A hemophagocytic lymphohistiocytosis case with newly defined UNC13D (C.175G>C; p.Ala59Pro) mutation and a rare complication

Hemophagocytic lymphohistiocytosis (HLH) represents a severe hyperinflammatory condition with cardinal symptoms of prolonged fever, cytopenias, hepatosplenomegaly, and hemophagocytosis by activated, morphologically benign macrophages with impaired function of natural killer cells and cytotoxic T lymphocytes. A 2-month-old girl, who was admitted with fever, was diagnosed with HLH and her genetic examination revealed a newly defined mutation in the UNC13D (c.175G>C; p.Ala59Pro) gene. She was treated with dexamethasone, etoposide, and intrathecal methotrexate. During the second week of treatment, after three doses of etoposide, it was noticed that there was a necrotic plaque lesion on the soft palate. Pathologic examination of debrided material in PAS and Grocott staining revealed lots of septated hyphae, which was consistent with aspergillosis infection. Etoposide was stopped and amphotericin B treatment was given for six weeks. HLH 2004 protocol was completed to eight weeks with cyclosporine A orally. There was no patient with invasive aspergillosis infection as severe as causing palate and nasal septum perforation during HLH therapy. In immuncompromised patients, fungal infections may cause nasal septum perforation and treatment could be achieved by antifungal therapy and debridement of necrotic tissue. © 2015 Turkish Society of Hematology. All rights reserved

Observing and modelling the young solar analogue EK Draconis: starspot distribution, elemental abundances, and evolutionary status

Observations and modelling of stars with near-solar masses in their early phases of evolution is critical for a better understanding of how dynamos of solar-type stars evolve. We examine the chemical composition and the spot distribution of the pre-main-sequence solar analogue EK Dra. Using spectra from the HERMES Spectrograph (La Palma), we obtain the abundances of 23 elements with respect to the solar ones, which lead to a $[{\rm Fe/H}]=0.03$, with significant overabundance of Li and Ba. The s-process elements Sr, Y, and Ce are marginally overabundant, while Co, Ni, Cu, Zn are marginally deficient compared to solar abundances. The overabundance of Ba is most likely due to the assumption of depth-independent microturbulent velocity. Li abundance is consistent with the age and the other abundances may indicate distinct initial conditions of the pre-stellar nebula. We estimate a mass of 1.04 $M_\odot$ and an age of $27^{+11}_{-8}$\,Myr using various spectroscopic and photometric indicators. We study the surface distribution of dark spots, using 17 spectra collected during 15 nights using the CAFE Spectrograph (Calar Alto). We also conduct flux emergence and transport (FEAT) simulations for EK Dra's parameters and produce 15-day-averaged synoptic maps of the likely starspot distributions. Using Doppler imaging, we reconstruct the surface brightness distributions for the observed spectra and FEAT simulations, which show overall agreement for polar and mid-latitude spots, while in the simulations there is a lack of low-latitude spots compared to the observed image. We find indications that cross-equatorial extensions of mid-latitude spots can be artefacts of the less visible southern-hemisphere activity.Comment: 15 pages, 16 figures, accepted for publication in MNRAS; corrections in Abstract and Introductio

Gas and seismicity within the Istanbul seismic gap

Understanding micro-seismicity is a critical question for earthquake hazard assessment. Since the devastating earthquakes of Izmit and Duzce in 1999, the seismicity along the submerged section of North Anatolian Fault within the Sea of Marmara (comprising the “Istanbul seismic gap”) has been extensively studied in order to infer its mechanical behaviour (creeping vs locked). So far, the seismicity has been interpreted only in terms of being tectonic-driven, although the Main Marmara Fault (MMF) is known to strike across multiple hydrocarbon gas sources. Here, we show that a large number of the aftershocks that followed the M 5.1 earthquake of July, 25th 2011 in the western Sea of Marmara, occurred within a zone of gas overpressuring in the 1.5–5 km depth range, from where pressurized gas is expected to migrate along the MMF, up to the surface sediment layers. Hence, gas-related processes should also be considered for a complete interpretation of the micro-seismicity (~M < 3) within the Istanbul offshore domain

X-ray activity and rotation of the young stars in IC 348

The physical origin of the strong magnetic activity in T Tauri stars and its relation to stellar rotation is not yet well understood. We investigate the relation between the X-ray activity, rotation, and Rossby number for a sample of 82 young stars in the ~3 Myr old cluster IC 348. We use the data of four Chandra observations of IC 348 to derive the X-ray luminosities of the young stars. The young stars in IC 348 show no correlation between X-ray activity and rotation period. Considering the Rossby numbers, nearly all IC 348 stars are in the saturated regime of the activity-rotation relation defined by main-sequence stars. Searching for possible super-saturation effects, we find a marginal (but statistically in-significant) trend that the stars with the smallest Rossby numbers show slightly lower X-ray activity levels. We compare the dispersion of fractional X-ray luminosities of the stars in the saturated rotation regime in IC 348 to that seen in younger and older stellar populations. The scatter seen in the ~3 Myr old IC 348 is considerably smaller than for the ~1 Myr old ONC, but, at the same time, considerably larger than the dispersion seen in the ~30 Myr old cluster NGC 2547 and in main-sequence stars. The results of our X-ray analysis of IC 348 show that neither the rotation rates nor the presence/absence of circumstellar disks are of fundamental importance for determining the level of X-ray activity in TTS. Our results suggest that the scatter of X-ray activity levels shown by the fast-rotating members of young clusters decreases with the age of the stellar population. We interpret this as a signature of the changing interior structure of PMS stars and the consequent changes in the dynamo mechanisms that are responsible for the magnetic field generation.Comment: accepted by A&A on 15th of December 2011 published in Astronomy & Astrophysics Februar 201

High-K volcanism in the Afyon region, western Turkey: from Si-oversaturated to Si-undersaturated volcanism

Volcanic rocks of the Afyon province (eastern part of western Anatolia) make up a multistage potassic and ultrapotassic alkaline series dated from 14 to 12 Ma. The early-stage Si-oversaturated volcanic rocks around the Afyon city and further southward are trachyandesitic volcanic activity (14.23 ± 0.09 Ma). Late-stage Si-undersaturated volcanism in the southernmost part of the Afyon volcanic province took place in three episodes inferred from their stratigraphic relationships and ages. Melilite– leucitites (11.50 ± 0.03 Ma), spotted rachyandesites, tephryphonolites and lamproites (11.91 ± 0.13 Ma) formed in the first episode; trachyandesites in the second episode and finally phonotephrites, phonolite, basaltic trachyandesites and nosean-bearing trachyandesites during the last episode. The parameter Q [normative q-(ne + lc + kls + ol)] of western Anatolia volcanism clearly decreased southward with time becoming zero in the time interval 10–15 Ma. The magmatism experienced a sudden change in the extent of Si saturation after 14 Ma, during late-stage volcanic activity of Afyon volcanic province at around 12 Ma, though there was some coexistence of Si-oversaturated and Si-undersaturated magmas during the whole life of Afyon volcanic province

Supply Chain Intelligence

This chapter provides on overall picture of business intelligence (BI) and supply chain analytics (SCA) as a means to support supply chain management (SCM) and decision-making. Based on the literature review, we clarify the needs of BI and performance measurement in the SCM sphere, and discuss its potential to enhance decision-making in strategic, tactical and operational levels. We also make a closer look in to SCA in different areas and functions of SCM. Our findings indicate that the main challenge for harnessing the full potential of SCA is the lack of holistic and integrated BI approaches that originates from the fact that each functional area is using its own IT applications without necessary integration in to the company’s overall BI system. Following this examination, we construct a holistic framework that illustrates how an integrated, managerially planned BI system can be developed. Finally, we discuss the main competency requirements, as well as the challenges still prohibiting the great majority of firms from building smart and comprehensive BI systems for SCM.fi=vertaisarvioitu|en=peerReviewed

Plot by plot: plotting urbanism as an ordinary process of urbanization

With this paper, we analyse an ordinary urban process, which has received little attention so far and propose a new concept to take account of it: plotting urbanism. It is usually subsumed under terms like “urban informality” or “incremental urbanism” and not studied as a distinct process. In comparing Lagos, Istanbul and Shenzhen we captured four defining features of plotting urbanism: first, it unfolds in a piecemeal fashion with limited comprehensive planning. Second, it emerges from conflicts between multiple overlapping modes of territorial regulation, land tenure and property rights, which result in specific territorial compromises. Third, plotting is based on commodification of housing and land, which might accentuate socio-economic differentiations between property-owners, who often live in the same area, and their tenants. The term “plotting” highlights the key role of the plot in the process. It also alludes to strategic acts of collaboration for individual and collaborative benefit

Operational Research: methods and applications

This is the final version. Available on open access from Taylor & Francis via the DOI in this recordThroughout its history, Operational Research has evolved to include methods, models and algorithms that have been applied to a wide range of contexts. This encyclopedic article consists of two main sections: methods and applications. The first summarises the up-to-date knowledge and provides an overview of the state-of-the-art methods and key developments in the various subdomains of the field. The second offers a wide-ranging list of areas where Operational Research has been applied. The article is meant to be read in a nonlinear fashion and used as a point of reference by a diverse pool of readers: academics, researchers, students, and practitioners. The entries within the methods and applications sections are presented in alphabetical order. The authors dedicate this paper to the 2023 Turkey/Syria earthquake victims. We sincerely hope that advances in OR will play a role towards minimising the pain and suffering caused by this and future catastrophes