Aims. - To assess the practical implementation of international guidelines and their impact on syncope management in a 500-bed general hospital.Patients and methods. - Three groups of 63 consecutive patients admitted for syncope to the emergency care unit (ECU) were studied: group 1, before the guidelines delivered to the practitioners, group 2 immediately after the diffusion of guidelines and group 3, one year later. The study evaluates the mean duration of stay (MDS) and the relevance of the diagnostic strategy.Results. - In group 1 compared to group 2, MDS were respectively 6.8 +/- 5.5 and 5.4 +/- 2.8 days (P = 0.07) and the unexplained syncope number respectively 22% and 24% (P = 0.8). The search of orthostatic hypotension became more systematic (13% versus 86% in group 1 and 2 respectively, P < 0.001). The agreement (kappa coefficient) between initial and final diagnostic increased in 0.34 to 0.44. One year later MDS in group 3 was 7.1 +/- 4.7 clays (P=0.8 versus group 1 and P=0.015 versus group 2) with only 6.3% systematic search for orthostatic hypotension (P < 0.001).Conclusions. - Guidelines optimize the syncope management in the ECU and the agreement between the emergency and discharge diagnostic without change of unexplained syncope and. MDS tend to be shorter when guidelines are actively implemented. Nevertheless, the positive impact of guidelines implementation is of limited duration. (C) 2012 Elsevier Masson SAS. All rights reserved.

Non-specific inflammation occurs at the time of acute thromboembolic disease and makes it difficult to interpret the measurement of some coagulation factors, such as factor (F) VIII:C and FXI [1]. An increase in FVIII:C is a risk factor for venous thromboembolic disease (VTE), and each 10 IU dL−1 increase in FVIII results in a 10% increase in the risk of VTE [2-4]. Limited data suggest that an increase in FXI is also a risk factor for VTE [5]. Studies by O'Donnel et al. [6] and Kamphuisen et al. [7] pointed out that the risk for VTE conferred by increased levels of FVIII:C is not due to acute-phase reactants such as C-reactive protein (CRP), and a temporal association has not been demonstrated between markers of inflammation and the development of VTE. FVIII:C and FXI are usually measured at least 3 months after acute VTE. However, no data are available regarding the optimal time to measure these coagulation factors after acute VTE. We therefore investigated the plasma levels of FVIII:C and FXI in the year following an acute event. Unselected cancer-free patients were included in the study at the time of acute deep venous thrombosis or pulmonary embolism proven by objective testing (Duplex ultrasound for deep vein thrombosis, CT scan for pulmonary embolism). FVIII:C and FXI were serially measured at the time of acute VTE (M0), and 3 (M3), 6 (M6) and 12 months (M12) after, together with erythrocyte sedimentation rate (ESR), CRP and fibrinogen. FVIII:C and FXI were measured by a one-stage chronometric method (STA automaton, STA®-Deficient VIII plasma and STA®-Deficient XI plasma, C.K. Prest reagent, STA Stago, Gennevilliers, France). Fibrinogen was measured by the von Clauss coagulometric method (STA Stago; Fibriquick Thrombin reagent, Biomerieux, Marcy l'Etoile, France). Statistical analysis was performed using Spearman's correlation test to study the correlation between FVIII:C or XI and the inflammatory markers. Wilcoxon's test was used to compare the levels of FVIII:C and XI at different times. The study population included 42 patients (22 females, 20 males, mean age 68 ± 16 years). All patients were followed up for 6 months, and 33 patients for 12 months. VTE was provoked in six patients (five recent surgery, one intensive care) and 36 patients had spontaneous VTE. The median duration of anti-vitamin K treatment was 11 months (range 7–16 months) and 19 patients were still treated with anti-vitamin K at M12. Levels of FVIII:C significantly decreased at M3, M6 and M12 (Table 1). Results were quite similar in the patients with unprovoked VTE, the FVIII:C median levels (range) were 207% (97–385), 193% (90–430), 182% (93–375) and 189% (95–441) at M0, M3, M6 and M12 respectively. In the whole sample, as in the unprovoked thrombosis group, decrease was significant at all times when compared with baseline but the decrease was not significant between M3 and M6 or M6 and M12. Three patients experienced late decrease below 150% at M6 and three at M12. A significant correlation was present only at M0 between FVIII:C and CRP (r = 0.38, P = 0.01) or ESR (r = 0.37, P = 0.04), but not subsequently. No correlation was found between fibrinogen and FVIII:C or FXI at any time. FXI did not significantly decrease after M0; only one patient with moderately elevated FXI (218%) experienced a decrease below 150%. No correlation was found at any time between FXI and CRP, ESR or fibrinogen. In conclusion, FVIII:C should be measured at least 3 months, preferably 12 months, after acute VTE. If FVIII:C is measured at M3 and found to be increased, another measurement should be done at M12. FXI level is not correlated with inflammatory parameters and can be measured at any time after acute VTE.

BACKGROUND:B-type peptide assay (brain natriuretic peptide [BNP] and N-terminal prohormone brain natriuretic peptide [NT-proBNP]) is useful for the diagnosis of heart failure (HF), but few data are available on the use of these markers in elderly subjects. The aim of this study was to evaluate NT-proBNP assay for the diagnosis of acute left HF in patients older than 70 years hospitalized for acute dyspnea. METHODS:We prospectively enrolled 256 elderly patients with acute dyspnea. They were categorized by 2 cardiologists unaware of NT-proBNP values into a cardiac dyspnea subgroup (left HF) and a noncardiac dyspnea subgroup (all other causes). RESULTS:Mean age was 81 +/- 7 years, and 52% of the patients were women. The diagnoses made in the emergency setting were incorrect or uncertain in 45% of cases. The median NT-proBNP value was higher (P < .0001) in patients with cardiac dyspnea (n = 142; 7906 pg/mL) than in patients with noncardiac dyspnea (n = 112; 1066 pg/mL). The area under the receiver operating characteristic curve was 0.86 (95% CI 0.81-0.91). At a cutoff of 2000 pg/mL, NT-proBNP had a sensitivity of 86%, a specificity of 71%, and an overall accuracy of 80% for cardiac dyspnea. The use of 2 cutoffs (< 1200 and > 4500 pg/mL) resulted in an 8% error rate and a gray area englobing 32% of values. CONCLUSION:NT-proBNP appears to be a sensitive and specific means of distinguishing pulmonary from cardiac causes of dyspnea in elderly patients. An optimal diagnostic strategy requires the use of 2 cutoffs and further investigations of patients with values in the gray area.

L’association MURCS comporte une aplasie des dérivés mülleriens (MU), des malformations rénales (R) et des malformations des somites cervicothoraciques (CS). C’est une affection rare (1/50 000 femmes) et sporadique, sans étiologie connue. Dans l’observation rapportée, il existe deux anomalies qui n’ont pas été décrites, une atrésie de l’œsophage et un tératome mature ovarien. L’atrésie de l’œsophage a fait initialement porter le diagnostic d’association VACTERL, plus connue car plus fréquente, illustrant ainsi la difficulté à différencier ces associations malformatives qui présentent des anomalies communes et des limites floues. La présence d’une hypoplasie des dérivés mülleriens a fait corriger le diagnostic en faveur du MURCS car il n’existe pas d’hypoplasie des dérivés mülleriens dans l’association VACTERL. En pratique, la présence de malformations rénales et cervicales isolées ou associées à d’autres éléments de l’association VACTERL doit inciter à rechercher une anomalie des dérivés mülleriens.

MURCS association includes Mullerrian duct aplasia-hypoplasia (MU), renal malformations (R) and cervicothoracic somite dysplasia (CS). This rare disease (1/50 000 females) is sporadic and of unknown aetiology. The reported case is the first one with additional esophageal atresia and ovarian mature teratoma. Esophageal atresia first led to the diagnosis of VACTERL association, which is more frequent and well known, showing that the identification of such malformative association may be challenging. The presence of mullerrian abnormality has allowed the diagnosis of MURCS association, as there is no mullerrian hypoplasia in VACTERL association. Therefore the association of isolated or combined renal and cervical malformation with VACTERL features should lead to the search for mullerrian abnormalities.

La prise en charge de qualité d'un patient suivi en oncologie pédiatrique dépasse largement le cadre des traitements anti-tumoraux et englobe un certain nombre de thématiques en lien avec les soins de support. Certains, communs aux soins de support proposés en oncologie adulte, comme la prise en charge de la douleur et le support nutritionnel présentent cependant des particularités pédiatriques. D'autres sont plus spécifiques à l'enfant comme la scolarité et l'information au jeune patient et exigent un cadre précis et des outils adaptés. Le jeune âge des patients et l'amélioration des taux de survie en oncologie pédiatrique conduisent également à interroger la temporalité des soins de support en ayant une réflexion sur leur accès au-delà de la phase active de la maladie. La synthèse présentée explore ces différents sujets : l'information et la communication au patient et à ses parents, l'évaluation et la gestion de la douleur, le soutien nutritionnel mais aussi la scolarisation et le suivi à long terme et le dépistage des séquelles induites par la maladie et ses traitements.Organization of health care of a patient followed in pediatric oncology is not limited to cancer treatment. It includes a whole range of supportive care. Some are common to the supportive care offered in adult oncology, such as pain management and nutritional support. However, there are pediatric specificities. Others are more peculiar to children, such as education and information for young patients, and require a specific framework and innovative tools. The young age of patients and the improvement in survival rates in pediatric oncology also lead to questioning the temporality of supportive care by considering access to supportive care beyond the active phase of the disease. This review explains some of these different specificities: information and communication to the patient and his parents, assessment and management of pain, nutritional support but also schooling, long-term follow-up and screening sequelae induced by the disease and its treatments.

This paper presents a critical assessment of protein C (PC) and protein S (PS) functional and immunological approaches with regard to DNA sequencing in a large hospital recruitment for thrombosis exploration in more than 1700 consecutive patients. After examination of clinical status and PC and PS phenotype, a genotypic study was implemented for 17 PC-deficient and 28 PS-deficient patients (activity < 70%). Sixty-five percent of the genotyped PC-deficient patients were found to have heterozygous mutations. Among the < 70% values, decreases in PC activity without gene mutation were always slight (mean value 64 +/- 7%) while patients presenting a PC gene mutation had a mean 50 +/- 17% activity (P < 0.05). Among the eight PC mutations found, only one has previously been described. A novel mutation in the promoter region (-1522), located in the HNF-1 site and associated with the Y226H heterozygous mutation, was found in a 9-month-old girl with 4% PC activity. Determination of PS functional activity was considerably improved by contemporaneous measurement of calibration and samples in a single step. Only 50% of the genotyped PS-deficient patients demonstrated heterozygous alterations of the gene. The benefit of sequencing to identify putative causal mutations was only 39% in PS-deficient women, while it was 90% in men. Among the nine PS mutations found, six have not yet been published. In the present paper, we explain our methodological choices and diagnostic strategy.

Samples with Bi2·05Sr1·9Ca1·05(Cu1-xFex)2O8+δ(0≤x≤0·15) compositions were synthesized by a liquid-mix process able to give single phase compounds. The superconducting phase was carefully investigated by SEM and EDX. Substitution of x Fe atoms in the Bi2Sr2CaCu2O8+δ grains corresponds to the removal of 1·7x Cu atoms and to the addition of 0·75x Bi atoms whereas the sum Sr+Ca remains quite constant. In a first step, this evolution may be explained by the following basic model: Fe2O3→2Fecu•+Vcu″+3 CuO which assumes the creation of vacancies in the copper sites of the Bi2Sr2CaCu2O8+δ lattice. However, the apparent increase of Bi content suggests the formation of microdomains of Bi2Sr2CuO6 phase intergrown in the Bi2·05Sr1·9Ca1·05(Cu1-xFex)2O8+δ grains, as inferred from X-ray diffraction and SEM which reveals the concomitant (SrCa)14Cu24O41 secondary phase. The EDX results are more consistent with a Bi2Sr2CuO6–Bi2Sr2CaCu2O8+δ intergrowth model in which Fe is accommodated in the Bi2Sr2CuO6 microdomains than with the model of substitution in a Bi2Sr2CaCu2O8+δ single phase.

Antiphospholipid/cofactor antibodies are detected in only 60% of patients with systemic lupus erythematosus (SLE) with thrombosis.1 Therefore, we studied thrombophilia factors and their relation with thrombosis in patients with SLE. Forty eight consecutive patients with SLE were included (39 women, 9 men), 15 with and 33 without past thrombosis (Th and NTh group, respectively). Twenty thrombotic events were identified: 17 deep venous and 1 arterial thrombosis, 2 osteonecrosis. Both groups had comparable clinical, biological, therapeutic data, and mean (SD) SLE disease activity index (SLEDAI) (5 (4.6) v 5.3 (4.8)). Patients were examined at least one month after thrombosis (>3 months in 11 out of 15). The following parameters were determined: protein C, total and free …

AIM:To compare the haemodynamic status during high-frequency oscillatory ventilation and conventional mechanical ventilation in very preterm infants with respiratory distress syndrome. METHODS:Thirty-two neonates of less than 30 wk gestation randomly assigned to high-frequency oscillatory ventilation (n = 15) or conventional mechanical ventilation (n = 17) had three echocardiographies and one cerebral Doppler-echography under the same ventilation during the first 48 h of life. RESULTS:Mean airway pressure was 2 cm H2O higher in infants ventilated with high-frequency oscillatory ventilation at the different echocardiographies. Comparable right ventricular indexes were observed in the two groups. Reduction of the ductus arteriosus diameter and ductal closure were significant only in neonates ventilated conventionally. Left ventricular performance and left ventricular contractility did not differ between the groups. The high-frequency group had lower end diastolic velocity and a higher resistance index in the anterior cerebral artery. CONCLUSION:Compared with conventional mechanical ventilation, high-frequency oscillatory ventilation was achieved without altering cardiac function. However, the inability of the left ventricle to improve its performance in the presence of a significant ductal shunt suggests a narrow range of optimal pressures under this ventilatory mode.