bluuebunny/crossref_metadata_2025_split huggingface dataset:
Synthesis of heat exchanger networks at subambient conditions with compression and expansion of process streams
Authors: Wechsung Achim , Aspelund Audun , Gundersen Truls , Barton Paul I. 
Published: August 2010

AbstractThis article presents an optimization formulation for the synthesis of heat exchanger networks where pressure levels of process streams can be adjusted to improve heat integration. Especially important at subambient conditions, this allows for the interconversion of work, temperature, and pressure‐based exergy and leads to reduced usage of expensive cold utility. Furthermore, stream temperatures and pressures are tuned for close tracking of the composite curves yielding increased exergy efficiency. The formulation is showcased on a simple example and applied to a case study drawn from the design of an offshore natural gas liquefaction process. Aided by the optimization, it is demonstrated how the process can extract exergy from liquid nitrogen and carbon dioxide streams to support the liquefaction of a natural gas stream without additional utilities. This process is part of a liquefied energy chain, which, supplies natural gas for power generation while facilitating carbon dioxide sequestration. © 2010 American Institute of Chemical Engineers AIChE J, 2011
Photocatalytic hydrogen production from water with nonfood hydrocarbons as oxidizing sacrifice agents
Authors: Deguchi Seiichi , Takeichi Toshinori , Shimasaki Shoji , Ogawa Muneaki , Isu Norifumi 
Published: August 2010

AbstractTo enhance photocatalytic water splitting, various oxidizing sacrifice agents (OSA) have been added to the system in order to scavenge the coproduced O2, and, thus, to hinder the reverse reactions. In the aim of achieving carbon‐neutral photocatalytic water splitting, nonfood hydrocarbons of castor‐ and jojoba‐oils were evaluated as OSA. Moreover, various surfactants were tested as emulsifiers for W/O binary solution for promoting photocatalytic water splitting rate. Among the OSA used, the castor‐oil was found to be more suitable candidate compared to jojoba‐oil, which was attributed to its smaller carbon chain numbers of mainly 18. Without surfactants, around 20 vol %‐castor‐oil aqueous binary solution with TiO2/Pt(0.10 wt %) provided the highest water splitting rate of about 30 mL‐H2/(m2·h). Among tested surfactants, liquid‐detergent was the best due to its optical transparency. 40 vol %‐ or 60 vol %‐castor‐oil emulsion with a drop of liquid‐detergent resulted in a water splitting rate of 125 mL‐H2/(m2·h), which was four times greater that the aforementioned highest value. © 2010 American Institute of Chemical Engineers AIChE J, 2011
Particle grinding by high‐intensity ultrasound: Kinetic modeling and identification of breakage mechanisms
Authors: Raman Vinay , Abbas Ali , Zhu Wenting 
Published: August 2010

AbstractHigh‐intensity ultrasound, is sought as a means to break particles. A horn‐type ultrasonic transducer is used to apply HIU into a suspension of alumina particles causing breakage to occur. The rate of particle breakage is monitored continuously via in‐line laser‐based particle chord length measurement. Kapur function analysis is used to arrive at the grinding kinetics under variations of ultrasonic power, particle loading, temperature of the suspension and particle size. The first Kapur function increases monotonically with increase in input ultrasonic power. Increasing temperature also increases the first Kapur function but an optimum in the range investigated (10–50°C) is observed near 25°C. An exponential relation is found for the variation of first Kapur function with particle size, this being unique to ultrasound‐mediated particle breakage. The breakage mechanism is attributed mainly to particle abrasion. Different breakage mechanisms are observed at different temperatures. © 2010 American Institute of Chemical Engineers AIChE J, 2011
Comprehensive evaluation of time‐to‐response parameter as a predictor of treatment failure following imatinib therapy in chronic phase chronic myeloid leukemia: Which parameter at which time‐point does matter?
Authors: Kim Dong Hwan (Dennis) , Sriharsha Lakshmi , Jung Chul Won , Kamel‐Reid Suzanne , Radich Jerald P. , Lipton Jeffrey H. 
Published: August 2010

AbstractEarly recognition of high‐risk patient is important to improve long‐term outcomes following imatinib therapy for chronic myeloid leukemia (CML). Some controversy surrounds the question, which of short‐term response parameters at which time‐point, including complete cytogenetic response (CCyR) or major molecular response (MMR) at 6 or 12 months, is the best predictor for treatment outcomes. In this comprehensive analysis, we adopted landmark analysis method, time‐dependent Cox's proportional hazard model, and receiver‐operating characteristics (ROC) method to analyze time‐to‐response parameter as predictor of long‐term outcomes in 187 chronic phase (CP) CML patients. Regardless of the methods of analysis, earlier achievement of short‐term response such as CCyR or MMR could predict the higher probability of achieving better interim outcome (such as treatment failure or loss of response [LOR]). Similar to the findings from other studies, our ROC analysis provided cutoff time points for MMR (18–36 months) and CCyR (6–12 months) that were the best predictors for LOR or treatment failure, which can be an indirect evidence supporting the ELN recommendation. The patient who achieves short‐term response rapidly will have a lower risk of losing response or failing after imatinib therapy in CML patients. Am. J. Hematol., 2010. © 2010 Wiley‐Liss, Inc.
Micrornas: Emerging key regulators of hematopoiesis
Authors: Havelange Violaine , Garzon Ramiro 
Published: August 2010

AbstractMicroRNAs (miRNAs) are small noncoding RNAs that regulate gene expression by pairing with their target mRNAs, thereby inducing protein translation inhibition or/and mRNA degradation. There is now strong evidence that miRNAs play a crucial role in the regulation of hematopoiesis. Several groups have shown that miRNA expression change dynamically during hematopoietic differentiation and functional studies demonstrated that miRNAs control not only differentiation but also activity of hematopoietic cells by targeting transcription factors, growth factor receptors, and specific transcripts involved in the modulation of cellular responses to external stimuli. In this review, we will summarize the current knowledge of miRNA expression and function during hematopoiesis and discuss controversies and future directions. Am. J. Hematol., 2010. © 2010 Wiley‐Liss, Inc.
Expression profiling of cytogenetically normal acute myeloid leukemia identifies MicroRNAs that target genes involved in monocytic differentiation
Authors: Lutherborrow Mark , Bryant Adam , Jayaswal Vivek , Agapiou David , Palma Catalina , Yang Yee Hwa , Ma David D.F. 
Published: August 2010

AbstractMicroRNAs are short ribonucleic acids (RNAs) that play an important role in many aspects of cellular biology such as differentiation and apoptosis, due to their role in the regulation of gene expression. Using microRNA microarrays, we characterized the microRNA gene expression of 27 patients with acute myeloid leukemia (AML) with normal cytogenetics, focusing on the microRNAs differentially expressed between the M1 and M5 French–American–British (FAB) subtypes. An accurate delineation of these two AML entities was observed based on the expression of 12 microRNAs. We hypothesized that these microRNAs may potentially be involved in the differentiation block of M1 blasts and consequently monocytic differentiation. Using publically available mRNA data and microRNA target prediction software, we identified several key myeloid factors that may be targeted by our candidate microRNAs. The expression changes of the candidate microRNAs during monocytic differentiation of AML cell lines treated with Vitamin D and phorbol 12‐myristate 13‐acetate were examined. All six candidate microRNAs were significantly down‐regulated over the time course by quantitative reverse transcriptase polymerase chain reaction suggesting a link between these microRNAs and monocytic differentiation. To further characterize these microRNAs, we confirmed by luciferase assays that these microRNA target several key myeloid factors such as MAFB, IRF8, and KLF4 identifying a possible mechanism for the control of differentiation by these microRNAs. Am. J. Hematol., 2011. © 2010 Wiley‐Liss, Inc.
Sedentary work, low physical job demand, and obesity in US workers
Authors: Choi BongKyoo , Schnall Peter L. , Yang Haiou , Dobson Marnie , Landsbergis Paul , Israel Leslie , Karasek Robert , Baker Dean 
Published: August 2010

AbstractBackgroundLittle is known about the role of low physical activity at work (sedentary work or low physical job demand) in the increasing prevalence of obesity of US workers.MethodsThis cross‐sectional and secondary data analysis included 1,001 male and 1,018 female workers (age range: 32–69) from the National Survey of Midlife Development in the United States (MIDUS) II study (2004–2006). Sedentary work and physical job demand were measured by questionnaire items. Total obesity (based on body mass index) and central obesity (based on waist circumference) were defined using WHO criteria.ResultsAfter controlling for covariates (socio‐demographic, psychosocial working conditions, health status, and health behaviors), sedentary work, low physical job demand, or their combination increased the risk for total and central obesity in male workers, particularly when they worked longer than 40 hr per week. Sedentary work marginally increased the risk for total and central obesity in female workers.ConclusionsLow physical activity at work is a significant risk factor for total and central obesity in middle‐aged US male workers. Am. J. Ind. Med. 53:1088–1101, 2010. © 2010 Wiley‐Liss, Inc.
Work‐related effort thrombosis in a millwright: A case report
Authors: Pysklywec Michael , Cina Claudio S. 
Published: August 2010

AbstractBackgroundEffort thrombosis refers to axillosubclavian vein thrombosis secondary to physical activities of the upper extremity.MethodsThis report describes the clinical presentation of effort thrombosis in a millwright and reviews the literature for occupational reports of this condition.ResultsWhile there is a paucity of literature reporting an occupational association, work related physical demands on the upper extremity appears to increase the risk of axillosubclavian impingement and thrombosis in certain patients.ConclusionEffort thrombosis is a rare vascular condition of the upper extremity that may be seen in workers with repetitive, forceful, or overhead arm activities. Am. J. Ind. Med. 54:244–247, 2011. © 2010 Wiley‐Liss, Inc.
Barriers and enablers to returning to work from long‐term sickness absence: Part I—A quantitative perspective
Authors: Board Belinda J. , Brown Jennifer 
Published: August 2010

AbstractBackgroundLong‐term sickness absence (LTSA) in the United Kingdom labor market has become a major health issue in recent years. In contrast to short‐term sickness absence, rates for LTSA have been on the increase. This paper, part 1 of a two‐part paper, identifies individual domain barriers to returning to work (RTW) from LTSA across the work disability timeline in the UK labor market.MethodsThis is a retrospective cohort study of 6,246 workers from an occupationally diverse Police Force within the UK using a large administrative database. A series of chi‐squared analyses were conducted to analyze the between and within group associations. Next, multiple logistic regression analyses using the Enter method were performed to develop a predictive model for RTW and Absence Phase.ResultsFindings substantiated the presence of individual domain barriers to RTW and predictors of RTW outcome and established the absence phase specificity of a number of risk factors of prolonged work disability. In particular, injury/illness especially mental ill health (MIH), physical job demands, sex, and number of episodes of LTSA are significant individual domain barriers to RTW and represent important risk factors for prolonged work disability.ConclusionsDuration of work disability is associated with medical diagnosis, especially MIH, physical job demands, sex, and number of LTSA episodes. Findings also support the importance of using the outcome measure of absence phase of risk factors in addition to RTW outcome. Am. J. Ind. Med. 54:307–324, 2011. © 2010 Wiley‐Liss, Inc.
Craniofacial birth defects: The role of neural crest cells in the etiology and pathogenesis of Treacher Collins syndrome and the potential for prevention
Authors: Trainor Paul A. 
Published: August 2010

AbstractOf all the babies born with birth defects, approximately one‐third display anomalies of the head and face [Gorlin et al., 1990] including cleft lip, cleft palate, small or absent facial and skull bones and improperly formed nose, eyes, ears, and teeth. Craniofacial disorders are a primary cause of infant mortality and have serious lifetime functional, esthetic, and social consequences that are devastating to both children and parents alike. Comprehensive surgery, dental care, psychological counseling, and rehabilitation can help ameliorate‐specific problems but at great cost over many years which dramatically affects national health care budgets. For example, the Center for Disease Control and Prevention estimates that the lifetime cost of treating the children born each year with cleft lip and/or cleft palate alone to be US$697 million. Treating craniofacial malformations, of which in excess of 700 distinct syndromes have been described, through comprehensive, well‐coordinated and integrated strategies can provide satisfactory management of individual conditions, however, the results are often variable and rarely fully corrective. Therefore, better techniques for tissue repair and regeneration need to be developed and therapeutic avenues of prevention need to be explored in order to eliminate the devastating consequences of head and facial birth defects. To do this requires a thorough understanding of the normal events that control craniofacial development during embryogenesis. This review therefore focuses on recent advances in our understanding of the basic etiology and pathogenesis of a rare craniofacial disorder known as Treacher Collins syndrome and emerging prospects for prevention that may have broad application to congenital craniofacial birth defects. © 2010 Wiley‐Liss, Inc.
A novel EFNB1 mutation (c.712delG) in a family with craniofrontonasal syndrome and diaphragmatic hernia
Authors: Hogue Jacob , Shankar Suma , Perry Hazel , Patel Reena , Vargervik Karin , Slavotinek Anne 
Published: August 2010

AbstractWe report on the occurrence of congenital diaphragmatic hernia in a family with craniofrontonasal syndrome found to have a previously unreported mutation in EFNB1. The female proband presented with hypertelorism, telecanthus, bifid nasal tip, widow's peak, frontal bossing, and a widened metopic suture. Her father was noted to have hypertelorism, telecanthus, widow's peak, and a history of pectus carinatum. He was found to have a previously unreported mutation in exon 5 of EFNB1 predicted to cause premature protein truncation. The parents of the proband previously had a female fetus with congenital diaphragmatic hernia. The occurrence of congenital diaphragmatic hernia, phenotypic differences between males and females, and utility of molecular testing in craniofrontonasal syndrome are demonstrated. © 2010 Wiley‐Liss, Inc.
Caudal dysgenesis and sirenomelia‐single centre experience suggests common pathogenic basis
Authors: Thottungal Anjana D. , Charles Adrian K. , Dickinson Jan E. , Bower Carol 
Published: August 2010

AbstractAbnormally formed lower limbs with varying degrees of fusion are the major feature of sirenomelia whereas maldeveloped lower limbs without fusion are found in association with caudal dysgenesis (CD). The relationship between these two entities has been a topic of debate for many years. The presence of a single umbilical artery originating from the abdominal aorta was considered a major feature distinguishing sirenomelia from CD. Based on this finding, the vascular steal theory was put forward as the causative mechanism of sirenomelia. CD and sirenomelia were considered to be two entirely different entities with distinct pathogenic mechanisms. However, it is now clear that a single umbilical artery can be found in some patients of CD and normal umbilical arteries in some patients of sirenomelia. The hypothesis of primary deficiency of caudal mesoderm caused by early developmental disruption suggests that sirenomelia and CD are two ends of a spectrum of maldevelopment of caudal mesoderm. In this paper we report on the clinical and pathological features of 16 patients of CD and 9 patients of sirenomelia from our institution and review the literature. This series of cases is notable for the significant association with neural tube defects, refining the renal and urogenital pathology associated with these conditions, and supporting the concept of a continuum of the disease spectrum. © 2010 Wiley‐Liss, Inc.
Clericuzio‐type poikiloderma with neutropenia syndrome in three sibs with mutations in the C16orf57 gene: Delineation of the phenotype
Authors: Concolino D. , Roversi G. , Muzzi G.L. , Sestito S. , Colombo E.A. , Volpi L. , Larizza L. , Strisciuglio P. 
Published: August 2010

AbstractWe report on three sibs who have autosomal recessive Clericuzio‐type poikiloderma neutropenia (PN) syndrome. Recently, this consanguineous family was reported and shown to be informative in identifying the C16orf57 gene as the causative gene for this syndrome. Here we present the clinical data in detail. PN is a distinct and recognizable entity belonging to the group of poikiloderma syndromes among which Rothmund–Thomson is perhaps the best described and understood. PN is characterized by cutaneous poikiloderma, hyperkeratotic nails, generalized hyperkeratosis on palms and soles, neutropenia, short stature, and recurrent pulmonary infections. In order to delineate the phenotype of this rare genodermatosis, the clinical presentation together with the molecular investigations in our patients are reported and compared to those from the literature. © 2010 Wiley‐Liss, Inc.
Somatic mosaicism for chromosome X and Y aneuploidies in monozygotic twins heterozygous for sickle cell disease mutation
Authors: Razzaghian Hamid Reza , Shahi Mehdi Hayat , Forsberg Lars A. , de Ståhl Teresita Diaz , Absher Devin , Dahl Niklas , Westerman Maxwell P. , Dumanski Jan P. 
Published: August 2010

AbstractSomatic genetic variation in health and disease is poorly explored. Monozygotic (MZ) twins are a suitable model for studies of somatic mosaicism since genetic differences in twins derived from the same zygote represent an irrefutable example of somatic variation. We report the analysis of a pair of generally healthy female MZ twins, discordant for somatic mosaicism for aneuploidy of chromosomes X and Y. Both twins are heterozygous carriers of sickle cell disease mutation. Genotyping of blood DNA from both twins using Illumina Human 610 SNP array revealed a copy number imbalance for chromosome X in a proportion of cells in one twin. Fluorescent in situ hybridization (FISH) analysis confirmed monosomy X (45,X) in 7% of proband nucleated blood cells. Unexpectedly, FISH analysis of cells from the other twin revealed 45,X and 46,XY lineages, both present in 1% of cells. The mechanism behind formation of these aneuploidies suggests several aberrant chromosome segregation events in meiosis and mitoses following conception. Our report contributes to the delineation of the frequency of somatic structural genomic variation in normal MZ twins. These results also illustrate the plasticity of the human genome for tolerating large copy number changes in healthy subjects and show the sensitivity of the Illumina platform for detection of aberrations that are present in a minority of the studied cells. © 2010 Wiley‐Liss, Inc.
Recurrence risk for offspring of twins discordant for oral cleft: A population‐based cohort study of the Danish 1936–2004 cleft twin cohort
Authors: Grosen Dorthe , Bille Camilla , Pedersen Jacob Krabbe , Skytthe Axel , Murray Jeffrey C , Christensen Kaare 
Published: August 2010

AbstractOur objective in this Danish population‐based cohort study was to estimate the recurrence risk of isolated oral cleft (OC) for offspring of the unaffected co‐twins of OC discordant twin pairs and to compare this risk to the recurrence risk in the offspring of the affected co‐twin as well as to the risk in the background population. During 1936–2004, 207 twin pairs were ascertained, among whom at least one twin had an OC. The index persons were twins discordant for OC who had children (N = 117), and their offspring (N = 239). The participants were ascertained by linkage between The Danish Facial Cleft Database, The Danish Twin Registry and The Danish Civil Registration System. In the study OC recurrence risk for offspring of the affected and unaffected twin and relative risk were compared to the background prevalence. We found that among 110 children of the 54 OC affected twins, two (1.8%) children had OC corresponding to a significantly increased relative risk (RR = 10; 95% CI 1.2–35) when compared to the frequency in the background population. Among the 129 children of the 63 unaffected twins, three (2.3%) children were affected, corresponding to a significantly increased relative risk (RR = 13; 95% CI 2.6–36) when compared the background prevalence. We concluded that in OC discordant twin pairs similar increased recurrence risks were found among offspring of both OC affected and OC unaffected twins. This provides further evidence for a genetic component in cleft etiology and is useful information for genetic counseling of twin pairs discordant for clefting. © 2010 Wiley‐Liss, Inc.
A de novo p.Asp18Asn mutation in TREX1 in a patient with Aicardi–Goutières syndrome
Authors: Haaxma Charlotte A. , Crow Yanick J. , van Steensel Maurice A.M. , Lammens Martin M.Y. , Rice Gillian I. , Verbeek Marcel M. , Willemsen Michèl A.A.P. 
Published: August 2010

AbstractAicardi–Goutières syndrome is a rare, genetically determined encephalopathy often resembling congenital infection. Mutations in the TREX1 gene are found in approximately 25% of patients. Aicardi–Goutières syndrome is usually inherited as an autosomal recessive trait, although a single case of a heterozygous TREX1 mutation associated with the syndrome has been reported. We present a second case of a de novo heterozygous TREX1 mutation causing an autosomal dominant phenotype of Aicardi–Goutières syndrome with additional features indicative of mitochondrial dysfunction. This report serves to enhance awareness of de novo heterozygous mutations underlying Aicardi–Goutières syndrome—with a concomitant low risk of recurrence. © 2010 Wiley‐Liss, Inc.
De novo ACTA2 mutation causes a novel syndrome of multisystemic smooth muscle dysfunction
Authors: Milewicz Dianna M. , Østergaard John R. , Ala‐Kokko Leena M. , Khan Nadia , Grange Dorothy K. , Mendoza‐Londono Roberto , Bradley Timothy J. , Olney Ann Haskins , Adès Lesley , Maher Joseph F. , Guo Dongchuan , Buja L. Maximilian , Kim Dong , Hyland James C. , Regalado Ellen S. 
Published: August 2010

AbstractSmooth muscle cells (SMCs) contract to perform many physiological functions, including regulation of blood flow and pressure in arteries, contraction of the pupils, peristalsis of the gut, and voiding of the bladder. SMC lineage in these organs is characterized by cellular expression of the SMC isoform of α‐actin, encoded by the ACTA2 gene. We report here on a unique and de novo mutation in ACTA2, R179H, that causes a syndrome characterized by dysfunction of SMCs throughout the body, leading to aortic and cerebrovascular disease, fixed dilated pupils, hypotonic bladder, malrotation, and hypoperistalsis of the gut and pulmonary hypertension. © 2010 Wiley‐Liss, Inc.