Cardiac involvement in pediatric hemolytic uremic syndrome.

BACKGROUND: Cardiac involvement is a known but rare complication of pediatric hemolytic uremic syndrome (HUS). We conducted a nationwide observational, retrospective case-control study describing factors associated with the occurrence of myocarditis among HUS patients. METHODS: Cases were defined as hospitalized children affected by any form of HUS with co-existent myocarditis in 8 French Pediatric Intensive Care Units (PICU) between January 2007 and December 2018. Control subjects were children, consecutively admitted with any form of HUS without coexistent myocarditis, at a single PICU in Lyon, France, during the same time period. RESULTS: A total of 20 cases of myocarditis were reported among 8 PICUs, with a mean age of 34.3 ± 31.9 months; 66 controls were identified. There were no differences between the two groups concerning the season and the typical, Shiga toxin-producing Escherichia coli (STEC-HUS), or atypical HUS (aHUS). Maximal leukocyte count was higher in the myocarditis group (29.1 ± 16.3G/L versus 21.0 ± 9.9G/L, p = 0.04). The median time between admission and first cardiac symptoms was of 3 days (range 0-19 days), and 4 patients displayed myocarditis at admission. The fatality rate in the myocarditis group was higher than in the control group (40.0% versus 1.5%, p < 0.001). Thirteen (65%) children from the myocarditis group received platelet transfusion compared to 19 (29%) in the control group (p = 0.03). CONCLUSION: Our study confirms that myocarditis is potentially lethal and identifies higher leukocyte count and platelet transfusion as possible risk factors of myocarditis. A higher resolution version of the Graphical abstract is available as Supplementary information.

Ultrasound guidance for urgent arterial and venous catheterisation: randomised controlled study.

BACKGROUND: Haemodynamically unstable patients often require arterial and venous catheter insertion urgently. We hypothesised that ultrasound-guided arterial and venous catheterisation would reduce mechanical complications. METHODS: We performed a prospective RCT, where patients requiring both urgent arterial and venous femoral catheterisation were randomised to either ultrasound-guided or landmark-guided catheterisation. Complications and characteristics of catheter insertion (procedure duration, number of punctures, and procedure success) were recorded at the time of insertion (immediate complications). Late complications were investigated by ultrasound examination performed between the third and seventh days after randomisation. Primary outcome was the proportion of patients with at least one mechanical complication (immediate or late), by intention-to-treat analysis. Secondary outcomes included success rate, procedure time, and number of punctures. RESULTS: We analysed 136 subjects (102 [75%] male; age range: 27-62 yr) by intention to treat. The proportion of subjects with one or more complications was lower in 22/67 (33%) subjects undergoing ultrasound-guided catheterisation compared with landmark-guided catheterisation (40/69 [58%]; odds ratio: 0.35 [95% confidence interval: 0.18-0.71]; P=0.003). Ultrasound-guided catheterisation reduced both immediate (27%, compared with 51% in the landmark approach group; P=0.004) and late (10%, compared with 23% in the landmark approach group; P=0.047) complications. Ultrasound guidance also reduced the proportion of patients who developed deep vein thrombosis (4%, compared with 22% following landmark approach; P=0.012), and achieved a higher procedural success rate (96% vs 78%; P=0.004). CONCLUSIONS: An ultrasound-guided approach reduced mechanical complications after urgent femoral arterial and venous catheterisation, while increasing procedural success. CLINICAL TRIAL REGISTRATION: NCT02820909.

Comparison of clinical outcomes and accuracy of electrode placement between robot-assisted and conventional deep brain stimulation of the subthalamic nucleus: a single-center study.

BACKGROUND: Several surgical methods are used for deep brain stimulation (DBS) of the subthalamic nucleus (STN) in Parkinson's disease (PD). This study aimed to compare clinical outcomes and electrode placement accuracy after robot-assisted (RAS) versus frame-based stereotactic (FSS) STN DBS in Parkinson's disease. METHODS: In this single-center open-label study, we prospectively collected data from 48 consecutive PD patients who underwent RAS (Neuromate®; n = 20) or FSS (n = 28) STN DBS with the same MRI-based STN targeting between October 2016 and December 2018 in the university neurological hospital of Lyon, France. Clinical variables were assessed before and 1 year after surgery. The number of electrode contacts within the STN was determined by merging post-operative CT and pre-operative MRI using Brainlab® GUIDE™XT software. RESULTS: One year after surgery, the improvement of motor manifestations (p = 0.18), motor complications (p = 0.80), and quality of life (p= 0.30) and the reduction of dopaminergic treatment (p = 0.94) and the rate of complications (p = 0.99) were similar in the two groups. Surgery duration was longer in the RAS group (p = 0.0001). There was no difference in the number of electrode contacts within the STN. CONCLUSION: This study demonstrates that RAS and FSS STN DBS for PD provide similar clinical outcomes and accuracy of electrode placement.

Poor Involvement of General Practitioners in Decisions of Withholding or Withdrawing Life-Sustaining Treatment in Emergency Departments.

BACKGROUND: Decisions of withholding or withdrawing life-sustaining treatment are frequent in emergency departments (ED) and patients are often unable to communicate their wishes concerning end of life desires. OBJECTIVE: To evaluate the participation of general practitioners (GPs) during the decision-making process of withholding or withdrawing life-sustaining treatments in ED. DESIGN: Prospective observational multicenter study. PATIENTS: We included patients for whom a decision of withdrawing or withholding life-sustaining treatments was made in ED. For each patient, we enrolled one general practitioner. MAIN MEASURES: GPs were interviewed about their perception of end of life patient's management and the communication with ED and families. KEY RESULTS: There were 109 potential patient participants. We obtained answers from 54 (49.5%) of the patient's associated GPs. Only 4 (7.4%) GPs were involved during the decision-making process of withholding or withdrawing life-sustaining treatments. Among GPs, 29 (53.7%) were contacted by family after the decision, most often to talk about their difficult experience with the decision. A majority (94%) believed their involvements in these decisions were important and 68% wished to "always" participate in end of life decisions despite the fact that they usually don't participate in these decisions. Finally, 66% of GPs believed that management of end of life in the emergency department was a failure and should be anticipated. CONCLUSIONS: GPs would like to be more involved and barriers to GP involvement need to be overcome. We do not have any outcome data to suggest that routine involvement of GPs in all end of life patients improves their outcomes. Moreover, it requires major system and process-based changes to involve all primary care physicians in ED decision-making. NIH TRIAL REGISTRY NUMBER: NCT02844972.

Gabapentin and Memantine for Treatment of Acquired Pendular Nystagmus: Effects on Visual Outcomes.

BACKGROUND: The most common causes of acquired pendular nystagmus (APN) are multiple sclerosis (MS) and oculopalatal tremor (OPT), both of which result in poor visual quality of life. The objective of our study was to evaluate the effects of memantine and gabapentin treatments on visual function. We also sought to correlate visual outcomes with ocular motor measures and to describe the side effects of our treatments. METHODS: This study was single-center cross-over trial. A total of 16 patients with chronic pendular nystagmus, 10 with MS and 6 with OPT were enrolled. Visual acuity (in logarithm of the minimum angle of resolution [LogMAR]), oscillopsia amplitude and direction, eye movement recordings, and visual function questionnaires (25-Item National Eye Institute Visual Functioning Questionnaire [NEI-VFQ-25]) were performed before and during the treatments (gabapentin: 300 mg 4 times a day and memantine: 10 mg 4 times a day). RESULTS: A total of 29 eyes with nystagmus were evaluated. Median near monocular visual acuity improved in both treatment arms, by 0.18 LogMAR on memantine and 0.12 LogMAR on gabapentin. Distance oscillopsia improved on memantine and on gabapentin. Median near oscillopsia did not significantly change on memantine or gabapentin. Significant improvement in ocular motor parameters was observed on both treatments. Because of side effects, 18.8% of patients discontinued memantine treatment-one of them for a serious adverse event. Only 6.7% of patients discontinued gabapentin. Baseline near oscillopsia was greater among those with higher nystagmus amplitude and velocity. CONCLUSIONS: This study demonstrated that both memantine and gabapentin reduce APN, improving functional visual outcomes. Gabapentin showed a better tolerability, suggesting that this agent should be used as a first-line agent for APN. Data from our investigation emphasize the importance of visual functional outcome evaluations in clinical trials for APN.

Liver disease related to alpha1-antitrypsin deficiency in French children: The DEFI-ALPHA cohort.

BACKGROUND & AIMS: To identify prognostic factors for liver disease in children with alpha-1 antitrypsin deficiency, irrespective of phenotype, using the DEFI-ALPHA cohort. METHODS: Retrospective, then prospective from 2010, multicentre study including children known to have alpha-1 antitrypsin blood concentration below 0.8 g/L, born in France since 1989. Clinical and biological data were collected. Liver disease was classified as "severe" (portal hypertension, liver failure, liver transplantation or death); "moderate" (persistent abnormal liver biology without portal hypertension); and "mild/none" (normal or almost normal liver biology and native liver). Prognostic factors for severe liver disease were evaluated using a Cox semiparametric model. RESULTS: In January 2017, 153 patients from 19 centres had been included; genotypes were PIZZ in 81.9%, PISZ in 8.1%, other in 10.0%. Mean ± SD follow-up was 4.7 ± 2.1 years. Half of patients had moderate liver disease. Twenty-eight children (18.3%) had severe liver disease (mean age 2.5 years, range: 0-11.6): diagnosis of alpha-1 antitrypsin deficiency was made before two months of age in 65.4%, genotypes were PIZZ in 25 (89.3%), PISZ in 2, PIMlike Z in 1, 15 children underwent liver transplantation, 1 child died at 3 years of age. Neonatal cholestasis was significantly associated with severe liver disease (P = 0.007). CONCLUSION: Alpha-1 antitrypsin-deficient patients presenting with neonatal cholestasis were likely to develop severe liver disease. Some patients with non-homozygous ZZ genotype can develop severe liver disease, such as PISZ and M variants, when associated with predisposing factors. Further genetic studies will help to identify other factors involved in the development of liver complications.

Mortality, complication, and fusion rates of patients with odontoid fracture: the impact of age and comorbidities in 204 cases.

PURPOSE: The French Society of Spine Surgery (SFCR) conducted a prospective epidemiologic multicenter study. The purpose was to investigate mortality, complication, and fusion rates in patients with odontoid fracture, depending on age, comorbidities, fracture type, and treatment. METHODS: Out of 204 patients, 60 were ≤ 70 years and 144 were > 70 years. Demographic data, comorbidities, treatment types and complications (general medical, infectious, neurologic, and mechanical), and death were registered within the first year. Fractures were classified according to Anderson-D'Alonzo and Roy-Camille on the initial CT. A 1-year follow-up CT was available in 144 patients to evaluate fracture consolidation. RESULTS: Type II and oblique-posterior fractures were the most frequent patterns. The treatment was conservative in 52.5% and surgical in 47.5%. The mortality rate in patients ≤ 70 was 3.3% and 16.7% in patients > 70 years (p = 0.0002). Fracture pattern and treatment type did not influence mortality. General medical complications were significantly more frequent > 70 years (p = 0.021) and after surgical treatment (p = 0.028). Neurologic complications occurred in 0.5%, postoperative infections in 2.0%, and implant-related mechanical complications in 10.3% (associated with pseudarthrosis). Fracture fusion was observed in 93.5% of patients ≤ 70 years and in 62.5% >70 years (p < 0.0001). Pseudarthrosis was present in 31.5% of oblique-posterior fractures and in 24.3% after conservative treatment. CONCLUSIONS: Age and comorbidities influenced mortality and medical complication rates most regardless of fracture type and treatment choice. Pseudarthrosis represented the main complication, which increased with age. Pseudarthrosis was most frequent in type II and oblique-posterior fractures after conservative treatment.

Faltering growth in the critically ill child: prevalence, risk factors, and impaired outcome.

Low body mass index (BMI) z score is commonly used to define undernutrition, but faltering growth allows for a complementary dynamic assessment of nutritional status. We studied the prevalence of undernutrition and faltering growth at admission in the pediatric intensive care (PICU) setting and their impacts on outcome. All (685) consecutive children (aged 0 to 18 years old) admitted in a single-center PICU over a 1-year period were prospectively enrolled. Nutritional status assessment was based on anthropometric measurements performed at admission and collected from medical files. Undernutrition was considered when z score BMI for age was < - 2SD. Faltering growth was considered when the weight for age curve presented a deceleration of > - 1 z score in the previous 3 months. Undernutrition was diagnosed in 13% of children enrolled, and faltering growth in 13.7% mostly in children with a normal BMI. Faltering growth was significantly associated with a history of underlying chronic disease, and independently with extended length of PICU stay in a multivariate analysis. CONCLUSION: Assessment of nutritional status in critically ill children should include both undernutrition and faltering growth. This study highlights that faltering growth is independently associated with suboptimal outcome in PICU. What is Known: • Malnutrition, defined according to BMI-for-age z score, is correlated with poor outcome in the critically ill child. • In this setting, nutritional assessment should consist not only of a static assessment based on BMI-for-age z score but also of a dynamic assessment to identify recent faltering growth. What is New: • Critically ill children frequently present with faltering growth at admission. • Faltering growth is a newly identified independent associated factor of suboptimal outcome in this setting (extended length of stay).

Uncontrolled donation after circulatory death: comparison of two kidney preservation protocols on graft outcomes.

BACKGROUND: Kidney transplantation following uncontrolled donation after circulatory death (uDCD) presents a high risk of delayed graft function due to prolonged warm ischemia time. In order to minimise the effects of ischemia/reperfusion injury during warm ischemia, normothermic recirculation recently replaced in situ perfusion prior to implantation in several institutions. The aim of this study was to compare these preservation methods on kidney graft outcomes. METHODS: The primary endpoint was the one-year measured graft filtration rate (mGFR). We collected retrospective data from 64 consecutive uDCD recipients transplanted over a seven-year period in a single centre. RESULTS: Thirty-two grafts were preserved by in situ perfusion and 32 by normothermic recirculation. The mean ± SD mGFR at 1 year post-transplantation was 43.0 ± 12.8 mL/min/1.73 m2 in the in situ perfusion group and 53.2 ± 12.8 mL/min/1.73 m2 in the normothermic recirculation group (p = 0.01). Estimated GFR levels were significantly higher in the normothermic recirculation group at 12 months (p = 0.01) and 24 months (p = 0.03) of follow-up. We did not find any difference between groups regarding patient and graft survival, delayed graft function, graft rejection, or interstitial fibrosis. CONCLUSIONS: Function of grafts preserved by normothermic recirculation was better at 1 year and the results suggest that this persists at 2 years, although no difference was found in short-term outcomes. Despite the retrospective design, this study provides an additional argument in favour of normothermic recirculation.

Returning to work after multimodal treatment in glioblastoma patients.

OBJECTIVE Although multimodal treatment for glioblastoma (GBM) has resulted in longer survival, uncertainties exist regarding health-related quality of life and functional performance. Employment represents a useful functional end point and an indicator of social reintegration. The authors evaluated the rate of patients resuming their employment and the factors related to work capacity. METHODS The authors performed a retrospective study of working-age patients treated with surgery and radiochemotherapy between 2012 and 2015. Data were collected before and after surgery and at 6, 12, 18, and 24 months. Employment was categorized according to the French Socio-Professional Groups and analyzed regarding demographic and clinical data, performance status, socio-professional category, radiological features, type, and quality of resection. RESULTS A total of 125 patients, mean age 48.2 years, were identified. The mean follow-up was 20.7 months with a median survival of 22.9 months. Overall, 21 patients (18.3%) went back to work, most on a part-time basis (61.9%). Of the patients who were alive at 6, 12, 18, and 24 months after diagnosis, 8.7%, 13.8%, 15.3%, and 28.2%, respectively, were working. Patients going back to work were younger (p = 0.03), had fewer comorbidities (p = 0.02), and had a different distribution of socio-professional groups, with more patients belonging to higher occupation categories (p = 0.02). Treatment-related symptoms (36.2%) represented one of the main factors that prevented the resumption of work. Employment was strongly associated with performance status (p = 0.002) as well as gross-total removal (p = 0.04). No statistically significant difference was found regarding radiological or molecular features and the occurrence of complications after surgery. CONCLUSIONS GBM diagnosis and treatment has a significant socio-professional impact with only a minority of patients resuming work, mostly on a part-time basis.

Worldwide view of nephropathic cystinosis: results from a survey from 30 countries.

BACKGROUND: Nephropathic cystinosis is a rare inherited metabolic disorder leading to progressive renal failure and extra-renal comorbidity. The prognosis relies on early adherence to cysteamine treatment and symptomatic therapies. Developing nations [DiN] experience many challenges for management of cystinosis. The aim of this study was to assess the management characteristics in DiN compared with developed nations [DeN]. METHODS: A questionnaire was sent between April 2010 and May 2011 to 87 members of the International Pediatric Nephrology Association, in 50 countries. RESULTS: A total of 213 patients were included from 41 centres in 30 nations (109 from 17 DiN and 104 from 13 DeN). 7% of DiN patients died at a median age of 5 years whereas no death was observed in DeN. DiN patients were older at the time of diagnosis. In DiN, leukocyte cystine measurement was only available in selected cases for diagnosis but never for continuous monitoring. More patients had reached end-stage renal disease in DiN (53.2 vs. 37.9%, p = 0.03), within a shorter time of evolution (8 vs. 10 yrs., p = 0.0008). The earlier the cysteamine treatment, the better the renal outcome, since the median renal survival increased up to 16.1 [12.5-/] yrs. in patients from DeN treated before the age of 2.5 years of age (p = 0.0001). However, the renal survival was not statistically different between DeN and DiN when patients initiated cysteamine after 2.5 years of age. The number of transplantations and the time from onset of ESRD to transplantation were not different in DeN and DiN. More patients were kept under maintenance dialysis in DiN (26% vs.19%, p = 0.02); 79% of patients from DiN vs. 45% in DeN underwent peritoneal dialysis. CONCLUSIONS: Major discrepancies between DiN and DeN in the management of nephropathic cystinosis remain a current concern for many patients living in countries with limited financial resources.

Trigeminal neuralgia due to venous neurovascular conflicts: outcome after microvascular decompression in a series of 55 consecutive patients.

BACKGROUND: Implication of veins as neurovascular conflict (NVC) in the genesis of trigeminal neuralgia (TN) remains a matter of debate. Few reports dealing with venous NVC have been published. The objective of this study is to describe the outcome in a historical cohort of consecutive patients with classical TN due to venous compression. METHODS: All patients with TN treated by microvascular decompression (MVD) from 2005 to 2013 were included if a marked venous compression was found at the surgery either alone or accompanied by an artery. Patients were evaluated for clinical presentation, operative findings and the long-term outcome. Outcome was considered favourable if patients were classed as BNI I or II (i.e. not requiring any medication). Kaplan-Meier analysis was used to determine probability of a favourable outcome at 10 years of follow-up. RESULTS: Out of the overall series of 313 patients having been treated by MVD and considered for the study, in 55 (17.5 %) a vein was the main compressive vessel; in 26 (8.3 %) it was the only compressive vessel. Probability of relief with no need for medication at 10 years was 70.6 %. The patients with focal arachnoiditis had a poor long-term outcome, i.e. BNI III-V, in 85.7 % compared with 20.8 % without arachnoiditis (p = 0.0037 Fisher's exact test). No differences in outcome were found between patients presenting with purely venous compression and patients with mixed compression. Outcome was similarly good for patients with atypical neuralgia when compared to patients with typical clinical presentation. CONCLUSIONS: Venous NVC as a cause of TN is far from rare. MVD with complete liberation of the entire root in cases with clear-cut venous compression on imaging studies gives a good probability of long-term pain relief, thus encouraging to propose surgery for such patients.

Pediatric combined liver-kidney transplantation: a single-center experience of 18 cases.

BACKGROUND: Experience in combined liver-kidney transplantation (CLKT) in children is limited. METHODS: We conducted a retrospective study of all pediatric CLKTs performed at our medical institution between 1992 and 2013. RESULTS: We identified 18 pediatric patients (9 girls) who had undergone CLKT at our institution during the study period. The median age [range] and body weight [range] of this patient group was 3.6 [1.0-18.6] years and 13 [10-40] kg, respectively; 11 patients weighed <15 kg at the time of CLKT. Indications for CLKT were primary hyperoxaluria (PH1; n = 14), association of hepatic fibrosis and end-stage renal disease (n = 3) and methylmalonic acidemia (n = 1). In the early postoperative period, eight patients required dialysis. Median stay in the pediatric intensive care unit was 10 [6-29] days. One patient died from cardiovascular disease 10 years after CLKT. There were no liver graft losses despite six acute liver rejection episodes, whereas four kidney grafts were lost. At last follow-up (6 [0.5-21] years) for patients with a functioning renal graft, the glomerular filtration rate was 71 [26-146] mL/min/1.73 m(2). In PH1 patients, urine oxalate normalized in six patients within 3 years after CLKT, but three patients still presented with elevated oxaluria at 1, 2 and 3 years after CLKT. CONCLUSIONS: Pediatric CLKT provides encouraging results in the long term, even in the youngest patients.

Burnout among physicians in palliative care: Impact of clinical settings.

OBJECTIVE: Burnout syndrome is a work-related professional distress. Palliative care physicians often have to deal with complex end-of-life situations and are at risk of presenting with burnout syndrome, which has been little studied in this population. Our study aims to identify the impact of clinical settings (in a palliative care unit (PCU) or on a palliative care mobile team (PCMT)) on palliative care physicians. METHOD: We undertook a cross-sectional study using a questionnaire that included the Maslach Burnout Inventory (MBI), and we gathered sociodemographic and professional data. The questionnaire was sent to all 590 physicians working in palliative care in France between July of 2012 and February of 2013. RESULTS: The response rate was 61, 8% after three reminders. Some 27 (9%) participants showed high emotional exhaustion, 12 (4%) suffered from a high degree of depersonalization, and 71 (18%) had feelings of low personal accomplishment. Physicians working on a PCMT tended (p = 0.051) to be more likely to suffer from emotional exhaustion than their colleagues. Physicians working on a PCMT worked on smaller teams (fewer physicians, p < 0.001; fewer nonphysicians, p < 0.001). They spent less time doing research (p = 0.019), had fewer resources (p = 0.004), and their expertise seemed to be underrecognized by their colleagues (p = 0.023). SIGNIFICANCE OF RESULTS: The prevalence of burnout in palliative care physicians was low and in fact lower than that reported in other populations (e.g., oncologists). Working on a palliative care mobile team can be a more risky situation, associated with a lack of medical and paramedical staff.

A training program for anthropometric measurements by a dedicated nutrition support team improves nutritional status assessment of the critically ill child.

OBJECTIVES: The cornerstone of an optimal nutrition approach in PICUs is to evaluate the nutritional status of any patient. Anthropometric measurements and nutritional indices calculation allow for nutritional status assessment, which is not often part of routine management, as it is considered difficult to perform in this setting. We designed a study to evaluate the impact of a training program by the PICU nutritional support team on the implementation of routine anthropometric measurements on our PICU. DESIGN: A prospective study was performed over a 2-year period, which included: a baseline evaluation of nutritional assessment, knowledge, anthropometric measurements (weight, height, and head and mid upper arm circumferences), and nutritional indices calculation in patient files. This was followed by a training program to implement the newly developed nutrition assessment guidelines, which included anthropometrical measurements and also the interpretation of these. The impact of this nutritional assessment program was reviewed annually for 2 years after the implementation. SETTING: PICU--Lyon, France. PATIENTS AND SUBJECTS: PICU nursing and medical staff, and patients admitted in February 2011, 2012, and 2013. INTERVENTIONS: Training program. MEASUREMENTS AND MAIN RESULTS: Ninety-nine percent of staff (n = 145) attended the individual teaching. We found significant progress in nutritional awareness and confidence about nutritional assessment following the teaching program. In addition, an improvement in staff knowledge about undernutrition and its consequences were found. We enrolled 41, 55, and 91 patients in 2011, 2012, and 2013, respectively. There was a significant increase in anthropometric measurements during this time: 32%, 65% (p = 0.002), and 96% in 2013 (p < 0.001). Nutritional indices were calculated in 20%, 74% (p < 0.001), and 96% (p < 0.001) of cases. CONCLUSIONS: This is the first study, showing that a targeted nutritional assessment teaching program that highlights both the importance and techniques of anthropometrical measurements has successfully been implemented in a PICU. It managed to improve staff knowledge and nutritional practice.

Endoscopic papillectomy for early ampullary tumors: long-term results from a large multicenter prospective study.

BACKGROUND AND STUDY AIMS: Endoscopic papillectomy of early tumors of the ampulla of Vater is an alternative to surgery. This large prospective multicenter study was aimed at evaluating the long-term results of endoscopic papillectomy. PATIENTS AND METHODS: Between September 2003 and January 2006, 10 centers included all patients referred for endoscopic papillectomy and meeting the inclusion criteria: biopsies showing at least adenoma, a uT1N0 lesion without intraductal involvement at endoscopic ultrasound (EUS), and no previous treatment. A standardized endoscopic papillectomy was done, with endoscopic monitoring with biopsies 4 - 8 weeks later where complications were recorded and complementary resection performed when necessary. Follow-up with duodenoscopy, biopsies, and EUS was done at 6, 12, 18, 24 and 36 months. Therapeutic success was defined as complete resection (no residual tumor found at early monitoring) without duodenal submucosal invasion in the resection specimen in the case of adenocarcinoma and without relapse during follow-up. RESULTS: 93 patients were enrolled. Mortality was 0.9 % and morbidity 35 %, including pancreatitis in 20 %, bleeding 10 %, biliary complications 7 %, perforation 3.6 %, and papillary stenosis in 1.8 %. Adenoma was not confirmed in the resection specimen in 14 patients who were therefore excluded. Initial treatment was insufficient in 9 cases (8 carcinoma with submucosal invasion; 1 persistence of adenoma). During follow-up, 5 patients had tumor recurrence and 7 died from unrelated diseases without recurrence. Finally, 81.0 % of patients were cured (95 % confidence interval 72.3 % - 89.7 %). CONCLUSION: Endoscopic papillectomy of selected ampullary tumors is curative in 81.0 % of cases. It must be considered to be the first-line treatment for early tumors of the ampulla of Vater without intraductal invasion.

Fibrinogen and von Willebrand factor and susceptibility vessel sign on T2*-weighted gradient echo imaging.

BACKGROUND: We investigated the relationship between von Willebrand factor (vWF), fibrin monomers (FM), fibrinogen baseline levels and the presence of susceptibility vessel sign (SVS) on T2*-weighted gradient echo imaging in acute ischemic stroke. METHODS: SVS was assessed at admission using T2*-weighted GRE. Plasmatic levels of vWF, FM and fibrinogen were evaluated before the initiation of intravenous thrombolysis. RESULTS: Forty-four patients were enrolled in this study. SVS was noted in 26 patients. Univariate analysis revealed that vWF >160% (p = 0.02) and fibrinogen >4 g/l (p = 0.03) were associated with a significant decrease in the likelihood of SVS. Multivariate analysis confirmed that higher levels of vWF or fibrinogen predicted the absence of SVS. CONCLUSIONS: The increased activity of vWF may promote a fibrin-platelet recruitment mainly contributing to the absence of (SVS).

Dual kidney transplantation from uncontrolled deceased donors after cardiac arrest: a possible option.

Organ shortage is a major problem in organ transplantation. For this reason, transplantation teams have found it necessary to revisit their organ acceptance criteria. Uncontrolled deceased donors after cardiac arrest could increase the donor pool by 20%, but at the same time there is a greater risk of delayed graft function and primary non-function. Dual kidney transplantation is an option when single kidney transplantation cannot be carried out because of lack of organ quality. We report for the first time our four first dual kidney transplantation from uncontrolled deceased donors after cardiac arrest with a follow up longer than 1 year. We described graft outcomes until 5 years, and histology at 3 and 12 months after transplantation. All organs were machine perfused in order to assess their quality leading to a single kidney transplantation or dual kidney transplantation decision. After 1 year of follow up, all grafts were functional with a mean estimated glomerular filtration rate of 44.5 ± 3.3 mL/min/1.73 m(2), and a mean inulin clearance of 43.7 ± 13.6 mL/mn/1.73 m(2). These findings suggest that dual kidney transplantation can represent a viable option for kidneys unsuitable for single kidney transplantation without increasing the rate of surgical complications. Successful transplantation is linked to histological, biological and donor clinical criteria, as well as perfusion parameters.

Use of 5-ALA fluorescence-guided surgery versus white-light conventional microsurgery for the resection of newly diagnosed glioblastomas (RESECT study): a French multicenter randomized phase III study.

OBJECTIVE: Only one phase III prospective randomized study, published in 2006, has assessed the performance of 5-aminolevulinic acid (5-ALA) fluorescence-guided surgery (FGS) for glioblastoma resection. The aim of the RESECT study was to compare the onco-functional results associated with 5-ALA fluorescence and with white-light conventional microsurgery in patients with glioblastoma managed according to the current standards of care. METHODS: This was a phase III prospective randomized single-blinded study, involving 21 French neurosurgical centers, comparing 5-ALA FGS with white-light conventional microsurgery in patients with glioblastoma managed according to the current standards of care, including neuronavigation use and postoperative radiochemotherapy. Randomization was performed in a 1:1 ratio stratified by institution. 5-ALA (20 mg/kg) or placebo (ascorbic acid) was administered orally 3-5 hours before the incision. The primary endpoint was the rate of gross-total resection (GTR) blindly assessed by an independent committee. Patients without a confirmed pathological diagnosis of glioblastoma or with unavailable postoperative MRI studies were excluded from the per-protocol analysis. RESULTS: Between March 2013 and August 2016, a total of 171 patients were assigned to the 5-ALA fluorescence group (n = 88) or to the placebo group (n = 83). Twenty-four cases were excluded because the WHO histological criteria of grade 4 glioma were not met. The proportion of GTR was significantly higher in the 5-ALA fluorescence group (53/67, 79.1%) than in the placebo group (33/69, 47.8%; p = 0.0002). After adjustment for age, preoperative Karnofsky Performance Scale score, and tumor location, GTR was still associated with 5-ALA fluorescence (OR 4.13 [95% CI 1.94-8.79]). The mean 7-day postoperative Karnofsky Performance Scale score (≥ 80% in 49/71, 69.0% [5-ALA group]; 50/71, 70.4% [placebo group], p = 0.86) and the proportion of patients with a worsened neurological status 3 months postoperatively (9/68, 13.2% [5-ALA group]; 9/70, 12.9% [placebo group], p = 0.95) were similar between groups. Adverse events related to 5-ALA intake were rare and consisted of photosensitization in 4/87 (4.6%) patients and hepatic cytolysis in 1/87 (1.1%) patients. The 6-month PFS (70.2% [95% CI 57.7%-79.6%] and 68.4% [95% CI 55.7%-78.1%]; p = 0.39) and 24-month OS (30.1% [95% CI 18.9%-42.0%] and 37.7% [95% CI 25.8%-49.5%]; p = 0.89) did not significantly differ. In multivariate analysis, GTR was an independent predictor of PFS (hazard ratio 0.56 [95% CI 0.36-0.86], p = 0.008) and OS (hazard ratio 0.65 [95% CI 0.42-1.01], p = 0.05). The use of 5-ALA FGS generates a significant extra cost of 2732.36€ (95% CI 1658.40€-3794.11€). CONCLUSIONS: The authors found that 5-ALA FGS is an easy-to-use, cost-effective, and minimally time-consuming technique that safely optimizes the extent of resection in patients harboring glioblastoma amenable to a large resection.