Braucht die Wirtschaft eine neue Ordnungspolitik?

In der deutschen Volkswirtschaftslehre wird ein Streit zwischen Anhängern der Ordnungspolitik und Anhängern der formal-theoretischen Ökonomik ausgetragen. Die Ordnungspolitiker setzen dabei vor allem auf Regeln und Institutionen, die der Marktwirtschaft einen Rahmen geben sollen. Den theoretischen Ökonomen werfen sie Realitätsferne vor. Entsprechend bezieht sich der Streit auch auf die Politikberatung. Hier müssen sich die Ökonomen mit dem Vorwurf auseinandersetzen, bei der Vorhersage der Finanzkrise versagt zu haben

Clinical and immunologic phenotype associated with activated phosphoinositide 3-kinase δ syndrome 2: A cohort study

Background Activated phosphoinositide 3-kinase δ syndrome (APDS) 2 (p110δ-activating mutations causing senescent T cells, lymphadenopathy, and immunodeficiency [PASLI]–R1), a recently described primary immunodeficiency, results from autosomal dominant mutations in PIK3R1, the gene encoding the regulatory subunit (p85α, p55α, and p50α) of class IA phosphoinositide 3-kinases. Objectives We sought to review the clinical, immunologic, and histopathologic phenotypes of APDS2 in a genetically defined international patient cohort. Methods The medical and biological records of 36 patients with genetically diagnosed APDS2 were collected and reviewed. Results Mutations within splice acceptor and donor sites of exon 11 of the PIK3R1 gene lead to APDS2. Recurrent upper respiratory tract infections (100%), pneumonitis (71%), and chronic lymphoproliferation (89%, including adenopathy [75%], splenomegaly [43%], and upper respiratory tract lymphoid hyperplasia [48%]) were the most common features. Growth retardation was frequently noticed (45%). Other complications were mild neurodevelopmental delay (31%); malignant diseases (28%), most of them being B-cell lymphomas; autoimmunity (17%); bronchiectasis (18%); and chronic diarrhea (24%). Decreased serum IgA and IgG levels (87%), increased IgM levels (58%), B-cell lymphopenia (88%) associated with an increased frequency of transitional B cells (93%), and decreased numbers of naive CD4 and naive CD8 cells but increased numbers of CD8 effector/memory T cells were predominant immunologic features. The majority of patients (89%) received immunoglobulin replacement; 3 patients were treated with rituximab, and 6 were treated with rapamycin initiated after diagnosis of APDS2. Five patients died from APDS2-related complications. Conclusion APDS2 is a combined immunodeficiency with a variable clinical phenotype. Complications are frequent, such as severe bacterial and viral infections, lymphoproliferation, and lymphoma similar to APDS1/PASLI-CD. Immunoglobulin replacement therapy, rapamycin, and, likely in the near future, selective phosphoinositide 3-kinase δ inhibitors are possible treatment options

An overview of test standards and regulations relevant to the fire testing of pressure vessels

Fire exposure of storage and transportation vessels of hazardous materials (including pressure liquefied gases) can result in BLEVEs and other high-consequence incidents with large societal and economic impacts. To reduce risk most countries have numerous regulations, codes of practice and guidance notes covering the design, operation and maintenance of vessels and thermal protection systems. Yet despite such regulations there remains no internationally accepted fire test procedure for pressure vessel and accompanying thermal protection systems that is capable of meeting a range of regulatory requirements. This paper considers some of the regulations in place in the western world and considers the origin of these based on large and medium-scale testing conducted to date. It examines conditions found in these tests to propose a set of recommendations on which to base a standard method of test. These recommendations are proposed as being representative of a credible large pool fire scenario that may occur