed with logistic regression.
RESULTS: A total of 1520 patients with aSAH from five regions were included in the study. Mean age of the patients were 60.6 years and 58% were female. Mortality within 180 days of admission was 30% (n = 456) of which 17% (n = 258) died during intensive care. A majority of the patients were treated at one hospital and in one ICU (70%, n = 1062). More than half of the patients (59%, n = 897) had their first intensive care admission at a hospital with a NICU. Patients in the North region had the lowest median GCS (10) and the highest SAPS3 score (60) when admitted to NICU. Treatment with invasive mechanical ventilation differed significantly between regions; 91% (n = 80) in the region with highest proportion versus 56% (n = 94) in the region with the lowest proportion, as did mortality; 16% (n = 44) versus 8% (n = 23). No differences between regions were found regarding age, sex and length of stay.
CONCLUSIONS: Patients with aSAH treated in a NICU or in an ICU in Sweden differs in characteristics. The study further showed some differences between regions which might be reduced if there were national consensus and treatment guidelines implemented.

BACKGROUND: Providing secondary prevention through structured and comprehensive cardiac rehabilitation programmes to patients after a myocardial infarction (MI) reduces mortality and morbidity and improves health-related quality of life. Cardiac rehabilitation has the highest recommendation in current guidelines. While treatment target attainment rates at Swedish cardiac rehabilitation centres is among the highest in Europe, there are considerable differences in service delivery and variations in patient-level outcomes between centres. In this trial, we aim to study whether centre-level guideline adherence and patient-level outcomes across Swedish cardiac rehabilitation centres can be improved through a) regular audit and feedback of cardiac rehabilitation structure and processes through a national quality registry and b) supporting cardiac rehabilitation centres in implementing guidelines on secondary prevention. Furthermore, we aim to evaluate the implementation process and costs.
METHODS: The study is an open-label cluster-randomized effectiveness-implementation hybrid trial including all 78 cardiac rehabilitation centres (attending to approximately 10 000 MI patients/year) that report to the SWEDEHEART registry. The centres will be randomized 1:1:1 to three clusters: 1) reporting cardiac rehabilitation structure and process variables to SWEDEHEART every six months (audit intervention) and being offered implementation support to implement guidelines on secondary prevention (implementation support intervention); 2) audit intervention only; or 3) no intervention offered. Baseline cardiac rehabilitation structure and process variables will be collected. The primary outcome is an adherence score measuring centre-level adherence to secondary prevention guidelines. Secondary outcomes include patient-level secondary prevention risk factor goal attainment at one-year after MI and major adverse coronary outcomes for up to five-years post-MI. Implementation outcomes include barriers and facilitators to guideline adherence evaluated using semi-structured focus-group interviews and relevant questionnaires, as well as costs and cost-effectiveness assessed by a comparative health economic evaluation.
CONCLUSIONS: Optimizing cardiac rehabilitation centres\' delivery of services to meet standards set in guidelines may lead to improvement in cardiovascular risk factors, including lifestyle factors, and ultimately a decrease in morbidity and mortality after MI.
BACKGROUND: ClinicalTrials.gov. Identifier: NCT05889416 . Registered 2023-03-23.

BACKGROUND: In the context of severe unexplained haemorrhage (SH), it is usual to seek haematological evaluation and investigate for an inherited rare bleeding disorder (IRBD). In such circumstances, appropriate screen can discriminate between IRBD and suspected child abuse. Yet, little information is available about the frequency of SH in the population of patients with IRBD.
OBJECTIVE: To collect epidemiologic data about SH and IRBD.
METHODS: The database of the FranceCoag network has collected information about IRBD since January 2004. Based on data gathered up to 16 March 2022, a retrospective search was conducted for of SH events having occurred before or at the time of IRBD diagnosis. Demographics and diagnosis circumstances were retrieved, as well as information about SH, defined as any life-threatening bleeding or intracranial haemorrhage.
RESULTS: Among the 13,433 patients of the database, 109 (0.8%) fulfilled inclusion criteria including a known date of IRBD diagnosis, haemophilia A or B (HA/HB) being the most frequent (82.5%). IRBD was discovered as a consequence of an SH event in 82.6% of the cases while CNS was involved in 55%. Severe and moderate HA/HB and other severe IRBD presented significantly more intracranial haemorrhage (p < .02) and a lower age at diagnosis (p = .03).
CONCLUSIONS: These data support that any unusual SH should raise a suspicion of IRBD. Particularly before 1-year of age, it is suggested to first confirm moderate or severe haemophilia and severe IRBD by standard coagulation tests (APTT, PT and fibrinogen), combined with a clotting FXIII assay as first-line investigation. Subsequent assays of coagulation factors should be performed in the case of abnormal values, in second-line investigation.