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Nanocomposite materials containing carbon nanotubes (CNTs) coated with platinum nanoparticles were fabricated as part of the investigations undertaken. The purpose of the works was the multifaceted characterization of the materials produced including examination of the structure, morphology, assessment of platinum nanoparticles distribution on the surface of CNTs and determination of the average size of platinum crystals. High quality multiwalled CNTs with the length of 100–200 μm and diameter of 10–20 nm obtained in chemical vapor deposition were used in the investigations. Raw nanotubes did not contain metallic impurities or amorphous carbon deposits. An indirect method of bonding the earlier produced platinum nanoparticles to the surface of functionalized CNTs was employed to deposit platinum nanoparticles onto the surface of CNTs. Nanocomposite materials containing CNTs coated with platinum nanoparticles with a 5% weight fraction were obtained as part of the experiments made. The following research techniques were employed during the investigations aimed at recognizing the structure and morphology of the nanocomposites obtained: transmission electron microscopy, scanning transmission electron microscopy, scanning electron microscopy, Raman spectroscopy, and X-ray structure analysis (XRD).

Familial hypercholesterolemia (FH) is a genetic disease that causes accelerated atherosclerosis and a high risk of occurrence of cardiovascular events. Atherosclerosis in the course of FH develops insidiously and reaches an advanced stage before the onset of clinical symptoms. Homozygous form of FH occurs in the Caucasian population with a frequency of one per million, while the heterozygous form of FH in European countries applies on average in 1 person per 500. FH diagnosis is an indication to take the whole family under medical care, education, and to introduce dietary and pharmacological treatment. The aim of treatment in children with FH is to achieve more than 50% reduction in LDL level or to achieve LDL-cholesterol concentrations below 130 mg/dl and below 100 mg/dl in diabetic children. The effectiveness of low fat diet in the treatment of FH is limited. The medicaments of first choice in the treatment of FH are statins. After initiation of therapy in children, cholesterol levels and the side effects of therapy and its impact on children's development, nutritional status, degree of sexual maturity should be routinely evaluated. Familial hypercholesterolemia is a relatively common metabolic disorder, but still quite rarely recognized and not properly treated. Early diagnosis and appropriate treatment of FH in children and adolescents can significantly reduce the risk of cardiovascular disease and sudden death in adults. The purpose of these recommendations is to describe the current epidemiological situation in Poland, to establish the guidelines for identifying FH in children and adolescents and to enable the introduction of effective treatment. This document is a supplement to the position of Lipid Expert Forum on FH in adults.

1Katedra i Klinika Pediatrii, Diabetologii i Endokrynologii, Gdanski Uniwersytet Medyczny, Gdansk 2Klinika Pediatrii, Endokrynologii, Diabetologii, Chorob Metabolicznych i Kardiologii Wieku Rozwojowego, Pomorski Uniwersytet Medyczny, Szczecin 3Klinika Pediatrii, Endokrynologii i Diabetologii Dzieciecej, Śląski Uniwersytet Medyczny, Katowice 4Klinika Patologii Noworodka, Instytut Pediatrii, Centrum Zdrowia Dziecka, Warszawa 5Instytut Żywności i Żywienia, Warszawa 6I Katedra i Klinika Kardiologii, Warszawski Uniwersytet Medyczny, Warszawa 7Wydzial Medyczny, Uniwersytet Rzeszowski, Rzeszow 8Klinika Pediatrii, Hematologii, Onkologii i Diabetologii, Łodzki Uniwersytet Medyczny, Łodź 9I Katedra i Klinika Kardiologii, Gdanski Uniwersytet Medyczny, Gdansk 10Katedra i Klinika Transplantacji Szpiku, Onkologii i Hematologii Dzieciecej, Wroclawski Uniwersytet Medyczny, Wroclaw 11Klinika Gastroenterologii, Hepatologii i Żywienia Dzieci, Centrum Zdrowia Dziecka, Warszawa 12Zaklad Genetyki, Instytut Psychiatrii i Neurologii, Warszawa 13Zaklad Prewencji i Dydaktyki, Katedra Nadciśnienia Tetniczego i Diabetologii, Gdanski Uniwersytet Medyczny, Gdansk 14Katedra i Zaklad Biologii i Genetyki, Gdanski Uniwersytet Medyczny, Gdansk 15Katedra Nefrologii i Nadciśnienia Tetniczego, Łodzki Uniwersytet Medyczny, Łodź

Tetraploidy is a condition in which there are four complete sets of chromosomes in a single cell. In humans, this would be 92 pairs of chromosomes per cell. A great majority of pregnancies with a tetraploid fetus end in miscarriage, or if the pregnancy goes to full term, the infant dies shortly after birth. Longer surveillance is rarely described. The only method for confirming tetraploidy is karyotyping, in many cases using classical G-banding methods. In this paper we would like to present another living individual with tetraploidy and to revise the syndrome in the light of its diagnostics in the era of molecular karyotyping, with array Comparative Genomic Hybridization (aCGH, arrayCGH).

There are many reports discussing the conformational flexibility of calix[4]arenes in solution. On the other hand, similar studies concerning the solid state have received considerably less attention, even for the more extended and simultaneously more plastic calixarenes. Furthermore, no studies have been performed on single crystals, most likely because they are generally still perceived as unalterable and rigid, despite an increasing number of reports proving that the opposite is true. We report herein a phenomenon taking place in a single crystal of homodithiacalix[4]arene, the first member of a novel family of “homodiheteracalix[4]arenes”, with diatomic hetera bridges introduced in the macrocyclic ring, in this case disulfide. In contrast with the extensive literature on single-crystal-to-single-crystal transformations (SCSCT) taking place in metal–organic networks, the number of reports regarding SCSCT for organic solids is very limited to date and only encompasses a couple of examples. This is attributed to the fact that cooperative molecular motion is much more easily achieved for frameworks than in molecular crystals. To the best of our knowledge, reversible, solvent-induced conformational switching in a single crystal of an organic solid has not been reported to date. Moreover, the process is correlated with the formation/disappearance of channels in the structure. Jones et al. observed a change in conformation of an organic cage induced by solvent; however the single crystal did not survive this transformation, and a detailed study was performed on powdered bulk material instead. Keeping in mind that the structural changes observed in our case do not lead to loss of the crystal integrity, the transformation seems to be quite remarkable. The compound I (Scheme 1) was obtained by applying the concept of dynamic covalent chemistry (DCC), an attractive approach whereby a reversible reaction is performed under

The purpose of the article is to discuss the process of oxidation of carbon nanotubes subsequently subjected to the process of decoration with rhenium nanoparticles. The influence of functionalization in an oxidizing medium is presented and the results of investigations using Raman spectroscopy and infrared spectroscopy are discussed. Multiwalled carbon nanotubes rhenium-type nanocomposites with the weight percentage of 10%, 20% and 30% of rhenium are also presented in the article. The structural components of such nanocomposites are carbon nanotubes decorated with rhenium nanoparticles. Microscopic examinations under transmission electron microscope and scanning transmission electron microscope using the bright and dark field confirm that nanocomposites containing about 20% of rhenium have the most homogenous structure.

Przedmiotem niniejszego artykułu jest problem ochrony danych osobowych świadka w kontekście obowiązku składania przez niego zeznań na rozprawie głównej w polskim procesie karnym. Analiza obejmuje zarówno problematykę związaną z rolą świadka w procesie karnym, jak i zakresu dostępu do danych osobowych w toku rozprawy głównej, a także relacji, jaka zachodzi między zasadą jawności postępowania a zasadą ochrony danych osobowych. Przedstawiono także instytucje, których obowiązywanie w procesie karnym przyczynia się do ochrony danych osobowych świadka, zarówno w bezpośredni jak i pośredni sposób. Wskazano również, na zagrożenia jakie niesie za sobą obecnie dostrzegalna, w polskiej procedurze karnej, tendencja do zwiększania zakresu jawności postępowania oraz dostępu mediów do relacjonowania jego przebiegu, w kontekście ochrony danych osobowych. The subject matter of this article is the protection of personal data of a witness in the context of obligation to testify on the main trial in the Polish criminal proceedings. The analysis pertains to the role of the witness in criminal proceedings, the scope of access to personal data in the course of the main trial, and relation between the principle of open proceedings and the principle of personal data protection. Additionally, the article presents the institutions which have both direct and indirect impact on the protection of personal data of a witness in the criminal proceedings. The article also indicates threats to personal data protection caused by the tendency to increase the openness of court proceedings and media access to report them, which can be observed in the Polish criminal procedure.

Recent updates of the European, American and Polish nutritional recommendations require updates of the nutritional norm for children. We present the Polish Expert Group statement (2012) on the intake of selected nutrients (proteins, lipids, carbohydrates, vitamin D and E) essential for nutrition in children aged 1–3 years. For this purpose the Expert Group reviewed available scientific data consisting of the recent guidelines, nutritional norms and recommendations, systematic reviews and expert opinions as well as original publications, in relation to the specific requirements for the Polish population.

Summary Background: Chondrodysplasia punctata (CDP) is a rare, heterogeneous congenital skeletal dysplasia, characterized by punctate or dot-like calcium deposits in cartilage observed on neonatal radiograms. A number of inborn metabolic diseases are associated with CDP, including peroxisomal and cholesterol biosynthesis dysfunction and other inborn errors of metabolism such as: mucolipidosis type II, mucopolysacharidosis type III, GM1 gangliosidosis. CDP is also related to disruption of vitamin K-dependent metabolism, causing secondary effects on the embryo, as well as fetal alcohol syndrome (FAS), chromosomal abnormalities that include trisomies 18 and 21, Turner syndrome. Case Report: This article presents clinical data and diagnostic imaging findings of two newborn babies with chondrodysplasia punctata. Children presented with skeletal and cartilage anomalies, dysmorphic facial feature, muscles tone abnormalities, skin changes and breathing difficulties. One of the patients demonstrated critical stenosis of spinal canal with anterior subluxation of C1 vertebra relative to C2. The aim of this article is to present cases and briefly describe current knowledge on etiopathogenesis as well as radiological and clinical symptoms of diseases coexisting with CDP. Conclusions: Radiological diagnostic imaging allows for visualization of punctate focal mineralization in bone epiphyses during neonatal age and infancy. Determining the etiology of chondrodysplasia punctata requires performing various basic as well as additional examinations, including genetic studies.

Barth syndrome (BTHS) is an X-linked mitochondrial defect characterised by dilated cardiomyopathy, neutropaenia and 3-methylglutaconic aciduria (3-MGCA). We report on two affected brothers with c.646G > A (p.G216R) TAZ gene mutations. The pathogenicity of the mutation, as indicated by the structure-based functional analyses, was further confirmed by abnormal monolysocardiolipin/cardiolipin ratio in dry blood spots of the patients as well as the occurrence of this mutation in another reported BTHS proband. In both brothers, 2D-echocardiography revealed some features of left ventricular noncompaction (LVNC) despite marked differences in the course of the disease; the eldest child presented with isolated cardiomyopathy from late infancy, whereas the youngest showed severe lactic acidosis without 3-MGCA during the neonatal period. An examination of the patients’ fibroblast cultures revealed that extremely low mitochondrial membrane potentials (mtΔΨ about 50 % of the control value) dominated other unspecific mitochondrial changes detected (respiratory chain dysfunction, abnormal ROS production and depressed antioxidant defense). 1) Our studies confirm generalised mitochondrial dysfunction in the skeletal muscle and the fibroblasts of BTHS patients, especially a severe impairment in the mtΔΨ and the inhibition of complex V activity. It can be hypothesised that impaired mtΔΨ and mitochondrial ATP synthase activity may contribute to episodes of cardiac arrhythmia that occurred unexpectedly in BTHS patients. 2) Severe lactic acidosis without 3-methylglutaconic aciduria in male neonates as well as an asymptomatic mild left ventricular noncompaction may characterise the ranges of natural history of Barth syndrome. Electronic supplementary material The online version of this article (doi:10.1007/s10545-013-9584-4) contains supplementary material, which is available to authorised users.

Site 1 in Zofipole east of Krakow is known for long-standing research conducted in 1930s and 1940s. In 1986 test excavations were conducted in the eastern, up to then unrecognized part of the area. This research led to the discovery of a part of a small settlement of the Baden culture yielding interesting and diversified (while not numerous) sources for studies into this culture in Lesser Poland. This research is worth discussing due to new radiocarbon data, animal bones analyses, the discoveries of a dwelling feature and presumed symbolic burial and fragments of pottery for salt production

Cytomegalovirus (CMV) infections are common and their incidence increases with age. In immunocompetent people they are usually asymptomatic or manifest as a mild, self-limiting mononucleosis syndrome. CMV infection in patients with immune deficiency as well as congenital infections may cause a considerable problem. A group of experts designated by the Polish Society of Epidemiology and Infectious Diseases has prepared recommendations on the diagnosis and treatment of CMV infections, with particular emphasis on the management of patients with immunodeficiencies and congenital infections.

Two phosphine ligands of [Pd(PPh3)4] were substituted by π(CS) coordination of 4-bromodithiobenzoic acid methyl ester resulting in complex 1. The same ester, after alkylation, afforded the dicationic complex bis(μ-methanethiolato)tetrakis(triphenylphosphine)dipalladium(2+) bis(tetrafluoroborate) (2) from the same palladium source. A related thiolato-bridged complex, bis(μ-methanethiolato)bis(1-methylpyridin-2(1H)-ylidene)bis(triphenylphosphine)dipalladium(2+) bis(tetrafluoroborate) (4) and the trinuclear cluster tris(μ-methanethiolato)tris(triphenylphosphine)tripalladium(+)(3PdPd) (5) resulted from treatment of a known cationic pyridinylidene complex with MeSLi. The double oxidative substitution reaction of [Pd(PPh3)4] with 1,5-dichloro-9,10-anthraquinone afforded trans-dichloro[μ-(9,10-dihydro-9,10-dioxoanthracene-1,5-diyl)]tetrakis(triphenylphosphine)dipalladium (6). Some of these complexes could be fully characterized by 1H-, 13C-, and 31P-NMR spectroscopy, mass spectrometry, and elemental analysis. The crystal and molecular structures of all of them, and of trans-bis(1,3-dihydro-1,3-dimethyl-2H-imidazol-2-ylidene)diiodopalladium (3), were determined by single-crystal X-ray diffraction.

Recent updates of the European, American and Polish nutritional recommendations require updates of the nutritional norm for children. We present the Polish Expert Group statement (2012) on the intake of selected nutrients (proteins, lipids, carbohydrates, vitamin D and E) essential for nutrition in children aged 1–3 years. For this purpose the Expert Group reviewed available scientific data consisting of the recent guidelines, nutritional norms and recommendations, systematic reviews and expert opinions as well as original publications, in relation to the specific requirements for the Polish population.

5-Lithiated 4,4-dimethyl-2-(2-thienyl)oxazoline reacted by halide and tetrahydrothiophene (THT) substitution with [AuCl(THT)] to form a cyclic trinuclear compound (1) in satisfactory yield. Spectroscopic studies and single-crystal X-ray analyses clarified the bonding within the ring system. The structure determination of two THF solvates revealed differences in the supramolecular assembly of the constituting molecules.

The purpose of the article is to present the unique properties of casting magnesium alloys subjected to surface treatment with the high power diode laser, to indicate the development outlooks of the technology and to present its application opportunities in the automotive industry. The laser alloying and cladding of hard titanium and tungsten carbides particles into the substrate surface improves the mechanical and functional properties of the investigated materials to a satisfactory extent. In view of the demand from the automotive sector for components and parts made of lightweight alloys, the development prospects of their effective manufacturing technology meeting the expected product properties, including surface laser treatment, were evaluated to be very high.

Despite the recent reports on transition-metal catalyzed cycloisomerization strategies toward helicenes, the amount of palladium catalyzed routes remains rather scarce. Within this letter the successful preparation and characterization of novel dioxa-aza[7]helicenes using palladium mediated coupling reactions is presented.

A series of AgI coordination compounds with a new ligand, 1,2-bis[(imidazol-2-yl)thiomethyl]benzene, and counterions such as PF6–, SbF6–, CF3SO3–, BF4– and NO3– was characterised by powder and single-crystal XRD. Powder XRD revealed the presence of a mixture of crystallographic phases that were further identified by single-crystal XRD. Despite conformational flexibility, 1,2-bis[(imidazol-2-yl)thiomethyl]benzene has a tendency to form N,N-chelated AgI mononuclear cationic complexes with a linear N–AgI–N unit. However, T-shaped geometry (N2O) around the silver ion was also observed, which involved solvent molecules and/or counterions. The formation of a cationic 1D chain with the N,N′-bridging ligand was revealed with the BF4– counterion. Coordination compounds that possess different nuclearity were formed with the NO3– counterion. In the dinuclear and trinuclear complexes, the ligand showed bischelating behaviour with N-donor atoms that interact with one and S-donor atoms with another metal centre. Furthermore, the crystal structure of the trinuclear complex revealed the presence of two crystallographically independent cationic moieties in the asymmetric unit, which only differ by a single O-donor ligand (NO3– vs. H2O), where both of the moieties possess three different geometries around the constituent silver ions. Argentophilic interactions are present in the majority of the reported structures. Available amine N atoms facilitate hydrogen bond formation and promote the occurrence of solvates.

Throughout history, a substantial or often leap technological progress, was determined by the availability of new engineering materials, which usually stimulated the improvement in the quality of life. The development of engineering materials constitutes one of the most significant elements in the field of science and technology, and innovative policy in Poland and Europe as regards the knowledge-based economy (KBE). The enhancement of the product functional properties, required by the new strategies of engineering materials development, as well as materials processing technologies is frequently connected with appropriate formation of the engineering materials surface layer structure and properties. Functional properties of many products depend not only on the possibility of transferring the mechanical load across the whole element section or its physicochemical properties, but, most often, they depend mainly on the surface layer structure and properties. The application of foresight methodology in the assessment of the state and prospects regarding the development of technology related to the formation of the materials surface structure and properties provides the possibility to manage knowledge in this field. It can be achieved through a formalization of the explicit and tacit knowledge gathering and use in order to increase production competitiveness, effectiveness, and innovation, improve the quality of life and achieve sustainable development. The objectives accomplished in such a way contribute to the increase in intellectual capital by enhancing the effectiveness of using the existing knowledge, creating new knowledge, as well as increasing the level of understanding and disseminating knowledge. The implemented foresight project will result in identifying the priority innovative technologies and strategic research trends in the scope of surface structure and properties formation of engineering materials and biomaterials whose development in the country will be of key importance within next 20 years.

A novel bidentate ligand, H8-BIFOL, was successfully prepared and resolved starting from readily available reagents. The reactivity and selectivity was evaluated in the alkynylation of benzaldehyde, resulting in ee’s up to 56%.

Summary Congenital chylous ascites is a rare entity, conditioned by numerous factors and with changing dynamics of the disease. Because of the lack of therapeutic and diagnostic standards, this disease constitutes to be a medical challenge. This article presents current knowledge on pathogenesis, diagnostics and management of this disease, as well as a case of a newborn with primary congenital chylous ascites in the abdominal cavity.

Streszczenie Stosowanie antybiotykow związane jest z mozliwością wystąpienia biegunki. Jej przebieg moze byc rozny: od najcześciej wystepującej, lagodnej i samoograniczającej sie biegunki, poprzez zapalenie jelit i/lub okreznicy, do najciezszej postaci, jaką jest rzekomobloniaste zapalenie jelita grubego. Biegunka związana z antybiotykoterapią najcześciej wystepuje po amoksycylinie, amoksycylinie z kwasem klawulanowym oraz cefuroksymie. W leczeniu biegunki związanej z antybiotykoterapią zasadniczym elementem jest odstawienie antybiotyku, a w przypadku rzekomobloniastego zapalenia jelit zastosowanie metronidazolu lub wankomycyny. W profilaktyce biegunki związanej z antybiotykoterapią zastosowanie mogą miec probiotyki o udokumentowanym dzialaniu.

Changes in annual tree ring width (TRW) and its size depends not only on the changing climate and natural stress factors such as extreme air temperatures, shortages in rainfall and excess rainfall during the growing season, but also on anthropogenic stress, including chemical compounds emitted to the atmosphere or lowering of the groundwater table caused by the operations of plants with high environmental impact. The purpose of this article is to assess the impact of meteorological conditions and anthropogenic factors on the size of annual growth of Scots pine tree-stands in the conditions of the climate of central-eastern Poland. On the basis of five created site chronologies in the vicinity of Zakłady Azotowe Puławy (nitrogen factories in Puławy) and using the moving correlation analysis and multiple regression analysis, a significant influence of temperature and precipitation conditions on the TRW size is proved. A significant, positive influence of air temperature on TRW was proved for the majority of chronologies created in the period of January&ndash, March, as well as in June, while it remained negative in May. The wide rings of Scots pines were formed when the precipitation of October and January (prior to the resumption of cambium activity) was lower than the average, and higher in April and in June&ndash, August. After including the anthropopressure factors in the regression equations, the description of the variability of the annual tree ring width was corrected. The coefficient of determination ranged from approx. 29% to even above 45% and was higher, on average by 10%, for all studied chronologies of Scots pine compared to the one calculated for constructed equations considering only meteorological conditions. The strength and direction of the impact of the independent variables (SO2, NH3, NOx) analysed on TRW mainly depended on the distance from the plants, as well as on the direction of inflow of industrial pollution to the stands examined. In light of the proven climate changes in central and eastern Poland, the growth conditions of pine stands will most likely deteriorate.

Cyclic 2:2 complexes of Au I and bitmb [1,3-bis(imidazol-1-ylmethyl)-2,4,6-trimethylbenzene] were prepared and the structures of [Au 2 (μ-bitmb) 2 ](BF 4 ) 2 ·2CH 3 CN ( 1 ·2CH 3 CN), [Au 2 (μ-bitmb) 2 ](BF 4 ) 2 ·2CH 2 Cl 2 ( 1 ·2CH 2 Cl 2 ) and [Au 2 (μ-bitmb) 2 ](CF 3 SO 3 ) 2 ·2CH 2 Cl 2 ( 2 ·2CH 2 Cl 2 ) were determined by single crystal X-ray diffraction. The frameworks of the compounds consist of dicationic rectangular heterometallacycles linked by aurophilic interactions along the b axis and edge-to-face C–H⋯π contacts. The latter expand the structures in zigzag fashion along the a axis leading to a 3D assembly. Interactions between the counterions and imidazole rings are also present in all structures. When crystals of 2 ·2CH 2 Cl 2 are subjected to a dynamic vacuum at 90 °C remarkably only 1 mol equivalent of CH 2 Cl 2 is removed, but without loss of crystallinity, affording the structure of 2 ·CH 2 Cl 2 .