Contributions of the US Centers for Disease Control and Prevention in Implementing the Global Health Security Agenda in 17 Partner Countries.

The Global Health Security Agenda (GHSA), a partnership of nations, international organizations, and civil society, was launched in 2014 with a mission to build countries' capacities to respond to infectious disease threats and to foster global compliance with the International Health Regulations (IHR 2005). The US Centers for Disease Control and Prevention (CDC) assists partner nations to improve IHR 2005 capacities and achieve GHSA targets. To assess progress through these CDC-supported efforts, we analyzed country activity reports dating from April 2015 through March 2017. Our analysis shows that CDC helped 17 Phase I countries achieve 675 major GHSA accomplishments, particularly in the cross-cutting areas of public health surveillance, laboratory systems, workforce development, and emergency response management. CDC's engagement has been critical to these accomplishments, but sustained support is needed until countries attain IHR 2005 capacities, thereby fostering national and regional health protection and ensuring a world safer and more secure from global health threats.

World-wide architecture of osteoporosis research: density-equalizing mapping studies and gender analysis.

OBJECTIVE: While research activities on osteoporosis grow constantly, no concise description of the global research architecture exists. Hence, we aim to analyze and depict the world-wide scientific output on osteoporosis combining bibliometric tools, density-equalizing mapping projections and gender analysis. METHOD: Using the NewQIS platform, we analyzed all osteoporosis-related publications authored from 1900 to 2012 and indexed by the Web of Science. Bibliometric details were analyzed related to quantitative and semi-qualitative aspects. RESULTS: The majority of 57 453 identified publications were original research articles. The USA and Western Europe dominated the field regarding cooperation activity, publication and citation performance. Asia, Africa and South America played a minimal role. Gender analysis revealed a dominance of male scientists in almost all countries except Brazil. CONCLUSION: Although the scientific performance on osteoporosis is increasing world-wide, a significant disparity in terms of research output was visible between developed and low-income countries. This finding is particularly concerning since epidemiologic evaluations of future osteoporosis prevalences predict enormous challenges for the health-care systems in low-resource countries. Hence, our study underscores the need to address these disparities by fostering future research endeavors in these nations with the aim to successfully prevent a growing global burden related to osteoporosis.

The systemic control of circadian gene expression.

The mammalian circadian timing system consists of a central pacemaker in the brain's suprachiasmatic nucleus (SCN) and subsidiary oscillators in nearly all body cells. The SCN clock, which is adjusted to geophysical time by the photoperiod, synchronizes peripheral clocks through a wide variety of systemic cues. The latter include signals depending on feeding cycles, glucocorticoid hormones, rhythmic blood-borne signals eliciting daily changes in actin dynamics and serum response factor (SRF) activity, and sensors of body temperature rhythms, such as heat shock transcription factors and the cold-inducible RNA-binding protein CIRP. To study these systemic signalling pathways, we designed and engineered a novel, highly photosensitive apparatus, dubbed RT-Biolumicorder. This device enables us to record circadian luciferase reporter gene expression in the liver and other organs of freely moving mice over months in real time. Owing to the multitude of systemic signalling pathway involved in the phase resetting of peripheral clocks the disruption of any particular one has only minor effects on the steady state phase of circadian gene expression in organs such as the liver. Nonetheless, the implication of specific pathways in the synchronization of clock gene expression can readily be assessed by monitoring the phase-shifting kinetics using the RT-Biolumicorder.

[Rhinosinusitis in cystic fibrosis]. / Rhinosinusitis bei Mukoviszidose

In cystic fibrosis (CF) mucociliary clearance of the entire respiratory system is impaired. This allows pathogens, such as Pseudomonas aeruginosa to persist and proliferate, which by progressive pulmonary destruction causes 90 % of premature deaths due to this inherited disease. The dramatic improvement in life expectation of patients due to intensive therapy has resulted in the inevitable but variably expressed sinonasal involvement coming into the clinical and scientific focus. Thereby, almost all CF patients reveal sinonasal pathology and many suffer from chronic rhinosinusitis. Recently, the sinonasal niche has been recognized as a site of initial and persistent colonization by pathogens. This article presents the pathophysiological background of this multiorgan disease as well as general diagnostic and therapeutic standards. The focus of this article is on sinonasal involvement and conservative and surgical options for treatment. Prevention of pathogen acquisition is an essential issue in the otorhinolaryngological treatment of CF patients.

Hepatitis B: global scientific development from a critical point of view.

Hepatitis B is the tenth leading cause of death worldwide. Countries with high endemicity, such as China and Taiwan show high scientific productivity in this field and dominate the top ten list of the most productive authors worldwide, providing four of them. This is remarkable, as the USA and Europe usually maintain leading positions, not only regarding country-specific scientific productivity, but also top ten ranking of most productive and most cited authors in other important medical sectors. So far, a scientometric analysis of the topic 'hepatitis B' has not been generated despite an increased need for it in times of modified evaluation criteria for academic personnel and a subsequent tendency to co-authorship and author self-citation. In this study, scientometric methods and large-scale data analysis were used to evaluate quality and quantity of scientific research dealing with the topic 'hepatitis B' and to contribute to distinguish relevant research output. Data were gained from Pubmed and ISI-Web. In the time span of 1971-2011, 49 166 items were published by 250 countries, of which the USA have been the most productive supplier with 28% of all publications, followed in considerable distance by Germany, China, the United Kingdom, Japan, France, Italy and Taiwan, respectively. The USA have established their position as centre of international cooperation. Their cooperation with China proves to be the most productive one. The most prolific journals in the field of hepatitis b were 'Hepatology', the 'Journal of Hepatology' and the 'Journal of Medical Virology'. h-index, citation rate and impact factor, commonly used for assessment of scientific quality, were determined and discussed critically with regard to distortion by bias of self-citation and co-authorship.

High expression of FOXP3 in primary melanoma is associated with tumour progression.

BACKGROUND: The antitumour immune response plays an important role in the prognosis of melanoma. High numbers of circulating regulatory T cells have been associated with rapid disease progression. OBJECTIVES: To assess the influence of forkhead box protein (FOXP)3, CD1a and langerin expression on the prognosis of primary melanoma. METHODS: We analysed 185 primary melanomas by immunohistochemical staining for expression of the regulatory T-cell marker FOXP3 and the dendritic cell markers langerin and CD1a, and correlated marker expression with clinical outcome. RESULTS: Disease-free survival and overall survival were significantly longer in patients expressing low levels of FOXP3 in the primary melanoma, whereas they were associated with high expression of CD1a. The negative prognostic value of FOXP3 expression was independent of the Breslow tumour thickness. Langerin expression did not correlate with the clinical outcome. CONCLUSIONS: High expression of FOXP3 in the primary melanoma may be used as an additional independent prognostic marker for early tumour progression in patients with melanoma.

Nasotracheal intubation depth in paediatric patients.

BACKGROUND: The aim of this study was to compare intubation depth using the Microcuff paediatric endotracheal tube (PET) placed with the intubation depth mark between the vocal cords with that of different published formulae/recommendations for nasotracheal intubation depth in children. METHODS: Children aged from birth to 10 yr undergoing elective surgery with nasotracheal intubation were included. Tracheal tubes were adjusted according to the intubation depth mark between the vocal cords using direct laryngoscopy. Nasal intubation depth was recorded and the distance 'tube tip to carina' was measured endoscopically. Based on the recorded nasal intubation depth and measured distance 'tube tip to carina', the position of tube tip and cuff was calculated according to six published formulae/recommendations. RESULTS: Seventy-six children were studied. For the Microcuff PET, the median tube tip advancement within the trachea was 52.9% (41.1-73.8%) of tracheal length. The shortest distance from the 'tube tip to carina' was 15 mm for a 3.5 mm internal diameter tube. If the six published formulae/recommendations had been used, this would have resulted in endobronchial tube placement in up to 9.1% of cases, and the tube tip would have been placed above the glottis in up to 2.6% of cases. The upper border of the cuff would have been placed in the subglottic area in up to 42.1% of cases and in a supraglottic position in up to 63.2% of cases. CONCLUSIONS: This study indicates that nasal intubation with the intubation depth mark placed between the vocal cords was superior to formula-based nasotracheal tube positioning. The latter would result in a high rate of endobronchial intubations, excessively high cuff positions and even tracheal extubations.

A Longitudinal Study of Thrombosis and Bleeding Outcomes With Thromboprophylaxis in Pregnant Women at Intermediate and High Risk of VTE.

BACKGROUND: The efficacy and safety of thromboprophylaxis in pregnancy at intermediate to high risk of venous thrombo-embolism (VTE) is an area of ongoing research. AIM: This study aimed to assess thrombosis and bleeding outcomes associated with thromboprophylaxis in women at risk of VTE. METHODS: A cohort of 129 pregnancies, who received thromboprophylaxis for the prevention of VTE, were identified from a specialist obstetric clinic in Johannesburg, South Africa. Intermediate-risk pregnancies, with medical comorbidities or multiple low risks, were managed with fixed low-dose enoxaparin antepartum and for a median (interquartile range) of 4 (4) weeks postpartum. High-risk pregnancies, with a history of previous VTE, were managed with anti-Xa adjusted enoxaparin antepartum and for a median of 6 (0) weeks postpartum. Pregnancy-related VTE was objectively confirmed. Major bleeding, clinically relevant nonmajor bleeding (CRNMB) and minor bleeding were defined according to the International Society on Thrombosis and Hemostasis Scientific Subcommittee. RESULTS: Venous thrombo-embolism occurred antepartum in 1.4% (95% CI: 0.04-7.7) of intermediate and 3.4% (95% CI: 0.4-11.7) of high-risk pregnancies. Bleeding events occurred in 7.1% (95% CI: 2.4-15.9) of intermediate and 8.5% (95% CI: 2.8-18.7) of high-risk pregnancies. Of these bleeding events, 3.1% (95% CI: 1.0-8.0) were classified as major bleeding. On univariate analysis, no independent predictors of bleeding were identified. CONCLUSION: The rates of thrombosis and bleeding in this predominantly African population were consistent with similar studies and can be used to inform pregnant women of the benefits of anticoagulation and the risks of potential bleeding.

[Low-dose droperidol in children: rescue therapy for persistent postoperative nausea and vomiting]. / "Low-dose"-Droperidol-Gabe bei Kindern: "Rescue"-Therapie gegen persistierende postoperative Übelkeit und Erbrechen.

BACKGROUND: Droperidol had been used as an effective antiemetic since the 1970s but was withdrawn from the market in 2001 because of a black box warning about QT prolongation and possible cardiac arrhythmia after high doses. In the meantime the black box warning has seriously been questioned and parenteral droperidol has again been licensed in 2008. Because droperidol acts on dopaminergic receptors different to 5-HT(3) antagonists and dexamethasone, it could possibly serve as a rescue drug after failed postoperative nausea and vomiting (PONV) prophylaxis. Persistent PONV after the recommended prophylaxis is a significant problem in pediatric anesthesia but a satisfactory strategy has not yet been defined. Therefore a retrospective audit was performed in order to evaluate whether low-dose droperidol (10 µg/kgBW) would be an effective rescue drug for failed antiemetic prophylaxis. PATIENTS AND METHODS: The electronic anesthesia patient data base of the University Children's Hospital Zurich was searched from 2004-2009 for patients who received low-dose droperidol in the postanesthesia care unit as rescue therapy for persistent PONV after antiemetic prophylaxis. Based on the recorded electronic data the effectiveness of low-dose droperidol as PONV rescue therapy and possible side effects were analyzed. RESULTS: A total of 338 patients who received droperidol were found from a total of 34,032 patients and the charts were analyzed. Of these patients 134 were excluded because they had received droperidol for indications other than PONV, 43 patients were excluded because they had not received antiemetic prophylaxis before droperidol and in 17 patients the data were incomplete, leaving 144 patients with an average age of 12.3 years (interquartile range IQR 9.5-15.2 years) for analysis. The upper range of ages resulted from patients with chronic diseases who were still being treated in the Children's Hospital. Low-dose droperidol was given because of persistent nausea to 59 patients (41%) and to 85 patients (59%) for persistent vomiting. Initial antiemetic prophylaxis and/or therapy had consisted of dexamethasone plus tropisetrone in 80 patients and tropisetrone or dexamethasone alone in 64 patients. In 128 patients (89%) rescue therapy with a median dose of 10.9 µg/kgBW droperidol was effective but vomiting persisted in 16 patients (11%). Sedation was the only side effect recorded and this was observed in 39 patients (27%). CONCLUSIONS: Low-dose droperidol (10 µg/kgBW) was found to be effective as rescue medication in pediatric patients experiencing PONV despite various prophylactic antiemetic regimens. No neurological or cardiopulmonary side effects were recorded after this low dosage.

Cuffed endotracheal tubes in children reduce sevoflurane and medical gas consumption and related costs.

BACKGROUND: This study aims to evaluate sevoflurane and anaesthetic gas consumption using uncuffed vs. cuffed endotracheal tubes (ETT) in paediatric surgical patients. METHODS: Uncuffed or cuffed ETT were used in paediatric patients (newborn to 5 years) undergoing elective surgery in a randomized order. Duration of assessment, lowest possible fresh gas flow (minimal allowed FGF: 0.5 l/min) and sevoflurane concentrations used were recorded. Consumption and costs for sevoflurane and medical gases were calculated. RESULTS: Seventy children (35 uncuffed ETT/35 cuffed ETT), aged 1.73 (0.01-4.80) years, were enrolled. No significant differences in patient characteristics, study period and sevoflurane concentrations used were found between the two groups. Lowest possible FGF was significantly lower in the cuffed ETT group [1.0 (0.5-1.0) l/min] than in the uncuffed ETT group [2.0 (0.5-4.3) l/min], P<0.001. Sevoflurane consumption per patient was 16.1 (6.4-82.8) ml in the uncuffed ETT group and 6.2 (1.1-14.9) ml in the cuffed ETT group, P=0.003. Medical gas consumption was 129 (53-552) l in the uncuffed ETT group vs. 46 (9-149) l in the cuffed ETT group, P<0.001. The total costs for sevoflurane and medical gases were 13.4 (6.0-67.3)euro/patient in the uncuffed ETT group and 5.2 (1.0-12.5)euro/patient in the cuffed ETT group, P<0.001. CONCLUSIONS: The use of cuffed ETT in children significantly reduced the costs of sevoflurane and medical gas consumption during anaesthesia. Increased costs for cuffed compared with uncuffed ETT were completely compensated by a reduction in sevoflurane and medical gas consumption.

European Academy of Paediatrics Research in Ambulatory Setting network (EAPRASnet): a multi-national general paediatric research network for better child health.

BACKGROUND: In 2008, the European Academy of Paediatrics launched a paediatric-based research network - EAPRASnet (European Academy of Paediatrics Research in Ambulatory Setting network). The network has recruited primary care and general paediatricians from European and Mediterranean countries. METHODS: Every paediatrician joining the network has been asked to complete a recruitment survey. The aims of the survey were to characterize paediatrician's demographics, practice arrangements and patient's demographics, to define main incentives for research, and to learn what paediatricians view as unsolved issues that need to be studied. RESULTS: A total of 156 paediatricians from 19 countries were recruited with 144 completing the questionnaire (92%). Majority of respondents (89%) were general paediatricians for more than half of their time. Practice arrangement of 47% of paediatricians was solo practice, with 40% in group practice. Electronic medical records were being used by 72% of respondents. Over 70% of the paediatricians had more than 1000 patients under their clinical care, and patients younger than 6 years old contributed nearly half of the patient population. Areas of most interest for research were: quality of care indicators, communication with parents, obesity, attention deficit hyperactivity disorder and effective well child care. Main incentives for participation in a research project were interest in the topic (81%) and effort to improve quality of care (71%). Lack of time was the leading reported obstacle for research activity (72%). EAPRASnet is growing, and the network's structure, operation and funding are described. Methods for joining the network and the process of study development are presented. CONCLUSION: A core group of EAP general paediatricians are committed to research in their practices. The information gathered will serve for future planning of research projects in the EAPRASnet to harmonize and optimize the care given to children in the primary care setting in Europe.

[Methods of health economic evaluation for health services research]. / Methoden der gesundheitsökonomischen Evaluation in der Versorgungsforschung.

On August 30, 2010, the German Network for Health Services Research [Deutsches Netzwerk Versorgungsforschung e. V. (DNVF e. V.)] approved the Memorandum III "Methods for Health Services Research", supported by the member societies mentioned as authors and published in this Journal [Gesundheitswesen 2010; 72: 739-748]. The present paper focuses on methodological issues of economic evaluation of health care technologies. It complements the Memorandum III "Methods for Health Services Research", part 2. First, general methodological principles of the economic evaluations of health care technologies are outlined. In order to adequately reflect costs and outcomes of health care interventions in the routine health care, data from different sources are required (e. g., comparative efficacy or effectiveness studies, registers, administrative data, etc.). Therefore, various data sources, which might be used for economic evaluations, are presented, and their strengths and limitations are stated. Finally, the need for methodological advancement with regard to data collection and analysis and issues pertaining to communication and dissemination of results of health economic evaluations are discussed.

The nocebo effect: a reason for patients' non-adherence to generic substitution?

Substituting generic formulations of the same chemical agent is a common practice in German health care on the basis of so called rebate contracts. The substitution of a medication may affect the patients' adherence or result in adverse events. While adverse events which may be caused by the pharmacological activity of the agent itself can be explained, some non-specific side effects cannot be substantiated referring to pharmacological factors. These adverse reactions are summarized under the term nocebo effect. Since patients experiencing a nocebo effect can subsequently become non-adherent or even discontinue an appropiate therapy, the aim of this article is to study patients' adherence to generic substitution and the extent of the nocebo effect. In MEDLINE and EMBASE, a search was carried out for articles which were published between March 25th, 1989 and March 25th, 2009 by using the following search terms: generic substitution, adherence, non-adherence, non-persistence, rebate contracts, patients' attitude, nocebo, negative placebo effects, placebo adverse reactions, placebo induced side effects and negative placebo responses. In addition a manual search was performed in the reference lists of the articles retrieved. 14 studies met the inclusion and exclusion criteria and were included in this article. The generic substitution was generally accepted by over two thirds of the study populations. But up to 34% of patients being treated for psychological diseases confronted with a change of their medication had additional adverse events. On the basis of the studies analysed, the conclusion can be drawn that the nocebo effect can play a crucial role in the treatment of psychological diseases. Therefore, physicians and pharmacists should be responsible to prevent the nocebo effect through adequately educating the patients.

[Intrathecal opioid medication for perioperative analgesia in severely handicapped children undergoing spinal operations]. / Intrathekale Opioidmedikation zur perioperativen Analgesie bei schwer behinderten Kindern mit Wirbelsäulenoperationen.

PURPOSE OF THE STUDY: Intrathecal opioids have been shown to be safe and effective for postoperative analgesia in healthy children for spinal surgery. The aim of this study was to evaluate the applicability of intrathecal opioids in severely handicapped children scheduled for spinal surgery. METHODS: With hospital ethical committee approval, patients with physical states III and IV of the ASA classification requiring spinal surgery were retrospectively studied. In addition to inhalational anesthesia with sevoflurane or intravenous anesthesia using propofol, morphine 20 microg/kgBW and sufentanil 1.5 microg/kgBW were administered intrathecally before surgery. After surgery an infusion of nalbuphine was started. Need for additional intraoperative and postoperative analgesics, time of extubation, postoperative pain scores and p(a)CO2 values as well as adverse effects were recorded. RESULTS: A total of 28 patients aged from 2.8 to 18.5 years (median 11.6 years) were studied. Immediate tracheal extubation in the operating room was possible in 17 patients and for 11 patients delayed extubation was elected. All patients were extubated within 24 h except for 1 patient who received massive postoperative transfusions. In 26 out of 28 patients (93%) the combination of intrathecal opioids with postoperative nalbuphine provided adequate analgesia. Observed side effects were post-operative nausea and vomiting (PONV), pruritus and moderate hypoventilation. In two patients a change to intravenous morphine therapy was necessary. CONCLUSION: The use of intrathecal opioids for perioperative pain control from spinal fusion in severely handicapped children is feasible. Intrathecal opioids provide adequate postoperative analgesia and allow early extubation without persisting relevant respiratory compromise in most of these patients.

[IQWiG's methods for the cost-benefit assessment : Comparison with an international reference scenario]. / Wo steht die Kosten-Nutzen-Bewertung des IQWiG : Abgleich mit einem internationalen Referenzszenario?

Standardization of international health economic guidelines has been repeatedly requested. In this context, an international reference case was proposed, which constitutes an agreed approach for the key elements of health economic evaluation including study perspective, comparators, source of effectiveness data, role of modeling, main (economic) outcome, source of utilities, characterizing uncertainty. It is, however, questionable whether such a reference scenario can reasonably be applied across all health care systems. Our analysis pursues the question to which degree the Institute for Quality and Efficiency in Health Care's (Institut für Qualität und Wirtschaftlichkeit im Gesundheitswesen, IQWiG) "General methods for evaluating the relation between cost and benefit" comply with the key elements of the reference case. In case of divergences, they will be described and discussed in light of the German social legislation and in consideration of current scientific evidence. In conclusion, the analysis revealed that IQWiG complied with the reference case in almost all aspects. Differences were found only with respect to the choice of main (economic) outcome and the source of utilities. These differences seem justified and well explained in the context of the German social legislation as well as in view of the weaknesses of the quality-adjusted life year (QALY) concept.

Neural correlates of subjective arousal and valence in health and panic disorder.

Aberrant emotion processing is a core characteristic of panic disorder (PD). Findings concerning the underlying neural pathways remain inconsistent. We applied functional magnetic resonance imaging (fMRI) in the context of a task based on the circumplex model of affect. This model links affective states to two underlying neurophysiological systems: arousal and valence. Twenty-two healthy participants and 20 participants with PD rated arousal and valence in response to affective faces during fMRI. In healthy controls, we found that arousal modulated the hemodynamic response in the parahippocampus, the ventromedial prefrontal cortex and the cuneus during face perception. Valence and extreme ratings of valence modulated the hemodynamic response in temporal, parietal, somatosensory, premotor and cerebellar regions. Comparing healthy controls to participants with PD, we found that healthy controls showed a stronger modulation of the hemodynamic response during face perception associated with extreme ratings of valence in the parahippocampus and the supplementary motor area. This suggests parahippocampal dysfunction in the processing of highly valenced affective faces in PD, which may underlie aberrant contextualization of strong affective stimuli. Our findings need to be interpreted with care as they were adjusted for multiple comparisons using a liberal correction procedure.

An adenosine deaminase that generates inosine at the wobble position of tRNAs.

Several transfer RNAs (tRNAs) contain inosine (I) at the first position of their anticodon (position 34); this modification is thought to enlarge the codon recognition capacity during protein synthesis. The tRNA-specific adenosine deaminase of Saccharomyces cerevisiae that forms I(34) in tRNAs is described. The heterodimeric enzyme consists of two sequence-related subunits (Tad2p/ADAT2 and Tad3p/ADAT3), both of which contain cytidine deaminase (CDA) motifs. Each subunit is encoded by an essential gene (TAD2 and TAD3), indicating that I(34) is an indispensable base modification in elongating tRNAs. These results provide an evolutionary link between the CDA superfamily and RNA-dependent adenosine deaminases (ADARs/ADATs).

Prospective randomized controlled multi-centre trial of cuffed or uncuffed endotracheal tubes in small children.

BACKGROUND: The use of cuffed tracheal tubes (TTs) in small children is still controversial. The aim of this study was to compare post-extubation morbidity and TT exchange rates when using cuffed vs uncuffed tubes in small children. METHODS: Patients aged from birth to 5 yr requiring general anaesthesia with TT intubation were included in 24 European paediatric anaesthesia centres. Patients were prospectively randomized into a cuffed TT group (Microcuff PET) and an uncuffed TT group (Mallinckrodt, Portex, Rüsch, Sheridan). Endpoints were incidence of post-extubation stridor and the number of TT exchanges to find an appropriate-sized tube. For cuffed TTs, minimal cuff pressure required to seal the airway was noted; maximal cuff pressure was limited at 20 cm H(2)O with a pressure release valve. Data are mean (SD). RESULTS: A total of 2246 children were studied (1119/1127 cuffed/uncuffed). The age was 1.93 (1.48) yr in the cuffed and 1.87 (1.45) yr in the uncuffed groups. Post-extubation stridor was noted in 4.4% of patients with cuffed and in 4.7% with uncuffed TTs (P=0.543). TT exchange rate was 2.1% in the cuffed and 30.8% in the uncuffed groups (P<0.0001). Minimal cuff pressure required to seal the trachea was 10.6 (4.3) cm H(2)O. CONCLUSIONS: The use of cuffed TTs in small children provides a reliably sealed airway at cuff pressures of

RNA editing by base deamination: more enzymes, more targets, new mysteries.

The posttranscriptional modification of messenger RNA precursors (pre-mRNAs) by base deamination can profoundly alter the physiological function of the encoded proteins. The recent identification of tRNA-specific adenosine deaminases (ADATs) has led to the suggestion that these enzymes, as well as the cytidine and adenosine deaminases acting on pre-mRNAs (CDARs and ADARs), belong to a superfamily of RNA-dependent deaminases. This superfamily might have evolved from an ancient cytidine deaminase. This article reviews the reactions catalysed by these enzymes and discusses their evolutionary relationships.

[Epidemiology of systemic lupus erythematosus in Reunion Island, Indian Ocean: A case-series in adult patients from a University Hospital]. / Caractéristiques du lupus érythémateux systémique à La Réunion : étude rétrospective en population adulte au CHU de Saint-Denis.

OBJECTIVES: The study aimed at assessing the epidemiology and clinical features of systemic lupus erythematosus (SLE) in Reunion Island, South West of Indian Ocean. PATIENTS AND METHODS: A retrospective study was conducted at the University Hospital of La Reunion (Saint-Denis) by charts review from 2004 to 2015. Patients with a SLE diagnosed over 15 years according to SLICC 2012 criteria were included. Incidence and prevalence were inferred from national health insurance database and population census results. RESULTS: In total, 123 patients met inclusion criteria: 116 were women (94%) and mean age at diagnosis was 34.7±13.4 years. Ten percent of all patients had a least one parent with autoimmune disease, and 4% with lupus. The main manifestations were musculoskeletal (89%) and mucocutaneous with acute or subacute lesions (76%), alopecia (25%), ulcers (15%) and discoid lupus (11%). Lupus nephropathy occurred in 39%, serositis in 31% and neurological features in 15%. Antinuclear antibodies were positive in 99% (threshold >1/80), and associated to anti-DNA (70%), anti-SSA (47%), anti-RNP (42%), and anti-Sm (37%). APL syndrome was diagnosed in 15%. The average annual standardized incidence between 2010 and 2016 was 6.3 cases per 100,000 inhabitants (95% confidence interval [CI]: 5.6-6.9). The prevalence was 76 cases per 100,000 inhabitants in 2016 (95% CI: 70-82). CONCLUSION: Lupus in the multi-ethnic population of Reunion Island is characterized by high incidence and high rates of articular and renal manifestations, as well as anti-ENA antibodies.