Both innate and adaptive immune responses are dependent on activation of nuclear factor κB (NF-κB), induced upon binding of pathogen-associated molecular patterns to Toll-like receptors (TLRs). In murine models, defects in NF-κB pathway are often lethal and viable knockout mice have severe immune defects. Similarly, defects in the human NF-κB pathway described to date lead to severe clinical disease. Here, we describe a patient with a hyper immunoglobulin M–like immunodeficiency syndrome and ectodermal dysplasia. Monocytes did not produce interleukin 12p40 upon stimulation with various TLR stimuli and nuclear translocation of NF-κB was impaired. T cell receptor–mediated proliferation was also impaired. A heterozygous mutation was found at serine 32 in IκBα. Interestingly, his father has the same mutation but displays complex mosaicism. He does not display features of ectodermal dysplasia and did not suffer from serious infections with the exception of a relapsing Salmonella typhimurium infection. His monocyte function was impaired, whereas T cell function was relatively normal. Consistent with this, his T cells almost exclusively displayed the wild-type allele, whereas both alleles were present in his monocytes. We propose that the T and B cell compartment of the mosaic father arose as a result of selection of wild-type cells and that this underlies the widely different clinical phenotype.

In recent years, communication scholars have expressed concerns about the diversity of news media content. While we live in an era of ‘news abundance’ – the number of outlets and channels has increased enormously – the available news is argued to have become more of the same, but has it? As empirical evidence is lacking, this article verifies whether newspapers in Flanders (Belgium), over time, have indeed become less diverse in terms of the news stories they cover. Based on data from a longitudinal content analysis of nine Flemish newspapers at four points in time (1983, 1993, 2003, and 2013), it shows that (1) newspapers, in general, have not become more alike in terms of news stories; (2) newspapers with a similar profile (elite/popular) are less diverse than newspapers with dissimilar profiles; and (3) newspapers owned by the same media group (concentration of media ownership) are more alike than independently owned newspapers.

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Highlights NCA's media merger reviews are evaluated against the width of consumers' interests. Theoretical insights from competition law and media policy are brought together. A claim analysis is applied on NCA's reviews of eight media mergers. NCAs pay limited attention to non-economic interests of consumers and remain vague. It is argued that the potential of the concept of consumer welfare can be increased. To this end, the media's political and socio-cultural role should be considered. Economic, political and socio-cultural welfare in media merger control: An analysis of the Belgian and Dutch competition authorities' reviews of media mergersAbstract: The premise of consumer welfare in competition law entails that National Competition Authorities (NCAs) weigh both economic and non-economic interests of consumers against those of producers. This contribution distinguishes between economic, socio-cultural and political welfare to evaluate whether NCAs examine a merger's impact against the width of consumer interest. A claim analysis is conducted of the NCAs' formal decisions on eight selected cases of proposed media mergers. The analysis shows that, in recent years, these NCAs pay attention to non-economic interests of consumers, but remain vague as to, first, what interests in particular are at stake; second, who the stakeholders are; and, third, how these interests are weighed. The results suggest potential to maximise consumer welfare by safeguarding the media's political and socio-cultural role in particular.To this end, first, the perspective of individuals as citizens must prevail; second, specific test must review the impact of media mergers on political and socio-cultural welfare; and, third, NCAs and Media Regulatory Authorities (MRAs) must bundle strengths.