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Tuberous sclerosis complex is a rare genetic disorder leading to the growth of hamartomas in multiple organs, including cardiac rhabdomyomas. Children with symptomatic cardiac rhabdomyoma require frequent admissions to intensive care units, have major complications, namely, arrhythmias, cardiac outflow tract obstruction and heart failure, affecting the quality of life and taking on high healthcare cost. Currently, there is no standard pharmacological treatment for this condition, and the management includes a conservative approach and supportive care. Everolimus has shown positive effects on subependymal giant cell astrocytomas, renal angiomyolipoma and refractory seizures associated with tuberous sclerosis complex. However, evidence supporting efficacy in symptomatic cardiac rhabdomyoma is limited to case reports. The ORACLE trial is the first randomised clinical trial assessing the efficacy of everolimus as a specific therapy for symptomatic cardiac rhabdomyoma.
ORACLE is a phase II, prospective, randomised, placebo-controlled, double-blind, multicentre protocol trial. A total of 40 children with symptomatic cardiac rhabdomyoma secondary to tuberous sclerosis complex will be randomised to receive oral everolimus or placebo for 3 months. The primary outcome is 50% or more reduction in the tumour size related to baseline. As secondary outcomes we include the presence of arrhythmias, pericardial effusion, intracardiac obstruction, adverse events, progression of tumour reduction and effect on heart failure.
ORACLE protocol addresses a relevant unmet need in children with tuberous sclerosis complex and cardiac rhabdomyoma. The results of the trial will potentially support the first evidence-based therapy for this condition.
Based on wettability and reaction interfaces previously reported, as well as on thermodynamic considerations, a likely mechanism has been proposed for the chemical interaction taking place at the metal/ceramic interface during wettability experiments carried out by the so-called “sessile drop” method. The experiments involved three Ag-Cu-based brazing alloys [Cusil (Ag-28wt.%Cu), Cusil-ABA (Ag-34.6wt.%Cu-1.58wt.%Ti) and Incusil-ABA (Ag-26.6wt.%Cu-12.4wt.%In-0.89wt.%Ti)] and as polished and pre-oxidized pressure-less sintered silicon carbide (PLS-SiC), with a total holding time of 90 minutes at 850 °C, under a Zr sponge-gettered vacuum of 10-4/10-5 Torr.
An alternative method for the standardless quantitative x-ray diffraction analysis of mixtures of inorganic crystalline phases proposed in the literature several years ago is presented. Our method requires only previously calculated μ*i values from tabulated data for all phases present in the mixtures. It does not require either the determination of calibration constants or the use of external standards, but it does require that the number of analyzed mixtures is larger than or equal to the number of phases present in them, and that the chemical composition of the mixtures are significantly different from each other. The integrated intensities of the chemically pure phases are estimated by a least-squares procedure from XRD data obtained from the mixtures. The method was tested against data published in the literature, with good results. Finally, a general expression for the “Normalized Height Law” proposed on an empirical basis by other researchers, has been theoretically derived.
An initial mixture of raw materials (batch) typically used for the manufacture of conventional soda-lime float glass was subjected to a mechanical activation process for 30 or 60 minutes in a planetary ball mill. An intensification of the chemical reactivity of the batch, which was directly related with the increase in the milling time, was observed. This accelerated the chemical reactions that took place during the batch melting process between sodium, calcium and magnesium carbonates and other components of the mixture, which happened at significantly lower temperatures with respect to the batch without mechanical activation. The heat of fusion of the batch, estimated using a methodology previously reported in the literature, indicated that the mechanical activation given to the initial mixture of raw materials decreased the energy consumed during the batch melting. This was also evidenced by a decrease in the temperature at which the release of CO2 ended, which was considerably larger than that previously reported in the literature based solely on the decrease in the particle size of a batch of similar composition achieved by dry sieving.
We present a scintillator-based detector able to measure the proton energy and the spatial distribution with a relatively simple design. It has been designed and built at the Spanish Center for Pulsed Lasers (CLPU) in Salamanca and tested in the proton accelerator at the Centro de Micro-Análisis de Materiales (CMAM) in Madrid. The detector is capable of being set in the high repetition rate (HRR) mode and reproduces the performance of the radiochromic film detector. It represents a new class of online detectors for laser–plasma physics experiments in the newly emerging high power laser laboratories working at HRR.
The alpaca fiber diameter (FD) varies from 18 to 36 μm, being the finer fiber categories highly appreciated. However, the alpaca fiber presents some limitations in the textile industry due to the high incidence of fiber medullation and diameter variability, both reduces the comfort feeling of the garments. Decreasing or even removing medullation could be a possible selection objective in alpaca breeding programs for increasing economic value of the alpaca fiber. Therefore, the present work aimed to estimate genetic parameters regarding medullation traits, as well as the genetic correlations with other economical important traits, to be able to select the appropriate criteria to reduce or remove medullation on alpaca fiber and help to reduce the prickle factor in the garments. The data was collected from 2000 to 2017 and belonged to the Pacomarca experimental farm. There were 3698 medullation records corresponding to 1869 Huacaya and 414 Suri genetic types. The fiber samples were taken from the mid side, and were analyzed in an OFDA 100® device. The traits analyzed were percentage of medullation (PM), medullated fiber diameter (MFD), FD, standard deviation of FD, greasy fleece weight as fiber traits; density, crimp in Huacaya and lock structure in Suri, head conformation, leg coverage as morphological traits; weaning weight and age at first calving as secondary and functional traits. Genetic parameters were estimated via a multitrait restricted maximum likelihood. The heritabilities for PM and MFD were 0.225 and 0.237 in Huacaya genetic type and 0.664 and 0.237 in Suri genetic type, respectively; heritabilities for other traits were moderate for productive and morphological traits, and low to moderate for secondary and functional traits. The genetic correlations PM–FD and MFD–FD were high and favorable in both genetic types, between 0.531 and 0.975; the genetic correlation PM–MFD was 0.121 in Huacaya and 0.427 in Suri. The rest of genetic correlations with other traits were in general moderate and favorable. The repeatabilities were 0.556 and 0.668 for PM, and 0.322 and 0.293 for MFD in Huacaya and Suri genetic types, respectively. As a conclusion, PM was identified to be a good selection criterion, probably combined in an index with FD to reduce prickling factor.
Although many mental health care systems provide care interventions that are not related to direct health care, little is known about the interfaces between the latter and core health care. ‘Core health care’ refers to services whose explicit aim is direct clinical treatment which is usually provided by health professionals, i.e., physicians, nurses, psychologists. ‘Other care’ is typically provided by other staff and includes accommodation, training, promotion of independence, employment support and social skills. In such a definition, ‘other care’ does not necessarily mean being funded or governed differently. The aims of the study were: (1) using a standard classification system (Description and Evaluation of Services and Directories in Europe for Long Term Care, DESDE-LTC) to identify ‘core health’ and ‘other care’ services provided to adults with mental health problems; and (2) to investigate the balance of care by analysing the types and characteristics of core health and other care services.
The study was conducted in eight selected local areas in eight European countries with different mental health systems. All publicly funded mental health services, regardless of the funding agency, for people over 18 years old were identified and coded. The availability, capacity and the workforce of the local mental health services were described using their functional main activity or ‘Main Types of Care’ (MTC) as the standard for international comparison, following the DESDE-LTC system.
In these European study areas, 822 MTCs were identified as providing core health care and 448 provided other types of care. Even though one-third of mental health services in the selected study areas provided interventions that were coded as ‘other care’, significant variation was found in the typology and characteristics of these services across the eight study areas.
The functional distinction between core health and other care overcomes the traditional division between ‘health’ and ‘social’ sectors based on governance and funding. The overall balance between core health and other care services varied significantly across the European sites. Mental health systems cannot be understood or planned without taking into account the availability and capacity of all services specifically available for this target population, including those outside the health sector.
We determined the molecular epidemiology of Bordetella pertussis isolates to evaluate its potential impact on pertussis reemergence in a population of Mexico. Symptomatic and asymptomatic cases were included. Pertussis infection was confirmed by culture and real-time polymerase chain reaction (PCR). Selected B. pertussis isolates were further analysed; i.e. clonality was analysed by pulsed-field gel electrophoresis (PFGE) and ptxP-ptxA, prn, fim2 and fim3 typing was performed by PCR and sequencing. Out of 11 864 analysed samples, 687 (5.8%) were positive for pertussis, with 244 (36%) confirmed by both culture and PCR whereas 115 (17%) were positive only by culture and 328 (48%) were positive only by PCR. One predominant clone (clone A, n = 62/113; 55%) and three major subtypes (A1, A2 and A3) were identified by PFGE. All 113 selected isolates had the allelic combination ptxP3-ptxA1. The predominant clone A and the three major subtypes (A1, A2 and A3) corresponded to the emerging genotypes ptxP3-ptxA1-prn2-fim2-1-fim3-2 and ptxP3-ptxA1-prn2-fim2-1-fim3-1. In conclusion, the presence of an endemic clone and three predominant subtypes belonging to the genotypes ptxP3-ptxA1-prn2-fim2-1-fim3-2 and ptxP3-ptxA1-prn2-fim2-1-fim3-1 were detected. This finding supports the global spread/expansion reported for these outbreaks associated genotypes.
For more than 60 years, Colombia experienced an armed conflict involving government forces, guerrillas, and other illegal armed groups. Violence, including torture and massacres, has caused displacement of entire rural communities to urban areas. Lack of information on the problems displaced communities face and on their perceptions on potential solutions to these problems may prevent programs from delivering appropriate services to these communities. This study explores the problems of Afro-Colombian survivors from two major cities in Colombia; the activities they do to take care of themselves, their families, and their community; and possible solutions to these problems.
This was a qualitative, interview-based study conducted in Quibdó and Buenaventura (Colombia). Free-list interviews and focus groups explored the problems of survivors and the activities they do to take care of themselves, their families, and their community. Key-informant interviews explored details of the identified mental health problems and possible solutions.
In Buenaventura, 24 free-list interviews, one focus group, and 17 key-informant interviews were completed. In Quibdó, 29 free-list interviews, one focus group, and 15 key-informant interviews were completed. Mental health problems identified included: (1) problems related to exposure to torture/violent events; (2) problems with adaptation to the new social context; and (3) problems related to current poverty, lack of employment, and ongoing violence. These problems were similar to trauma symptoms and features of depression and anxiety, as described in other populations. Solutions included psychological help, talking to friends/family, relying on God’s help, and getting trained in different task or jobs.
Afro-Colombian survivors of torture and violence described mental health problems similar to those of other trauma-affected populations. These results suggest that existing interventions that address trauma-related symptoms and current ongoing stressors may be appropriate for improving the mental health of survivors in this population.
Santaella-TenorioJ, Bonilla-EscobarFJ, Nieto-GilL, Fandiño-LosadaA, Gutiérrez-MartínezMI, BassJ, BoltonP. Mental Health and Psychosocial Problems and Needs of Violence Survivors in the Colombian Pacific Coast: A Qualitative Study in Buenaventura and Quibdó. Prehosp Disaster Med. 2018;33(6):567–574.
To compare the epidemiology, clinical characteristics, and mortality of patients with bloodstream infections (BSI) caused by extended-spectrum β-lactamase (ESBL)-producing Escherichia coli (ESBL-EC) versus ESBL-producing Klebsiella pneumoniae (ESBL-KP) and to examine the differences in clinical characteristics and outcome between BSIs caused by isolates with CTX-M versus other ESBL genotypes
As part of the INCREMENT project, 33 tertiary hospitals in 12 countries retrospectively collected data on adult patients diagnosed with ESBL-EC BSI or ESBL-KP BSI between 2004 and 2013. Risk factors for ESBL-EC versus ESBL-KP BSI and for 30-day mortality were examined by bivariate analysis followed by multivariable logistic regression.
The study included 909 patients: 687 with ESBL-EC BSI and 222 with ESBL-KP BSI. ESBL genotype by polymerase chain reaction amplification of 286 isolates was available. ESBL-KP BSI was associated with intensive care unit admission, cardiovascular and neurological comorbidities, length of stay to bacteremia >14 days from admission, and a nonurinary source. Overall, 30-day mortality was significantly higher in patients with ESBL-KP BSI than ESBL-EC BSI (33.7% vs 17.4%; odds ratio, 1.64; P=.016). CTX-M was the most prevalent ESBL subtype identified (218 of 286 polymerase chain reaction-tested isolates, 76%). No differences in clinical characteristics or in mortality between CTX-M and non–CTX-M ESBLs were detected.
Clinical characteristics and risk of mortality differ significantly between ESBL-EC and ESBL-KP BSI. Therefore, all ESBL-producing Enterobacteriaceae should not be considered a homogeneous group. No differences in outcomes between genotypes were detected.
Radionuclide scanning images published in Nature by Di Chiro in 1964 showed a downward migration along the spinal canal of particle tracers injected in the brain ventricles while also showing an upward flow of tracers injected in the lumbar region of the canal. These observations, since then corroborated by many radiological measurements, have been the basis for the hypothesis that there must be an active circulation mechanism associated with the transport of cerebrospinal fluid (CSF) deep down into the spinal canal and subsequently returning a portion back to the cranial vault. However, to date, there has been no physical explanation for the mechanism responsible for the establishment of such a bulk recirculating motion. To investigate the origin and characteristics of this recirculating flow, we have analyzed the motion of the CSF in the subarachnoid space of the spinal canal. Our analysis accounts for the slender geometry of the spinal canal, the small compliance of the dura membrane enclosing the CSF in the canal, and the fact that the CSF is confined to a thin annular subarachnoid space surrounding the spinal cord. We apply this general formulation to study the characteristics of the flow generated in a simplified model of the spinal canal consisting of a slender compliant cylindrical pipe with a coaxial cylindrical inclusion, closed at its distal end, and subjected to small periodic pressure pulsations at its open entrance. We show that the balance between the local acceleration and viscous forces produces a leading-order flow consisting of pure oscillatory motion with axial velocities on the order of a few centimetres per second and amplitudes monotonically decreasing along the length of the canal. We then demonstrate that the nonlinear term associated with the convective acceleration contributes to a second-order correction consisting of a steady streaming that generates a bulk recirculating motion of the CSF along the length of the canal with characteristic velocities two orders of magnitude smaller than the leading-order oscillatory flow. The results of the analysis of this idealized geometry of the spinal canal are shown to be in good agreement not only with experimental measurements in an in-vitro model but also with radiological measurements conducted in human adults.
Composites from carbon nanotubes and polymers have been synthesized and studied. The composites were obtained joining carbon nanotubes with polymethyl methacrylate, nylon-6 and polystyrene. The materials were observed through scanning electron microscopy to evaluate the carbon nanotubes dispersion in the polymeric matrices. FTIR and Raman spectroscopies were used to analyze the interactions among functionalized and non-functionalized multiwalled carbon nanotubes and polymers, demonstrating affinity and peculiar spectra behaviors for each composite with different carbon nanotubes loads.
PA6 and PMMA polymers with different MWCNTs addition (5, 7 and 9 wt %) were synthetized through casting solution, resulting in improvement properties in contrast to pristine polymers. SEM images showed the MWCNTs embedded into polymeric matrices. D, G and G´ bands of MWCNTs were confirmed by Raman spectroscopy and functional groups observed in both nanocomposites by FTIR demonstrated a strong interaction. A significant increasing in electrical conductivity and microhardness was observed in all the nanocomposites. Major microhardness values were obtained in MWCNTs/PA6 (50 HV) however the MWCNTs/PMMA nanocomposites showed the highest electrical conductivity value (6.4×10-4 S/cm).
This work presents a study about synthesis of polypyrrole films electrochemically doped with iodine by luminescent discharge plasma in aqueous sodium sulfate solution, and its structural, morphological and electrical characterization. The synthesis is carried out at atmospheric pressure with square pulse from -1.06 to 1.16 V for 120 minutes. Doping times of the films are 10, 30 and 50 minutes. The films have thicknesses of 6, 12.7, 20.32 and 25.4 μm, and linear growth trend according to the time exposure of their synthesis. The main chemical groups exhibited in the films are C-H, N-H and CH2I, which are characteristics of film doped with Iodine. The electrical conductivity calculated of polypyrrole films is in the range 10-5-10-3 S/cm, and its activation energy is between 0.052 and 1.77 eV.
Suckling lamb meat is traditionally produced in Mediterranean Europe. Breed can affect the quality of the lamb carcass and meat. This study is aimed at comparing the carcass and meat quality between suckling lambs from a local and a non-native dairy breed, Churra and Assaf. Churra is included in the Spanish Protected Geographical Indication (PGI) ‘Lechazo de Castilla y León’, whereas Assaf is not. However, Assaf breeders have requested the inclusion of the breed in the PGI. Carcasses and meat from 16 male lambs (eight Churra and eight Assaf) were used in this study. The lambs were all raised under an intensive rearing system and fed on a milk substitute to minimise maternal influence. The carcasses were evaluated for conformation, fatness, joint and leg tissue proportions and the meat was analysed for composition (i.e. proximate composition, iron, haematin, fatty acids and volatiles) and technological quality traits (i.e. texture, water holding capacity, colour and lipid stability). Churra carcasses were larger than Assaf carcasses. However, the proportions of commercial joints and main tissues did not differ between breeds. Cavity and intermuscular leg fat, but not total leg fat, were higher in Churra carcasses. Churra meat showed a higher proportion of n-6 fatty acids, higher redness and better colour stability during aerobic storage. In contrast, Assaf lamb was more resistant to lipid oxidation after cooking. This is a preliminary study to measure the influence of breed on a wide range of quality characteristics in Churra and Assaf suckling lamb carcass and meat. It may be of relevance for breeders, consumers and food policy makers, setting the basis for future studies that include larger commercial populations.
There is a need of more quantitative standardised data to compare local Mental Health Systems (MHSs) across international jurisdictions. Problems related to terminological variability and commensurability in the evaluation of services hamper like-with-like comparisons and hinder the development of work in this area. This study was aimed to provide standard assessment and comparison of MHS in selected local areas in Europe, contributing to a better understanding of MHS and related allocation of resources at local level and to lessen the scarcity in standard service comparison in Europe. This study is part of the Seventh Framework programme REFINEMENT (Research on Financing Systems’ Effect on the Quality of Mental Health Care in Europe) project.
A total of eight study areas from European countries with different systems of care (Austria, England, Finland, France, Italy, Norway, Romania, Spain) were analysed using a standard open-access classification system (Description and Evaluation of Services for Long Term Care in Europe, DESDE-LTC). All publicly funded services universally accessible to adults (≥18 years) with a psychiatric disorder were coded. Care availability, diversity and capacity were compared across these eight local MHS.
The comparison of MHS revealed more community-oriented delivery systems in the areas of England (Hampshire) and Southern European countries (Verona – Italy and Girona – Spain). Community-oriented systems with a higher proportion of hospital care were identified in Austria (Industrieviertel) and Scandinavian countries (Sør-Trøndelag in Norway and Helsinki-Uusimaa in Finland), while Loiret (France) was considered as a predominantly hospital-based system. The MHS in Suceava (Romania) was still in transition to community care.
There is a significant variation in care availability and capacity across MHS of local areas in Europe. This information is relevant for understanding the process of implementation of community-oriented mental health care in local areas. Standard comparison of care provision in local areas is important for context analysis and policy planning.