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To link to full-text access for this article, visit this link: http://dx.doi.org/10.1016/j.respol.2005.07.003 Byline: Paul B. de Laat Keywords: Commons; Intellectual property rights; Licensing; Open source; Software Abbreviations: BSD, Berkeley Software Distribution; FSF, Free Software Foundation; GPL, General Public License; IPRs, intellectual property rights; LGPL, Library (or Lesser) General Public License; MPL, Mozilla Public License; NPL, Netscape Public License; OSI, Open Source Initiative Abstract: Two property regimes for software development may be distinguished. Within corporations, on the one hand, a Private Regime obtains which excludes all outsiders from access to a firm's software assets. It is shown how the protective instruments of secrecy and both copyright and patent have been strengthened considerably during the last two decades. On the other, a Public Regime among hackers may be distinguished, initiated by individuals, organizations or firms, in which source code is freely exchanged. It is argued that copyright is put to novel use here: claiming their rights, authors write 'open source licenses' that allow public usage of the code, while at the same time regulating the inclusion of users. A 'regulated commons' is created. The analysis focuses successively on the most important open source licenses to emerge, the problem of possible incompatibility between them (especially as far as the dominant General Public License is concerned), and the fragmentation into several user communities that may result. Author Affiliation: Faculty of Philosophy, University of Groningen, Oude Boteringestraat 52, 9712 GL Groningen, The Netherlands Article History: Received 22 April 2004; Revised 6 July 2005; Accepted 7 July 2005

BackgroundThe mitochondrial DNA (mDNA) 3243A>G variant is the most common pathogenic variant of the mDNA. To interpret results of clinical trials in mitochondrial disease, it is important to have a clear understanding of the natural course of disease. To obtain more insight into the disease burden and the progression of disease in carriers of the mDNA 3243 A>G variant, we followed a cohort of 151 carriers from 61 families prospectively for up to 6 years.MethodsThe disease severity was scored using the Newcastle Mitochondrial Disease Adult Scale (NMDAS), including SF-36 quality of life (QoL) scores. Heteroplasmy levels were measured in urinary epithelial cells (UEC), leucocytes and saliva. The progression of the disease was studied using linear mixed model analysis.ResultsOne hundred twenty-four carriers (out of 151) were symptomatic. Four clinical groups were identified: 1) classical mitochondrial encephalomyopathy, lactic acidosis and stroke-like episodes syndrome (n=7), 2) maternally inherited diabetes deafness syndrome (n=60), 3) ‘other’ (n=57) and 4) dormant carriers (n=27). A yearly increase of NMDAS score of 0.47 point was measured in the total group. Heteroplasmy levels in both leucocytes and UEC were only weakly correlated with disease severity. Physical QoL declined with age. The most important determinants of QoL decline were hearing loss, speech problems, exercise intolerance, gait instability, psychiatric problems and gastrointestinal involvement.ConclusionThe mDNA 3243 A>G variant causes a slowly progressive disease, with a yearly increase of NMDAS score of ~0.5 point overall with the clinical phenotype being the only determinant of disease progression.

The m.3243A>G mutation is the most prevalent, disease-causing mitochondrial DNA (mtDNA) mutation. In a national cohort study of 48 families harbouring the m.3243A>G mutation, we identified three families in which the mutation appeared to occur sporadically within these families. In this report we describe these three families. Based on detailed mtDNA analysis of three different tissues using two different quantitative pyrosequencing assays with sensitivity to a level of 1% mutated mtDNA, we conclude that the m.3243A>G mutation has arisen de novoin each of these families. The symptomatic carriers presented with a variety of symptoms frequently observed in patients harbouring the m.3243A>G mutation. A more severe phenotype is seen in the de novofamilies compared to recent cohort studies, which might be due to reporting bias.

To determine the value of fibroblast growth factor 21 (FGF21), a recently discovered biomarker for mitochondrial disease, in predicting clinical disease severity and disease progression in adult carriers of the m.3243A>G mutation.

In `real' space, third partieshave always been useful to facilitatetransactions. With cyberspace opening up, it isto be expected that intermediation will alsodevelop in a virtual fashion. The articlefocuses upon new cyberroles for third partiesthat seem to announce themselves clearly.First, virtualization of the market place haspaved the way for `cybermediaries', who brokerbetween supply and demand of material andinformational goods. Secondly,cybercommunication has created newuncertainties concerning informational securityand privacy. Also, as in real space,transacting supposes some decency with one'spartners. These needs are being addressed byTrusted Third Parties, anonymizers, escrowarrangements, facilitators and externalauditing. Virtual reputation trackingmechanisms are being developed as well.Finally, in order to resolve disputes,mediators and arbitrators have started offeringtheir services online. In the closing sectionthese emerging cyberroles are assessedcritically. It is argued in particular, thatboth cybermediaries and cyberjustice poseserious threats to privacy. Moreover, onlinedispute resolution, as it is practised now,neglects its duties of public accounting.

Graves’ disease is the most prevalent cause of hyperthyroidism in children. The treatment commonly involves antithyroid therapy using a thionamide. We present a case of a 13-year-old girl with the antithyroid arthritis syndrome, presenting as a migratory polyarthritis, after the initiation of thionamide treatment for Graves’ disease. Antithyroid arthritis syndrome warranted immediate cessation of thionamide. Improvement of the arthritis was seen in subsequent days. As there are no other reversible treatment modalities for Graves’ disease in children, definitive treatment with radioactive iodine was needed to control the hyperthyroidism in this child. Antithyroid arthritis syndrome presenting as a migratory polyarthritis is a severe adverse effect of a common pediatric disease and should therefore be recognized by pediatricians.