5 research outputs found

    ‘The ICJ then and now’: interview with Professor Bruno Simma and Professor Georg Nolte

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    This year’s German National Rounds of the Philip C. Jessup International Law Moot Court Competition hosted a high-profile interview featuring Professor Bruno Simma, former judge at the International Court of Justice, and Professor Georg Nolte, who recently took his seat on the bench of the very same Court. The discussion was moderated by Patricia Nacimiento of Herbert Smith Freehills. The interview offers a unique and very intimate conversation between two judges of the World Court, sharing both their professional and personal views of the Court from different perspectives. The discussion covers topics ranging from the process of becoming a judge on the International Court of Justice, the everyday work of the Court and the changes to the personal and academic lives of someone elected to serve as a judge at The Hague. Both scholars also reflect on the benefits of moot court competitions both for themselves and for students today. We are hosting the video with the kind permission of Friedrich Alexander University Erlangen-NĂŒrnberg, who organized both this year’s German Jessup Competition and the interview

    Arbitration in Germany : the Model Law in practice /

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    At head of title: Kluwer Law International.Includes bibliographical references (p. [1195]-1205) and index

    Assessment of plasma chitotriosidase activity, CCL18/PARC concentration and NP-C suspicion index in the diagnosis of Niemann-Pick disease type C : A prospective observational study

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    Niemann-Pick disease type C (NP-C) is a rare, autosomal recessive neurodegenerative disease caused by mutations in either the NPC1 or NPC2 genes. The diagnosis of NP-C remains challenging due to the non-specific, heterogeneous nature of signs/symptoms. This study assessed the utility of plasma chitotriosidase (ChT) and Chemokine (C-C motif) ligand 18 (CCL18)/pulmonary and activation-regulated chemokine (PARC) in conjunction with the NP-C suspicion index (NP-C SI) for guiding confirmatory laboratory testing in patients with suspected NP-C. In a prospective observational cohort study, incorporating a retrospective determination of NP-C SI scores, two different diagnostic approaches were applied in two separate groups of unrelated patients from 51 Spanish medical centers (n = 118 in both groups). From Jan 2010 to Apr 2012 (Period 1), patients with ≄2 clinical signs/symptoms of NP-C were considered 'suspected NP-C' cases, and NPC1/NPC2 sequencing, plasma chitotriosidase (ChT), CCL18/PARC and sphingomyelinase levels were assessed. Based on findings in Period 1, plasma ChT and CCL18/PARC, and NP-C SI prediction scores were determined in a second group of patients between May 2012 and Apr 2014 (Period 2), and NPC1 and NPC2 were sequenced only in those with elevated ChT and/or elevated CCL18/PARC and/or NP-C SI ≄70. Filipin staining and 7-ketocholesterol (7-KC) measurements were performed in all patients with NP-C gene mutations, where possible. In total across Periods 1 and 2, 10/236 (4%) patients had a confirmed diagnosis o NP-C based on gene sequencing (5/118 [4.2%] in each Period): all of these patients had two causal NPC1 mutations. Single mutant NPC1 alleles were detected in 8/236 (3%) patients, overall. Positive filipin staining results comprised three classical and five variant biochemical phenotypes. No NPC2 mutations were detected. All patients with NPC1 mutations had high ChT activity, high CCL18/PARC concentrations and/or NP-C SI scores ≄70. Plasma 7-KC was higher than control cut-off values in all patients with two NPC1 mutations, and in the majority of patients with single mutations. Family studies identified three further NP-C patients. This approach may be very useful for laboratories that do not have mass spectrometry facilities and therefore, they cannot use other NP-C biomarkers for diagnosis
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