Measurement of the

^{140}Ce(n,γ) is a key reaction for slow neutron-capture (s-process) nucleosynthesis due to being a bottleneck in the reaction flow. For this reason, it was measured with high accuracy (uncertainty ≈5%) at the n_TOF facility, with an unprecedented combination of a high purity sample and low neutron-sensitivity detectors. The measured Maxwellian averaged cross section is up to 40% higher than previously accepted values. Stellar model calculations indicate a reduction around 20% of the s-process contribution to the Galactic cerium abundance and smaller sizeable differences for most of the heavier elements. No variations are found in the nucleosynthesis from massive stars.

Identification of the first homozygous intragenic deletion in the YY1AP1 gene in a consanguineous family: New insights into the phenotypic variability associated with Grange syndrome.

Grange syndrome (GRNG-MIM#135580) is a rare recessive disorder associating variable features including diffuse vascular stenosis, brachysyndactyly, osteopenia with increased bone fragility, cardiac malformations, and variable developmental delay. Since its first description in 1998, only 15 individuals from 10 families have been reported, carrying homozygous or compound heterozygous frameshift or nonsense variants in YY1AP1. In a patient with cutaneous and bone syndactyly and a hemorrhagic stroke at the age of 16 months, consistent with a clinical diagnosis of GRNG, we performed exome sequencing after negative array-CGH and congenital limb malformation panel results. Copy number variant analysis from exome data identified a homozygous intragenic out-of-frame deletion of 1.84 kb encompassing exons seven and eight of YY1AP1, confirming a molecular diagnosis of GRNG. Genetic counseling led to the identification of additional family members compatible with GRNG. Here, we provide new insights into the phenotypic variability associated with GRNG and highlight the utility of the detection of small copy number variants to identify the molecular causes of heterogeneous malformative genetic disorders.

74 Ge( n , γ ) cross section below 70 keV measured at n_TOF CERN.

Neutron capture reaction cross sections on 74 Ge are of importance to determine 74 Ge production during the astrophysical slow neutron capture process. We present new resonance data on 74 Ge( n , γ ) reactions below 70 keV neutron energy. We calculate Maxwellian averaged cross sections, combining our data below 70 keV with evaluated cross sections at higher neutron energies. Our stellar cross sections are in agreement with a previous activation measurement performed at Forschungszentrum Karlsruhe by Marganiec et al., once their data has been re-normalised to account for an update in the reference cross section used in that experiment.

[Prevalence and Risk Factors of Chlamydia Trachomatis Infection Among Women Consulting at the Sexually Transmitted Infection Centre in la Reunion: A Cross-Sectional Study]. / Prévalence et Déterminants de L'infection à Chlamydia Trachomatis chez les Femmes Consultant en Centres de Dépistage à la Réunion: Une Étude Transversale.

Introduction: Chlamydia trachomatis (CT) infection is the commonest bacterial sexually transmitted infection (STI) in the world. Often asymptomatic, it can lead to significant complications in women. In France, since 2003, systematic screening for CT in STI center has been recommended for women aged less than 25 year. The main objective of this study was to determine CT prevalence in patients attending STI centers in Reunion Island. The second objective was to explore the determinants of this infection. Method: A cross-sectional survey using an anonymous questionnaire was conducted among women attending STI center in two hospitals in western and southern Reunion Island during one year. All women who had performed a CT PCR, based on vaginal self-swabs, were included. Results: Among the 620 patients tested, the prevalence of infection was 6.6% (95% CI [4.7-8.6]). By age group, the highest prevalence was between 12 and 17 years with 14.3% positive tests compared to 7.5% and 3.9% respectively in 18-24 and 25-67 year age group (p = 0.003). The risk factors for CT were a young age (p = 0.02), a first sexual intercourse between 11 and 14 years old (p = 0.01), lack of previous STI screening history (p = 0.02), and the following motives for seeking screening: "partner unfaithfulness" (p = 0.01) and "infected partner" (p = 0.02). Conclusion: This study highlights the high prevalence of CT among Reunionese minors. A more systematic screening and a reinforcement of STI awareness among young people in Reunion Island seem to be essential.

Bordetella bronchiseptica infection.

OBJECTIVE: To collect data of all patients admitted to hospital with a positive test to Bordetella bronchiseptica between 2001 and 2015. METHODS: We performed a retrospective monocentric study of all hospitalized patients over the past 15 years with a positive test to B. bronchiseptica. RESULTS: Nine patients were included between 2001 and 2015; two presented with infectious relapses, i.e. a total of 14 positive test samples were observed. Age, induced immunodeficiency, and preexisting respiratory illnesses are risk factors. All patients showed symptoms at sample collection and the infection was exclusively respiratory. The diagnosis was obtained through a cytobacteriological test of sputum, bronchial aspiration, or bronchial fibroscopy with a bronchoalveolar lavage. The drug susceptibility test revealed a natural resistance to cephalosporins including ceftazidime, monobactam, and fosfomycin. There were cases of resistance to penicillin A and to the trimethoprim/sulfamethoxazole association. The classically used antibiotic treatment for community-acquired pneumonia is based on probability and may thus fail. Four patients died. The duration and nature of the antibiotics to use have not been codified. CONCLUSION: B. bronchiseptica infection mainly affects the elderly. All patients should be treated, regardless of the importance of the inoculum, and all infected animals should be treated.

The impact of using electrical impedance spectroscopy (ZedScan) on the performance of colposcopy in diagnosing high grade squamous lesions of the cervix.

OBJECTIVES: To determine the efficacy of colposcopy plus ZedScan, which measures changes in tissue electrical impedance, for detecting intraepithelial high-grade lesions compared to colposcopy alone. METHODS: A prospective study conducted at a university hospital colposcopy clinic. Patients referred following abnormal cervical cytology or colposcopic follow up were examined by colposcopy plus ZedScan to assess the cervix. The results of ZedScan directed and colposcopically directed biopsies were compared. RESULTS: Ninety-one patients were included in this study. The median age was 33 years. Eighty (88%) were referred with abnormal cytology; LSIL 45%, ASCUS 27%, ACS-H 8%, HSIL 7%, AGC 1% and 12% follow-up postconisation or colposcopic follow up. Colposcopy alone detected 18 high-grade lesions with 64 women undergoing biopsy with a total of 115 biopsies being taken with a sensitivity of 60% and NPV estimated at 81.7%. ZedScan with colposcopy increased the detection of high-grade lesions by 47.3%, identifying 27 high-grade lesions and one case of invasive cancer. Sensitivity was 93.1% and NPV estimated at 91.3%. A combination of normal colposcopy practice and ZedScan had a sensitivity and NPV of 100%. CONCLUSION: ZedScan used in conjunction with the colposcopy improves sensitivity in detecting high-grade lesions at the expense of a moderate increase in the number of biopsies.

Relating specific connexin co-expression ratio to connexon composition and gap junction function.

Cardiac connexin 43 (Cx43), Cx40 and Cx45 are co-expressed at distinct ratios in myocytes. This pattern is considered a key factor in regulating the gap junction channels composition, properties and function and remains poorly understood. This work aims to correlate gap junction function with the connexin composition of the channels at accurate ratios Cx43:Cx40 and Cx43:Cx45. Rat liver epithelial cells that endogenously express Cx43 were stably transfected to induce expression of accurate levels of Cx40 or Cx45 that may be present in various areas of the heart (e.g. atria and ventricular conduction system). Induction of Cx40 does not increase the amounts of junctional connexins (Cx43 and Cx40), whereas induction of Cx45 increases the amounts of junctional connexins (Cx43 and Cx45). Interestingly, the non-junctional fraction of Cx43 remains unaffected upon induction of Cx40 and Cx45. Co-immunoprecipitation studies show low level of Cx40/Cx43 heteromerisation and undetectable Cx45/Cx43 heteromerisation. Functional characterisation shows that induction of Cx40 and Cx45 decreases Lucifer Yellow transfer. Electrical coupling is decreased by Cx45 induction, whereas it is decreased at low induction of Cx40 and increased at high induction. These data indicate a fine regulation of the gap junction channel make-up in function of the type and the ratio of co-expressed Cxs that specifically regulates chemical and electrical coupling. This reflects specific gap junction function in regulating impulse propagation in the healthy heart, and a pro-arrhythmic potential of connexin remodelling in the diseased heart.

About three cases of ulceroglandular tularemia, is this the re-emergence of Francisella tularensis in Belgium?

Tularemia is a zoonosis caused by Francisella tularensis that can be transmitted by several ways to human being and cause different clinical manifestations. We report three clinical cases of tularemia with ulceroglandular presentation in young males acquired during outdoor activities in Southern Belgium. Confirmation of the diagnosis was established by serology. Only three cases of tularemia have been reported in Belgium between 1950 and 2012 by the National Reference Laboratory CODA-CERVA (Ref Lab CODA-CERVA) but re-emergence of tularemia is established in several European countries and F. tularensis is also well known to be present in animal reservoirs and vectors in Belgium. The diagnosis of tularemia has to be considered in case of suggestive clinical presentation associated with epidemiological risk factors.

Bk and Cf chromatographic separation and ²49Bk/²48Cm and ²49Cf/²48Cm elemental ratios determination by inductively coupled plasma quadrupole mass spectrometry.

The French Atomic Energy Commission has carried out several experiments for the study of minor-actinide transmutation processes in high intensity thermal neutron flux. In this context a Cm sample enriched in (248)Cm (∼97%) was irradiated in a thermal neutron flux at the High Flux Reactor (HFR) of the Laue-Langevin Institute (ILL). The precise and accurate determination of Cf isotope ratios and of (249)Bk/(248)Cm and (249)Cf/(248)Cm elemental ratios in the (248)Cm irradiated sample is crucial for the calculation of actinide neutron capture cross-sections. This work describes an analytical procedure for the separation and the isotope ratio measurement of Bk and Cf in the irradiated sample. The Bk and Cf separation is based on a lanthanides separation protocol previously developed by the laboratory. Well-defined retention times for Bk and Cf were obtained by coupling the Ionic Chromatography (IC) with an ICP-QMS. All conditions of element separation by IC and the different steps of the analytical protocol in order to obtain the isotopic and elemental ratios are presented. Relative uncertainties of Cf isotopic ratios range from 0.3% to 0.5% and the uncertainty of the (249)Bk/(248)Cm and (249)Cf/(248)Cm elemental ratios are respectively 6.1% and 3.2%. This level of uncertainty for both isotopic and elemental ratios is in perfect agreement with the requirement for transmutation studies.

Beyond UV radiation: a skin under challenge.

Since ancient times, human beings have been trying to protect their skin against the adverse effects of the sun. From the first mineral sunscreens used by Egyptians, to the current more sophisticated ultraviolet (UVA/UVB) organic sunscreens, progress has been made in terms of sun protection and deeper knowledge of skin physiology has been acquired in the process. The solar spectrum is composed of radiations of various wavelengths having specific, as well as overlapping effects on skin. UVB is mainly responsible for sunburn and DNA dimer formation that can lead to mutation. UVA generates oxidative reactions affecting DNA, proteins and lipids, and is also immunosuppressive. Recently, visible light and infrared radiation (IR) have been associated with oxidative damage and IR has been additionally linked to adverse heat effects on skin. Numerous other extrinsic factors, related to environment and lifestyle, also affect the appearance of skin, precipitating ageing. New molecular mechanisms linking sun and environmental factors to skin ageing have been identified: IR affects mitochondrial integrity and specific heat receptors also mediate some of its effects, tryptophan is a chromophore for UVB, and the aryl hydrocarbon receptor (AhR) is activated by light and xenobiotics to alter skin physiology. Integrating all these new elements is changing the way we think about skin extrinsic ageing. Is UVA/UVB sunscreen protection still enough for our skin?

Terahertz quantum cascade lasers operating up to ∼ 200 K with optimized oscillator strength and improved injection tunneling.

A new temperature performance record of 199.5 K for terahertz quantum cascade lasers is achieved by optimizing the lasing transition oscillator strength of the resonant phonon based three-well design. The optimum oscillator strength of 0.58 was found to be larger than that of the previous record (0.41) by Kumar et al. [Appl. Phys. Lett. 94, 131105 (2009)]. The choice of tunneling barrier thicknesses was determined with a simplified density matrix model, which converged towards higher tunneling coupling strengths than previously explored and nearly perfect alignment of the states across the injection and extraction barriers at the design electric field. At 8 K, the device showed a threshold current density of 1 kA/cm2, with a peak output power of ∼ 38 mW, and lasing frequency blue-shifting from 2.6 THz to 2.85 THz with increasing bias. The wavelength blue-shifted to 3.22 THz closer to the maximum operating temperature of 199.5 K, which corresponds to ∼ 1.28hω/κB. The voltage dependence of laser frequency is related to the Stark effect of two intersubband transitions and is compared with the simulated gain spectra obtained by a Monte Carlo approach.

Hepatitis C infection markers among blood donors in Kisangani, Democratic Republic of Congo

Objectives: The role of blood transfusion in the spread of hepatitis C virus (HCV) is of concern in the DRC. Screened since the end of 2004 in blood donors, few data are however available on HCV in Kisangani. A study is needed to determine the seroprevalence of HCV in blood donors. Patients and method: 1247 blood samples collected from all volunteer blood donors who donated blood from August 1, 2005 to April 30, 2006 at the Provincial Blood Transfusion Centre were tested for anti-HCV antibodies. At the same time as HCV serology, markers for HIV and HBV were tested. Results: A total of 51(4.1%) volunteer blood donors (Table I) were HCV antibody positive. Fifty-two (4.2%) of the subjects were HIV positive and 60 (4.8%) were HBV positive. The mean age of HCV-positive donors was 31.4 years (±13.1) (Table II). HCV-positive seropositivity is lower among donors aged 17 to 24 years compared with those aged 25 years and older (p < 0.05). Positive HCV seropositivity is not related to gender. Conclusion: The seroprevalence of hepatitis C virus is relatively high like that of HIV among volunteer blood donors in Kisangani. It justifies that every blood donor be tested for HCV in order to prevent its transmission in Kisangani

Multiple transfusions for sickle cell disease in the Democratic Republic of Congo: the importance of the hepatitis C virus.

BACKGROUND AND OBJECTIVES: Improvement of transfusion security in sub-Saharan countries requires the determination of priorities taking into account the specific context. PATIENTS AND METHODS: One hundred and forty patients with sickle cell disease (SCD) from one clinical centre for SCD in Kisangani, DRC were tested for HBsAg, anti-HIV antibodies, anti-HCV antibodies and for alloantibodies against red blood cells and human leucocyte antigens (HLA). RESULTS: Thirteen patients had not been transfused and were free of HBV, HIV or HCV infection. HBV, HIV and HCV infections were detected in 2/127 (1.6%), 1/127 (0.9%) and 10/127 (7.9%) transfused patients, respectively. All ten cases of HCV infection were associated with patients who had transfusions prior to the introduction of HCV testing in 2004 (P=0.043). Red blood cells and HLA alloantibodies were detected in 13/127 (10%) and 2/127 (1.6%), respectively. CONCLUSION: HCV testing should be a priority. The rhesus (Rh) phenotype, mainly the RhD antigen and the Kell antigen should be assessed in SCD patients. Further extended phenotyping and deleucocytation should not be considered as priorities.

Electrically switching transverse modes in high power THz quantum cascade lasers.

The design and fabrication of a high power THz quantum cascade laser (QCL), with electrically controllable transverse mode is presented. The switching of the beam pattern results in dynamic beam switching using a symmetric side current injection scheme. The angular-resolved L-I curves measurements, near-field and far-field patterns and angular-resolved lasing spectra are presented. The measurement results confirm that the quasi-TM(01) transverse mode lases first and dominates the lasing operation at lower current injection, while the quasi-TM(00) mode lases at a higher threshold current density and becomes dominant at high current injection. The near-field and far-field measurements confirm that the lasing THz beam is maneuvered by 25 degrees in emission angle, when the current density changes from 1.9 kA/cm(2) to 2.3 kA/cm(2). A two-dimension (2D) current and mode calculation provides a simple model to explain the behavior of each mode under different bias conditions.

Prevalence of sickle cell disease in a northeastern region of the Democratic Republic of Congo: what impact on transfusion policy?

Transfusion therapy may save the lives of patients with sickle cell disease (SCD), but it is also associated with a high risk of transmission of infection. The aims of this study were to determine the prevalence of SCD in a northeastern region of the Democratic Republic of Congo (DRC), and to define ways in which the procedures involved in the security of transfusions should be improved. During a 3-month period in 2006/2007, 520 samples of umbilical cord blood were obtained through neonatal screening in five health centres in Kisangani. The samples were analysed using an isoelectric focusing technique. The estimated prevalence of sickle cell trait and SCD in the population tested was 23.3 and 0.96%, respectively. These numbers will be presented for the attention of the health authorities in DRC with responsibility for SCD and they will be asked to consider improvements in treatment procedures for SCD, such as blood transfusions, as a public health priority.

Childhood spinal muscular atrophy induces alterations in contractile and regulatory protein isoform expressions.

AIMS: Although modifications of the survival motor neurone gene are responsible for most spinal muscular atrophy (SMA) cases, the molecular pathophysiology and the muscular target proteins involved are still unknown. The aim of this study was to compare the expression of contractile and regulatory protein isoforms in quadriceps muscles from SMA children with age-matched control quadriceps. METHODS: The isoform patterns of myosin heavy chains (MHC), troponin subunits (T, C and I) and tropomyosin were determined by immunoblotting, reverse transcription-polymerase chain reaction and mass spectrometry analyses. Depending on the disease severity, their expression levels were followed in specific variants of SMA populations (types I, II and III), with comparison with age-matched control muscles. RESULTS: The isoform transitions in SMA muscles were different from the fast-to-faster transitions occurring in normal muscles from children aged 1 month to 5 years old. Moreover, the expression of the neonatal MHC isoform was not repressed in SMA muscles. CONCLUSIONS: The presence of the neonatal MHC isoform in SMA muscles indicates an alteration of the phenotype in these diseased muscles. It is strongly suggested that MHC and troponin T proteins may be good markers for the SMA pathology.

Heart valve disease associated with treatment with ergot-derived dopamine agonists: a clinical and echocardiographic study of patients with Parkinson's disease.

OBJECTIVE: To elucidate the association between treatment with ergot-derived dopamine agonists (EDDA) and valvular abnormalities amongst patients with idiopathic Parkinson's disease (IPD) and secondly, to analyse the yield of clinical screening for valvular heart disease. DESIGN: A cross-sectional controlled study. SETTING: The cohort of IPD patients treated in the outpatient clinic, Department of Neurology, Aarhus University Hospital, Denmark. SUBJECTS: A total of 138 IPD patients [median age 64 (39-87) years, 62% men] treated with either EDDA (n = 85) or non-EDDA (n = 53) for at least 6 months. Interventions. Patients were screened for valvular heart disease by clinical means and by examiner-blinded echocardiography. Main outcome measure was valvular regurgitation revealed by echocardiography. RESULTS: Severe aortic regurgitation (n = 4) or moderate aortic (n = 12), mitral (n = 3) or tricuspidal valve regurgitation (n = 5) was found in 22 EDDA patients (25.9%). Two patients had coexistent moderate mitral and tricuspid valvular regurgitation. Two non-EDDA patients had moderate valve insufficiency (3.8%, P < 0.05). The adjusted relative risk for at least moderate valve insufficiency in the EDDA patients was 7.2% (P < 0.05). The sensitivity of detecting at least moderate valvular disease by cardiac murmur, dyspnoea, or the heart failure marker NT-proBNP (natriuretic peptide) was 62% for the neurologists and 93% for the cardiologist but with equally low specificity (30-35%). CONCLUSION: EDDA was associated with a clinically important and statistically significant risk of at least moderate valve regurgitation. Clinical screening for valve disease was inadequate and it seems advisable to offer EDDA patients control with echocardiography.

Severe tongue protrusion dystonia: clinical syndromes and possible treatment.

We describe intermittent or sustained severe involuntary tongue protrusion in patients with a dystonic syndrome. Speech, swallowing, and breathing difficulties can be severe enough to be life threatening. Causes include neuroacanthocytosis, pantothenate kinase-associated neurodegeneration, Lesch-Nyhan syndrome, and postanoxic and tardive dystonia. The pathophysiology of intermittent severe tongue protrusion remains unknown. Tongue protrusion dystonia is often unresponsive to oral drugs but may benefit from botulinum toxin injections into the genioglossus muscle. Bilateral deep brain pallidal stimulation was beneficial in two cases.

Earlier occurrence of severe alopecia areata in HLA-DRB1*11-positive patients.

BACKGROUND: Alopecia areata (AA) is a polygenic immune-mediated disorder affecting the hair follicle for which an association with human leukocyte antigen HLA-DRB1*11 has been described. OBJECTIVE: Two parameters including age of onset and extent of the disease (patchy AA and AT/AU forms) were correlated with the presence or absence of HLA-DRB1*11 and its alleles in 88 severe AA patients. METHODS: Patients and healthy controls were typed for HLA-DR and -DQ by molecular method. RESULTS: Among AA patients, 37.5% (a proportion rising to 72% when taking patients who began their first patch before the age of 20 years) were positive for HLA-DRB1*11 compared to 21.2% healthy controls (p = 0.004, RR = 2.1). DRB1*11-positive status was associated with earlier development of the first AA patch, at the mean age of 16 years compared to 27 years (p = 0.003) in DRB1*11-negative patients. Among the DRB1*11 alleles, the presence of DRB1*1104 was associated with the earliest occurrence of AA. CONCLUSION: Our data indicate that the HLA system largely through DRB1*1104 allele influences AA onset rather than extension considering patchy AA and AT/AU.