Main outcomes from the first 2 years of France's screening programme for neonatal permanent hearing loss through a descriptive study.

AIM: This study aimed to evaluate the implementation of France's neonatal hearing loss screening programme years after its launch, and to estimate permanent bilateral neonatal hearing loss (PBNHL) prevalence and distribution by severity. METHODS: This descriptive study used aggregated regional data on all births in France in 2015-2016. Screening coverage, refusal rate, positive predictive value (PPV), proportion of children with suspected PBNHL, PBNHL prevalence and distribution by severity were calculated. RESULTS: Eight hundred thousand neonates were eligible for the screening programme per year. Between 2015 and 2016, screening coverage increased (83.3% vs. 93.8%; p < 0.001), and the refusal rate remained stable (0.1%). In 2016, when considering the additional tests performed several weeks after birth, the proportion of suspected PBNHL neonates decreased (1.4% vs. 0.9%) while the PPV increased (4.7% vs. 7.6%). In 2015, the estimated prevalence of PBNHL (moderate to profound) was 0.09% (95% CI 0.08-0.10). Among neonates with >= 41 decibels deficit, 56.8%, 16.6%, and 26.6% had moderate, severe and profound hearing loss, respectively. CONCLUSION: The national target of 90% screening coverage was exceeded. The additional test could be useful to avoid overcrowding in diagnostic structures. Diagnostic data quality must be improved to confirm PBNHL prevalence and distribution by severity.

Impact of the SARS-CoV-2 pandemic and first lockdown on pregnancy monitoring in France: the COVIMATER cross-sectional study.

BACKGROUND: In the context of the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) pandemic, consultations and pregnancy monitoring examinations had to be reorganised urgently. In addition, women themselves may have postponed or cancelled their medical monitoring for organisational reasons, for fear of contracting the disease caused by SARS-CoV-2 (COVID-19) or for other reasons of their own. Delayed care can have deleterious consequences for both the mother and the child. Our objective was therefore to study the impact of the SARS-CoV-2 pandemic and the first lockdown in France on voluntary changes by pregnant women in the medical monitoring of their pregnancy and the associated factors. METHODS: A cross-sectional study was conducted in July 2020 using a web-questionnaire completed by 500 adult (> 18 years old) pregnant women during the first French lockdown (March-May 2020). A robust variance Poisson regression model was used to estimate adjusted prevalence ratios (aPRs). RESULTS: Almost one women of five (23.4%) reported having voluntarily postponed or foregone at least one consultation or pregnancy check-up during the lockdown. Women who were professionally inactive (aPR = 1.98, CI95%[1.24-3.16]), who had experienced serious disputes or violence during the lockdown (1.47, [1.00-2.16]), who felt they received little or no support (1.71, [1.07-2.71]), and those who changed health professionals during the lockdown (1.57, [1.04-2.36]) were all more likely to have voluntarily changed their pregnancy monitoring. Higher level of worry about the pandemic was associated with a lower probability of voluntarily changing pregnancy monitoring (0.66, [0.46-0.96]). CONCLUSIONS: Our results can guide prevention and support policies for pregnant women in the current and future pandemics.

FranceCoag: a 22-year prospective follow-up of the national French cohort of patients with inherited bleeding disorders.

FranceCoag is an ongoing open prospective multicentre cohort project aimed at improving epidemiological knowledge about inherited bleeding disorders in France. The main objective of this article was to evaluate the project's progress as of the 30th December 2016. Between 1994 and this date, of the 10,047 patients included in the study, 384 (3.8%) were reported by clinicians to have died and 159 (1.6%) to be lost to follow-up. Among the remaining 9504 patients still being followed up, 5748 (60.5%) had haemophilia A, 1300 (13.7%) haemophilia B, 1980 (20.8%) von Willebrand Disease while 476 (5.0%) had another clotting factor deficiency (Factor I, II, V, combined V and VIII, VII, X, XI and XIII). The median age of the population was 32 years (Inter-quartile range (IQR) 18-50 years) at data extraction on December 30th, 2016. The subgroup of children (i.e., < 18 years old) with severe haemophilia and comprehensive information available since the first exposure to treatment was identified as the PUPs (Previously Untreated Patients) cohort. Data for the 643 children included in the PUPs' cohort had been collected since their birth. Follow-up data were collected by the clinicians in haemophilia treatment centres (HTC) every 12.9 months on median (IQR 11.4-21.3). In the PUPS cohort, data were updated every 6.2 months on median (IQR 3.7-11.7). A unique patient number assigned at study inclusion was kept at individual HTC by participating clinicians. The data collected included demographic, clinical, therapeutic and biological items on standard electronic forms. As of December 30th 2016, a plasma and serum samples was available for 2581 patients (27.1%).

Analyses of the FranceCoag cohort support differences in immunogenicity among one plasma-derived and two recombinant factor VIII brands in boys with severe hemophilia A.

Around one third of boys with severe hemophilia A develop inhibitors (neutralizing antibodies) against their therapeutic factor VIII product. This adverse effect may result in more life-threatening bleeding, disability, impaired quality of life, and costly care. We compared the incidence of inhibitors in boys treated with the three factor VIII products most used in France: one plasma-derived (Factane) and two recombinant products (Advate and Kogenate Bayer). A previously untreated cohort of patients was created in 1994 to investigate risk factors for inhibitor development. We selected boys with severe hemophilia A (factor VIII <1 IU/dL) first treated with one of the three factor VIII products studied. Details of product infusions, inhibitor assays and main fixed and time-varying inhibitor risk factors were recorded for the first 75 exposure days. Three outcomes (all inhibitors, high-titer inhibitors and subsequently treated inhibitors) were analyzed by univariate and multivariate Cox models. We studied 395 boys first treated between 2001 and 2016 (131, 137, and 127 with Factane, Advate, and Kogenate Bayer, respectively). Clinically significant inhibitors were diagnosed in 121 patients (70 high-titer). The incidence of high-titer inhibitors was significantly associated with the factor VIII product received (P=0.005): the cumulative incidence at 75 exposure days was 12.7% (95% CI: 7.7-20.6) with Factane, 20.4% (95% CI: 14.0-29.1) with Advate, and 31.6% (95% CI: 23.5-41.7) with Kogenate Bayer. The low inhibitor incidence observed with Factane is concordant with recent findings from the SIPPET randomized trial. These consistent results from observational and experimental studies should lead to improved care for previously untreated patients and cost savings for healthcare systems worldwide.

Use of clinical practice guidelines on long-term prophylaxis in severe hemophilia in France: a retrospective audit.

OBJECTIVES: To evaluate the impact of the 2002 guidelines on the current status of prophylaxis in French children with severe hemophilia A or B. STUDY DESIGN: Clinical information was captured, in a prospective way, using FranceCoag Network. We retrospectively studied 291 patients with severe (<1 IU/dL) hemophilia A and B, with no history of inhibitors. RESULTS: Our results demonstrate that the availability of national medical guidelines has improved clinical practice in France. In the past decade, the proportion of children with severe hemophilia undergoing prophylaxis has shown a significant 2- to 3-fold increase: ∼80% of these children>3 years of age are now receiving prophylaxis. In severe hemophilia A and B, the age at which prophylaxis commences has significantly decreased: 4.0 and 6.1 years for the period 1996-1999 as opposed to 1.8 and 1.4 years for the period 2004-2007 (P=.0001). CONCLUSIONS: Long-term clinical and physical evaluations of patients will be necessary to establish the benefits of this increase in prophylactic treatment on the prevention of hemophilic arthropathy.

Mental health of pregnant women during the SARS-CoV-2 pandemic in France: Evolution of self-perceived psychological state during the first lockdown, and anxiety frequency two months after the lockdown ended.

Previous pandemics and related lockdowns have had a deleterious impact on pregnant women's mental health. We studied the impact of the SARS-CoV-2/Covid-19 pandemic and France's first lockdown on pregnant women's mental health. A cross-sectional study was conducted in July 2020 using a web-questionnaire completed by 500 adult women who were pregnant during the first lockdown in France (March-May 2020). Questions focused on their self-perceived psychological state and affects they felt before and during the lockdown and anxiety symptomatology (HAD) two months after it ended. A robust variance Poisson regression model was used to estimate adjusted prevalence ratios (aPR) for anxiety and self-perceived psychological state evolution. One in five respondents (21.1%) reported psychological deterioration during lockdown. Associated determinants were: i) little or no social support (self-perceived) (aRP = 1.77, 95%CI[1.18-2.66]), ii) increased workload (1.65, [1.02-2.66]), and iii) poor/moderate knowledge about SARS-CoV-2 transmission (1.60, [1.09-2.35]). Seven percent of women reporting psychological deterioration had access to professional psychological support during lockdown, while 19% did not despite wanting it. Women reported heightened powerlessness (60.3%), frustration (64%) and fear (59.2%) during lockdown. One in seven respondents (14.2%, 95%CI[10.9-18.2]) had anxiety symptoms. Determinants associated: i) at least one pregnancy-related pathology (aPR = 1.82, 95%CI[1.15-2.88]), ii) overweightness or obesity (1.61, [1.07-2.43]), iii) one child under the age of six years in the household during the lockdown (3.26, [1.24-8.53]), iv) little or no social support (self-perceived) during the lockdown (1.66, [1.07-2.58]), v) friend or relatives diagnosed with Covid-19 or with symptoms of the disease (1.66; [1.06-2.60]), vi) no access to medication for psychological distress (2.86, [1.74-4.71]), and vii) unsuccessfully seeking exchanges with healthcare professionals about their pregnancy during the pandemic (1.66, [1.08-2.55]). Our results can guide prevention and support policies for pregnant women during pandemics, current or future, with or without lockdowns. Preventing perinatal mental health problems is essential to ensure a supportive environment for the child's development.

Perception of the SARS-CoV-2 pandemic by pregnant women during the first lockdown in France: worry, perceived vulnerability, adoption and maintenance of prevention measures according to the Covimater study.

Background: We aimed to describe pregnant women's worry about the SARS-CoV-2 pandemic, the associated reasons, their perceived vulnerability to this infection, and factors influencing continued poor/non-existent or decreased implementation of preventive measures over time. Method: A cross-sectional study was conducted in July 2020 using a web-questionnaire completed by 500 women who were pregnant during the first lockdown in France (March-May 2020). Questions focused on worry caused by the pandemic, perceived vulnerability to infection by SARS-CoV-2 and implementation of preventive measures during and after lockdown. A robust variance Poisson regression model was used to estimate adjusted prevalence ratios (aPR) for perceived vulnerability and continued poor/non-existent or decreased implementation of preventive measures. Results: Participants felt significantly more vulnerable to infection than women of childbearing age who were included in a parallel study on the French general population, but were significantly less worried about the pandemic. Obese participants and those who unsuccessfully sought exchanges with healthcare professionals about their infection risk felt significantly more vulnerable (aPR = 1.32 95%CI[1.05-1.64] and 1.88 [1.43-2.48], respectively). Participants with continued poor/non-existent or decreased implementation of preventive measures two months after the lockdown ended were more likely to have experienced violence during the lockdown (2.06, [1.32-3.22]), or to live in areas less affected by the pandemic (1.66 [1.05-2.62]). A good knowledge of viral transmission (0.54 [0.30-0.97]) and a high perceived vulnerability score (0.66 [0.44-0.99]) were associated with maintained/increased implementation of preventive measures. Conclusions: Our results can guide prevention and support policies for pregnant women during pandemics, current or future.

Pregnant women's unmet need to communicate with a health professional during the SARS-CoV-2 pandemic lockdown in France: The Covimater cross-sectional study.

During the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) pandemic lockdown, communication between pregnant women and health professionals may have become complicated due to restrictions on movement and saturated health services. This could have impacts on pregnancy monitoring and women's wellbeing. We aimed to i) describe the unmet need of pregnant women living in France to communicate with health professionals about the pandemic and their pregnancy during the lockdown, ii) assess the socio-demographic, medical and contextual factors associated with this unmet need. The Covimater cross-sectional study, conducted in July 2020, includes data on 500 adult women's experiences of pregnancy during the first lockdown period in France (i.e., from March to May 2020). The women, all residents in metropolitan France, answered a web-based questionnaire about their conversations with health professionals during the lockdown, as well as their social and medical characteristics. A robust variance Poisson regression model was used to estimate crude or adjusted prevalence ratios (aPRs) for their unmet need to communicate with health professionals about the pandemic and their pregnancy. Forty-one percent of participants reported an unmet need to communicate with a health professional during the lockdown, mainly about the risk of transmitting SARS-CoV-2 to their baby and the consequences for the latter. Factors associated were: i) being professionally inactive (aPR = 1.58,CI95%[(1.14-2.21]), ii) having an educational level below secondary school diploma (1.38,[1.05,-1.81]), iii) having experienced serious arguments/violence (2.12,[1.28-3.52]), iv) being very worried about the pandemic (1.41,[1.11-1.78]), v) being primiparous (1.36,[1.06-1.74]) and vi) having had pregnancy consultations postponed/cancelled by health professionals during the lockdown (1.35,[1.06-1.73]). These results can be used to develop targeted strategies that ensure pregnant women are able to i) communicate with health professionals about the potential impact of the SARS-CoV-2 pandemic on their pregnancy, and ii) access up-to-date and reliable information on the consequences of SARS-CoV-2 for themselves and their child.