Fibrose rétropéritonéale: cause rare d'insuffisance rénale postrénale, a ne pas méconnaître [Retroperitoneal fibrosis: a rare cause of postrenal kidney failure, no to be missed].

We report the observation of a fifty years old man, admitted in the emergency room for bilateral lumbar pain and hyperkaliemic metabolic acidosis, and postrenal kidney failure induced by bilateral hydronephrosis. Radiographic exploration and histologic studies of biopsy confirmed an idiopathic retroperitoneal fibrosis that clinically and biologicaly responded to three seances of hemodialysis, and insertion in each uretere of one double J stent, and long term corticotherapy. The retroperitoneal fibrosis is a little common inflammatory disease, characterized by the development of a fibrous mass around the retroperitoneal structures. His diagnostic means evolved. On the other hand, his treatment was the object of no checked controlled and randomized trial. This article proposes an updating of the knowledge on this subject.

An unusual cause of hemoptysis--aberrant origin of the left pulmonary artery from the ascending aorta in the adult.

Aberrant origin of a pulmonary artery from the ascending aorta is an uncommon congenital vascular malformation with poor survival without surgery. In this case report, we describe the unusual late diagnosis of this congenital malformation in an otherwise asymptomatic young man presenting with mild hemoptysis. We review the natural and modified history of this defect and the relevant aspects of follow-up in adult life.

El just-in-time i la fallida dels sistemes productius al Japó al març de 2011

El document descriu com el just-in-time va afavorir la fallida dels sistemes productius de les empreses d'automoció al Japó durant el gran terratrèmol de l'any 2011.

Just the Facts for 2012

There are 20,552 full-time employees who work for the State of Iowa Executive Branch(excluding Fair Authority, Community-Based Corrections, and the Regents employees). These employees are undoubtedly the most valuable resource for providing timely and quality services to Iowans. To strategically manage this resource, state departments and policymakers must have thorough and accurate information. The information in “Just the Facts for 2008” is a snapshot of the workforce, collected,compiled, and presented in a format that will aid agencies and decision makers in strategic planning. In many cases, data cover a number of years and are presented to give the reader a sense of trends. While the Department of Administrative Services, Human Resource Enterprise (DAS/HRE)wants to present data in its purest form so readers can draw their own conclusions, we also have a responsibility to clarify anything that may be confusing or misleading. It is important to highlight workforce trends and explain their significance to the work of Iowa state government. The following chapter summaries are intended to do that.

Just the Facts for 2013

DAS/HRE publishes "Just the Facts" an almanac on the Executive Branch workforce. To assist state decision makers in preparing for the future. Thorough strategic planning is requisite to state government's future ability to provide quality products and services. The almanac is intended as an historical source of workforce date that can be used for comparative purposes as the workforce continues to change in Iowa.

Mutations in B3GALT6, which encodes a glycosaminoglycan linker region enzyme, cause a spectrum of skeletal and connective tissue disorders.

Proteoglycans (PGs) are a major component of the extracellular matrix in many tissues and function as structural and regulatory molecules. PGs are composed of core proteins and glycosaminoglycan (GAG) side chains. The biosynthesis of GAGs starts with the linker region that consists of four sugar residues and is followed by repeating disaccharide units. By exome sequencing, we found that B3GALT6 encoding an enzyme involved in the biosynthesis of the GAG linker region is responsible for a severe skeletal dysplasia, spondyloepimetaphyseal dysplasia with joint laxity type 1 (SEMD-JL1). B3GALT6 loss-of-function mutations were found in individuals with SEMD-JL1 from seven families. In a subsequent candidate gene study based on the phenotypic similarity, we found that B3GALT6 is also responsible for a connective tissue disease, Ehlers-Danlos syndrome (progeroid form). Recessive loss-of-function mutations in B3GALT6 result in a spectrum of disorders affecting a broad range of skeletal and connective tissues characterized by lax skin, muscle hypotonia, joint dislocation, and spinal deformity. The pleiotropic phenotypes of the disorders indicate that B3GALT6 plays a critical role in a wide range of biological processes in various tissues, including skin, bone, cartilage, tendon, and ligament.

Recueil des lois, ordonnances, décrets et règlements relatifs aux alignements, à l'expropriation pour cause d'utilité publique, spécialement dans les voies de Paris / dressé sous la direction de M. Alphand,... par MM. A. Deville,... L. Rez,...

[Acte royal. 1607-1885]