Search

People have been accompanied by colours throughout the history and through all periods of life. In different eras, colours were also associated with status symbols of different social classes or mythological beliefs. We are often addressed emotionally by colour combinations, since colour perception is always and exclusively a sensorial experience. Various colour combinations can have a pleasant effect on us or leave us cold as well as in a state of shock. All of the above, presented the starting point of this research. Detection and perception of sensations through colour was accomplished by preparing a questionnaire related to 12 selected colours. The research included 302 participants from Slovenia, of both sexes and different ages, born from 1940 to 2004. From the obtained results, it could be concluded how popular a certain colour is in a material, design and spiritual sense. Moreover, the results were validated and compared regarding the gender and age of participants, and further compared with the results of previous studies.

This paper analyses approaches to involving young people in the processes of valuation, interpretation and utilization of local heritage to meet contemporary development challenges. The starting point is the finding that European development strategies highlight the various potentials of cultural heritage, but young people from western Slovenia hardly see any of these potentials. Moreover, the presented ethnographic research results show that young people are not very interested in heritage practices undertaken in their cities or villages. This paper’s results demonstrate how heritage may be introduced to young generations, how youth understand local heritage and its values, and what they think about voluntary activities in cultural fields., U radu se analiziraju pristupi uključivanja mladih u procese vrednovanja, interpretacije i korištenja lokalne baštine ususret suvremenim izazovima razvoja. Polazi se od toga da europske razvojne strategije naglašavaju različite potencijale kulturne baštine, no mladi iz zapadne Slovenije teško uviđaju te potencijale. Nadalje, rezultati etnografskog istraživanja pokazuju da mladi nisu značajnije zainteresirani za prakse vezane uz baštinu koje se provode u njihovim gradovima i selima. Rezultati ovog rada pokazuju na koji se način baština može predstaviti mlađim generacijama, kako mladi razumiju lokalnu baštinu i njezine vrijednosti te što misle o volonterskim aktivnostima u području kulture.

The main objective of the presented research was to study the possibility of using natural colourants obtained through the aqueous extraction of fresh leaves from the Junglans regia (J. regia), or common walnut tree, for the dyeing of wool yarn. A unique principle was explored by combining the phases of extraction and mordanting into one with the aim of shortening the dyeing procedure, while at the same time extracting more colouring components. Spectrophotometric studies revealed the signifi cant impact of both mordant addition and dye-bath pH on the absorbance curve and thus on the colour and K/S values of the dyed samples. A meta-ordanting technique using ferrous sulphate produced a greater depth of shade at a wavelength of 400 nm, with respect to the concentration of mordant and the liquid ratio of the extraction. Finally, wool yarn dyed with pure leaf extracts exhibited a potent inhibiting activity against Candida albicans (C. albicans) with a moderate reduction rate of 59%, and an inhibited response against Staphylococcus aureus (S. aureus) with a low reduction rate of 38.6%.

Graphical abstract, Highlights • Three novel gelsolin amyloidogenic substitutions cluster at the G4:G5 interface. • Mutations impair stability through strand distortion, charge and steric repulsion. • Mutations do not increase sensitivity to furin, the first step of the canonical pathway. • In the unproteolysed form these variants tend to aggregate and are proteotoxic. • The variants aggregate via an alternative, likely proteolysis-independent, mechanism., Gelsolin comprises six homologous domains, named G1 to G6. Single point substitutions in this protein are responsible for AGel amyloidosis, a hereditary disease causing progressive corneal lattice dystrophy, cutis laxa, and polyneuropathy. Although several different amyloidogenic variants of gelsolin have been identified, only the most common mutants present in the G2 domain have been thoroughly characterized, leading to clarification of the functional mechanism. The molecular events underlying the pathological aggregation of 3 recently identified mutations, namely A551P, E553K and M517R, all localized at the interface between G4 and G5, are here explored for the first time. Structural studies point to destabilization of the interface between G4 and G5 due to three structural determinants: β-strand breaking, steric hindrance and/or charge repulsion, all implying impairment of interdomain contacts. Such rearrangements decrease the temperature and pressure stability of gelsolin but do not alter its susceptibility to furin cleavage, the first event in the canonical aggregation pathway. These variants also have a greater tendency to aggregate in the unproteolysed forms and exhibit higher proteotoxicity in a C. elegans-based assay. Our data suggest that aggregation of G4G5 variants follows an alternative, likely proteolysis-independent, pathway.

The presented research examined five adsorbents, i.e., zeolite 4A, a mixture of three zeolites (4A, 13X, and ZSM-5), natural zeolite (tuff), activated carbon, and peat, and their potential capability for removal of exceeded ions of lead (Pb), antimony (Sb), sulphates (SO42−), and fluorides (F−) from real wastewater generated in the crystal glass industry, which was previously treated in-situ by flocculation, with the aim to attain the statutory values for discharge into watercourses or possible recycling. The screening experiment evidenced that the tuff was the most suitable adsorbent for the reduction of Pb (93.8%) and F− (98.1%). It also lowered wastewater’s pH sufficiently from 9.6 to 7.8, although it was less appropriate for the reduction of Sb (66.7%) as compared to activated carbon (96.7%) or peat (99.9%). By adjusting the pH of the initial wastewater to pH 5, its adsorption capacity even enlarged. Results from the tuff-filled column experiment revealed reduction of Pb up to 97%, Sb up to 80%, and F− up to 96%, depending on the velocity flow, and thus it could be used for post-treatment (and recycling) of wastewaters from the crystal glass industry. Moreover, the system showed an explicit buffering capacity, but negligible reduction of the SO42−.

In contrast to USH2A, variants in ADGRV1 are a minor cause of Usher syndrome type 2, and the associated phenotype is less known. The purpose of the study was to characterize the retinal phenotype of 18 ADGRV1 patients (9 male, 9 female, median age 52 years) and compare it with that of 204 USH2A patients (111 male, 93 female, median age 43 years) in terms of nyctalopia onset, best corrected visual acuity (BCVA), fundus autofluorescence (FAF), and optical coherence tomography (OCT) features. There was no statistical difference in the median age at onset (30 and 18 years, Mann–Whitney U test, p = 0.13), the mean age when 50% of the patients reached legal blindness (≥1.0 log MAR) based on visual acuity (64 years for both groups, log-rank, p = 0.3), the risk of developing advanced retinal degeneration (patch or atrophy) with age (multiple logistic regression, p = 0.8), or the frequency of cystoid macular edema (31% vs. 26%, Fisher’s exact test, p = 0.4). ADGRV1 and USH2A retinopathy were indistinguishable in all major functional and structural characteristics, suggesting that the loss of function of the corresponding proteins produces similar effects in the retina. The results are important for counseling ADGRV1 patients, who represent the minor patient subgroup.

Achromatopsia has been proposed to be a morphologically predominately stable retinopathy with rare reports of progression of structural changes in the macula. A five-grade system of optical coherence tomography (OCT) features has been used for the classification of structural macular changes. However, their association with age remains questionable. We characterized the Slovenian cohort of 12 patients with pathogenic variants in CNGA3 or CNGB3 who had been followed up with OCT for up to 9 years. Based on observed structural changes in association with age, the following four-stage classification of retinal morphological changes was proposed: (I) preserved inner segment ellipsoid band (Ise), (II) disrupted ISe, (III) ISe loss and (IV) ISe and RPE loss. Data from six previously published studies reporting OCT morphology in CNGA3 and CNGB3 patients were additionally collected, forming the largest CNGA3/CNGB3 cohort to date, comprising 126 patients aged 1–71 years. Multiple regression analysis showed a significant correlation of OCT stage with age (p &lt, 0.001) and no correlation with gene (p &gt, 0.05). The median ages of patients with stages I–IV were 12 years, 23 years, 27 years and 48 years, respectively, and no patient older than 50 years had continuous ISe. Our findings suggest that achromatopsia presents with slowly but steadily progressive structural changes of the macular outer retinal layers. However, whether morphological changes in time follow the proposed four-stage linear pattern needs to be confirmed in a long-term study.

This paper focuses on the Kras/Carso Food Tour to present the challenges of developing a luxury tourism product. The tour's design followed the current strategy of the Slovenian Tourist Board, which defines specific criteria for a luxury experience. Contextualized by the experiential trends in tourism, the paper juxtaposes the bottom-up and top-down perspectives on luxury experiences. The authors argue that mediation by experts familiar with academic discourses and local culture is beneficial for the development of a successful tourism product. The study contributes to the debates on luxury tourism, which have neglected bottom-up perceptions in tourist discourse and overlooked the dilemmas people face when "luxurifying" their traditions and heritage practices.

Achromatopsia has been proposed to be a morphologically predominately stable retinopathy with rare reports of progression of structural changes in the macula. A five-grade system of optical coherence tomography (OCT) features has been used for the classification of structural macular changes. However, their association with age remains questionable. We characterized the Slovenian cohort of 12 patients with pathogenic variants in

Purpose: Missing heritability in human diseases represents a major challenge, and this is particularly true for ABCA4-associated Stargardt disease (STGD1). We aimed to elucidate the genomic and transcriptomic variation in 1054 unsolved STGD and STGD-like probands. Methods: Sequencing of the complete 128-kb ABCA4 gene was performed using single-molecule molecular inversion probes (smMIPs), based on a semiautomated and cost-effective method. Structural variants (SVs) were identified using relative read coverage analyses and putative splice defects were studied using in vitro assays. Results: In 448 biallelic probands 14 known and 13 novel deep-intronic variants were found, resulting in pseudoexon (PE) insertions or exon elongations in 105 alleles. Intriguingly, intron 13 variants c.1938-621G>A and c.1938-514G>A resulted in dual PE insertions consisting of the same upstream, but different downstream PEs. The intron 44 variant c.6148-84A>T resulted in two PE insertions and flanking exon deletions. Eleven distinct large deletions were found, two of which contained small inverted segments. Uniparental isodisomy of chromosome 1 was identified in one proband. Conclusion: Deep sequencing of ABCA4 and midigene-based splice assays allowed the identification of SVs and causal deep-intronic variants in 25% of biallelic STGD1 cases, which represents a model study that can be applied to other inherited diseases. Fil: Khan, Mubeen. Radboud University Nijmegen Medical Centre; Países Bajos Fil: Cornelis, Stéphanie S.. Radboud University Nijmegen Medical Centre; Países Bajos Fil: Del Pozo Valero, Marta. Hospital Universitario Fundación Jiménez Díaz; España. Radboud University Nijmegen Medical Centre; Países Bajos Fil: Whelan, Laura. Trinity College; Estados Unidos Fil: Runhart, Esmee H.. Radboud University Nijmegen Medical Centre; Países Bajos Fil: Mishra, Ketan. Radboud University Nijmegen Medical Centre; Países Bajos Fil: Bults, Femke. Radboud University Nijmegen Medical Centre; Países Bajos Fil: AlSwaiti, Yahya. St John of Jerusalem Eye Hospital Group; Palestina (ANP) Fil: AlTalbishi, Alaa. St John of Jerusalem Eye Hospital Group; Palestina (ANP) Fil: De Baere, Elfride. University of Ghent; Bélgica Fil: Banfi, Sandro. Seconda Universita Degli Studi Di Napoli; Italia Fil: Banin, Eyal. The Hebrew University of Jerusalem; Israel Fil: Bauwens, Miriam. University of Ghent; Bélgica Fil: Ben Yosef, Tamar. The Ruth And Bruce Rappaport Faculty Of Medicine; Israel Fil: Boon, Camiel J. F.. Leiden University. Leiden University Medical Center; Países Bajos Fil: van den Born, L. Ingeborgh. Rotterdam Ophthalmic Institute; Países Bajos Fil: Defoort, Sabine. Universite Lille; Francia Fil: Devos, Aurore. Universite Lille; Francia Fil: Dockery, Adrian. Trinity College; Estados Unidos Fil: Dudakova, Lubica. Charles University and General University Hospital; República Checa Fil: Fakin, Ana. Charles University and General University Hospital; República Checa Fil: Farrar, G. Jane. Trinity College; Estados Unidos Fil: Ferraz Sallum, Juliana Maria. Universidade Federal de Sao Paulo; Brasil Fil: Fujinami, Kaoru. UCL Institute of Ophthalmology; Reino Unido Fil: Gilissen, Christian. Radboud University Nijmegen Medical Centre; Países Bajos Fil: Glavac, Damjan. University of Ljubljana; Eslovenia Fil: Gorin, Michael B.. University of California at Los Angeles. School of Medicine; Estados Unidos Fil: Greenberg, Jacquie. University of Cape Town; Sudáfrica Fil: Hayashi, Takaaki. The Jikei University School of Medicine; Japón Fil: Hettinga, Ymkje M.. Bartiméus Diagnostic Center for Complex Visual Disorders; Países Bajos Fil: Hoischen, Alexander. Radboud University Nijmegen Medical Centre; Países Bajos Fil: Hoyng, Carel B.. Radboud University Nijmegen Medical Centre; Países Bajos Fil: Hufendiek, Karsten. University Eye Hospital Hannover Medical School; Alemania Fil: Jägle, Herbert. University Regensburg; Alemania Fil: Kamakari, Smaragda. OMMA Ophthalmological Institute of Athens; Grecia Fil: Karali, Marianthi. Seconda Universita Degli Studi Di Napoli; Italia Fil: Kellner, Ulrich. No especifíca; Fil: Klaver, Caroline C. W.. Radboud University Nijmegen Medical Centre; Países Bajos Fil: Kousal, Bohdan. Charles University and General University Hospital; República Checa Fil: Lamey, Tina M.. University of Western Australia; Australia Fil: MacDonald, Ian M.. University of Alberta; Canadá Fil: Matynia, Anna. University of California at Los Angeles. School of Medicine; Estados Unidos Fil: McLaren, Terri L.. University of Western Australia; Australia Fil: Mena, Marcela D.. Consejo Nacional de Investigaciones Científicas y Técnicas. Oficina de Coordinación Administrativa Parque Centenario. Instituto de Investigaciones Bioquímicas de Buenos Aires. Fundación Instituto Leloir. Instituto de Investigaciones Bioquímicas de Buenos Aires; Argentina Fil: Meunier, Isabelle. Université Montpellier II; Francia Fil: Miller, Rianne. Radboud University Nijmegen Medical Centre; Países Bajos Fil: Newman, Hadas. Universitat Tel Aviv; Israel Fil: Ntozini, Buhle. University of Cape Town; Sudáfrica Fil: Oldak, Monika. No especifíca; Fil: Pieterse, Marc. Radboud University Nijmegen Medical Centre; Países Bajos Fil: Podhajcer, Osvaldo Luis. Consejo Nacional de Investigaciones Científicas y Técnicas. Oficina de Coordinación Administrativa Parque Centenario. Instituto de Investigaciones Bioquímicas de Buenos Aires. Fundación Instituto Leloir. Instituto de Investigaciones Bioquímicas de Buenos Aires; Argentina Fil: Puech, Bernard. Universite Lille; Francia Fil: Ramesar, Raj. University of Cape Town; Sudáfrica Fil: Rüther, Klaus. No especifíca; Fil: Salameh, Manar. No especifíca; Fil: Salles, Mariana Vallim. Universidade de Sao Paulo; Brasil Fil: Sharon, Dror. The Hebrew University of Jerusalem; Israel Fil: Simonelli, Francesca. Seconda Universita Degli Studi Di Napoli; Italia Fil: Spital, Georg. No especifíca; Fil: Steehouwer, Marloes. Radboud University Nijmegen Medical Centre; Países Bajos Fil: Szaflik, Jacek P.. No especifíca; Fil: Thompson, Jennifer A.. No especifíca; Fil: Thuillier, Caroline. Universite Lille; Francia Fil: Tracewska, Anna M.. No especifíca; Fil: van Zweeden, Martine. Radboud University Nijmegen Medical Centre; Países Bajos Fil: Vincent, Andrea L.. University of Auckland; Nueva Zelanda Fil: Zanlonghi, Xavier. No especifíca; Fil: Liskova, Petra. Charles University and General University Hospital; República Checa Fil: Stöhr, Heidi. Universitat Regensburg; Alemania Fil: De Roach, John N.. University of Western Australia; Australia Fil: Ayuso, Carmen. Hospital Universitario Fundación Jiménez Díaz; España Fil: Roberts, Lisa. University of Cape Town; Sudáfrica Fil: Weber, Bernhard H. F.. Universitat Regensburg; Alemania Fil: Dhaenens, Claire Marie. Universite Lille; Francia Fil: Cremers, Frans P. M.. Radboud University Nijmegen Medical Centre; Países Bajos

PURPOSE: ABCA4-associated disease, a recessive retinal dystrophy, is hallmarked by a large proportion of patients with only one pathogenic ABCA4 variant, suggestive for missing heritability. METHODS: By locus-specific analysis of ABCA4, combined with extensive functional studies, we aimed to unravel the missing alleles in a cohort of 67 patients (p), with one (p = 64) or no (p = 3) identified coding pathogenic variants of ABCA4. RESULTS: We identified eight pathogenic (deep-)intronic ABCA4 splice variants, of which five are novel and six structural variants, four of which are novel, including two duplications. Together, these variants account for the missing alleles in 40.3% of patients. Furthermore, two novel variants with a putative cis-regulatory effect were identified. The common hypomorphic variant c.5603A>T p.(Asn1868Ile) was found as a candidate second allele in 43.3% of patients. Overall, we have elucidated the missing heritability in 83.6% of our cohort. In addition, we successfully rescued three deep-intronic variants using antisense oligonucleotide (AON)-mediated treatment in HEK 293-T cells and in patient-derived fibroblast cells. CONCLUSION: Noncoding pathogenic variants, novel structural variants, and a common hypomorphic allele of the ABCA4 gene explain the majority of unsolved cases with ABCA4-associated disease, rendering this retinopathy a model for missing heritability in autosomal recessive disorders. ispartof: GENETICS IN MEDICINE vol:21 issue:8 pages:1761-1771 ispartof: location:United States status: published

Purpose: Using exome sequencing, the underlying variants in many persons with autosomal recessive diseases remain undetected. We explored autosomal recessive Stargardt disease (STGD1) as a model to identify the missing heritability.Methods: Sequencing of ABCA4 was performed in 8 STGD1 cases with one variant and p.Asn1868Ile in trans, 25 cases with one variant, and 3 cases with no ABCA4 variant. The effect of intronic variants was analyzed using in vitro splice assays in HEK293T cells and patient-derived fibroblasts. Antisense oligonucleotides were used to correct splice defects.Results: In 24 of the probands (67%), one known and five novel deep-intronic variants were found. The five novel variants resulted in messenger RNA pseudoexon inclusions, due to strengthening of cryptic splice sites or by disrupting a splicing silencer motif. Variant c.769-784C>T showed partial insertion of a pseudoexon and was found in cis with c.5603A>T (p.Asn1868Ile), so its causal role could not be fully established. Variant c.4253+43G>A resulted in partial skipping of exon 28. Remarkably, antisense oligonucleotides targeting the aberrant splice processes resulted in (partial) correction of all splicing defects.Conclusion: Our data demonstrate the importance of assessing noncoding variants in genetic diseases, and show the great potential of splice modulation therapy for deep-intronic variants.

Mutations in rhodopsin gene (RHO) are a frequent cause of retinitis pigmentosa (RP) and less often, congenital stationary night blindness (CSNB). Mutation p.G90D has previously been associated with CSNB based on the examination of one family. This study screened 60 patients. Out of these 60 patients, 32 were affected and a full characterization was conducted in 15 patients. We described the clinical characteristics of these 15 patients (12 male, median age 42 years, range 8–71) from three families including visual field (Campus Goldmann), fundus autofluorescence (FAF), optical coherence tomography (OCT) and electrophysiology. Phenotypes were classified into four categories: CSNB (N = 3, 20%) sector RP (N = 3, 20%), pericentral RP (N = 1, 6.7%) and classic RP (N = 8, 53.3% (8/15)). The phenotypes were not associated with family, sex or age (Kruskal–Wallis, p &gt, 0.05), however, cystoid macular edema (CME) was observed only in one family. Among the subjects reporting nyctalopia, 69% (22/32) were male. The clinical characteristics of the largest p.G90D cohort so far showed a large frequency of progressive retinal degeneration with 53.3% developing RP, contrary to the previous report.

Sveučilište Sjever jedina je visokoškolska institucija u RH koja se uključila u Program energetske obnove zgrada javnog sektora u razdoblju 2014. – 2020. Sljedeći korak nakon cjelovite obnove jest istraživanje parametara ugode u prostoru te je u radu opisan način na koji će se uz potrošnju energije pratiti temperatura, relativna vlažnost, razina ugljikova dioksida prisutnog u prostoru, te hlapljivi organski spojevi (VOC) u prostoru. Na temelju toga analizirat će se ostvarenje zajamčenih ušteda i procijeniti potencijal za dodatne uštede.

Sveučilište Sjever je jedina visokoškolska institucija u RH koje se uključila u Program energetske obnove zgrada javnog sektora u razdoblju 2014-2020. Nakon provedene cjelovite obnove, započeto je istraživanje parametara ugode u prostoru, te su u ovom radu opisani načini na koji se uz potrošnju energije prati temperatura, RH, CO2 i VOC u prostoru. U radu su prikazani i prvi rezultati analize navedenih parametara, kao i utjecaj ponašanja korisnika na potrošnju energije. Navedeno će se promatrati u cilju unaprjeđenja postojećeg modela ugovaranja po energetskom učinku (ESCO model).

Prisotnost barvil v odpadni vodi je nesprejemljiva tako iz estetskega kot tudi iz ekološkega stališča. Problematika prvega izhaja iz dejstva, da so odpadne vode, ki vsebujejo barvila, intenzivno obarvane že pri zelo nizkih koncentracijah barvil. Bolj pereča kot estetska je okoljska problematika barvil v odpadni vodi. Objektivno tveganje, ki ga predstavljajo za okolje, je odvisno od strukturnih in fizikalnih karakteristik barvil, njihove koncentracije ter časa izpostavitve v okolju. V okviru predmeta se prouči razpoložljive tehnologije čiščenja obarvanih vod, kar pa kaže na to, da ni enostavnih, učinkovitih in hkrati cenovno sprejemljivih načinov čiščenja tako kompleksnih odpadnih vod, kot so obarvane odpadne vode.

Vaje »Sodobni postopki barvanja in barva« obravnavajo sistematiko in nomenklaturo barvil, fizikalno kemijo barvalnih procesov ter uporabo naprednih materialov in tehnik pri procesu barvanja. Največji del je posvečen posebnim postopkom, ki omogočajo sodoben način barvanja ob istočasnem doseganju višjega nivoja kakovosti tako v smislu ekologije, kot v doseganju več funkcionalnih lastnosti materialov in barvanju z naravnimi barvili. Postopki, ki jih posebej proučujemo so barvanje pri pogojih super kritičnega CO2, barvanje ob souporabi ultrazvoka ali predhodne plazma obdelave in barvanje s funkcionalnimi barvil. Ta vrsta barvil nam omogoča posebnosti kot so: termokromija, fotokromija, elektrokromija, solvatokromija in ostale učinke povezane z uporabo teh barvil.

Simple Summary Gelsolin amyloidosis is a rare autosomal dominant genetic disease, which typically affects the cornea, skin and sometimes other organ systems and is caused by mutations in a gene coding for gelsolin protein (GSN). We describe a novel mutation of GSN gene, p.Glu580Lys, associated with gelsolin amyloidosis in six members of a two-generation family, who exhibited lattice corneal dystrophy, loose facial skin and irregular heart rhythm. In one patient we reported optic nerve impairment, which is possibly a novel feature associated with gelsolin amyloidosis. Abstract Gelsolin amyloidosis typically presents with corneal lattice dystrophy and is most frequently associated with pathogenic GSN variant p.Asp214Asn. Here we report clinical and histopathological features of gelsolin amyloidosis associated with a novel GSN variant p.Glu580Lys. We studied DNA samples of seven members of a two-generation family. Exome sequencing was performed in the proband, and targeted Sanger sequencing in the others. The heterozygous GSN variant p.Glu580Lys was identified in six patients. The patients exhibited corneal dystrophy (5/6), loose skin (5/6) and/or heart arrhythmia (3/6) and one presented with bilateral optic neuropathy. The impact of the mutation on the protein structure was evaluated in silico. The substitution is located in the fifth domain of gelsolin protein, homologous to the second domain harboring the most common pathogenic variant p.Asp214Asn. Structural investigation revealed that the mutation might affect protein folding. Histopathological analysis showed amyloid deposits in the skin. The p.Glu580Lys is associated with corneal dystrophy, strengthening the association of the fifth domain of gelsolin protein with the typical amyloidosis phenotype. Furthermore, optic neuropathy may be related to the disease and is essential to identify before discussing corneal transplantation.

The aim of this research was to apply three different types of zeolites and the combination thereof in the form of a very fine powder, together with different chemicals and additives on polyamide knitted fabric according to an industrially acceptable exhaustion procedure in order to study changes in the morphology, optical properties and wettability of surfaces. Zeolites were analysed using Scanning Electron Microscopy (SEM), Fourier Transform Infrared Spectroscopy (FTIR) and gas physiosorption. Additionally, the morphology of zeolite-coated surfaces was examined closely using SEM, while changes in molecular-chemical level were examined by means of IR spectroscopy. Optical properties were studied using CIE colour measurement and diff use reflectance profi le determination, while the hydrophilic/hydrophobic character was examined using goniometry. The obtained results show the suitability of the employed exhaustion procedure, depending on the type of zeolite and the composition of the treatment bath. The results also provided evidence of the enhanced wettability of PA fabrics using 4A and 13X zeolites in combination with selected additives.

Background Breast augmentation in trans-women requires special consideration and thorough preoperative planning. Objectives This study aimed to present our long-term outcome and experience gained over the past 21 years. Methods Trans-women who have undergone breast augmentation since 1995 were reviewed for primary surgery, type of incision, implant site, implant size and shape, and revisions. Results A total of 138 patients with a follow-up of 4.6 years (range, 2.0-13.3) were included. In 112 patients (82.4%), the inframammary incision was chosen. Subpectoral implantation was performed in 70 patients (51.5%). Round breast implants (91 patients, 66.9%, P = 0.010) with low projection (103 patients, 75.7%, P < 0.001) were favored. The implant size used during 2011-2016 of 363.3 ± 97.3 cc was significantly increased as compared to the implant size reported during 1995-1999 (mean difference, +142.1 cc, P < 0.001) and 2000-2004 (mean difference, +113.5 cc, P < 0.001). Simultaneously, revision rates dropped significantly over time (52.9% during 1995-1999 as compared to 6.9% during 2011-2016, P < 0.001). Overall, request for larger implants was the most common indication for revision (13 patients, 9.4%) after 6 months (range, 4.0-18.7). At revision, implant volume was 355.4 ± 132.8 cc, showing significant increase of 107.0 ± 48.1 cc as compared to primary implants (+30.0%, P = 0.048). Conclusions Request for larger breast implants was the most common reason for revision. Depending on the various degrees of breast tissue growth due to hormonal therapy, whenever applicable, we recommend round, low projection implants with a mean size of 360 cc in the prepectoral pocket. Level of Evidence: 4

Purpose: Usher syndrome (USH) is a multisensory deficiency involving vision, hearing and the vestibular system. The purpose of this study is to report on the functional data (i.e. electroretinography, visual fields, visual acuity) of patients with retinitis pigmentosa (RP) due to Usher syndrome that were collected in a multicentre European study (TREATRUSH). Methods: A total of 268 genetically confirmed USH patients underwent electrophysiological examinations in the context of multimodal ophthalmological examination in the study (75 USH1, 189 USH2 and four USH3). Full-field electroretinography (ERG) was performed according to ISCEV standards, visual field determination was carried out with either the Octopus or Goldmann perimeters and visual acuity was examined with either ETDRS or Snellen charts. The data were compared between USH subtypes (USH1/USH2/USH3) and correlated with age. Results: Visual acuity decreases significantly with age for both USH1 and USH2 (p < 0.001), without a difference between the two cohorts. When corrected for age, the preserved kinetic visual field was significantly larger in USH2 than in USH1 (p = 0.04). Furthermore, the preserved kinetic visual field area showed a significant decrease with age (based on an exponential fit) in both USH1 and USH2 (p < 0.001). In USH1 patients, however, the visual field was already vastly reduced at an early age. The ERG results were abnormal in all patients. Detectable data for scotopic ERG were obtained from nine patients, and data of photopic ERG were obtained from 24 patients, without a difference between USH1 and USH2 subtypes. Conclusions: There are differences in the phenotypes of RP in USH subtypes, most visible in the progression of visual fields between USH1 and USH2. The perimetric reduction occurs earlier in USH1 than in USH2. In both subtypes, visual acuity decreases significantly with age and the ERG is not detectable already at early ages. Purpose: Usher syndrome (USH) is a multisensory deficiency involving vision, hearing and the vestibular system. The purpose of this study is to report on the functional data (i.e. electroretinography, visual fields, visual acuity) of patients with retinitis pigmentosa (RP) due to Usher syndrome that were collected in a multicentre European study (TREATRUSH). Methods: A total of 268 genetically confirmed USH patients underwent electrophysiological examinations in the context of multimodal ophthalmological examination in the study (75 USH1, 189 USH2 and four USH3). Full-field electroretinography (ERG) was performed according to ISCEV standards, visual field determination was carried out with either the Octopus or Goldmann perimeters and visual acuity was examined with either ETDRS or Snellen charts. The data were compared between USH subtypes (USH1/USH2/USH3) and correlated with age. Results: Visual acuity decreases significantly with age for both USH1 and USH2 (p < 0.001), without a difference between the two cohorts. When corrected for age, the preserved kinetic visual field was significantly larger in USH2 than in USH1 (p = 0.04). Furthermore, the preserved kinetic visual field area showed a significant decrease with age (based on an exponential fit) in both USH1 and USH2 (p < 0.001). In USH1 patients, however, the visual field was already vastly reduced at an early age. The ERG results were abnormal in all patients. Detectable data for scotopic ERG were obtained from nine patients, and data of photopic ERG were obtained from 24 patients, without a difference between USH1 and USH2 subtypes. Conclusions: There are differences in the phenotypes of RP in USH subtypes, most visible in the progression of visual fields between USH1 and USH2. The perimetric reduction occurs earlier in USH1 than in USH2. In both subtypes, visual acuity decreases significantly with age and the ERG is not detectable already at early ages.

Background The worldwide spread of a novel coronavirus disease (COVID-19) has led to a near total stop of non-urgent, elective surgeries across all specialties in most affected countries. In the field of aesthetic surgery, the self-imposed moratorium for all aesthetic surgery procedures recommended by most international scientific societies has been adopted by many surgeons worldwide and resulted in a huge socioeconomic impact for most private practices and clinics. An important question still unanswered in most countries is when and how should elective/aesthetic procedures be scheduled again and what kind of organizational changes are necessary to protect patients and healthcare workers when clinics and practices reopen. Defining manageable, evidence-based protocols for testing, surgical/procedural risk mitigation and clinical flow management/contamination management will be paramount for the safety of non-urgent surgical procedures. Methods We conducted a MEDLINE/PubMed research for all available publications on COVID-19 and surgery and COVID-19 and anesthesia. Articles and referenced literature describing possible procedural impact factors leading to exacerbation of the clinical evolution of COVID-19-positive patients were identified to perform risk stratification for elective surgery. Based on these impact factors, considerations for patient selection, choice of procedural complexity, duration of procedure, type of anesthesia, etc., are discussed in this article and translated into algorithms for surgical/anesthesia risk management and clinical management. Current recommendations and published protocols on contamination control, avoidance of cross-contamination and procedural patient flow are reviewed. A COVID-19 testing guideline protocol for patients planning to undergo elective aesthetic surgery is presented and recommendations are made regarding adaptation of current patient information/informed consent forms and patient health questionnaires. Conclusion The COVID-19 crisis has led to unprecedented challenges in the acute management of the crisis, and the wave only recently seems to flatten out in some countries. The adaptation of surgical and procedural steps for a risk-minimizing management of potential COVID-19-positive patients seeking to undergo elective aesthetic procedures in the wake of that wave will present the next big challenge for the aesthetic surgery community. We propose a clinical algorithm to enhance patient safety in elective surgery in the context of COVID-19 and to minimize cross-contamination between healthcare workers and patients. New evidence-based guidelines regarding surgical risk stratification, testing, and clinical flow management/contamination management are proposed. We believe that only the continuous development and broad implementation of guidelines like the ones proposed in this paper will allow an early reintegration of all aesthetic procedures into the scope of surgical care currently performed and to prepare the elective surgical specialties better for a possible second wave of the pandemic. Level of Evidence V This journal requires that authors assign a level of evidence to each article. For a full description of these Evidence-Based Medicine ratings, please refer to the Table of Contents or the online Instructions to Authors www.springer.com/00266.

Cultural practices of exploring, revitalizing, interpreting and developing cultural heritage have become an important driving force in alleviating the social, cultural, environmental and economic challenges of today's world and in initiating sustainable progress in local communities. The interpretation of cultural uniqueness is not only in the hands of professionals from heritage institutions or research centres, but also in the hands of local residents, who are recognized as heritage bearers, cultural activists, practitioners and transmitters of heritage to the younger generations. The main objective of the model is to describe steps to achieve community involvement in cultural heritage projects and guidelines for carrying out the valorisation process with the active participation of the local population. The model outlines interesting and instructive case studies of how different interactive and "soft" methods can be used to build communities by following both top-down and bottom-up approaches. In addition, presented practices also show how to convince the younger generation that local heritage offers many opportunities to put bold ideas into practice.

X-linked retinopathies represent a significant proportion of monogenic retinal disease. They include progressive and stationary conditions, with and without syndromic features. Many are X-linked recessive, but several exhibit a phenotype in female carriers, which can help establish diagnosis and yield insights into disease mechanisms. The presence of affected carriers can misleadingly suggest autosomal dominant inheritance. Some disorders (such as RPGR-associated retinopathy) show diverse phenotypes from variants in the same gene and also highlight limitations of current genetic sequencing methods. X-linked disease frequently arises from loss of function, implying potential for benefit from gene replacement strategies. We review X-inactivation and X-linked inheritance, and explore burden of disease attributable to X-linked genes in our clinically and genetically characterised retinal disease cohort, finding correlation between gene transcript length and numbers of families. We list relevant genes and discuss key clinical features, disease mechanisms, carrier phenotypes and novel experimental therapies. We consider in detail the following: RPGR (associated with retinitis pigmentosa, cone and cone-rod dystrophy), RP2 (retinitis pigmentosa), CHM (choroideremia), RS1 (X-linked retinoschisis), NYX (complete congenital stationary night blindness (CSNB)), CACNA1F (incomplete CSNB), OPN1LW/OPN1MW (blue cone monochromacy, Bornholm eye disease, cone dystrophy), GPR143 (ocular albinism), COL4A5 (Alport syndrome), and NDP (Norrie disease and X-linked familial exudative vitreoretinopathy (FEVR)). We use a recently published transcriptome analysis to explore expression by cell-type and discuss insights from electrophysiology. In the final section, we present an algorithm for genes to consider in diagnosing males with non-syndromic X-linked retinopathy, summarise current experimental therapeutic approaches, and consider questions for future research.

Breast augmentation surgery is one of the crucial procedures in male-to-female gender reassignment surgery. It is performed by implantation of silicone prostheses and/or lipofilling and can, if requested, be well integrated within the vaginoplasty surgical session, hence avoiding further anesthesia. This procedure, however, remains challenging as there are fundamental anatomical landmarks and differences between trans woman and cis woman chest. This is particularly relevant in assessing the thoracic proportions, breast and nipple positioning, their forms and sizes, as well as the synchrony with the rest of the body. Furthermore, the treatment should be personalized, addressing the patient’s individual desires and significantly contributing to the healing process of gender dysphoria.