Spatial and temporal distribution of Kutum (Rutilus frisii kutum, Kamensky 1901) in Iranian waters of the Caspian Sea

Caspian Kutum (Rutilus frisii kutum, Kamensky 1901) is among the main commercial species in the Caspian Sea. The present study aimed to determine the spatial and temporal distribution patterns of Kutum in depth ranges of 10 to 100 m by bottom trawl net in Iranian waters of the Caspian Sea during 2008 to 2010. The results showed that the maximum catch and CPUE were 2903 kg and 57.08 kg/0.5 h in winter 2009. The minimum catch and CPUE were 79 kg and 1.41 kg/0.5 h in spring 2010. During the warm seasons (spring and summer) most Kutums occurred in depths less than 20 m. While, during cold seasons they occurred at all depths. The average (±SE) CPUE was 94.0 ± 78.53 kg/0.5 h in depths 20-50 m in autumn and 128.3 ± 77.64 kg/0.5 h in depths >50 m in winter

A review of research on the growth of multinational enterprises: a Penrosean lens

We provide a comprehensive review of the growth of multinational enterprises (MNEs) based on a quarter-century (25 years) of scholarly international business publications. We synthesize research insights on the determinants of the growth of MNEs through the lens of Penrose’s theory of firm growth, which is the most influential theoretical perspective on firm growth. The review takes stock of the research findings on the facilitators, constraints, and the trajectory of growth of MNEs, and elaborates on the commonalities and differences between research on the growth of MNEs that draws on Penrose’s theory and research that does not. This comparison highlights the opportunity to build an internally coherent theory of the growth of MNEs, that is, a theory that connects ‘what an MNE is’ to ‘what determines an MNE’s growth.’ It also indicates that past research placed a strong emphasis on exogenous factors (e.g., cross-country distances) as key constraints on growth even though the Penrosean lens suggests a close consideration of endogenous factors that drive international growth, as well as endogenizing the ostensibly exogenous growth determinants. The review highlights the importance of firm-specific managerial knowledge and learning, which shapes the direction of the growth of MNEs and ensures its administrative coherence

Stock assessment of juvenile sturgeons in the Iranian water of the Caspian Sea by bottom trawl survey

The sturgeon stock assessment was performed to aim at estimation of absolute and relative abundance and determination of species composition at lower 10 m depths using the Si-Sara2 RV vessel in the Iranian coasts of the Caspian Sea in Guilan, Mazandaran and Golestan provinces during 6-30 September 2011-2012. In this study, 40 stations were selected on the basis of stratified random sampling design and then the stock estimation was performed using the swept area method. The study was carried out using bottom trawling with 9 m head rope. The time and speed of trawling in each station were 30 minutes and 2.5 knots respectively. The Catch per Unit of Effort (CPUE) in 2011–2012 were 7.03 and 6.96 individuals per trawling, respectively. The catch per unit of area in these years were found to be 1662 and 1644 fish in nm2, respectively.Total abundance of sturgeon juveniles was 13,327,164 individuals in 2011. So, the species composition included A. persicus (87.8%) and A. stellatus (12.2%). Total abundance of sturgeon juveniles was found to be 14,364,882 individuals in 2012 and the species composition comprised A. persicus (61.4%) and A. stellatus (38.6%). In 2011 the biomass of sturgeons in Iranian coastal water of the Caspian Sea was 295 tons and the composition of biomass included A. persicus (81.5%) and A. stellatus (18.5%), respectively. In the cruises conducted in 2011, this amount was estimated to be 217 tons comprising A. persicus (54.2%) and A. stellatus (45.8%), respectively. The results of this study in 2011–2012 showed remarkable abundance of juvenile sturgeons in Iranian coastal waters of the Caspian Sea in late summer and early autumn. So, by conserving these valuable stocks, the number of spawners will be increased in the future

Twenty-seven mutations with three novel pathologenic variants causing biotinidase deficiency: a report of 203 patients from the southeastern part of Turkey

BACKGROUND: Biotinidase deficiency (BD) is an autosomal recessive inborn error of metabolism characterized by neurologic and cutaneous symptoms and can be detected by newborn screening. Newborn screening for BD was implemented in Turkey at the end of 2008. METHODS: In total, 203 patients who were identified among the infants detected by the newborn screening were later confirmed to have BD through measurement of serum biotinidase activity. We also performed BTD mutation analysis to characterize the genetic profile. RESULTS: Twenty-seven mutations were identified. The most commonly found variants were c.1330G>C (p.D444H), c.1595C>T (p.T532M), c.470G>A (p.R157H), and c.198_104delGCGGCTGinsTCC (p.C33Ffs ) with allele frequencies of 0.387, 0.175, 0.165 and 0.049, respectively. Three novel pathogenic and likely pathogenic variants were identified: p.W140* (c.419G>A), p.S319F (c.956C>T) and p.L69Hfs*24 (c.192_193insCATC). We also identified three mutations reported in just one patient in the past (p.V442Sfs*59 [c.1324delG], p.H447R [c.1340A>G] and p.198delV [c.592_594delGTC]). Although all of the patients were asymptomatic under the treatment of biotin, only one patient, who had the novel c.419G>A homozygous mutation became symptomatic during an episode of acute gastroenteritis with a presentation of ketosis and metabolic acidosis. Among the screened patients, 156 had partial and 47 had profound BD. CONCLUSIONS: We determined the mutation spectra of BD from the southeastern part of Turkey. The results of this study add three more mutations to the total number of mutations described as causing BD

VERTICAL FARMING CONCEPTIN THAILAND: IMPORTANT DECISION VARIABLES

Abstract: This study conducts an exploratory study related to a systematic model of decision variables for the vertical farming concept found in Thailand. Utilizing the model of decision variables found in three paradigms, the different available sources of data were synthesized to understand the concept as a phenomenon and to find a valid model for a new construction and product development strategy using the vertical farming concept for Thailand in the future

Comprehensive approach to reintegration of disability benefit recipients with multiple problems (CARm) into the labour market:results of a randomized controlled trial

PURPOSE: Although most clients on work disability benefits face multiple problems, most traditional interventions for (re)integration focus on a single problem. The aim of this study was to evaluate the "Comprehensive Approach to Reintegrate clients with multiple problems" (CARm), which provides a strategy for labour experts to build a relationship with each client in order to support clients in their needs and mobilize their social networks.METHODS: This study is a stratified, two-armed, non-blinded randomized controlled trial (RCT), with a 12-month follow-up period. Outcome measures were: having paid work, level of functioning, general health, quality of life, and social support.RESULTS: We included a total of 207 clients in our study; 97 in the intervention group and 110 in the care as usual (CAU) group. The clients' mean age was 35.4 years (SD 12.8), 53.1% were female, and 179 (86.5%) reported multiple problems. We found the CARm intervention to have no significant effects superior to those of the CAU group on all outcomes.CONCLUSION: As we found no superior effect of the CARm intervention compared to CAU, we cannot recommend widespread adoption of CARm. A process evaluation will give more insight into possible implementation failure of the intervention.IMPLICATIONS FOR REHABILITATIONMost traditional interventions for (re)integration into the labour market are problem-centred, i.e., focusing on a single problem, and have limited effectiveness in persons with multiple problems.A strength-based intervention may be suitable for vocational rehabilitation and disability settings, since it contains many elements (e.g., being strength-based, focused on clients' wishes and goals, and involving activation of the social environment) also likely to improve chances of re-employment of persons with multiple problems.In this study a strength-based intervention did not show a superior effect on paid employment and functioning within one year follow-up compared to care as usual in people with multiple problems on a work disability benefit.</p

Use of decongestants may disrupt cell signaling pathways that control Tbx gene expression, leading to hypoplastic left heart syndrome

Hypoplastic left heart syndrome (HLHS) collectively refers to a range of congenital heart defects, all involving some degree of left ventricular hypoplasia, or underdevelopment of the left ventricle. Additionally, HLHS often involves coarctation of the aorta, and can also include hypoplasia of the ascending aorta, as well as mitral and/or aortic valve stenosis or atresia. HLHS is extremely rare, as it has been reported to occur in only 1 in 5000 live births each year. The cause of HLHS is currently unknown, however much research is being done to discover how and why these defects occur. HLHS is known to be familially inherited in some instances and is also associated with many well-characterized genetic disorders, including Holt-Oram syndrome, Turner’s syndrome, Noonan syndrome, Smith-Lemli-Opitz syndrome, as well as trisomies 13, 18, and 21. Additionally, an autosomal recessive pattern of inheritance has been found amongst some siblings, however, no specific genes have been implicated. Incidence of HLHS also varies significantly in certain geographical regions and some studies have found a seasonal correlation in HLHS, indicating a possible environmental cause

Penrose's The Theory of the Growth of the Firm: an exemplar of engaged scholarship

Edith Penrose's (1959) classic book, The Theory of the Growth of the Firm, made a substantial impact on strategic management research, especially in the context of the resource-based view of the firm, and the ripple effects of her impact continue to unfold today in various disciplines. The book serves as a remarkably rich source of inspiration for scholarly research and a generative source of ideas, which are waiting to be further developed. In this paper, we examine Penrose's (1959) classic and provide: (1) the process by which this book came about; (2) a summary of its key ideas; (3) the subsequent impact of the book, in which we focus on mathematical models; and (4) a discussion of some of the research lessons learned from this exemplar of engaged scholarship. We invite management science and operations scholars to discover the rich scientific world of Edith Penrose and experience the product and process of her research creativit

The outcome of 41 Late-Diagnosed Turkish GA-1 Patients: A Candidate for the Turkish NBS

Background: Glutaric aciduria type 1(GA-1) is an inherited cerebral organic aciduria. Untreated patients with GA-1 have a risk of acute encephalopathic crises during the first 6 years of life. In so far as GA-1 desperately does not exist in Turkish newborn screening (NBS) program, most patients in our study were late-diagnosed. / Method: This study included 41 patients diagnosed with acylcarnitine profile, urinary organic acids, mutation analyses in the symptomatic period. We presented with clinical, neuroradiological, and molecular data of our 41 patients. / Results: The mean age at diagnosis was 14.8 13.9 (15 days to 72 months) and, high blood glutaconic acid, glutarylcarnitine and urinary glutaric acid (GA) levels in 41 patients were revealed. Seventeen different mutations in the glutaryl-CoA dehydrogenase gene were identified, five of which were novel. The patients, most of whom were late-diagnosed, had a poor neurological outcome. Treatment strategies made a little improvement in dystonia and the frequency of encephalopathic attacks. / Conclusion: All GA-1 patients in our study were severely affected since they were latediagnosed, while others show that GA-1 is a treatable metabolic disorder if it is diagnosed with NBS. This study provides an essential perspective of the severe impact on GA-1 patients unless it is diagnosed with NBS. We immediately advocate GA-1 to be included in the Turkish NBS

Mucopolysaccharidosis Type-II with Pathognomonic Skin Appearance: A Case with Pebbling Sign

Mucopolysaccharidosis type-II (MPS-II) is an X-linked lysosomal storage disorder. Here, we report an 8-year-old boy with pebbling sign in the scapular region, coarse facies, claw hand, diastolic murmur, and hepatomegaly. With decreased iduronate-2-sulfatase activity and hemizygous mutation in the IDS gene, the diagnosis was MPS-II. Pebbling sign is a rare but pathognomonic sign of MPS-II