Ehlers Danlos type VI is a rare autosomal recessive connective tissue disease involving primarily the skin and joints. The main feature of the condition is neonatal hypotonia and rare complications ...are ruptures of arteries and the eye globe. A 4 year old girl with a typical clinical presentation and molecular diagnosis of EDS VI is presented. Sequencing of PLOD1 gene revealed a homozygous deletion in exon 13 (c.1362delC), leading to a frameshift and truncation of the lysyl hydroxylase, an enzyme necessary for collagen biosynthesis. Early diagnosis allowed treatment with high doses of ascorbic acid in order to prevent complications, genetic counseling of the family and prenatal diagnosis of an unaffected embryo.
Opuntia ficus indica je vrsta kaktusa koja pripada familiji Cactaceae. Ova familija kaktusa obuhvata oko 200 podvrsta kaktusa koji su rasprostranjeni najviše u Americi. Najčešću kulinarsku primjenu ...ima Indijska Fig Opuntia (Opuntia ficus indica ), jer se njeni listovi i plodovi koriste kao svježe salate. Plod kaktusa se konzumira zreo u ljetnom periodu. Najviše su rasprostranjeni na zapadnoj hemisferi (Meksiko) i zapadnim dijelovima Amerike, ali mogu se naći i u drugim dijelovima svijeta (Galapagos ostrva, Australija, Mediteran). Prva istraživanja Opuntia ficus indica započela su ispitivanjem sastava listova, a zatim i plodova u pogledu diuretskih efekata, a preliminarne studije su pokazale diuretska i antioksidativna svojstva koja su usko vezana za konzumaciju svježeg ploda.
Biološki aktivni sastojci preparata koji se koriste u suplementima hrane ili OTC dobiveni su postupnim tehnološkim procesom usitnjavanja, presovanja, klarifikacije i vakum evaporacije ploda Opuntia ficus indica . Dobivena praškasta forma ploda Opuntia ficus indica sadrži ukupno 65-85 % betalaina i to u obliku idiksantin i betanin. Rezultati navedeni u literaturi, dobijeni u različitim studijama sprovedenim na pacovima, pokazali su pozitivan efekat primjene praškaste forme kaktusovog ploda (Opuntia ficus indica). Pozitivni rezultati dokazuju veoma značajan nutricionisticki benefit ploda kaktusa Opuntia ficus indica , upravo zbog klinički dokazanih efekata. Sva otkrivena i dokazana svojstva ploda kaktusa Opuntia ficus indica zaslužuju značajnu pažnju primjene ovog ploda kod diuretskih
bolesti, antioksidativne zaštite organizma kao i očuvanju normalne tjelesne težine.
Provider: - Institution: - Data provided by Europeana Collections- Food allergy is an IgE mediated reaction of the immune system to food components, most commonly to food proteins. Food allergies ...develop after primary sensitization through the gastrointestinal tract and the mechanism behind this route of sensitization has not yet been elucidated. It has been postulated that in order to sensitize the organism through an oral route, food allergens must reach the gastrointestinal mucosa in at least a partially intact form in order to interact with the immune system. In addition to the unclear mechanism of sensitization, food allergies also present a challenge in the field of diagnostics, while the only effective therapy method to date is the strict avoidance of allergenic substances. During the last 30 years, since its first appearance on the world market, kiwifruit has become one of the more popular components of human nutrition, and yet, at the same time, it has emerged as one of the ten most frequent elicitors of allergic reactions to food. Actinidin, a cysteine protease from the papain family, is the most abundant protein in the pulp of kiwifruit and is considered the major allergen of this fruit. In this thesis the structural and immunological characteristics of actinidin were examined in order to shed light on the mechanism of oral sensitization and to aid in the improvement of kiwifruit allergy diagnosis. It was shown that actinidin purified from fresh kiwifruit under native conditions represents a mixture of the active and inactive enzyme. Differences in electrophoretic mobility, IgE binding in immunoblot and patient response in skin prick tests were shown for proteolytically active and inhibited actinidin. It was demonstrated that only the active enzyme survives conditions of simulated gastric and intestinal fluid while preserving its immunogenicity and proteolytic activity. The observed differences between the active and inhibited form of actinidin are most likely a consequence of differences in the conformation of the enzyme that arise upon inhibition, a hypothesis supported by results from CD spectroscopy and fluorimetry. Results presented in this thesis contribute to the effort of elucidating the mechanism of oral sensitization to kiwifruit by shedding light on the features of actinidin which enable it to reach the intestinal mucosa in a proteolytically active form. Additionally, since the influence of the activity (conformation) of actinidin on results of in vitro and in vivo diagnostic assays was demonstrated, results from this thesis could contribute to the improvement of kiwifruit allergy diagnosis. In addition, it was shown that the relative ratio of active and inhibited actinidin in kiwifruit protein extract depends on the chosen method for extract preparation. Apart from influencing future kiwifruit allergy diagnostic procedures, these results can be used to optimise the extraction procedure for kiwifruit protein extracts. In this thesis the possibility of using kiwifruit extract as a plant milk clotting agent was analysed. It was shown that the addition of kiwifruit extract prepared at pH 5.0 to cow milk leads to the formation of a casein clot. The obtained clot and whey were shown to have identical protein profiles to the clot and whey obtained through the activity of calf chymosin. The ratio between coagulating and proteolytical activity towards cow milk was only 30 % less favorable when using kiwifruit extract.- Alergije na hranu su IgE antitelima posredovane imunološke reakcije na komponente hrane, najčešće proteine. Razvijaju se nakon primarne senzitizacije preko gastrointestinalnog trakta, i za sada nije poznat mehanizam senzitizacije ovim putem. Smatra se da bi alergeni proteini hrane morali da prođu kroz gastrointestinalni trakt barem delimično očuvane strukture kako bi doveli do senzitizacije. Pored nerazjašnjenog mehanizma senzitizacije, alergije na hranu predstavljaju i izazov na polju dijagnostike, dok je jedina do sada potpuno uspešna metoda terapije strogo izbegavanje unosa alergene namirnice. Tokom svega 30-tak godina od svog prvog pojavljivanja na svetskom tržištu pa do danas plod kivija je postao jedna od omiljenijih namirnica u ljudskoj ishrani, ali je istovremeno dospeo i među deset najčešćih uzročnika alergije na hranu. Aktinidin, cistein proteaza iz papainske familije, je najzastupljeniji protein pulpe kivija i smatra se glavnim alergenim molekulom ovog izvora. U oviru ove disertacije ispitivane su strukturne i imunološke osobine aktinidina u cilju rasvetljavanja puta oralne senzitizacije i poboljšanja dijagnostike alergije na kivi. Pokazano je da aktinidin prečišćen iz svežeg ploda kivija pod nativnim uslovima predstavlja smešu aktivnog i neaktivnog enzima. Između proteolitički aktivnog i neaktivnog aktinidina postoji razlika u elektroforetskoj pokretljivosti, sposobnosti vezivanja IgE u imunoblotu i obrascima reagovanja pacijenata u kožnim probama. Pokazano je da samo aktivni enzim preživljava uslove simuliranog želudačnog i crevnog soka i da nakon ovih tretmana pokazuje očuvanu imunogenost i proteolitičku aktivnost. Uočene razlike između aktivnog i inhibiranog oblika aktinidina najverovatnije su posledica razlika u strukturi enzima koje nastaju prilikom inhibicije, a ova pretpostavka je potkrepljena rezultatima CD spektroskopije i fluorimetrije. Rezultati dobijeni u ovoj disertaciji doprinose rasvetljavanju mehanizama oralne senzitizacije na kivi ukazujući na strukturne osobine aktinidina koje mu omogućavaju da u proteolitički aktivnom obliku dospe do intestinalne mukoze. S obzirom da je pokazan uticaj aktivnosti (konformacije) aktinidina na in vivo i in vitro dijagnostičke eseje prikazani rezultati mogu doprineti i poboljšanju dijagnostike alergije na kivi. Pokazano je i da relativni odnos aktivnog i inhibiranog aktinidina u ekstraktu kivija zavisi od načina pripreme ekstrakta. Ovi rezultati, osim u poboljšanju dijagnostike alergije na kivi, mogu biti upotrebljeni i za optimizaciju proteinskih ekstrakata kivija i njihovu upotrebu u industriji mleka. U ovoj disertaciji je ispitana moguća upotreba ekstrakta kivija kao sredstva za koagulaciju mleka u procesu proizvodnje sira. Dobijeni rezultati su pokazali da dodavanjem proteinskog ekstrakta kivija pripremljenog na pH 5.0 u kravlje mleko dolazi do nastanka gruševine i surutke identičnih proteinskih profila kao prilikom dodavanja himozina poreklom iz želuca teleta. Izmeren odnos koagulacione i proteolitičke aktivnost ovog ekstrakta prema mleku je bio svega 30% lošiji od himozina.- All metadata published by Europeana are available free of restriction under the Creative Commons CC0 1.0 Universal Public Domain Dedication. However, Europeana requests that you actively acknowledge and give attribution to all metadata sources including Europeana
Provider: - Institution: - Data provided by Europeana Collections- Currant (Ribes sp.) is a very important berry fruit species ranking the second, after strawberry, for its commercial production on a ...world scale. In Serbia, currant is unjustifiably neglected fruit species because Serbia has very favorable agro-climate conditions for its successful growing and market demands for this fruit are persistent. Sporadic cultivation on small size areas or individual holdings, resulting in low yields and poor quality fruit, does not meet the demands of domestic market. Therefore, the production of this high-profitable fruit species must be intensified, which is possible to achieve not only by planting orchards based on modern technology of fruit growing principles but also by introducing new varieties of high quality fruits. Currant cultivars, especially representatives of black currant, demonstrate potent anti-oxidative, antiinflammatory, anti-microbial and anti-cancer activities due to the presence of phenolic compounds and vitamins in their fruits. Taking into account currant fruit species’ outstanding nutritional and medicinal properties as well as excellent production characteristics, the aim of investigations was to examine newly introduced cultivars of black, red and white currant and establish the suitability for their larger-scale cultivation in agro-ecological conditions of Serbia. The obtained results will be used to choose the cultivars with best properties to be recommended for exploitation in more intensive growing orchards covering larger production areas. Investigations predominantly focused on currant fruit biochemical properties and juices, and fatty oils quality from seeds of various cultivars was determined via essential fatty acids contents. Due to steady consumption needs for this fruit, analysis involved the effects of technological treatments (freezing and processing) on the contents and activities of polyphenolic components in them. Investigations were carried out in a currant collection orchard, the property of the “Omega” nursery (Mislodjin village, Obrenovacka Posavina), in the period 2007- 2009. The experimental orchard was planted in 2006, using the hedge system (a bush form), at 1.8 m of inter-row and 0.8 m intra-row spacing. Investigations comprised 29 currant cultivars (13 black currant cultivars, 11 red currant cultivars and 5 white currant cultivars). Laboratory analyses were performed at the Chair of Fruit Science, Faculty of Agriculture, Belgrade University, and at the Institute for Medicinal Plant Research “Dr. Josif Pancic’, Belgrade. During three-year studies, the following parameters were monitored: phenological properties of currant cultivars (date of the first leaf emergence, date of inflorescence emergence, date of initiation and full flowering, date of the first berry formed, date of fruit ripening – fruit harvest); vegetative growth potential of currant cultivars (number of newly formed sprouts per bush, dimensions, bush volume and weight removed by pruning); generative potential of currant cultivars (number of fruit buds per bush, number of newly formed inflorescences per fruit bud, number of inflorescences per bush, number of flowers in an inflorescence, per cent of fruit setting, cluster weight, cluster length, number of berries per cluster, berry weight, yield per bush and unit of area). Studies of currant fruit, juice and seed characterization involved the following compounds from a group of primary metabolites: contents of soluble dry matter, total and invert sugars, total acids and vitamin C. Studies of secondary metabolites included: contents of total phenols, total anthocyanins and individual aglicones of the anthocyanin as well as anti-oxidative activities of juices and quantitative and quantitative analysis of oils. Black currant cultivars entered statistically significantly earlier into the initial phenophases of vegetative growth compared to red currant and white currant cultivars (March, 6th and 22th). As vegetative growth progressed, the difference decreased between the dates of currant cultivars entering into later phenophases. Also, black currant cultivars had higher vegetative growth potential. A representative picture of the studied varieties’ vigor was indicated by the values of bush volume and bush weight removed by pruning. Average value of bush volume in black currant varieties amounted to 0.52 m3 and in red and white currants to 0.38 m3. According to bush volume, about 40% of black currant varieties had vigorous and very vigorous bush, while in red and white currants such vigor was exhibited by 12% of studied cultivars only. At the same time, the weight of sprouts and stems discarded by pruning from black currant bush was by about 70% higher than the weight of sprouts and stems removed from red and white currant cultivars. Taking into account all parameters of vegetative growth potential, red currant cultivars can be grown in the densest spacing, which additionally increases their production value per unit of cultivation area. Compared to black currant cultivars, the cultivars of red and white currant possessed higher generative potential: they formed a higher number of fruit buds/bush (204.30 : 129.86), inflorescences/bush (204.30 : 175.93), flowers in an inflorescence (20.19 : 9.51) and flowers/bush (4124.82 : 1673.09). Also, values of the majority of parameters for realizing generative potential level were higher than in black currant cultivars. Consequently, red and white currant cultivars produced considerably higher average yields (1.83 kg/bush and 12.71 t/ha). The fruits of black currant varieties had better nutritional and anti-oxidative properties compared to the fruits of red and white currant cultivars, which is reflected in significantly higher contents of soluble dry matter (14.09% : 11.35%) and total sugars (by approx. 20%). The most prominent difference between studied groups of cultivars was found in vitamin C contents. The fruits of red currant cultivars had 2.6 times higher contents of vitamin C than red and white currant fruits. As for total phenol contents, the values were by 2.1 times higher than in fruits of red and white currant cultivars, and ranged from 137.6 mg GAE/100g in cv. Bona to 278.9 mg GAE/100g in cv. Ometa. The contents of total anthocyanins were 5.6 times higher in black currant cultivars than in red currant cultivars, and delphinidin was the most present aglicone of the anthocyanin, while cyanidin was that in fruits of red currant cultivars. In juices produced from fruits of studied currant cultivars a decline in the contents of total phenols was recorded ranging from 44.11 - 76.02% in black currant cultivars and from 37.2 – 68.62% in cultivars of red and white currant. Preservation of fruits and juices in a frozen state at -200C during a one-year period caused qualitative and quantitative changes in their chemical composition and exhibited anti-oxidative activities. The levels of those changes were primarily determined by the cultivars’ genetic basis. Namely, the contents of total phenols were increased and the contents of total anthocyanins were decreased in the fruits of all black currant cultivars, while total phenols were decreased in white currant cultivars. In the fruits of four red currant cultivars a higher content of total phenols was registered, but it was lower in all other cultivars. However, increase of the contents of total anthocyanins was found in all red currant cultivars. In juices of all studied currant cultivars, kept in a frozen state, total phenol contents were increased. Black currant juices exhibited the most expressed antioxidative activity (IC50 value of 1.9 mg/ml in cv. Ben Lomond to 4.0 mg/ml in cv. Ojebin), then red currant juices (IC50 value of 1.9 mg/ml in cv. Redpul to 12.3 mg/ml in cv. Slovakia) and, lastly, white currant juices (IC50 value of 3.48 mg/ml in cv. Primus to 9.94 mg/ml in cv. White Champagne). Therefore, the lowest amount of juice from black currant cultivars and the highest amount of juice from white currant cultivars is needed to neutralize free radicals. In general, fruits and juices of black currant cultivars demonstrated considerably better quality nutritional properties and higher anti-oxidative capacity compared to the cultivars of red and white currant. Fruit processing into juices caused reduction of secondary metabolites content in them, except for total anthocyanins content in seven red currant cultivars. Preservation of fruits and juices in a frozen state increased the content of total phenols, in some cases as high as 171.8%. During preservation, total anthocyanins content decrease was registered in black currant cultivars, however its increase in red currant cultivars. After freezing, juices obtained from black currant cultivars and six red currant cultivars had higher anti-radical activity. Higher content of fatty oils was found in the seeds of black currant cultivars, but red and white currant cultivars had more favorable composition of essential fatty acids. A comprehensive analysis of currant cultivars grown in the Obrenovacka Posavina agro-ecological conditions proved that the best production, nutritional and anti-oxidative properties are shown by black currant cultivars: Bona, Ben Sarek, Tsema and Ometa; red currant cultivars: Јunifer, Rondom and Redpoll, and white currant cultivar Primus.- Ribizla (Ribes sp.) je veoma značajna vrsta jagodastih voćaka, koja se po obimu proizvodnje u svetskim razmerama nalazi na drugom mestu, odmah iza jagode. U Srbiji je ribizla neopravdano zapostavljena vrsta, iako postoje izuzetno pogodni agroklimatski uslovi za njeno uspešno gajenje i konstantna tražnja na tržištu za ovim voćem. Sporadično gajenje na malim površinama ili okućnicama, praćeno niskim prinosima i lošijim kvalitetom ploda, ne zadovoljava potrebe domaćeg tržišta. Stoga, nameće se potreba za intenziviranjem proizvodnje ove visokorentabilne vrste voćak
Rad se bavi pitanjima nezakonitih dokaza u okvirima hrvatskoga kaznenog postu ̋povnog prava dajući kratki pregled temeljnih načela i polazišta de- finiranih primjenjivim pravnim izvorištima, ...odnosno nacionalnim postupovnim zakonodavstvom, Ustavom RH i Europskom konvencijom. Konkretnije, smisao rada jest analiza valjanosti uporabe (iskoristivosti) sadržaja prikupljenih mjerama tajnog snimanja telefonskih razgovora prema trećima osobama, odnosno osobama prema kojima takve mjere nisu bile inicijalno određene, a sve u smislu odredbe članka 8. Europske konvencije i opravdanih očekivanja u zaštiti temeljnih prava na privatnost i općenje. Ova pitanja analizirana su komparacijom nacionalnog sudovanja i temeljnog standarda zaštite prava fundiranog na presudama ESLJP-a, sa zaključkom kako su određene promjene u nacionalnom zakonodavstvu (Zakon o kaznenom postupku) nužne, želi li se ispuniti postavljene standarde.
The paper deals with the issue of
unlawful evidences within terms of Croatian criminal legal system providing
brief overview of the basic principles and framework as defined by applicable
legal sources, i.e. national procedural law, the Constitution and the European
Convention. More precisely, it aims to analyse the validity of usage of
contents gathered by means of covert recording of telephone conversation
against the third party or the person against those specific measures have not
been ordered, within the scope of the Art. 8. of European Convention and
reasonable expectations in protection of basic rights of privacy and
communication. These issues have been analysed through comparison of the
national judiciary and basic rights protection standard brought by ECHR,
concluding that certain changes in Croatian legislation (The Criminal
proceedings act) will have to take place in order to comply with the highest
standards developed.
Provider: - Institution: - Data provided by Europeana Collections- Original language summary:
Populační politika a péče o novorozence v socialistickém Československu a rozhovor s profesorem Švejcarem ...o omylech, které se matky dopouštějí u malých dětí.- Extended description:
V roce 1974 byl v Československu největší přírůstek obyvatelstva za posledních 20 let. Dítěti u nás je věnována velká péče už od narození. Profesor Josef Švejcar se věnuje dětem už 55 let. Proto jsme se ho zeptali na chyby a omyly, kterých se matky dopouštějí u malých dětí. Dítě především se musí odmalička otužovat, pobývat hodně na čerstvém vzduchu, nabýt přehnaně oblečeno. Má také spát v místnosti, kde je otevřené okno. Nejlepší dětskou výživou pro dítě je mateřské mléko. V něm jsou obsaženy také protilátky proti infekci. Navíc kojením vzniká mezi matkou a dítětem citové pouto, které ho provází další život.
In 1974, there was the largest increase in population over the past 20 years in Czechoslovakia. A child is given a maximum care since its birth in our country. Professor Josef Švejcar deals with children for 55 years. Therefore, we asked what errors and mistakes do mothers of small children make. First of all, a child should be hardened from the early childhood; it should stay in fresh air and be dressed appropriately according to the weather. It should also sleep in a room with an open window. Breast milk is the best food for a baby. It contains antidotes against infection. Moreover, breast feeding creates an emotional bond between mother and child which accompanies it through its whole life.- Information:- Population policy and newborn care in socialist Czechoslovakia and an interview with Professor Švejcar of mistakes that are committed by mothers with small children- All metadata published by Europeana are available free of restriction under the Creative Commons CC0 1.0 Universal Public Domain Dedication. However, Europeana requests that you actively acknowledge and give attribution to all metadata sources including Europeana
Provider: Swedish Open Cultural Heritage | K-samsök - Institution: - Data provided by Europeana Collections- Plog från Palmcrantz & Co i Stockholm.- All metadata published by Europeana are available ...free of restriction under the Creative Commons CC0 1.0 Universal Public Domain Dedication. However, Europeana requests that you actively acknowledge and give attribution to all metadata sources including Europeana
U radu su analizirane sorte jabuke Cripps Pink, Granny Smith, Jonagold, Idared, Golden Delicious i Braeburn u svrhu pogodnosti plodova za sušenje. Rezultati istraživanja, odnosno polinomne jednadžbe ...krivulje sušenja, ukazuju da su neke sorte jabuka pogodnije za proizvodnju čipsa od jabuka (Cripps Pink, Golden Delicious, Idared). Kako bi se sačuvala kvaliteta listića (čips) i smanjili troškovi, nužno je prosec sušenja podijeliti u više faza. Priprema jabuka za dobivanje čipsa dosta je zahtjevna, što izravno utječe na troškove, pa je stoga nužno pripremne faze maksimalno mehanizirati.
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