996 resultados para Unstable hemoglobins


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OBJECTIVE: To evaluate fracture patterns in bicondylar tibial plateau fractures and their impact on treatment strategy. DESIGN: Prospective data analysis with documentation of initial injury and treatment strategy, computed tomography scans, conventional x-rays, long-term evaluation of radiographs, and functional assessments. SETTING: Level 1 regional trauma center. PATIENTS: Prospective data acquisition of 14 consecutive patients (10 male and 4 female) with a bicondylar tibial plateau fracture (AO Type C). INTERVENTION: Application of a stepwise reconstruction strategy of the tibial plateau starting with the reposition and fixation of the posteromedial split fragment using a 3.5 buttress plate, followed by reposition and grafting of the lateral compartment and lateral fixation with a 3.5 plate in 90 degree to the medial fixation device. MAIN OUTCOME MEASUREMENTS: All patients were evaluated with full-length standing film, standardized x-rays, Lysholm score for functional assessment, and patient's self-appraisal. RESULTS: Most of the complex bicondylar fractures follow a regular pattern in that the medial compartment is split in a mediolateral direction with a posteromedial main fragment, combined with various amounts of multifragmental lateral compartment depression. The technique introduced allows for accurate and stable reduction and fixation of this fracture type. The final Lysholm knee score showed an average of 83.5 points (range: 64.5-92). CONCLUSIONS: Complex bicondylar tibial plateau fractures follow a regular pattern, which is not represented in existing 2-dimensional fracture classifications. A 2-incision technique starting with the reduction of the posteromedial edge results in accurate fracture reduction with low complication rates and excellent knee function.

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Social work is experiencing an unprecedented degree of institutional instability, particularly in the advanced industrial nations which, to varying degrees and via differing paths, have abandoned the Keynesian Welfare State. It has been replaced with a fundamentally different workfare regime in which operates on quite different assumptions – all of which pose fundamental challenges to social work. The degree of change is such that it can be understood as institutional change. The profession needs a number of strategies in response the contemporary de-stabilization. Drawing on theoretical and empirical literature about institutional change we show why it is that professional leadership is crucial in the current environment. The paper reviews what in currently know about leadership, both in general and in relation to social work. Referring to the notion of institutional entrepreneurs and on the role played by other non-social work professional associations in situations of change, we articulate what role leadership can play. We conclude with recommendations about how leadership could be promoted, particularly by the professional associations.

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Myotonic dystrophy (DM), an autosomal dominant disorder mapping to human chromosome 19q13.3, is the most common neuromuscular disease in human adults.^ Following the identification of the mutation underlying the DM phenotype, an unstable (CTG)$\sb{n}$ trinucleotide repeat in the 3$\prime$ untranslated region (UTR) of a gene encoding a ser/thr protein kinase named DM protein kinase (DMPK), the study was targeted at two questions: (1) the identification of the disease-causing mechanism(s) of the unstable repeat, and at a more basic level, (2) the identification of the origin and the mechanism(s) involved in repeat instability. The first goal was to identify the pathophysiological mechanisms of the (CTG)$\sb{n}$ repeat.^ The normal repeat is transcribed but not translated; therefore, initial studies centered on the effect on RNA transcript levels. The vast majority of DM affecteds are heterozygous for the mutant expansion, so that the normal allele interferes with the analysis of the mutant allele. A quantitative allele-specific RT-PCR procedure was developed and applied to a spectrum of patient tissue samples and cell lines. Equal levels of unprocessed pre-mRNA were determined for the wild type (+) and disease (DM) alleles in skeletal muscle and cell lines of heterozygous DM patients, indicating that any nucleosome binding has no effect at the level of transcriptional initiation and transcription of the mutant DMPK locus. In contrast, processed mRNA levels from the DM allele were reduced relative to the + allele as the size of the expansion increased. The unstable repeat, therefore, impairs post-transcriptional processing of DM allele transcripts. This phenomenon has profound effects on overall DMPK locus steady-state transcript levels in cells missing a wild type allele and does not appear to be mediated by imprinting, decreased mRNA stability, generation of aberrant splice forms, or absence of polyadenylation of the mutant allele.^ In Caucasian DM subjects, the unstable repeat is in complete linkage disequlibrium with a single haplotype composed of nine alleles within and flanking DMPK over a physical distance of 30 kb. A detailed haplotype analysis of the DM region was conducted on a Nigerian (Yoruba) DM family, the only indigenous sub-Saharan DM case reported to date. Each affected member of this family had an expanded (CTG)$\sb{n}$ repeat in one of their DMPK alleles. However, unlike all other DM populations studied thus far, disassociation of the (CTG)$\sb{n}$ repeat expansion from other alleles of the putative predisposing haplotype was found. Thus, the expanded (CTG)$\sb{n}$ repeat in this family was the result of an independent mutational event. Consequently, the origin of DM is unlikely the result of a single mutational event, and the hypothesis that a single ancestral haplotype predisposes to repeat expansion is not compelling. (Abstract shortened by UMI.) ^

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A growing body of longitudinal studies suggests that low self-esteem is a risk factor for depression. However, it is unclear whether other characteristics of self-esteem, besides its level, explain incremental or even greater variance in subsequent depression. We examined the prospective effects of self-esteem level, instability (i.e., the degree of variability in self-esteem across short periods), and contingency (i.e., the degree to which self-esteem fluctuates in response to self-relevant events) on depressive symptoms in 1 overarching model, using data from 2 longitudinal studies. In Study 1, 372 adults were assessed at 2 waves over 6 months, including 40 daily diary assessments at Wave 1. In Study 2, 235 young adults were assessed at 2 waves over 6 weeks, including about 6 daily diary assessments at each wave. Self-esteem contingency was measured by self-report and by a statistical index based on the diary data (capturing event-related fluctuations in self-esteem). In both studies self-esteem level, but not self-esteem contingency, predicted subsequent depressive symptoms. Self-esteem instability predicted subsequent depressive symptoms in Study 2 only, with a smaller effect size than self-esteem level. Also, level, instability, and contingency of self-esteem did not interact in the prediction of depressive symptoms. Moreover, the effect of self-esteem level held when controlling for neuroticism and for all other Big Five personality traits. Thus, the findings provide converging evidence for a vulnerability effect of self-esteem level, tentative evidence for a smaller vulnerability effect of self-esteem instability, and no evidence for a vulnerability effect of self-esteem contingency.

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This study explored the effects of unstable shoe design on oxygen consumption. Methods. Oxygen consumption (VO2) and heart rate (HR) were measured in 16 individuals while barefoot, wearing unstable shoes (Masai Barefoot Technology) and wearing conventional sport shoes while standing and walking on a treadmill and for 5 individuals while walking around a 400 m track. Results. When wearing the MBT shoes, a significant (p < 0.01) increase of 9.3 ± 5.2% in VO2 was measured while standing quietly for 6 min. No differences in VO2 and HR were observed between the MBT shoes or weight-adjusted conventional shoes (to match the weight of the MBT shoes) while walking on a treadmill. However, significant increases (p < 0.01) in VO2 (4.4 ± 8.2%) and HR (3.6 ± 7.3%) were observed for the MBT shoes compared with being barefoot. No significant differences in VO2 and HR were recorded while walking around a 400 m track either with MBT shoes, weight-adjusted conventional shoes or barefoot. Nonetheless, a comparison of the MBT shoes with barefoot revealed a tendency for VO2 to be higher when wearing the MBT shoes (7.1 ± 6.5%, p < 0.1) although HR was not significantly affected. Conclusions. The unstable shoe design predominantly effects oxygen consumption while standing, most likely due to increased muscle activity of the lower extremities.

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Loss of function of the urea cycle enzyme argininosuccinate lyase (ASL) is caused by mutations in the ASL gene leading to ASL deficiency (ASLD). ASLD has a broad clinical spectrum ranging from life-threatening severe neonatal to asymptomatic forms. Different levels of residual ASL activity probably contribute to the phenotypic variability but reliable expression systems allowing clinically useful conclusions are not yet available. In order to define the molecular characteristics underlying the phenotypic variability, we investigated all ASL mutations that were hitherto identified in patients with late onset or mild clinical and biochemical courses by ASL expression in human embryonic kidney 293 T cells. We found residual activities >3 % of ASL wild type (WT) in nine of 11 ASL mutations. Six ASL mutations (p.Arg95Cys, p.Ile100Thr, p.Val178Met, p.Glu189Gly, p.Val335Leu, and p.Arg379Cys) with residual activities ≥16 % of ASL WT showed no significant or less than twofold reduced Km values, but displayed thermal instability. Computational structural analysis supported the biochemical findings by revealing multiple effects including protein instability, disruption of ionic interactions and hydrogen bonds between residues in the monomeric form of the protein, and disruption of contacts between adjacent monomeric units in the ASL tetramer. These findings suggest that the clinical and biochemical course in variant forms of ASLD is associated with relevant residual levels of ASL activity as well as instability of mutant ASL proteins. Since about 30 % of known ASLD genotypes are affected by mutations studied here, ASLD should be considered as a candidate for chaperone treatment to improve mutant protein stability.

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PURPOSE Open surgical management of unstable pelvic ring injuries has been discussed controversially compared to percutaneous techniques in terms of surgical site morbidity especially in older patients. Thus, we assessed the impact of age on the outcome following fixation of unstable pelvic ring injuries through the modified Stoppa approach. METHODS Out of a consecutive series of 92 patients eligible for the study, 63 patients (mean age 50 years, range 19-78) were evaluated [accuracy of reduction, complications, failures, Majeed-Score, Oswestry Disability Questionnaire (ODI), Mainz Pain Staging System (MPSS)] at a mean follow-up of 3.3 years (range 1.0-7.9). Logistic multivariate regression analysis was performed to assess the outcome in relation to increasing patient age and/or Injury Severity Score (ISS). RESULTS Out of 63 patients, in 36 an "anatomic" reduction was achieved. Ten postoperative complications occurred in eight patients. In five patients, failure of fixation was noted at the anterior and/or posterior pelvic ring. In 49 patients, an "excellent" or "good" Majeed-Score was obtained; the mean ODI was 14 % (range 0-76 %); 50 patients reported either no or only minor chronic pelvic pain (MPSS). Only an increasing ISS conferred an increased likelihood of the occurrence of a non-anatomical reduction, a "poor" or "fair" Majeed-Score, or an ODI >20 %. CONCLUSIONS Increasing age did not impact the analysed parameters. Open reduction and internal fixation of the anterior pelvic ring through a modified Stoppa approach in unstable pelvic ring injuries did not result in an unfavourable outcome with increasing age of patients.

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The long-term risk associated with different coronary artery disease (CAD) presentations in women undergoing percutaneous coronary intervention (PCI) with drug-eluting stents (DES) is poorly characterized. We pooled patient-level data for women enrolled in 26 randomized clinical trials. Of 11,577 women included in the pooled database, 10,133 with known clinical presentation received a DES. Of them, 5,760 (57%) had stable angina pectoris (SAP), 3,594 (35%) had unstable angina pectoris (UAP) or non-ST-segment-elevation myocardial infarction (NSTEMI), and 779 (8%) had ST-segment-elevation myocardial infarction (STEMI) as clinical presentation. A stepwise increase in 3-year crude cumulative mortality was observed in the transition from SAP to STEMI (4.9% vs 6.1% vs 9.4%; p <0.01). Conversely, no differences in crude mortality rates were observed between 1 and 3 years across clinical presentations. After multivariable adjustment, STEMI was independently associated with greater risk of 3-year mortality (hazard ratio [HR] 3.45; 95% confidence interval [CI] 1.99 to 5.98; p <0.01), whereas no differences were observed between UAP or NSTEMI and SAP (HR 0.99; 95% CI 0.73 to 1.34; p = 0.94). In women with ACS, use of new-generation DES was associated with reduced risk of major adverse cardiac events (HR 0.58; 95% CI 0.34 to 0.98). The magnitude and direction of the effect with new-generation DES was uniform between women with or without ACS (pinteraction = 0.66). In conclusion, in women across the clinical spectrum of CAD, STEMI was associated with a greater risk of long-term mortality. Conversely, the adjusted risk of mortality between UAP or NSTEMI and SAP was similar. New-generation DESs provide improved long-term clinical outcomes irrespective of the clinical presentation in women.

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Ocean planets are volatile-rich planets, not present in our Solar system, which are thought to be dominated by deep, global oceans. This results in the formation of high-pressure water ice, separating the planetary crust from the liquid ocean and, thus, also from the atmosphere. Therefore, instead of a carbonate-silicate cycle like on the Earth, the atmospheric carbon dioxide concentration is governed by the capability of the ocean to dissolve carbon dioxide (CO2). In our study, we focus on the CO2 cycle between the atmosphere and the ocean which determines the atmospheric CO2 content. The atmospheric amount of CO2 is a fundamental quantity for assessing the potential habitability of the planet's surface because of its strong greenhouse effect, which determines the planetary surface temperature to a large degree. In contrast to the stabilizing carbonate-silicate cycle regulating the long-term CO2 inventory of the Earth atmosphere, we find that the CO2 cycle feedback on ocean planets is negative and has strong destabilizing effects on the planetary climate. By using a chemistry model for oceanic CO2 dissolution and an atmospheric model for exoplanets, we show that the CO2 feedback cycle can severely limit the extension of the habitable zone for ocean planets.

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This paper proposes asymptotically optimal tests for unstable parameter process under the feasible circumstance that the researcher has little information about the unstable parameter process and the error distribution, and suggests conditions under which the knowledge of those processes does not provide asymptotic power gains. I first derive a test under known error distribution, which is asymptotically equivalent to LR tests for correctly identified unstable parameter processes under suitable conditions. The conditions are weak enough to cover a wide range of unstable processes such as various types of structural breaks and time varying parameter processes. The test is then extended to semiparametric models in which the underlying distribution in unknown but treated as unknown infinite dimensional nuisance parameter. The semiparametric test is adaptive in the sense that its asymptotic power function is equivalent to the power envelope under known error distribution.

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In recent years, a large and expanding literature has examined the properties of developing economies with regard to the macroeconomic cycle.1 One such property that is characteristic of developing economies is large fluctuations in consumption. Meanwhile, aid for the low income countries is extremely volatile, and under certain circumstances, the volatile aid amplifies the consumption volatility. This document examines whether it is possible that the volatile aid yields high consumption volatility in African countries that constitute the majority of the low income countries. Our numerical analysis reveals that the strongly influential aid disbursements yield a considerably large fluctuation in consumption.

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During the years 1992-1996 a climatic change took place in the Iberian Peninsula, changing from dry years to humid years, giving place to numerous and abundant rainfalls that, in the zone of Andalusia caused enough problems in embankments of the roads.

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We report conditions on a switching signal that guarantee that solutions of a switched linear systems converge asymptotically to zero. These conditions are apply to continuous, discrete-time and hybrid switched linear systems, both those having stable subsystems and mixtures of stable and unstable subsystems.