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Background

Frequent painful vaso-occlusive crises (VOCs) were associated with mortality in the Cooperative Study of Sickle Cell Disease (CSSCD) over twenty years ago. Modern therapies for sickle cell anemia (SCA) like hydroxyurea are believed to have improved overall patient survival. The current study sought to determine the relevance of the association between more frequent VOCs and death and its relative impact upon overall mortality compared to other known risk factors in a contemporary adult SCA cohort.

Methods

Two hundred sixty four SCA adults were assigned into two groups based on patient reported outcomes for emergency department (ED) visits or hospitalizations for painful VOC treatment during the 12 months prior to evaluation.

Results

Higher baseline hematocrit (p = 0.0008), ferritin (p = 0.005), and HDL cholesterol (p = 0.01) were independently associated with 1 or more painful VOCs requiring an ED visit or hospitalization for acute pain. During a median follow-up of 5 years, mortality was higher in the ED visit/hospitalization group (relative risk [RR] 2.68, 95% CI 1.1-6.5, p = 0.03). Higher tricuspid regurgitatant jet velocity (TRV) (RR 2.41, 95% CI 1.5-3.9, p < 0.0001), elevated ferritin (RR 4.00, 95% CI 1.8-9.0, p = 0.001) and lower glomerular filtration rate (RR=2.73, 95% CI 1.6-4.6, p < 0.0001) were also independent risk factors for mortality.

Conclusions

Severe painful VOCs remain a marker for SCA disease severity and premature mortality in a modern cohort along with other known risk factors for death including high TRV, high ferritin and lower renal function. The number of patient reported pain crises requiring healthcare utilization is an easily obtained outcome that could help to identify high risk patients for disease modifying therapies.

Trial Registration

ClinicalTrials.gov NCT00011648 http://clinicaltrials.gov/  相似文献   
3.
Effects of medium viscosity on kinetic parameters of poly(U) hydrolysis catalyzed by RNase from Bac. intermedius 7P (binase) were studied in solutions of sucrose (4-50 wt. %) and glycerol (35-62 wt. %) in Tris--sodium acetate buffer (pH 7.5) at 25 degreesC. The rate constant of reaction kcat was practically unchanged over a wide range of viscosities (1-15 cP for sucrose and 2.5-3 cP for glycerol). In glycerol solutions, kcat slightly increased with viscosity increase from 4 to 10 cP. Addition of NaCl to the buffer medium resulted in an inhibitory effect of Na+ on kcat, prevented by 50% sucrose or 60% glycerol. It is concluded that binase-catalyzed poly(U) cleavage occurs through a "tense"-substrate mechanism, similarly to reactions catalyzed by alpha-chymotrypsin, trypsin, and laccase.  相似文献   
4.
Summary : FT is a tool written in C++, which implements the Fourier analysis method to locate periodicities in aminoacid or DNA sequences. It is provided for free public use on a WWW server with a Java interface. Availability : The server address is http://o2.db. uoa.gr/FT Contact : shamodr@atlas.uoa.gr   相似文献   
5.
Cicadas are widely distributed in the Mediterranean area and are mainly distinguished by male acoustic signals, which act as specific mate recognition systems. Within the genus Cicada L. several species have diverged in their calling songs without showing external morphological differences, so acoustic recordings and genetic studies are particularly useful for systematic, biogeography and evolutionary studies. This study assesses sequence variation in closely related species of Cicada from the Mediterranean area, using domain III of the 12S rRNA mitochondrial gene in order to determine the phylogenetic relationships and the evolutionary history of this group, as well as the population structure of the two most common species, C. orni and C. barbara. Five distinct haplogroups were identified, C. orni, C. barbara, C. mordoganensis, C. cretensis and C. lodosi, each corresponding to a distinct evolutionary group. C. barbara was the most divergent species within this group, while C. orni and C. mordoganensis were the most similar. The population structure and demographic parameters of the species were not completely resolved. However, there is evidence for the separation of the C. orni Greek populations from the rest of Europe and also for demographic expansions probably related to Pleistocene climate changes. © 2009 The Linnean Society of London, Zoological Journal of the Linnean Society, 2009, 155 , 266–288.  相似文献   
6.
Desjardins, P.R., Mángano, M.G., Buatois, L.A. & Pratt, B.R. 2010: Skolithos pipe rock and associated ichnofabrics from the southern Rocky Mountains, Canada: colonization trends and environmental controls in an early Cambrian sand‐sheet complex. Lethaia, 10.1111/j.1502‐3931.2009.00214.x The Lower Cambrian Gog Group of the southern Rocky Mountains of western Canada offers an opportunity to explore animal–sediment relationships in a high‐energy setting, during the early phase of Phanerozoic diversification. Its strata record a sand‐sheet complex on the broad pericontinental shelf of West Laurentia. Six ichnofabrics are recognized: Skolithos IF‐1 characterized by Skolithos linearis in planar cross‐stratified sandstone; Skolithos IF‐2 with Skolithos linearis and accessory Diplocraterion parallelum in ripple cross‐laminated, and planar and trough cross‐stratified sandstone; Skolithos IF‐3 with S. linearis and Planolites montanus in wavy‐ and flaser‐bedded sandstone, interfingering with bioturbated mudstone exhibiting P. montanus and Teichichnus rectus; Skolithos IF‐4, characterized by abundant Skolithos linearis in planar and trough cross‐stratified sandstone; Rosselia IF‐1 consisting of Rosselia isp. in planar cross‐stratified sandstone; and Rosselia IF‐2 consisting of scattered small Rosselia isp. in hummocky, cross‐stratified sandstone. The presence of contrasting ichnofabrics within a single early Cambrian sand‐sheet complex illuminates how the colonization trends of suspension and detritus feeders were controlled by factors specific to the various sub‐environments. □Gog Group, Ichnofabric, Lower Cambrian, Pipe Rock, Rosselia, Skolithos.  相似文献   
7.
The simultaneous study of the temporal dynamics of foraging behaviour, diet and seed abundance is essential to assess the way in which resources affect the behaviour and ecology of harvester ants. Here, we evaluate how fluctuations in grass seed abundance during three consecutive growing seasons influenced the foraging behaviour and diet of the harvester ants Pogonomyrmex rastratus, P. mendozanus and P. inermis in the central Monte desert, Argentina. Seed abundance of the most consumed grasses varied greatly through ant activity season, and ants altered their foraging behaviour in response to those changes. Foragers spent more time travelling and searching for food, and their foraging trips took longer during the low seed availability season. Foraging distance was very similar among species and, contrary to our expectations, did not vary between seasons. Foraging success of P. rastratus and P. inermis increased during the high availability season. This matched the seasonal pattern of foraging activity, suggesting that colonies may detect seed abundance and regulate their foraging effort with the rate of forager success. Although grass seeds were the main item in the diet of the three species, P. mendozanus, and to a lesser extent P. rastratus, turned more generalist when grass seeds were scarce. In contrast, P. inermis showed a very narrow diet breadth, only harvesting grass seeds in both seasons. Our results indicate the relevance of seed availability on foraging behaviour of harvester ants, which should be taken into account when predicting and evaluating the effect of ants on seed resources as well as numerical responses of harvester ant populations to the temporal and spatial variations in grass seed abundance.  相似文献   
8.
The role played by abandoned nests of leaf‐cutting ants (Atta spp.) as a small‐scale disturbance regime that affects plant recruitment, species coexistence and forest regeneration remains poorly investigated. Here we examine whether abandoned nests of Atta cephalotes serve as regeneration niches and operate as particular plant recruitment habitats, favouring forest regeneration after ant activities cease and leading to the establishment of taxonomically/ecologically distinct plant assemblages. Soil properties, canopy openness, light availability and regenerating plant assemblages were evaluated across 18 nests and adjacent control plots in a large remnant of Atlantic Forest in north‐east Brazil from December 2004 to December 2005. Surprisingly, nests and control plots exhibited very similar light environments irrespective of nest age, but nest soils exhibited substantial reductions in carbon content (1.45 ± 0.24 vs. 1.79 ± 0.13%) and organic matter (2.50 ± 0.41 vs. 3.08 ± 0.23%), and proved to be much more resistant to penetration (30.57 ± 6.08 vs. 39.48 ± 7.53 mm). Functional signature of regenerating plant assemblages exhibited little variation across both habitat types, as they were dominated by pioneer, small‐seeded and vertebrate‐dispersed species. However, abandoned nests exhibited less dense, impoverished and more homogeneous regenerating plant assemblages at local and landscape scale; they clearly lacked nest‐dependent plant species and represented floristic subsets of the flora inhabiting the undisturbed forest. This recruitment bottleneck was transient in the long term because nest‐related effects ameliorated in older nests. Our results suggest that, unlike treefall gaps, abandoned nests represent temporary (relatively long‐lasting) islands of unsuitable substrate that reduce plant recruitment, retard forest regeneration, and fail in providing a special regeneration niche able to promote species coexistence and plant diversity.  相似文献   
9.
Abstract: Since 1997 the Mexican government has promoted Management Units for Conservation and Sustainable Use of Wildlife (UMAs) on private and community lands as an economically attractive mean for the conservation of biodiversity. To date, compliance of UMAs with stated sustainability goals has not been evaluated. Thus, we designed multicriteria evaluation framework based on the stated objectives of the national UMA program and applied it to a sample of 6 UMAs operating in peasant communities near or in the Calakmul Biosphere Reserve in Campeche, Mexico. Evaluation criteria covered thematic areas of environment, economy, social development, and laws and rules. We formulated 15 criteria with 29 indicators, and 133 verifiers. Data for the verifiers were based on direct observation, interviews with key actors in the peasant communities, participation in regional meetings, scrutiny of government reports, and databases. We calculated sustainability indexes by weighting, summing, and standardizing verifiers to percentage scales, and aggregating to the successive higher hierarchical levels of the evaluation framework. We found an average overall sustainability index of 45.7% for the UMAs evaluated. Scores were highest for the environment thematic area (55.2%), followed by economy (43.4%), social development (42.3%), and laws and rules (41.7%). We observed particularly low indicators for management strategies, habitat monitoring, environmental education, and knowledge of wildlife laws. We conclude that the contribution of UMAs to the conservation and sustainable use of biodiversity could be improved by a number of actions. These include developing wildlife management educational programs with a strong environmental legislation component, the correct enforcement of wildlife laws, and creating country-wise links among academic and governmental institutions promoting the work with regional and national wildlife experts.  相似文献   
10.
Morphological variation of the Y chromosome has been observed in different populations. This variation is mostly related to the heteromorphic Yq12 band, which is composed of a variable block of constitutive heterochromatin. The Yqh+ heteromorphism has a worldwide frequency of 2.85% and is considered clinically innocuous. The aim of this study was to identify the ancestry of the Yqh+ heteromorphism present in individuals from western Mexico. For this purpose, 17 Y-chromosome single nucleotide polymorphisms were analysed by multiplex polymerase chain reaction and SNaPshot assays. In 28 Yqh+ males, only five haplogroups were observed; with a haplogroup diversity of 0.4841 ± 0.1094, which was less than that observed in a study of unselected Mexican mestizo population. Differences were specifically conferred by the high frequencies of haplogroups R1b1 and P*(xQ,R), and by the absence of the Amerindian haplogroup Q (Q*(xQ1a3a) plus Q1a3a) from the Yqh+ group. This study suggests a post-1492 incorporation for Yqh+ chromosomes into the Mexican northwestern population.  相似文献   
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