Family Formation and Employment Changes Among Descendants of Immigrants in France: A Multiprocess Analysis.

This paper investigates the association between family formation and the labour market trajectories of immigrants' descendants over the life course. Using rich data from the Trajectories and Origins survey from France, we apply multilevel event history models to analyse the transitions in and out of employment for both men and women by parity. We account for unobserved co-determinants of childbearing and employment by applying a simultaneous-equations modelling. Our analysis shows that women's professional careers are negatively associated with childbirth. There are differences across descendant groups. The female descendants of Turkish immigrants are more likely to exit employment and less likely to re-enter employment following childbirth than women from other groups. The negative impact of childbearing on employment is slightly overestimated among women due to unobserved selection effects. Among men, the descendants of European immigrants are less likely to exit employment after having a child than other descendant groups. The study demonstrates the negative effect of childbearing on women's employment, which is pronounced for some minority groups suggesting the need for further policies to help women reconcile work with family life.

Cost-effectiveness and public health impact of using high dose quadrivalent influenza vaccine in the French older adults population.

INTRODUCTION: Seasonal influenza outbreaks in France cause a surge in patients, exacerbating the overburdened healthcare system each winter. Older adults are particularly vulnerable to serious events related to influenza. Quadrivalent influenza high dose (QIV HD) vaccines have been developed to offer better clinical protection in older adults, who often exhibit suboptimal immune response to quadrivalent influenza standard dose vaccines (QIV SD). This study aims to evaluate the public health impact and cost-effectiveness of administering HD versus SD vaccines to individuals aged 65+ in France. METHODOLOGY: Using a static model and decision-tree approach, the study analyzed health outcomes such as influenza cases, GP (general practitioner) visits, hospitalizations, and mortality; relative vaccine efficacy (rVE) estimates were derived from a pivotal randomized-controlled trial and a meta-analysis comparing HD to SD vaccines. Two approaches were implemented to model hospitalizations (conditional on influenza or not), and analyses on bed occupancy were performed. RESULTS: Results showed that using QIV HD instead of QIV SD during an average influenza season in France led to the prevention of 57,209 additional cases of influenza, 13,704 GP visits, and 764 influenza-related deaths. Moreover, switching to QIV HD resulted in additional 1,728 to 15,970 hospitalizations avoided and 15,124 to 138,367 reduced days of hospitalization depending on the hospitalization approach used. The cost-utility analysis showed a cost per quality-adjusted life year (QALY) gained ranging from 24,020 €/QALY to 5,036 €/QALY. CONCLUSIONS: Switching to QIV HD in older adults showed to be cost-effective, with even greater public health benefits at higher coverage rate, regardless the season severity.

Is health-related quality of life sufficiently addressed in trials for breast cancer treatments? An assessment based on reimbursement opinions from the French health technology assessment body, 2009-2023.

BACKGROUND: Breast cancer treatments can impact the patients' health-related quality of life (HR-QoL). This criterion is relevant for drug reimbursement decisions. We wanted to assess the usage of HR-QoL in health technology assessments (HTA). METHODS: All HAS (Haute Autorité de Santé, the French HTA body) opinions published between January 1, 2009 and March 31, 2023 for the reimbursement of breast cancer drugs were analysed. RESULTS: 51 distinct appraisals were found during the period, corresponding to 45 product-specific indications, of which 36 (80 %) including clinical studies in which HR-QoL was an endpoint. HAS explicitly rejected HR-QoL data in 25 out of 36 (69 %) indications with such data. Rejections are justified by methodological weaknesses, including lack of adjustment for type I error inflation (n=21 indications), open-label treatment (n=7), lack of a pre-specified clinically relevant HR-QoL threshold (n=6) or missing data (n=6). Regardless of rejection status, HR-QoL results were not mentioned as a determinant of value assessment in 3/36 (8 %) instances (2/25 for rejected data). CONCLUSIONS: HR-QoL data are inconsistently present in HTA assessments of new breast cancer drugs. Their methodological quality often hinders their use in determining the drug's value. POLICY SUMMARY: A rigorous and acceptable comparative experimental framework is expected for HR-QoL assessments. More detail on the precise impact of the absence or presence of HR-QoL data in the determination of the drug's added value could help understanding how this dimension is influential in the assessments.

End-of-life medical decisions in French overseas departments: results of a retrospective survey.

BACKGROUND: French laws governing end-of-life medical practices forbid euthanasia and affirm patients' right to deep and continuous sedation until death. Cultural traditions and disparities in health care provision, as in overseas France, could limit the enforcement of such laws and modify end-of-life medical practices. AIM: This research aims to describe end-of-life medical decisions in overseas France and to compare with those described in mainland France. METHODS: A retrospective study of a random sample of adult patients who died between March 2020 and February 2021 was conducted in four overseas French departments. Physicians who certified the deaths were asked to describe end-of-life care and medical decisions in a questionnaire. RESULTS: A total of 1815 deaths were analysed over 8730 questionnaires sent. Withholding treatments was the most frequent decision (41%), treatment for pain or symptoms was intensified for a third of patients, Deep and continuous sedation until death was implemented in 13.3% cases. The use of drugs to deliberately end life was mentioned in 1.3% deaths. At least one decision was made in 61.6% deaths. More decisions that may hasten death were made before predictable deaths. Intensification of pain and symptoms treatment was more frequent in 2022 than in 2010. Deep and continuous sedation was introduced by law in 2016 without prejudice to other decisions. CONCLUSION: Physicians in overseas France have implemented recent changes in end-of-life laws, including deep and continuous sedation. Comparisons with 2010 mainland France survey show a better implementation of palliative medicine in 2022, with higher proportions of treatment withholding.

Health economic impact of patients with phenylketonuria (PKU) in France - A nationwide study of health insurance claims data.

Background: Phenylketonuria (PKU) is an inherited metabolic disease. If left untreated, it can lead to severe irreversible intellectual disability and can cause seizures, behavior disturbance, and white matter disease. This study aimed at evaluating the health economic impact of patients with PKU in France. Methods: This retrospective observational study used health insurance claims data from the French SNDS (Système National des Données de Santé) database, which contains data from over 66 million French inhabitants. Patients with PKU were identified by ICD-10 diagnosis codes E70.0 (PKU) and E70.1 (Other hyperphenylalaninemia) documented as a chronic condition (affection de longue durée - ALD) or in the inpatient setting in the SNDS database between 2006 and 2018. Patients with PKU were matched to controls without PKU by age, sex, and region. Patients with early- and late-diagnosed PKU were defined as patients born after and before the implementation of nationwide newborn screening in France in 1972, respectively. Outcomes were analyzed for the year 2018. Results: Overall, 3549 patients with PKU were identified in the database on January 1st, 2018. Of those, 3158 patients versus 15,703 controls with at least one healthcare consumption in 2018 were available for outcome analyses. Patients with PKU had 7.7 times higher healthcare costs than non-PKU controls in 2018 (€11,144 versus 1456 mean costs; p < 0.0001). Pharmaceutical costs including dietary amino acid supplements were the cost driver and contributed 80.0% of the overall mean difference (MD) between patients with PKU and matched non-PKU controls. More than half (52.4%) of the mean pharmaceutical costs per patient with PKU was attributable to medical foods including dietary amino acid supplements.Of the 3158 patients with PKU, 2548 (80.7%) were classified as early-diagnosed and 610 (19.7%) as late-diagnosed. Increased healthcare costs, in comparison to non-PKU controls, were more evident in early-diagnosed patients (€11,263 versus €855 mean costs; 13.2-fold increase; p < 0.0001). For patients with late-diagnosed PKU, healthcare costs were 2.7-fold higher compared to matched non-PKU controls (€10,644 versus €3951 mean costs; p < 0.0001). Outpatient pharmaceutical costs accounted for 89.1% of the MD between early-diagnosed patients and controls. Among late-diagnosed patients, 55.5% of the MD were attributable to costs for inpatient care, followed by costs for outpatient care (23.9%) and outpatient pharmaceutical costs (20.6%). Conclusion: The results indicate that PKU is associated with substantially increased health care costs compared to non-PKU controls in France. The health economic impact was most evident in patients with early-diagnosed PKU due to increased outpatient pharmaceutical costs, especially for medical foods including dietary amino acid supplements. For late-diagnosed and by definition older patients with PKU, the excess costs compared with matched controls were mostly driven by costs for inpatient care.

Health Care Resource Use and Total Mortality After Hospital Admission for Severe COVID-19 Infections During the Initial Pandemic Wave in France: Descriptive Study.

BACKGROUND: Little is known about post-hospital health care resource use (HRU) of patients admitted for severe COVID-19, specifically for the care of patients with postacute COVID-19 syndrome (PACS). OBJECTIVE: A list of HRU domains and items potentially related to PACS was defined, and potential PACS-related HRU (PPRH) was compared between the pre- and post-COVID-19 periods, to identify new outpatient care likely related to PACS. METHODS: A retrospective cohort study was conducted with the French National Health System claims data (SNDS). All patients hospitalized for COVID-19 between February 1, 2020, and June 30, 2020 were described and investigated for 6 months, using discharge date as index date. Patients who died during index stay or within 30 days after discharge were excluded. PPRH was assessed over the 5 months from day 31 after index date to end of follow-up, that is, for the post-COVID-19 period. For each patient, a pre-COVID-19 period was defined that covered the same calendar time in 2019, and pre-COVID-19 PPRH was assessed. Post- or pre- ratios (PP ratios) of the percentage of users were computed with their 95% CIs, and PP ratios>1.2 were considered as "major HRU change." RESULTS: The final study population included 68,822 patients (median age 64.8 years, 47% women, median follow-up duration 179.3 days). Altogether, 23% of the patients admitted due to severe COVID-19 died during the hospital stay or within the 6 months following discharge. A total of 8 HRU domains were selected to study PPRH: medical visits, technical procedures, dispensed medications, biological analyses, oxygen therapy, rehabilitation, rehospitalizations, and nurse visits. PPRs showed novel outpatient care in all domains and in most items, without specificity, with the highest ratios observed for the care of thoracic conditions. CONCLUSIONS: Patients hospitalized for severe COVID-19 during the initial pandemic wave had high morbi-mortality. The analysis of HRU domains and items most likely to be related to PACS showed that new care was commonly initiated after discharge but with no specificity, potentially suggesting that any impact of PACS was part of the overall high HRU of this population after hospital discharge. These purely descriptive results need to be completed with methods for controlling for confusion bias through subgroup analyses. TRIAL REGISTRATION: ClinicalTrials.gov NCT05073328; https://clinicaltrials.gov/ct2/show/NCT05073328.

Disease burden of patients with moderate to severe ulcerative colitis: A French multicenter real-life study (THEFAR).

BACKGROUND: Specific studies on the impact of ulcerative colitis (UC) and bowel urgency (BU) on disability and quality of life (QoL) of patients on advanced therapies are missing. METHODS: Clinical and therapeutic management data were collected by Gastroenterologists from adult patients with UC treated with advanced therapies. Patients reported outcomes on QoL were collected using patient-reported questionnaires. RESULTS: Forty-one sites enrolled 293 patients. Median age was 42.0 years, median disease duration was 6.0 years. 38.9 % had active disease (partial Mayo score>1). Median treatment duration was 16.9 months. 166 (57.0 %) patients had BU [median UNRS=2] and 78.3 % had fecal incontinence [median Wexner score=8.0]. Moderate to severe disability (IBD-Disk score≥40) was reported in 37.8 % patients. BU patients had a higher Wexner score [10.0 vs 5.2, p < 0.0001] and moderate to severe disability rate (53.7% vs 16.9 %, p < 0.0001), lower QoL and work productivity than those in BU remission: mean EQ-5D-5 L utility [0.846 vs 0.943, p < 0.0001], VAS for self-rated health [66.2 vs 82.1, p < 0.0001], and overall work impairment [35.7% vs 11.3 %, p < 0.0001]. CONCLUSION: The burden of moderate to severe UC, especially in patients with BU, is high. These findings highlight that BU control remains an unmet medical need in UC patients and underscore the need for new innovative treatments.

Should the French approval of baclofen for alcohol dependence be extended to abstinence maintenance?

Ever since 2018 France has been the only country to approve the gamma aminobutyric acid type B (GABA-B) receptor agonist baclofen for alcohol dependence. This authorization follows a ten-year period of intensive off-label use during which baclofen was used in doses of up to 300 and even 400mg per day to support the gradual reduction of alcohol consumption in patients suffering from alcohol dependence. However, in international clinical trials, baclofen has mainly been studied to support the maintenance of abstinence. The French use of baclofen was therefore somewhat atypical as it paved the way for drug-supported approaches to reducing alcohol consumption, even before nalmefene was marketed. In line with this specific use of baclofen, approval was granted only for alcohol reduction support. However, a recent Cochrane systematic review and meta-analysis by Agabio et al. found significant efficacy only for abstinence maintenance, while no significant effect was found on alcohol reduction outcomes and no dose-response relationship was identified in the analyses. The safety of baclofen was judged to be good. Based on these substantial new results, the Société française d'alcoologie (SFA) now considers that baclofen should also be approved for the maintenance of abstinence. The extension of approval should not lead to the removal of the initial indication or the possibility of using high doses, as some patients have found this therapeutic regimen particularly useful for them. France, which has been a open skies national laboratory on the use of baclofen in alcohol dependence for over ten years, should let this original therapeutic option available to patients. However, it should update the regulatory framework defining the main conditions of access to treatment for patients based on the latest and highest scientific evidence.

Variation in the prion protein gene (PRNP) open reading frame sequence in French cervids.

The recent emergence of chronic wasting disease (CWD) in Europe has become a new public health risk for monitoring of wild and farmed cervids. This disease, due to prions, has proliferated in North America in a contagious manner. In several mammalian species, polymorphisms in the prion protein gene (PRNP) play a crucial role in the susceptibility to prions and their spread. To obtain a reliable picture of the distribution of PRNP polymorphisms in the two most common cervid species in France, we sequenced the open reading frame (ORF) of this gene in 2114 animals, 1116 roe deer (Capreolus capreolus) and 998 red deer (Cervus elaphus). Selection criteria such as historical origin, spatial distribution and sex ratio have been integrated to establish this sample collection. Except for one heterozygous animal with a non-synonymous mutation at codon 37 (G37A), all the 1116 French roe deer were monomorphic. Red deer showed greater variation with two non-synonymous substitutions (T98A; Q226E), three synonymous substitutions (codons 21, 78 and 136) and a new 24pb deletion (Δ69-77). We found significant regional variations between French regions in the frequency of the identified substitutions. After cloning of the PRNP ORF from animals presenting multiple non-synonymous polymorphisms, we identified six haplotypes and obtained a total of twelve genotypes. As in other European countries, we highlighted the apparent homogeneity of PRNP in the French roe deer and the existence of a greater diversity in the red deer. These results were in line with European phylogeographic studies on these two species.

Post-pandemic occupational medicine and telemedicine in Portugal and France: what does the future look like?

Based on personal experience over several years, we carried out a comparative analysis of two different European health systems, in Portugal and France, from a perspective comparing occupational medicine and use of telemedicine in a postpandemic context. This analysis addressed four aspects: Health System; Occupational Medicine; Telemedicine/Telework; and Future and Suggestions. The study employed searches and review of recent articles, guidelines, and recommendations from the authorities responsible for regulation (Medical Doctors Order, Labor Legislation, and Medical Collegiate Recommendations) and analysis of some statistical indicators from recent studies. Three tables on Occupational Health and Medicine present some relevant data and facilitate comparisons. Despite the difficulties of comparison, given the basic differences between these two systems (Beveridge vs. Bismark), it can be concluded that there is a greater acceptance of judicious use of teleconsultation in France (from 15 to 35%). This includes its use by occupational nurses, in the context of the "Visite de Information et Prevention", with good acceptance among employers and employees. There are still some difficulties to be resolved concerning security, conducting biometrics, and objective examinations. We expect that these issues will be overcome with improved biosensing, adequate training, and proper regulation. Given the shortage of occupational physicians and the customary overrunning of legal deadlines, we believe that these possibilities and suggestions should be explored and adopted by the specialty's Collegiates. Certain recommendations to this effect are made.

Com base na experiência pessoal de vários anos, foi realizada uma análise comparativa de dois sistemas de saúde europeus diferentes, de Portugal e da França, em uma perspectiva direcionada a uma comparação da medicina do trabalho e a utilização da telemedicina no contexto pós-pandêmico, em que ela se normalizou. Esta análise comparativa incide em quatro aspectos: sistema de saúde; medicina do trabalho; telemedicina/teletrabalho; e futuro e sugestões. Foram incluídas pesquisa e revisão documental de vários artigos recentes, normativas e recomendações das autoridades responsáveis pela tutela (Recomendações Colegiais, Ordem dos Médicos, Código do Trabalho), e foram feitas comparações de alguns indicadores estatísticos e estudos recentes. Três tabelas sobre a saúde, a medicina do trabalho e a telemedicina apresentam alguns dados relevantes e facilitam a comparação. Apesar da comparação difícil dadas as diferenças de base dos dois sistemas (modelo Beveridge versus Bismarck), parece ser possível concluir que existe uma maior abertura para a utilização criteriosa da teleconsulta na França (entre 15 e 35%), incluindo o seu uso por enfermeiras no contexto da visite d'information et de prévention, com uma boa aceitação pelos empregadores e trabalhadores. Ainda existem dificuldades por resolver, nomeadamente sobre a segurança e as dificuldades na sua utilização ou na realização da biometria ou exame objetivo. Por meio de melhorias no biosensing e de uma formação adequada, essas questões serão ultrapassadas. Dada a falta de médicos do trabalho e a habitual dilação dos prazos legais, essa possibilidade e as sugestões deveriam ser exploradas e enquadradas pelo Colégio da especialidade. Realizam-se algumas sugestões nesse sentido.

Online collaborative research on seasonal work. Collective capabilities to resist on precarious work and living conditions.

BACKGROUND: The number of seasonal workers in the agricultural sector in France is increasing and their working conditions are difficult and disgraceful. While they have been shown in various studies to be subject to processes that result in them being unable to act on occupational health, some of them, mobilized online, have formed a collective whose is permitted them to develop collective actions. OBJECTIVES: Our article aims to describe this online collective and how the power to act emerged from the discussions. The purpose it's also to determine how the functioning of these groups fosters the expression of a collective point of view and the achievement of goals that is not attained elsewhere. METHODS: We carried out a collaborative research online with a collective of seasonal workers, which consisted in following and taking part in discussions about conditions at work and outside of work, using an instant messaging tool. The results of this collaborative research, included testimonies registration and co-produced with seasonal workers, has been analyzed mobilizing A. Sen's capabilities approach. RESULTS: During their online discussions, the seasonal workers allow themselves to share the situations of injustice they are subject to with the other members. Here, we present their output and collective actions which were made possible by the mobilization of the resources of some of their members or by collaborating with other actors. CONCLUSIONS: In spite of the remarkable conversion factors made available through this collective action online, certain "failures" show that in order for these workers to have better access to their rights, effective support by public policy is indispensable.

Syphilis testing in blood donors, France, 2007 to 2022.

BackgroundSyphilis in blood donors (BD) has increased in many countries.AimWe aimed to describe trends in syphilis seroposivity in BD in France, to identify risk factors and assess if a non-treponemic test (NTT) could define BD having recovered from syphilis for more than 1 year.MethodsThe analysis covered the period 2007 to 2022 and 45,875,939 donations. Of the 474 BD syphilis-positive in 2022, 429 underwent additional investigations with an NTT. History of syphilis was obtained at the post-donation interview or based on serology results for repeat donors.ResultsUntil 2021, positivity rates remained stable (mean: 1.18/10,000 donations, range: 1.01-1.38). An increased rate was observed in 2022 (1.74/10,000; p = 0.02). Over the whole study period, prevalence was 2.2 times higher in male than in female BD (4.1 times higher in 2022). The proportion of males with an identified risk factor who have sex with men increased from 16.7% in 2007 to 64.9% in 2022. Based on NTT, 79 (18%) of the donors who were seropositive in 2022 were classified as having been infected in the previous year. History of syphilis was available for 30 of them. All had an infection within the previous 3 years. Among seven donors with a syphilis < 12 months before testing, one had an NTT titre ≥ 8, three a titre between 1 and 4, three were negative.ConclusionSyphilis seropositivity increased considerably in BDs in 2022, mostly in males, notably MSM. Available data did not allow appropriate evaluation of the NTT to distinguish recent from past infection.

Perceptions, expectations, and recommendations of trans adults on gender-affirming care in France: a qualitative study.

Trans people have diverse life experiences which may include gender-affirming care (GAC). GAC positively impacts the quality of life of trans adults. However, they are often met with barriers to care and are particularly vulnerable within the healthcare system. The needs and expectations surrounding GAC may vary between individual patients. This article aims to analyze trans adults' perceptions, expectations, and recommendations on GAC. Twenty-seven semi-structured interviews were conducted by a team of academic and peer researchers; transcribed interviews were then analyzed using a codebook and thematic analysis. Three main themes were identified; the liberating experience of GAC; the uneven distribution of knowledge and power between patients and providers; and the recommended practices in GAC. Additional training and research are necessary to facilitate high-quality care for trans adults accessing GAC.

Family trajectories among immigrants and their descendants in three European countries: A multistate approach in comparative research.

This study investigates partnership changes and childbearing among immigrants and their descendants in the UK, France, and Germany. Our analysis of longitudinal data shows, first, significant diversity in family trajectories among immigrants and their descendants in Europe. Immigrants from other European countries and their descendants tend to cohabit prior to marriage, and their fertility in unions is often similar to that of the native population. In contrast, South Asians and Turkish populations exhibit marriage-centred family behaviour with elevated third-birth rates. Individuals of sub-Saharan African or Caribbean origin display higher rates of non-marital family transitions. Second, we observe some changes in partnership and childbearing patterns across migrant generations; these are stronger for fertility than for partnership patterns. Third, migration background is particularly associated with partnership patterns, whereas the destination country context influences childbearing patterns. We expect some patterns to persist across future migrant generations (e.g. preference for marriage vs cohabitation), whereas others are likely to vanish (e.g. large families).

Evaluation of a New Telemedicine System for Early Detection of Cardiac Instability in Patients With Chronic Heart Failure: Real-Life Out-of-Hospital Study.

BACKGROUND: For a decade, despite results from many studies, telemedicine systems have suffered from a lack of recommendations for chronic heart failure (CHF) care because of variable study results. Another limitation is the hospital-based architecture of most telemedicine systems. Some systems use an algorithm based on daily weight, transcutaneous oxygen measurement, and heart rate to detect and treat acute heart failure (AHF) in patients with CHF as early on as possible. OBJECTIVE: The aim of this study is to determine the efficacy of a telemonitoring system in detecting clinical destabilization in real-life settings (out-of-hospital management) without generating too many false positive alerts. METHODS: All patients self-monitoring at home using the system after a congestive AHF event treated at a cardiology clinic in France between March 2020 and March 2021 with at least 75% compliance on daily measurements were included retrospectively. New-onset AHF was defined by the presence of at least 1 of the following criteria: transcutaneous oxygen saturation loss, defined as a transcutaneous oxygen measurement under 90%; rise of cardiac frequency above 110 beats per minute; weight gain of at least 2 kg; and symptoms of congestive AHF, described over the phone. An AHF alert was generated when the criteria reached our definition of new-onset acute congestive heart failure (HF). RESULTS: A total of 111 consecutive patients (n=70 men) with a median age of 76.60 (IQR 69.5-83.4) years receiving the telemonitoring system were included. Thirty-nine patients (35.1%) reached the HF warning level, and 28 patients (25%) had confirmed HF destabilization during follow-up. No patient had AHF without being detected by the telemonitoring system. Among incorrect AHF alerts (n=11), 5 patients (45%) had taken inaccurate measurements, 3 patients (27%) had supraventricular arrhythmia, 1 patient (9%) had a pulmonary bacterial infection, and 1 patient (9%) contracted COVID-19. A weight gain of at least 2 kg within 4 days was significantly associated with a correct AHF alert (P=.004), and a heart rate of more than 110 beats per minute was more significantly associated with an incorrect AHF alert (P=.007). CONCLUSIONS: This single-center study highlighted the efficacy of the telemedicine system in detecting and quickly treating cardiac instability complicating the course of CHF by detecting new-onset AHF as well as supraventricular arrhythmia, thus helping cardiologists provide better follow-up to ambulatory patients.

Acid sphingomyelinase deficiency in France: a retrospective survival study.

BACKGROUND: Acid sphingomyelinase deficiency (ASMD) or Niemann-Pick disease types A, A/B, and B is a progressive, life-limiting, autosomal recessive disorder caused by sphingomyelin phosphodiesterase 1 (SMPD1) gene mutations. There is a need to increase the understanding of morbidity and mortality across children to adults diagnosed with ASMD. METHODS: This observational retrospective survey analysed medical records of patients with ASMD with retrievable data from 27 hospitals in France, diagnosed/followed up between 1st January 1990 and 31st December 2020. Eligible records were abstracted to collect demographic, medical/developmental history, and mortality data. Survival outcomes were estimated from birth until death using Kaplan-Meier survival analyses; standardised mortality ratio (SMR) was also explored. RESULTS: A total of 118 medical records of patients with ASMD (type B [n = 94], type A [n = 15], and type A/B [n = 9]) were assessed. The majority of patients were males (63.6%); the median [range] age at diagnosis was 8.0 [1.0-18.0] months (type A), 1.0 [0-3] year (type A/B), and 5.5 [0-73] years (type B). Overall, 30 patients were deceased at the study completion date; the median [range] age at death for patients with ASMD type A (n = 14) was 1 [0-3.6] year, type A/B (n = 6) was 8.5 [3.0-30.9] years, and type B (n = 10) was 57.6 [3.4-74.1] years. The median [95% confidence interval (CI)] survival age from birth in patients with ASMD type A and type A/B was 2.0 [1.8-2.7] years and 11.4 [5.5-18.5] years, respectively. Survival analysis in ASMD type B was explored using SMR [95% CI] analysis (3.5 [1.6-5.9]), which showed that age-specific deaths in the ASMD type B population were 3.5 times more frequent than those in the general French population. The causes of death were mostly severe progressive neurodegeneration (type A: 16.7%), cancer (type B: 16.7%), or unspecified (across groups: 33.3%). CONCLUSIONS: This study illustrated a substantial burden of illness with high mortality rates in patients with ASMD, including adults with ASMD type B, in France.

Serosurvey of canine leishmaniasis in five departments near an identified human clinical case in Marseille (France).

Leishmania infantum is a protozoan parasite of the family Trypanosomatidae, transmitted by the bite of phlebotomine sand flies (Diptera: Psychodidae). It is responsible for human and canine leishmaniasis in countries bordering the Mediterranean basin. Here we describe a clinical case of human cutaneous leishmaniasis in a 76-year-old female patient living in Marseille. Upon interrogation, the patient had no history of recent travel or contact with animals. The study involved clinical, serological, and molecular investigation of the occurrence of Leishmania infection in 718 dogs from five departments within a 130 km radius perimeter around Marseille. Five dogs showed signs of moderate leishmaniasis. Additionally, the serological survey of dogs revealed a global seroprevalence of 5.1%, with a significantly different prevalence in the Vaucluse department. Molecular analysis and phylogenetic studies highlighted the close relatedness of Leishmania strains between human and canine hosts with 99.6% of identity, indicating local transmission. The findings underscore the importance of serological surveillance in dogs and human. In a One Health approach, it is necessary to continue investigating Leishmania infection in all potential reservoirs, especially for zoonotic L. infantum in wildlife (red foxes, leporids, rodents, etc.) but also in dogs, cats, and equids.

Multi-Elemental Analysis of Edible Insects, Scorpions, and Tarantulas from French (Online) Market and Human Health Risk Assessment Due to Their Consumption: A Pilot Study.

Edible insects are becoming increasingly popular as protein alternatives to traditional animal-based products. As such, information on their elemental composition is important to ensure they are safe for human consumption. This article describes the development and validation of a rapid, reliable method for the simultaneous determination of 19 elements (Al, As, B, Ba, Ca, Cd, Co, Cr, Cu, Fe, K, Mg, Mn, Mo, Na, Pb, Se, Sr, and Zn) in edible insects by inductively coupled plasma mass spectrometry (ICP-MS) following closed vessel microwave digestion. The method was validated using three insect certified reference materials, namely black soldier fly larvae meal (BFLY-1), cricket flour (KRIK-1), and mealworm powder (VORM-1). The method was applied to analyze twelve different (whole) insect species. The maximum amount of each sample was calculated for As, Cd, and Pb with respect to their provisional tolerable daily intake values established by the Food and Agricultural Organization/World Health Organization. Most of the samples, except for scorpions and tarantulas, were safe to consume at large doses (1000-10,000 insects per day). Furthermore, most of the samples contained high levels of Fe, K, Na, and Zn, providing a preliminary overview of the nutritional profile of these novel protein alternatives.

Phlebotomine sand fly distribution and abundance in France: A systematic review.

Global changes in climate are contributing to modified Phlebotomine sand fly presence and activity, and the distribution of the pathogens they transmit (e.g., Leishmania and Phlebovirus), and are leading to their possible extension toward northern France. To predict the evolution of these pathogens and control their spread, it is essential to identify and characterize the presence and abundance of potential vectors. However, there are no recent publications describing sand fly species distribution in France. Consequently, we carried out a systematic review to provide distribution and abundance maps over time, along with a simplified dichotomous key for species in France. The review adhered to PRISMA guidelines, resulting in 172 relevant capture reports from 168 studies out of the 2646 documents retrieved, of which 552 were read and 228 analyzed. Seven species were recorded and categorized into three groups based on their abundance: low abundance species, abundant but little-studied species, and abundant vector species. Sand flies are certainly present throughout France but there is a greater diversity of species in the Mediterranean region. Phlebotomus perniciosus and Ph. ariasi are the most abundant and widely distributed species, playing a role as vectors of Leishmania. Sergentomyia minuta, though very abundant, remains under-studied, highlighting the need for further research. Phlebotomus papatasi, Ph. perfiliewi, Ph. sergenti, and Ph. mascittii are present in low numbers and are less documented, limiting understanding of their potential role as vectors. This work provides the necessary basis for comparison of field data generated in the future.

Title: Répartition et abondance des phlébotomes en France : revue systématique. Abstract: Les changements globaux du climat contribuent à modifier la présence et l'activité des phlébotomes, ainsi que la répartition des pathogènes qu'ils transmettent (par exemple Leishmania et Phlebovirus), et conduisent à leur éventuelle extension vers le nord de la France. Pour prédire l'évolution de ces pathogènes et contrôler leur propagation, il est essentiel d'identifier et de caractériser la présence et l'abondance des vecteurs potentiels. Il n'existe cependant aucune publication récente décrivant la répartition des espèces de phlébotomes en France. Par conséquent, nous avons réalisé une revue systématique pour fournir des cartes de répartition et d'abondance dans le temps, ainsi qu'une clé dichotomique simplifiée pour les espèces françaises. La revue a respecté les lignes directrices PRISMA, aboutissant à 172 rapports de capture pertinents provenant de 168 études sur les 2 646 documents récupérés, dont 552 ont été lus et 228 analysés. Sept espèces ont été recensées et classées en trois groupes en fonction de leur abondance : les espèces de faible abondance, les espèces abondantes mais peu étudiées et les espèces vectrices abondantes. Les phlébotomes sont certes présents partout en France mais on trouve une plus grande diversité d'espèces dans le bassin méditerranéen. Phlebotomus perniciosus et Ph. ariasi sont les espèces les plus abondantes et les plus largement réparties, jouant un rôle de vecteurs de Leishmania. Sergentomyia minuta, bien que très abondant, reste sous-étudié, ce qui souligne la nécessité de recherches plus approfondies. Phlebotomus papatasi, Ph. perfiliewi, Ph. sergenti et Ph. mascittii sont présents en faibles nombres et sont moins documentés, ce qui limite la compréhension de leur rôle potentiel en tant que vecteurs. Ce travail fournit la base nécessaire pour la comparaison des données de terrain générées à l'avenir.