Miracle-dz/UniFAR-FTUs huggingface dataset:
Inherited epidermolysis bullosa: recent basic and clinical advances

Purpose of review This review highlights key findings, both clinical and basic, that have been published in the field of inherited epidermolysis bullosa within the past few years. Recent findings New epidermolysis bullosa phenotypes, genotypes and modes of transmission have been identified, resulting in a revised classification system. Detailed evidence-based data are now available on the risk of extracutaneous complications in each of the major epidermolysis bullosa subtypes. Studies are now underway to try to better explain the biological aggressiveness of squamous cell carcinomas arising in epidermolysis bullosa skin. Cell and animal models have been refined and used to ascertain the feasibility of gene replacement therapy, stem cell transplantation, and treatment with injected allogeneic fibroblasts or recombinant type VII collagen. As a result, clinical trials are now being pursued to test each of these in humans. Summary Epidermolysis bullosa is caused by mutations in at least 14 genes, leading to a broad spectrum of entities, each of which has its own relative risk for the development of specific extracutaneous complications and/or premature death. Intensive research, both basic and clinical, is bringing us closer to more effective treatments and possibly even a cure.
Comparison of Recovery Profile After Ambulatory Anesthesia with Propofol, Isoflurane, Sevoflurane and Desflurane: A Systematic Review

In this systematic review we focused on postoperative recovery and complications using four different anesthetic techniques. The database MEDLINE was searched via PubMed (1966 to June 2002) using the search words “anesthesia” and with ambulatory surgical procedures limited to randomized controlled trials in adults (>19 yr), in the English language, and in humans. A second search strategy was used combining two of the words “propofol,” “isoflurane,” “sevoflurane,” or “desflurane”. Screening and data extraction produced 58 articles that were included in the final meta-analysis. No differences were found between propofol and isoflurane in early recovery. However, early recovery was faster with desflurane compared with propofol and isoflurane and with sevoflurane compared with isoflurane. A minor difference was found in home readiness between sevoflurane and isoflurane (5 min) but not among the other anesthetics. Nausea, vomiting, headache, and postdischarge nausea and vomiting incidence were in favor of propofol compared with isoflurane (P < 0.05). A larger number of patients in the inhaled anesthesia groups required antiemetics compared with the propofol group. We conclude that the differences in early recovery times among the different anesthetics were small and in favor of the inhaled anesthetics. The incidence of side effects, specifically postoperative nausea and vomiting, was less frequent with propofol.
Ellipsoidal sets for static output feedback

Abstract The static output feedback synthesis for LTI systems is considered. It is shown to have analogies with robust analysis, in particular the existence of an output feedback gain is equivalent to the existence of some quadratic separator. These considerations lead to formulate the problem as the synthesis of an ellipsoidal set of stabilising controllers. Based on this formulation important issues are developed for fragility, restricted controllers and also root-clustering.
Caco-2 Invasion by Cronobacter sakazakii and Salmonella enterica Exposed to Drying and Heat Treatments in Dried State in Milk Powder

Due to the ability of foodborne pathogens to survive in low moisture food, the decontamination of milk powder is an important issue in food protection. The safety of food products is, however, not always insured and the different steps in the processing of food involve physiological and metabolic changes in bacteria. Among these changes, virulence properties may also be affected. In this study, the effect of drying and successive thermal treatments on the invasion capacity of Salmonella Typhimurium, Salmonella Senftenberg, and Cronobacter sakazakii was assessed. Bacteria were dried on milk powder at three different water activity levels (0.25, 0.58, and 0.80) and heated at two different temperatures (90°C and 100°C) for 30 and 120 s. After recovery, stressed bacterial populations were placed in contact with Caco-2 cells to estimate their invasion capacity. Our results show that drying increases the invasion capacity of foodborne pathogens, but that heat treatment in the dried state did not exert a selective pressure on bacterial cells depending on their invasion capacity after drying. Taken together, our findings add to the sum of knowledge on food safety in dried food products and provide insight into the effects of food processing.
[Paraproteinemic neuropathies: foundations for a specific therapy related to patients' quality of life].

INTRODUCTION Monoclonal gammopathy (MG) is reported in 1% of subjects above 50 years of age and in 3% of those above the age of 70. Of all patients with MG, 3% present polyneuropathy (PNP). The abbreviation MGUS (monoclonal gammopathy of undetermined significance) is used to describe a benign proliferation of M component (monoclonal) according to Kyle's criteria, with a potential to be malignant that is indicated by clinical, biochemical and haematological parameters. Aims. The aim of this study was to evaluate the effect of the clinical exacerbation of MGUS associated neuropathy, by referring to the existing literature and to our own experience in order to set out a number of proposals for treatment based on the deterioration of the patient's quality of life. CASE REPORTS Case 1: a 53 year old female with predominantly sensory sensory motor demyelinating polyradiculoneuropathy, associated to IgG lambda MGUS. Case 2: a 65 year old female with demyelinating sensory motor polyradiculoneuropathy associated to IgM kappa MGUS. In both cases there was a rapidly progressing neurological deterioration and no other data exist to suggest malign lymphoproliferation. CONCLUSIONS In addition to the treatments established for MG linked PNP, which are based on the modulation of the immune response, it has also been proved that when malign proliferation of plasmacytes exists specific treatment of the gammopathy can significantly stabilise or improve the neuropathic symptoms. We propose gammopathy specific haematological treatment in patients who still meet Kyle's criteria for MGUS with a torpid neurological course and scarce response to therapy with immunomodulators, prior to an important deterioration in the quality of life. We also suggest establishing this rapidly progressing clinical course as a criterion that indicates the process of becoming malign as put forward by Eurelings et al.
Indications for Anterior Lumbar Interbody Fusion

Anterior lumbar interbody fusion (ALIF) has become a widely recognized surgical technique for degenerative pathology of the lumbar spine. Spinal fusion has evolved dramatically ever since the first successful internal fixation by Hadra in 1891 who used a posterior approach to wire adjacent cervical vertebrae in the treatment of fracture‐dislocation. Advancements were made to reduce morbidity including bone grafting substitutes, metallic hardware instrumentation and improved surgical technique. The controversy regarding which surgical approach is best for treating various pathologies of the lumbar spine still exists. Despite being an established treatment modality, current indications of ALIF are yet to be clearly defined in the literature. This article discusses the current literature on indications on ALIF surgery.
Evaluation du niveau d'activité physique dans un service Marocain d'hémodialyse chronique

Introduction L'activité physique est souvent réduite chez le patient hémodialysé chronique. Plusieurs études ont montré l'intérêt du maintien ou de la reprise d'une activité physique sur l’état de santé des patients. Le but de notre travail est d'analyser le niveau d'activité physique chez nos patients hémodialysé chronique. Méthodes Nous avons réalisé une étude transversale au centre d'hémodialyse de l'hôpital Al Farabi d'Oujda. Nous avons évalué l'activité physique de nos patients à l'aide du Questionnaire de Baecke traduit en dialectal Marocain et adapté au contexte socioculturel Marocain. Nous avons relevé les paramètres démographiques et clinico-biologiques ainsi que le niveau d'activité physique. L'analyse statistique est réalisée par le logiciel SPSS 11.5. Résultats Notre étude a inclus 83 patients. L’âge moyen était de 47,3±13,2 ans, avec une prédominance masculine. 15,6% déclarent n'avoir aucune activité physique. 68% des patients rapportent des difficultés à effectuer des efforts physiques importants (courir, soulever un objet lourd). 16,4% des patients déclarent avoir une activité physique et sportive régulière (marche, foot-ball etc.). La diminution de l'activité physique est corrélée de façon significative à l’âge avancée, l'indice de masse corporelle bas, l'ancienneté en hémodialyse et les affections cardio-vasculaires. Conclusion Un programme d'activité physique adapté à notre population d'hémodialysés et élaboré en concertation pluridisciplinaire (cardiologues, orthopédistes, rhumatologues, kinésithérapeutes) paraît intéressant dans le cadre d'une prise en charge globale du patient hémodialysé chronique.
How Should Unfunded Social Security be Financed ?

The U.S. Social Security program is almost entirely nanced with payroll taxes. However, other taxes exist that could be used (at least partially) to nance Social Security bene ts. Without arguing in favour of the old and controversial idea of reallocating general tax revenue to nance Social Security, in this study a di¤erent question is asked: What type of economic activity is it more appropriate to tax in order to nance a given level of pension bene ts, while preserving the current Social Security systems bene t entitlements and pay-as-you-go nature, and not altering its legislated distributional aspects? To answer this question we build a calibrated general equilibrium model with rational and inattentive (low-saving) agents, uncertain longevity, income heterogeneity, endogenous retirement, Social Security, non-payroll taxes and productive government spending. We nd that lowering the payroll tax and re nancing Social Security with higher consumption taxes improves the welfare of the rich, factor prices and raises the GDP, while the welfare of the poor is nearly una¤ected. Despite potential concerns that in an economy where a sizable fraction of the population saves very little, making it unlikely for the capital stock to increase su¢ ciently in response to higher consumption taxes, we nd this is not the case in the general equilibrium environment with endogenous retirement. a JEL Classi cation: H55, E21, E62, D58
Review of standards for electronic imaging for facsimile systems

Although facsimile systems have existed for more than 25 years, only recently have we seen explosive growth in the use of facsimile, with a corresponding increase in facsimile standards activity. Current standards work concentrates on improving the quaity and speed of image transmission and on increasing functionality. The goal is to benefit from the developing technology-higher resolution scanners and printers, and improved processing, storage and communication technology. Topics now under discussion indude (but by no means are limited to) transmission of color images, improved compression of bilevel images, facsimile routing, facsimile carried by the Integrated Services Digital Network (ISDN), and facsimile applied to teleconferencing. Personal computers can operate as or can be used to add functionality to facsimile terminals. The result is new standards activity (for example, binary file transfer, database storage and retrieval, computer-facsimile communication, and an Applications Programming Interface). Facsimile and facsimile-related standards activity are described with particular emphasis on function and performance. Image compression algorithms are an important factor. Communication protocols are also addressed.
The Influence of Indian Ocean Atmospheric Circulation on Warm Pool Hydroclimate During the Holocene Epoch

Existing paleoclimate data suggest a complex evolution of hydroclimate within theIndo-Pacific Warm Pool (IPWP) during the Holocene epoch. Here we introduce a new leafwax isotope record from Sulawesi, Indonesia and compare proxy water isotope data withocean-atmosphere general circulation model (OAGCM) simulations to identifymechanisms influencing Holocene IPWP hydroclimate. Modeling simulations suggestthat orbital forcing causes heterogenous changes in precipitation across the IPWPon a seasonal basis that may account for the differences in time-evolution of the proxydata at respective sites. Both the proxies and simulations suggest that precipitationvariability during the September–November (SON) season is important for hydroclimatein Borneo. The preeminence of the SON season suggests that a seasonally laggedrelationship between the Indian monsoon and Indian Ocean Walker circulation influencesIPWP hydroclimatic variability during the Holocene.
Persistent Rash on the Face and Lips

A 52-year-old man was referred to the dermatology clinic for a persistent rash on his face and lips of 5 months' duration, despite the use of a mild steroid cream prescribed by his primary care physician. He noted that the rash had “seemed to spread” and was now on his hands as well. In addition, he had hard nodules on his fingers that had become noticeable in the previous 2 weeks. He reported a history of long-standing gastroesophageal reflux disease that was well controlled with proton pump inhibitors. Physical examination revealed matlike telangiectasias on the patient's face, lips, and hands (Figure ​(Figure11), hard subcutaneous nodules on fingers that appeared edematous and tight (Figures ​(Figures22 and ​and33), and a loud P2 component on cardiac auscultation. Upon further questioning, he reported a long history of cold intolerance, with cold weather causing aching hands that “turn blue. ” Figure 1 Matlike telangiectasias on the face and lips. Figure 2 Nonpitting edema of hands and fingers with matlike telangiectasias. The skin is waxy and tight with no hair on the dorsum of the hands. (File photo of another patient with the same disorder.) Figure 3 Subcutaneous calcium hydroxyapatite nodules in the fingertips. DIAGNOSIS: CREST syndrome.
El Paleolítico Medio y Superior en el sector central de Andalucía (Córdoba y Málaga)

Este trabajo de investigacion recurre al analisis de la cultura material (industrias litica y sobre asta/hueso, arte mobiliar y elementos de adorno) de 14 colecciones del yacimiento de Cueva Bajondillo (Torremolinos, Malaga) y otras 4 de El Pirulejo (Priego de Cordoba), contextualizadas a nivel cronologico, paleoambiental e historiografico: para el primer apartado disponemos de 23 dataciones absolutas (U/Th, AMS y TL) obtenidas en Cueva Bajondillo, en otros casos empleamos una asignacion cronologica de caracter relativo y en otras ocasiones recurrimos a los paralelos tecnotipologicos de las conjuntos. En el ambito paleoclimatico disponemos de estudios sobre los edificios travertinicos de Priego de Cordoba y Torremolinos, las dinamicas glacioeustaticas y litoral de la Bahia de Malaga y analisis litoestratigraficos y polinicos de Cueva Bajondillo. Por ultimo, hemos contrastado los datos obtenidos con la secuencia del Paleolitico Medio meridional y del Paleolitico Superior de ambito iberico. En el apartado metodologico hemos aplicado un sistema tecnologico de estudio de las industrias liticas talladas, que considera que los modelos de gestion de estas responden a las necesidades economicas y tecnofuncionales, asi como a las tradiciones culturales de las comunidades prehistoricas que las generaron. Las aportaciones cronologicas, paleoclimaticas o tecnoculturales de Cueva Bajondillo y El Pirulejo permiten abordar, con nuevos datos, la vertebracion de la secuencia cronologica, cultural y paleoambiental del Paleolitico Medio y Superior del sector central de Andalucia. Los datos litoestratigraficos y polinicos consolidan un modelo paleoambiental para el sur de la Peninsula Iberica que participa, sin ningun genero de duda aunque con los matices que impone su ubicacion geografica meridional, de los procesos climaticos de rango mundial que acontecieron durante el Pleistoceno Superior, suministra
Date me if you can: Ein systematischer Überblick über den aktuellen Forschungsstand von Online-Dating

Übersicht Online-Dating ist mittlerweile zum Massenphänomen geworden, was angesichts veränderter Lebensformen, zunehmender Technologisierung und verknappter Zeitressourcen nicht verwundert. Empirische Forschungsarbeiten zu Online-Dating werden seit 1995 in stetig wachsender Zahl veröffentlicht. Der vorliegende Beitrag fasst den aktuellen Forschungsstand in seiner thematischen Breite zusammen und behandelt zentrale Forschungsergebnisse aus den folgenden Schwerpunkten: Beliebte Dating-Plattformen und Dating-Apps, typische Nutzergruppen, Plattformauswahl, Profilerstellung, Partnerwahl, mediale Kommunikation und Outcome. In der Gesamtschau zeigt sich, dass Online-Dating eine alltägliche und mittlerweile gesellschaftlich akzeptierte Form der Kontaktanbahnung ist. Je nach Alter, Geschlecht, Persönlichkeit und Lebenssituation wird Online-Dating allerdings aus unterschiedlichen Motiven heraus genutzt, und auch Art und Umfang der Nutzung differieren. Daraus ergeben sich nicht nur unterschiedliche Dating-Resultate, sondern ebenfalls verschiedene Gefahrenpotenziale. Auf Forschungslücken in den einzelnen Themenbereichen wird hingewiesen.
Motion of the Human Mandibular Condyle during Mastication

It has been reported that loading to the mandible during closing movement makes the condylar path move more in the superior direction than that during the free closing movement. In this study, the hypothesis was tested that the displacement of the condyle on the chewing side is greater in the direction of the mandibular fossa than that on the non-chewing side. Using a six-degrees-of-freedom jaw movement recording system, we recorded condylar motion in 12 healthy adults without TMD, during the chewing of a large hard gummy jelly. The maximum displacements at the condyle on the chewing side from the maximum intercuspation (CO) position were significantly larger in the superior and medial directions at the initial stage and in the posterior direction at all stages (0.5 mm, 0.5 mm, and 0.6 mm, respectively) than those on the non-chewing side (0.0 mm, 0.1 mm, and 0.1 mm, respectively). This suggests that, in healthy adults, the condyles at CO are located in a position such that excessive load is not applied to the temporomandibular joint when there are the aforementioned displacements.
POLLINATOR CHOICE AND STABILIZING SELECTION FOR FLOWER COLOR IN DELPHINIUM NELSONII

Bright colors are characteristic of the flowers of many higher plants. For the last century these colors have been interpreted as adaptations by which flowers signal their presence to animal pollinators (Darwin, 1876; Muller, 1883; Plateau, 1899; Clements and Long, 1923; Kugler, 1943; Daumer, 1958; Heinrich, 1975; Jones, 1978; Kevan, 1978). If this is correct, the exact color(s) found within populations of single species, and the array of colors represented in an assemblage of sympatric species, may be explicable largely as evolutionary consequences of pollinator foraging behavior. Several plant species in the Rocky Mountains of Colorado exhibit polymorphisms well suited for studies of relationships between flower color, pollinator behavior, and plant fitness. Although flowers of these species are normally brightly colored, we have noticed rare individuals whose flowers are white or pale. Such color changes are likely to have a genetic basis, as we shall discuss later. Species with such flower color polymorphisms include Delphinium nelsonii Greene (Ranunculaceae), Lupinus argenteus Pursh (Leguminosae), Ipomopsis aggregata (Pursh) V. Grant (Polemoniaceae), Iris missouriensis Nutt. (Iridaceae), and Linum lewisii Pursh (Linaceae). The low frequencies of "albino" individuals in populations of these species suggest an equilibrium between their production by spontaneous mutation and some form of selection against them (cf. Crow and Kimura, 1970; Harding, 1970). Selection against albinos could take at least two forms. Mutations causing reduced flower pigmentation might be associated with physiological or developmental changes (e. g., reduced ovule number, stigma size, or receptivity) which lower intrinsic fecundity. Alternatively, pollinators might discriminate against albinos and thus exert stabilizing selection for flower color. In this paper we describe experiments and observations with one of the species listed above, Delphinium nelsonii, a small perennial herb whose flowers are normally deep blue. Near the Rocky Mountain Biological Laboratory (RMBL) in west-central Colorado, D. nelsonii is pollinated primarily by broad-tailed hummingbirds (Selasphorus platycercus Swainson) and bumblebee queens (B ombus appositus Cresson, B. flavifrons Cresson, B. californicus F. Smith, and B. nevadensis Cresson) (Waser, 1978; Price and Waser, 1979). We present evidence that paleor white-flowered albinos are rare in populations near the RMBL; that selection against albinos occurs in the form of reduced seed sets relative to normal blueflowered individuals; that intrinsic fecundity of albinos is not lower than that of normal individuals; that both hummingbirds and bumblebees discriminate against albinos in a way sufficient to explain their low seed sets and consequent rarity; and that discrimination may be due to difficulty pollinators experience in extracting nectar from albino flowers.
Rate of change in renal function and mortality in elderly treated hypertensive patients.

BACKGROUND AND OBJECTIVES Evidence relating the rate of change in renal function, measured as eGFR, after antihypertensive treatment in elderly patients to clinical outcome is sparse. This study characterized the rate of change in eGFR after commencement of antihypertensive treatment in an elderly population, the factors associated with eGFR rate change, and the rate's association with all-cause and cardiovascular mortality. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS Data from the Second Australian National Blood Pressure study were used, where 6083 hypertensive participants aged ≥65 years were enrolled during 1995-1997 and followed for a median of 4.1 years (in-trial). Following the Second Australian National Blood Pressure study, participants were followed-up for a further median 6.9 years (post-trial). The annual rate of change in the eGFR was calculated in 4940 participants using creatinine measurements during the in-trial period and classified into quintiles (Q) on the basis of the following eGFR changes: rapid decline (Q1), decline (Q2), stable (Q3), increase (Q4), and rapid increase (Q5). RESULTS A rapid decline in eGFR in comparison with those with stable eGFRs during the in-trial period was associated with older age, living in a rural area, wider pulse pressure at baseline, receiving diuretic-based therapy, taking multiple antihypertensive drugs, and having blood pressure <140/90 mmHg during the study. However, a rapid increase in eGFR was observed in younger women and those with a higher cholesterol level. After adjustment for baseline and in-trial covariates, Cox-proportional hazard models showed a significantly greater risk for both all-cause (hazard ratio, 1.28; 95% confidence interval, 1.09 to 1.52; P=0.003) and cardiovascular (hazard ratio, 1.40; 95% confidence interval, 1.11 to 1.76; P=0.004) mortality in the rapid decline group compared with the stable group over a median of 7.2 years after the last eGFR measure. No significant association with mortality was observed for a rapid increase in eGFR. CONCLUSIONS In elderly persons with treated hypertension, a rapid decline in eGFR is associated with a higher risk of mortality.