Pohjoismainen hyvinvointivaltio ja keskiluokka

Teema: pohjoismainen hyvinvointivaltio. Artikkeli perustuu alustukseen, joka pidettiin 28.1.2008 Suomalaisen Tiedeakatemian juhlaseminaarissa "Pohjoismaat: malli vai umpikuja?"

FNA: rossz ötlet a jóléti állam szempontjából

Bo Rothstein: UBI: A Bad Idea For The Welfare State, in Philippe van Parijs (ed.): Basic Income And The Left: A European Debate, Social Europe Edition London, 2017, 103–110

CORRUPTION, GENDER EQUALITY AND FEMINIST STRATEGIES

ABSTRACT The following arguments are presented. 1) Corruption in its various forms is a serious social ill. 2) Democracy is not a safe cure against corruption. 3) Increased gender equality seems to be one important factor behind getting corruption under control. 4) Impartiality in the exercise of public power, not least, when it "translates" into meritocratic recruitment and promotion in the public administration, has a powerful effect on lowering corruption. 5) While some aspects of impartiality are central for gender equality, research results are mixed. Some show that impartial principles promotes gender equality, others show that gender bias exists also in many processes designed to be impartial. Going from these results to policy recommendation is thus fraught with many difficulties. One is how to handle problems of legitimacy in the implementation process for various forms of preferential treatment of discriminated groups. Since these problems are impossible to handle, we may be in for a "Churchillian" argument. Like representative democracy, meritocracy may be a far from ideal solution for lowering corruption and thereby promoting human well-being, but it may be the least bad of existing alternatives

Association of Variants in the SPTLC1 Gene With Juvenile Amyotrophic Lateral Sclerosis

Importance: Juvenile amyotrophic lateral sclerosis (ALS) is a rare form of ALS characterized by age of symptom onset less than 25 years and a variable presentation.Objective: To identify the genetic variants associated with juvenile ALS.Design, Setting, and Participants: In this multicenter family-based genetic study, trio whole-exome sequencing was performed to identify the disease-associated gene in a case series of unrelated patients diagnosed with juvenile ALS and severe growth retardation. The patients and their family members were enrolled at academic hospitals and a government research facility between March 1, 2016, and March 13, 2020, and were observed until October 1, 2020. Whole-exome sequencing was also performed in a series of patients with juvenile ALS. A total of 66 patients with juvenile ALS and 6258 adult patients with ALS participated in the study. Patients were selected for the study based on their diagnosis, and all eligible participants were enrolled in the study. None of the participants had a family history of neurological disorders, suggesting de novo variants as the underlying genetic mechanism.Main Outcomes and Measures: De novo variants present only in the index case and not in unaffected family members.Results: Trio whole-exome sequencing was performed in 3 patients diagnosed with juvenile ALS and their parents. An additional 63 patients with juvenile ALS and 6258 adult patients with ALS were subsequently screened for variants in the SPTLC1 gene. De novo variants in SPTLC1 (p.Ala20Ser in 2 patients and p.Ser331Tyr in 1 patient) were identified in 3 unrelated patients diagnosed with juvenile ALS and failure to thrive. A fourth variant (p.Leu39del) was identified in a patient with juvenile ALS where parental DNA was unavailable. Variants in this gene have been previously shown to be associated with autosomal-dominant hereditary sensory autonomic neuropathy, type 1A, by disrupting an essential enzyme complex in the sphingolipid synthesis pathway.Conclusions and Relevance: These data broaden the phenotype associated with SPTLC1 and suggest that patients presenting with juvenile ALS should be screened for variants in this gene.</p