Re: Sodium Valproate-Induced Myopathy in a Child

N

Seizure as Initial Manifestation of Aortic Dissection Type A

Background: Seizure as the initial manifestation of aortic dissection is rare.Case report: An 88-year-old female experienced a first generalized tonic clonic seizure, which was terminated with midazolam. Acute cerebral magnetic resonance imaging and angiography were non-informative. After awaking she complained about cramping pain in the right upper extremity, which was accompanied by involuntary flexion movements of the right upper extremity. Blood pressure was initially normal. Blood gases revealed metabolic acidosis and blood chemical investigations a markedly increased D-dimer. Consecutively blood pressure declined and transthoracic echocardiography showed pericardial effusion. A computed tomography scan of the thorax revealed an aortic dissection type A. The patient died 16 hours after admission after cardiothoracic surgeons had refused surgical treatment.Conclusion: This case shows that a generalized tonic-clonic seizure may be the initial manifestation of an aortic dissection type A in the absence of thoracic chest pain and that brachyalgia may not develop earlier than with progression of the dissection. [West J Emerg Med. 2010; 11(5):510-511.

1,2,3,4-Tetra­methyl­cyclo­pent-2-ene-1,4-diol

The title compound, C9H16O2, crystallizes with two mol­ecules in the asymmetric unit. The structure displays inter­molecular O—H⋯O hydrogen bonding

The inner membrane protein Mdm33 controls mitochondrial morphology in yeast

Mitochondrial distribution and morphology depend on MDM33, a Saccharomyces cerevisiae gene encoding a novel protein of the mitochondrial inner membrane. Cells lacking Mdm33 contain ring-shaped, mostly interconnected mitochondria, which are able to form large hollow spheres. On the ultrastructural level, these aberrant organelles display extremely elongated stretches of outer and inner membranes enclosing a very narrow matrix space. Dilated parts of Δmdm33 mitochondria contain well-developed cristae. Overexpression of Mdm33 leads to growth arrest, aggregation of mitochondria, and generation of aberrant inner membrane structures, including septa, inner membrane fragments, and loss of inner membrane cristae. The MDM33 gene is required for the formation of net-like mitochondria in mutants lacking components of the outer membrane fission machinery, and mitochondrial fusion is required for the formation of extended ring-like mitochondria in cells lacking the MDM33 gene. The Mdm33 protein assembles into an oligomeric complex in the inner membrane where it performs homotypic protein–protein interactions. Our results indicate that Mdm33 plays a distinct role in the mitochondrial inner membrane to control mitochondrial morphology. We propose that Mdm33 is involved in fission of the mitochondrial inner membrane

Информационное обеспечение в процессно-ориентированной модели управления предприятиями

Приведено описание информационного обеспечения как составной части менеджмента производственного предприятия в его современном, процессном представлении. Информационное обеспечение предприятий связано с качеством функционирования, которое с точки зрения процессно-ориентированных стандартов характеризуется результативностью и эффективностью.It has been accomplished the description of data-ware as a component of manufacturing enterprise management in its modern process presentation. The data-ware of enterprise is connected with quality of functioning, which is defined by effectiveness and efficiency of the according process-oriented standards

Late Holocene current patterns in the northern Patagonian fjords recorded by sediment drifts in Aysén Fjord

Present-day circulation patterns in the southeastern Pacific Ocean are driven by the Antarctic Circumpolar Current, directing subantarctic surface water into the Patagonian fjords since at least the early Holocene. In this way, bottom current patterns in the area are regulated by the regional climate, although the complex bathymetry of the fjords has a significant impact as well. To understand the potential interplay of climate, seafloor topography and circulation patterns, we study the sedimentary infill of Aysén Fjord (~45°S) and reveal the first active sediment drifts in the region. These allow constraining the present-day circulation patterns in northern Patagonia and show an incoming (southward) as well as returning (northward) flow direction. While the general sedimentary evolution of the fjord (and thus also the sediment drifts) is climate-driven (i.e., it reflects variability in southern westerly wind strength), the onset of drift formation at ~3.7 ka does not seem to have originated from an abrupt change in regional climate. Instead, we propose that a megathrust earthquake described in paleoseismic records in the area could have resulted in subsidence of one (or more) of the many bathymetric highs in the Patagonian fjords, thus contributing to enhanced spilling of subantarctic water into the fjord. This study underscores the importance of multidisciplinary research to understand past and present bottom current circulation patterns and disentangle different possible feedback mechanisms

Expression patterns of transcribed human endogenous retrovirus HERV-K(HML-2) loci in human tissues and the need for a HERV Transcriptome Project

Background: A significant proportion of the human genome is comprised of human endogenous retroviruses (HERVs). HERV transcripts are found in every human tissue. Expression of proviruses of the HERV-K(HML-2) family has been associated with development of human tumors, in particular germ cell tumors (GCT). Very little is known about transcriptional activity of individual HML-2 loci in human tissues, though. Results: By employing private nucleotide differences between loci, we assigned ~1500 HML-2 cDNAs to individual HML-2 loci, identifying, in total, 23 transcriptionally active HML-2 proviruses. Several loci are active in various human tissue types. Transcription levels of some HML-2 loci appear higher than those of other loci. Several HML-2 Rec-encoding loci are expressed in GCT and non-GCT tissues. A provirus on chromosome 22q11.21 appears strongly upregulated in pathologic GCT tissues and may explain high HML-2 Gag protein levels in GCTs. Presence of Gag and Env antibodies in GCT patients is not correlated with activation of individual loci. HML-2 proviruses previously reported capable of forming an infectious HML-2 variant are transcriptionally active in germ cell tissue. Our study furthermore shows that Expressed Sequence Tag (EST) data are insufficient to describe transcriptional activity of HML-2 and other HERV loci in tissues of interest. Conclusion: Our, to date, largest-scale study reveals in greater detail expression patterns of individual HML-2 loci in human tissues of clinical interest. Moreover, large-scale, specialized studies are indicated to better comprehend transcriptional activity and regulation of HERVs. We thus emphasize the need for a specialized HERV Transcriptome Project

SNEV is an evolutionarily conserved splicing factor whose oligomerization is necessary for spliceosome assembly

We have isolated the human protein SNEV as downregulated in replicatively senescent cells. Sequence homology to the yeast splicing factor Prp19 suggested that SNEV might be the orthologue of Prp19 and therefore might also be involved in pre-mRNA splicing. We have used various approaches including gene complementation studies in yeast using a temperature sensitive mutant with a pleiotropic phenotype and SNEV immunodepletion from human HeLa nuclear extracts to determine its function. A human–yeast chimera was indeed capable of restoring the wild-type phenotype of the yeast mutant strain. In addition, immunodepletion of SNEV from human nuclear extracts resulted in a decrease of in vitro pre-mRNA splicing efficiency. Furthermore, as part of our analysis of protein–protein interactions within the CDC5L complex, we found that SNEV interacts with itself. The self-interaction domain was mapped to amino acids 56–74 in the protein's sequence and synthetic peptides derived from this region inhibit in vitro splicing by surprisingly interfering with spliceosome formation and stability. These results indicate that SNEV is the human orthologue of yeast PRP19, functions in splicing and that homo-oligomerization of SNEV in HeLa nuclear extract is essential for spliceosome assembly and that it might also be important for spliceosome stability

Управление финансовой устойчивостью и рентабельностью предприятия

Целью статьи является изучение значения управления финансовой устойчивостью и рентабельностью предприятия в современных условиях хозяйствования

Protocol and statistical analysis plan for the REstricted fluid therapy VERsus Standard trEatment in Acute Kidney Injury – REVERSE-AKI randomized controlled pilot trial

Abstract Background Fluid accumulation frequently coexists with acute kidney injury (AKI) and is associated with increased risk for AKI progression and mortality. Among septic shock patients, restricted use of resuscitation fluid has been reported to reduce the risk of worsening of AKI. Restrictive fluid therapy, however, has not been studied in the setting of established AKI. Here, we present the protocol and statistical analysis plan of the REstricted fluid therapy VERsus Standard trEatment in Acute Kidney Injury - the REVERSE-AKI trial that compares a restrictive fluid therapy regimen to standard therapy in critically ill patients with AKI. Methods REVERSE-AKI is an investigator-initiated, multinational, open-label, randomized, controlled, feasibility pilot trial conducted in 7 ICUs in 5 countries. We aim to randomize 100 critically ill patients with AKI to a restrictive fluid treatment regimen versus standard management. In the restrictive fluid therapy regimen, the daily fluid balance target is neutral or negative. The primary outcome is the cumulative fluid balance assessed after 72 hrs from randomization. Secondary outcomes include safety, feasibility, duration and severity of AKI, and outcome at 90 days (mortality and dialysis dependence). Conclusions This is the first multinational trial investigating the feasibility and safety of a restrictive fluid therapy regimen in critically ill patients with AKI.Peer reviewe