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This work contains the elements which are necessary to understand how the clinical trials about neurofibromatosis type 1 (NF1) have been developed and the bases on which they have relied in order to determine the prospects of treatment for the patients suffering from this genetic disorder. Therefore follows the clinical description of this condition as well as its genetic origin, the functions of the neurofibromin, the protein encoded by the NF1 gene, the up to date knowledge acquired on the signaling pathways and a briefoverview of animal data (orthologs).As NF1 is a multisystem disease with highly variable expressivity, it seems important to focus on a more specific domain: plexiform neurofibromas. Due to the specific nature and natural history of these neurofibromas at bedside, experts elaborated a distinct clinical tool for a more appropriate evaluation for treatment efficacy measurement (ReINS). This scale is here reported. Numerous clinical trials have been conducted with different molecules. Four molecules are here fully described: imatinib, sirolimus, pirfenidone and tipifamib. For each molecule, pharmacology, mechanisms of action and indication for treatment extracted from different phases of trial to clinical use in plexiform neurofibromas are reported. The focus on these 4 allow to draw the lines of evidence and may serve as a good background to explore the research and development strengths and caveats for other new drugs.In conclusion, the various studies once analyzed have revealed the pertinent use of the volumetric analyze of plexiform neurofibromas by M.R.I and to use evaluation tools the quality of life, pain and patient appreciation on treatment. They also helped to improve the knowledge conceming NF1 and the use of medicine in this particular disease."contributory" although insufficient effect. The knowledge acquired to date offers no treatment for patients suffering from NF 1 but the ongoing studies should guarantee progress for a better treatment and let .us hope to cure. Le présent mémoire contient les éléments permettant de comprendre sur quelles bases se sont appuyés et comment ont pu être élaborés les essais cliniques dans le cadre de la Neurofibromatose de type 1(NF1) et ce afin de déterminer quelles sont les perspectives de traitement pour les patients atteints de cette maladie génétique. Ainsi, sont présentées successivement : la description clinique de cette phacomatose, son origine génétique, les fonctions de la neurofibromine encodée par le gène NF 1, les connaissances acquises au niveau biologique (voies de signalisation) et un bref aperçu des données issues des recherches chez l'animal. La NF1 étant une maladie multisystémique, il m'a semblé important de cibler un domaine plus particulier : celui des neurofibromes plexiformes. De nombreux essais cliniques ont été effectués avec différentes molécules, dont 4 sont abordées de manière détaillée : l'imatinib, le sirolimus, la pirfénidone et le tipifarnib. L'intérêt de chaque molécule dans le traitement des neurofibromes plexiformes à partir des indications et des mécanismes maîtrisés actuellement a été précisé. Notons également que d'autres molécules, citées dans ce travail, ont été testées ou sont actuellement en cours d'étude. Avant de décrire chacune des filières médicamenteuses, la méthodologie spécifique, « ReiNS », développée pour les essais cliniques effectués dans le cadre de la NF1 est détaillée. En conclusion, les études analysées ont, tout d'abord, permis de préciser la pertinence de l'analyse volumétrique des neurofibromes plexiformes par IRM et de déterminer les outils d'évaluation de la qualité de vie, de la douleur... Elles ont également contribué à améliorer les connaissances de la NF1 et l'utilisation des médicaments dans ce cadre particulier. Seuls l'imatinib et le sirolimus semblent montrer un effet « contributif » mais malgré tout insuffisant. Les connaissances acquises, à ce jour, n'offrent donc aucunes perspectives de traitement aux patients atteints de NF1 et présentant un neurofibrome plexiforme. Cependant, les études en cours et l'intérêt scientifique pour cette problématique leur permettent de garder espoir.

Neurofibromatosis 1 --- Clinical Trial

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Avenging Lincoln's Death: The Trial of John Wilkes Booth's Accomplices dissects the trial of eight alleged accomplices of John Wilkes Booth, showing that the trial was unconstitutional because Congress never authorized trial by military commission and President Johnson exceeded his authority by appointing a military commission when the Habeas Corpus Act of 1863 directed him to turn over the accomplices to civilian authorities. The trial was also flawed by admission of irrelevant evidence of Confederate atrocities and perjured testimony.

Lincoln Assassination Conspiracy Trial, Washington, D.C., 1865.

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This paper estimates the short-term, partial-equilibrium impacts of a public-private partnership program for low-cost private secondary schools in Uganda. The public-private partnership program is part of a broader strategy to absorb large increases in secondary enrollment following the introduction of universal secondary education. Under the program, the government offers a per-student subsidy to participating private schools. Program implementation allowed for a randomized phase-in study design to estimate the causal impacts of the program on private school performance. The study finds that the public-private partnership program helped absorb large numbers of eligible students in secondary schools. Student performance in participating private schools was significantly better than in nonparticipating private schools. The study finds that improved student performance is potentially linked to increased input availability, as well as positive selection of government aided students in private schools. Suggestive evidence indicates that this selection most likely occurs on the part of households rather than schools.

Education, Randomized Controlled Trial --- Public-Private Partnership --- Universal Access

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With the acceptance of international criminal procedure as a self-sustaining discipline and as the tribunals established to try the most serious crimes in the former Yugoslavia, Sierra Leone, and Rwanda have completed or are beginning to wind up their activities, the time is ripe for a critical evaluation of these international criminal tribunals and their legacy. By examining the due process standards embraced by the five contemporary international criminaltribunals, the author draws conclusions about how the right to a fair trial should be interpreted in international criminal law.

International criminal law --- Fair trial --- Right to a fair trial --- Trial, Fair --- Due process of law --- Criminal law, International --- ICL (International criminal law) --- Criminal law --- International law --- Criminal jurisdiction --- International crimes

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Civil procedure --- Procedure (Law) --- Actions and defenses --- Appellate procedure --- Trial practice --- Law and legislation