Hematology oncology practice in the Asia-Pacific APHCON survey results from the 6th international hematologic malignancies conference: bridging the gap 2015, Beijing, China

This report serves as a snapshot of the state-of-knowledge in the Asia Pacific (APAC) Hematology Oncology community, and establishes a baseline for longitudinal investigations to follow changes in best practices over time. The objective of this study was to understand the approach to hematologic diseases, common standards of care and best practices, issues that remain controversial or debated, and educational or resource gaps that warrant attention. We used mobile application to disseminate and distribute questionnaires to delegates during the 6th international hematologic malignancies conference hosted by the APAC Hematology Consortium at Beijing, China. User responses were collected in an anonymous fashion. We report survey results in two ways: the overall responses, and responses as stratified between Chinese physicians and 'Other' represented nationalities. Overall geographical concordance in survey responses was positive and strong. Perhaps more interesting than instances of absolute agreement, these data provide a unique opportunity to identify topics in which physician knowledge or opinions diverge. We assigned questions from all modules to broad categories of: patient information; diagnosis; treatment preference; transplantation; and general knowledge/opinion. On average, we observed a geographic difference of 15% for any particular answer choice, and this was fairly constant across survey modules. These results reveal utility and need for widespread and ongoing initiatives to assess knowledge and provide evidence-based education in real time. The data will be made more valuable by longitudinal participation, such that we can monitor changes in the state of the art over time.published_or_final_versio

High-resolution spatio-temporal analyses of drought episodes in the western Mediterranean basin (Spanish mainland, Iberian Peninsula)

12 Pags.- 6 Figs.- 1 Tabl. © The Author(s) 2018. Open Access. This article is distributed under the terms of the Creative Commons Attribution 4.0 International License, which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made.The purpose of this research was to identify major drought events on the Spanish mainland between 1961 and 2014 by means of two drought indices, and analyze the spatial propagation of drought conditions. The indices applied were the standardized precipitation index (SPI) and the standardized evaporation precipitation index (SPEI). The first was calculated as standardized anomalies of precipitation at various temporal intervals, while the second examined the climatic balance normalized at monthly scale, incorporating the relationship between precipitation and the atmospheric water demand. The daily meteorological data from Spanish Meteorological Archives (AEMet) were used in performing the analyses. Within the framework of the DESEMON project, original data were converted into a high spatial resolution grid (1.1 km2) following exhaustive quality control. Values of both indices were calculated on a weekly scale and different timescales (12, 24 and 36 months). The results show that during the first half of the study period, the SPI usually returned a higher identification of drought areas, while the reverse was true from the 1990s, suggesting that the effect from atmospheric evaporative demand could have increased. The temporal propagation from 12- to 24-month and 36-month timescales analyzed in the paper seems to be a far from straightforward phenomenon that does not follow a simple rule of time lag, because events at different temporal scales can overlap in time and space. Spatially, the propagation of drought events affecting more than 25% of the total land indicates the existence of various spatial gradients of drought propagation, mostly east–west or west–east, but also north–south have been found. No generalized episodes were found with a radial pattern, i.e., from inland to the coast.This work is supported by the projects CGL2014-52135-C03-01, CGL2014-52135-C3-3-R and PCIN-2015-220, financed by the Spanish Government, Ministry of the Economy and Finance and FEDER, IMDROFLOOD financed by the Water Works 2014 co-funded call of the European Commission, INDECIS, financed by the ERA-NET Cofund for Climate Services of the European Research Area for Climate Services, and the Regional Government of Aragón DGA-FSE (Grupo de Investigación Consolidado ‘Clima, Agua, Cambio Global y Sistemas Naturales’). Celia Salinas is a FPI-PhD student supported by the Ministry of the Economy and Finance. Miquel Tomas-Burguera was supported by the predoctoral FPU program 2013 (Ministry of Education, Culture and Sport).Peer reviewe

Identification of genetic variants associated with Huntington's disease progression: a genome-wide association study

Huntington's disease is caused by a CAG repeat expansion in the huntingtin gene, HTT. Age at onset has been used as a quantitative phenotype in genetic analysis looking for Huntington's disease modifiers, but is hard to define and not always available. Therefore, we aimed to generate a novel measure of disease progression and to identify genetic markers associated with this progression measure

Clinical and genetic characteristics of late-onset Huntington's disease

Background: The frequency of late-onset Huntington's disease (>59 years) is assumed to be low and the clinical course milder. However, previous literature on late-onset disease is scarce and inconclusive. Objective: Our aim is to study clinical characteristics of late-onset compared to common-onset HD patients in a large cohort of HD patients from the Registry database. Methods: Participants with late- and common-onset (30\u201350 years)were compared for first clinical symptoms, disease progression, CAG repeat size and family history. Participants with a missing CAG repeat size, a repeat size of 6435 or a UHDRS motor score of 645 were excluded. Results: Of 6007 eligible participants, 687 had late-onset (11.4%) and 3216 (53.5%) common-onset HD. Late-onset (n = 577) had significantly more gait and balance problems as first symptom compared to common-onset (n = 2408) (P <.001). Overall motor and cognitive performance (P <.001) were worse, however only disease motor progression was slower (coefficient, 120.58; SE 0.16; P <.001) compared to the common-onset group. Repeat size was significantly lower in the late-onset (n = 40.8; SD 1.6) compared to common-onset (n = 44.4; SD 2.8) (P <.001). Fewer late-onset patients (n = 451) had a positive family history compared to common-onset (n = 2940) (P <.001). Conclusions: Late-onset patients present more frequently with gait and balance problems as first symptom, and disease progression is not milder compared to common-onset HD patients apart from motor progression. The family history is likely to be negative, which might make diagnosing HD more difficult in this population. However, the balance and gait problems might be helpful in diagnosing HD in elderly patients