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Goldenhar syndrome: current perspectives

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World Journal of Pediatrics 2017年第5期13卷 405-415页

作者： katarzyna Bogusiak Aleksandra Puch Piotr Arkuszewski Craniomaxillofacial and Oncological Surgery Clinic Medical University of LodzLodzPoland Department of Dentistry Medical University of LodzLodzPoland

Background:Progress in medical branches that has taken place since the first child with Goldenhare syndrome (GS) had been described in 1952 by Maurice Goldenhar,facilitated better understanding of this congenital *** also gave new perspectives and the opportunity to achieve satisfactory treatment results,mainly due to development of surgical *** sources:Based on the literature and own experience,we discussed the phenotype of presentation of GS,ethiopathogenesis,genetic counselling and treatment with particular emphasis on surgery correction of hemifacial ***:The spectrum of GS abnormalities ranges from mild to severe ones and include patients with barely noticeable facial asymmetry to very pronounced facial defect with more or less severe abnormalities of internal organs and/ or *** is characterized most commonly by impaired development of eyes,ears,lips,tongue,palate,mandible,maxifla,zygomatic and orbital structures and deformations of the teeth *** is multifactorial and dependent on genetic and environmental factors but there are still many unknowns about the syndrome which should be ***:Patients with GS due to a large variety of abnormalities and different severity of symptoms pose a challenge for *** of this necessitate an individual approach to each single patient and involvement a team of specialists in treatment *** is a complex,long-lasting,multidisciplinary process and should be divided into stages,according to patient's age,as well as the extent and severity of observed *** and pediatricians are involved in care of these patients from the onset.

关键词： abnormalities Goldenhar syndrome oculoauriculovertebral syndrome

Dietary carnosic acid and seleno-compounds change concentrations of fatty acids, cholesterol, tocopherols and malondialdehyde in fat and heart of lambs

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Animal Nutrition 2021年第3期7卷 812-822页

作者： Malgorzata Bialek Marian Czauderna Kamil Zaworski katarzyna Krajewska The Kielanowski Institute of Animal Physiology and Nutrition Polish Academy of Sciences05-110 JablonnaPoland

The aim of the current study was to evaluate the impact of carnosic acid(CA),selenised yeast(^(Y)Se)and selenate(^(VI)Se)supplemented to diets,including fish oil(FO)and rapeseed oil(RO),on the content of fatty acids,total cholesterol(TCh),tocopherols and malondialdehyde in the fat located between the thigh muscles and the heart in ***-four male Corriedale lambs were divided into 4 groups of 6 *** were fed a diet with FO and RO(the control diet)or experimental diets containing RO,FO and CA with/without Se(as^(Y)Se or^(VI)Se).The experimental diets without/with^(Y)Se or^(VI)Se changed concentrations of fatty acids in the fat and heart compared to the *** experimental diets increased the levels of c11c14C20:2,c5c8c11c14C20:4,c5c8c11c14c17C20:5 and the sums of long-chain polyunsaturated fatty acids(LPUFA)and conjugated linoleic acid isomers in the fat compared to the *** experimental diet containing^(Y)Se or^(VI)Se increased the content of Se,TCh,c11c14C20:2,c8c11c14C20:3,c5c8c11c14C20:4,c5c8c11c14c17C20:5,c7c10c13c16c19C22:5,c4c7c10c13c16c19C22:6 and the concentration sum of n-3LPUFA,n-6LPUFA and tocopherols in the heart in comparison with the control diet and the diet containing only *** diets reduced the concentration of malon-dialdehyde in the fat and heart in comparison with the control *** dietary intervention has great potential for future practical and commercial implementations.

关键词： Selenium Carnosic acid Fatty acid Tocopherol Cholesterol Lamb

Drosophila melanogaster as a model system of aluminum toxicity and aging

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Insect Science 2014年第2期21卷 189-202页

作者： Ewelina Kijak Ezio Rosato katarzyna Knapczyk Elzbieta Pyza Department of Cell Biology and Imaging Institute of Zoology Jagiellonian University Krakow Poland Department of Genetics University of Leicester Leicester LE1 7RH United Kingdom

The aim of this study was to investigate the toxic effects of aluminum （A1） on the model organism-Drosophila melanogaster. The study is especially concerned with the effects of aluminum on the fruit fly＇s development, life span, and circadian rhythm in rest and activity. Flies were exposed to aluminum in concentrations from 40 to 280 mg/kg in rearing media or the flies were raised on control medium. Moreover, the life span of insects exposed to aluminum containing 40, 120, or 240 mg/kg of A1 in the medium, only during their larval development, during the whole life cycle and only in their adult life was tested. To check if aluminum and aging cause changes in D. melanogaster behavior, the locomotor activity of flies at different ages was recorded. Results showed that aluminum is toxic in concentrations above 160 mg/kg in the rearing medium. Depending on A1 concentration and time of exposure, the life span of the flies was shortened. At intermediate concentrations （120 mg/kg）, however, A1 had a stimulating effect on males increasing their life span and level of locomotor activity. At higher concentration the aluminum exposure increased or decreased the level of locomotor activity ofD. melanogaster depending on age of flies. In addition, in the oldest insects reared on aluminum supplemented media and in mid-aged flies reared on the highest concentration of A1 the daily rhythm of activity was disrupted.

关键词： circadian rhythms development life-span locomotor activity rest/activity cycle survival

Assessing discharge periodicity in mountain catchments using classified environmental conditions(Tatra Mountains, Poland)

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Journal of Mountain Science 2022年第1期19卷 16-32页

作者： BOROWska-PAKUŁA Joanna MIĘSIAK-WÓJCIK katarzyna Department of Hydrology and Climatology Institute of Earth and Envronmental ScienceMaria Curie-Skłodowska UniversityAleja Kraśnicka 2D20-031 LublinPoland

The periodicity of a river expressed in cycles of various lengths(monthly, seasonal,multiannual) is a result of climatic factors and overlapping environmental conditions within its catchment. In uncontrolled or poorly surveyed catchments, it is very difficult to determine the duration of a stream’s hydrological activity. This is especially relevant for catchments with complicated water circulation in karstic rocks. The present study concerns the small catchment of the Str??yski Potok river located in the area of the Tatra National Park, in the Western Tatras. The observation period covered the 2015 hydrological year, which differed hydrologically from average conditions. This study aims to develop a simple method to explain the processes shaping the mountain stream discharge periodicity. The research employed periodic field observations linked with climatic and non-climatic factors. Environmental conditions were assessed as four classes reflecting their influence on appearance or disappearance of mountain stream water. Class boundaries were the values of quartiles. The degree of correspondence between environmental factors and stream field observations was described via the Index DC(Degree Correspondence Index) *** correspondence(Index DC =0) was found in 23% catchments, a weak relationship between conditions favouring discharge and actual condition(Index DC=-1, +1) was noted within 11 catchments,while in 9 catchments, no such relationship was found(Index DC =-2, +2). The obtained results indicate a correspondence or lack thereof between the environmental potential of the catchment and its discharge periodicity. The discrepancies between the assessment of the influence of climatic and nonclimatic factors and the data collected during field observations provide a basis for more detailed *** of these studies based on the proposed classifications will allow for a more complete explanation of water disappearance in river channels and the d

关键词： Discharge periodicity Small mountain catchment Tatra Mountains Environmental analysis

Challenges and opportunities for using formate to store, transport, and use hydrogen

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Journal of Energy Chemistry 2020年第2期29卷 216-224页

作者： katarzyna Grubel Hyangsoo Jeong Chang Won Yoon Tom Autrey Pacific Northwest National Laboratory 902 Battelle Boulevard.P.O.Box 999.Richland.WA 99352.United States Center for Hydrogen and Fuel Cell Research Korea Institute of Science and Technology(KIST).5 Hwarang-ro 14-gil.Seongbuk-gu.Seoul 02792Republic of Korea Divison of Energy and Environment Technology KIST SchoolKorea University of Science and Technology5 Hwarung-ro 14-gil.Seongbuk-guSeoul 02792.Republic of Korea KHU-KIST Department of Converging Science and Technology Kyung Hee University26 Kyungheedae-ro.Dongdaemun-guSeoul 02447Republic of Korea

In this perspective article,the synthesis and thermodynamic properties of aqueous solutions of formate salts(FS,HCO2-)are described in relationship to the concept of *** physiochemical properties of solid FS,aqueous formate solutions,and aqueous bicarbonate solutions set the limitations for storage capacity,deliverable capacity,and usable H2capacity of these H2carriers,*** parameters will help in the design of systems that use H2carriers for storage and transport of H2for fuel cell power ***,as well as admixtures with formic acid(FA,H2CO2),have potential to address the goals outlined in the *** of Energy’s H2@scale initiative to store in chemical bonds a significant quantity of energy(hundreds of megawatts)obtained from large scale renewable resources.

关键词： Hydrogen carriers Formate salts Bicarbonate salts Thermodynamics

Inhalable microparticles as drug delivery systems to the lungs in a dry powder formulations

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Regenerative Biomaterials 2023年第1期10卷 218-248页

作者： Karolina Knap Konrad Kwiecien katarzyna Reczynska-Kolman Elzbieta Pamuła Department of Biomaterials and Composites Faculty of Materials Science and CeramicsAGH University of Science and Technology30-059 KrakowPoland

Inhalation-administrated drugs remain an interesting possibility of addressing pulmonary *** drug delivery to the lungs allows one to obtain high concentration in the site of action with limited systemic distribution,leading to a more effective therapy with reduced required doses and side *** the other hand,there are several difficulties in obtaining a formulation that would meet all the criteria related to physicochemical,aerodynamic and biological properties,which is the reason why only very few of the investigated systems can reach the clinical trial phase and proceed to everyday use as a ***,we focused on powders consisting of polysaccharides,lipids,proteins or natural and synthetic polymers in the form of microparticles that are delivered by inhalation to the lungs as drug *** summarized the most common trends in research today to provide the best dry powders in the right fraction for inhalation that would be able to release the drug before being removed by natural *** review article addresses the most common manufacturing methods with novel modifications,pros and cons of different materials,drug loading capacities with release profiles,and biological properties such as cytocompatibility,bactericidal or anticancer properties.

关键词： pulmonary therapies drug delivery systems to lungs microparticles inhalers dry powder inhalers

The increase of adenylate kinase activity in the blood can control aggregation of platelets in coronary or peripheral arterial ischemia

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Health 2010年第3期2卷 246-252页

作者： Bozena Studzińska Anna Seroka Marta Lepicka katarzyna Roszek Michal Komoszyński Nicolaus Copernicus University Toruń Poland

Activation and aggregation of blood platelets is crucial for hemostasis and thrombosis. In the vascular system adenine nucleotides are important signaling molecules playing a key role in hemostasis. ADP was the first low molecular weight agent recognized to cause blood platelets activation and aggregation. NTPDases and adenylate kinase (AK) are the main enzymes involved in metabolism of extracellular adenine nucleotides. The majority of studies concentrated on the role of NTPDase1 (apyrase) in the inhibition of platelets aggregation. Up to now, there are still insufficient data concerning the role of AK in this process. We found that adenylate kinase activity in the serum of patients with myocardial infarction is significantly increased when compared to the healthy volunteers. The elevated activity of AK is connected to appearance of another isoform of that enzyme, expressed in patients with myocardial infarction. The influence of AK on the pig blood platelets aggregation induced by 20 μM ADP or 7.5 μg/ml rat collagen was examined. 1U of adenylate kinase added to platelet-rich plasma (PRP) before ADP or collagen, inhibited the platelets aggregation. One minute after induction of platelets activation by ADP as much as 5U of adenylate kinase was necessary to stop the platelet aggregation. In the case of collagen activated aggregation, only 2U of AK added 1 or 5 minutes after initiation of the aggregation process were sufficient for disaggregation of platelets. The increase of ATP: ADP ratio is probably responsible for the initiation of disaggregation process. We conclude that adenylate kinase is involved in regulation of plate-lets aggregation. Anticoagulative role of AK indicates the possibility of using this enzyme in the treatment of cardiovascular diseases.

关键词： Hemostasis Platelets Aggregation Adenylate Kinase

Gastric hyperplastic polyp with focal cancer

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Gastroenterology Report 2016年第2期4卷 158-161,I0003页

作者： Adam Roman Markowski katarzyna Guzinska-Ustymowicz Department of Internal Medicine and Gastroenterology Polish Red Cross Memorial Municipal HospitalBialystokPoland Endoscopy Unit and Outpatient Department Hospital of the Ministry of InteriorBialystokPoland Department of General Pathomorphology Medical University of BialystokBialystokPoland

This paper reports a rare case of early adenocarcinoma within the gastric hyperplastic polyp,that was completely resected during an endoscopic procedure,and discusses current recommendations in such *** resection of polyps with focal dysplasia or cancer is commonly indicated,as long as the procedure can be performed *** complete excision of a polyp with atypical focal lesion,endoscopic surveillance is *** frequency of surveillance endoscopy should depend on the precise histopathological diagnosis and possibility of confirming the completeness of the endoscopic *** the completeness of the procedure is confirmed both macro-and microscopically,gastric resection does not have to be performed.A follow-up esophago-gastroduodenoscopy should be performed at 1 year and then at 3 years.

关键词： gastric hyperplastic polyp gastric cancer surveillance

Molecular diffusion in ternary poly(vinyl alcohol)solutions

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Frontiers of Chemical Science and Engineering 2022年第6期16卷 1003-1016页

作者： katarzyna Majerczak Ophelie Squillace Zhiwei Shi Zhanping Zhang Zhenyu J.Zhang School of Chemical Engineering University of BirminghamBirmingham B152TTUK The Procter and Gamble Company CincinnatiOH 45040USA

The diffusion kinetics of a molecular probe-rhodamine B-in ternary aqueous solutions containing poly(vinyl alcohol),glycerol,and surfactants was investigated using fluorescence correlation spectroscopy and dynamic light *** show that the diffusion characteristics of rhodamine B in such complex systems is determined by a synergistic effect of molecular crowding and intermolecular interactions between chemical *** presence of glycerol has no noticeable impact on rhodamine B diffusion at low concentration,but significantly slows down the diffiision of rhodamine B above 3.9%(w/v)due to a dominating steric inhibition ***,introducing surfactants(cationic/nonionic/anionic)to the system results in a decreased diffusion coefficient of the molecular *** solutions containing nonionic surfactant,this can be explained by an increased crowding *** ternary poly(vinyl alcohol)solutions containing cationic or anionic surfactant,surfactant-polymer and surfactant-rhodamine B interactions alongside the crowding effect of the molecules slow down the overall diffiisivity of rhodamine *** results advance our insight of molecular migration in a broad range of industrial complex formulations that incorporate multiple compounds,and highlight the importance of selecting the appropriate additives and surfactants in formulated products.

关键词： fluorescence correlation spectroscopy poly(vinyl alcohol) anomalous diffusion crowding effects dynamic light scattering binding effects rhodamine B