Measurement of the Atmospheric Muon Spectrum from 20 to 3000 GeV

The absolute muon flux between 20 GeV and 3000 GeV is measured with the L3 magnetic muon spectrometer for zenith angles ranging from 0 degree to 58 degree. Due to the large exposure of about 150 m2 sr d, and the excellent momentum resolution of the L3 muon chambers, a precision of 2.3 % at 150 GeV in the vertical direction is achieved. The ratio of positive to negative muons is studied between 20 GeV and 500 GeV, and the average vertical muon charge ratio is found to be 1.285 +- 0.003 (stat.) +- 0.019 (syst.).Comment: Total 32 pages, 9Figure

De kredietcrisis

ARTIKELEN: 1. W.G. Wolters - Spelen met financiële risico's in een onzekere wereld 2. W. Huisman - Kredietcrisis en organisatiecriminaliteit: vier mogelijkheden 3. B.M.J. Slot - Elke crisis haar eigen crimineel 4. E. Mecking - De kredietcrisis en de lessen van Kondratieff; een doemscenario 5. B. Unger - Wie is verantwoordelijk voor de financiële crisis? 6. D. Schoenmaker - Financieel toezicht op Europees niveau 7. W.F. van Raaij - De economische crisis en het herstel van vertrouwen 8. Boekrecensie: J. J. van Duijn over 'Animal spirits: how human psychology drives the economy, and why it matters for global capitalism' van G.A. Akerlof en R.J. Shiller 9. Boekrecensie: P.W. Zuidhof over 'Op naar de volgende crisis! Over het verleidende vermogen van de financiële markt' van O. Velthuis en L. Noordegraaf-Eelens SAMENVATTING: In dit themanummer is gekozen voor een brede benadering van het fenomeen kredietcrisis. Zo komt de relatie tussen kredietcrisis en criminaliteit aan de orde en wordt aandacht besteed aan financiële zwendel in heden en verleden en aan de (verschuivende) grenzen tussen moreel, immoreel en crimineel handelen. Het onderwerp 'vertrouwen' komt in verschilende bijdragen aan bod, evenals de gevolgen van de kredietcrisis voor de inrichting van het toezicht op de financiële sector. Om een en ander in een context te plaatsen zijn ook enkele meer economisch getinte bijdragen opgenomen waarin wordt ingegaan op de mogelijke oorzaken van de crisis

Chylous ascites in cirrhosis: a case report and review of the literature

Chylous ascites is an uncommon clinical entity which results from the accumulation of fat, predominantly chylomicrons, in the ascitic fluid. Conventional treatment methods are unsatisfactory. A patient is reported with chylous ascites associated with cirrhosis and portal hypertension in whom the ascites, the renal insufficiency and the fluid and electrolyte disturbances were corrected by the insertion of a Denver peritoneovenous shunt

Leri's pleonosteosis, a congenital rheumatic disease, results from microduplication at 8q22.1 encompassing GDF6 and SDC2 and provides insight into systemic sclerosis pathogenesis.

OBJECTIVES: Leri's pleonosteosis (LP) is an autosomal dominant rheumatic condition characterised by flexion contractures of the interphalangeal joints, limited motion of multiple joints, and short broad metacarpals, metatarsals and phalanges. Scleroderma-like skin thickening can be seen in some individuals with LP. We undertook a study to characterise the phenotype of LP and identify its genetic basis. METHODS AND RESULTS: Whole-genome single-nucleotide polymorphism genotyping in two families with LP defined microduplications of chromosome 8q22.1 as the cause of this condition. Expression analysis of dermal fibroblasts from affected individuals showed overexpression of two genes, GDF6 and SDC2, within the duplicated region, leading to dysregulation of genes that encode proteins of the extracellular matrix and downstream players in the transforming growth factor (TGF)-β pathway. Western blot analysis revealed markedly decreased inhibitory SMAD6 levels in patients with LP. Furthermore, in a cohort of 330 systemic sclerosis cases, we show that the minor allele of a missense SDC2 variant, p.Ser71Thr, could confer protection against disease (p<1×10(-5)). CONCLUSIONS: Our work identifies the genetic cause of LP in these two families, demonstrates the phenotypic range of the condition, implicates dysregulation of extracellular matrix homoeostasis genes in its pathogenesis, and highlights the link between TGF-β/SMAD signalling, growth/differentiation factor 6 and syndecan-2. We propose that LP is an additional member of the growing 'TGF-β-pathies' group of musculoskeletal disorders, which includes Myhre syndrome, acromicric dysplasia, geleophysic dysplasias, Weill-Marchesani syndromes and stiff skin syndrome. Identification of a systemic sclerosis-protective SDC2 variant lays the foundation for exploration of the role of syndecan-2 in systemic sclerosis in the future