The frequency of Duchenne muscular dystrophy/Becker muscular dystrophy and Pompe disease in children with isolated transaminase elevation: results from the observational VICTORIA study

IntroductionElevated transaminases and/or creatine phosphokinase can indicate underlying muscle disease. Therefore, this study aims to determine the frequency of Duchenne muscular dystrophy/Becker muscular dystrophy (DMD/BMD) in male children and Pompe disease (PD) in male and female children with isolated hypertransaminasemia.MethodsThis multi-center, prospective study enrolled patients aged 3–216 months with serum alanine transaminase (ALT) and/or aspartate transaminase (AST) levels >2× the upper limit of normal (ULN) for ≥3 months. Patients with a known history of liver or muscle disease or physical examination findings suggestive of liver disease were excluded. Patients were screened for creatinine phosphokinase (CPK) levels, and molecular genetic tests for DMD/BMD in male patients and enzyme analysis for PD in male and female patients with elevated CPK levels were performed. Genetic analyses confirmed PD. Demographic, clinical, and laboratory characteristics of the patients were analyzed.ResultsOverall, 589 patients [66.8% male, mean age of 63.4 months (standard deviation: 60.5)] were included. In total, 251 patients (188 male and 63 female) had CPK levels above the ULN. Of the patients assessed, 47% (85/182) of male patients were diagnosed with DMD/BMD and 1% (3/228) of male and female patients were diagnosed with PD. The median ALT, AST, and CPK levels were statistically significantly higher, and the questioned neurological symptoms and previously unnoticed examination findings were more common in DMD/BMD patients than those without DMD/BMD or PD (p < 0.001).DiscussionQuestioning neurological symptoms, conducting a complete physical examination, and testing for CPK levels in patients with isolated hypertransaminasemia will prevent costly and time-consuming investigations for liver diseases and will lead to the diagnosis of occult neuromuscular diseases. Trial RegistrationClinicaltrials.gov NCT04120168

La formation d'une bourgeoisie commerçante turque en Belgique

The making of a Turkish trading bourgeoisie in Belgium Altay MANÇO and Oya AKHAN In this work, the hypothesis of the emergence in Belgium of a business class of Turkish origin is investigated on the basis of the most recent statistic and bibliographical sources. Some characteristics of those professionals are described, as well as differences between the immigrated Turkish investors in Belgium and in other EEC countries such as Germany and France. In addition, a qualitative survey, based on 20 interviews of Turkish grocers from the Province of Liege, analyses the development conditions of this kind of business.La formation d'une bourgeoisie commerçante turque en Belgique Altay MANÇO et Oya AKHAN Dans cet article, l'hypothèse de l'émergence en Belgique d'une classe d'affaire d'origine turque est examinée d'abord à travers les sources statistiques et bibliographiques les plus récentes. Certaines caractéristiques des indépendants sont présentées. Sont également envisagées les raisons des différences observées dans le comportement des investisseurs turcs de Belgique avec leurs homologues dans divers pays de la CEE, dont l'Allemagne et la France. Enfin, 20 interviews réalisés avec des épiciers turcs de la Province de Liège permettent, dans une approche qualitative, d'identifier avec précision les conditions de développement de ce genre d'entreprises.La formación de una burgesía comerciante turca en Belgica Altay MANÇO y Oya AKHAN En este artículo, la hipótesis de la aparición en Bélgica de una clase de negociantes de origen turco se examina a través de las fuentes estadísticas y bibliográficas más recientes. Algunas características de los independientes están presentadas. Se encaran igualmente las razones de las diferencias observadas en el comportamiento de los inversionistas turcos de Bélgica con sus homólogos en los diversos países de la CEE, como Alemania y Francia. Finalmente, 20 entrevistas realizadas con tenderos turcos de la provincia de Lieja permiten, en un enfoque cualitativo, identifïcar con precisión las condiciones de desarrollo de este tipo de empresas.Belçika'da bir Türk işadami sinifinin gelişimi Altay MANÇO et Oya AKHAN Bu yazida, Belçika'da bir Türk serbest meslek sahibi sinifinin gelişme varsayimi en yeni sayisal verilere dayanilarak değerlendirilmiştir. Serbest meslek sahiplerinin çeşitli yanlari tanitilarak, basta Fransa ve Almanya olmak üzere diger Avrupa ülkelerine göre Belçika'daki Türk yatirimcilarinin gösterdikleri eğilim farkliliklari incelenmiştir. Son bölüm Liège vilayetinde hizmet veren 20 Türk bakkali ile yapilan görüşmelerin nicel çözümlemesine ayrilmiştir. Bu alan çalişmasi çerçevesinde Belçika'daki Türk ticaretinin gelişme koşullarinin belirlenmesine çalişilmistir.Manço Altay, Akhan Oya. La formation d'une bourgeoisie commerçante turque en Belgique. In: Revue européenne des migrations internationales, vol. 10, n°2,1994. pp. 149-162

A comparative study of two HPLC methods for dissolved monosaccharide analysis in seawater using 2-amino benzamide and 2-amino pyrazine as pre-column derivatization reagents

© 2021 Elsevier B.V.Two reversed-phase HPLC methods for molecular analysis of dissolved free monosaccharides in seawater were investigated comparatively by optimizing chromatographic separations and pre-column derivatization reactions. Monosaccharides derivatized with 2-amino benzamide or 2-amino pyrazine, and reduced with 2-picoline borane or 2-dimethylamino borane. According to results of optimization, separation performance, and detection limits, 2-amino benzamide method gave better results than 2-amino pyrazine method. Among 12 monosaccharides tested, it was possible to quantify glucose + galactose, galacturonic acid, glucuronic acid, xylose + arabinose, ribose, mannose, and N-acetyl-D-glucosamine with detection limits between 1.2 and 11 nM with intra-day repeatability of 2–9% and inter-day repeatability of 3–9%. The optimized method has the same level of detection limit with a widely used anion exchange chromatography method. Besides the preliminary results reported in this study, it may be possible to achieve higher sensitivity and to detect more monosaccharides by the use of shorter and narrow-bore columns at different polarities in further studies

Eutrophication in ızmır bay, eastern aegean

The polluted waters of İzmir Bay were studied to measure the variations of biochemical and physical characteristics monthly or three-monthly. The spatial and temporal changes of nutrients (nitrate, nitrite, and ortho phosphate) and chlorophyll-a, temperature and salinity in the bay were monitored during the period of February 1990 - December 1992. The evaluated results were compared with the results obtained from the relatively unpolluted waters of the Aegean Sea. Eutrophication of the inner bay has already started, spreading progressively to the rest of the bay. The degree of eutrophication in the bay was assessed. The concentration of nutrients ranged between 0.21-2.51 µg-at 1-1 for PO4-P; 0.44-4.20 µg-at l-1 for N03-N; 0.11-3.50 µg-at I-1 for NO2-N. Chlorophyll-a concentration is between 0.7-39.1 µg l-1 and estimated primary productivity in the bay is 7–10 times higher than the offshore production in the region. The main reason for high primary production is the high terrestrial input. As expected total suspended solids concentrations is high, so that the water is turbid having extremely low penetration, the average depth of secchi disk is between 2–10 m. © 1995, Taylor & Francis Group, LLC. All rights reserved

Spatial Distribution Patterns, Sources of Heavy Metals, and Relation to Ecological Risk of Surface Sediments of the Cyprus Northern Shelf (Eastern Mediterranean)

In this study, the spatial distribution of metals and the sources of metal pollution were investigated along the Northern Shelf of Cyprus (Mediterranean Sea). The concentrations of heavy metals and organic matter were measured in sediments collected from the Gemi Konagi, Girne, and Gazi Magusa areas. Measured metal values were compared with Mediterranean background concentrations. Cu and Zn concentrations at the Gemi Konagi area and all of the Cr values were higher than Mediterranean backgrounds. The metal levels were evaluated by the enrichment factor (EF), contamination factor (C-f) and degree of contamination (C-d). EF results indicated that heavy metal sources were probably originated from natural processes and mining activities. The C-f values of Hg indicated low contamination. The C-d (degree of contamination) values for all heavy metals also showed a low degree of contamination at the study area. Metal levels were also compared with the numerical Sediment Quality Guidelines (SQG) for an environmental risk assessment. Results showed that sediments were classified as heavily polluted by Cu and moderately polluted by Zn at Gemi Konagi and heavily polluted by Cr and Ni contamination at all sampling areas per the SQG