Diet therapy and metabolic control among Chilean adults with a neonatal diagnosis of Phenylketonuria

Abstract Phenylketonuria (PKU) is an autosomal recessive defect affecting the metabolic pathway of phenylalanine (Phe), causing hyperphenylalaninemia and neurotoxicity. Diagnosis must occur in the neonatal period and treatment should begin as early as possible. Evidence implies that treatment adherence declines as age advances. The aim was to describe the diet of a subgroup of Chilean adults with PKU currently in follow-up. Fifty-three subjects (49% women) followed up between January 2021 to April 2023 were considered. The concentration of Phe (PheC) in dried blood spots measured by fluorometry and 24-hour dietary recalls were analyzed. The median PheC of the sample was 438µmol/L (interquartile range(IQR):351-585µmol/L). A protein intake of 1.35±0.3 gr/Kg/d was observed of which 87% came from the protein substitute without Phe. Participants had a median Phe intake of 459mg/d (IQR:327-976) and 13.1g/d of fiber intake. Most participants, 51% and 92% reported consuming fruits and vegetables, respectively, and 32% consumed Low-Protein foods. Regarding micronutrients, all participants exceeded 90% adequacy according to recommendations. For vitamin-D and vitamin-B12, 100% is provided by the protein substitute. According to our results, it is mandatory to establish transition programs toward adulthood, to constantly maintain good metabolic control, and to adapt diet therapy to their new lifestyle.

Resultados de un programa de descolonización de Staphylococcus aureus en cirugía protésica primaria de cadera y rodilla / Results of a preoperative screening and decolonization program for Staphylococcus aureus in primary hip and knee arthroplasty

Introducción: La detección y descolonización del Staphylococcus aureus previo a la cirugía, se postula como la opción para disminuir el riesgo de infección en artroplastias. El objetivo de este estudio fue evaluar la efectividad de un programa de cribado de S. aureus en la artroplastia total de rodilla (ATR) y en la artroplastia total de cadera (ATC), la incidencia de infección respecto a una cohorte histórica y su viabilidad económica. Material y métodos: Estudio pre-postintervención en pacientes intervenidos de ATR y ATC en al año 2021. Se realizó protocolo de detección de colonización nasal por S. aureus y erradicación si procedía, con mupirocina intranasal, cultivo postratamiento con el resultado de 3 semanas entre cultivo postratamiento y cirugía. Se evalúan medidas de eficacia, se analizan costes y se comprara la incidencia de infección respecto a una serie histórica de pacientes intervenidos entre enero y diciembre de 2019, realizando análisis estadístico descriptivo y comparativo. Resultados: Los grupos fueron comparables estadísticamente. Se realizó el cultivo en el 89%, siendo 19 (13%) pacientes positivos. Se confirmó el tratamiento en 18, cultivo control en 14, todos descolonizados; ninguno sufrió infección. Un paciente con cultivo negativo sufrió infección por S. epidermidis. En cohorte histórica: 3 sufrieron infección profunda por S. epidermidis, E. cloacae y S. aureus. El coste del programa fue de 1.661,85€. Conclusión: El programa de cribado detectó el 89% de los pacientes. La prevalencia de infección en el grupo intervención era menor que en la cohorte, siendo S.epidermidis el microorganismo causante, diferente a S. aureus descrito en la literatura y en la cohorte. Consideramos que este programa es económicamente viable, siendo sus costes reducidos y asumibles.(AU)

Introduction: Detection and decolonization of Staphylococcus aureus prior to surgery is postulated as an option to reduce the risk of infection in arthroplasties. The aim of this study was to evaluate the effectiveness of a screening program for S. aureus in total knee arthroplasty (TKA) and total hip arthroplasty (THA), the incidence of infection with respect to a historical cohort, and its economic viability. Material and methods: Pre-post intervention study in patients undergoing primary knee and hip prostheses in 2021, a protocol was carried out to detect nasal colonization by S. aureus and eradication if appropriate, with intranasal mupirocin, post-treatment culture with results three weeks between post-treatment culture and surgery. Efficacy measures are evaluated, costs are analyzed and the incidence of infection is compared with respect to a historical series of patients operated on between January and December 2019, performing a descriptive and comparative statistical analysis. Results: The groups were statistically comparable. Culture was performed in 89%, with 19 (13%) positive patients. Treatment was confirmed in 18, control culture in 14, all decolonized; none suffered infection. One culture-negative patient suffered from Staphylococcus epidermidis infection. In historical cohort: 3 suffered deep infection by S. epidermidis, Enterobacter cloacae, S. aureus. The cost of the program is €1661.85. Conclusion: The screening program detected 89% of the patients. The prevalence of infection in the intervention group was lower than in the cohort, with S. epidermidis being the main microorganism, different from S. aureus described in the literature and in the cohort. We believe that this program is economically viable, as its costs are low and affordable.(AU)

[Translated article] Results of a preoperative screening and decolonization programme for Staphylococcus aureus in primary hip and knee arthroplasty / Resultados de un programa de descolonización de Staphylococcus aureus en cirugía protésica primaria de cadera y rodilla

Introducción: La detección y descolonización del Staphylococcus aureus previo a la cirugía, se postula como la opción para disminuir el riesgo de infección en artroplastias. El objetivo de este estudio fue evaluar la efectividad de un programa de cribado de S. aureus en la artroplastia total de rodilla (ATR) y en la artroplastia total de cadera (ATC), la incidencia de infección respecto a una cohorte histórica y su viabilidad económica. Material y métodos: Estudio pre-postintervención en pacientes intervenidos de ATR y ATC en al año 2021. Se realizó protocolo de detección de colonización nasal por S. aureus y erradicación si procedía, con mupirocina intranasal, cultivo postratamiento con el resultado de 3 semanas entre cultivo postratamiento y cirugía. Se evalúan medidas de eficacia, se analizan costes y se comprara la incidencia de infección respecto a una serie histórica de pacientes intervenidos entre enero y diciembre de 2019, realizando análisis estadístico descriptivo y comparativo. Resultados: Los grupos fueron comparables estadísticamente. Se realizó el cultivo en el 89%, siendo 19 (13%) pacientes positivos. Se confirmó el tratamiento en 18, cultivo control en 14, todos descolonizados; ninguno sufrió infección. Un paciente con cultivo negativo sufrió infección por S. epidermidis. En cohorte histórica: 3 sufrieron infección profunda por S. epidermidis, E. cloacae y S. aureus. El coste del programa fue de 1.661,85€. Conclusión: El programa de cribado detectó el 89% de los pacientes. La prevalencia de infección en el grupo intervención era menor que en la cohorte, siendo S.epidermidis el microorganismo causante, diferente a S. aureus descrito en la literatura y en la cohorte. Consideramos que este programa es económicamente viable, siendo sus costes reducidos y asumibles.(AU)

Introduction: Detection and decolonization of Staphylococcus aureus prior to surgery is postulated as an option to reduce the risk of infection in arthroplasties. The aim of this study was to evaluate the effectiveness of a screening program for S. aureus in total knee arthroplasty (TKA) and total hip arthroplasty (THA), the incidence of infection with respect to a historical cohort, and its economic viability. Material and methods: Pre-post intervention study in patients undergoing primary knee and hip prostheses in 2021, a protocol was carried out to detect nasal colonization by S. aureus and eradication if appropriate, with intranasal mupirocin, post-treatment culture with results three weeks between post-treatment culture and surgery. Efficacy measures are evaluated, costs are analyzed and the incidence of infection is compared with respect to a historical series of patients operated on between January and December 2019, performing a descriptive and comparative statistical analysis. Results: The groups were statistically comparable. Culture was performed in 89%, with 19 (13%) positive patients. Treatment was confirmed in 18, control culture in 14, all decolonized; none suffered infection. One culture-negative patient suffered from Staphylococcus epidermidis infection. In historical cohort: 3 suffered deep infection by S. epidermidis, Enterobacter cloacae, S. aureus. The cost of the program is €1661.85. Conclusion: The screening program detected 89% of the patients. The prevalence of infection in the intervention group was lower than in the cohort, with S. epidermidis being the main microorganism, different from S. aureus described in the literature and in the cohort. We believe that this program is economically viable, as its costs are low and affordable.(AU)

Prioridades de investigación en términos de fluidoterapia perioperatoria y monitorización hemodinámica: encuesta de consenso Delphi del Subcomité de Fluidoterapia y Monitorización hemodinámica de la Sección de Hemostasia, Medicina Transfusional y Fluidoterapia (SHTF) de la Sociedad española de Anestesiología y Reanimación (SEDAR) / Research priorities in perioperative fluid therapy and hemodynamic monitoring: A Delphi Consensus Survey from the Fluid Therapy and Hemodynamic Monitoring Subcommittee of the Hemostasis, Transfusion Medicine and Fluid Therapy Section (SHTF) of the Spanish Society of Anesthesiology and Critical Care (SEDAR)

Antecedentes: La investigación sobre fluidoterapia y monitorización hemodinámica perioperatorias es difícil y costosa. Los objetivos del presente estudio fueron resumir y priorizar estas cuestiones, en orden de importancia investigadora. Métodos: Cuestionario estructurado electrónico Delphi a lo largo de tres rondas entre 30 expertos en fluidoterapia y monitorización hemodinámica identificados a través del Subcomité de Fluidoterapia y Monitorización hemodinámica de la Sección de Hemostasia, Medicina Transfusional y Fluidoterapia de la Sociedad Española de Anestesiología y Reanimación. Resultados: Se identificaron 77 cuestiones, que se clasificaron en orden de priorización. Las cuestiones se categorizaron en temas de cristaloides, coloides, monitorización hemodinámica y otros. Se categorizaron 31 cuestiones como de prioridad investigadora esencial, para determinar si los algoritmos de optimización hemodinámica intraoperatorios basados en el índice de predicción de hipotensión invasivo o no invasivo frente a otras estrategias de manejo podrían reducir la incidencia de complicaciones postoperatorias, así como si el uso de biomarcadores del estrés renal junto con un protocolo de fluidoterapia dirigido por objetivos podría reducir la estancia hospitalaria y la incidencia de insuficiencia renal aguda en pacientes adultos sometidos a cirugía no cardiaca, lográndose el más alto consenso. Conclusiones: El Subcomité de Fluidoterapia y Monitorización hemodinámica de la Sección de Hemostasia, Medicina Transfusional y Fluidoterapia de la Sociedad Española de Anestesiología y Reanimación utilizará estos resultados para la realización de investigación.(AU)

Background: Research on perioperative fluid therapy and hemodynamic monitoring is difficult and expensive. The objectives of the present study were to summarize and prioritize these issues, in order of research importance. research importance. Methods: Structured electronic Delphi questionnaire over three rounds among 30 experts in fluid therapy and hemodynamic monitoring identified through the Subcommittee on Fluid Therapy and Hemodynamic Monitoring Subcommittee of the Hemostasis, Transfusion Medicine and Fluid Therapy Section of the Spanish Society of Anesthesiology and Resuscitation. Results: 77 issues were identified, which were ranked in order of prioritization. The were categorized into crystalloid, colloid, hemodynamic monitoring and others. and others. Thirty-one issues were categorized as of essential research priority, to determine whether intraoperative hemodynamic optimization algorithms based on the index of invasive or noninvasive hypotension prediction index versus other management strategies could reduce the incidence of postoperative reduce the incidence of postoperative complications, as well as whether the use of biomarkers of renal stress in conjunction biomarkers of renal stress in conjunction with a goal-directed fluid therapy protocol could reduce hospital stay and the hospital stay and the incidence of acute renal failure in adult patients undergoing non-cardiac surgery, and whether the non-cardiac surgery, achieving the highest consensus. Conclusions: The Subcommittee on Fluid Therapy and Hemodynamic Monitoring of the Section of Hemostasis, Transfusion Medicine and Fluid Therapy of the Spanish Society of Anesthesiology and Resuscitation of the Spanish Society of Anesthesiology and Resuscitation will use these results for further research.(AU)

Research priorities in perioperative fluid therapy and hemodynamic monitoring: A Delphi Consensus Survey from the Fluid Therapy and Hemodynamic Monitoring Subcommittee of the Hemostasis, Transfusion Medicine and Fluid Therapy Section (SHTF) of the Spanish Society of Anesthesiology and Critical Care (SEDAR).

BACKGROUND: Research in fluid therapy and perioperative hemodynamic monitoring is difficult and expensive. The objectives of this study were to summarize these topics and to prioritize these topics in order of research importance. METHODS: Electronic structured Delphi questionnaire over three rounds among 30 experts in fluid therapy and hemodynamic monitoring identified through the Fluid Therapy and Hemodynamic Monitoring Subcommittee of the Hemostasis, Transfusion Medicine and Fluid Therapy Section of the Spanish Society of Anesthesiology and Critical Care. RESULTS: 77 topics were identified and ranked in order of prioritization. Topics were categorized into themes of crystalloids, colloids, hemodynamic monitoring and others. 31 topics were ranked as essential research priority. To determine whether intraoperative hemodynamic optimization algorithms based on the invasive or noninvasive Hypotension Prediction Index versus other management strategies could decrease the incidence of postoperative complications. As well as whether the use of renal stress biomarkers together with a goal-directed fluid therapy protocol could reduce hospital stay and the incidence of acute kidney injury in adult patients undergoing non-cardiac surgery, reached the highest consensus. CONCLUSIONS: The Fluid Therapy and Hemodynamic Monitoring Subcommittee of the Hemostasis, Transfusion Medicine and Fluid Therapy Section of the Spanish Society of Anesthesiology and Critical Care will use these results to carry out the research.

[Translated article] Results of a preoperative screening and decolonization programme for Staphylococcus aureus in primary hip and knee arthroplasty.

INTRODUCTION: Detection and decolonization of Staphylococcus aureus prior to surgery is postulated as an option to reduce the risk of infection in arthroplasties. The aim of this study was to evaluate the effectiveness of a screening programme for S. aureus in total knee arthroplasty (TKA) and total hip arthroplasty (THA), the incidence of infection with respect to a historical cohort, and its economic viability. MATERIAL AND METHODS: Pre-post intervention study in patients undergoing primary knee and hip prostheses in 2021, a protocol was carried out to detect nasal colonization by S. aureus and eradication if appropriate, with intranasal mupirocin, post-treatment culture with results three weeks between post-treatment culture and surgery. Efficacy measures are evaluated, costs are analyzed and the incidence of infection is compared with respect to a historical series of patients operated on between January and December 2019, performing a descriptive and comparative statistical analysis. RESULTS: The groups were statistically comparable. Culture was performed in 89%, with 19 (13%) positive patients. Treatment was confirmed in 18, control culture in 14, all decolonized; none suffered infection. One culture-negative patient suffered from Staphylococcus epidermidis infection. In historical cohort: three suffered deep infection by S. epidermidis, Enterobacter cloacae, Staphylococcus aureus. The cost of the programme is €1661.85. CONCLUSION: The screening programme detected 89% of the patients. The prevalence of infection in the intervention group was lower than in the cohort, with S. epidermidis being the main micro-organism, different from S. aureus described in the literature and in the cohort. We believe that this programme is economically viable, as its costs are low and affordable.

Implementation of a major trauma team. Analysis of activation and care times in patients admitted to the ICU.

OBJECTIVE: To analyze the factors associated with the activation of the severe trauma care team (STAT) in patients admitted to the ICU, to measure its impact on care times, and to analyze the groups of patients according to activation and level of anatomical involvement. DESIGN: Prospective cohort study of severe trauma admitted to the ICU. From June 2017 to May 2019. Risk factors for the activation of the STAT analysed with logistic regression and CART type classification tree. SETTING: Second level hospital ICU. PATIENTS: Patients admitted consecutively. INTERVENTIONS: No. MAIN VARIABLES OF INTEREST: STAT activation. Demographic variables. Injury severity (ISS), intentionality, mechanism, assistance times, evolutionary complications, and mortality. RESULTS: A total of 188 patients were admitted (46.8% of STAT activation), median age of 52 (37-64) years (activated 47 (27-62) vs not activated 55 (42-67) P = 0.023), males 84.0%. No difference in mortality according to activation. The logistic model finds as factors: care (16.6 (2.1-13.2)) and prehospital intubation (4.2 (1.8-9.8)) and severe lower extremity injury (4.4 (1.6-12.3)). Accidental fall (0.2 (0.1-0.6)) makes activation less likely. The CART model selects the type of trauma mechanism and can separate high and low energy trauma. CONCLUSIONS: Factors associated with STAT activation were prehospital care, requiring prior intubation, high-energy mechanisms, and severe lower extremity injuries. Shorter care times if activated without influencing mortality. We must improve activation in older patients with low-energy trauma and without prehospital care.

Results of a preoperative screening and decolonization program for Staphylococcus aureus in primary hip and knee arthroplasty. / Resultados de un programa de descolonización de Staphylococcus aureus en cirugía protésica primaria de cadera y rodilla.

INTRODUCTION: Detection and decolonization of Staphylococcus aureus prior to surgery is postulated as an option to reduce the risk of infection in arthroplasties. The aim of this study was to evaluate the effectiveness of a screening program for S. aureus in total knee arthroplasty (TKA) and total hip arthroplasty (THA), the incidence of infection with respect to a historical cohort, and its economic viability. MATERIAL AND METHODS: Pre-post intervention study in patients undergoing primary knee and hip prostheses in 2021, a protocol was carried out to detect nasal colonization by S. aureus and eradication if appropriate, with intranasal mupirocin, post-treatment culture with results three weeks between post-treatment culture and surgery. Efficacy measures are evaluated, costs are analyzed and the incidence of infection is compared with respect to a historical series of patients operated on between January and December 2019, performing a descriptive and comparative statistical analysis. RESULTS: The groups were statistically comparable. Culture was performed in 89%, with 19 (13%) positive patients. Treatment was confirmed in 18, control culture in 14, all decolonized; none suffered infection. One culture-negative patient suffered from Staphylococcus epidermidis infection. In historical cohort: 3 suffered deep infection by S. epidermidis, Enterobacter cloacae, S. aureus. The cost of the program is €1661.85. CONCLUSION: The screening program detected 89% of the patients. The prevalence of infection in the intervention group was lower than in the cohort, with S. epidermidis being the main microorganism, different from S. aureus described in the literature and in the cohort. We believe that this program is economically viable, as its costs are low and affordable.

Controlled release of growth factors using synthetic glycosaminoglycans in a modular macroporous scaffold for tissue regeneration.

Healthy regeneration of tissue relies on a well-orchestrated release of growth factors. Herein, we show the use of synthetic glycosaminoglycans for controlled binding and release of growth factors to induce a desired cellular response. First, we screened glycosaminoglycans with growth factors of interest to determine kon (association rate constant), koff (dissociation rate constant), and Kd (equilibrium rate constant). As proof-of-concept, we functionalized an elastin-like recombinamer (ELR) hydrogel with a synthetic glycosaminoglycan and immobilized fibroblast growth factor 2 (FGF2), demonstrating that human umbilical vein endothelial cells cultured on top of ELR hydrogel differentiated into tube-like structures. Taking this concept further, we developed a tunable macroporous ELR cryogel material, containing a synthetic glycosaminoglycan and FGF2 that showed increased blood vessel formation and reduced immune response compared to control when implanted in a subcutaneous mouse model. These results demonstrated the possibility for specific release of desired growth factors in/from a modular 3D scaffold in vitro and in vivo.

Biohybrid elastin-like venous valve with potential for in situ tissue engineering.

Chronic venous insufficiency (CVI) is a leading vascular disease whose clinical manifestations include varicose veins, edemas, venous ulcers, and venous hypertension, among others. Therapies targeting this medical issue are scarce, and so far, no single venous valve prosthesis is clinically available. Herein, we have designed a bi-leaflet transcatheter venous valve that consists of (i) elastin-like recombinamers, (ii) a textile mesh reinforcement, and (iii) a bioabsorbable magnesium stent structure. Mechanical characterization of the resulting biohybrid elastin-like venous valves (EVV) showed an anisotropic behavior equivalent to the native bovine saphenous vein valves and mechanical strength suitable for vascular implantation. The EVV also featured minimal hemolysis and platelet adhesion, besides actively supporting endothelialization in vitro, thus setting the basis for its application as an in situ tissue engineering implant. In addition, the hydrodynamic testing in a pulsatile bioreactor demonstrated excellent hemodynamic valve performance, with minimal regurgitation (<10%) and pressure drop (<5 mmHg). No stagnation points were detected and an in vitro simulated transcatheter delivery showed the ability of the venous valve to withstand the implantation procedure. These results present a promising concept of a biohybrid transcatheter venous valve as an off-the-shelf implant, with great potential to provide clinical solutions for CVI treatment.

Impact of aromatic residues on the intrinsic disorder and transitional behaviour of model IDPs.

Understanding the interplay between order and disorder in intrinsically disorder proteins (IDPs), and its impact on the properties and features of materials manufactured from them, is a major challenge in the design of protein-based synthetic polymers intended for advanced functions. In this paper an elastin-like diblock co-recombinamer amphiphile (Phe-ELR) based on a hydrophobic block containing five phenylalanine (Phe) residues proximal to the carboxyl function of a glutamic acid (Glu) residue upon folding, and with Glu as the guest residue in the hydrophilic part, was engineered and its assembly behaviour compared with another amphiphilic ELR used as control. Phe-ELR was tailored in order to clarify the impact of the presence of aromatic residues in the amino acid sequence, which even in early studies by Urry's group already demonstrated a certain out-of-trend behaviour compared with other apolar amino acids, especially non-aromatic ones, on ELR behaviour. The combination of several experimental techniques indicates strong molecular interactions associated with the Phe residue, thus resulting in limited reversible character of the temperature-induced transitions during sequential thermal cycles, a lower than expected transition enthalpy, and clear differences in its supramolecular assembly with respect to the control ELR. A distinctive pre-aggregated state for the Phe-ELR under any condition of pH and temperature is found. Eventually, this state gives rise to Phe-core micelles or a solid jelly-like material, depending on the concentration, pH and presence of salts. In conclusion, it appears that the presence of aromatic residues and their ability to promote strong inter- and intramolecular interactions at any temperature and pH causes a complete modification of the order-disorder interplay present in other, non-aromatic ELRs. These molecular events have a profound impact on the physical properties of the resulting polymer when compared with other ELRs. This work helps to shed light on the limits that govern intrinsic disorder in ELRs beyond its inverse temperature transition.

Carbon and nitrogen isotopic similarity between the endangered Darwin's fox (Lycalopex fulvipes) and sympatric free-ranging dogs in Chiloé Island, Chile.

Darwin's fox is an opportunistic omnivorous predator native to Chile classified as endangered by the IUCN Red List. Habitat use by Darwin's foxes can be negatively affected by the presence of free-ranging dogs that range freely across native and non-native habitats and can be a source of fox mortality. The objective of this study was to analyze the isotopic similarity of Darwin's fox and sympatric free-ranging dogs in Chiloé Island to determine the impact of anthropogenic environmental alterations on wild predators. We use hair samples to characterise and compare their δ13C and δ15N values and to evaluate isotopic similarity and isotope niches overlap. A generalised linear model was used to associate the isotope value with landscape variables (forest cover and vegetation type) and distance to the nearest house. We found no significant differences in δ13C or δ15N values between foxes and dogs, and a marginally significant isotope niche overlap (59.4 %). None of the selected variables at landscape and site scale were related to isotope values. Although our study is not a probe of direct contact between foxes and free-ranging dogs, the high isotopic similarity highlights the risk of pathogen spillover from free-ranging dogs to Darwin's foxes.

A serosurvey for spotted fever group Rickettsia and Coxiella burnetii antibodies in rural dogs and foxes, Chile.

Antibodies against Spotted Fever Group (SFG) Rickettsia and Coxiella burnetii, investigated through indirect antibody immunofluorescence tests, were detected in serum samples from 3.1% and 0% of 358 rural dogs, respectively, and in none of 32 wild foxes tested. SFG Rickettsia seropositive dogs were only detected in the Mountain Desert (8%) and the Steppe-Mediterranean (9%) regions. Exposure in the Mountain Desert, where no ticks and fleas were found on any dog, could correspond to a new SFG Rickettsia sp. recently described in soft ticks or to a related agent. Our survey confirms low endemicity in the country of C. burnetii, as observed in recent serosurveys in humans.

Assessing Progress Towards SDGs Implementation Using Multiple Reference Point Based Multicriteria Methods: The Case Study of the European Countries.

To achieve the UN 2030 Agenda Goals, and considering their complexity and multidisciplinary, Multi-criteria analysis appears to be a suitable approach to give a true support to public decision makers in defining policy lines. This study focuses on the application of the Multiple Reference Point Weak-Strong Composite Indicators (MRP-WSCI) and its partially compensatory version (MRP-PCI), to assess, in the framework of the UN 2030 Agenda, the sustainability of the 28 members of the European Union (pre-Brexit). Countries were analyzed and compared according to their conditions and progress against the 17 Sustainable Development Goals, considering three reference years: 2007, 2012 and 2017. The analysis shows that Nordic countries reach a good level of global sustainability, with values of the indicators, W-W-W and S-W-W, between 2 and 3; while the States of east Europe, in particular Romania, Bulgaria and Greece, stay at the worst levels, having overall indicators values less than 1.5. Furthermore, the results highlight how countries in the lower group have difficulties especially in social and economic sustainability. On the other hand, states with a good overall condition record the worst results in the environmental dimension, such as the Netherlands, which shows, for the year 2017, a value for this sphere less than 2, while in the other two show a good value (over 2.5).

Fast and reversible crosslinking of a silk elastin-like polymer.

Elastin-like polymers (ELPs) and their chimeric subfamily the silk elastin-like polymers (SELPs) exhibit a lower critical solvation temperature (LCST) behavior in water which has been extensively studied from theoretical, computational and experimental perspectives. The inclusion of silk domains in the backbone of the ELPs effects the molecular dynamics of the elastin-like domains in response to increased temperature above its transition temperature and confers gelation ability. This response has been studied in terms of initial and long-term changes in structures, however, intermediate transition states have been less investigated. Moreover, little is known about the effects of reversible hydration on the elastin versus silk domains in the physical crosslinks. We used spectroscopic techniques to analyze initial, intermediate and long-term states of the crosslinks in SELPs. A combination of thermoanalytical and rheological measurements demonstrated that the fast reversible rehydration of the elastin motifs adjacent to the relatively small silk domains was capable of breaking the silk physical crosslinks. This feature can be exploited to tailor the dynamics of these types of crosslinks in SELPs. STATEMENT OF SIGNIFICANCE: The combination of silk and elastin in a single molecule results in synergy via their interactions to impact the protein polymer properties. The ability of the silk domains to crosslink affects the thermoresponsive properties of the elastin domains. These interactions have been studied at early and late states of the physical crosslinking, while the intermediate states were the focus of the present study to understand the reversible phase-transitions of the elastin domains over the silk physical crosslinking. The thermoresponsive properties of the elastin domains at the initial, intermediate and late states of silk crosslinking were characterized to demonstrate that reversible hydration of the elastin domains influenced the reversibility of the silk crosslinks.

Topographically guided hierarchical mineralization.

Material platforms based on interaction between organic and inorganic phases offer enormous potential to develop materials that can recreate the structural and functional properties of biological systems. However, the capability of organic-mediated mineralizing strategies to guide mineralization with spatial control remains a major limitation. Here, we report on the integration of a protein-based mineralizing matrix with surface topographies to grow spatially guided mineralized structures. We reveal how well-defined geometrical spaces defined within the organic matrix by the surface topographies can trigger subtle changes in single nanocrystal co-alignment, which are then translated to drastic changes in mineralization at the microscale and macroscale. Furthermore, through systematic modifications of the surface topographies, we demonstrate the possibility of selectively guiding the growth of hierarchically mineralized structures. We foresee that the capacity to direct the anisotropic growth of such structures would have important implications in the design of biomineralizing synthetic materials to repair or regenerate hard tissues.

Antimicrobial activity of graphite oxide doped with silver against Bacillus subtilis, Candida albicans, Escherichia coli, and Staphylococcus aureus by agar well diffusion test: Synthesis and characterization.

In the present study, graphite oxide, graphite oxide doped with 0.8 g AgNO3 named GrO-0.5Ag, and graphite oxide doped with 1.25 g AgNO3 named GrO-1.0Ag (silver-doped graphitic materials) were synthesized by the modified Hummers and chemical doping methods. Subsequently, its antimicrobial activity against Bacillus subtilis, Candida albicans, Escherichia coli, and Staphylococcus aureus microorganisms was evaluated by agar well diffusion test with 1 and 2 mg·ml-1 of material concentrations. The silver-doped graphitic materials were characterized by X-Ray Diffraction, Fourier-Transform Infrared, Raman, X-Ray Photoelectron spectroscopies, and Scanning Electron Microscopy. The GrO-0.5Ag material showed a percentage of inhibition effect of 86, 82, 48, and 45% with respect to the positive control for Staphylococcus aureus, Bacillus subtilis, Escherichia coli, and Candida albicans microorganisms, respectively. Otherwise, the GrO-1.0Ag material showed a percentage of inhibition effect of 54% with respect to the positive control for Bacillus subtilis and less than 50% for the other microorganisms. The material concentrations showed a slight change in the percentage of inhibition effect for all microorganisms. Graphite oxide functional groups, small crystal size, free silver ions, and silver carbonate formation in silver-doped graphitic materials presented a viable alternative to inhibit bacterial growth.

25 Hydroxy Vitamin D Level, Bone Health, Vitamin D and Calcium Intake in Chilean Patients with Phenylketonuria and Hyperphenylalaninemias

Abstract It has been shown that there is a decrease in the concentrations of 25 hydroxyvitamin D (25-OHD) and bone mineral density (BMD) in patients with phenylketonuria (PKU) in their follow-up. Our objective was to determine concentrations of 25-OHD in subjects with PKU and hyperphenylalaninemia (HPA). Transversal analytical study considered three groups: G1-PKU with neonatal diagnosis and formula intake without Phe; G2-HPA, without specific treatment and G3-C control group. Sixteen patients per group (aged 6-23) were included. Levels of 25-OHD, lumbar spine (L2-L4), femur and total BMD, intact parathormone (PTH) and vitamin D (VitD) and calcium intake were calculated. The Kruskal-Wallis statistical test was applied (p-value<0,05). Significant differences were detected in concentrations of 25-OHD between G1-PKU and G2-HPA (38.9 ng/mL; 28 ng/mL, respectively) (NV: >30 ng/mL). G1-PKU had a higher intake of VitD, with differences among groups. There were no significant differences among groups in relation to BMD and intact PTH. In conclusion, G1-PKU under treatment and with good adherence, does not present VitD deficiency and no BMD alterations are observed. In contrast, G2-HPA had a lower intake of VitD and decreased 25-OHD concentrations which could affect the bone architecture in the long term. Further studies on the G2-HPA are suggested.

Update on Dietary Compliance, Nutritional Status, and Neuropsychological Functioning in the Chilean Phenylketonuria cohort

Abstract Since 1992, Chile has had a Newborn Screening Program for Phenylketonuria (PKU), which currently has an incidence of 1:18,916 newborns. The objective of the current study was to describe the 2020 follow up of the Chilean PKU cohort. The variables analyzed were: nutritional status, dietary compliance and neuropsychological functioning. We conducted a descriptive cross-sectional statistical analysis. The 271 subjects with PKU had an average age of diagnosis of 17±8 days and a phenylalanine (Phe) level of 1122±546 umol/L. Approximately 80% of protein requirement came from a protein substitute. For those <18 years of age, 80% had good dietary compliance with Phe level between 120-360 umol/L and those >18 years had a median of 522 umol/L (95%CI 468 - 636). Forty-four percent of the active PKU cohort had overweight/obesity. Eighty-five percent of the cohort >4 years of age had a normal intelligence quotient (IQ) (score 80-120). We observed a negative correlation (p <0.001; 95% CI: - 0.5, -0.2) between IQ score and Phe level. The Chilean protocol and protein substitute subsidy for life, together with the follow-up and continuous education carried out by the clinical team has encouraged compliance.

Antibiofilm coatings based on protein-engineered polymers and antimicrobial peptides for preventing implant-associated infections.

Implant-associated infections (IAIs) are one of the leading concerns in orthopedics and dentistry as they commonly lead to implant failure. The presence of biofilms and, increasingly frequently, drug-resistant bacteria further impairs the efficacy of conventional antibiotics. Immobilization of antimicrobial peptides (AMPs) on implant surfaces is a promising alternative to antibiotics for prevention of IAIs. In addition, the use of functional linkers for the AMP tethering enables to increase the antimicrobial potential and the bioactivities of the coating. In this study, an extracellular-matrix-mimicking system based on elastin-like recombinamers (ELRs) has been developed for the covalent anchoring of AMPs and investigated for use as a hybrid antibiofilm coating. A drip-flow biofilm reactor was used to simulate in vivo environmental dynamic conditions, thus showing that the presence of the AMPs in the hybrid coatings provided strong antibiofilm activity against monospecies and microcosm biofilm models of clinical relevance. These results, together with an excellent cytocompatibility towards primary gingival fibroblasts, encourage the use of ELRs as multivalent platforms for AMPs and open up a wide range of possibilities in the biofabrication of advanced coatings combining the antibiofilm potential of AMPs and the outstanding tunability and biomechanical properties of the ELRs.