Esclerosis tuberosa: hallazgos clinicos en 67 pacientes

BACKGROUND: Tuberous sclerosis is an uncommon neurocutaneous syndrome characterized by the appearance of hamartomas in multiple organs. Diagnosis is based on clinical criteria. OBJECTIVE. To report the clinical findings in a series of 67 patients with tuberous sclerosis. MATERIAL AND METHODS: This was a descriptive and observational retrospective study of patients with tuberous sclerosis referred to our dermatology clinics between January 1994 and March 2007. RESULTS: All patients presented neurological or dermatological disorders. Other disorders, in descending frequency, were psychiatric (55.5%), renal (32.8%), cardiac (22.4%), skeletal and pulmonary (13.4%), and ophthalmological (11.9%). CONCLUSIONS: We report the clinical findings in a series of patients with tuberous sclerosis. According to our literature search, this is the first such study in the Spanish population. Overall, our findings support those already publishe

Frequency analysis of vocalisations in relation to the growth in broiler chicken

Poultry is one of the lowest cost sources of animal protein in the world and, more than 40 billion chickens are produced every year globally. For reasons of public concern and due to the large number of animals involved, it is considered by many people to be important to take care of the welfare and health status of the chickens reared under intensive farm conditions. Precision Livestock Farming (PLF) can support the farmer in his day to day routine management through the use of sensors, cameras and microphones, and these have the potential to improve production and to enable monitoring of welfare status. In this context, the 7FP EU-PLF project aims to test the efficiency of the use of those sensors at farm level. In particular, the aim of this study was to record and analyse broiler vocalisations under normal farm conditions and to identify the relation between animal sounds, and growth trends. Recordings were made at regular intervals, for the entire short production life of the birds, in order to evaluate the variation of frequency and bandwidth of the sounds emitted by the animals during the cycle of production. The recordings were made in an automated, non-invasive and non-intrusive way and the sound data was compared with the weight of the birds automatically measured by a 'step on scale' placed on the floor of the broiler house. Sound analysis was performed based on the amplitude and frequency of the sound signal in audio files recorded at farm level. Through analysis of the sounds recorded, a significant correlation (P<0.001) between the frequencies of the vocalisations recorded and the weight of the broilers was found across all production cycles and farms assessed. The ongoing goal will be the development of a tool able to automatically detect the growth of the animals based on the frequency of the vocalisation emitted by the birds at different ages, and as a possible tool for determining deviations from their expected growth trend

Hybrid Refining Approach of PrOnto Ontology

This paper presents a refinement of PrOnto ontology using a validation test based on legal experts’ annotation of privacy policies combined with an Open Knowledge Extraction (OKE) algorithm. To ensure robustness of the results while preserving an interdisciplinary approach, the integration of legal and technical knowledge has been carried out as follows. The set of privacy policies was first analysed by the legal experts to discover legal concepts and map the text into PrOnto. The mapping was then provided to computer scientists to perform the OKE analysis. Results were validated by the legal experts, who provided feedbacks and refinements (i.e. new classes and modules) of the ontology according to MeLOn methodology. Three iterations were performed on a set of (development) policies, and a final test using a new set of privacy policies. The results are 75,43% of detection of concepts in the policy texts and an increase of roughly 33% in the accuracy gain on the test set, using the new refined version of PrOnto enriched with SKOS-XL lexicon terms and definitions

Experience With Bexarotene to Treat Cutaneous T-Cell Lymphomas: A Study of the Spanish Working Group of Cutaneous Lymphomas

Background and objectives: Bexarotene has been approved to treat advanced stage cutaneous T -cell lymphomas (CTCL) since 1999. However, very few data have been published on its long-term safety and efficacy profile. The aim of this study is to determine the tolerability to bexarotene and outcomes by collecting the 2nd largest case series to date on its long-term use vs CTCL. Material and method: This was a multicenter retrospective review of 216 patients with mycosis fungoides (174), or S & eacute;zary syndrome (42) on a 10 -year course of bexarotene alone or in combination with other therapies at 19 tertiary referral teaching hospitals. Results: A total of 133 men (62%) and 83 women (38%) were included, with a mean age of 63.5 year (27 - 95). A total of 45% were on bexarotene monotherapy for the entire study period, 22% started on bexarotene but eventually received an additional therapy, 13% were on another treatment but eventually received bexarotene while the remaining 20% received a combination therapy since the beginning. The median course of treatment was 20.78 months (1 - 114); and the overall response rate, 70.3%. Complete and partial response rates were achieved in 26% and 45% of the patients, respectively. Treatment was well tolerated, being the most common toxicities hypertriglyceridemia (79%), hypercholesterolemia (71%), and hypothyroidism (52%). No treatment -related grade 5 adverse events were reported. Conclusions: Our study confirms bexarotene is a safe and effective therapy for the long-term treatment of CTCL. (c) 2024 AEDV. Published by Elsevier Espana, S.L.U. This is an open access article under the CC BY -NC -ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/)

Исследование желаемого образа семьи молодежи, проживающей в больших, средних и малых городах

Funding: EPSRC EP/J01771X, Royal Society Wolfson Research Merit AwardBackground Topical Photodynamic therapy (PDT) is an effective treatment for superficial non-melanoma skin cancers (NMSC) and dysplasia. During PDT light activates the photosensitiser (PpIX), metabolised from a topical pro-drug. A combination of PpIX, light and molecular oxygen results in inflammation and cell death. However, the outcomes of the treatment could be better. Insufficient biosynthesis of PpIX may be one of the causes of incomplete response or recurrence. Measuring surface fluorescence is usually employed as a means of studying PpIX formation. The aim of this work was to develop a device and a method for convenient fluorescence imaging in clinical settings to gather information on PpIX metabolism in healthy skin and NMSC with a view to improving PDT regimes. Methods A handheld fluorescence camera and a time course imaging method was developed and used in healthy volunteers and patients diagnosed with basal cell carcinoma (BCC) and actinic keratosis (AK). The photosensitiser (precursor) creams used were 5-aminolaevulinic acid (ALA; Ameluz®) and methyl aminolevulinate (MAL; Metvix®). Pain was assessed using a visual analogue score immediately after the PDT. Results Fluorescence due to PpIX increases over three hours incubation in healthy skin and in lesional BCC and AK. Distribution of PpIX fluorescence varies between the lesion types and between subjects. There was no significant correlation between PpIX fluorescence characteristics and pro-drug, diagnosis or pain experienced. However, there was a clear dependence on body site. Conclusion The device and the method developed can be used to assess the characteristics of PpIX fluorescence, quantitative analysis and time course. Our findings show that body site influences PpIX fluorescence which we suggest may be due to the difference in skin temperature at different body sites.PostprintPeer reviewe

Anisotropy studies around the galactic centre at EeV energies with the Auger Observatory

Data from the Pierre Auger Observatory are analyzed to search for anisotropies near the direction of the Galactic Centre at EeV energies. The exposure of the surface array in this part of the sky is already significantly larger than that of the fore-runner experiments. Our results do not support previous findings of localized excesses in the AGASA and SUGAR data. We set an upper bound on a point-like flux of cosmic rays arriving from the Galactic Centre which excludes several scenarios predicting sources of EeV neutrons from Sagittarius $A$. Also the events detected simultaneously by the surface and fluorescence detectors (the `hybrid' data set), which have better pointing accuracy but are less numerous than those of the surface array alone, do not show any significant localized excess from this direction.Comment: Matches published versio

The Role of Purported Mucoprotectants in Dealing with Irritable Bowel Syndrome, Functional Diarrhea, and Other Chronic Diarrheal Disorders in Adults

Chronic diarrhea is a frequent presenting symptom, both in primary care medicine and in specialized gastroenterology units. It is estimated that more than 5% of the global population suffers from chronic diarrhea. and that about 40% of these subjects are older than 60 years. The clinician is frequently faced with the need to decide which is the best therapeutic approach for these patients. While the origin of chronic diarrhea is diverse, impairment of intestinal barrier function, dysbiosis. and mucosal micro-inflammation are being increasingly recognized as underlying phenomena characterizing a variety of chronic diarrheal diseases. In addition to current pharmacological therapies, there is growing interest in alternative products such as mucoprotectants, which form a mucoadhesive film over the epithelium to reduce and protect against the development of altered intestinal permeability, dysbiosis, and mucosal micro-inflammation. This manuscript focuses on chronic diarrhea in adults, and we will review recent evidence on the ability of these natural compounds to improve symptoms associated with chronic diarrhea and to exert protective effects for the intestinal barrier

EUSO-SPB1 mission and science

The Extreme Universe Space Observatory on a Super Pressure Balloon 1 (EUSO-SPB1) was launched in 2017 April from Wanaka, New Zealand. The plan of this mission of opportunity on a NASA super pressure balloon test flight was to circle the southern hemisphere. The primary scientific goal was to make the first observations of ultra-high-energy cosmic-ray extensive air showers (EASs) by looking down on the atmosphere with an ultraviolet (UV) fluorescence telescope from suborbital altitude (33 km). After 12 days and 4 h aloft, the flight was terminated prematurely in the Pacific Ocean. Before the flight, the instrument was tested extensively in the West Desert of Utah, USA, with UV point sources and lasers. The test results indicated that the instrument had sensitivity to EASs of ⪆ 3 EeV. Simulations of the telescope system, telescope on time, and realized flight trajectory predicted an observation of about 1 event assuming clear sky conditions. The effects of high clouds were estimated to reduce this value by approximately a factor of 2. A manual search and a machine-learning-based search did not find any EAS signals in these data. Here we review the EUSO-SPB1 instrument and flight and the EAS search