Open Door, Summer 2003

The Open Door newsletter includes information about the latest happenings at the Iowa Library for the Blind and Physically Handicapped.

Rituximab treatment in Myasthenia gravis: an open-label trial

Myasthenia gravis (MG), an antibody (AB)-mediated autoimmune disorder, responds to treatments targeting the humoral response such as intravenous immunoglobulines (IVIG) and plasma exchange treatments (PEX). Rituximab (RTX), a monoclonal anti-CD20 AB that depletes the specific B lymphocyte population should be efficient and is being used for resistant MG patients in small cohorts. Objectives: This is an observational prospective study that aims to determine the efficacy of RTX in MG, the duration of the clinical effect after treatment and the possible sparing effect on other immunosuppressive drugs.Methods: Between January 2009 and December 2010, 8 MG (2 with anti-MUSK AB) patients (62.5% female) with mean age of 41 years (range 24-79 yo), were treated by RTX. The patients treated were those who experienced serious side-effects and/or treatment failure. In three cases the criteria for treatment was the need to spare frequent recurrent plasmapheresis or IGIV treatment. We compared the functional tests before and prospectively after the treatment (schema used for one cure: 2 9 1gr within 15 days interval), the duration of the efficiency (follow-up of 4-24 months) and we repeated the cures based on clinical criteria.Results: Two patients (25%) underwent 3 RTX cures, 2 (25%) underwent 2 cures and the others (50%) one cure. No adverse events were observed. Six patients (75%) showed a clinical response with improvement of the functional scores and reduction of the concomitant immunosuppressive treatments (75% for prednisone, 35% for other immunosuppressive drugs) that persists over a period of 4-9 months. Follow-up of clinical state and lymphocyte count showed an inverse correlation between the CD 19 count and the clinical state of the patients.Conclusion: In this small series of patients RTX treatment shows significant improvement of clinical state of MG refractory to conventional treatment patients, without side-effects reported, even in patients that were retreated. Larger studies should be held to determine if RTX could be an alternative to plasmapheresis and IVIG as second-line treatment in MG.

Transforming growth factor-beta: the breaking open of a black box.

Transforming growth factor-beta (TGF-beta) and its related proteins regulate broad aspects of body development, including cell proliferation, differentiation, apoptosis and gene expression, in various organisms. Deregulated TGF-beta function has been causally implicated in the generation of human fibrotic disorders and in tumor progression. Nevertheless, the molecular mechanisms of TGF-beta action remained essentially unknown until recently. Here, we discuss recent progress in our understanding of the mechanism of TGF-beta signal transduction with respect to the regulation of gene expression, the control of cell phenotype and the potential usage of TGF-beta for the treatment of human diseases.

Desarrollo de un portal web para instituciones deportivas utilizando componentes Open Source

El proyecto puede dividirse en dos grandes partes, diseño de un portal deportivo y análisis de una herramienta Open Source para el desarrollo de portales web. El apartado de diseño cuenta con un club de atletismo que proporciona unos requerimientos y necesidades de cara a tener un problema real y no basado en especulaciones. Se ha diseñado tanto la base de datos como la estructura del portal y se tienen en cuenta las necesidades del cliente. La parte de análisis de una herramienta Open Source desglosa los módulos de esta, viendo que necesidades cubre cada uno y que pueden hacer, que tecnologías usan y que soluciones pueden dar al problema planteado.

Correction of pectus excavatum combined with open heart surgery in a patient with Marfan's syndrome.

We report a patient with Marfan's syndrome and pectus excavatum who underwent open heart surgery with simultaneous correction of the sternal malformation. Permanent internal stabilization, achieved by bilateral overlapping of the bevelled ends of the lowest ribs and reinforced with sternal closure wires offered a maintained postoperative chest wall stability, avoided the potential postoperative complications of cardiac compression, and improved the aesthetic appearance of the anterior chest wall. The increased risk of bleeding due to extensive dissection was minimized by postponing the repair of pectus excavatum to when protamin is administered after termination of cardiopulmonary bypass.

Free-and open source software for a course on network management: authoring and enactment of scripts based on collaborative learning strategies

This paper describes a Computer-Supported Collaborative Learning (CSCL) case study in engineering education carried out within the context of a network management course. The case study shows that the use of two computing tools developed by the authors and based on Free- and Open-Source Software (FOSS) provide significant educational benefits over traditional engineering pedagogical approaches in terms of both concepts and engineering competencies acquisition. First, the Collage authoring tool guides and supports the course teacher in the process of authoring computer-interpretable representations (using the IMS Learning Design standard notation) of effective collaborative pedagogical designs. Besides, the Gridcole system supports the enactment of that design by guiding the students throughout the prescribed sequence of learning activities. The paper introduces the goals and context of the case study, elaborates onhow Collage and Gridcole were employed, describes the applied evaluation methodology, anddiscusses the most significant findings derived from the case study.

Closed and open grade I and II tibial shaft fractures treated by reamed intramedullary nailing

RESUME Objectif : Le but de cette étude est d'évaluer les résultats du traitement par enclouage centre-médullaire alésé des fractures diaphysaires du tibia aussi bien fermées que pour les fractures ouvertes de stade I et II selon Gustillo. Méthodes: Entre 1997 et 2000, 119 patients présentant une fracture diaphysaire du tibia ont été traités dans notre service par un enclouage centre-médullaire alésé. En postopératoire, 96 patients, soit 70 fractures fermées et 26 fractures ouvertes I et II selon Gustillo, ont été suivis cliniquement et radiologiquement pour une période de plus d'une année et demi. L'introduction du clou centro-médullaire a été faite selon la technique habituelle, soit transtendineuse ou paratendineusé rotulienne. Tous les clous ont été alésés jusqu'à 1,5 mm au-dessus du diamètre du clou. Les patients ayant une fracture isolée du tibia ont été immédiatement mobilisés en charge partielle pour une période de 6 semaines. Ceux qui avaient des lésions associées, notamment au niveau de la cheville épsilatérale, ont nécessité la mise en place d'un plâtre de Type Sarmiento. Résultats : Six patients (6,3%) ont développé un syndrome des loges après chirurgie. Quarante-huit cas (50%) ont nécessité une dynamisation du clou après une période moyenne de 12 semaines en raison d'un retard de consolidation. En général, 90,6% des fractures ont consolidé après 24 semaines postopératoires en moyenne, sans aucune différence significative entre les fractures fermées et les fractures ouvertes. Deux patients (2,1 %) présentant une fracture ouverte degré II ont développé une infection profonde ayant nécessité un traitement. Nous avons également observé 9,4% de cals vicieux minimes et sans conséquence clinique. Huit patients (8,3%) ont eu une brisure des vis de .verrouillage mais également sans conséquence clinique. Au dernier contrôle, 52% des patients, dont ('introduction du clou s'est faite en transtendineux ont des douleurs antérieures du genou contre 14% parmi ceux où l'introduction était paratendineuse. Conclusion : L'enclouage centre-médullaire reste le traitement de choix pour les fractures diaphysaires du tibia, qu'elles soient fermées ou ouvertes degré I ou II selon Gustillo.

Intraoral open reduction and internal fixation of displaced mandibular angle fractures using a specific ad hoc reduction-compression forceps: a preliminary study

OBJECTIVES: To preliminarily evaluate prospectively the accuracy and reliability of a specific ad hoc reduction-compression forceps in intraoral open reduction of transverse and displaced mandibular angle fractures. STUDY DESIGN: We analyzed the clinical and radiologic data of 7 patients with 7 single transverse and displaced angle fractures. An intraoral approach was performed in all of the patients without using perioperative intermaxillary fixation. A single Arbeitsgemeinschaft Osteosynthese (AO) unilock reconstruction plate was fixed to each stable fragment with 3 locking screws (2.0 mm in 5 patients and 2.4 mm in 2 patients) at the basilar border of the mandible, according to AO/American Society of Internal Fixation (ASIF) principles. Follow-up was at 1, 3, 6, and 12 months, and we noted the status of healing and complications, if any. RESULTS: All of the patients had satisfactory fracture reduction as well as a successful treatment outcome without complications. CONCLUSION: This preliminary study demonstrated that the intraoral reduction of transverse and displaced angle fractures using a specific ad hoc reduction-forceps results in a high rate of success.

Vemurafenib in patients with BRAF(V600) mutation-positive melanoma with symptomatic brain metastases: final results of an open-label pilot study.

BACKGROUND & AIM: Brain metastases are frequent in patients with metastatic melanoma, indicating poor prognosis. We investigated the BRAF kinase inhibitor vemurafenib in patients with advanced melanoma with symptomatic brain metastases. METHODS: This open-label trial assessed vemurafenib (960mg twice a day) in patients with BRAF(V600) mutation-positive metastatic melanoma with non-resectable, previously treated brain metastases. The primary end-point was safety. Secondary end-points included best overall response rate, and progression-free and overall survival. RESULTS: Twenty-four patients received vemurafenib for a median treatment duration of 3.8 (0.1-11.3) months. The majority of discontinuations were due to disease progression (n=22). Twenty-three of 24 patients reported at least one adverse event (AE). Grade 3 AEs were reported in four (17%; 95% confidence interval [CI], 4.7-37.4%) patients and included cutaneous squamous cell carcinoma in four patients. Median progression-free survival was 3.9 (95% CI, 3.0-5.5) months, and median survival was 5.3 (95% CI, 3.9-6.6) months. An overall partial response (PR) at both intracranial and extracranial sites was achieved in 10 of 24 (42%; 95% CI, 22.1-63.4) evaluable patients, with stable disease in nine (38%; 95% CI, 18.8-59.4) patients. Of 19 patients with measurable intracranial disease, seven (37%) achieved >30% intracranial tumour regression, and three (16%; 95% CI, 3.4-39.6%) achieved a confirmed PR. Other signs of improvement included reduced need for corticosteroids and enhanced performance status. CONCLUSIONS: Vemurafenib can be safely used in patients with advanced symptomatic melanoma that has metastasised to the brain and can result in meaningful tumour regression.

Open source business strategies in the domain of embedded systems

El tema de este estudio es el aumento de la comprensión teórica y empírica de la estrategia de negocio de código abierto en el dominio de sistemas embebidos por investigar modelos de negocios de código abierto, retos, recursos y capacidades operativas y dinámicas.

Open Door Newsletter of the Library, Summer 2006

The Open Door newsletter is produced by the Iowa Commission for the Blind. It is published twice a year with information about the library programs and what is going on.

Open Door Newsletter of the Library, Winter 2003

The Open Door newsletter is produced by the Iowa Commission for the Blind. It is published twice a year with information about the library programs and what is going on.

Open Door Newsletter of the Library, Winter 2003

The Open Door newsletter is produced by the Iowa Commission for the Blind. It is published twice a year with information about the library programs and what is going on.