Evaluation of poly(lactic acid) and ECOVIO based biocomposites loaded with antimicrobial sodium phosphate microparticles.

Herein, biobased composite materials based on poly(lactic acid) (PLA) and poly(butylene adipate-co-terephthalate) (PBAT) as matrices, sodium hexametaphosphate microparticles (E452i, food additive microparticles, 1 and 5 wt%) as antimicrobial filler and acetyl tributyl citrate (ATBC, 15 wt%) as plasticizer, were developed for potential food packaging applications. Two set of composite films were obtained by melt-extrusion and compression molding, i) based on PLA matrix and ii) based on Ecovio® matrix (PLA/PBAT blend). Thermal characterization by thermogravimetric analysis and differential scanning calorimetry demonstrated that the incorporation of E452i particles improved thermal stability and crystallinity, while the mechanical test showed an increase in the Young's modulus. E452i particles also provide antimicrobial properties to the films against food-borne bacteria Listeria innocua and Staphylococcus aureus, with bacterial reduction percentages higher than 50 % in films with 5 wt% of particles. The films also preserved their disintegradability as demonstrated by an exhaustive characterization of the films under industrial composting conditions. Therefore, the results obtained in this work reveal the potential of these biocomposites as appropriated materials for antibacterial and compostable food packaging films.

Synthesis and characterization of thiazolium chitosan derivative with enhanced antimicrobial properties and its use as component of chitosan based films.

In this work, chemical modification of chitosan using cationic thiazolium groups was investigated with the aim to improve water solubility and antimicrobial properties of chitosan. Enzymatic synthesis and ethyl-3-(3-dimethylaminopropyl) carbodiimide/N-hydroxysuccinimide (EDC/NHS) chemistry were employed to synthesize and attach to chitosan through the amine groups the molecule bearing thiazolium moieties, quaternized 4-(2-(4-methylthiazol-5-yl) ethoxy)-4-oxobutanoic acid (MTBAQ). On the basis of Fourier transform infrared spectroscopy (FTIR), elemental analysis and solid state nuclear magnetic resonance (ssNMR), around 95 % of the available amine groups of chitosan (of 25 % degree of acetylation) reacted. The resulting derivative was water soluble at physiological pH and exhibit excellent antimicrobial activity against Listeria innocua, Staphylococcus epidermidis, Staphylococcus aureus and Methicillin Resistant S. aureus Gram-positive bacteria (MIC = 8-32 µg/ mL), whereas its efficiency decreases against fungi Candida albicans and Eschericia coli Gram-negative bacterium. Subsequently, the thiazolium chitosan derivative was employed as antimicrobial component (up to 7 wt%) of chitosan/glycerol based films. The incorporation of the chitosan derivative does not modify significantly the characteristics of the film in terms of thermal and mechanical properties, while enhances considerably the antimicrobial activity.

PLA and PBAT-Based Electrospun Fibers Functionalized with Antibacterial Bio-Based Polymers.

Antimicrobial fibers based on biodegradable polymers, poly(lactic acid) (PLA), and poly(butylene adipate-co-terephthalate) (PBAT) are prepared by electrospinning. For this purpose, a biodegradable/bio-based polyitaconate containing azoles groups (PTTI) is incorporated at 10 wt.% into the electrospinning formulations. The resulting fibers functionalized with azole moieties are uniform and free of beads. Then, the accessible azole groups are subjected to N-alkylation, treatment that provides cationic azolium groups with antibacterial activity at the surface of fibers. The positive charge density, roughness, and wettability of the cationic fibers are evaluated and compared with flat films. It is confirmed that these parameters exert an important effect on the antimicrobial properties, as well as the length of the alkylating agent and the hydrophobicity of the matrix. The quaternized PLA/PTTI fibers exhibit the highest efficiency against the tested bacteria, yielding a 4-Log reduction against S. aureus and 1.7-Log against MRSA. Then, biocompatibility and bioactivity of the fibers are evaluated in terms of adhesion, morphology and viability of fibroblasts. The results show no cytotoxic effect of the samples, however, a cytostatic effect is appreciated, which is ascribed to the strong electrostatic interactions between the positive charge at the fiber surface and the negative charge of the cell membranes.

Estudio de utilización de linezolid en el Hospital Central de la Defensa «Gómez Ulla» / Study of the Linezolid usage at Hospital Central de la Defensa Gómez Ulla

El linezolid es un antimicrobiano del grupo de las oxazolidinonas, desarrollado en la década de 1990, siendo elprimero comercializado en su grupo a principios de los años 2000. Está indicado, según ficha técnica, en el tratamiento de la neumonía nosocomial y de la neumonía adquirida en la comunidad, cuando se conoce o se sospecha que está causada por bacteriasgram-positivas, y en el tratamiento de infecciones complicadas de piel y partes blandas (IPPBC) con certeza de microorganismosgram-positivos. Debido a su buena farmacocinética, rentabilidad e indicaciones anteriormente mencionadas ha cobrado especial importancia tanto en ámbito hospitalario como ambulatorio. Sujetos y métodos: Se llevó a cabo un estudio de utilizaciónde medicamentos (EUM) prescripción-indicación retrospectivo, en el Hospital Central de la Defensa (HCD) «Gómez Ulla»,durante el mes de septiembre de 2019, con el objetivo de conocer el patrón de uso de linezolid (indicaciones, adecuación o no aficha técnica, pauta posológica, vía de adminsitración utilizada, sospecha de reacciones adversas medicamentosas, características socio-demográficas y clínicas de los pacientes). El tipo de muestreo fue consecutivo no probabilístico. Se realizó un análisisestadístico descriptivo a través del programa informático SPSS 25.0. Resultados: Un total de 28 pacientes recibieron durante elmes de septiembre de 2019 al menos una dosis de linezolid. (AU)

Linezolid is an antimicrobial from the group of oxazolidinones, developed in the 1990s, being the first marketed inits group in the early 2000s. It is indicated, according to the data sheet, in the treatment of nosocomial pneumonia. and community-acquired pneumonia, when known or suspected to be caused by gram positive bacteria, and in the treatment of complicatedskin and soft tissue infections with certain gram positive organisms. Due to its good pharmacokinetics, profitability and aforementioned indications, it has gained special importance both in hospital and outpatient settings. Subjects and methods: A retrospectiveprescription-indication drug use study was carried out at the Central Defense Hospital (HCD) «Gómez Ulla», during the monthof September 2019, with the objective of knowing the pattern of use of linezolid (indications, adequacy or not to the data sheet,dosage regimen, route of administration used, suspected adverse drug reactions, sociodemographic and clinical characteristics ofthe patients). The type of test was non-probabilistic consecutive. A descriptive statistical analysis was performed using the SPSS 25.0computer program. Results: A total of 28 patients received at least one dose of linezolid during the month of September 2019. IPPBwas the most prevalent indication (53.5%) (15/28) followed by pneumonia (28.5%) (8/28). It was not adapted in 5 cases (17.8%) (5/28)to the indications of the data sheet: Complicated Urinary Tract Infection (3/5), Complicated Intra-abdominal Infection (1/5) andfebrile syndrome without source (1/5). (AU)

Electrospun Polylactic Acid-Based Fibers Loaded with Multifunctional Antibacterial Biobased Polymers.

Here, we report the development of antibacterial and compostable electrospun polylactic acid (PLA) fibers by incorporation of a multifunctional biobased polymer in the process. The multifunctional polymer was synthesized from the bio-sourced itaconic acid building block by radical polymerization followed by click chemistry reaction with hydantoin groups. The resulting polymer possesses triazole and hydantoin groups available for further N-alkylation and chlorination reaction, which provide antibacterial activity. This polymer was added to the electrospinning PLA solution at 10 wt %, and fiber mats were successfully prepared. The obtained fibers were surface-modified through the accessible functional groups, leading to the corresponding cationic triazolium and N-halamine groups. The fibers with both antibacterial functionalities demonstrated high antibacterial activity against Gram-positive and Gram-negative bacteria. While the fibers with cationic surface groups are only effective against Gram-positive bacteria (Staphylococcus epidermidis and Staphylococcus aureus), upon chlorination, the activity against Gram-negative Escherichia coli and Pseudomonas aeruginosa is significantly improved. In addition, the compostability of the electrospun fibers was tested under industrial composting conditions, showing that the incorporation of the antibacterial polymer does not impede the disintegrability of the material. Overall, this study demonstrates the feasibility of this biobased multifunctional polymer as an antibacterial agent for biodegradable polymeric materials with potential application in medical uses.

Patients with Parkinson's Disease Show Alteration in their Visuospatial Abilities and in their Egocentric and Allocentric Spatial Orientation Measured by Card Placing Tests.

BACKGROUND: Visuospatial skills are impaired in Parkinson's disease (PD). Other related skills exist, such as spatial orientation have been poorly studied. The egocentric (based on internal cues) and allocentric frameworks (based on external cues) are used in daily spatial orientation. Depending on PD onset, the allocentric framework may have a higher level of impairment in tremor-dominant and the egocentric one in akinetic-rigid. OBJECTIVE: To evaluate spatial orientation and visuospatial functions in PD patients and controls, and to assess whether their performance is related to disease duration and the PD subtype (tremor-dominant and akinetic-rigid). METHODS: We evaluated egocentric and allocentric spatial orientation (Egocentric and Allocentric Spatial Memory Tasks) and visuospatial abilities, span and working memory in 59 PD patients and 51 healthy controls. RESULTS: Visuospatial skills, visuospatial span, and egocentric and allocentric orientation are affected in PD. Visuospatial skills and allocentric orientation undergo deterioration during the first 5 years of the disease progression, while egocentric orientation and visuospatial span do so at later stages (9-11 years). The akinetic-rigid subtype presents worse results in all the spatial abilities that were measured when compared to controls, and worse scores in visuospatial working memory, visuospatial abilities and allocentric orientation when compared to the tremor-dominant group. The tremor-dominant group performed worse than controls in egocentric and allocentric orientation. CONCLUSION: PD patients show deficits in their visuospatial abilities and in their egocentric and allocentric spatial orientation compared to controls, specifically in akinetic-rigid PD. Only spatial orientation are affected in tremor-dominant PD patients. Allocentric orientation is affected earlier in the progression of the disease.

Vertical cutoff methods in serum protein electrophoresis for the measurement of monoclonal protein concentrations: Which is best?

BACKGROUND: Monoclonal protein (M-protein) concentrations are measured by serum protein electrophoresis (SPE). Two methods are used for demarcating the M-protein area in the electropherogram: perpendicular drop (PD) and tangent skimming (TS). The aim of this study was tocompare both methods and to establish which is the most accurate and precise. METHODS: We studied 24 sera containing M-protein (5-44 g/L). The systematic error (SE) was evaluated in a dilution series of 12 sera. Within-day, between-day, and interobserver variability were assessed. SPE was performed by capillary and agarose gel electrophoresis. M-protein concentrations were measured using both cutoff methods. RESULTS: The PD method shows a constant SE ranged 1.00-2.27 g/L, while constant SE for TS is ranged -0.30--0.57 g/L. None of the cutoff methods or electrophoretic methods showed a proportional SE, with the exception of the TS method in capillary electrophoresis for ß-migrating M-protein. The PD method was more precise than the TS method in all three estimates of imprecision. An increased CV for concentrations < 10 g/L in between-day imprecision was observed with the TS method. Interobserver imprecision was greater for M-protein concentrations < 17 g/L for both cutoff methods (14.85%, 26.42% respectively). CONCLUSIONS: Despite being less precise, the TS method provides a more accurate measurement of M-protein concentration.

Hemolytic and Antimicrobial Activities of a Series of Cationic Amphiphilic Copolymers Comprised of Same Centered Comonomers with Thiazole Moieties and Polyethylene Glycol Derivatives.

A series of well-defined antimicrobial polymers composed of comonomers bearing thiazole ring (2-(((2-(4-methylthiazol-5-yl)ethoxy)carbonyl)oxy)ethyl methacrylate monomer (MTZ)) and non-hemotoxic poly(ethylene glycol) side chains (poly(ethylene glycol) methyl ether methacrylate (PEGMA)) were synthesized by reversible addition-fragmentation chain transfer (RAFT) polymerization. By post-polymerization functionalization strategy, polymers were quaternized with either butyl or octyl iodides to result in cationic amphiphilic copolymers incorporating thiazolium groups, thus with variable hydrophobic/hydrophilic balance associated to the length of the alkylating agent. Likewise, the molar percentage of PEGMA was modulated in the copolymers, also affecting the amphiphilicity. The antimicrobial activities of these cationic polymers were determined against Gram-positive and Gram-negative bacteria and fungi. Minimum inhibitory concentration (MIC) was found to be dependent on both length of the alkyl hydrophobic chain and the content of PEGMA in the copolymers. More hydrophobic octylated copolymers were found to be more effective against all tested microorganisms. The incorporation of non-ionic hydrophilic units, PEGMA, reduces the hydrophobicity of the system and the activity is markedly reduced. This effect is dramatic in the case of butylated copolymers, in which the hydrophobic/hydrophilic balance is highly affected. The hemolytic properties of polymers analyzed against human red blood cells were greatly affected by the hydrophobic/hydrophilic balance of the copolymers and the content of PEGMA, which drastically reduces the hemotoxicity. The copolymers containing longer hydrophobic chain, octyl, are much more hemotoxic than their corresponding butylated copolymers.

Tuberculosis miliar y tuberculoma coroideo en una niña de 3 meses: diagnóstico y seguimiento de un caso / Miliary tuberculosis and choroidal tuberculoma in a three-month old baby girl: Diagnosis and follow-up of a case

Se presenta a una niña de 3 meses de edad con un cuadro febril sin foco y con empeoramiento. La exploración fundoscópica mostró una lesión coroidea en el ojo izquierdo junto con lesiones en otros órganos sugestivas de tuberculosis diseminada. La evolución fue favorable tras tratamiento con cuádruple terapia antibiótica asociada a corticoterapia. La tuberculosis diseminada debe tenerse en cuenta ante un cuadro febril de tórpida evolución. La exploración oftalmológica de estos pacientes es fundamental, ya que, aunque es poco frecuente, los signos característicos de las uveítis posteriores nos orientarán hacia el diagnóstico y tratamiento precoz de esta enfermedad

A three-month old baby girl presented with fever of unknown origin and with signs of worsening of this episode. Funduscopy showed a solitary choroidal lesion in her left eye, as well as extraocular lesions suggesting disseminated tuberculosis. A favourable outcome was achieved after quadruple antibiotic course and cortisone therapy. Disseminated tuberculosis should be considered in cases of fever of unknown origin in children with an unsatisfactory evolution. Ocular examination is mandatory, due to the possible posterior uveitis signs that can help with early diagnosis and treatment of some diseases

Miliary tuberculosis and choroidal tuberculoma in a three-month old baby girl: Diagnosis and follow-up of a case. / Tuberculosis miliar y tuberculoma coroideo en una niña de 3 meses: diagnóstico y seguimiento de un caso.

A three-month old baby girl presented with fever of unknown origin and with signs of worsening of this episode. Funduscopy showed a solitary choroidal lesion in her left eye, as well as extraocular lesions suggesting disseminated tuberculosis. A favourable outcome was achieved after quadruple antibiotic course and cortisone therapy. Disseminated tuberculosis should be considered in cases of fever of unknown origin in children with an unsatisfactory evolution. Ocular examination is mandatory, due to the possible posterior uveitis signs that can help with early diagnosis and treatment of some diseases.

[Reliability and concurrent validity of the app Goniometer Pro vs Universal Goniometer in the determination of passive knee flexion]. / Fiabilidad y validez concurrente de la app Goniometer Pro vs goniómetro universal en la determinación de la flexión pasiva de rodilla.

BACKGROUND: Smartphones have become a fundamental part of our society, not only everyday, but also professional, since many applications have emerged for the health field. The objective of the study was to analyze the reliability and concurrent validity of an app for iPhone in relation to traditional measurement. MATERIAL AND METHODS: A non-experimental, double-blind validation and descriptive study. On a sample of 21 subjects, four measurements of knee mobility were performed. Two using universal goniometer (GU) and two through the application Goniometer-Pro (G-Pro) for IPhone. The X-ray was used as a reference measure of the knee flexion angle and was analyzed by a single evaluator who compared this result with that of the other evaluators. RESULTS: The difference between the average intra-group values was 3.148o (± 2.669o) for GU and 2.476o (± 2.638o) for G-Pro. The inter-group mean difference reached 5.45o. The Inter-observer reliability was 0.990 for GU and 0.993 for G-Pro; as for validity, the values obtained were 0.976 for GU and 0.992 for G-Pro. CONCLUSIONS: The G-Pro app is a reliable tool with a high accuracy to measure the knee flexion angle. Its results are slightly superior and more accurate to those of traditional instruments.

ANTECEDENTES: Los teléfonos inteligentes se han convertido en una parte fundamental de nuestra sociedad, no sólo cotidiana, sino también profesional, pues han surgido numerosas aplicaciones para el ámbito sanitario. El objetivo del estudio fue analizar la fiabilidad y validez concurrente de una app para iPhone en relación con la goniometría tradicional. MATERIAL Y MÉTODOS: Estudio comparativo y descriptivo de validación no experimental a doble ciego. En una muestra de 21 sujetos se realizaron cuatro mediciones de movilidad de rodilla: dos mediante goniometría universal (GU) y dos mediante la aplicación Goniometer-Pro (G-Pro) para iPhone. La radiografía se empleó como medida de referencia del ángulo de flexión de rodilla y fue analizada por un único evaluador que comparó este resultado con el de los otros evaluadores. RESULTADOS: La diferencia entre los valores medios intragrupos fue de 3.148o (± 2.669o) para GU y 2.476o (± 2.638o) para G-Pro. La diferencia de medias intergrupos alcanzó 5.45o. La fiabilidad interobservador fue de 0.990 para GU y 0.993 para G-Pro; en cuanto a la validez, los valores obtenidos fueron 0.976 para GU y 0.992 para G-Pro. CONCLUSIONES: La app G-Pro es una herramienta fiable y con una elevada exactitud para medir el ángulo de flexión de la rodilla. Sus resultados son ligeramente superiores y más precisos a los de la goniometría tradicional.

Double-walled iron oxide nanotubes via selective chemical etching and Kirkendall process.

Double-walled oxide nanotube structures are interesting for a wide range of applications, from photocatalysis to drug delivery. In this work, a progressive oxidation method to fabricate double-walled nanotube structures is reported in detail. The approach is based on the electrodeposition of metallic iron nanowires, in porous alumina templates, followed by a selective chemical etching, nanoscale Kirkendall effect, a fast oxidation and out-diffusion of the metallic core structure during thermal annealing. To validate the formation mechanism of such core-shell structure, chemical composition and atomic structure were assessed. The resulting hematite nanotubes have a high degree of uniformity, along several microns, and a nanoscopic double-walled structure.

The Novel Role of SOX2 as an Early Predictor of Cancer Risk in Patients with Laryngeal Precancerous Lesions.

The SOX2 gene located at 3q26 is frequently amplified and overexpressed in multiple cancers, including head and neck squamous cell carcinomas (HNSCC). The tumor-promoting activity and involvement of SOX2 in tumor progression has been extensively demonstrated, thereby emerging as a promising therapeutic target. However, the role of SOX2 in early stages of tumorigenesis and its possible contribution to malignant transformation remain unexplored. This study investigates for the first time SOX2 protein expression by immunohistochemistry and gene amplification by real-time PCR using a large series of 94 laryngeal precancerous lesions. Correlations with the histopathological classification and the risk of progression to invasive carcinoma were established. Nuclear SOX2 expression was frequently detected in 38 (40%) laryngeal dysplasias, whereas stromal cells and normal adjacent epithelia showed negative expression. SOX2 gene amplification was detected in 18 (33%) of 55 laryngeal dysplasias. Univariate Cox analysis showed that SOX2 gene amplification (p = 0.046) and protein expression (p < 0.001) but not histological grading (p = 0.432) were significantly associated with laryngeal cancer risk. In multivariate stepwise analysis including age, tobacco, histology, SOX2 gene amplification and SOX2 expression, SOX2 expression (HR = 3.531, 95% CI 1.144 to 10.904; p = 0.028) was the only significant independent predictor of laryngeal cancer development. These findings underscore the relevant role of SOX2 in early tumorigenesis and a novel clinical application of SOX2 expression as independent predictor of laryngeal cancer risk in patients with precancerous lesions beyond current WHO histological grading. Therefore, targeting SOX2 could lead to effective strategies for both cancer prevention and treatment.

Altered myogenesis and premature senescence underlie human TRIM32-related myopathy.

TRIM32 is a E3 ubiquitin -ligase containing RING, B-box, coiled-coil and six C-terminal NHL domains. Mutations involving NHL and coiled-coil domains result in a pure myopathy (LGMD2H/STM) while the only described mutation in the B-box domain is associated with a multisystemic disorder without myopathy (Bardet-Biedl syndrome type11), suggesting that these domains are involved in distinct processes. Knock-out (T32KO) and knock-in mice carrying the c.1465G > A (p.D489N) involving the NHL domain (T32KI) show alterations in muscle regrowth after atrophy and satellite cells senescence. Here, we present phenotypical description and functional characterization of mutations in the RING, coiled-coil and NHL domains of TRIM32 causing a muscle dystrophy. Reduced levels of TRIM32 protein was observed in all patient muscle studied, regardless of the type of mutation (missense, single amino acid deletion, and frameshift) or the mutated domain. The affected patients presented with variable phenotypes but predominantly proximal weakness. Two patients had symptoms of both muscular dystrophy and Bardet-Biedl syndrome. The muscle magnetic resonance imaging (MRI) pattern is highly variable among patients and families. Primary myoblast culture from these patients demonstrated common findings consistent with reduced proliferation and differentiation, diminished satellite cell pool, accelerated senescence of muscle, and signs of autophagy activation.

Frequent Alteration of Annexin A9 and A10 in HPV-Negative Head and Neck Squamous Cell Carcinomas: Correlation with the Histopathological Differentiation Grade.

The annexin protein superfamily has been implicated in multiple physiological and pathological processes, including carcinogenesis. Altered expression of various annexins has frequently been observed and linked to the development and progression of various human malignancies. However, information is lacking on the expression and clinical significance of annexin A9 (ANXA9) and A10 (ANXA10) in head and neck squamous cell carcinomas (HNSCC). ANXA9 and ANXA10 expression was evaluated in a large cohort of 372 surgically treated HPV-negative HNSCC patients and correlated with the clinicopathologic parameters and disease outcomes. Down-regulation of ANXA9 expression was found in 42% of HNSCC tissue samples, compared to normal epithelia. ANXA9 expression in tumors was significantly associated with oropharyngeal location and histological differentiation grade (P < 0.001). In marked contrast, ANXA10 expression was absent in normal epithelium, but variably detected in the cytoplasm of cancer cells. Positive ANXA10 expression was found in 64% of tumors, and was significantly associated with differentiation grade (P < 0.001), being also more frequent in oropharyngeal tumors (P = 0.019). These results reveal that the expression of both ANXA9 and ANXA10 is frequently altered in HNSCC and associated to the tumor differentiation grade, suggesting that they could be implicated in the pathogenesis of these cancers.

Fiabilidad y validez concurrente de la app Goniometer Pro vs goniómetro universal en la determinación de la flexión pasiva de rodilla / Reliability and concurrent validity of the app Goniometer Pro vs Universal Goniometer in the determination of passive knee flexion

Resumen: Antecedentes: Los teléfonos inteligentes se han convertido en una parte fundamental de nuestra sociedad, no sólo cotidiana, sino también profesional, pues han surgido numerosas aplicaciones para el ámbito sanitario. El objetivo del estudio fue analizar la fiabilidad y validez concurrente de una app para iPhone en relación con la goniometría tradicional. Material y métodos: Estudio comparativo y descriptivo de validación no experimental a doble ciego. En una muestra de 21 sujetos se realizaron cuatro mediciones de movilidad de rodilla: dos mediante goniometría universal (GU) y dos mediante la aplicación Goniometer-Pro (G-Pro) para iPhone. La radiografía se empleó como medida de referencia del ángulo de flexión de rodilla y fue analizada por un único evaluador que comparó este resultado con el de los otros evaluadores. Resultados: La diferencia entre los valores medios intragrupos fue de 3.148o (± 2.669o) para GU y 2.476o (± 2.638o) para G-Pro. La diferencia de medias intergrupos alcanzó 5.45o. La fiabilidad interobservador fue de 0.990 para GU y 0.993 para G-Pro; en cuanto a la validez, los valores obtenidos fueron 0.976 para GU y 0.992 para G-Pro. Conclusiones: La app G-Pro es una herramienta fiable y con una elevada exactitud para medir el ángulo de flexión de la rodilla. Sus resultados son ligeramente superiores y más precisos a los de la goniometría tradicional.

Abstract: Background: Smartphones have become a fundamental part of our society, not only everyday, but also professional, since many applications have emerged for the health field. The objective of the study was to analyze the reliability and concurrent validity of an app for iPhone in relation to traditional measurement. Material and methods: A non-experimental, double-blind validation and descriptive study. On a sample of 21 subjects, four measurements of knee mobility were performed. Two using universal goniometer (GU) and two through the application Goniometer-Pro (G-Pro) for IPhone. The X-ray was used as a reference measure of the knee flexion angle and was analyzed by a single evaluator who compared this result with that of the other evaluators. Results: The difference between the average intra-group values was 3.148o (± 2.669o) for GU and 2.476o (± 2.638o) for G-Pro. The inter-group mean difference reached 5.45o. The Inter-observer reliability was 0.990 for GU and 0.993 for G-Pro; as for validity, the values obtained were 0.976 for GU and 0.992 for G-Pro. Conclusions: The G-Pro app is a reliable tool with a high accuracy to measure the knee flexion angle. Its results are slightly superior and more accurate to those of traditional instruments.

XAS/DRIFTS/MS spectroscopy for time-resolved operando investigations at high temperature.

The combination of complementary techniques in the characterization of catalysts under working conditions is a very powerful tool for an accurate and in-depth comprehension of the system investigated. In particular, X-ray absorption spectroscopy (XAS) coupled with diffuse reflectance infrared Fourier transform spectroscopy (DRIFTS) and mass spectroscopy (MS) is a powerful combination since XAS characterizes the main elements of the catalytic system (selecting the absorption edge) and DRIFTS monitors surface adsorbates while MS enables product identification and quantification. In the present manuscript, a new reactor cell and an experimental setup optimized to perform time-resolved experiments on heterogeneous catalysts under working conditions are reported. A key feature of this setup is the possibility to work at high temperature and pressure, with a small cell dead volume. To demonstrate these capabilities, performance tests with and without X-rays are performed. The effective temperature at the sample surface, the speed to purge the gas volume inside the cell and catalytic activity have been evaluated to demonstrate the reliability and usefulness of the cell. The setup capability of combining XAS, DRIFTS and MS spectroscopies is demonstrated in a time-resolved experiment, following the reduction of NO by Rh nanoparticles supported on alumina.

Non-compaction cardiomyopathy and early respiratory failure in an adult symptomatic female carrier of centronuclear myopathy caused by a MTM1 mutation.

X-linked myotubular myopathy (XLMTM) is a rare neuromuscular condition caused by mutations in the MTM1 gene. Female carriers are believed to be usually asymptomatic; nevertheless, recent reports have displayed a wide a spectrum of clinical involvement in females suggesting that MTM1 mutations might be underestimated in this population. Here we report a 55-year-old woman manifesting with an abrupt respiratory decline, whose respiratory function tests revealed a severe restrictive ventilatory defect. The neurological examination identified mild proximal leg weakness and her cardiac evaluation showed a non-compaction cardiomyopathy with normal left ventricle function. Muscle biopsy was consistent with centronuclear myopathy. Next-generation sequencing of 49 genes related to congenital myopathies allowed the identification of a 4 bp deletion in the MTM1 gene, leading to a truncating mutation previously described in males but for the first time reported in a female patient.