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Background: Post-intensive care syndrome could be responsible for inability to receive proper cancer treatment after ICU stay in patients with solid tumors (ST). Our purpose was to determine the factors associated with cancer treatment resumption and the impact of cancer treatment on the outcome of patients with ST after ICU stay. Methods: We conducted a retrospective study including all patients with ST admitted to the ICU between 2014 and 2019 in a French University-affiliated Hospital. Results: A total of 219 patients were included. Median SAPS II at ICU admission was 44.0 [IQR 32.8, 66.3]. Among the 136 patients who survived the ICU stay, 81 (59.6%) received cancer treatment after ICU discharge. There was an important increase in patients with poor performance status (PS) of 3 or 4 after ICU stay (16.2% at admission vs. 44.5% of patients who survived), with significant PS decline following the ICU stay (median difference − 1.5, 95% confidence interval [-1.5-1.0], p < 0.001). The difference between the PS after and before ICU stay (delta PS) was independently associated with inability to receive cancer treatment (Odds ratio OR 0.34, 95%CI 0.18–0.56, p value < 0.001) and with 1-year mortality in patients who survived at ICU discharge (Hazard ratio HR 1.76, 95%CI 1.34–2.31, p value < 0.001). PS before ICU stay (OR 3.73, 95%IC 2.01–7.82, p value < 0.001) and length of stay (OR 1.23, 95%CI 1.06–1.49, p value 0.018) were independently associated with poor PS after ICU stay. Survival rates at ICU discharge, at 1 and 3 years were 62.3% (n = 136), 27.3% (n = 59) and 17.1% (n = 37), respectively. The median survival for patients who resumed cancer treatment after ICU stay was 771 days (95%CI 376–1058), compared to 29 days (95%CI 15–49) for those who did not resume treatment (p < 0.001). Conclusion: Delta PS, before and after ICU stay, stands out as a critical determinant of cancer treatment resumption and survival after ICU stay. Multidisciplinary intervention to improve the general condition of these patients, in ICU and after ICU stay, may improve access to cancer treatment and long-term survival. [ABSTRACT FROM AUTHOR]

Purpose: We aimed to assess risk factors of candida-related Vascular Graft Infections (VGIs). Methods: We did a case–control study (1:4) matched by age and year of infection, nested in a cohort of patient with a history of VGIs. Cases were defined by a positive culture for Candida spp. in biological samples and controls were defined by a positive culture for bacterial strains only in biological samples. Risk factors for Candida-related VGIs were investigated using multivariate logistic regression. Mortality were compared using survival analysis. Results: 16 Candida-related VGIs were matched to 64 bacterial-related VGIs. The two groups were comparable regarding medical history and clinical presentation. Candida-related VGIs were associated with bacterial strains in 88% (14/16). Gas/fluid-containing collection on abdominal CT scan and the presence of an aortic endoprosthesis were risk factors for Candidaspp.-related VGIs [RRa 10.43 [1.81–60.21] p = 0.009 RRa and 6.46 [1.17–35.73] p = 0.03, respectively]. Candida-related VGIs were associated with a higher mortality when compared to bacterial-related VGIs (p = 0.002). Conclusions: Candida-related VGIs are severe. Early markers of Candidaspp. infection are needed to improve their outcome. The suspicion of aortic endoprosthesis infection may necessitate probabilistic treatment with antifungal agents. [ABSTRACT FROM AUTHOR]

Background: Solid pseudopapillary neoplasms of the pancreas (SPNP) are rare tumors predominantly in young women. We report the largest single-center cohort study comparing resection of SPNP by laparoscopic approach (LA) and the open approach (OA). Method: Between 2001 and 2021, 102 patients (84% women, median age: 30) underwent pancreatectomy for SPNP and were retrospectively studied. Demographic, perioperative, pathological, early and the long-term results were evaluated between patients operated by LA and those by OA. Results: Population included 40 LA and 62 OA. There were no significant differences in demographics data between the groups. A preoperative biopsy by endoscopic ultrasound was performed in 45 patients (44%) with no difference between the groups. Pancreatoduodenectomy (PD) was less frequently performed by LA (25 vs 53%, p = 0.004) and distal pancreatectomy (DP) was more frequently performed by LA (40 vs 16%, p = 0.003). In the subgroup analysis by surgical procedure, LA-PD was associated with one mortality, less median blood loss (180 vs 200 ml, p = 0.034) and fewer harvested lymph nodes (11 vs 15, p = 0.02). LA-DP was associated with smaller median tumor size on imaging (40 vs 80mm, p = 0.048), shorter surgery (135 vs 190 min, p = 0.028), and fewer complications according to the median comprehensive complication index score (0 vs 8.7, p = 0.048). LA-Central pancreatectomy was associated with shorter surgery (160 vs 240, p = 0.037), less median blood loss (60 vs 200, p = 0.043), and less harvested lymph nodes (5 vs 2, p = 0.025). After a median follow-up of 60 months, two recurrences (2%) were observed and were unrelated to the approach. Conclusions: The LA for SPNP appears to be safe, should be applied cautiously in case of PD for large lesion, and was not associated with recurrence. [ABSTRACT FROM AUTHOR]

Objectives To describe the characteristics, treatment and outcome of isolated ANCA-associated scleritis at diagnosis compared with idiopathic scleritis with negative ANCA tests. Methods This retrospective multicentre case–control study was performed within the French Vasculitis Study Group (FVSG) network and in three French tertiary ophthalmologic centres. Data from patients with scleritis without any systemic manifestation and with positive ANCA results were compared with those of a control group of patients with idiopathic scleritis with negative ANCA tests. Results A total of 120 patients, including 38 patients with ANCA-associated scleritis and 82 control patients, diagnosed between January 2007 and April 2022 were included. The median follow-up was 28 months (IQR 10–60). The median age at diagnosis was 48 years (IQR 33–60) and 75% were females. Scleromalacia was more frequent in ANCA-positive patients (P  = 0.027) and 54% had associated ophthalmologic manifestations, without significant differences. ANCA-associated scleritis more frequently required systemic medications, including glucocorticoids (76% vs 34%; P  < 0.001), and rituximab (P  = 0.03) and had a lower remission rate after the first- and second-line treatment. Systemic ANCA-associated vasculitis (AAV) occurred in 30.7% of patients with PR3- or MPO-ANCA, after a median interval of 30 months (IQR 16.3–44). Increased CRP >5 mg/l at diagnosis was the only significant risk factor of progression to systemic AAV [adjusted hazard ratio 5.85 (95% CI 1.10, 31.01), P  = 0.038]. Conclusion Isolated ANCA-associated scleritis is mostly anterior scleritis with a higher risk of scleromalacia than ANCA-negative idiopathic scleritis and is more often difficult to treat. One-third of patients with PR3- or MPO-ANCA scleritis progressed to systemic AAV. [ABSTRACT FROM AUTHOR]

Data are scarce regarding epidemiology and management of critically ill patients with lung abscesses. What are the clinical and microbiological characteristics of critically ill patients with lung abscesses, how are they managed in the ICU, and what are the risk factors of in-ICU mortality? This was a retrospective observational multicenter study, based on International Classification of Diseases, 10th Revision, codes, between 2015 and 2022 in France. In-ICU mortality-associated factors were determined by multivariate logistic regression. We analyzed 171 ICU patients with pulmonary abscesses. Seventy-eight percent were male, with a mean age of 56.5 ± 16.4 years; 20.4% misused alcohol, 25.2% had a chronic lung disease (14% COPD), and 20.5% had a history of cancer. Overall, 40.9% were immunocompromised and 38% qualified for nosocomial infection. Presenting symptoms included fatigue or weight loss in 62%, fever (50.3%), and dyspnea (47.4%). Hemoptysis was reported in 21.7%. A polymicrobial infection was present in 35.6%. The most frequent pathogens were Enterobacteriaceae in 31%, Staphylococcus aureus in 22%, and Pseudomonas aeruginosa in 19.3%. Fungal infections were found in 10.5%. Several clusters of clinicoradiologic patterns were associated with specific microbiological documentation and could guide empiric antibiotic regimen. Percutaneous abscess drainage was performed in 11.7%; surgery was performed in 12.7%, and 12% required bronchial artery embolization for hemoptysis. In-ICU mortality was 21.5%, and age (OR: 1.05 [1.02-1.91], P =.007], renal replacement therapy during ICU stay (OR, 3.56 [1.24-10.57], P =.019), and fungal infection (OR, 9.12 [2.69-34.5], P =.0006) were independent predictors of mortality after multivariate logistic regression, and drainage or surgery were not. Pulmonary abscesses in the ICU are a rare but severe disease often resulting from a polymicrobial infection, with a high proportion of Enterobacteriaceae, S aureus , and P aeruginosa. Percutaneous drainage, surgery, or arterial embolization was required in more than one-third of cases. Further prospective studies focusing on first-line antimicrobial therapy and source control procedure are warranted to improve and standardize patient management. [Display omitted] [ABSTRACT FROM AUTHOR]

Background The use of myocardial reperfusion—mainly via angioplasty—has increased in our region to over 95%. We wondered whether old and very old patients have benefited from this development. Methods Setting: Greater Paris Area (Ile-de-France). Data Regional registry, prospective, including since 2003, data from 39 mobile intensive care units performing prehospital treatment of patients with ST segment elevation myocardial infarction (STEMI) (<24 h). Parameters Demographic, decision to perform reperfusion and outcome (in-hospital mortality). Primary endpoint Reperfusion decision rate by decade over age 70. Secondary endpoint Outcome. Results We analysed the prehospital management of 27,294 patients. There were 21,311 (78%) men and 5,919 (22%) women with a median age of 61 (52–73 years). Among these patients, 8,138 (30%) were > 70 years, 3,784 (14%) > 80 years and 672 (2%) > 90 years. The reperfusion decision rate was 94%. It decreased significantly with age: 93, 90 and 76% in patients in their seventh, eighth and ninth decade, respectively. The reperfusion decision rate increased significantly over time. It increased in all age groups, especially the higher ones. Mortality was 6%. It increased significantly with age: 8, 16 and 25% in patients in their seventh, eighth and ninth decade, respectively. It significantly decreased over time in all age groups. The odds ratio of the impact of reperfusion decision on mortality reached 0.42 (0.26–0.68) in patients over 90 years. Conclusion the increase in the reperfusion decision rate was the greatest in the oldest patients. It reduced mortality even in patients over 90 years of age. [ABSTRACT FROM AUTHOR]

Emergent stenting in tandem occlusions and mechanical thrombectomy (MT) of acute ischemic stroke related to large vessel occlusion (LVO-AIS) with a large core are tested independently. We aim to assess the impact of reperfusion with MT in patients with LVO-AIS with a large core and a tandem occlusion and to compare the safety of reperfusion between large core with tandem and nontandem occlusions in current practice. We analyzed data of all consecutive patients included in the prospective Endovascular Treatment in Ischemic Stroke Registry in France between January 2015 and March 2023 who presented with a pretreatment ASPECTS (Alberta Stroke Program Early CT Score) of 0–5 and angiographically proven tandem occlusion. The primary end point was a favorable outcome defined by a modified Rankin Scale (mRS) score of 0–3 at 90 days. Among 262 included patients with a tandem occlusion and ASPECTS 0–5, 203 patients (77.5%) had a successful reperfusion (modified Thrombolysis in Cerebral Infarction grade 2b-3). Reperfused patients had a favorable shift in the overall mRS score distribution (adjusted odds ratio [aOR], 1.57 [1.22–2.03]; P < 0.001), higher rates of mRS score 0–3 (aOR, 7.03 [2.60–19.01]; P < 0.001) and mRS score 0–2 at 90 days (aOR, 3.85 [1.39–10.68]; P = 0.009) compared with nonreperfused. There was a trend between the occurrence of successful reperfusion and a decreased rate of symptomatic intracranial hemorrhage (aOR, 0.5 [0.22–1.13]; P = 0.096). Similar safety outcomes were observed after large core reperfusion in tandem and nontandem occlusions. Successful reperfusion was associated with a higher rate of favorable outcome in large core LVO-AIS with a tandem occlusion, with a safety profile similar to nontandem occlusion. [ABSTRACT FROM AUTHOR]

Background: Both AIDS-defining and non-AIDS-defining cancers (ADC/NADC) predispose people living with HIV (PLHIV) to critical illnesses. The objective of this multicentre study was to investigate the prognostic impact of ADC and NADC in PLHIV admitted to the intensive care unit (ICU). Methods: All PLHIV admitted over the 2015–2020 period in 12 university-affiliated ICUs in France were included in the study cohort. The effect of ADC and NADC on in-hospital mortality (primary study endpoint) was measured through logistic regression with augmented backward elimination of potential independent variables. The association between ADC/NADC and treatment limitation decision (TLD) during the ICU stay (secondary study endpoint) was analysed. One-year mortality in patients discharged alive from the index hospital admission (exploratory study endpoint) was compared between those with ADC, NADC or no cancer. Results: Amongst the 939 included PLHIV (median age, 52 [43–59] years; combination antiretroviral therapy, 74.4%), 97 (10.3%) and 106 (11.3%) presented with an active NADC (mostly lung and intestinal neoplasms) and an active ADC (predominantly AIDS-defining non-Hodgkin lymphoma), respectively. Inaugural admissions were common. Bacterial sepsis and non-infectious neoplasm-related complications accounted for most of admissions in these subgroups. Hospital mortality was 12.4% in patients without cancer, 30.2% in ADC patients and 45.4% in NADC patients (P < 0.0001). NADC (adjusted odds ratio [aOR], 7.00; 95% confidence interval [CI], 4.07–12.05) and ADC (aOR, 3.11; 95% CI 1.76–5.51) were independently associated with in-hospital death after adjustment on severity and frailty markers. The prevalence of TLD was 8.0% in patients without cancer, 17.9% in ADC patients and 33.0% in NADC patients (P < 0.0001)—organ failures and non-neoplastic comorbidities were less often considered in patients with cancer. One-year mortality in survivors of the index hospital admission was 7.8% in patients without cancer, 17.0% in ADC patients and 33.3% in NADC patients (P < 0.0001). Conclusions: NADC and ADC are equally prevalent, stand as a leading argument for TLD, and strongly predict in-hospital death in the current population of PLHIV requiring ICU admission. [ABSTRACT FROM AUTHOR]

Background: Changes in the epidemiology of traumatic brain injury (TBI) in older patients have received attention, but limited data are available on the outcome of these patients after admission to intensive care units (ICUs). The aim of this study was to evaluate the outcomes of patients over 65 years of age who were admitted to an ICU for TBI. Methods: This was a multicentre, retrospective, observational study conducted from January 2013 to February 2019 in the surgical ICUs of 5 level 1 trauma centres in France. Patients aged ≥ 65 years who were hospitalized in the ICU for TBI with or without extracranial injuries were included. The main objective was to determine the risk factors for unfavourable neurological outcome at 3 months defined as an Extended Glasgow Outcome Scale (GOSE) score < 5. Results: Among the 349 intensive care patients analysed, the GOSE score at 3 months was ≤ 4 and ≥ 5 in 233 (67%) and 116 (33%) patients, respectively. The mortality rate at 3 months was 157/233 (67%), and only 7 patients (2%) fully recovered or had minor symptoms. Withdrawal or withholding of life-sustaining therapies in the ICU was identified in 140 patients (40.1%). Multivariate analysis showed that age (OR 1.09, CI 95% 1.04–1.14), male sex (OR 2.94, CI95% 1.70–5.11), baseline Glasgow Coma Scale score (OR 1.20, CI95% 1.13–1.29), injury severity score (ISS; OR 1.04, CI95% 1.02–1.06) and use of osmotherapy (OR 2.42, CI95% 1.26–4.65) were associated with unfavourable outcomes (AUC = 0.79, CI 95% [0.74–0.84]). According to multivariate analysis, the variables providing the best sensitivity and specificity were age ≥ 77 years, Glasgow Coma Scale score ≤ 9 and ISS ≥ 25 (AUC = 0.79, CI 95% [0.74–0.84]). Conclusions: Among intensive care patients aged ≥ 65 years suffering from TBI, age (≥ 77 years), male sex, baseline Glasgow coma scale score (≤ 9), ISS (≥ 25) and use of osmotherapy were predictors of unfavourable neurological outcome. Trial registration: ClinicalTrials.gov Identifier: NCT04651803. Registered 03/12/2020. Retrospectively registered. [ABSTRACT FROM AUTHOR]

Objectives To assess whether two cluster analysis-based axial SpA (axSpA) endotypes (A for purely axial; B for both axial and peripheral) are stable over time and are associated with different long-term disease outcomes. Methods K-means cluster analysis was performed at each visit (until 5 years) on 584 patients from the DESIR cohort, who completed all planned visits, and validated in 232 consecutive axSpA patients from the BeGiant cohort. Cluster stability overtime was assessed by kappa statistics. A generalized linear mixed-effect analysis was applied to compare outcomes between clusters. Classification and regression tree (CART) analysis was performed to determine a decision rule able to assign a given patient to a definite cluster at onset. Results Both endotypes remained remarkably stable over time. In the DESIR cohort, patients in cluster B showed higher disease activity, worse functional outcome and higher need for anti-rheumatic drugs than patients in cluster A. CART analysis yielded three main clinical features (arthritis, enthesitis and dactylitis) that accurately determined cluster assignment. These results could be replicated in the Be-GIANT cohort. Conclusion Cluster-based axSpA endotypes were reproducible in two different cohorts, stable over time and associated with different long-term outcome. The axSpA endotype with additional peripheral disease manifestations is associated with more severe disease and requires more intensive drug therapy Clinical trial registration clinicaltrials.gov, https://clinicaltrials.gov , NCT01648907. [ABSTRACT FROM AUTHOR]

Background: Opioid use disorder (OUD) poses a global health challenge, and despite medications for opioid use disorder (MOUD) and psychosocial interventions, relapse remains a significant concern. Comorbid psychiatric disorders, including attention deficit hyperactivity disorder (ADHD), are one of the major factors associated with poor OUD treatment outcome. We aimed to estimate the frequency of probable ADHD (in childhood and in adulthood) in patients with OUD; to assess the factors associated with this comorbidity; and to explore the factors that mediate the relationship between ADHD and OUD treatment outcome., Methods: We conducted an observational study using a sample of 229 patients aged 18 years and older who were diagnosed with OUD and had received MOUD for at least six months. Participants were assessed through a structured interview and self-report questionnaires. Multivariate logistic regressions and a mediation analysis were performed., Results: Almost half of the participants reported probable ADHD in childhood, and ADHD persisted into adulthood among two-thirds of the patients. The factors associated with poor OUD treatment outcome included earlier onset of OUD, lower education, and greater impulsivity. There was no direct effect of probable ADHD in childhood on OUD treatment outcome, but there was an indirect effect through negative urgency, the tendency to respond impulsively to negatively connoted emotional experiences., Conclusions: The findings suggest that ADHD symptoms, particularly impulsivity, may contribute to vulnerability in opioid use and play a crucial role in treatment outcomes for this population., Trial Registration: ClinicalTrials identifier NCT01847729., (© 2024. The Author(s).)

Objectives: There is little data on pregnant women with imported malaria in high-income countries, especially regarding offspring outcomes. We wanted to determine pregnancy outcomes of imported malaria in pregnant women in mainland France., Patients and Methods: We conducted a retrospective, descriptive study of outcomes in pregnant women hospitalized with malaria from 2004 to 2014 in two regions of mainland France. An adverse outcome was defined as a miscarriage, stillbirth, preterm birth (<35 weeks of gestation), low birth weight (LBW) defined as less than 2500 g, or congenital malaria., Results: Of 60 pregnancies, 5 were excluded because of elective abortions; 55 were investigated, of which 11 were primigravidae and 44 multigravidae. Pregnancies were singleton (n = 51) or twin (n = 4). Mean age was 30.4 years (range:19-45 y). Among the 55 cases, 9 ended in a miscarriage (8 singletons and 1 twin pregnancy) and 1 had a stillbirth at 21 weeks of gestation, all immediately after the malarial episode. 45 gave birth (29 vaginal deliveries and 16 caesarean sections) to 48 (42 singletons and 6 twins) newborns. Amongst these, 30 were healthy full-term newborns, 10 had LBW, and 8 were preterm. Overall, 26 of 55 (47.3%) pregnancies, and 29 of 59 (49.2%) offsprings had adverse outcomes. Compared to singleton pregnancies, twin pregnancies were associated with adverse outcomes (p = 0.0438)., Conclusions: Imported malaria has a severe impact on pregnancy outcomes. Prevention and management of imported malaria in pregnancy should be optimized., Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2024 The Authors. Published by Elsevier Ltd.. All rights reserved.)

Aims  In low-gradient aortic stenosis (LGAS), the high valvulo-arterial impedance observed despite low valvular gradient suggests a high vascular load. Thoracic aortic calcifications (TACs) and valvular aortic calcifications (VACs) are, respectively, surrogates of aortic load and aortic valvular gradient. The aim of this study was to compare the respective contributions of TAC and VAC on 3-year cardiovascular (CV) mortality following TAVI in LGAS vs. high-gradient aortic stenosis (HGAS) patients. Methods and results  A total of 1396 consecutive patients were included. TAC and VAC were measured on the pre-TAVI CT-scan. About 435 (31.2%) patients had LGAS and 961 (68.8%) HGAS. LGAS patients were more prone to have diabetes, coronary artery disease (CAD), atrial fibrillation (AF), and lower left ventricular ejection fraction (LVEF), P <0.05 for all. During the 3 years after TAVI, 245(17.8%) patients experienced CV mortality, 92(21.6%) in LGAS and 153(16.2%) in HGAS patients, P =0.018. Multivariate analysis adjusted for age, gender, diabetes, AF, CAD, LVEF, renal function, vascular access, and aortic regurgitation showed that TAC but not VAC was associated with CV mortality in LGAS, hazard ratio (HR) 1.085 confidence interval (CI) (1.019–1.156), P =0.011, and HR 0.713 CI (0.439–1.8), P =0.235; the opposite was observed in HGAS patients with VAC but not TAC being associated with CV mortality, HR 1.342 CI (1.034–1.742), P =0.027, and HR 1.015 CI (0.955–1.079), P =0.626. Conclusion  TAC plays a major prognostic role in LGAS while VAC remains the key in HGAS patients. This confirms that LGAS is a complex vascular and valvular disease. [ABSTRACT FROM AUTHOR]

Objective To report the 10-year outcome of an inception cohort of patients with early rheumatoid arthritis (RA), the ESPOIR cohort, and predictors of outcome. Methods From 2003 to 2005, 813 patients were included if they had early arthritis (<6 months) with a high probability of RA and had never been prescribed DMARDs. Multivariate analysis was used to evaluate predictors of outcome. Results In total, 521 (64.1%) RA patients were followed up for 10 years; 35 (4.3%) died, which appears to be similar to the French general population. Overall, 480 (92.1%) patients received a DMARD; 174 (33.4%) received at least one biologic DMARD, 13.6% within 2 years. At year 10, 273 (52.4%) patients were in DAS28 remission, 40.1% in sustained remission, 14.1% in drug-free remission, 39.7% in CDAI remission. Half of the patients achieved a health assessment questionnaire-disability index (HAQ-DI) < 0.5. SF-36 physical component and pain were well controlled. Structural progression was weak, with a mean change from baseline in modified Sharp score of 11.0  (17.9). Only 34 (6.5%) patients required major joint surgery. A substantial number of patients showed new comorbidities over 10 years. Positivity for anti-citrullinated peptides antibodies (ACPA) was confirmed as a robust predictor of long-term outcome. Conclusions We report a very mild 10-year outcome of a large cohort of patients with early RA diagnosed in the early 2000s, which was much better than results for a previous cohort of patients who were recruited in 1993. This current favourable outcome may be related to more intensive care for real-life patients. [ABSTRACT FROM AUTHOR]

Background and Purpose: Patients with suspected stroke are referred to the nearest hospital and are managed either in a spoke center (SC), a primary stroke center (PSC), or a comprehensive stroke center (CSC) in order to benefit from early intravenous thrombolysis (IVT). In case of large vessel occlusion (LVO), mechanical thrombectomy (MT) is only performed in the CSC, whereas the effectiveness of MT is highly time-dependent. There is a debate about the best management model of patients with suspected LVO. Therefore, we aimed to compare functional and safety outcomes of LVO patients eligible for MT managed through our regional telestroke system., Method: We performed a retrospective analysis of our observational prospective clinical registry in all consecutive subjects with LVO within six hours of onset who were admitted to the SC, PSC, or CSC in the east of France between October 2017 and November 2022. The primary endpoint was the functional independence defined as modified Rankin scale (mRS) score 0 to 2 at 90 days. Secondary endpoints were functional outcome, early neurological improvement, symptomatic intracranial hemorrhage and 90-day mortality., Results: Among the 794 included patients with LVO who underwent MT, 122 (15.4%) were managed by a SC, 403 (50.8%) were first admitted to a PSC, and 269 (33.9%) were first admitted to the CSC. The overall median NIHSS and ASPECTS score were 16 and 8, respectively. Multivariate analysis did not find any significant difference for the primary endpoint between patients managed by PSC versus CSC (OR 1.06 [95% CI 0.64;1.76], P=0.82) and between patient managed by SC versus CSC (OR 0.69 [0.34;1.40], P=0.30). No difference between the three groups was found except for the parenchymal hematoma rate between PSC and CSC (15.7 versus 7.4%, OR 2.25 [1.07;4.74], P=0.032)., Conclusions: Compared with a first admission to a CSC, the clinical outcomes of stroke patients with LVO eligible for MT first admitted to a SC or a PSC are similar., (Copyright © 2023 Elsevier Masson SAS. All rights reserved.)

Background: Transcatheter (TAVR) has supplanted surgical (SAVR) aortic valve replacement (AVR)., Aim: To evaluate whether adoption of this technology has varied according to centre volume at the nationwide level., Methods: From an administrative hospital-discharge database, we collected data on all AVRs performed in France between 2007 and 2019. Centres were divided into terciles based on the annual number of SAVRs performed in 2007-2009 ("before TAVR era")., Results: A total of 192,773 AVRs (134,662 SAVRs and 58,111 TAVRs) were performed in 47 centres. The annual number of AVRs and TAVRs increased significantly and linearly in low-volume (<152 SAVRs/year; median 106, interquartile range [IQR] 75-129), middle-volume (152-219 SAVRs/year; median 197, IQR 172-212) and high-volume (>219 SAVRs/year; median 303, IQR 268-513) terciles, but to a greater degree in the latter (+14, +16 and +24 AVRs/centre/year and +16, +19 and +31 TAVRs/centre/year, respectively; P ANCOVA <0.001). Charlson Comorbidity Index and in-hospital death rates declined from 2010 to 2019 in all terciles (all P trend <0.05). In 2017-2019, after adjusting for age, sex and Charlson Comorbidity Index, there was a trend toward lower death rates in the high-volume tercile (P=0.06) for SAVR, whereas death rates were similar for TAVR irrespective of tercile (P=0.27). Similar results were obtained when terciles were defined based on number of interventions performed in the last instead of the first 3years. Importantly, even centres in the lowest-volume tercile performed a relatively high number of interventions (150 TAVRs/year/centre)., Conclusions: In a centralized public healthcare system, the total number of AVRs increased linearly between 2007 and 2019, mostly due to an increase in TAVR, irrespective of centre volume. Progressive declines in patient risk profiles and death rates were observed in all terciles; in 2017-2019 death rates were similar in all terciles, although lower in high-volume centres for SAVR., (Copyright © 2024 The Author(s). Published by Elsevier Masson SAS.. All rights reserved.)

Background the ongoing growing and ageing population is associated with an increase in older patients suffering a stroke. We aimed to assess the current profile of these patients in a population-based setting. Methods all patients with acute stroke were prospectively identified among residents of Dijon, France, between 2013 and 2017, using a population-based registry. Characteristics and early outcome of patients were compared according to age groups. Results 1,288 stroke cases were recorded (median age: 81.1 years, interquartile range: 66.1–86.7, 54% women). Patients aged 75–85 years and those >85 years accounted for 27.6 and 33.9% of overall cases. Increasing age was associated with a greater prevalence of vascular risk factors, pre-existing cognitive impairment and handicap, higher initial severity, more frequent cardioembolic ischemic stroke, post-stroke pulmonary infection and delirium. Only 41% of patients aged 75–85 years and 18% of those aged >85 years had a good early recovery. Compared with patients aged <75 years, patients aged 75–85 years [adjusted odds ratio (OR) = 2.61; 95% confidence interval (CI): 1.74–3.93, P  < 0.001] and those aged >85 years (adjusted OR = 7.18; 95% CI: 4.58–11.3, P  < 0.001) had an increased risk of poor post-stroke functional outcome. Among survivors, the proportion of patients discharged to home was 60% in age group <75 years, compared with 49% in patients aged 75–85 years and 29% in those aged >85 years. Thirty per cent of patients >85 years old required a long-term care institution. Conclusion the increasing burden of stroke in older people has major implications for future treatment strategies and need for dedicated care facilities. [ABSTRACT FROM AUTHOR]

Background: Few data are available on patients who have experienced anaphylaxis and were admitted to ICUs. The purpose of this observational study was to describe the epidemiology and management of these patients.Methods: This was a multicentre retrospective study carried out in 23 French ICUs between 2012 and 2017. All patients who suffered anaphylaxis and were transferred to an ICU were included. Data were collected using an electronic database after approval by an ethics committee.Results: A total of 339 patients were included, and 17 (5%) died secondary to anaphylaxis. The main triggers were drugs (77%), contrast media (11%), and food (7%). Epinephrine was administered before ICU admission in 88% of patients with Grade III anaphylaxis and 100% of patients with Grade IV anaphylaxis. Most patients with Grades III and IV anaphylaxes did not receive the recommended dose of i.v. fluid of 30 ml kg-1 within the first 4 h of ICU admission. The time to epinephrine administration was not statistically different between survivors and non-survivors, but non-survivors received a higher dose of epinephrine (median: 5 [3-10] vs 3 [2-7] mg; P<0.0001), which suggests that some forms of anaphylactic shock may be resistant to epinephrine. In multivariate analysis, only lactate concentration at ICU admission was a predictor of death (odds ratio: 1.47 [1.15-1.88]; P=0.002).Conclusions: Lactate concentration at ICU admission appeared to be the most reliable criterion for assessing prognosis. Epinephrine is widely used during anaphylaxis, but the volume of fluid resuscitation was consistently lower than recommended.Clinical Trial Registration: NCT04290507. [ABSTRACT FROM AUTHOR]

Background: Critically ill patients with systemic rheumatic disease (SRD) have benefited from better provision of rheumatic and critical care in recent years. Recent comprehensive data regarding in-hospital mortality rates and, most importantly, long-term outcomes are scarce.Research Question: The aim of this study was to assess short and long-term outcome of patients with SRD who were admitted to the ICU.Study Design and Methods: All records of patients with SRD who were admitted to ICU between 2006 and 2016 were reviewed. In-hospital and one-year mortality rates were assessed, and predictive factors of death were identified.Results: A total of 525 patients with SRD were included. Causes of admission were most frequently shock (40.8%) and acute respiratory failure (31.8%). Main diagnoses were infection (39%) and SRD flare-up (35%). In-hospital and one-year mortality rates were 30.5% and 37.7%, respectively. Predictive factors that were associated with in-hospital and one-year mortalities were, respectively, age, prior corticosteroid therapy, simplified acute physiology score II ≥50, need for invasive mechanical ventilation, or need for renal replacement therapy. Knaus scale C or D and prior conventional disease modifying antirheumatic drug therapy was associated independently with death one-year after ICU admission.Interpretation: Critically ill patients with SRD had a fair outcome after an ICU stay. Increased age, prior corticosteroid therapy, and severity of critical illness were associated significantly with short- and long-term mortality rates. The one-year mortality rate was also associated with prior health status and conventional disease modifying antirheumatic drug therapy. [ABSTRACT FROM AUTHOR]

Purpose: This pilot study describes the socio-professional development and quality of life of young adults with Asperger syndrome in France. Methods: Those young adults, between 18 and 30 years old, receiving care in a child psychiatry department for autistic spectrum disorder, were requested to respond to the Ad Hoc, World Health Organization Quality Of Life – Bref and Copenhagen psychosocial questionnaires regarding their socio-professional background and feelings about their future. Results: Of the 79 eligible subjects, 24 were selected to participate in our study. Their average age at the time of the pilot study was 22.2 years (standard deviation 3.4 years), and their average age when they were diagnosed was 17.5 years (standard deviation 3.7 years.). There were 54% who reported a psychiatric comorbidity anxiety disorder. Half stated they had completed secondary school and benefitted from being professionally employed. During this study, only six were employed, while the others remained financially dependent on their parents. The group's quality of life self-assessment scores were significantly lower compared to the French general population in overall psychology (43.6 versus 68.7) and social relationships (48.9 versus 76.5). However, the study's participants perceived work as an important means to their personal development. Hence, in order to cope with their difficulties, they hoped to benefit from customized support adapted to their autistic disorder and for their workplace colleagues to be better informed about Asperger syndrome. Conclusions: Our results are in line with international data. Additional studies need to be done in order to determine socio-professional integration factors and, in particular, the integration of potential contributions by occupational health departments with those social and medical teams supporting these young adults. Young adults with Asperger syndrome benefit from the support of their family in determining their professional goals. Support may be required to enhance social and communicative abilities to help integration. Employees would benefit from information on the syndrome and how best to support. [ABSTRACT FROM AUTHOR]

Background: Laparoscopic right hemicolectomy (LRHC) is increasingly performed for the treatment of right colon disease. Nevertheless, standardization of the surgical technique regarding the performance of intracorporeal (IC) or extracorporeal (EC) anastomosis is lacking. The purpose of this study was to compare short-term postoperative outcomes in patients who had laparoscopic right colectomy either with IC or EC. Methods: This was a retrospective, non-randomized and multicenter study conducted from January 2005 to December 2015 on the CLIMHET study group cohort from five tertiary centers in France. Data were collected for all patients with LRHC to compare patient characteristics, intraoperative data and postoperative outcomes in terms of medical and surgical complications, duration of hospitalization and mortality. A multivariate analysis was performed to compare the results in the two groups. Results: Of the 597 patients undergoing LRHC, 150 had IC and 447 had EC. The incidence of medical complications (cardiac, vascular, and pulmonary complications) was lower in the IC group than in the EC group (13 vs 20%, p = 0.049). This difference remained significant in multivariate analysis after adjusting to field characteristics and patient histories (p = 0.009). Additionally, a shorter hospital stay (7 vs 8 days, p = 0.003) was observed in the IC group as compared to the EC group. This difference remained significant in favor of the IC group in multivariate analysis (p = 0.029). There was no difference between the groups as regards: surgical complications (p = 0.76), time of mobilization (p = 0.93), reintervention rate (p = 1) and 90-day mortality (p = 0.47). Conclusions: Our results show that IC anastomosis in LRHC is associated with fewer medical complications and shorter hospital stays compared to EC anastomosis. [ABSTRACT FROM AUTHOR]

Background: Cancer patients have been reported to be at higher risk of COVID-19 complications and deaths. We report the characteristics and outcome of patients diagnosed with COVID-19 during breast cancer treatment at Institut Curie hospitals (ICH, Paris area, France).Methods: An IRB-approved prospective registry was set up at ICH on March 13, 2020, for all breast cancer patients with COVID-19 symptoms or radiologic signs. Registered data included patient history, tumor characteristics and treatments, COVID-19 symptoms, radiological features, and outcome. Data extraction was done on April 25, 2020. COVID-19 patients were defined as those with either a positive RNA test or typical, newly appeared lung CT scan abnormalities.Results: Among 15,600 patients actively treated for early or metastatic breast cancer during the last 4 months at ICH, 76 patients with suspected COVID-19 infection were included in the registry and followed. Fifty-nine of these patients were diagnosed with COVID-19 based on viral RNA testing (N = 41) or typical radiologic signs: 37/59 (63%) COVID-19 patients were treated for metastatic breast cancer, and 13/59 (22%) of them were taking corticosteroids daily. Common clinical features mostly consisted of fever and/or cough, while ground-glass opacities were the most common radiologic sign at diagnosis. We found no association between prior radiation therapy fields or extent of radiation therapy sequelae and extent of COVID-19 lung lesions. Twenty-eight of these 59 patients (47%) were hospitalized, and 6 (10%) were transferred to an intensive care unit. At the time of analysis, 45/59 (76%) patients were recovering or had been cured, 10/59 (17%) were still followed, and 4/59 (7%) had died from COVID-19. All 4 patients who died had significant non-cancer comorbidities. In univariate analysis, hypertension and age (> 70) were the two factors associated with a higher risk of intensive care unit admission and/or death.Conclusions: This prospective registry analysis suggests that the COVID-19 mortality rate in breast cancer patients depends more on comorbidities than prior radiation therapy or current anti-cancer treatment. Special attention must be paid to comorbidities when estimating the risk of severe COVID-19 in breast cancer patients. [ABSTRACT FROM AUTHOR]

Introduction: Awake brain surgery (ABS) represents a rare surgical procedure in children as age and psychological aspects, which are considered to interfere with its feasibility and psychological outcome and limit its application. Only few pediatric case series have been reported so far, indicating a more complex translation of this surgical approach to children. However, the advances in neuropsychological testing and monitoring may have a substantial impact on ameliorating the eligibility of children undergoing awake procedures. This study addresses the condition of ABS in a pediatric cohort, focusing on its practicability and diversified outcome aspects. Methods: We performed a retrospective review and prospective outcome analysis of pediatric patients with CNS lesions undergoing ABS between 2005 and 2018, completed at the University of Lyon, France. Results: Eighteen children were considered for ABS with respect to the eloquent location of their CNS lesions documented in their pre-operative MRI. Seventeen of them underwent asleep–awake–asleep brain surgery. The cohort included 5 males and 12 females. The median age at surgery was 14.8 years, (range 9.4 to 17.6 years). Intraoperative testing included electrocortical stimulation while pursuing speech or motor activity. Most of the lesions were intrinsic tumors of glial origin. A complete tumor removal was achieved in 11 patients (65%). Post-operative neurological deficits were transiently observed in 2 patients, whereas severe psychological reactions occurred in 1 child. Persistent attention deficits were found in 2 patients. One patient experienced an infectious complication requiring antibiotic treatment. Two patients died during follow-up due to tumor progression. The mean duration of follow up was 22.2 months (range 3.4 to 46.8 months). Conclusions: ABS was shown to be beneficial in terms of efficient tumor resection besides simultaneous preservation of neurological functions. Psychological preparation of the families and the children is essential to increase the number and age range of patients, who can benefit from this technique. Neuropsychological testing before and after surgery is essential to determine cognitive outcome, which can be altered in a minority of patients. [ABSTRACT FROM AUTHOR]

Background: Decompressive craniectomy (DC) has been shown to be an effective treatment for malignant cerebral infarction (MCI). There are limited nationwide studies evaluating outcome after craniectomy for MCI.Objective: To describe the evolution in DC practices for MCI, long-term survival, and associated prognostic factors.Methods: We searched the French medico-administrative national database to retrieve patients who underwent DC between 2008 and 2017.Results: A total of 1841 cases of DC were performed over 10 years in 51 centers. Mean age at procedure was 50.9 years, 18% were above 60 years, and 64.4% were male. There was a significant increase in DC for MCI over the 10 years (p < 0.001), and the annual volume of procedures more than doubled (95/year vs. 243/year). Early survival at one week and one month was 86%, 95%CI (84.5, 87.6) and 79.7%, 95%CI (77.8, 81.5), respectively. Long-term survival at 1 and 5 years were 73.6%, 95%CI (71.6, 75.7) and 68.9%, 95%CI (66.5, 71.4), respectively. Patients below 60 years at the time of DC (HR = 0.5; 95%CI [0.4, 0.7], p < 0.001), DC being performed in a center with a high surgical activity (HR = 0.8; 95%CI [0.6, 0.9], p = 0.002), and the patients having unimpaired consciousness (HR = 0.6; 95%CI [0.5, 0.8], p < 0.001) were associated with increased survival in both univariate and adjusted Cox regressions. 18.7% of the survivors had a cranioplasty inserted within 3 months and 57.8% within 6 months. The probability of having a cranioplasty at one year was 75.6%, 95%CI (77.9, 73.1).Conclusion: Over the past 10 years in France, DC has been increasingly performed for MCI regardless of age. However, in-hospital mortality remains considerable, as about one quarter of patients died within the first weeks. For those who survive beyond 6 months, the risk of death significantly decreases. Early mortality is especially high for comatose patients above 60 years operated in inexperienced centers. Most of those who remain in good functional status tend to undergo a cranioplasty within the year following DC. [ABSTRACT FROM AUTHOR]

Background: In France, the incidence of severe imported malaria cases increased since early 2000. Artesunate was available (temporarily use authorization) since mid-2011 in France and commonly used for severe malaria since early 2013. Thus, the study objectives were to describe the patients with severe imported malaria admitted in intensive care unit (ICU) and assess the changes in clinical presentation and outcomes before and after this date. Methods: Retrospective observational single-center study in the infectious diseases ICU of a referral university hospital, conducted on patients admitted for severe imported malaria from 2004 to 2017. Demographic variables, severity scores, WHO's severity criteria on admission, treatment, and ICU and hospital lengths of stay were collected. Patients' characteristics and outcomes were compared between both periods. A poor outcome was defined as the composite endpoint of death, or requirement for vasopressors, invasive mechanical ventilation and/or renal replacement therapy. Results: 189 patients were included, 98 in 2004–2012 and 91 in 2013–2017, most often from West and Central African countries (96%). The number of WHO criteria for severe malaria was comparable in both groups, but SAPS II, SOFA and ICU length of stay were significantly higher in 2004–2012, while patients of African origin living in France were less frequent (p < 0.01). The outcome was poor for 41/98 cases in 2004–2012 and 12/91 cases in 2013–2017 (p < 0.01). The risk factors of poor outcome on the multivariate logistic regression were a neurological failure (adjusted odds ratio (adjOR = 3.23; 95% CI (1.03–10.08), p = 0.004), cardio-circulatory failure (adjOR = 9.92; 95% CI (2.34–42), p = <0.01) and creatinine blood levels > 265 µmol/L (adjOR = 10.76; 95% CI (3.17–36.53), p < 0.01). In the multivariate analysis, IV artesunate was not associated with a better outcome. Patients of African origin did not seem to have a better outcome than Caucasian patients or those from other origins (adjOR = 0.59; 95% CI (0.21–1.65), p = 0.31). Conclusion: Patients with imported malaria admitted in ICU in 2013–2017 were less severely ill than those in 2004–2012. These trends could be partially explained by the increasing proportion of African patients visiting friends or relatives or living in endemic areas. [ABSTRACT FROM AUTHOR]

Open in new tab Download slide Open in new tab Download slide OBJECTIVES The transcarotid (TC) approach for transcatheter aortic valve implantation (TAVI) is potentially an optimal alternative to the transfemoral (TF) approach. Our goal was to compare the safety and efficacy of TC- and TF-TAVI. METHODS Patients who underwent TF-TAVI or TC-TAVI in the prospectively collected FRANCE TAVI registry between January 2013 and December 2015 were compared. Propensity score inverse probability weighting methods were employed to minimize the impact of bias related to non-random treatment assignment. RESULTS Of the 11 033 patients included in the current study, 10 598 (96%) underwent a TF-TAVI and 435 (4.1%) had a TC-TAVI. Patients in the TC-TAVI access group presented with a higher risk profile but were significantly younger. There were no differences in the perioperative and 2-year mortality rates after adjustment [odds ratio (OR) 1.02, 95% confidence interval (CI) 0.62–1.68; P  = 0.99 and hazard ratio 1.03, 95% CI 0.7–1.35; P  = 0.83). TC-TAVI was associated with a significant risk of stroke (OR 2.42, 95% CI 2.01–2.92; P  < 0.001), ST-elevation myocardial infarction (OR 7.32, 95% CI 3.87–13.87; P  < 0.001), infections (OR 2.36, 95% CI 2.04–2.71; P  < 0.001), bleeding (OR 2.01, 95% CI 1.76–2.29; P  < 0.001), renal failure (OR 2.23, 95% CI 1.90–2.60; P  < 0.001) and need for dialysis (OR 2.36, 95% CI 2.01–2.76, P  < 0.001). Conversely, TC-TAVI was not confirmed as a risk factor for pacemaker implantation after adjustment (OR 1.05, 95% CI 0.96–1.15; P  < 0.28) and was a protective factor for vascular complications (OR 0.37, 95% CI 0.32–0.43; P  < 0.001). CONCLUSIONS TC-TAVI is a safe procedure compared to TF-TAVI, although it holds an increased risk of perioperative complications. It should be considered in case of non-femoral peripheral access as the second access choice, to increase the overall safety of TAVI procedures. [ABSTRACT FROM AUTHOR]

Purpose: To report the incidence, risk factors, clinical presentation, and outcome predictors of severe leptospirosis requiring intensive care unit (ICU) admission in a temperate zone.Methods: LEPTOREA was a retrospective multicentre study conducted in 79 ICUs in metropolitan France. Consecutive adults admitted to the ICU for proven severe leptospirosis from January 2012 to September 2016 were included. Multiple correspondence analysis (MCA) and hierarchical classification on principal components (HCPC) were performed to distinguish different clinical phenotypes.Results: The 160 included patients (0.04% of all ICU admissions) had median values of 54 years [38-65] for age, 40 [28-58] for the SAPSII, and 11 [8-14] for the SOFA score. Hospital mortality was 9% and was associated with older age; worse SOFA score and early need for endotracheal ventilation and/or renal replacement therapy; chronic alcohol abuse and worse hepatic dysfunction; confusion; and higher leucocyte count. Four phenotypes were identified: moderately severe leptospirosis (n = 34, 21%) with less organ failure and better outcomes; hepato-renal leptospirosis (n = 101, 63%) with prominent liver and kidney dysfunction; neurological leptospirosis (n = 8, 5%) with the most severe organ failures and highest mortality; and respiratory leptospirosis (n = 17, 11%) with pulmonary haemorrhage. The main risk factors for leptospirosis contamination were contact with animals, contact with river or lake water, and specific occupations.Conclusions: Severe leptospirosis was an uncommon reason for ICU admission in metropolitan France and carried a lower mortality rate than expected based on the high severity and organ-failure scores. The identification in our population of several clinical presentations may help clinicians establish an appropriate index of suspicion for severe leptospirosis. [ABSTRACT FROM AUTHOR]

Introduction: Guillain-Barré syndrome (GBS) is an inflammatory polyradiculoneuritis. Our aim in this study was to describe the clinical characteristics and the long-term sequelae of GBS in a French pediatric population.Methods: In this multicenter, retrospective study we evaluated clinical signs, radiological examinations, laboratory tests, treatments, and outcomes.Results: One hundred ten children were included in this investigation. These children presented with walking difficulties, muscle weakness, and cranial nerve impairment. Electrodiagnostic testing revealed 70% with acute inflammatory demyelinating polyradiculoneuropathy (AIDP) and 16% with acute motor axonal neuropathy (AMAN). One hundred children received immunoglobulins. At follow-up, 77% were cured, whereas 9% had sequelae, associated with an axonal form (P < .01) and a short interval between symptom onset and hospitalization (P < .01). The need for intubation was correlated with peripheral facial paralysis (P < .01) and dysautonomia (P < .01).Discussion: Although AIDP and AMAN present in a similar way, the axonal form is associated with a worse outcome. [ABSTRACT FROM AUTHOR]

Background: A high perioperative blood lactate level has been reported to be associated with poor outcomes after cardiac surgery. More than isolated peaks of lactate values, it should be more interesting to take into account changes in intraoperative blood lactate level (∆Lact). This large-scale retrospective study evaluated the relationship between ∆Lact and overall intensive care unit morbidity and 30-day all-cause mortality. Methods: Perioperative data from consecutive patients undergoing on-pump cardiac surgery between September 2010 and June 2016 were retrospectively analysed through our institutional database including clinical, transfusion and laboratory test results implemented prospectively by physicians. Blood lactate levels were initially measured after induction of anaesthesia (baseline) and periodically during the surgery. The ∆Lact was defined as the difference between the highest intraoperative blood lactate and the baseline lactate level and offered the opportunity to stratify patients into four subgroups: ⩽0, 0.1-0.9, 1-1.9 and ⩾2 mmol L−1. Results: From the 7,795 patients found eligible during the study period, 7,447 patients were analysed. The median ∆Lact of our patients was 0.6 (0.3-1) mmol L−1. Most of the studied patients (65.9%) exhibited a ∆Lact between 0.1 and 0.9 mmol L−1. A concentration-dependent relationship was observed between ∆Lact and intensive care unit morbidity and 30-day mortality. After adjustment for co-variables, all ∆Lact > 0 was associated with an increase in overall intensive care unit morbidity. An independent relationship was also found between ∆Lact and 30-day mortality as of a 1 mmol L−1 increase. Conclusion: Our results suggest that ∆Lact is associated with poor short-term outcomes in adult cardiac surgical patients. [ABSTRACT FROM AUTHOR]

To analyze the impact of chronic kidney disease (CKD) on major clinical outcome in SLE by using a nationwide database. Characteristics of all admitted SLE patients experiencing CKD (eGFR <60 mL/min/1.73 m2) in France from 2009 to 2015 were analyzed through the French medico- administrative database. Factors associated with CKD and major clinical outcomes such as end-stage renal disease (ESRD), cardiovascular event (CVE), septic shock and death were assessed. We used a multivariate Cox proportional hazard model and subdistribution hazard models to analyze survival without major clinical events according to the presence of CKD. From 2009 to 2015, 26,320 SLE patients were hospitalized in France. Among them, 6439 (86.5% women; mean age 45.7 [16.5] years old) had a baseline stay in 2009 during which CKD was reported in 428 (6.7%) cases. Multivariate analysis showed that lupus nephritis (OR 6.6 [5.2–8.4]), high blood pressure (OR 3.5 [2.8–4.5]), septic shock (OR 3.2 [1.7–6.0]) and past cardiovascular history (OR 1.4 [1.0–2.0]) were associated with CKD status. From 2009 to 2015, ESRD, CVE, septic shock, and death occurred in 4.0%, 14.4%, 6.3% and 9.6% of the 6439 SLE patients. CKD at baseline was independently and strongly associated with the occurrence of ESRD (sdHR 15.9 [11.6–21.9]), CVE (sdHR 1.7 [1.4–2.2]), septic shock (sdHR 2.1 [1.5–2.8]) and death (HR 1.7 [1.3–2.2]) during the follow up. CKD is a major risk factor for overall morbidity and mortality in SLE patients, highlighting the need for early pre-CKD lupus nephritis diagnosis and treatment. • Nationwide database analysis of major outcomes in SLE was performed. • CKD is a major risk factor for overall morbidity and mortality in SLE. • Early pre-CKD lupus nephritis diagnosis and treatment is mandatory. [ABSTRACT FROM AUTHOR]

Objectives: We aimed to investigate center effects on hospital mortality of ICU patients with hematologic malignancies and to explore interactions between center and patients characteristics.Design: Multicenter prospective cohort.Setting: Seventeen ICUs across France and Belgium.Patients: One-thousand eleven patients with hematologic malignancies hospitalized in ICUs.Interventions: Reanalysis of the original data using state-of-the-art statistical methods with permutation procedures for testing multiple random effects.Measurements and Main Results: Average crude mortality was 39% and varied from 11% to 58% across centers. There was a significant center effect on the mean hospital mortality, after adjustment on individual prognostic factors (p < 0.001; median adjusted odds ratio for center effect 1.57 [interquartile range, 1.24-2.18]). There was also a quantitative interaction between center and the effect of the Sequential Organ Failure Assessment score: higher scores were associated with higher mortality (odds ratio for 1 point = 1.24 on average; 95% CI, 1.15-1.33) but with a magnitude that depended on center (p = 0.028).Conclusions: Between-center heterogeneity in hospital mortality was confirmed after adjustment for individual prognostic factors. It was partially explained by center experience in treating oncology patients. Interestingly, center effect was similar in magnitude to that of known mortality risk factors. [ABSTRACT FROM AUTHOR]

Abstract Over the last 20 years, numerous studies have fairly consistently reported an improvement in the prognosis of patients with severe trauma after the establishment of a trauma network. These systems can be either exclusive, in which all patients are referred only to a small number of specifically designated centres that meet strict criteria, or inclusive, in which patients may be referred to any hospital of a particular area according to capacity, which is observed in France. Hospitals are classified (level 1 to level 3) according to their technical facilities and the number of patients admitted for severe trauma, knowing that studies have also shown an improvement of the outcome for the most severely injured patients (haemorrhagic shock, severe head trauma), in hospitals with the greatest technical facilities and the most important activity. The triage of the patients to a suitable centre must be done after careful prehospital evaluation, which is made on clinical criteria (mechanism, injury, medical history), measurement of vital signs, calculation of scores (RTS, MGAP) or based on classifications. According to this assessment, the patients will then be triaged to a centre that has the capacity for the optimal and definitive management of these injuries. The goal is then to avoid under triage which is synonymous of retransfer, loss of time, and probably also prognosis worsening, and to avoid over triage that may induce an inadequate use of resources, activity overload and cost increase. Thus, it seems essential to develop trauma networks to improve mortality and morbidity of patients that undergone a severe injury. These trauma networks will then have to be evaluated and a register set up. [ABSTRACT FROM AUTHOR]

Background: Calciphylaxis (CPX) is a rare and life-threatening disease characterized by vascular calcification and development of painful and necrotizing skin lesions with a challenging management. Mechanisms of CPX are complex and include an imbalance between vascular calcification promoters and inhibitors, and frequently vitamin K deficiency.Objectives: To describe the various presentations and identify predictive factors of death in patients with CPX.Methods: In this multicenter retrospective study, we included 71 CPX patients followed in South-West France (n = 26) and in French Polynesia (n = 45), and who all received sodium thiosulfate (25 g thrice weekly for a median of 61 days).Results: Characteristics at presentation significantly differed between metropolitan and Polynesian French patients. Polynesians were less frequently on regular dialysis at the onset of CPX, had a higher incidence of diabetes mellitus and obesity, more disturbances of calcium-phosphorus metabolism, and received vitamin K antagonists less frequently than patients from South-West France. Despite intensive management, the 1-year mortality rate was 66% and median time to death was 200 days (IQR, 40; 514). The number of body areas involved (i.e., three: OR 2.70 [1.09; 6.65], p = 0.031; four: OR 8.79 [1.54; 50.29], p = 0.015) was the only predictive factor for death, whereas application of topical cerium nitrate-silver sulfadiazine was protective (OR 0.44 [0.20; 0.99], p = 0.046). Surgical debridement, hyperbaric oxygenation therapy, and geographical origin were not associated with overall outcomes.Conclusions: Cerium nitrate may lead to vascular decalcification and chelation of reactive oxygen species, and prevent infection. Cerium nitrate-silver sulfadiazine was associated with better outcomes and should be tested in a prospective comparative trial in CPX patients. [ABSTRACT FROM AUTHOR]

Summary: The study objective was to estimate the effect of social deprivation estimated by the European Deprivation Index (EDI) on the risk of death and graft failure on renal transplantation in France. EDI was calculated for 8701 of 9205 patients receiving a first renal transplantation between 2010 and 2014. Patients were separated in EDI quintiles of the general population. A Cox model (cs‐HR: cause‐specific hazard ratio of death or graft failure) and a Fine and Gray model (sd‐HR: subdistribution hazard ratio of death and graft failure) were used for the analysis. The 5th quintile group (most deprived) accounted for 32% of patients [2818 of 8701]. In the multivariate analysis, compared with quintile 1, the risk of death was higher for the 5th quintile group in the complete cohort [cs‐HR: 1.31, 95% CI: (1.01–1.70), sd‐HR: 1.29, 95% CI: (1.00–1.68)], in the deceased donor group [cs‐HR: 1.31, 95% CI: (1.00–1.71), sd‐HR: 1.30, 95% CI: (1.00–1.70)] but not in living donor transplant patients. There was no association between the EDI groups and the risk of transplant failure. Social deprivation estimated by the EDI is associated with an increased risk of death in transplantation in France but not with the chance of allograft loss. [ABSTRACT FROM AUTHOR]

The influence of raised fibrinogen levels on outcome in stable outpatients with peripheral arterial disease (PAD) has not been consistently investigated. We used data from the Factores de Riesgo y ENfermedad Arterial (FRENA) registry to compare ischemic events, major bleeding, and mortality in stable outpatients with PAD, according to their baseline plasma fibrinogen levels. Of 1363 outpatients with PAD recruited in FRENA, 558 (41%) had fibrinogen levels >450 mg/100 mL. Over 18 months, 43 patients presented with acute myocardial infarction, 37 had an ischemic stroke, 51 underwent limb amputation, 19 had major bleeding, and 90 died. Compared to patients with normal levels, those with raised fibrinogen levels had an over 2-fold higher rate of ischemic stroke (rate ratio [RR]: 2.30; 95% confidence interval [CI]: 1.19-4.59), limb amputation (RR: 2.58; 95% CI: 1.46-4.67), or death (RR: 2.27; 95% CI: 1.49-3.51) and an over 3-fold higher rate of major bleeding (RR: 3.90; 95% CI: 1.45-12.1). On multivariate analysis, patients with raised fibrinogen levels had an increased risk of developing subsequent ischemic events (hazard ratio [HR]: 1.61; 95% CI: 1.11-2.32) and major bleeding (HR: 3.42; 95% CI: 1.22-9.61). Stable outpatients with PAD and raised plasma fibrinogen levels had increased rates of subsequent ischemic events and major bleeding. [ABSTRACT FROM AUTHOR]

Objectives: To determine the proportion and the characteristics of patients who did or did not respond after 3 months of ulipristal acetate (UPA) therapy.Study Design: In this retrospective cohort study conducted in the University Hospital of Bordeaux (France) and University Medical Center Ljubljana (Slovenia), symptomatic non-menopausal patients with fibroids that qualified for surgery were pretreated by 3 months of oral UPA 5 mg/day. Clinical success was defined by normalization of the bleeding score, and/or regression of pelvic pain, and/or abdominal distension. Imaging success was defined by reduction in fibroid volume ≥ 25%.Results: The clinical and imaging success rates were 54/66 (82%) and 39/66 (59%) respectively. The absence of previous pregnancy (p = 0.004) and the size of the dominant fibroid ≥ 80 mm (p = 0.004) were independent factors associated with clinical failure. Age <35 years (p = 0.02) was the only independent factor associated with imaging failure.Conclusion: Young women developing fibroids and/or women with large fibroids may be resistant to ulipristal acetate therapy. [ABSTRACT FROM AUTHOR]

Background: Early-onset ventilator-associated pneumonia (EOVAP) occurs frequently in severe traumatic brain-injured patients, but potential consequences on cerebral oxygenation and outcome have been poorly studied. The objective of this study was to describe the incidence, risk factors for, and consequences on cerebral oxygenation and outcome of EOVAP after severe traumatic brain injury (TBI).Methods: We conducted a retrospective, observational study including all intubated TBI admitted in the trauma center. An EOVAP was defined as a clinical pulmonary infection score >6, and then confirmed by an invasive method. Patient characteristics, computed tomography (CT) scan results, and outcome were extracted from a prospective register of all intubated TBI admitted in the intensive care unit (ICU). Data concerning the cerebral oxygenation monitoring by PbtO2 and characteristics of EOVAP were retrieved from patient files. Multivariate logistic regression models were developed to determine the risk factors of EOVAP and to describe the factors independently associated with poor outcome at 1-year follow-up.Results: During 7 years, 175 patients with severe TBI were included. The overall incidence of EOVAP was 60.6% (47.4/1000 days of ventilation). Significant risk factors of EOVAP were: therapeutic hypothermia (OR 3.4; 95% CI [1.2-10.0]), thoracic AIS score ≥3 (OR 2.4; 95% CI [1.1-5.7]), and gastric aspiration (OR 5.2, 95% CI [1.7-15.9]). Prophylactic antibiotics administration was a protective factor against EOVAP (OR 0.3, 95% CI [0.1-0.8]). EOVAP had negative consequences on cerebral oxygenation. The PbtO2 was lower during EOVAP: 23.5 versus 26.4 mmHg (p <0.0001), and there were more brain hypoxia episodes: 32 versus 27% (p = 0.03). Finally, after adjusting for confounders, an EOVAP was an independent factor associated with unfavorable neurologic functional outcome at the 1-year follow-up (OR 2.71; 95% CI [1.01-7.25]).Conclusions: EOVAP is frequent after a severe TBI (overall rate: 61%), with therapeutic hypothermia, severe thoracic lesion, and gastric aspiration as main risk factors. EOVAP had a negative impact on cerebral oxygenation measured by PbtO2 and was independently associated with unfavorable outcome at 1-year follow-up. This suggests that all precautions available should be taken to prevent EOVAP in this population. [ABSTRACT FROM AUTHOR]

To evaluate the impact of the 2015 European Resuscitation Council (ERC) guidelines on patient outcomes following traumatic cardiac arrest (TCA) and on advanced life support interventions carried out by physician-staffed ambulances. Data of TCA patients aged ≥18 years were extracted from the French nationwide cardiac arrest registry. A pre- (2011–2015) and a post-publication period (2016–2020) were defined. In the guidelines, a specific TCA management algorithm was introduced to prioritise the treatment of reversible causes. Its impact was evaluated using adjusted interrupted time series analysis. 4,980 patients were treated (2,145 during the pre-publication period and 2,739 during the post-publication period). There was no significant change in the rates of prehospital ROSC (22.4% vs. 20.2%, p = 0.07 in the pre- and post- intervention respectively), survival (1.4% vs. 1.4%, p = 0.87) or good neurological outcome (71.4% vs. 66.7%, p = 0.93) or in the incidence of organ donation (1.6% vs. 1.3%, p = 0.50). There were nonsignificant changes in the adjusted temporal trend for ROSC (aOR 0.88; 95% CI [0.77; 1.00]), survival (aOR 1.34; 95% CI [0.83;2.17]), good neurological outcome (aOR 1.57; 95% CI [0.82;3.05]), and organ donation (aOR 1.06; 95% CI [0.71;1.60]). The use of intraosseous catheters (13.0% vs. 19.2%, p < 0.001), external haemorrhage control measures (23.9% vs. 64.8%, p < 0.001), bilateral chest decompression (13.7% vs. 16.5%, p = 0.009), and packed red cell transfusion (2.7% vs. 6.5%, p < 0.001) increased in the post-publication period. Despite the increased frequency of trauma rescue interventions performed by on-scene physicians, no change in patient-centred outcomes was associated with the publication of the 2015 ERC guidelines in France. [ABSTRACT FROM AUTHOR]

Purpose: As post-cardiac arrest care may influence patients' outcome, characteristics of receiving hospitals should be integrated in the evaluation of survival. We aimed at assessing the influence of care level center on patients' survival at hospital discharge using a regional registry of out-of-hospital cardiac arrest patients (OHCA).Methods: We retrospectively analysed a Utstein and in-hospital data prospectively collected for all non-traumatic OHCA patients, in whom a successful return of spontaneous circulation (ROSC) had been obtained, from a large metropolitan area (Great Paris). Receiving hospitals were categorized in 3 groups as follows: A centers (High-case volume with cath-lab 24/7), B centers (Intermediate-case volume with cath-lab partly available) and C centers (Low-case volume and no cath-lab) We compared patients' characteristics and outcome in the 3 groups and performed a multivariate logistic regression using survival to discharge as primary endpoint.Results: Between May 2011 and December 2013, 1476 patients were admitted in 48 hospitals (group A: n=917; group B: n=428; group C: n=91). Overall survival rate at discharge was 433/1436 (30%). Patients' baseline characteristics significantly differed, as hospitals from group A received younger patients with a higher rate of shockable cardiac rhythms (p<0.001). Unadjusted survival rate differed significantly among the 3 groups of hospitals (respectively 34%, 25% and 15.4% for A-C, p<0.01). In multivariate analysis, the category of receiving hospital was no longer associated with survival, even in the subgroup of witnessed arrest and shockable patients.Conclusion: In this population-based study, characteristics of receiving hospitals are not associated with survival rate at discharge. This might be partially explained by the prehospital triage organization used in France. [ABSTRACT FROM AUTHOR]

Depression and anxiety are commonly associated with anorexia nervosa (AN) and contribute to difficulties in social integration, a negative factor for outcome in AN. The link between those disorders and AN has been poorly studied. Thus, our objective was to investigate (1) the link between outcome nine years after hospitalisation for AN and the occurrence of lifetime anxious or depressive comorbidities; (2) the prognostic value of these comorbidities on patient outcome; 181 female patients were hospitalised for AN (between 13 and 22 years old), and were re-evaluated for their psychological, dietary, physical and social outcomes, from 6 to 12 years after their hospitalisation. The link between anxious and depressive disorders (premorbid to AN and lifetime) and the outcome assessment criteria were tested through multivariate analyses; 63% of the participants had good or intermediate outcome, 83% had presented at least one anxiety or depression disorder in the course of their lives, half of them before the onset of AN. Premorbid obsessive compulsive disorders (OCD), BMI at admission, and premenarchal AN all contribute to poor prognosis. Social phobia and agoraphobia affect the subjects' quality of life and increase eating disorder symptoms. These results encourage a systematic assessment of, and care for, anxiety and depression comorbidities among female adolescent patients with a particular focus on premorbid OCD. [ABSTRACT FROM AUTHOR]

BACKGROUND: We aimed to test the performance of PRESERVE and RESP scores to predict death in patients with severe ARDS receiving extracorporeal membrane oxygenation (ECMO) with different case mixes. METHODS: All consecutive patients treated with ECMO for refractory ARDS, regardless of cause, in the Caen University Hospital in northwestern France over the last decade were included in a retrospective cohort study. The receiver operating characteristic curves of each score were plotted, and the area under the curve was computed to assess their performance in predicting mortality (c-index). RESULTS: Forty-one subjects were included. Pre-ECMO ventilator settings were: mean VT, 6.1 ± 0.9 mL/kg; breathing frequency, 32 ± 4 breaths/min; PEEP, 11 ± 4 cm H2O; peak inspiratory pressure, 48 ± 9 cm H2O; plateau pressure, 30.4 ± 4.4 cm H2O. At ECMO initiation, blood gas results were: pH 7.22 ± 0.17, PaO2/... = 63 ± 22 mm Hg; ... = 56 ± 18 mm Hg; ... = 99 ± 2%. Pre-ECMO data were available in 35 and 27 subjects for calculation of the PRESERVE score and RESP score, respectively. Pre-ECMO scoring system results were: median PRESERVE score, 4 (interquartile range 2-5), and median RESP score, 0 (interquartile range -2 to 2). Twenty-three subjects (56%) died, including 19 receiving ECMO. In univariate analysis, plateau pressure (P = .031), driving pressure (P = <.001), and compliance (P = .02) recorded at the time of ECMO initiation as well as the PRESERVE score (P = .032) were significantly associated with mortality. With a c-index of 0.69 (95% CI 0.53-0.87), the PRESERVE score had better discrimination than the RESP score (c-index of 0.60 [95% CI 0.41-0.78]) for predicting mortality. CONCLUSIONS: The use of these scores in helping physicians to determine the patients with ARDS most likely to benefit from ECMO should be limited in clinical practice because of their relatively poor performance in predicting death in subjects with severe ARDS receiving ECMO support. Before widespread use is initiated, these scoring systems should be tested in large prospective studies of subjects with severe ARDS undergoing ECMO treatment. [ABSTRACT FROM AUTHOR]

Objectives: To assess predictors and indicators of disability and quality-of-life 4 years after severe traumatic brain injury (TBI), using structural equation modelling (SEM). Methods: The PariS-TBI study is a longitudinal multi-centre inception cohort study of 504 patients with severe TBI. Among 245 survivors, 147 patients were evaluated upon 4-year follow-up, and 85 completed the full assessment. Two outcome measures were analysed separately using SEM: the Glasgow Outcome Scale-extended (GOS-E), to measure disability, and the QOLIBRI, to assess quality-of-life. Four groups of variables were entered in the model: demographics; injury severity; mood and cognitive impairments; somatic impairments. Results: The GOS-E was directly significantly related to mood and cognition, injury severity, and somatic impairments. Age and education had an indirect effect, mediated by mood/cognition or somatic deficiencies. In contrast, the only direct predictor of QOLIBRI was mood and cognition. Age and somatic impairments had an indirect influence on the QOLIBRI. Conclusion: Although this study should be considered as explorative, it suggests that disability and quality-of-life were directly influenced by different factors. While disability appeared to result from an interaction of a wide range of factors, quality-of-life was solely directly related to psycho-cognitive factors. [ABSTRACT FROM PUBLISHER]

Background In the context of the development of telemedicine in France to address low thrombolysis rates and limited stroke infrastructures, a star-shaped telestroke network was implemented in Burgundy (1.6 million inhabitants). We evaluated the safety and effectiveness of this network for thrombolysis in acute ischemic stroke patients. Methods One hundred and thirty-two consecutive patients who received intravenous thrombolysis during a telemedicine procedure (2012-2014) and 222 consecutive patients who were treated at the stroke center of Dijon University Hospital, France (2011-2012) were included. Main outcomes were the modified Rankin scale ( mRS) score and case fatality at 3 months. Comparisons between groups were made using multivariable ordinal logistic regression and logistic regression analyses, respectively. Results Baseline characteristics of telethrombolysis patients were similar to those of patients undergoing thrombolysis locally except for a higher frequency of previous cancer and pre-morbid handicap, and a trend towards greater severity at admission in the former. The distribution of mRS scores at 3 months was similar between groups, as were case-fatality rates (18.9% in the telethrombolysis group versus 16.5%, P = 0.56). In multivariable models, telethrombolysis did not independently influence functional outcomes at 3 months ( odds ratio for a shift towards a worse outcome on the mRS, 1.11; 95% confidence interval , 0.74-1.66, P = 0.62) or death ( odds ratio, 0.86; 95% confidence interval, 0.44-1.69, P = 0.66). Conclusion The implementation of a regional telemedicine network for the management of acute ischemic stroke appeared to be effective and safe. Thanks to this network, the proportion of patients who benefit from thrombolysis will increase. Further research is needed to evaluate economic benefits. [ABSTRACT FROM AUTHOR]

Drowning affects more than 500,000 people worldwide and is responsible for at least 350,000 deaths each year. In France, 1235 drowning resulting in 496 deaths were recorded in the summer 2012. This retrospective study has investigated the epidemiology of drowning in the city of Marseille (South of France) between 2000 and 2011. We identified 449 cases of unintentional drowning. The highest incidence was found among males with a median age of 36 years. The incidence was 5.3 victims per 10,000 inhabitants with a mortality rate of 1.2 per 10,000. These accidents occurred mainly at sea (89%) and during the summer season. A majority of drowning victims (69%) were admitted in a hospital. This is the only study in France to analyse data on drowning throughout the year and over a long period. Drowning is a serious condition burdened by 22% of victims who die. [ABSTRACT FROM PUBLISHER]

We wanted to describe the characteristics, treatment and outcome of autoimmune and inflammatory diseases (SAIDs) associated with chronic myelomonocytic leukemia (CMML), and conducted a French multicenter retrospective study and a literature review. We included 26 cases of CMML (median age 75 years, 54% female), 80% with CMML-1. CPSS score was low (0 or 1) in 75% of cases. SAIDS was systemic vasculitis in 54%. Diagnosis of the 2 diseases was concomitant in 31% cases, and CMML was diagnosed before SAIDs in 12 cases (46%). First line treatment for SAIDs consisted mostly of steroid, with 85% of response. Second-line treatment was needed in 40% cases. Six patients received hypomethylating agents, with 66% response on SAIDs. A literature review found 49 cases of CMML-associated SAIDs, in whom SAIDs was systemic vasculitis in 29% cases. Hence, vasculitis is the most frequent SAIDs associated with CMML. After initial response to steroids, recurrence and steroid-dependence were frequent. Hypomethylating agents may be interesting in this context. [ABSTRACT FROM AUTHOR]

Aim Practitioners are faced with a substantial challenge when considering recombinant tissue plasminogen activator (rt- PA) therapy for older patients with ischemic stroke. Patients aged over 80 years suffer from the most severe cerebral infarcts. The benefit of rt- PA treatment compared with single standard care only in stroke units remains to be clearly assessed. Methods We collected data from 321 patients aged over 80 years admitted for acute cerebral infarction to the stroke unit of Nancy University Hospital in France between 1 January 2009 and 31 December 2012. Patients were stratified into two groups: treated or not with rt- PA. Baseline characteristics and outcome were collected and compared between both groups. Good outcome at 3 months was defined as modified Rankin Scale score ≤2. Results The 55 patients treated with rt- PA had a higher National Institute of Health Stroke Scale score on admission than those without (15 vs 5; P < 0.001). They were more likely to have intracranial haemorrhage (20 vs 5%; P < 0.001) without an increased mortality rate (28 vs 27%; P = 0.95). Multivariate analysis showed a more favorable outcome (odds ratio 7, 95% confidence interval 3-16.5; P < 0.001). Slightly higher percentages of patients with modified Rankin Scale ≤2 were found after intention-to-treat analysis (49 vs 45%) and after exclusion of patients with baseline modified Rankin Scale >2 (57 vs 54 %), but without reaching significance ( P > 0.05). Conclusions Rt- PA therapy would appear to improve prognosis in the elderly with ischemic stroke. This suggests that age alone should no longer be a barrier to rt- PA therapy. Geriatr Gerontol Int 2016; 16: 843-849. [ABSTRACT FROM AUTHOR]

Background: Parenteral nutrition (PN) is the main treatment for intestinal failure. Objective: We aimed to review the indications for home parenteral nutrition (HPN) in children and describe the outcome over a 14-y period from a single center. Design: We conducted a retrospective study that included all children who were referred to our institution and discharged while receiving HPN between 1 January 2000 and 31 December 2013. The indications for HPN were divided into primary digestive diseases (PDDs) and primary nondigestive diseases (PNDDs). We compared our results to a previous study that was performed in our unit from 1980 to 2000 and included 302 patients. Results: A total of 251 patients were included: 217 (86%) had a PDD. The mean ± SD age at HPN onset was 0.7 ± 0.3 y, with a mean duration of 1.9 ± 0.4 y. The indications for HPN were short bowel syndrome (SBS) (59%), PNDD (14%), congenital enteropathies (10%), chronic intestinal pseudo-obstruction syndromes (9%), inflammatory bowel diseases (5%), and other digestive diseases (3%). By 31 December 2013, 52% of children were weaned off of HPN, 9% of the PDD subgroup had intestinal transplantation, and 10% died mostly because of immune deficiency. The major complications of HPN were catheter-related bloodstream infections (CRBSIs) (1.7/1000 d of PN) and intestinal failure-associated liver disease (IFALD) (51 children; 20% of cohort). An increased rate of CRBSIs was observed compared with our previous study, but we saw a decreasing trend since 2012. No noteworthy deceleration of growth was observed in SBS children 6 mo after weaning off HPN. Conclusions: SBS was the major indication for HPN in our cohort. IFALD and CRBSIs were potentially life-threatening problems. Nevertheless, complication rates were low, and deaths resulted mostly from the underlying disease. [ABSTRACT FROM AUTHOR]

Objective. Outcome of JDM is highly heterogeneous. Our objective was to determine clinical and muscle biopsy features associated with poor outcome and response to treatment. Methods. Clinical data and muscle biopsy were obtained from a monocentric cohort of 29 patients. Clinical subgroups were defined by latent class model analysis of initial and follow-up parameters. Myopathological features were analysed using validated scores. Capillary loss was determined on reconstructions of transversal sections and assessed in the different age groups to take into account variations of muscle capillarization during post-natal development. Regression models were used to identify initial predictors of therapeutic response. Results. Two distinct homogeneous subgroups of patients were identified according to clinical severity and pathological findings. The smallest group of patients (7/29) presented with severe JDM. Compared with the other group (22/29), patients had more severe muscle weakness at disease onset, low remission rate at 12 months, frequent subcutaneous limb oedema or gastrointestinal (GI) involvement and higher myopathological scores (capillary dropout, perifascicular necrosis/regeneration, fibres with internal myonuclei and fibrosis subscores). Relevance of capillary dropout to JDM severity was substantiated by agebased analysis, confirming its major role in JDM pathophysiology. Most of these manifestations could be related to vasculopathy (limb oedema, GI involvement, capillary dropout). Furthermore, Childhood Myositis Assessment Scale <34 with either GI involvement or muscle endomysial fibrosis at disease onset were the best predictors of poor response to treatment. Conclusion. Vasculopathy is prominent in severe JDM. Simple criteria can be used at initial evaluation to identify patients requiring a more intensive therapy. [ABSTRACT FROM AUTHOR]

Objective. We describe myelodysplastic syndrome (MDS)-associated systemic inflammatory and autoimmune diseases (SIADs), their treatments and outcomes and the impact of SIADs on overall survival in a French multicentre retrospective study. Methods. In this study, 123 patients with MDS and SIADs were analysed. Results. Mean age was 70 years (S.D. 13) and the male:female ratio was 2. The SIADs were systemic vasculitis in 39 (32%) cases, CTD in 31 (25%) cases, inflammatory arthritis in 28 (23%) cases, a neutro-philic disorder in 12 (10%) cases and unclassified in 13 cases (11%). The SIADs fulfilled the usual classification criteria in 75 (66%) cases, while complete criteria were not reached in 21 (19%) cases. A significant association was shown between chronic myelomonocytic leukaemia (CMML) and systemic vasculitis (P = 0.0024). One hundred and eighteen (96%) SIAD patients were treated (91% with steroids), with an 83% response to first-line treatment, including 80% for steroids alone. A second-line treatment for SIADs was required for steroid dependence or relapse in 48% of cases. The effect of MDS treatment on SIADs could be assessed in 11 patients treated with azacytidine and SIAD response was achieved in 9/11 (80%) and 6/11 (55%) patients at 3 and 6 months, respectively. Compared with 665 MDS/CMML patients without SIADs, MDS/CMML patients with SIADs were younger (P<0.01), male (P = 0.03), less often had refractory anaemia with ring sideroblasts (P<0.01), more often had a poor karyotype (16% vs 11%, P = 0.04) and less frequently belonged to low and intermediate-1 International Prognostic Scoring System categories, but no survival difference was seen between patients with MDS-associated SIADs and without SIADs (P = 0.5). Conclusion. The spectrum of SIADs associated to MDS is heterogeneous, steroid sensitive, but often steroid dependent. [ABSTRACT FROM AUTHOR]