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Fragility
Kinds of Fragility Terms modified by Fragility Selected AbstractsThe Factor Structure of Chinese Personality TermsJOURNAL OF PERSONALITY, Issue 2 2009Xinyue Zhou ABSTRACT From the Contemporary Chinese Dictionary, 3,159 personality descriptors were selected and then ranked by the frequency of use. Among those, the top 413 terms with the highest frequency were administered to two independent large samples in China for self-ratings and peer ratings to explore the emic Chinese personality structure as well as to test the universality of other models. One- and two-factor structures found in previous studies of other languages were well replicated. Previous structures with more than two factors were not well replicated, but six- and seven-factor models were at least as well supported as the Big Five. Emic analysis indicated that a seven-factor structure was the most informative structure relatively salient across subsamples of self-ratings and peer ratings, across original and ipsatized data, and across differences in variable selections. These factors can be called Extraversion, Conscientiousness/Diligence, Unselfishness, Negative Valence, Emotional Volatility, Intellect/Positive Valence, and Dependency/Fragility. [source] Bone Fragility Contributes to the Risk of Fracture in Children, Even After Moderate and Severe Trauma,,JOURNAL OF BONE AND MINERAL RESEARCH, Issue 2 2008Emma M Clark Abstract We prospectively examined whether the relationship between skeletal fragility and fracture risk in children 9.9 ± 0.3 (SD) yr is affected by trauma level. Bone size relative to body size and humeral vBMD showed similar inverse relationships with fracture risk, irrespective of whether fractures followed slight or moderate/severe trauma. Introduction: Fracture risk in childhood is related to underlying skeletal fragility. However, whether this relationship is confined to low-trauma fractures or whether skeletal fragility also contributes to the risk of fracture caused by higher levels of trauma is currently unknown. Materials and Methods: Total body DXA scan results obtained at 9.9 yr of age were linked to reported fractures over the following 2 yr in children from the Avon Longitudinal Study of Parents and Children. DXA scan results that were subsequently derived included total body less head (TBLH) bone size relative to body size (calculated from TBLH area adjusted for height and weight) and humeral volumetric BMD (vBMD; derived from subregional analysis at this site). Trauma level was assigned using the Landin classification based on a questionnaire asking about precipitating causes. Results: Of the 6204 children with available data, 549 (8.9%) reported at least one fracture over the follow-up period, and trauma level was assigned in 280 as follows: slight trauma, 56.1%; moderate trauma, 41.0%; severe trauma, 2.9%. Compared with children without fractures, after adjustment for age, sex, socioeconomic status, and ethnicity, children with fractures from both slight and moderate/severe trauma had a reduced bone size relative to body size (1133 cm2 in nonfractured children versus 1112 cm2 for slight trauma fractures, p < 0.001; 1112 cm2 for moderate/severe trauma fractures, p = 0.001) and reduced humeral vBMD (0.494 g/cm3 in nonfractured children versus 0.484 g/cm3 for slight trauma fractures, p = 0.036; and 0.482g/cm3 for moderate/severe trauma fractures, p = 0.016). Conclusions: Skeletal fragility contributes to fracture risk in children, not only in fractures caused by slight trauma but also in those that result from moderate or severe trauma. [source] Evidence From Data Searches and Life-Table Analyses for Gender-Related Differences in Absolute Risk of Hip Fracture After Colles' or Spine Fracture: Colles' Fracture as an Early and Sensitive Marker of Skeletal Fragility in White Men,JOURNAL OF BONE AND MINERAL RESEARCH, Issue 12 2004Patrick Haentjens Abstract Based on data searches and life-table analyses, we determined the long-term (remaining lifetime) and short-term (10- and 5-year) absolute risks of hip fracture after sustaining a Colles' or spine fracture and searched for potential gender-related differences. In aging men, Colles' fractures carry a higher absolute risk for hip fracture than spinal fractures in contrast to women. These findings support the concept that forearm fracture is an early and sensitive marker of male skeletal fragility. Introduction: Colles' fracture occurrence has been largely ignored in public health approaches to identify target populations at risk for hip fracture. The aim of this study was to estimate the long-term and short-term absolute risks of hip fracture after sustaining a Colles' or spine fracture and to search for potential gender-related differences in the relationship between fracture history and future fracture risk. Materials and Methods: To determine the long-term (remaining lifetime) and short-term (10- and 5-year) absolute risks of hip fracture, we applied life-table methods using U.S. age- and sex-specific hip fracture incidence rates, U.S. age-specific mortality rates for white women and men, pooled hazard ratios for mortality after Colles' and spine fracture, and pooled relative risks for hip fracture after Colles' and spine fracture, estimated from cohort studies by standard meta-analytic methods. Results: Our results indicate that the estimated remaining lifetime risks are dependent on age in both genders. In women, remaining lifetime risks increase until the age of 80 years, when they start to decline because of the competing probabilities of fracture and death. The same pattern is found in men until the age of 85 years, the increment in lifetime risk being even more pronounced. As expected, the risk of sustaining a hip fracture was found to be higher in postmenopausal women with a previous spine fracture compared with those with a history of Colles' fracture. In men, on the other hand, the prospective association between fracture history and subsequent hip fracture risk seemed to be strongest for Colles' fracture. At the age of 50, for example, the remaining lifetime risk was 13% in women with a previous Colles' fracture compared with 15% in the context of a previous spine fracture and 9% among women of the general population. In men at the age of 50 years, the corresponding risk estimates were 8%, 6%, and 3%, respectively. Similar trends were observed when calculating 5- and 10-year risks. Conclusions: In aging men, Colles' fractures carry a higher absolute risk for hip fracture than spinal fractures in contrast to women. These findings support the concept that forearm fracture is an early and sensitive marker of male skeletal fragility. The gender-related differences reported in this analysis should be taken into account when designing screening and treatment strategies for prevention of hip fracture in men. [source] Bone Fragility and Collagen Cross-Links,JOURNAL OF BONE AND MINERAL RESEARCH, Issue 12 2004Eleftherios P Paschalis Abstract Infrared imaging analysis of iliac crest biopsy specimens from patients with osteoporotic and multiple spontaneous fractures shows significant differences in the spatial variation of the nonreducible:reducible collagen cross-links at bone-forming trabecular surfaces compared with normal bone. Introduction: Although the role of BMC and bone mineral quality in determining fracture risk has been extensively studied, considerably less attention has been paid to the quality of collagen in fragile bone. Materials and Methods: In this study, the technique of Fourier transform infrared imaging (FTIRI) was used to determine the ratio of nonreducible:reducible cross-links, in 2- to 4-,m-thick sections, from human iliac crest biopsy specimens (N = 27) at bone-forming trabecular surfaces. The biopsy specimens were obtained from patients that had been diagnosed as high- or low-turnover osteoporosis, as well as premenopausal women <40 years of age, with normal BMD and biochemistry, who suffered multiple spontaneous fractures. The obtained values were compared with previously published analyses of trabecular bone from normal non-osteoporotic subjects (N = 14, 6 males and 8 females; age range, 51,70 years). Results and Conclusions: Collagen cross-links distribution within the first 50 ,m at forming trabecular surfaces in patients with fragile bone was markedly different compared with normal bone. [source] Gnathodiaphyseal Dysplasia: A Syndrome of Fibro-Osseous Lesions of Jawbones, Bone Fragility, and Long Bone BowingJOURNAL OF BONE AND MINERAL RESEARCH, Issue 9 2001Mara Riminucci Abstract We report an unusual generalized skeletal syndrome characterized by fibro-osseous lesions of the jawbones with a prominent psammomatoid body component, bone fragility, and bowing/sclerosis of tubular bones. The case fits with the emerging profile of a distinct syndrome with similarities to previously reported cases, some with an autosomal dominant inheritance and others sporadic. We suggest that the syndrome be named gnathodiaphyseal dysplasia. The patient had been diagnosed previously with polyostotic fibrous dysplasia (PFD) elsewhere, but further clinical evaluation, histopathological study, and mutation analysis excluded this diagnosis. In addition to providing a novel observation of an as yet poorly characterized syndrome, the case illustrates the need for stringent diagnostic criteria for FD. The jaw lesions showed fibro-osseous features with the histopathological characteristics of cemento-ossifying fibroma, psammomatoid variant. This case emphasizes that the boundaries between genuine GNAS1 mutation-positive FD and other fibro-osseous lesions occurring in the jawbones should be kept sharply defined, contrary to a prevailing tendency in the literature. A detailed pathological study revealed previously unreported features of cemento-ossifying fibroma, including the participation of myofibroblasts and the occurrence of psammomatoid bodies and aberrant mineralization, within the walls of blood vessels. Transplantation of stromal cells grown from the lesion into immunocompromised mice resulted in a close mimicry of the native lesion, including the sporadic formation of psammomatoid bodies, suggesting an intrinsic abnormality of bone-forming cells. [source] Inaccuracies Inherent in Dual-Energy X-Ray Absorptiometry In Vivo Bone Mineral Density Can Seriously Mislead Diagnostic/Prognostic Interpretations of Patient-Specific Bone FragilityJOURNAL OF BONE AND MINERAL RESEARCH, Issue 5 2001H. H. Bolotin First page of article [source] Heterogeneity in the Growth of the Axial and Appendicular Skeleton in Boys: Implications for the Pathogenesis of Bone Fragility in MenJOURNAL OF BONE AND MINERAL RESEARCH, Issue 10 2000Michelle Bradney Abstract Men with spine fractures have reduced vertebral body (VB) volume and volumetric bone mineral density (vBMD). Men with hip fractures have reduced femoral neck (FN) volume and vBMD, site-specific deficits that may have their origins in growth. To describe the tempo of growth in regional bone size, bone mineral content (BMC), and vBMD, we measured bone length, periosteal and endocortical diameters, BMC, and vBMD using dual-energy X-ray absorptiometry in 184 boys aged between 7 and 17 years. Before puberty, growth was more rapid in the legs than in the trunk. During puberty, leg growth slowed while trunk length accelerated. Bone size was more advanced than BMC in all regions, being ,70% and ,35% of their predicted peaks at 7 years of age, respectively. At 16 years of age, bone size had reached its adult peak while BMC was still 10% below its predicted peak. The legs accounted for 48%, whereas the spine accounted for 10%, of the 1878 g BMC accrued between 7 and 17 years. Peripubertal growth contributed (i) 55% of the increase in leg length but 78% of the mineral accrued and (ii) 69% of the increase in spine length but 87% of the mineral accrued. Increased metacarpal and midfemoral cortical thickness was caused by respective periosteal expansion with minimal change in the endocortical diameter. Total femur and VB vBMD increased by 30,40% while size and BMC increased by 200,300%. Thus, growth builds a bigger but only slightly denser skeleton. We speculate that effect of disease or a risk factor during growth depends on the regions maturational stage at the time of exposure. The earlier growth of a regions size than mass, and the differing growth patterns from region to region, predispose to site-specific deficits in bone size, vBMD, or both. Regions further from their peak may be more severely affected by illness than those nearer completion of growth. Bone fragility in old age is likely to have its foundations partly established during growth. [source] Seismic design of bridges accounting for spatial variability of ground motionEARTHQUAKE ENGINEERING AND STRUCTURAL DYNAMICS, Issue 4-5 2005A. Lupoi Abstract The effects of the spatial variability of the ground motion on the response of bridge structures are investigated in this study. Following a well-established convention, the phenomenon is represented as the combined effect of three causes: the loss of coherence of the motion with distance, the wave-passage, and the local site conditions. Since the nature and amount of non-synchronism vary within ample limits a statistical approach is adopted. A parametric study is carried out on a representative set of bridges subjected to carefully selected combinations of the factors inducing spatial variability. The investigation has shown that the phenomenon affects the response considerably and, hence, the level of protection of these structures. It is observed that for all bridge types considered, the ductility demands at the base of the piers in the presence of spatial variability increase in the majority of cases. Further, for a given bridge type, the probabilities of failure vary by more than one order of magnitude depending on the combination of the parameters. Attention has been focused on a parameter representing the ratio between the maximum curvature ductility demand and the same quantity for the case of fully synchronous motion. This parameter has been used to correct the conventional synchronous design procedure by increasing the available ductility. The re-analysis of all the cases with a modified ductility capacity shows that the procedure is effective in reducing the fragilities to the values corresponding to synchronous input. Copyright © 2005 John Wiley & Sons, Ltd. [source] US Credit Crisis and Spillovers to AsiaASIAN ECONOMIC POLICY REVIEW, Issue 2 2009Morris GOLDSTEIN F41; F34; F31; F37 We review key highlights of the global credit crisis. We then consider how financial turmoil in the largest advanced economies might be transmitted to East Asia. The focus is on foreign trade links, international capital flows, currency market pressures and mismatches, financial sector fragilities, and countercyclical monetary and fiscal policy actions. We introduce a set of vulnerability indicators and explore whether an ordinal ranking of East Asian economies according to these vulnerability indicators seems to be related to the cross-country differences in estimated slowdowns of economic growth during the crisis. Finally, we discuss how Asian economies might encourage the adoption of a stronger regulatory and supervisory framework in the USA and whether some Asian economies and the USA might pursue a more "balanced" growth strategy after the crisis. [source] Occupational nail fragility in a professional violistCONTACT DERMATITIS, Issue 1 2004Bianca Maria Piraccini No abstract is available for this article. [source] Analysing Macro-Poverty Linkages of External Liberalisation: Gaps, Achievements and AlternativesDEVELOPMENT POLICY REVIEW, Issue 3 2005Bernhard G. Gunter CGE modelling has dominated analysis of the impact of external liberalisation on poverty. This article provides a structuralist critique of standard neo-classical CGE models. It highlights five sets of gaps and partial achievements in the modelling of issues affecting the poverty impact of macroeconomic policies: duality and structural rigidities; efficiency gains and quota rents; the investment and savings specification; the nature of public expenditures; and the modelling of financial fragility, risk premia and issues of credibility. It outlines a model that makes it possible to analyse more plausible stories about the impact of both current and capital account liberalisation and questions the realism of existing approaches to ex-ante poverty impact assessment. [source] PERORAL PANCREATOSCOPY: CURRENT STATUS AND FUTURE EXPECTATIONS USING NARROW BAND IMAGINGDIGESTIVE ENDOSCOPY, Issue 2007Yoshifumi Arisaka Peroral pancreatoscopy (POPS) under duodenoscopic assistance provide direct visual assessment of the pancreatic duct, tissue sampling, and therapeutic interventions. Sometimes, pancreatoscopy can confirm accurate diagnosis, such as differential diagnosis of filling defects between intraductal tumors and stones. However, it is often difficult to differentiate malignant from benign strictures solely on pancreatoscopy. It is currently considered that intraductal papillary mucinous neoplasm (IPMN) is the most suitable indication of POPS. POPS has several problems: image resolution, fragility and maneuverability. Concerning image resolution, the quality has been improved with the development of a video pancreatoscope. Moreover, the recently developed endoscopic optical technology of narrow band imaging (NBI) is now available to video pancreatoscopy. This will allow direct visual assessment. Although currently POPS has several problems, further improvement will assist POPS to become a useful modality in combination with NBI. [source] Error estimation of closed-form solution for annual rate of structural collapseEARTHQUAKE ENGINEERING AND STRUCTURAL DYNAMICS, Issue 15 2008Brendon A. Bradley Abstract With the increasing emphasis of performance-based earthquake engineering in the engineering community, several investigations have been presented outlining simplified approaches suitable for performance-based seismic design (PBSD). Central to most of these PBSD approaches is the use of closed-form analytical solutions to the probabilistic integral equations representing the rate of exceedance of key performance measures. Situations where such closed-form solutions are not appropriate primarily relate to the problem of extrapolation outside of the region in which parameters of the closed-form solution are fit. This study presents a critical review of the closed-form solution for the annual rate of structural collapse. The closed-form solution requires the assumptions of lognormality of the collapse fragility and power model form of the ground motion hazard, of which the latter is more significant regarding the error of the closed-form solution. Via a parametric study, the key variables contributing to the error between the closed-form solution and solution via numerical integration are illustrated. As these key variables cannot be easily measured, it casts doubt on the use of such closed-form solutions in future PBSD, especially considering the simple and efficient nature of using direct numerical integration to obtain the solution. Copyright © 2008 John Wiley & Sons, Ltd. [source] Hb Woodville, a rare , -globin variant, caused by codon 6 mutation of the ,1 geneEUROPEAN JOURNAL OF HAEMATOLOGY, Issue 1 2006Vip Viprakasit Abstract:, Since 1995, the national programme for the prevention and control of severe thalassaemia has been implemented in Thailand. This programme is composed of the population screening in pregnant women and couples by osmotic fragility, HbE screening and the confirmation test using haemoglobin analyses by electrophoresis or chromatography. Thereafter, several hitherto unidentified haemoglobins (Hbs) with structural defects are increasingly described and these variants are now easily studied using DNA technology. In this study, the authors describe the haematology and molecular analyses in a 28-yr-old healthy female who was identified as having an exceptionally ,high HbA2' from haemoglobin analysis. Subsequent analyses demonstrated that observed atypical ,HbA2' was, in fact, a rare innocuous , -globin variant, called Hb Woodville [alpha 2 6(A4); Asp , Tyr]. For the first time, this abnormal Hb species is characterised at the molecular level. [source] De novo mutations in monilethrixEXPERIMENTAL DERMATOLOGY, Issue 6 2003Liran Horev Abstract: Mutations in the hair keratins hHb1 and hHb6 have been recently reported to cause monilethrix, an autosomal dominant hair shaft disorder, characterized by variable degrees of hair fragility and follicular hyperkeratosis. We found 10 families with monilethrix in which the parents were not clinically affected, and sequenced the hair keratin hHb1, hHb3 and hHb6 genes in seven patients. In five patients no mutations were found, while in two patients we identified de novo germline missense mutations at the helix termination motif: E402K (hHb6) and E413K (hHb1). [source] Genotype,phenotype correlation in skin fragility-ectodermal dysplasia syndrome resulting from mutations in plakophilin 1EXPERIMENTAL DERMATOLOGY, Issue 2 2002T. Hamada Abstract: We report a 42-year-old Japanese man with an unusual autosomal recessive genodermatosis. The clinical features comprised normal skin at birth, loss of scalp hair at 3-months of age after a febrile illness, progressive nail dystrophy during infancy, palmoplantar keratoderma starting around the age of 18 years and trauma-induced skin fragility and blisters noted from the age of 20 years. Skin biopsy of rubbed non-lesional skin revealed widening of spaces between adjacent keratinocytes from the suprabasal layer upwards. Electron microscopy demonstrated a reduced number of hypoplastic desmosomes. Immunohistochemical labeling showed a reduction in intercellular staining for the desmosome component plakophilin 1. Mutation analysis revealed a homozygous intron 11 donor splice site mutation in the plakophilin 1 gene, 2021+1 G>A (GenBank no. Z34974). RT-PCR, using RNA extracted from the skin biopsy, provided evidence for residual low levels of the full-length wild-type transcript (,8%) as well as multiple other near full-length transcripts, one of which was in frame leading to deletion of 17 amino acids from the 9th arm-repeat unit of the plakophilin 1 tail domain. Thus, the molecular findings help explain the clinical features in the patient, who has a similar but milder phenotype to previously reported patients with skin fragility-ectodermal dysplasia syndrome associated with complete ablation of plakophilin 1 (OMIM 604536). This new ,mitis' phenotype provides further clinicopathological evidence for the role of plakophilin 1 in keratinocyte cell,cell adhesion and ectodermal development. [source] Novel keratin 16 mutations and protein expression studies in pachyonychia congenita type 1 and focal palmoplantar keratodermaEXPERIMENTAL DERMATOLOGY, Issue 3 2000F. J. D. Smith Abstract: Pachyonychia congenita type 1 (PC-1) is an autosomal dominant ectodermal dysplasia characterized by nail dystrophy, focal non-epidermolytic palmoplantar keratoderma (FNEPPK) and oral lesions. We have previously shown that mutations in keratin 16 (K16) cause fragility of specific epithelia resulting in phenotypes of PC-1 or FNEPPK alone. Here, we report 2 novel mutations in K16 causing distinct phenotypes. A heterozygous missense mutation (L124R) was detected in a kindred with PC-1. In a family where mild FNEPPK was the only phenotype, a 23 bp deletion and a separate 1 bp deletion downstream were found in exon 6: [1244,1266del; 1270delG]. At the protein level, these mutations remove 8 residues and substitute 2 residues in the helix termination motif (HTM) of the K16 polypeptide. The HTM sequence is conserved in all known intermediate filament proteins and for convenience, this complex mutation was designated ,HTM. Transient expression of K16 cDNAs carrying either the L124R or the ,HTM mutation in epithelial cell line PtK2 produced aggregation of the keratin cytoskeleton. However, the aggregates observed with the ,HTM mutation were morphologically different and appeared to be less disruptive to the endogenous cytoskeleton. Therefore, loss of the HTM sequence may render this mutant K16 less capable of contributing to filament assembly and decrease its dominant-negative effect, resulting in the milder FNEPPK phenotype. [source] The Origins of Modern DivorceFAMILY PROCESS, Issue 1 2007STEPHANIE COONTZ High rates of marital dissolution and easy access to divorce are not unprecedented, historically or cross-culturally. But contemporary divorce in North America and Western Europe has different origins and features than divorce in previous cultures. The origins of modern divorce patterns date back more than 200 years, to the invention of the historically unprecedented idea that marriage should be based on love and mutual affection. Ironically, then, the fragility of modern marriage stems from the same values that have elevated the marital relationship above all other personal and familial commitments: the concentration of emotion, passion, personal identity, and self-validation in the couple relationship and the attenuation of emotional attachments and obligations beyond the conjugal unit. The immediate causes of divorce may range from factors as diverse as the personal psychological characteristics of one or both spouses to the stresses of economic hardship and community disintegration. But in a larger perspective, the role of divorce in modern societies and its relatively high occurrence both flow from the same complex of factors that have made good marriages so much more central to people's happiness than through most of the past, and deterioration of a marital relationship so much more traumatic. [source] Modesty and Excellence: Gender and Sports Culture in Dutch Catholic Schooling, 1900,40GENDER & HISTORY, Issue 1 2008Marjet Derks This article focuses on the construction of gendered identities in Dutch schools run by religious orders during the process of Catholic emancipation. It discusses the fragility of identity, the simultaneity of tradition and modernity, and the role that gender plays in all these interactions. Two schools in the city of Nijmegen, the Netherlands, are compared during the first half of the twentieth century: the Jesuit institution for boys, Canisius College, and the Ursuline college for girls next door, Mater Dei. At both, sport played an important role in the transmission of notions of Catholicism and gender. [source] Chromosomal fragile sites FRA3B and FRA16D show correlated expression and association with failure of apoptosis in lymphocytes from patients with thyroid cancerGENES, CHROMOSOMES AND CANCER, Issue 5 2006Isabella Sbrana It has been suggested that common fragile sites (cFSs) are related to cancer development. This appears to be the case for FRA3B and FRA16D, localized in two tumor-suppressor genes (FHIT and WWOX, respectively) that are altered by deletions or loss of heterozygosity (LOH) in many cancers. The features responsible for fragility have not yet been identified. Furthermore, it is still unclear whether instability at these regions causes chance deletions and loss of function of the associated genes, or whether the gene function itself is related to the appearance of fragility. In this study, we analyzed cFS expression in lymphocytes from 20 healthy or thyroid cancer,affected subjects exposed to radiation after the Chernobyl accident. The same cells were examined for apoptosis, a principal function of both the FHIT and WWOX genes. Exceptionally elevated chromosome fragility was observed, particularly in cancer patients, affecting FRA3B, FRA16D, and a cluster of less highly expressed cFSs; levels of chromosome fragility were found to be correlated among these cFSs. Interestingly, most expressed cFSs were sites of LOH reported for thyroid tumors; moreover, cells with the highest fragility also had a reduced ability to undergo apoptosis. These findings reveal previously unknown genetic interactions affecting fragile loci, suggestive of a shared function inside mitotic cells. Attenuation of checkpoint control and apoptosis resistance seem to be the cell phenotypes associated with unusual chromosome fragility. We propose that breakage at specific cFS could derive from early epigenetic events at loci involved in radiation carcinogenesis. This article contains Supplementary Material available at http://www.interscience.wiley.com/jpages/1045-2257/suppmat. © 2006 Wiley-Liss, Inc. [source] Post-excavation deterioration of the Copney Bronze Age Stone Circle Complex: A geomorphological perspectiveGEOARCHAEOLOGY: AN INTERNATIONAL JOURNAL, Issue 5 2010Patricia A. Warke Post-excavation deterioration of stones from under blanket peat at the Copney Bronze Age Stone Circle Complex in County Tyrone, Northern Ireland, proceeded through widespread scaling, flaking, and splitting of stones. Investigation showed that prior to burial the porphyritic stones already possessed a complex legacy of geological weaknesses derived from hydrothermal alteration and tectonic deformation. Analysis indicated that significant alteration occurred during approximately 2000 years of burial under acidic peat cover, with development of a secondary porosity, alteration of primary minerals, and the opening of preexisting lines of weakness within the stones. Burial under peat also resulted in "bleaching" the stones so that they appeared white in color following excavation. These alterations during burial left the stones in a significantly weakened state and particularly susceptible to the effects of subaerial weathering processes. Data underline the potential fragility of excavated stonework and highlight the need to plan for its aftercare before complete excavation is undertaken. © 2010 Wiley Periodicals, Inc. [source] Quality, imagery and marketing: producer perspectives on quality products and services in the lagging rural regions of the European UnionGEOGRAFISKA ANNALER SERIES B: HUMAN GEOGRAPHY, Issue 1 2001Brian Ilbery A range of factors, including consumer concerns about food safety, the growing popularity of rural tourism and policy initiatives to promote endogenous rural development, is converging to promote a relocalization of food production and service provision, especially in those regions marginalized by the globalization of the food supply system. The recent outbreak of foot and mouth disease in the UK has starkly illustrated the fragility of localized systems which depend heavily on consumers travelling to them. Within such a context, the importance of successful marketing strategies has become even more apparent. This paper reports on a questionnaire survey which investigated promotional and marketing strategies among a diverse range of producers and service providers in marginal agricultural areas of the EU. The findings suggest that many producers are situated towards the "formal" end of a marketing continuum, whereby ability to promote quality products and services (QPS) lies with a range of intermediaries. This raises doubts about the future economic benefits of QPS, should current marketing structures persist. The discussion offers critical reflections on interdisciplinary and international research of this nature, and advocates further theoretical and methodological development in order to explore in more depth many of the aspects raised in this exploratory investigation. [source] COL5A1 signal peptide mutations interfere with protein secretion and cause classic Ehlers-Danlos syndrome,HUMAN MUTATION, Issue 2 2009Sofie Symoens Abstract Classic Ehlers-Danlos syndrome (EDS) is a heritable connective tissue disease characterized by skin hyperextensibility, atrophic scarring, joint hypermobility and generalized tissue fragility. Mutations in COL5A1 and COL5A2, encoding the type V collagen pro,1- and pro,2-chain, are found in ,50% of patients with classic EDS. The majority of mutations lead to a non-functional COL5A1 allele, as a result of the introduction of a premature stopcodon in one COL5A1 transcript. A minority of mutations affect the structure of the type V collagen central helical domain. We show that mutations in the signal peptide (SP) domain of the preproá1(V)-collagen chain cause classic EDS. The missense mutations (p.L25R and p.L25P) are located in the crucial hydrophobic SP core, which is indispensible for preprotein translocation into the endoplasmic reticulum. As a result, mutant type V procollagen is retained within the cell, leading to a decreased amount of type V collagen in the extracellular matrix and disturbed collagen fibrillogenesis. Our findings further support the observation that decreased availability of type V (pro)collagen is a key factor and a shared mechanism in the pathogenesis of classic EDS. © 2008 Wiley-Liss, Inc. [source] Angiopoietin-2 in experimental colitisINFLAMMATORY BOWEL DISEASES, Issue 6 2010Vijay C. Ganta PhD Abstract Background: The pathophysiology of inflammatory bowel disease (IBD) includes leukocyte infiltration, blood and lymphatic remodeling, weight loss and protein enteropathy. The roles of angiopoietin-2 (Ang-2) in initiating gut inflammation, leukocyte infiltration and angiogenesis are not well understood. Methods: Disease activity index, histopathological scoring, myeloperoxidase assay, immunohistochemistry and sodium dodecyl sulphate- polyacrylamide gel electrophoretic methods were employed in the present study to addess the roles of Ang-2 in experimental colitis. Results: Several important differences were seen in the development of experimental IBD in Ang-2,/, mice. Although weight change and disease activity differ only slightly in WT and Ang-2,/, + DSS treated mice, leukocyte infiltration, inflammation and blood and lymphatic vessel density is significantly attenuated compared to WT + DSS mice. Gut capillary fragility and water export (stool blood and form) appear significantly earlier in Ang-2,/, + DSS mice vs. WT. Colon lengths were also significantly reduced in Ang-2,/, and gut histopathology was less severe in Ang-2,/, compared to WT + DSS. Lastly, the decrease in serum protein content in WT + DSS was less severe in Ang-2,/, + DSS, thus protein losing enteropathy (PLE) a feature of IBD is relieved by Ang-2,/,. Conclusion: These data demonstrate that in DSS colitis, Ang-2 mediates inflammatory hemangiogenesis, lymphangiogenesis and neutrophil infiltration to reduce some, but not all clinical features of IBD. The implications for Ang-2 manipulation in the development of IBD and other inflammatory diseases and treatments involving Ang-2 are discussed. (Inflamm Bowel Dis 2009) [source] The Y chromosome and its fragilityINTERNATIONAL JOURNAL OF ANDROLOGY, Issue 4 2008C. Krausz No abstract is available for this article. [source] Lichenoid nail changes as sole external manifestation of graft vs. host diseaseINTERNATIONAL JOURNAL OF DERMATOLOGY, Issue 1 2002Sara Isabel Palencia MD A 56-year-old-man who had refractory anemia with an excess of blasts underwent an allogeneic peripheral blood stem cell transplantation (PBSCT) from his brother after preparation with melphalan and fludarabin. He received GvHD (graft-vs.-host disease) prophylaxis with cyclosporine from day ,1 at a daily dose of 5 mg/kg of body weight. The daily dosage was tapered gradually from day +20. On post-PBSCT day 68 he developed acute cutaneous GvHD grade 3 and acute gastrointestinal GvHD grade 2,3, which was resolved with a daily dose of 1 mg/kg of body weight of prednisone. The patient was discharged in good clinical condition and without signs of GvHD, and he started tapering his immunosuppressive treatment. By day 160 he developed oral lichen planus-like changes, with several reticulate white lesions on the oral mucosa. A biopsy specimen was microscopically consistent with lichenoid GvHD (Fig. 1). By day 150 after PBSCT, when he was being treated with CsA 100 mg once daily and prednisone 10 mg once daily, his fingernails started to grow abnormally and gradually became dystrophic and painful. Two months later his toenails became similarly affected. Although affecting all finger and toe nails, the lesions were especially important in both thumbs. Physical examination revealed multiple findings on his nails (Fig. 2): thickening, fragility, onycholysis, longitudinal striations, and even pterygium. The micological cultures were negative. A biopsy specimen showed an sparse papillary dermis lymphoid infiltrate with focal exocytosis and presence of isolated multiple necrotic keratinocytes (Fig. 3). These findings were interpreted as a lichenoid GvHD with oral and nail involvement. The patient did not have other associated cutaneous lesions. He did not develop signs or symptoms consistent with hepatic GvHD. In May 2000 thalidomide was added to the immunosuppressive therapy, at a daily dose from 100 to 300 mg according to tolerance (constipation, sedation, ,). The lesions on the oral mucous showed a substantial improvement, but the nail changes remained more or less stable. Thalidomide was discontinued after 7 months because the patient displayed numbness and tingling in the hands and feet consistent with a peripheral neuropathy. Twenty days later he stopped taking thalidomide and the oral lichenoid lesions worsened, resulting in difficulty in eating. He also developed periungueal erythema, swelling and intense pain after minimal trauma. The daily dose of prednisone increased to 20,30 mg with moderate improvement. However, the dose could not be increased because of the secondary immunosuppressive effects. Twenty-three months post-PBSCT the patient remains with intense oral and nail lichenoid lesions. Figure Figure 1 . Oral mucosa with a lichenoid hiperplasia and a band-like lymphoid infiltrate. Note the basal lymphocytosis with isolated necrotic keratinocytes Figure 2. Lichenoid graft-vs.-host disease showing marked nail involvement with a ridge in the midline Figure 3. Panoramic view of the nail epithelium. Dermal lymphocytes with basal exocytosis and apoptotic keratinocytes (arrow) are evident [source] Factors affecting wafer sheet qualityINTERNATIONAL JOURNAL OF FOOD SCIENCE & TECHNOLOGY, Issue 5 2006Ismail S. Dogan Summary Wafers are low-moisture-baked foods. They are formed from a batter and baked between hot plates. The quality of wafer sheets is mainly controlled by flour property, water level and temperature, mixing action, baking time and temperature. The quality is judged by attributes of the batter such as the density, viscosity, holding time and temperature, and by properties of the wafer such as weight, surface colour, fragility and moisture content. In this study, the batter-specific gravity of 1.11,1.19 was recorded. Water and gluten content did not affect density. Water level, but not gluten content, however affected viscosity. Batter holding time drastically changed viscosity. The temperature of plates did significantly affect bake time. For wafer sheets with a high quality, 155,165% water level, 170 °C baking temperature and 2 min of bake time were found to be adequate. Wafer sheets baked at the lower temperatures stuck to the plates and broke up to several pieces. At a lower water level (<145%) and baking temperature of 150 °C, tough and flinty sheets were obtained, whereas at a water level higher than 160% and a higher temperature (190 °C), fragile sheets were obtained. [source] Flow-cytometric analysis of erythrocytes and reticulocytes in congenital dyserythropoietic anaemia type II (CDA II): value in differential diagnosis with hereditary spherocytosisINTERNATIONAL JOURNAL OF LABORATORY HEMATOLOGY, Issue 1 2001P. Danise Congenital dyserythropoietic anaemia type II (CDA II) is the most common congenital dyserythropoietic anaemia. CDA II is frequently misdiagnosed as Hereditary Spherocytosis (HS) due to the presence of mild chronic haemolytic anaemia with splenomegaly, increased osmotic fragility, and presence of microspherocytes. Accurate diagnosis of CDA II is important to prevent severe iron overload. Erythrocyte and reticulocyte indices were assessed in 10 patients from six families with CDA II, 18 patients from eight families with HS, and 50 normal controls. Characteristic increases in distribution width were present in CDA II for cell volume (RDW, anisocytosis) and in HS for cell haemoglobin concentration (HDW, anisochromia), resulting in an RDW/HDW ratio which was significantly greater in CDA than HS (P < 0.0002). A cut-off value for RDW/HDW of 5.34 resulted in 89% sensitivity and 70% specificity in distinguishing CDA II from HS. Distribution width for cell haemoglobin content of reticulocytes (CHDWr) was characteristically increased in CDA II, resulting in a CHDW/CHDWr ratio significantly lower in CDA II than HS (P < 0.0002). A cut-off value of 0.98 provided 89% sensitivity and 80% specificity in distinguishing CDA II from HS. These differences in distribution widths of flow-cytometric parameters of reticulocytes and mature erythrocytes reflect the different pathogeneses of the two diseases and are helpful for the differential diagnosis of these two conditions. [source] Llong Casnewydd: the Newport Ship,a Personal ViewINTERNATIONAL JOURNAL OF NAUTICAL ARCHAEOLOGY, Issue 1 2004Owain T. P. Roberts The discovery of a late medieval ship on a building site at Newport (Casnewydd) in south Wales, UK, in 2002 raised public awareness of the fragility of such discoveries where they interrupt municipal building programmes. Within a tight schedule the ship remains were recovered and now await further work. The origins of the ship are unknown since the dendrochronology is as yet unmatched, though the structure indicates Scandinavian and possibly Basque influences. Artefacts indicate trading with Iberia during the first half of the 15th century. Much of the ship had been salvaged at the end of her useful life about 1467. © 2004 The Nautical Archaeology Society [source] The Political Economy of AIDS Treatment: Intellectual Property and the Transformation of Generic SupplyINTERNATIONAL STUDIES QUARTERLY, Issue 3 2007Kenneth C. Shadlen This article examines the relationship between intellectual property (IP) and public health, with a focus on the extension of AIDS treatment in the developing world. While most of the literature on IP and health examines the conditions affecting poor countries' capacities to acquire essential medicines, I show the distinct,and more complicated,political economy of production and supply. IP regulations alter the structure of generic pharmaceutical sectors in the countries capable of supplying essential medicines, and changes in market structure affect actors' economic and political interests and capacities. These new constellations of interests and capacities have profound implications for the creation and maintenance of political coalitions in support of on-going drug supply. The result is that the global AIDS treatment campaign becomes marked by mismatches of interests and capacities: those actors capable of taking the economic, legal, and political steps necessary to increase the supply and availability of essential drugs have diminished interest in doing so, and those actors with an interest in expanding treatment may lack the capacities to address the problem of undersupply. By focusing centrally on actors' interests in and capacities for economic and political action, the article restores political economy to analysis of an issue-area that has been dominated by attention to international law. And by examining the fragility of the coalitions supporting the production and supply of generic drugs, the article points to the limits of transnational activist networks as enduring agents of change. [source] | ||||||||||||||||||||||||||||||||||||||||||||||