French Centers Research Articles

T-Wave Oversensing with Contemporary Implantable Cardioverter-Defibrillators.

Implantable cardioverter-defibrillators (ICDs) need to reliably detect ventricular tachycardia (VT) and ventricular fibrillation (VF) while avoiding T-wave oversensing (TWOS), which is associated with a risk of inappropriate therapies. The incidence of TWOS with endovascular ICDs appears to differ between manufacturers. We aimed to evaluate the incidence and clinical consequences of TWOS with contemporary Medtronic and Boston Scientific ICDs. Consecutive patients implanted with a recent Medtronic or Boston Scientific ICD and remotely monitored at three French centers were included. All transmitted EGMs labelled as VF, VT, non-sustained VT (NSVT), or ventricular oversensing (Medtronic) were screened for TWOS. Among 7589 transmitted episodes from 674 patients with a Boston Scientific ICD, we did not identify a single case of TWOS. Among 16,790 transmitted episodes from 1733 patients with a Medtronic ICD, we identified 60 patients (3.4%) with at least one episode of TWOS. In 46 patients, TWOS was intermittent (NSVT episodes). In the remaining 14 patients, TWOS resulted in 60 sustained episodes (completed counters). No inappropriate therapies were delivered in 12 of these patients because no therapies were programmed (in monitor zones, 11 episodes) or because therapies were inhibited by the morphology discriminator (Wavelet, 19 episodes) or by the anti-TWOS algorithm (26 episodes). Two patients received inappropriate therapies due to TWOS (0.1% of patients with Medtronic ICDs). On review of 24,379 transmitted episodes from 2407 patients with endovascular ICDs, we found no case of TWOS with Boston Scientific devices, whereas TWOS was not uncommon with Medtronic devices. However, the risk of inappropriate therapy with Medtronic ICDs was very low (0.1%) due to the often intermittent nature of this phenomenon, the morphology discriminator, and the anti-TWOS algorithm.

Is the clinical phenotype impact the prognosis in dementia with Lewy bodies?

IntroductionThe first predominant clinical symptoms of dementia with Lewy bodies (DLB) are highly variable; however, the prognosis based on initial predominant symptoms remains poorly understood.MethodsMulticenter retrospective study in 4 French expert neurological centers. Patients were categorized in 3 groups according to their first more predominant symptoms: cognitive, psychiatric, or motor.ResultsAnalysis of 310 DLB patients. The mean age was 73.5 years old (SD 7.5) including 32.3% of women. The mean follow-up was 7.25 years (SD 3.6). We observed that the full clinical picture was more frequent in the motor group than in the cognitive group (p = 0.01); male gender and age at onset were associated with a significant excess risk of instantaneous mortality (p = 0.01).ConclusionInitial symptoms may affect the clinical course of patients, but no significant difference in mortality was observed.

Left atrial appendage closure in very elderly patients in the French National Registry

ObjectiveLeft atrial appendage closure (LAAC) is recommended to decrease the stroke risk in patients with atrial fibrillation and contraindications to anticoagulation. However, age-stratified data are scarce. The aim of this...

Characterization of 164 patients with NRAS mutated non-small cell lung cancer (NSCLC)

BackgroundNRAS mutations are observed in less than 1% of non-small cell lung cancer (NSCLC). Clinical data regarding this rare subset of lung cancer are scarce and response to systemic treatment such as chemotherapy or immune checkpoint inhibitors (ICI) has never been reported. MethodsAll consecutive patients with an NRAS mutated NSCLC, diagnosed between August 2014 and November 2020 in 14 French centers, were included. Clinical and molecular data were collected and reviewed from medical records. ResultsOut of the 164 included patients, 106 (64.6%) were men, 150 (91.5%) were current or former smokers, and 104 (63.4%) had stage IV NSCLC at diagnosis. The median age was 62 years, and the most frequent histology was adenocarcinoma (81.7%). NRAS activating mutations were mostly found in codon 61 (70%), while codon 12 and 13 alterations were observed in 16.5% and 4.9% of patients, respectively. Programmed death ligand-1 expression level <1%/1–49%/≥50% were respectively found in 30.8%/27.1%/42.1% of tumors. With a median follow-up of 12.5 months, median overall survival (OS) of stage IV patients was 15.3 months (95% CI 9.9–27.6). No significant difference in OS was found according to the type of mutation (codon 61 vs. other), HR = 1.12 (95% CI 0.65–1.95). Among stage IV patients treated with platinum-based doublet (n = 66), ICI (n = 48), or combination of both (n = 10), objective response rate, and median progression free survival were respectively 45% and 5.8 months, 35% and 6.9 months, 70% and 8.6 months. ConclusionNRAS mutated NSCLC are characterized by a high frequency of smoking history and codon 61 mutations. Further studies are needed to confirm the encouraging outcome of immunotherapy in combination with chemotherapy.

High-purified cannabidiol efficacy and safety in a cohort of adult patients with various types of drug-resistant epilepsies

About 30% of patients with epilepsy are drug resistant. Lennox-Gastaut syndrome (LGS), Dravet syndrome (DS) and tuberous sclerosis complex (TSC) are diseases for which high-purified-cannabidiol (CBD) known as Epidiolex® (GW pharma) can be prescribed in add-on of other medications in case of drug-resistance. Currently, there are only a few recent data in the literature about the efficacy and safety of CBD in other forms of refractory epilepsies especially focal epilepsies in adults. We report retrospectively the experience of high-purified-CBD use in two French reference medical centers for epilepsy in various forms of drug-resistant epilepsy. We distinguished two groups of patients: group A with epileptic encephalopathies and group B with focal or multifocal epilepsy. Safety and efficacy (% of responder patients) were evaluated. Finally, 73 patients (51 in group A and 22 in group B) used high-purified CBD as an add-on treatment for their drug-resistant epilepsy. Patients in group A were significantly younger (P=0.0155), with a longer exposition of treatment (P=0.0497) than group B and with higher doses (P=0.0300). Respectively, 15 patients (29.4%) and five patients (22.7%) were responders during the follow-up period (P=0.552). The association with clobazam was more frequent in responders than in non-responder patients (16 patients [80%] versus four [20%]). The most frequent side effect was somnolence. At the end of follow-up, 15 patients in group A (29.4%) and nine patients in group B (40.1%) had stopped the high-purified-CBD treatment due to aggravation of seizure, absence of positive effects, or adverse events. This study showed no significant difference regarding the type of drug-resistant epilepsy and suggests that this treatment may be of interest for all types of drug-resistant epilepsy.

Nephrometry scores to predict oncological outcomes following partial nephrectomy (UroCCR Study 70).

Partial nephrectomy (PN) for large or complex renal tumors can be difficult and associated with a higher risk of recurrence than radical nephrectomy. We aim to evaluate the clinical useful of nephrometry scores for predicting oncological outcomes in a large cohort of patients who underwent PN for renal cell carcinomas. Our analysis included patients who underwent PN for renal cell carcinoma in 21 French academic centers (2010-2020). RENAL, PADUA, and SPARE scores were calculated based on preoperative imaging. Uni- and multivariate cox models were performed to identify predictors of recurrence-free survival and overall survival. The area under the curve (AUC) was used to identify models with the highest discrimination. Decision curve analyses (DCAs) determined the net benefit associated with their use. A total of 1927 patients were analyzed with a median follow-up of 32months (14-45). RENAL score (p = 0.01), age (p = 0.002), histological type (p = 0.001), high nuclear grade (p = 0.001), necrotic component (p < 0.001), and positive margins (p = 0.005) were significantly related to recurrence in multivariate analyses. The discriminative performance of the 3 radiological scores was modest (65, 63, and 63%, respectively). All 3 scores showed good calibration, which, however, deteriorated with time. Decision curve analysis of the three models for the prediction of overall and recurrence-free survival was similar for all three scores and of limited clinical relevance. The association between nephrometry scores and oncological outcomes after NP is very weak. The use of these scores for predicting oncological outcomes in routine practice is therefore of limited clinical value.

Open Versus Laparoscopic Right Hepatectomy for Hepatocellular Carcinoma Following Sequential TACE-PVE: A Multicentric Comparative Study.

Right hepatectomy (RH) for hepatocellular carcinoma (HCC) is ideally preceded by transcatheter arterial chemoembolization (TACE) and portal vein embolization (PVE). Laparoscopic approach improves short-term outcome and textbook outcome (TO), which reflects the "ideal" surgical outcome, after RH. However, laparoscopic RH on an underlying diseased liver and after TACE/PVE remains a challenging procedure. The aim of this study was to compare the outcomes in patients who underwent laparoscopic liver resection (LLR) or open liver resection (OLR) following TACE/PVE. All patients with HCC who underwent RH after TACE/PVE in five French centers were retrospectively included. Outcomes were compared between the LLR group and the OLR group using propensity score matching (PSM). Quality of surgical care was defined by TO. Between 2005 and 2019, 117 patients were included (41 in LLR group, 76 in OLR group). Overall morbidity was comparable (51% versus 53%, p=0.24). In LLR group, TO was completed in 66% versus 37% in OLR group (p=0.02). LLR and absence of clamping were the only factors associated with TO completion [hazard ratio (HR) 4.27, [1.77-10.28], p=0.001]. After PSM, 5-year overall survival (OS) and progression-free survival (PFS) were 55% in matched LLR versus 77% in matched OLR, p=0.35, and 13% in matched LLR versus 17% in matched OLR, p=0.97. TO completion was independently associated with a better 5-year OS (65.2% versus 42.5%, p=0.007). Major LLR after TACE/PVE should be considered as a valuable option in expert centers to increase the chance of TO, the latter being associated with a better 5-year OS.

Prognostic score for synchronous metastatic rectal cancer: A real-world study.

Prognostic factors of metastatic rectal cancer are not well known. The objective of this study was to identify prognostic factors of overall survival (OS) in a cohort of patients with non-resectable synchronous metastatic rectal cancer. Patients were retrospectively enrolled from 18 French centres. Univariate and multivariate analyses were performed to identify prognostic factors for OS. A simple score was derived from this a development cohort RESULTS: A total of 243 patients with metastatic rectal cancer were included in the study. Median OS was 24.4 months, 95% CI [19.4-27.2]. Among patients with non-resected metastases (n=141), six independent prognostic factors associated with better OS were identified in multivariate analysis: primary tumour surgery, WHO score 0-1, middle or upper rectal tumour, lung metastases only, systemic chemotherapy and targeted agent in first line. A prognostic score individualized three groups, each factor counting for one point in the score (<3,=3 et > 3). Their median OS were respectively 27.9 months, 95% CI [21.7-35.1], 17.1 months [11.9-19.7] (HR2/1=2.08, 95%, CI [1.31-3.30], p2/1=0.002) and 9.1 months [4.9-11.7] (HR3/2=2.32, 95% CI [1.38-3.92], p3/2=0.001). A prognostic score for non-resectable synchronous metastatic rectal cancer can be proposed to classify patients in three prognostic groups.

Morbidity, mortality, and prognostic factors in gestational trophoblastic neoplasia with liver metastasis.

Liver metastases of gestational trophoblastic neoplasia (GTN) are rare, but associated with poor prognosis. The additional concomitant presence of brain or intra-abdominal metastases, with liver metastases has been described as worsening factors, but the literature on this topic is reduced. To estimate the overall mortality, specific hepatic morbidity, and mortality, and to identify prognostic factors for patients with GTN and liver metastases. The medical records of 26 GTN patients with liver metastases registered in the French Center for Trophoblastic Diseases and treated between November 1999 and December 2019 were reviewed. Overall survival was described using Kaplan-Meier estimates. Prognostic factors were identified using univariate and multivariate Cox analyses. The 5-year overall survival rate was 60.7% for all patients with liver metastasis. The survival rate was higher in patients who achieved complete remission after first-line chemotherapy than in those who did not (100% vs 20%, p=0.001). The only factor independently associated with prognosis was the presence of 6 or more liver metastases (5-year survival, 16.7% vs. 82.4% otherwise; HR =11.1, 95%CI, 2.3-53.1; p=0.003). None of the five patients with a single liver metastasis died. GTN with liver metastasis is very rare (1.6%). The prognosis of patients seems to be improving. The results of this study are also reassuring for patients with complete remission after first-line combination chemotherapy, as well as for those with a single liver metastasis.

The clinical and financial burden of nonhepatocellular carcinoma focal lesions detected during the surveillance of patients with cirrhosis.

HCC surveillance is challenged by the detection of hepatic focal lesions (HFLs) of other types. This study aimed to describe the incidence, characteristics, outcomes, and costs of non-HCC HFL detected during surveillance. We retrospectively analyzed nonstandardized workup performed in French patients included in HCC surveillance programs recruited in 57 French tertiary centers (ANRS CirVir and CIRRAL cohorts, HCC 2000 trial). The overall cost of workup was evaluated, with an estimation of an average cost per patient for the entire population and per lesion detected. A total of 3295 patients were followed up for 59.8 months, 391 (11.9%) patients developed HCCs (5-year incidence: 12.1%), and 633 (19.2%) developed non-HCC HFLs (5-year incidence: 21.8%). Characterization of non-HCC HFL required a median additional of 0.7 exams per year. A total of 11.8% of non-HCC HFLs were not confirmed on recall procedures, and 19.6% of non-HCC HFLs remained undetermined. A definite diagnosis of benign liver lesions was made in 65.1%, and malignant tumors were diagnosed in 3.5%. The survival of patients with benign or undetermined non-HCC HFL was similar to that of patients who never developed any HFL (5-year survival 92% vs. 88%, p = 0.07). The average cost of the diagnostic workup was 1087€ for non-HCC HFL and €1572 for HCC. Non-HCC HFLs are frequently detected in patients with cirrhosis, and do not impact prognosis, but trigger substantial costs. This burden must be considered in cost-effectiveness analyses of future personalized surveillance strategies.

CANTO: Skin Toxicities Evaluation of a Multicenter Large Prospective Cohort of Irradiated Patients for Early-Stage Breast Cancer

Skin damage is a common toxicity during oncology management. Its assessment and understanding of the factors influencing its occurrence, is a major issue in the management of patients treated for an early breast cancer (BC). CANTO (NCT01993498) is a prospective, multicentric, clinical cohort study of 11 400 patients with stage I-III BC, treated in 26 French cancer centers. In this study, we will evaluate the 8561 patients during their overall management for a BC. We are focus on specifical skin toxicities: Erythema, fibrosis, telangiectasia and skin color (CTCAE v4.0). These toxicities were assessed at 3-6 (M0), 12 (M12), 36 (M36), 60 (M60) months. The prevalence of toxicities of interest varied over time, so at M0, 30.4% of patients had erythema while 17.7% of patients had fibrosis. At M12, M36 and M60, the prevalence of erythema decreased from 5.5 % to 2.5 % then to 2 %, respectively, while fibrosis remained stable at about 19 %. The prevalence of telangiectasia increased from 0.8 % to 7 % from M0 to M60. While the modification of skin color was 51.1 % at M0, and 10.6 % at M60. After adjustments, at M0, there was a statistically significant association between the onset of cutaneous erythema and obesity, the presence of axillary dissection, the type of surgery the age of the patient as well as the tumor phenotype RH+/HER2+ [OR: 0.79 [0.67; 0.93]]. Concerning fibrosis, a statistically significant association was found, at M12, with the age of the patient, obesity, tumor grade, Charlson score, type of surgery and mitotic index. Obesity and the age of the patient represent at M12 and M36 a risk significantly associated with the onset of telangiectasia. Concerning the modification of skin color at M12, we find a link between the age of the patient, obesity, tobacco consumption, tumor grade, Charlson score, type of surgery, and alcohol consumption [OR: 0.69 [0.51; 0.91]]. In this study we identified several risk factors for acute and late skin toxicity. The knowledge of its predictive factors allows a personalized management of the patient by adapting our treatments and our monitoring according to these different factors.

Early Results of the French Multicenter, Randomized SHARE Trial Comparing Whole Breast Irradiation vs. Accelerated Partial Breast Irradiation in Postmenopausal Women with Early-Stage Breast Cancer

The aim is to report toxicity and cosmetic outcomes at 3 and up to 9 years of follow-up of post-menopausal patients randomized to receive either standard whole breast irradiation (WBI), including hypofractionated options, versus accelerated partial breast irradiation (APBI). From December 2010 to July 2015, 1006 patients were enrolled in 34 French centers. Among the whole population, 28 patients who did not meet the final selection criteria or withdrew consent were excluded leading to a modified intention to treat analysis dataset of 978 patients (WBI: n = 488; APBI: n = 490). Median age (65y) and tumor stage pT1 (99%) rates were similar in both arms. Patients had conservative surgery with clip placement in the tumor bed. Clear margins (> 2mm) were observed in 99% of the patients. In both arms, 96-97% of the patients had negative sentinel lymph node biopsy. The median time interval between surgery and radiotherapy was 57d in WBI vs 62d in APBI. WBI schedules consisted of 50 Gy in 25 fr+16 Gy (n = 212) or 40 Gy in 15 fr (n = 156) or 42.5 Gy in 16 fr (n = 120). APBI arm consisted of 38.5 Gy or 40 Gy in 10 fr, 2 fr/day. Overall, 94 patients from the APBI arm finally received standard WBI. For statistical considerations, SHARE trial, sponsored by UNICANCER (NCT01247233) is a non-inferiority randomized controlled trial comparing APBI versus WBI in terms of local control as primary objective. Secondary endpoints were severe toxicity (NCI-CTCAE v4 grade ≥ 2), and cosmetic results. For both outcomes, we estimated the cumulative incidences (CI) using Kalbfleish and Prentice method, considering disease relapse, secondary cancer or death as competing events. Treatment effect (APBI vs WBI) was estimated by cause-specific Hazard Ratios (cs-HR) from Cox models. Median follow-up was 5.8y (range, 0.13-9.5). The number of deaths was 27, and the number of local relapses was 8. Among the 978 patients, 582 and 396 had finally WBI and APBI, respectively. The rates of post-operative hematoma, edema and infection were low: 8-9%, 2%, 3-2%, respectively. When considering any type of severe toxicity, we observed a significant reduction rate in APBI compared to WBI: cs-HR = 0.73 (95% confidence interval: 0.61-0.88); p = 0.001, and 3-year CI of severe toxicity at 45% in WBI vs 36% in APBI arm. The difference was also in favor of APBI when considering breast skin toxicity alone: cs-HR = 0.55 (0.44-0.70), p<0.001 and 3-year CI at 36% in WBI vs 21% in APBI arm. Conversely, for breast other toxicities, WBI was found less toxic than APBI with a 3-year CI of 8% vs 15%, respectively. When considering cosmetic results, we observed no significant difference between the two arms in both evaluations by physicians and patients. Historically SHARE is the first APBI trial that included hypofractionated schedules in the standard arm. We report increased risk of severe toxicity and skin breast toxicity in standard arm as compared with APBI arm without any difference in terms of cosmetic results. Longer follow-up is needed.

Piecemeal Resection for Large Colorectal Adenomas Remains Essential in 2022: A Single-Center Experience in a Tertiary French Center.

Colorectal lesions measuring greater than 20 mm are unsuitable for en bloc endoscopic mucosal resection (EMR): piecemeal EMR (PM-EMR) and endoscopic submucosal dissection (ESD) are needed. The European Society of Gastrointestinal Endoscopy (ESGE) recommends ESD only for microinfiltrative lesions, although Japanese teams perform en bloc ESD for all lesions. We report the outcomes obtained in our endoscopy unit for these lesions and assess the hybrid "knife-assisted piecemeal EMR" (KAPM-EMR) technique. The main aim was to assess the short-term outcomes (C1). The secondary objectives were to evaluate the long-term results (C2), adverse event rate and management of recurrence. We retrospectively analyzed data from patients treated by PM-EMR, KAPM-EMR and ESD for a colorectal lesion measuring greater than 20 millimeters using prospective inclusion over four years. Data from 167 patients (median age: 70) with a median follow-up of 15.1 months were analyzed after excluding 95 patients. A total of 131 lesions were removed by PM-EMR, 24 by KAPM-EMR and 12 by ESD; 146/167 (87.4%) patients were considered in remission at C1. Recurrence was treated by endoscopy in 20/21 patients (95%); 86/89 (96.6%) were in remission at C2. A total of 16/167 patients developed adverse events, all of whom except one were endoscopically managed. KAPM-EMR was associated with a higher perforation risk (p=0.037). No differences in postoperative bleeding were found among the three groups (p=0.576). Piecemeal resection remains an effective and safe technique for large colorectal adenomas. KAPM-EMR may be useful but should be applied with caution due to the risk of perforation.

Prognostic impact of venous thromboembolism on the course of sarcoidosis: A multicenter retrospective case-control study

Sarcoidosis is an independent risk factor for venous thromboembolism (VTE). However, the characteristics and clinical evolution of sarcoidosis patients presenting a VTE (sarcoidosis/VTE group) in the course of their disease are not known. Consequently, if VTE occurrence is associated with a more severe disease is still pending.We conducted a retrospective case-control study of sarcoidosis/VTE patients compared to matched sarcoidosis controls without VTE in two French tertiary centers, analysed and compared the clinical, biological, functional, imaging and evolutive profiles of the two groups.Sixty-one patients were included with at least one episode of VTE during course of sarcoidosis. At sarcoidosis onset (before/at the time of VTE occurrence) the number of affected organs, radiological stages and pulmonary functional tests were not significantly different between the two groups. In contrast, we found that sarcoidosis/VTE patients required more frequently a systemic immunosuppressive therapy (corticosteroids and/or immunosuppressors, 79% versus 58%; p = 0.008). The functional course was also poorer in sarcoidosis/VTE patients with a more frequent decrease in functional vital capacity (33% versus 18% in sarcoidosis/VTE patients and controls, respectively, p = 0.008). Finally, sarcoidosis/VTE patients presented more frequently with pulmonary hypertension (10% versus 1% in patients and controls, respectively, p = 0.006), and their survival was significantly worse (log-rank p <0.001).The occurrence of VTE during sarcoidosis is associated with a more severe disease and a poorer prognosis. The occurrence of VTE during sarcoidosis might signal a more inflammatory and/or evolutive disease in sarcoidosis/VTE patients and should be taken in consideration when designing therapeutic strategies for them.

The France PCI registry: Design, methodology and key findings

Obstructive coronary artery disease is the main cause of death worldwide. By tracking events and gaining feedback on patient management, the most relevant information is provided to public health services to further improve prognosis. To create an inclusive and accurate registry of all percutaneous coronary intervention (PCI) procedures performed in France, to assess and improve the quality of care and create research incentives. Also, to describe the methodology of this French national registry of interventional cardiology, and present early key findings. The France PCI registry is a multicentre observational registry that includes consecutive patients undergoing coronary angiography and/or PCI. The registry was set up to provide online data analysis and structured reports of PCI activity, including process of care measures and assessment of risk-adjusted outcomes in all French PCI centres that are willing to participate. More than 150 baseline data items, describing demographic status, PCI indications and techniques, and in-hospital and 1-year outcomes, are captured into local reporting software by medical doctors and local research technicians, with subsequent encryption and internet transfer to central data servers. Annual activity reports and scoring tools available on the France PCI website enable users to benchmark and improve clinical practices. External validation and consistency assessments are performed, with feedback of data completeness to centres. Between 01 January 2014 and 31 December 2022, participating centres increased from six to 47, and collected 364,770 invasive coronary angiograms and 176,030 PCIs, including 54,049 non-ST-segment elevation myocardial infarction cases and 31,631 ST-segment elevation myocardial infarction cases. Fifteen studies stemming from the France PCI registry have already been published. This fully electronic, daily updated, high-quality, low-cost, national registry is sustainable, and is now expanding. Merging with medicoeconomic databases and nested randomized scientific studies are ongoing steps to expand its scientific potential.

Kidney Transplantation in Patients With AA Amyloidosis: Outcomes in a French Multicenter Cohort

Outcomes of kidney transplantation for patients with renal AA amyloidosis are uncertain, with reports of poor survival and high rates of disease recurrence. However, the data are inconclusive and mostly based on studies from the early 2000s and earlier. Retrospective multicenter cohort study. We searched the French national transplant database to identify all patients with renal AA amyloidosis who underwent kidney transplantation between 2008 and2018. Age, cause of amyloidosis, use of biotherapies, and C-reactive protein levels. Outcomes were all-cause mortality and allograft loss. We also reported amyloidosis allograft recurrence, occurrence of acute rejection episodes, as well as infectious, cardiovascular, and neoplastic disease events. Kaplan-Meier estimator for mortality and cumulative incidence function method for allograft loss. Factors associated with patient and allograft survival were investigated using a Cox proportional hazards model and a cause-specific hazards model, respectively. 86 patients who received kidney transplants for AA amyloidosis at 26 French centers were included. The median age was 49.4 years (IQR, 39.7-61.1). The main cause of amyloidosis was familial Mediterranean fever (37 cases; 43%). 16 (18.6%) patients received biotherapy after transplantation. Patient survival rates were 94.0% (95% CI, 89.1-99.2) at 1 year and 85.5% (77.8-94.0) at 5 years after transplantation. Cumulative incidences of allograft loss were 10.5% (4.0-17.0) at 1 year and 13.0% (5.8-20.1) at 5 years after transplantation. Histologically proven AA amyloidosis recurrence occurred in 5 transplants (5.8%). An infection requiring hospitalization developed in 55.8% of cases, and there was a 27.9% incidence of acute allograft rejection. Multivariable analysis showed that C-reactive protein concentration at the time of transplantation was associated with patient survival (HR, 1.01; 95% CI, 1.00-1.02; P=0.01) and allograft survival (HR, 1.68; 95% CI, 1.10-2.57; P=0.02). The study lacked a control group, and the effect of biotherapies on transplantation outcomes could not be explored. This relatively contemporary cohort of patients who received a kidney transplant for AA amyloidosis experienced favorable rates of survival and lower recurrence rates than previously reported. These data support the practice of treating these patients with kidney transplantation for end-stage kidney disease. AA amyloidosis is a severe and rare disease. Kidney involvement is frequent and leads to end-stage kidney disease. Because of the involvement of other organs, these patients are often frail, which has raised concerns about their suitability for kidney transplantation. We reviewed all patients with AA amyloidosis nephropathy who underwent kidney transplantation in France in the recent era (2008-2018) and found that the outcomes after kidney transplantation were favorable, with 85.5% of patients still alive 5 years after transplantation, a survival rate that is comparable to the outcomes of patients receiving a transplant for other forms of kidney diseases. Recurrence of amyloidosis in the transplanted kidney was infrequent (5.8%). These data support the practice of kidney transplantation for patients with AA amyloidosis who experience kidney failure.

Polynesian Agency and the Establishment of the French Centre for Pacific Tests

ABSTRACT Since the 1960s, the installation of the Centre for Pacific Tests (Centre d’expérimentation du Pacifique or CEP) in French Polynesia has given rise to important debates concerning the agency of the populations in relationship to a decision on which they were not consulted. Until now, these debates have often focused on the reactions of the political class. In this article, we propose another approach to these issues by focusing on economic and social questions, with particular attention paid to the multiple strategies implemented by Polynesian workers. As this article demonstrates, while local actors had no say in the decision to set up the CEP in French Polynesia, the actual process of infrastructure construction, the interactions induced by these activities and the transformation of local power relations constituted opportunities for Islanders to develop their own strategies based on their interests and desires, their opinions, and the constraints that weighed unequally on them.

BOB-ACG study: Pulse methylprednisolone to prevent bilateral ophthalmologic damage in giant cell arteritis. A multicentre retrospective study with propensity score analysis

IntroductionGiant cell arteritis (GCA) is complicated in 10 to 20% of cases by permanent visual ischemia (PVI). International guidelines advocate the use of intravenous pulse of methylprednisolone from 250 to 1000mg per day, for three days, followed by oral prednisone at 1mg/kg per day. The aim of this study is to assess whether this strategy significantly reduces the risk of early PVI of the second eye, compared with direct prednisone at 1mg/kg per day. MethodsWe conducted a multicentre retrospective observational study over the past 15 years in 13 French hospital centres. Inclusion criteria included: new case of GCA; strictly unilateral PVI, prednisone at dose greater than or equal to 0.9mg/kg per day; for the intravenous methylprednisolone (IV-MP) group, total dose between 900 and 5000mg, close follow-up and knowledge of visual status at 1 month of treatment, or earlier, in case of contralateral PVI. The groups were compared on demographic, clinical, biological, iconographic, and therapeutic parameters. Statistical analysis was optimised using propensity scores. ResultsOne hundred and sixteen patients were included, 86 in the IV-MP group and 30 in the direct prednisone group. One patient in the direct prednisone group and 13 in the IV-MP group bilateralised, without significant difference between the two strategies (3.3% vs 15.1%). Investigation of the association between IV-MP patients and contralateral PVI through classical logistic regression, matching or stratification on propensity score did not show a significant association. Weighting on propensity score shows a significant association between IV-MP patients and contralateral PVI (OR=12.9 [3.4; 94.3]; P<0.001). Improvement in visual acuity of the initially affected eye was not significantly associated with IV-MP (visual acuity difference 0.02 vs –0.28 LogMar), even in the case of early management, i.e., within the first 48hours after the onset of PVI (n=61; visual acuity difference –0.11 vs 0.25 LogMar). Complications attributable to corticosteroid therapy in the first month were significantly more frequent in the IV-MP group (31.8 vs 10.7%; P<0.05). DiscussionOur data do not support the routine use of pulse IV-MP for GCA complicated by unilateral PVI to avoid bilateral ophthalmologic damage. It might be safer to not give pulse IV-MP to selected patients with high risks of glucocorticoids pulse side effects. A prospective randomised multicentre study comparing pulse IV-MP and prednisone at 1mg/kg per day is desirable.

Value of rare diseases reference centers: impact on diagnosis and access to specialized care in fibrous dysplasia of bone

IntroductionReference Centers and Rare Disease Health Networks aim to improve the management of patients with rare diseases. The French reference center for Fibrous Dysplasia was certified in 2006. ObjectiveThe objective of our study was to assess the effectiveness of our reference center since its constitution. MethodsIn a retrospective cohort study, we compared the activity of our center, including the time elapsed between access to the center and the diagnostic delay of patients with Fibrous Dysplasia between two periods, 1994–2006 (before certification) and 2007–2019 (after certification). Data were extracted from patients’ records (Easily®). Wilcoxon and Fisher tests were performed, using R®. ResultsOur cohort included 527 patients with Fibrous Dysplasia/Mc Cune Albright syndrome. The activity of the Fibrous Dysplasia center increased from 139 patients in the first period (1994–2006) to an additional 388 patients for the second period (2007–2019). Mean time elapsed to diagnosis of Fibrous Dysplasia was 1.5 years before 2007 and 1.9 years after 2007 (p = 0.12). Diagnosis was made before referral in over 80% of patients. There was a non-significant decrease in the number of patients with delayed diagnosis: 37 patients (44%) in the first period had a diagnostic delay and 94 patients (33%) in the second period (p = 0.07). Patients were referred to our center on average 6.8 years (before 2007) and 7.9 years (after 2007) after their diagnosis (p = 0.77). ConclusionHealthcare organization with reference centers significantly impacted the management of patients with Fibrous Dysplasia/Mc Cune Albright syndrome, with a substantial increase in the activity of our center, that roughly tripled since certification. This healthcare organization was also associated with a trend toward decreasing diagnostic delay. However, diagnostic delay affected more than a third of patients and the time to access to the center remained extended (≈7–8 years after diagnosis). The current challenge lies in informing primary care providers and patients about education to rare diseases and existence of reference centers for earlier and more effective specialized management.

First results of the Terumo Aortic stentgraft in a French center for the treatment of aneurysmal changes in aortic dissections

First results of the Terumo Aortic stentgraft in a French center for the treatment of aneurysmal changes in aortic dissections