bluuebunny/crossref_metadata_2025_split huggingface dataset:
Founder Effect of the 669insA Mutation in BSCL2 Gene Causing Berardinelli‐Seip Congenital Lipodystrophy in a Cluster from Brazil
Authors: Gomes K. B. , Pardini V. C. , Ferreira A. C. S. , Fonseca C. G. , Fernandes A. P. 
Published: May 2007

SummaryCongenital generalized lipodystrophy (CGL) or Berardinelli‐Seip Syndrome (BSCL) is a rare autosomal recessive disease characterized by a nearly‐complete absence of adipose tissue from birth and severe metabolic alterations. The 669insA mutation in exon 4 of the BSCL2 gene was identified as the major genetic alteration leading to BSCL in a group of 22 patients from the northeastern Brazilian state of Rio Grande do Norte. Aiming to investigate the causes of the high frequency of BSCL in this region, a molecular genetic study was conducted using eight microsatelite markers located in chromosome 11. Additional investigations concerning the proportion of expected homozygous and heterozygous individuals, genetic diversity, fixation index and coefficient of endogamy were undertaken, and indicated significant differences by comparing the allelic and haplotypic frequencies observed for the BSCL affected families and the control group. It was concluded that a founder effect, genetic drift and consanguineous marriages have significantly affected the structure of this population, resulting in the highest frequency of BSCL in Brazil.
Variation in 5' Promoter Region of the APOE gene Contributes to Predicting Ischemic Heart Disease (IHD) in the Population at Large: the Copenhagen City Heart Study
Authors: Stengård J. H. , Frikke‐Schmidt R. , Tybjærg‐Hansen A. , Nordestgaard B.G. , Sing C.F. 
Published: May 2007

SummaryThe objective of this study was to evaluate whether an increased hazard of developing ischemic heart disease (IHD) is associated with any of the three genotypes A560T832/A560T832, A560T832/A560G832 and A560T832/T560T832, defined by variations in two non‐coding SNPs in the 5′ promoter region of the apolipoprotein E (APOE) gene. These genotypes were selected because they distinguished between high and low levels of HDL‐C, TG and/or T‐C in our earlier study of multiple samples defined by gender and population. We found a significant increase (p<0.05) in the hazard of IHD in females with the A560T832/T560T832 genotype that remained significant after fitting the effects of dyslipidemia, other established risk factors, and the structural isoform variations of the ApoE molecule. We discuss why this statistically significant genetic predictor may not be an appropriate screening test for IHD in the Danish population at large.
Evaluating the impact of public health notification of suspected transfusion‐transmissible hepatitis C virus infection and effectiveness of lookback and traceback investigations by Canadian Blood Services in British Columbia, Canada, August 2002 through February 2005
Authors: Whitlock Mandy , Lord Sandra , Buxton Jane A. , Doyle Patrick , Bigham Mark 
Published: May 2007

BACKGROUND:Suspected transfusion‐transmissible infections (TTIs) have been reported to public health (PH) in British Columbia (BC) since August 2002. The impact of PH notification of suspected transfusion‐transmissible hepatitis C virus (TT‐HCV) infection over the first 2.5 years and the effectiveness of HCV lookback (LB) and traceback (TB) investigations conducted by Canadian Blood Services (CBS) in BC were evaluated.STUDY DESIGN AND METHODS:Suspected TT‐HCV cases reported to CBS in BC between August 28, 2002, and February 28, 2005, were analyzed. The incremental yield of plausible TTIs from PH‐reported suspected TTIs was calculated. The effectiveness of LB and TB investigations was assessed with respect to the impact of improved anti‐HCV donor screening, the number of newly recognized HCV infections, and the timeliness of initiating investigations.RESULTS:Nine of 553 (1.6%) investigations were initiated after PH reporting, yielding an additional 2 of 237 (i.e., 0.8%) plausible TTIs. Ninety‐two percent of investigations with transfused units involved transfusions before implementing second‐generation anti‐HCV enzyme immunoassay (EIA) donor screening. Almost one‐third of HCV‐infected persons in linked investigations (i.e., LB triggered by a TB and vice versa) were newly identified. Recently tested, PH‐reported cases incurred a mean delay exceeding 6 months until initiating a LB or TB investigation.CONCLUSION:PH reporting of TTIs and investigating transfusions after second‐generation anti‐HCV EIA donor screening identified few plausible TT‐HCV infections. Many HCV‐infected recipients or lapsed donors first became aware of their infection status as a result of CBS investigations. The current process of reporting suspected TTIs incurs significant time delay.
Mapping of Epicardial Activation in a Rabbit Model of Chronic Myocardial Infarction:
Authors: WALKER NICOLA L. , BURTON FRANCIS L. , KETTLEWELL SARAH , SMITH GODFREY L. , COBBE STUART M. 
Published: May 2007

Introduction: This study examines the consequences of a large transmural apical infarct on the epicardial electrical activity in isolated rabbit hearts. Methods and Results: Hearts were isolated 8 weeks after coronary artery ligation. Membrane voltage from the epicardial surface of the left ventricle (LV) including the infarct was monitored using the voltage sensitive dye RH237. Optical action potentials were detected from the epicardial surface of the infarct; the signal amplitude was ∼20% of those in the noninfarcted zone (NZ). Epicardial activation mapping of the LV free wall showed that during right atrial (RA) pacing, the activation sequence was not significantly different between infarcted and sham‐operated groups. However, direct stimulation of the epicardium in the NZ revealed an area of slow conduction velocity (CV ∼5 cm/s−1, ∼10% of normal values) at the margin of the infarct zone (IZ). Within the IZ, CV was ∼50% of normal. A prominent endocardial rim of myocardium in the infarct was not the source of epicardial optical signals because chemical ablation of the endocardium did not affect the epicardial activation pattern. Concluson: Therefore, remnant groups of myocytes in the mid‐wall and epicardium of the infarct scar support normal electrical activation during RA pacing. Areas of delayed conduction emerge only on epicardial stimulation.
Prospective Randomized Comparison of 8‐mm Gold‐Tip, Externally Irrigated‐Tip and 8‐mm Platinum‐Iridium Tip Catheters for Cavotricuspid Isthmus Ablation
Authors: SACHER FRÉDÉRIC , O'NEILL MARK D. , JAIS PIERRE , HUFFER LINDA L. , LABORDERIE JULIEN , DERVAL NICOLAS , DEPLAGNE ANTOINE , TAKAHASHI YOSHIHIDE , JONNSON ANDERS , HOCINI MELEZE , CLEMENTY JACQUES , HAISSAGUERRE MICHEL 
Published: May 2007

Introduction: Radiofrequency (RF) ablation of the cavotricuspid isthmus (CTI) can be performed using different types of ablation catheter. Gold tip electrodes have the theoretical advantage of creating bigger lesions than standard platinum‐iridium electrode. This prospective, randomized study compares the clinical efficacy of 8‐mm gold tip catheter, externally irrigated and 8‐mm platinum‐iridium tip (Pt tip) catheters. Methods and Results: Sixty consecutive patients (51 men, 60 ± 10 years) undergoing de novo CTI ablation for documented typical atrial flutter were randomized to one of the following ablation catheters: 8‐mm gold tip catheter, an externally irrigated‐tip (Irr. tip) catheter, or an 8‐mm Pt tip catheter. The procedural endpoint was achievement of bidirectional isthmus conduction block with ≤ 20 minutes of RF energy application. The latter was achieved equally with the 3 catheters (95% for gold tip, 100% for irrigated tip, 95% for Pt tip) and the durations of RF (10 ± 6, 10 ± 4, 13 ± 8 minutes), fluoroscopy (12 ± 6, 12 ± 7, 15 ± 12 minutes) and the procedure (34 ± 23, 38 ± 24, 40 ± 30 minutes) were similar in all groups. The maximal targeted power could not be reached in at least one location in 40% of patients with gold tip and in 35% of patients with Pt tip catheters whereas it was always achieved with an Irr. tip catheter (P = 0.003, P = 0.008). The reduction in impedance during RF delivery was greater with Irr. tip (11 ± 7 Ω) than with gold (7 ± 4 Ω, P = 0.02) or Pt tip (5 ± 3 Ω, P = 0.001) catheters. Conclusion: This study demonstrates equivalent efficacies of gold, platinum‐iridium and externally Irr. tip catheters for successful de novo ablation of the CTI.
Current state of vitiligo therapy – evidence‐based analysis of the literature
Authors: Forschner Tobias , Buchholtz Stefan , Stockfleth Eggert 
Published: May 2007

SummaryVitiligo is a skin disease with a worldwide prevalence ranging from 0.5% to 4%. Conservative therapies include photochemotherapy, phototherapy with UVB radiation (broadband UVB 290–320 nm, narrow band UVB 311 nm), systemic steroids and pseudocatalase. Modern therapeutic options include treatment with topical immunomodulators (tacrolimus, pimecrolimus), analogues of vitamin D3, excimer laser and surgery/transplantation. Our analysis compares these therapies for vitiligo and the evidence levels supporting their effectiveness. Conclusions: The face and neck respond best to all therapeutic approaches, while the acral areas are least responsive. For generalized vitiligo, phototherapy with UVB radiation is most effective with the fewest side effects; PUVA is the second best choice.Topical corticosteroids are the preferred drugs for localized vitiligo. They may be replaced by topical immunomodulators which display comparable effectiveness and fewer side effects.The effectiveness of vitamin D analogues is controversial with limited data. Surgical therapy can be very successful, but requires an experienced surgeon and is very demanding of time and facilities, thus limiting its widespread use. L‐phenylalanine therapy appears effective on the face but enjoys neither widespread use nor extensive data support. No single therapy for vitiligo can be regarded as the most effective as the success of each treatment modality depends on the type and location of vitiligo.
Cryosurgical treatment of lentigo maligna
Authors: De Moraes Aparecida Machado , Pavarin Lilian Bianchi , Herreros Fernanda , De Aguiar Michelman Fábia , Velho Paulo Eduardo Neves Ferreira , De Souza Elemir Macedo 
Published: May 2007

Summary Background: Lentigo maligna (LM) is a common melanocytic malignancy which requires therapy because of the risk of progression to invasive lentigo maligna melanoma which a much worse prognosis. Patients and Methods: 18 patients with clinical and histopathological diagnosis of LM were treated with cryosurgery.The patients were older Caucasians (mean age 59.5 years) and 11 were male. They were chosen for cryosurgery because the lesion posed a surgical challenge or the patient was not a good surgical candidate. They were treated with two freeze‐thaw cycles of liquid nitrogen under local anesthesia in a single sitting. Lesions larger than 2 cm2 were divided into smaller segments for freezing. Results: The lesions resolved clinically in all cases, with no recurrence or metas‐tasis detected during a mean follow‐up of 75.5 months. Some patients developed hypopigmented scars. Conclusions: Cryosurgery with liquid nitrogen is an efficient, safe and in most cases aesthetically acceptable alternative method to treat LM.
Granulomatous jellyfish dermatitis
Authors: Ulrich Heidi , Landthaler Michael , Vogt Thomas 
Published: May 2007

SummaryThe induction of a granulomatous inflammation by jellyfish toxins is rare. More typically, acute toxic and urticarial reactions are seen. An 11‐year‐old boy developed a striated urticarial erythema on the left cheek after contact with a gelatinous mass while swimming in the sea in Croatia. After initial erosion, a striated induration developed in the area of contact. Histological examination revealed a granulomatous inflammation with some eosinophils. While topical steroid‐based antiinflammatory and antibacterial therapy over several weeks was not effective, topical therapy with tacrolimus 0.1% for two two‐week treatment periods led to healing of the skin changes with a slight scar. There was no clinical recurrence after 5 month of follow‐up.
Sex and Season Affect Individual‐Level Diet Variation in the Neotropical Marsupial Gracilinanus microtarsus (Didelphidae)
Authors: Martins Eduardo Guimarães , Araújo Márcio Silva , Bonato Vinícius , Reis Sérgio Furtado dos 
Published: May 2007

ABSTRACTIndividual‐level diet variation was investigated in Gracilinanus microtarsus, an insectivorous marsupial whose diet is affected by sex and season in the highly seasonal Brazilian Cerrado. To measure individual‐level diet variation, the diets of individual males and females were compared to that of their population in the warm‐wet and cool‐dry seasons using the proportional similarity index (PSi). This index varies from 1 (complete overlap between the individual i 's and population's diets) toward 0 (decreasing overlap). Mean PSi values were computed as a measure of the degree of interindividual diet variation (the larger the mean PSi value, the smaller the variation among individuals’ diets). Interindividual diet variation among females was similar between seasons, whereas among males it decreased from the warm‐wet to the cool‐dry season. Diet variation among males is probably reduced in the cool‐dry season because of constraints on food consumption generated by interactions between endogenous (physiological needs associated with high rates of body mass growth) and exogenous (food limitation) factors.
Population Patterns of a Riparian Frog (Rana swinhoana) Before and After an Earthquake in Subtropical Taiwan
Authors: Lai Yung‐Chih , Shieh Bao‐Sen , Kam Yeong‐Choy 
Published: May 2007

ABSTRACTWe compared the population dynamics of a riparian ranid frog, Rana swinhoana, before (1996–1999) and after (1999–2001) a strong earthquake. This earthquake caused little disturbance to the vegetation and landscape of the study site but the stream and ponds dried up within a week. Nearly all frogs marked (1002 of 1004) before the earthquake had disappeared after the earthquake. Smaller, unmarked frogs began to appear in stream habitats about 9 mo after the earthquake, and the frog population was much smaller than it was before the earthquake. Population dynamics and temporal and spatial distribution of frogs before and after the earthquake correlated closely with the hydrology of the stream and ponds. The movement patterns of frogs before and after the earthquake were similar, suggesting frog behavior did not change in response to drastic changes in hydrology, and frogs continued to exhibit strong site‐fidelity. Following the earthquake, stream water volume was much lower, especially in the summer, which allowed the normally winter‐breeding frogs to breed year‐round. Results demonstrate that a population of R. swinhoana can disappear suddenly as the result of a natural disturbance. We propose that anuran species that exhibit strong site‐fidelity are particularly susceptible to extirpation of local populations because frogs may lack the behavioral plasticity to respond to sudden water depletion.
On the Vibration
Isolation of Flexible Structures
Authors: Tu Y.
Q. , Zheng G.
T. 
Published: May 2007

Abstract
Although the study of vibration isolation has a very long history, when an isolated structure is so flexible that it cannot be properly approximated with a rigid body or a single-degree-of-freedom model, its vibration isolation brings about some new questions and problems. By transforming the dynamic equation of motion of the coupled structure formed by the isolator and the isolated structure into the modal space and following the tradition of studying features of the vibration transmissibility across the isolator, questions and problems associated with the flexible structure vibration isolation are studied. It is found from the study that a lower isolation frequency and a higher damping level can both increase the isolation effectiveness, the isolated structure is a vibration absorber to the isolator, and a combination of the vibration isolation and the vibration attenuation can be more effective in mitigating the vibration. A numerical example of the whole spacecraft vibration isolation has proved the above conclusions.
Age Differences in Deactivation: A Link to Cognitive Control?
Authors: Persson Jonas , Lustig Cindy , Nelson James K. , Reuter-Lorenz Patricia A. 
Published: May 2007

Abstract
The network of regions shown by functional imaging studies to be deactivated by experimental tasks relative to nominally more passive baselines (task &lt; baseline) may reflect processes engaged during the resting state or “default mode.” Deactivation may result when attention and resources are diverted from default-mode processes toward task processes. Aging is associated with altered patterns of deactivation which may be related to declining resources, difficulties with resource allocation, or both. These possibilities predict that greater task demand, which increases deactivation levels in younger adults, should exacerbate age-related declines in allocating resources away from the default mode. The present study investigated the magnitude and temporal properties of deactivations in young and older adults during tasks that varied in their demand for cognitive control. Two versions of a verb generation task that varied in their demand for selection among competing alternatives were compared to word reading and a fixation baseline condition. Consistent with our hypothesis, greater deactivations were found with increasing demand. Young and older adults showed equivalent deactivations in the minimal selection condition. By contrast, age differences in both the magnitude and time course of deactivation increased with selection demand: Compared to young adults', older adults' deactivation response showed less sensitivity to demand. Demand-related changes in deactivation magnitude correlated with performance changes, suggesting that individual and group differences in deactivation have functional significance.
Religious Culture Contested: The Sufi Ritual of Dawsa in Nineteenth-Century Cairo
Authors: Hatina Meir 
Published: May 2007

AbstractWith the entry of Muslim society into the modern era in the nineteenth century, Sufi beliefs and rituals became the focus of systematic debate and denunciation by local and foreign observers alike. An illuminating example is the dawsa ritual—a ceremony involving the shaykh of the Sa'diyya order riding his horse over the backs of his prostrate disciple s, which was particularly widespread in the Cairene milieu. This practice, intended to prove that true believers are protected from all harm, was officially abolished in 1881 in the name of enlightenment and human dignity. The present article traces the history of the dawsa and, more broadly, sheds light on the Sufi encounter with the challenges of modernity. It reveals a diverse picture of the anti-Sufi campaign carried out by various elements in Egypt—foreign consul s, government official s, modernists and nationalists—which resulted in a loss of influence by Sufi order s, yet fostered a capacity for survival and ideological rejuvenation.
Circulation Regimes: Chaotic Variability versus SST-Forced Predictability
Authors: Straus David M. , Corti Susanna , Molteni Franco 
Published: May 2007

Abstract
The circulation regimes in the Pacific–North American region are studied using the NCEP–NCAR reanalyses for the 18-winter period (1981/82–1998/99; NCEP18) and for the 54-winter period (1948/49–2001/02; NCEP54). The sampling properties of the regimes are estimated using very large ensembles (of size 55) of winter simulations made for the NCEP18 period with the atmospheric general circulation model of the Center for Ocean–Land–Atmosphere Studies, forced by observed SST and sea ice.
The regimes are identified using a modified version of the k-means method. From the NCEP54 dataset a set of four clusters was found [i.e., the Alaskan ridge (AR), Arctic low (AL), Pacific trough (PT), and the Arctic high (AH)], which are significant (vis-à-vis a multinormal background), and more reproducible (within randomly chosen half-length samples) than would be expected from a multinormal process. The frequency of occurrence of the PT (AH) has increased (decreased) significantly during the past two decades.
The PT cluster obtained from NCEP18 dataset more closely resembles the El Niño–forced seasonal mean pattern of recent decades than it does the traditional PNA.
The GCM simulates the AR, AL, and PT clusters (but not the AH). The simulated AR and PT patterns have errors (cf. the NCEP18 results), which are outside the range of internal variability. The simulated frequency of occurrence agrees with the NCEP18 results within sampling variability.
The differences in cluster properties of the PT and AR regimes between the NCEP18 and NCEP54 datasets are due to changes in SST forcing, not sampling error.
Year-to-year changes in the frequency of occurrence of the PT, AL, and AR clusters in the simulations and the NCEP18 dataset are generally consistent with each other.
Prediction Skill of the NAO and PNA from Daily to Seasonal Time Scales
Authors: Johansson Åke 
Published: May 2007

AbstractThe skill of state-of-the-art operational dynamical models in predicting the two most important modes of variability in the Northern Hemisphere extratropical atmosphere, the North Atlantic Oscillation (NAO) and Pacific–North American (PNA) teleconnection patterns, is investigated at time scales ranging from daily to seasonal. Two uncoupled atmospheric models used for deterministic forecasting in the short to medium range as well as eight fully coupled atmosphere–land–ocean forecast models used for monthly and seasonal forecasting are examined and compared.For the short to medium range, the level of forecast skill for the two indices is higher than that for the entire Northern Hemisphere extratropical flow. The forecast skill of the PNA is higher than that of the NAO. The forecast skill increases with the magnitude of the NAO and PNA indices, but the relationship is not pronounced. The probability density function (PDF) of the NAO and PNA indices is negatively skewed, in agreement with the distribution of skewness of the geopotential field. The models maintain approximately the observed PDF, including the negative skewness, for the first week.Extreme negative NAO/PNA events have larger absolute values than positive extremes in agreement with the negative skewness of the two indices. Recent large extreme events are generally well forecasted by the models.On the intraseasonal time scale it is found that both NAO and PNA have lingering forecast skill, in contrast to the Northern Hemisphere extratropical flow as a whole. This fact offers some hope for extended range forecasting, even though the skill is quite low. No conclusive positive benefit is seen from using higher horizontal resolution or coupling to the oceans.On the monthly and seasonal time scales, the level of forecast skill for the two indices is generally quite low, with the exception of winter predictions at short lead times.
Objectively Determined Resolution-Dependent Threshold Criteria for the Detection of Tropical Cyclones in Climate Models and Reanalyses
Authors: Walsh K. J. E. , Fiorino M. , Landsea C. W. , McInnes K. L. 
Published: May 2007

Abstract
Objectively derived resolution-dependent criteria are defined for the detection of tropical cyclones in model simulations and observationally based analyses. These criteria are derived from the wind profiles of observed tropical cyclones, averaged at various resolutions. Both an analytical wind profile model and two-dimensional observed wind analyses are used. The results show that the threshold wind speed of an observed tropical cyclone varies roughly linearly with resolution. The criteria derived here are compared to the numerous different criteria previously employed in climate model simulations. The resulting method provides a simple means of comparing climate model simulations and reanalyses.