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BackgroundThe best management of symptomatic patients with low-gradient (LG) severe aortic stenosis (AS) and preserved left ventricular ejection fraction (LVEF) has not been established. The Randomised study for the Optimal Treatment of symptomatic patients with low-gradient severe Aortic valve Stenosis (ROTAS) trial aimed to assess the superiority of aortic valve replacement (AVR) versus medical treatment (MT) in this specific group of AS patients.MethodsPatients with symptomatic LG severe AS and preserved LVEF (>50%) underwent dobutamine stress echocardiography and/or CT-aortic calcium score to confirm AS severity and were then randomised 1:1 to AVR or MT. The primary endpoint was a composite of overall death and/or cardiovascular hospitalisation.ResultsThe ROTAS study was stopped early because of insufficient recruitment. In the end, only 52 patients (age 79±7 years; women 54%; NYHA III-IV 27%; median STS score 3.3%) were included in the study. During follow-up (mean: 14±7 months), the primary endpoint occurred in 12 (23%) patients. Compared with MT, AVR was not associated with a significant prognostic benefit (events: 5/26 (19%) vs 7/26 (27%) (HR 0.76, 95% CI 0.24 to 2.39, p=0.63). During follow-up, 11 (42%) patients in the MT group developed class I criteria for AVR or severe symptoms justifying a cross-over to the AVR group.ConclusionsBecause of the small number of included patients and short follow-up the ROTAS trial was underpowered and unable to demonstrate a difference in the study endpoint between treatment arms. In patients in the MT arm, a regular echocardiographic and clinical assessment might be useful to disclose those developing class I indications of AVR or severe AS-related symptoms.Trial registration numberNCT01835028.

L’insuffisance cardiaque représente plus de 1,5 million de personnes en France, soit plus de 150 000 hospitalisations par an avec un taux de mortalité à 5ans de 50 % chez les personnes NYHA 3-4. L’assurance-maladie a mis en place depuis 2010 le service PRADO (programme d’accompagnement de retour à domicile) afin d’améliorer la prise en charge des patients avec pour objectif de diminuer les ré-hospitalisations. La télésurveillance basée sur la mesure ambulatoire et quotidienne de paramètres vitaux en association à l’accompagnement thérapeutique, serait un outil supplémentaire et complémentaire du PRADO.

Recognition that inflammation may contribute to the pathogenesis of various cardiac diseases has naturally led to the evaluation of the therapeutic potential of a range of anti-inflammatory approaches. Unfortunately, results in most settings have been disappointing. The majority of novel approaches fail despite promising preclinical data, partly attributable to off-target effects. The purpose of this review, focused on inflammation following acute myocardial ischemia, is to give a brief overview of the new insights regarding research on pro-inflammatory signaling cascades that could be targeted for cardioprotective therapeutic developments.

The new ternary compound Th3Co3Sb4was synthesized by arc melting. The compound crystallizes in the cubic space group I-43d(No. 220) with a=9.572(2) Å and adopts the Y3Au3Sb4structure, which is a filled version of the Th3P4structure type. The crystal structure was solved and refined with single-crystal X-ray diffraction. Final R1/Rw2=1.52/3.73%. Th3Co3Sb4is a metallic ferromagnet at room temperature. Electrical conductivity and thermoelectric power measurements show metallic behavior, which is also predicted by the electronic band structure calculations.

The four new intermetallic aluminum silicides RE4Fe2+xAl7-xSi8 (RE = Ce, Pr, Nd, Sm) crystallize from the reaction of Si, Fe, and RE (or rare earth oxides) in molten Al at 850 °C. All compounds share the same structure type as determined by single-crystal X-ray diffraction analysis. They form in the space group Cmmm (No. 65) with cell constants of a = 10.909(2) Å, b = 16.265(3) Å, c = 4.0804(8) Å, R1 = 0.0196, and wR2 = 0.0486 for the Sm analogue. The crystal structure is a complex three-dimensional network comprised of repeating layers containing Al, Si, and Fe connected by atoms between the layers. The RE³⁺ ions are then located within tunnels of the three-dimensional network, running parallel to the c axis with a coordination number of 14. Magnetic susceptibility measurements indicate that the rare earth ions are in a 3+ oxidation state, whereas the Fe atoms are in an effective diamagnetic state. Electronic band structure calculations, carried out on the hypothetical analogue Y4Fe2Al7Si8, predict metallic behavior and suggest Fe to be in a reduced state with almost filled d orbitals. Variable temperature electrical conductivity and thermopower measurements confirm the metallic nature of the compounds. The charge transport and magnetic properties of the Ce analogue are anomalous and indicative of f^1/0 valence fluctuations at T < 100 K.

Objectives. To describe the clinical manifestations of familial Mediterranean fever (FMF) in 91 patients from 47 families and provide data from the genetic study.
Patients and methods. We conducted a retrospective chart review of 91 patients (including 83 children aged <15 yr) from 47 families through a questionnaire and a specific database. The genetic analysis included complete screening of known mutations of the MEFV gene on chromosome 16p13.3. A positive diagnosis required at least two mutations, one on each chromosome.
Results. Our panel included 52 females and 39 males, with a mean age of 7.27 yr. Of the 47 families, 31 were non-Ashkenazi Jews, 10 were Armenians and six were from other ethnic groups. Clinical features included fever (100%), peritonitis (86%), pleuritis (56%), arthritis (34%) and myalgias (27%). We observed a high rate of cutaneous manifestations (47%); erythema, oedema and recurrent oral ulcers were the most frequent. Phenotype-genotype correlations showed a significant association of M694V homozygosity with earlier age of onset (P = 0.044), fever >39°C (P = 0.002), pleural crisis (P = 0.0044), splenomegaly (P = 0.0005) and arthritis (P = 0.001). Associations with mucocutaneous features were as follows: erysipelas-like erythema (P = 0.012), oedema (P = 0.61, not significant) and oral ulcers (P = 0.45, not significant).
Conclusion. New phenotype-genotype correlations emerged from our study: homozygosity for the M694V mutation was associated with intensity of fever, splenomegaly and with erysipelas-like erythema. Apart from erysipelas-like erythema, no significant association was found between other cutaneous features and the genotype.

Analytical capillary zone electrophoresis (CZE) conditions including concentration, pH value and nature (complexing or non‐complexing) of the running buffer with or without the addition of methanol, capillary temperature and applied voltage, were studied in order to optimize the separation and resolution of a mixture of 13 C‐glycosylflavones and one flavone O‐glucoside. Factors primarily responsible for differences in electrophoretic mobility of flavone C‐glycosides are identical to those defined from the separation of flavonoid O‐glycosides, i.e. number and position of the free hydroxy groups on the flavone skeleton, and number and type of attached sugar groups. Nevertheless, an increase in the pH value from 9 to 10 of the borate running buffer affects the elution order: such an increase induces greater relative electrophoretic mobilities of C‐glycoside derivatives with a free hydroxy group at C‐4′ compared with those of luteolin C‐glycoside derivatives. This change in the migration order could be explained both by a decrease of the net charge linked to an increase and stability of the borate–luteolin complexation and by an increase in molecular size. Moreover the 6‐C‐glycosylflavones present electrophoretic mobilities lower than their homologous 8‐C‐glucosyl isomers. Finally, the study of C‐glucosylflavones from 18 different samples of Passiflora incarnataby CZE shows a great variability in the qualitative composition. Copyright © 2000 John Wiley & Sons, Ltd.

Analytical capillary zone electrophoresis (CZE) conditions including concentration, pH value and nature (complexing or non-complexing) of the running buffer with or without the addition of methanol, capillary temperature and applied voltage, were studied in order to optimize the separation and resolution of a mixture of 13 C-glycosylflavones and one flavone O-glucoside. Factors primarily responsible for differences in electrophoretic mobility of flavone C-glycosides are identical to those defined from the separation of flavonoid O-glycosides, i.e. number and position of the free hydroxy groups on the flavone skeleton, and number and type of attached sugar groups. Nevertheless, an increase in the pH value from 9 to 10 of the borate running buffer affects the elution order: such an increase induces greater relative electrophoretic mobilities of C-glycoside derivatives with a free hydroxy group at C-4' compared with those of luteolin C-glycoside derivatives. This change in the migration order could be explained both by a decrease of the net charge linked to an increase and stability of the borate–luteolin complexation and by an increase in molecular size. Moreover the 6-C-glycosylflavones present electrophoretic mobilities lower than their homologous 8-C-glucosyl isomers. Finally, the study of C-glucosylflavones from 18 different samples of Passiflora incarnata by CZE shows a great variability in the qualitative composition. Copyright © 2000 John Wiley & Sons, Ltd.

Two ternary silicides, MNiSi3 (M = Sm, Y), have been synthesized from Sm, Ni, and Si in molten Ga at 850 °C in sealed silica tubes. Both compounds form black shiny crystals and are stable even in aqua regia. The structures, determined by single-crystal X-ray diffraction, are orthorhombic, Cmmm (No. 65) with Z = 4, and have lattice parameters a = 3.965(2) Å, b = 21.144(2) Å, c = 4.007(1) Å for M = Sm and a = 3.930(2) Å, b = 21.021(2) Å, c = 3.960(1) Å for M = Y, respectively. Refinement based on Fo² yielded R1 = 0.0319 and wR2 = 0.0712 [I > 2 σ(I)] for M = Sm and R1 = 0.0267 and wR2 = 0.0688 [I > 2σ(I)] for M = Y. The compounds adopt the SmNiGe3 structure type with zigzag Si chains and Si dimers and exhibit metallic p-type electrical conductivity. Variable temperature magnetic susceptibility data suggest that Sm is 3+ and Ni has no magnetic moment. SmNiSi3 has an antiferromagnetic transition at 12 K and follows the modified Curie−Weiss law above 12 K. Band structure calculations using density functional theory, generalized gradient approximation, full potential LAPW method, and also extended Hückel tight binding theory show that the materials are metallic and suggest that Ni is either neutral or in a reduced oxidation state. Additional insight into the bonding was obtained by extended Hückel calculations carried out on the [NiSi3]^3- framework under the assumption that Y is mostly 3+. These results suggest that the Si zigzag chain contains single bonds with a partial double bond character.

A new quaternary silicide, Sm2Ni(NixSi1-x)Al4Si6 (x = 0.18−0.27), has been synthesized from Sm2O3, NiO, and Si or Sm, Ni, and Si in Al metal flux at 800 °C. The structure, determined by single-crystal X-ray diffraction, is tetragonal, space group P4/nmm (No. 129) with Z = 2, and lattice parameters a = b = 5.8060(3) Å, c = 14.845(1) Å. Refinement based upon F² [I > 2σ(I)] yielded R1 = 0.0252 and wR2 = 0.0634. The compound exhibits a new structure type containing two different alternating layers which are linked together through Si/Ni−Si bonds to form a three-dimensional framework. One layer is formed by edge-shared (the edges parallel to c) NiAl8 cubes. The other layer is a Si-based net which consists of six-member, five-member, and square planar rings. The structure of this compound cannot be rationalized on the basis of the Zintl−Klemm concept. Extended Hückel, tight binding calculations were carried out for different hypothetical stoichiometries, besides the observed one, of the compound Sm2Ni(NixSi1-x)Al4Si6. Five models were investigated with x = 0, 0.25, 0.5, 0.75, and 1. The compounds with x = 0 and 0.25 are predicted to be more stable than the others. Electrical conductivity and thermopower data indicate that the compound is p-type metallic. The temperature-dependent magnetic susceptibility exhibits an antiferromagnetic ordering near 60 K and a weak ferromagnetic (WF) transition near 17 K. High temperature (150−300 K) magnetic susceptibility data suggest that Sm is in the 3+ oxidation state.

La drépanocytose est la maladie génétique la plus répandue en France avec une prévalence dominée par l’Île de France et les territoires d’outre-mer. L’atteinte vasculaire cérébrale représente une complication grave et précoce des syndromes drépanocytaires. L’écho-Doppler transcrânien permet d’identifier les enfants à plus haut risque d’ischémie cérébrale, mais les critères de dépistage d’une vasculopathie cérébrale chez l’adulte drépanocytaire ne sont pas établis.