Ex post evaluation of the management and implementation of cohesion policy 2000-06 (ERDF)

This report has been drafted by the European Policies Research Centre (University of Strathclyde) as part of an ex post evaluation of the management and implementation systems for Cohesion policy, 2000-06, which has been commissioned by DG REGIO and which is being managed by EPRC and Metis (Vienna) under European Commission contract no: 2007.CE.16.0.AT.034. The report provides an overview of the main features of management and implementation systems across the EU25 in the 2000-06 period (2004-06 for the EU10) and has been drafted by Professor John Bachtler, Laura Polverari and Frederike Gross, with assistance from Dr Sara Davies and Ruth Downes. The research is based on studies of individual countries undertaken by EPRC together with national experts from each of the EU25 Member States. The authors are grateful for helpful comments from the DG REGIO Evaluation Unit and Geographical Units, in particular Anna Burylo, Veronica Gaffey and Kai Stryczynski. Any errors or omissions remain the responsibility of the authors

Liver transplantation for type IV glycogen storage disease

TYPE IV glycogen storage disease is a rare autosomal recessive disorder (also called Andersen's disease1 or amylopectinosis) in which the activity of branching enzyme alpha-1, 4-glucan: alpha-1, 4-glucan 6-glucosyltransferase is deficient in the liver as well as in cultured skin fibroblasts and other tissues.2,3 This branching enzyme is responsible for creating branch points in the normal glycogen molecule. In the relative or absolute absence of this enzyme, an insoluble and irritating form of glycogen, an amylopectin-like polysaccharide that resembles plant starch, accumulates in the cells. The amylopectin-like form is less soluble than normal glycogen, with longer outer and inner chains. © 1991, Massachusetts Medical Society. All rights reserved

Real-time analysis of gene regulation by glucocorticoid hormones

There is increasing evidence that temporal factors are important in allowing cells to gain additional information from external factors, such as hormones and cytokines. We sought to discover how cell responses to glucocorticoids develop over time, and how the response kinetics vary according to ligand structure and concentration, and hence have developed a continuous gene transcription measurement system, based on an interleukin-6 (IL-6) luciferase reporter gene. We measured the time to maximal response, maximal response and integrated response, and have compared these results with a conventional, end point glucocorticoid bioassay. We studied natural glucocorticoids (corticosterone and cortisol), synthetic glucocorticoids (dexamethasone) and glucocorticoid precursors with weak, or absent bioactivity. We found a close correlation between half maximal effective concentration (EC50) for maximal response, and for integrated response, but with consistently higher EC50 for the latter. There was no relation between the concentration of ligand and the time to maximal response. A comparison between conventional end point assays and real-time measurement showed similar effects for dexamethasone and hydrocortisone, with a less effective inhibition of IL-6 seen with corticosterone. We profiled the activity of precursor steroids, and found pregnenolone, progesterone, 21-hydroxyprogesterone and 17-hydroxyprogesterone all to be ineffective in the real-time assay, but in contrast, progesterone and 21-hydroxyprogesterone showed an IL-6 inhibitory activity in the end point assay. Taken together, our data show how ligand concentration can alter the amplitude of glucocorticoid response, and also that a comparison between real-time and end point assays reveals an unexpected diversity of the function of glucocorticoid precursor steroids, with implications for human disorders associated with their overproduction

The Prenatal Environment in Twin Studies: A Review on Chorionicity

A literature search was conducted to identify articles examining the association of chorionicity (e.g., whether twins share a single chorion and thus placenta or have separate chorions/placentas) and genetics, psychiatry/behavior, and neurological manifestations in humans twins and higher-order multiples. The main aim was to assess how frequently chorionicity has been examined in relation to heritability estimates, and to assess which phenotypes may be most sensitive to, or affected by, bias in heritability estimates because of chorionicity. Consistent with the theory that some chorionicity effects could lead to overestimation and others to underestimation of heritability, there were instances of each across the many phenotypes reviewed. However, firm conclusions should not be drawn since some of the outcomes were only examined in one or few studies and often sample sizes were small. While the evidence for bias due to chorionicity was mixed or null for many outcomes, results do, however, consistently suggest that heritability estimates are underestimated for measures of birth weight and early growth when chorionicity is not taken into account

Traumatic quadriceps rupture in a patient with patellectomy: a case report

<p>Abstract</p> <p>Introduction</p> <p>Acute traumatic, unilateral, quadriceps rupture after patellectomy is rare.</p> <p>Case presentation</p> <p>We present a 42-year old male who experienced a unilateral left quadriceps tendon rupture following assault by four people. Twenty-seven years before this injury, the patient had suffered ipsilateral femur and comminuted patellar fractures, which were managed by intramedullary nailing and patellectomy respectively. We performed primary end to end repair of the torn tendon. Postoperatively, histology revealed findings consistent with pre-existent degenerative changes. The patient made good recovery, and returned to his former occupation which was reliant on his ability to drive.</p> <p>Conclusion</p> <p>Degenerative changes of the tendon of the extensor mechanism of knee following patellectomy may predispose the quadriceps tendon to traumatic rupture. Early operative intervention and protracted rehabilitation are required to obtain the best functional results.</p

The Hubble Space Telescope Treasury Program on the Orion Nebula Cluster

The Hubble Space Telescope (HST) Treasury Program on the Orion Nebula Cluster has used 104 orbits of HST time to image the Great Orion Nebula region with the Advanced Camera for Surveys (ACS), the Wide-Field/Planetary Camera 2 (WFPC2) and the Near Infrared Camera and Multi Object Spectrograph (NICMOS) instruments in 11 filters ranging from the U-band to the H-band equivalent of HST. The program has been intended to perform the definitive study of the stellar component of the ONC at visible wavelengths, addressing key questions like the cluster IMF, age spread, mass accretion, binarity and cirumstellar disk evolution. The scanning pattern allowed to cover a contiguous field of approximately 600 square arcminutes with both ACS and WFPC2, with a typical exposure time of approximately 11 minutes per ACS filter, corresponding to a point source depth AB(F435W) = 25.8 and AB(F775W)=25.2 with 0.2 magnitudes of photometric error. We describe the observations, data reduction and data products, including images, source catalogs and tools for quick look preview. In particular, we provide ACS photometry for 3399 stars, most of them detected at multiple epochs, WFPC2 photometry for 1643 stars, 1021 of them detected in the U-band, and NICMOS JH photometry for 2116 stars. We summarize the early science results that have been presented in a number of papers. The final set of images and the photometric catalogs are publicly available through the archive as High Level Science Products at the STScI Multimission Archive hosted by the Space Telescope Science Institute.Comment: Accepted for publication on the Astrophysical Journal Supplement Series, March 27, 201

Liver transplantation for type I and type IV glycogen storage disease

Progressive liver failure or hepatic complications of the primary disease led to orthotopic liver transplantation in eight children with glycogen storage disease over a 9-year period. One patient had glycogen storage disease (GSD) type I (von Gierke disease) and seven patients had type IV GSD (Andersen disease). As previously reported [19], a 16.5-year-old-girl with GSD type I was successfully treated in 1982 by orthotopic liver transplantation under cyclosporine and steroid immunosuppression. The metabolic consequences of the disease have been eliminated, the renal function and size have remained normal, and the patient has lived a normal young adult life. A late portal venous thrombosis was treated successfully with a distal splenorenal shunt. Orthotopic liver transplantation was performed in seven children with type N GSD who had progressive hepatic failure. Two patients died early from technical complications. The other five have no evidence of recurrent hepatic amylopectinosis after 1.1–5.8 postoperative years. They have had good physical and intellectual maturation. Amylopectin was found in many extrahepatic tissues prior to surgery, but cardiopathy and skeletal myopathy have not developed after transplantation. Postoperative heart biopsies from patients showed either minimal amylopectin deposits as long as 4.5 years following transplantation or a dramatic reduction in sequential biopsies from one patient who initially had dense myocardial deposits. Serious hepatic derangement is seen most commonly in types T and IV GSD. Liver transplantation cures the hepatic manifestations of both types. The extrahepatic deposition of abnormal glycogen appears not to be problematic in type I disease, and while potentially more threatening in type IV disease, may actually exhibit signs of regression after hepatic allografting