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Periurban forests are key to offsetting anthropogenic carbon emissions, but they are under constant threat from urbanization. In particular, secondary Neotropical forest types in Andean periurban areas have a high potential to store carbon, but are currently poorly characterized. To address this lack of information, we developed a method to estimate periurban aboveground biomass (AGB)—a proxy for multiple ecosystem services—of secondary Andean forests near Bogotá, Colombia, based on very high resolution (VHR) GeoEye-1, Pleiades-1A imagery and field-measured plot data. Specifically, we tested a series of different pre-processing workflows to derive six vegetation indices that were regressed against in situ estimates of AGB. Overall, the coupling of linear models and the Ratio Vegetation Index produced the most satisfactory results. Atmospheric and topographic correction proved to be key in improving model fit, especially in high aerosol and rugged terrain such as the Andes. Methods and findings provide baseline AGB and carbon stock information for little studied periurban Andean secondary forests. The methodological approach can also be used for integrating limited forest monitoring plot AGB data with very high resolution imagery for cost-effective modelling of ecosystem service provision from forests, monitoring reforestation and forest cover change, and for carbon offset assessments.

Trends in studies on autism in Spain: Publications and authorship networks (1974-2007). All the papers on autism published in journals by Spanish authors until 2007 were reviewed in order to identify changes and trends in studies, journals and authorship networks. A total of 567 works were analyzed. Results showed a continuous increase in the total number of publications and collaborative works (especially from 1999 onwards), as well as coincidence of the general framework of papers with the guidelines for research and treatment on autism that were published in the Anglo-Saxon arena for the same period. Some weak points were also identified, such as the low proportion of empirical and funded studies, low impact of the journals, and low author continuity, which have also been noted for other domains of research in Spain. We conclude that psychological and biomedical research on autism is currently a growing field in our country. However, important changes are needed, both in the way the authors conduct and communicate their studies, and in the commitment of some institutions (specially, universities and parent advocacy groups). Lastly, some proposals are suggested to improve the scientific quality of future studies and their usefulness for people with autism. [ABSTRACT FROM AUTHOR]

The great variability in the clinical presentation of Fabry Disease (FD), the difficulties for its diagnosis, and the availability of several alternatives for its treatment, are a great challenge for the professionals who treat patients with FD, that justify the elaboration of an evidence based clinical practice guideline (CPG) to help decision-making in the management of Fabry patients. METHODS: We applied both the guidances from NICE guideline development methods and GUIASALUD (Spanish NHS Guideline Methods). We incorporated GRADE methodology (Grading of Recommendations Assessment) for the evaluation of quality of scientific evidence and the development and weighing of recommendations. For the final elaboration of these recommendations, a structured consensus process was carried out with a panel of 9 experts proposed by different scientific societies, research centers and patients' associations in two rounds using the Delphi-RAND method. Finally, the proposed guideline was reviewed by stakeholders (pharma industries, scientific societies and patient associations). The guideline maintains a living process for annual updating. RESULTS: thirty-two PICO clinical questions were considered, gathered in 9 different steps of the process of care. Tables were prepared for the synthesis and evaluation of the quality of the evidence for each of the questions. Regarding the quality of the evidence, balance between benefits and risks, values and preferences of patients, equity and use of resources were considered. The FD-Guideline contains 92 specific recommendations for the management of FD including chapters on when to suspect the disease or how to support social and non-medical outcomes for patients and families. CONCLUSIONS: An evidence based CPG about Fabry disease is now available for both health care workers involved in this disease and patients. [ABSTRACT FROM AUTHOR]

The aim is to conduct a descriptive, population-based study in order to assess temporal and spatial changes in mortality due to granulomatosis with polyangiitis (GPA) in Spain from 1984 to 2016. Mortality data were obtained from the Spanish Annual Death Registry. Deaths in which GPA was the underlying cause were selected using the 446.4 and M31.3 codes from the International Classification of Diseases, 9th and 10th revision. Annual average age at death and age-adjusted mortality rates were calculated. Geographic analysis was performed at municipality and district level. Variations in mortality according to the type of municipality (urban, agro-urban or rural), district and geographic location (degrees of latitude) were assessed using standardized mortality ratios (SMRs) and smoothed-SMRs. Over the whole period, 620 deaths due to GPA were identified. Age at death increased at an average annual rate of 0.78% over the period 1987-2016 ( p < 0.05). Age-adjusted mortality rates increased by an annual average of 20.58% from 1984 to 1992, after which they fell by 1.91% a year ( p < 0.05). The agro-urban category had the highest percentage (4.57%) of municipalities with a significantly higher GPA mortality rate than expected. Geographic analysis revealed four districts with a higher risk of death due to GPA, two in the North of Spain and two in the South. This population-based study revealed an increase in the age at death attributed to GPA. Age-adjusted mortality rates went up sharply until 1992, after which they started to decline until the end of the study period. Geographic differences in mortality risk were identified but further studies will be necessary to ascertain the reasons for the distribution of GPA disease., Competing Interests: The authors declare no conflict of interest.

A large number of studies have reported on the validity of autism spectrum disorder (ASD) screening procedures. An overall understanding of these studies' findings cannot be based solely on the level of internal validity of each, since screening instruments might perform differently according to certain factors in different settings. Europe has led the field with the development of the first screening tool and first prospective screening study of autism. This paper seeks to provide an overview of ASD screening studies and ongoing programmes across Europe, and identify variables that have influenced the outcomes of such studies. Results show that, to date, over 70,000 children have been screened in Europe using 18 different screening procedures. Differences among findings across studies have enabled us to identify ten factors that may influence screening results. Although it is impossible to draw firm conclusions as to which screening procedure is most effective, this analysis might facilitate the choice of a screening method that best fits a specific scenario, and this, in turn, may eventually improve early ASD detection procedures.

Rare Diseases Epidemiology is a novel action field still largely unexplored. However, Rare Diseases is a topic of growing interest at world level. The aims of this chapter are to revise useful epidemiological tools and define areas where epidemiology can help improve the rare disease knowledge, and facilitate policy decisions taking into account the real burden of rare diseases in society. This chapter also seeks to describe: the problems of coding and classification of diseases, measuring disease frequency, the study designs and association studies, the causality, the evolution from descriptive to epigenetic epidemiology and the natural history of disease. One of the major challenges facing analytical epidemiology and clinical epidemiological research into rare diseases is that genes can be involved in both aetiology and prognosis. Despite the many similarities between genetic association studies and classic observational epidemiological studies, the former pose several specific limitations, including an unprecedented volume of new data and the likelihood of very small individual effects, as well other limitations. Selecting the appropriate pathway from among all those available, i.e. the one that best relates genes from the various known regions and disease mechanisms, is crucial for the success of this type of studies.

The rare disease community suffers from the absence of reliable epidemiological data on the prevalence and incidence of rare diseases in the national and global populations. The rare diseases community includes all of the stakeholders involved in the research and development and dissemination of products and information for the diagnosis, prevention or treatment of rare diseases or conditions. To replace many of the perceived myths with realities, several global efforts are required if we are going to sustain and increase the reported progress with the thousands of rare diseases. One is the identification and expansion of worldwide partnerships and collaborations of Patient Advocacy Groups for individual rare diseases. Another requirement is to develop a global research infrastructure of qualified investigators to stimulate and coordinate research efforts by seeking ways to provide access to clinical trials at multi-national research sites with common protocols and multi-disciplinary research teams. Providing ready access to the information about rare diseases, patient advocacy groups, research studies and products in research protocols will continue to improve the lives of patients and their families. Many scientists, public and private sector organizations, patient advocacy groups, foundations, and the pharmaceutical, biotechnology, and medical devices industries are committed to translating research discoveries that will be useful in the care of patients with rare diseases over their lifespan. Evidence from well constructed epidemiological studies will provide the evidence that point to the value of additional clinical studies to increase the understanding of rare diseases.

Objective: Scleroderma (systemic sclerosis) is a rare disease that results in great suffering. We estimated the burden of disease posed by scleroderma and its relative importance in the health of the Spanish population., Methods: We estimated scleroderma-based burden of disease following procedures developed for the Global Burden of Disease study to ensure comparability., Results: Despite its low prevalence, scleroderma generated 1732 disability-adjusted life-years (DALY) in Spain in 2001, comprising 562 (32%) years of life lost and 1170 (68%) years lived with disability. Most scleroderma-related DALY (73%) occurred in the population aged 15-54 years. Estimated DALY accounted for 0.59% of other musculoskeletal disorder-related DALY in the European A subregion (countries with low mortality rate in both adults and children in the World Health Organization classification), a significant value in the overall burden of disease., Conclusion: The burden of scleroderma in Spain was high, with disability being the major contributing factor. Burden of disease is an important measure in rare diseases, and may be an important indicator to be considered as a health unit in developed countries.

Aim: This paper reports an investigation of the quality of life of patients most severely affected by toxic oil syndrome and the association between quality of life and disability variables, handicaps and depression., Background: In 1981, an epidemic occurred in Central and Northwestern Spain. The illness, now called toxic oil syndrome (TOS), appeared to be caused by consumption of oil mixtures containing rapeseed oil denatured with aniline and affected over 20,000 individuals. Today, it is classified as a chronic disease that has resulted in a large number of sequelae., Method: From the 1031 people classified with permanent disability, a random sample of 292 individuals was selected. A structured interview was carried out to investigate the clinical aspects. The Nottingham Health Profile Questionnaire was used to assess quality of life. To study the relationship between quality of life and disability, handicap and depression, a univariate analysis was carried out. The chi-square test was used for inter-group comparisons. A P-value of 0.05 was adopted as the limit for inclusion of a variable in the multivariate analysis. Multivariate analysis was undertaken using logistic regression., Results: Two hundred and fourteen patients were interviewed (79% response rate). Mean age was 59.3 (sd: 12.7) years. Quality of life mean score was 52.8 (sd: 22.6). The variables associated with poorer quality of life perception were depression [odds ratio (OR) = 9.66, confidence interval (CI) = 3.71-25.15], role-related disabilities (occupation role: OR = 2.82 CI = 1.28-6.22) and mobility (bending/picking up: OR = 4.74, CI = 2.31-9.72), as well as economic problems (OR = 1.62, CI = 1.07-2.46)., Conclusion: The quality of life of those most severely affected by TOS is poor. Their health profile is characterized by depression and important functional and psycho-social disabilities that limit daily living activities and social roles, and are related to self-perceptions of poor quality of life.

Toxic oil syndrome (TOS) resulted from consumption of rapeseed oil denatured with 2% aniline and affected more than 20,000 persons. Eighteen years after the epidemic, many patients continue to report neurologic symptoms that are difficult to evaluate using conventional techniques. We conducted an epidemiologic study to determine whether an exposure to toxic oil 18 years ago was associated with current adverse neurobehavioral effects. We studied a case group of 80 adults exposed to toxic oil 18 years ago and a referent group of 79 adult age- and sex-frequency-matched unexposed subjects. We interviewed subjects for demographics, health status, exposures to neurotoxicants, and responses to the Kaufman Brief Intelligence Test (K-BIT), Programa Integrado de Exploracion Neuropsicologica (PIEN), and Goldberg depression questionnaires and administered quantitative neurobehavioral and neurophysiologic tests by computer or trained nurses. The groups did not differ with respect to educational background or other critical variables. We examined associations between case and referent groups and the neurobehavioral and neurophysiologic outcomes of interest. Decreased distal strength of the dominant and nondominant hands and increased vibrotactile thresholds of the fingers and toes were significantly associated with exposure to toxic oil. Finger tapping, simple reaction time latency, sequence B latency, symbol digit latency, and auditory digit span were also significantly associated with exposure. Case subjects also had statistically significantly more neuropsychologic symptoms compared with referents. Using quantitative neurologic tests, we found significant adverse central and peripheral neurologic effects in a group of TOS patients 18 years after exposure to toxic oil when compared with a nonexposed referent group. These effects were not documented by standard clinical examination and were found more frequently in women.

In 1981, in Spain, the ingestion of an oil fraudulently sold as olive oil caused an outbreak of a previously unrecorded condition, later known as toxic oil syndrome (TOS), clinically characterized by intense incapacitating myalgias, marked peripheral eosinophilia, and pulmonary infiltrates. Of the 20,000 persons affected, approximately 300 died shortly after the onset of the disease and a larger number developed chronic disease. For more than 15 years, a scientific committee supported by the World Health Organization's Regional Office for Europe and by the Institute of Health Carlos III in Madrid has guided investigation intended to identify the causal agent(s), to assess toxicity and mode of action, to establish the pathogenesis of the disease, and to detect late consequences. This report summarizes advances in research on this front. No late mortality excess has been detected. Among survivors, the prevalence of some chronic conditions (e.g., sclerodermia, neurologic changes) is high. Attempts to reproduce the condition in laboratory animals have been unsuccessful, and no condition similar to TOS has been reported in the scientific literature. Laboratory findings suggest an autoimmune mechanism for TOS, such as high levels of seric soluble interleukin-2 receptor. Epidemiologic studies integrated with chemical analyses of case-related oils have shown that the disease is strongly associated with the consumption of oils containing fatty acid esters of 3-(N-phenylamino)-1,2-propanediol (PAP). These chemicals have also been found in oils synthesized under conditions simulating those hypothesized to have occurred when the toxic oil was produced in 1981. Whether PAP esters are simply markers of toxicity of oils or have the capability to induce the disease remains to be elucidated.