Amicus Brief by Amnesty International and Others

On September 2, 2020, six Portuguese youth filed a complaint with the European Court of Human Rights against 33 countries. The complaint alleges that the respondents have violated human rights by failing to take sufficient action on climate change, and seeks an order requiring them to take more ambitious action. The complaint relies on Articles 2, 8, and 14 of the European Convention on Human Rights, which protect the right to life, right to privacy, and right to not experience discrimination. The complainants claim that their right to life is threatened by the effects of climate change in Portugal such as forest fires; that their right to privacy includes their physical and mental wellbeing, which is threatened by heatwaves that force them to spend more time indoors; and that as young people, they stand to experience the worst effects of climate change. The case is brought against the Member States of the Council of Europe (Austria, Belgium, Bulgaria, Cyprus, Czech Republic, Germany, Greece, Denmark, Estonia, Finland, France, Croatia, Hungary, Ireland, Italy, Lithuania, Luxembourg, Latvia, Malta, the Netherlands, Poland, Portugal, Romania, Slovak Republic, Slovenia, Spain and Sweden) as well as Norway, Russia, Switzerland, Turkey, Ukraine and the United Kingdom. The complainants allege that the respondents have fallen short of their human rights obligations by failing to agree to emissions reductions that will keep temperature rise to 1.5 degrees Celsius, as envisioned by the Paris Agreement. On November 30, 2020, The European Court of Human Rights fast-tracked and communicated the case to 33 defendant countries, requiring them to respond by the end of February 2021. According to the Global Legal Action Network (GLAN), who are supporting the case, only a tiny minority of cases before the Court are fast-tracked and communicated. On February 4, 2021, the Court rejected a motion by the defendant governments asking the Court to overturn its fast-tracking decision. The governments had asked the court to overturn priority treatment of the case and to hear arguments only on the admissibility of the case. The Court sent a letter to the parties rejecting these motions and gave the defendants until May 27, 2021 to submit a defense on both admissibility and the merits of the case. The Court also granted until May 6, 2021 third party interventions. Among other seven third-party intervention, on May 5, 2021, Amnesty International intervened in the case and submitted her written observations to the European Court of Human Rights. The submission supports the claimants\u27 position, providing legal arguments to the Court to show that international law requires states to not harm, and to not allow companies within their jurisdiction to harm, the human rights of people outside their borders. On May 19, 2021 a new intervention was made by the European Commission submitted her written observations to the European Court of Human Rights. Noting the pronounced impact of environmental degradation and climate change on human rights, the Commissioner argues that international environmental and children’s rights law instruments should play a significant role in defining the scope of states’ obligation to prevent human rights violations caused by environmental harm. The Commission bases its defense of EU policy in the field of environmental protection on sound legal reasoning and science-based evidence. The term ‘climate emergency’ expresses the political will to fulfill the obligations under the Paris Agreement. The Commissioner concludes that “the increasing number of climate change-related applications provide the Court with a unique opportunity to continue to forge the legal path towards a more complete implementation of the Convention and to offer real-life protection to individuals affected by environmental degradation and climate change.” On August 14, 2021, the claimants received the respondent governments’ respective defenses. However, on legal advice, the claimants have decided not to make them public. The claimants have until January 12, 2022 to respond to the governments’ defenses. On June 30, 2022, the Chamber of the European Court of Human Rights relinquished jurisdiction in favor of the Grand Chamber. The case is now going to be examined by the ECtHR\u27s Grand Chamber of 17 judges on account of the fact that the case raises a serious question affecting the interpretation of the Convention (Art 30 ECHR). At Issue: Youth filed human rights complaint against 33 governments

EuReCa ONE—27 Nations, ONE Europe, ONE Registry A prospective one month analysis of out-of-hospital cardiac arrest outcomes in 27 countries in Europe

AbstractIntroductionThe aim of the EuReCa ONE study was to determine the incidence, process, and outcome for out of hospital cardiac arrest (OHCA) throughout Europe.MethodsThis was an international, prospective, multi-centre one-month study. Patients who suffered an OHCA during October 2014 who were attended and/or treated by an Emergency Medical Service (EMS) were eligible for inclusion in the study. Data were extracted from national, regional or local registries.ResultsData on 10,682 confirmed OHCAs from 248 regions in 27 countries, covering an estimated population of 174 million. In 7146 (66%) cases, CPR was started by a bystander or by the EMS. The incidence of CPR attempts ranged from 19.0 to 104.0 per 100,000 population per year. 1735 had ROSC on arrival at hospital (25.2%), Overall, 662/6414 (10.3%) in all cases with CPR attempted survived for at least 30 days or to hospital discharge.ConclusionThe results of EuReCa ONE highlight that OHCA is still a major public health problem accounting for a substantial number of deaths in Europe.EuReCa ONE very clearly demonstrates marked differences in the processes for data collection and reported outcomes following OHCA all over Europe. Using these data and analyses, different countries, regions, systems, and concepts can benchmark themselves and may learn from each other to further improve survival following one of our major health care events

Genome-wide Analyses Identify KIF5A as a Novel ALS Gene

To identify novel genes associated with ALS, we undertook two lines of investigation. We carried out a genome-wide association study comparing 20,806 ALS cases and 59,804 controls. Independently, we performed a rare variant burden analysis comparing 1,138 index familial ALS cases and 19,494 controls. Through both approaches, we identified kinesin family member 5A (KIF5A) as a novel gene associated with ALS. Interestingly, mutations predominantly in the N-terminal motor domain of KIF5A are causative for two neurodegenerative diseases: hereditary spastic paraplegia (SPG10) and Charcot-Marie-Tooth type 2 (CMT2). In contrast, ALS-associated mutations are primarily located at the C-terminal cargo-binding tail domain and patients harboring loss-of-function mutations displayed an extended survival relative to typical ALS cases. Taken together, these results broaden the phenotype spectrum resulting from mutations in KIF5A and strengthen the role of cytoskeletal defects in the pathogenesis of ALS.Peer reviewe

Amicus Brief by Amnesty International and Others

On September 2, 2020, six Portuguese youth filed a complaint with the European Court of Human Rights against 33 countries. The complaint alleges that the respondents have violated human rights by failing to take sufficient action on climate change, and seeks an order requiring them to take more ambitious action. The complaint relies on Articles 2, 8, and 14 of the European Convention on Human Rights, which protect the right to life, right to privacy, and right to not experience discrimination. The complainants claim that their right to life is threatened by the effects of climate change in Portugal such as forest fires; that their right to privacy includes their physical and mental wellbeing, which is threatened by heatwaves that force them to spend more time indoors; and that as young people, they stand to experience the worst effects of climate change. The case is brought against the Member States of the Council of Europe (Austria, Belgium, Bulgaria, Cyprus, Czech Republic, Germany, Greece, Denmark, Estonia, Finland, France, Croatia, Hungary, Ireland, Italy, Lithuania, Luxembourg, Latvia, Malta, the Netherlands, Poland, Portugal, Romania, Slovak Republic, Slovenia, Spain and Sweden) as well as Norway, Russia, Switzerland, Turkey, Ukraine and the United Kingdom. The complainants allege that the respondents have fallen short of their human rights obligations by failing to agree to emissions reductions that will keep temperature rise to 1.5 degrees Celsius, as envisioned by the Paris Agreement. On November 30, 2020, The European Court of Human Rights fast-tracked and communicated the case to 33 defendant countries, requiring them to respond by the end of February 2021. According to the Global Legal Action Network (GLAN), who are supporting the case, only a tiny minority of cases before the Court are fast-tracked and communicated. On February 4, 2021, the Court rejected a motion by the defendant governments asking the Court to overturn its fast-tracking decision. The governments had asked the court to overturn priority treatment of the case and to hear arguments only on the admissibility of the case. The Court sent a letter to the parties rejecting these motions and gave the defendants until May 27, 2021 to submit a defense on both admissibility and the merits of the case. The Court also granted until May 6, 2021 third party interventions. Among other seven third-party intervention, on May 5, 2021, Amnesty International intervened in the case and submitted her written observations to the European Court of Human Rights. The submission supports the claimants\u27 position, providing legal arguments to the Court to show that international law requires states to not harm, and to not allow companies within their jurisdiction to harm, the human rights of people outside their borders. On May 19, 2021 a new intervention was made by the European Commission submitted her written observations to the European Court of Human Rights. Noting the pronounced impact of environmental degradation and climate change on human rights, the Commissioner argues that international environmental and children’s rights law instruments should play a significant role in defining the scope of states’ obligation to prevent human rights violations caused by environmental harm. The Commission bases its defense of EU policy in the field of environmental protection on sound legal reasoning and science-based evidence. The term ‘climate emergency’ expresses the political will to fulfill the obligations under the Paris Agreement. The Commissioner concludes that “the increasing number of climate change-related applications provide the Court with a unique opportunity to continue to forge the legal path towards a more complete implementation of the Convention and to offer real-life protection to individuals affected by environmental degradation and climate change.” On August 14, 2021, the claimants received the respondent governments’ respective defenses. However, on legal advice, the claimants have decided not to make them public. The claimants have until January 12, 2022 to respond to the governments’ defenses. On June 30, 2022, the Chamber of the European Court of Human Rights relinquished jurisdiction in favor of the Grand Chamber. The case is now going to be examined by the ECtHR\u27s Grand Chamber of 17 judges on account of the fact that the case raises a serious question affecting the interpretation of the Convention (Art 30 ECHR). At Issue: Youth filed human rights complaint against 33 governments

Review of Hair Cell Synapse Defects in Sensorineural Hearing Impairment

ObjectiveTo review new insights into the pathophysiology of sensorineural hearing impairment. Specifically, we address defects of the ribbon synapses between inner hair cells and spiral ganglion neurons that cause auditory synaptopathy.Data sources and study selectionHere, we review original publications on the genetics, animal models, and molecular mechanisms of hair cell ribbon synapses and their dysfunction.ConclusionHair cell ribbon synapses are highly specialized to enable indefatigable sound encoding with utmost temporal precision. Their dysfunctions, which we term auditory synaptopathies, impair audibility of sounds to varying degrees but commonly affect neural encoding of acoustic temporal cues essential for speech comprehension. Clinical features of auditory synaptopathies are similar to those accompanying auditory neuropathy, a group of genetic and acquired disorders of spiral ganglion neurons. Genetic auditory synaptopathies include alterations of glutamate loading of synaptic vesicles, synaptic Ca influx or synaptic vesicle turnover. Acquired synaptopathies include noise-induced hearing loss because of excitotoxic synaptic damage and subsequent gradual neural degeneration. Alterations of ribbon synapses likely also contribute to age-related hearing loss

Proceedings Of The 23Rd Paediatric Rheumatology European Society Congress: Part Two

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