bluuebunny/crossref_metadata_2025_split huggingface dataset:
FAMILIAL HYPOPHOSPHATEMIC VITAMIN D RESISTANT RICKETS
Authors: STICKLER GUNNAR B. 
Published: January 2008

SummarySurvey of all available data of infants with familial hypophosphatemia vitamin D resistant rickets observed partially or throughout the first year of life indicates that hypophosphatemia begins in the neonatal period regardless of whether or not the mother had been treated with vitamin D in high doses. Growth failure in vitamin D resistant rickets cannot be prevented even when treatment with high doses of vitamin D is begun early.
HEREDITARY DEFECT OF THE SACRUM AND COCCYX WITH ANTERIOR SACRAL MENINGOCELE
Authors: COHN JØRGEN , BAY‐NIELSEN ERIK 
Published: January 2008

SummarySix familial cases of partial absence of the sacrum and the coccyx are described. Four had sacral ventral meningocele. There was a sex‐linked dominant inheritance. The diagnosis of the meningocele was established by myelography. The symptoms were constipation and incontinence of urine. Three patients also had recurrent infections of the urinary tract, and three had congenital anal stenosis.
RENAL TUBULAR ACIDOSIS AND HYPERGAMMAGLOBULINAEMIC PURPURA IN A 10 YEAR OLD GIRL WITH ROENTGENOGRAPHIC SIGNS SUGGESTING MEDULLARY SPONGE KIDNEY
Authors: POPA M. , STÄNESCU V. 
Published: January 2008

SummaryA 10‐year‐old girl presenting roentgenographic signs suggesting medullary sponge kidney, renal tubular acidosis and hypergammaglobu‐linaemic purpura is reported. The possible relationships between this conditions are discussed.
CHILDHOOD HYPOGLYCAEMIA, AS A SEQUEL OF ERYTHROBLASTOSIS FOETALIS
Authors: DANKS D. M. 
Published: January 2008

SummaryHypoglycaemia is described in two siblings at seven months and 25 months of age, respectively. Both had suffered very severe erythroblastosis foetalis and it is suggested that the hypoglycaemia represents a late sequel of this disease. Erythroblastosis should be added to the list of conditions associated with hypoglycaemia in the newborn and also in older children. During the investigations an influence of the sight of food upon blood glucose levels was noted.
DIAGNOSIS OF SEVERE CONGENITAL HEART DISEASE IN INFANTS
Authors: BJÖRK L. , MICHAËLSSON M. , WENDEL H. 
Published: January 2008

SummaryIn a series of 103 consecutive cases of severe cardiac disease in infants the diagnostic value of clinical examination, electrocardiogram and conventional roentgen examination was found to be very limited. Emergency use of cardiac catheterization and angiocardiography is necessary to achieve a correct diagnosis in these patients.
INTRAVASCULAR COAGULATION IN GENERALIZED HERPES SIMPLEX INFECTION OF THE NEWBORN
Authors: SHERSHOW LEE W. , EKERT HENRY , SWANSON VIRGINIA L. , WRIGHT HARRY T. , GILCHRIST GERALD S. 
Published: January 2008

SummaryA case of disseminated herpes simplex virus infection in a newborn associated with a fatal bleeding diathesis is reported. The presence of intravascular coagulation was suggested by thrombocytopenia, fibrinogenopenia, and reduced Factor V level. At necropsy localized fibrin and platelet deposition was found in liver and lung. It is postulated that intravascular coagulation resulted from local tissue necrosis and the subsequent release of thrombo‐plastin‐like substances into the general circulation. Heparin therapy did not alter the fatal outcome.
Subendothelial infiltration of neutrophil granulocytes and liberation of matrix-destabilizing enzymes in an experimental model of human neo-intima
Authors: Torzewski Michael , Dahm Manfred , Kirkpatrick Charles , Lackner Karl J. , Vahl Christian-Friedrich , Dorweiler Bernhard 
Published: January 2008

SummaryIt was the objective of this study to examine the role of human neutrophil granulocytes (PMN) in an in-vitro model of human neo-intima developed for the study of atherosclerosis. Human granulocytes were subjected to a co-culture model of human endothelial and smooth muscle cells. Subendothelial lipid accumulation was achieved by addition of native LDL to the culture medium. Tissue samples were analyzed by immunohistochemistry and scanning/transmission electron microscopy, and culture supernatants were examined for the presence of interleukin- 8 (IL-8), MCP-1, GRO-α, elastase and matrixmetalloproteinase-8 (MMP-8). Following addition of 2 mg/ml LDL, adherence, transmigration and infiltration depth of PMN was increased significantly when compared to controls. LDL challenging was paralleled by a time- and dose-dependent secretion of IL-8 from intimal smooth muscle cells. PMN infiltration was mediated by the IL-8-signalling pathway and accompanied by release of elastase and MMP-8 into the supernatant and induction of endothelial cell apoptosis. In conclusion, LDL-induced secretion of IL-8 by intimal smooth muscle cells provides a potential mechanism of PMN-recruitment into culprit lesions. The concomitant release of potent matrix-degrading enzymes and the induction of EC apoptosis may have implications for plaque destabilization and cardiovascular events.
Real-time measurement of non-lethal platelet thromboembolic responses in the anaesthetized mouse
Authors: Tymvios Charalambos , Jones Sarah , Moore Christopher , Pitchford Simon C. , Page Clive P. , Emerson Michael 
Published: January 2008

SummaryIdentifying and evaluating new therapeutic targets in platelets requires advanced animal models in which platelet responses can be measured directly and in situ.This is important because platelet function is strongly influenced by external factors such as those originating from the vascular endothelium. Our objectives were to record graded, non-lethal thromboembolic platelet responses to platelet agonists in situ in the mouse and to demonstrate an inhibitory effect of aspirin in our model. Radiolabelled platelets were infused into anaesthetized mice and responses to ADP, collagen and thrombin measured as changes in platelet associated counts in miniaturized detection probes placed over the thoracic region. All agonists induced dose-dependent changes in platelet counts due to accumulation of thrombi in the pulmonary vasculature. We confirmed a specific platelet effect by comparing platelet and erythrocyte responses and showing platelet aggregates in the lung vasculature histologically. Simultaneous injection of collagen and adrenaline induced increased and protracted synergistic platelet responses compared with individual injection of these agents and aspirin inhibited collagen-induced responses. We confirmed the clinical relevance of our model by showing that platelet thromboembolism in the mouse, like pulmonary embolism in humans, impaired cardiovascular performance. We present a refined method for measuring platelet agonist dose-responses and thromboembolism in real-time without inducing mortality in the mouse. Our technique will be useful in investigating the molecular determinants of physiological and pathophysiological platelet function in an in-vivo context and will enable investigations of both platelet and non-platelets mediators of thrombus formation.
The use of the VerifyNow P2Y12 point-of-care device to monitor platelet function across a range of P2Y12 inhibition levels following prasugrel and clopidogrel administration
Authors: Payne Christopher D. , Li Ying G. , Brandt John T. , Small David S. , Farid Nagy A. , Salazar Daniel E. , Winters Kenneth J. , Jakubowski Joseph A. 
Published: January 2008

SummaryVariability in response to antiplatelet agents has prompted the development of point-of-care (POC) technology. In this study, we compared theVerifyNowTM P2Y12 (VN-P2Y12) POC device with light transmission aggregometry (LTA) in subjects switched directly from clopidogrel to prasugrel. Healthy subjects on aspirin were administered a clopidogrel 600 mg loading dose (LD) followed by a 75 mg/d maintenance dose (MD) for 10 days. Subjects were then switched to a prasugrel 60 mg LD and then 10 mg/d MD for 10 days (n=16), or to a prasugrel 10 mg/d MD for 11 days (n=19). Platelet function was measured by LTA andVN-P2Y12 at baseline and after dosing. Clopidogrel 600 mg LD/75 mg MD treatment led to a reduction in P2Y12 reaction units (PRU) from baseline. A switch from clopidogrel MD to prasugrel 60 mg LD/10 mg MD produced an immediate decrease in PRU, while a switch to prasugrel 10 mg MD resulted in a more gradual decline. Consistent with the reduction in PRU, device-reported percent inhibition increased during both clopidogrel and prasugrel regimens. Inhibition of platelet aggregation as measured by LTA showed a very similar pattern to that found with VN-P2Y12 measurement, irrespective of treatment regimens. The dynamic range of VN-P2Y12 appeared to be narrower than that of LTA. With two different thienopyridines, the VN-P2Y12 device, within a somewhat more limited range, reflected the overall magnitude of change in aggregation response determined by LTA. The determination of the clinical utility of such POC devices will require their use in clinical outcome studies.
Role of proteolysis in development of murine adipose tissue
Authors: Christiaens Valerie , Scroyen Ilse , Lijnen H. Roger 
Published: January 2008

SummaryObesity is a common disorder, and related diseases such as diabetes, atherosclerosis, hypertension, cardiovascular disease and cancer are a major cause of mortality and morbidity inWesterntype societies. Development of obesity is associated with extensive modifications in adipose tissue involving adipogenesis, angiogenesis and extracellular matrix proteolysis. The fibrinolytic (plasminogen/plasmin) and matrix metalloproteinase (MMP) systems cooperate in these processes. A nutritionally induced obesity model in transgenic mice has been used extensively to study the role of the fibrinolytic and MMP systems in the development of obesity. These studies support a role of both systems in adipogenesis and obesity, and suggest that modulation of proteolytic activity may affect development of adipose tissue.
Male-specific cardiac pathologies in mice lacking either the A or B subunit of factor XIII
Authors: Souri Masayoshi , Koseki-Kuno Shiori , Takeda Naoki , Yamakawa Mitsunori , Takeishi Yasuchika , Degen Jay L. , Ichinose Akitada 
Published: January 2008

SummaryFactor XIII (FXIII) is a proenzyme of plasma transglutaminase consisting of enzymatic A subunits (FXIII-A) and non-catalytic B subunits (FXIII-B), and acts in haemostasis and wound healing. We generated mice lacking either FXIII-A or FXIII-B to investigate the physiological functions of FXIII in vivo. A longitudinal study was carried out using the gene-targeted mice to explore the possible effects of FXIII deficiency on aging. Survival rates of FXIII-A-/- males decreased to approximately 50% at 10 months after birth, although most FXIII-A-/- females and both genders of wild-type mice survived. Four FXIII-A-/- males died of severe intra-thoracic haemorrhage, and a large haematoma was found in their hearts. Haemorrhage, haemosiderin deposition and/or fibrosis were observed in the hearts of other dead FXIII-A-/- males. Fibrosis together with haemosiderin deposition was also found in the hearts of FXIII-A-/- males sacrificed. The in-vivo cardiac function was normal in FXIII-A-/- mice when compared with wild-type mice despite the presence of significant cardiac fibrosis. Although survival rates for both genders of the FXIII-B-/- and wild-type mice did not differ, mild fibrosis together with haemosiderin deposits were only found in the hearts of the sacrificed FXIII-B-/- males. Carditis and fibrosis in FXIII-deficient mice might be caused by a faulty or delayed reparative process that was initiated by abnormal haemorrhagic events within heart tissue. It is important therefore to examine possible cardiac involvement in human patients with congenital FXIII deficiency.
Clearance of von Willebrand factor
Authors: Christophe Olivier D. , Oortwijn Beatrijs D. , Lenting Peter J. , Denis Cécile V. 
Published: January 2008

SummaryThe life cycle of von Willebrand factor (VWF) comprises a number of distinct steps, ranging from the controlled expression of theVWF gene in endothelial cells and megakaryocytes to the removal of VWF from the circulation. The various aspects of VWF clearance have been the objects of intense research in the last few years, stimulated by observations thatVWF clearance is a relatively common component of the pathogenesis of type 1 von Willebrand disease (VWD). Moreover, improving the survival of VWF is now considered as a viable therapeutic strategy to prolong the half-life of factor VIII in order to optimise treatment of haemophilia A. The present review aims to provide an overview of recent findings with regard to the molecular basis of VWF clearance. A number of parameters have been identified that influence VWF clearance, including its glycosylation profile and a number of VWF missense mutations. In addition, in-vivo studies have been used to identify cells that contribute to the catabolism of VWF, providing a starting point for the identification of receptors that mediate the cellular uptake ofVWF.Finally, we discuss recent data describing chemically modification of VWF as an approach to prolong the half-life of the VWF/FVIII complex.
Factor VII-activating protease (FSAP): Vascular functions and role in atherosclerosis
Authors: Parahuleva Mariana , Muhl Lars , Kemkes-Matthes Bettina , Sedding Daniel , Preissner Klaus T. , Kanse Sandip M. 
Published: January 2008

SummaryFSAP is a plasma serine protease for which a potential role in the regulation of coagulation and fibrinolysis is postulated, based on its property to activate factorVII (FVII) as well as pro-urokinase (uPA). In clinical studies, the G534E single nucleotide polymorphism (Marburg I) of FSAP has been linked to late complications of atherothrombosis and is associated with a low proteolytic activity, particularly, towards pro-uPA. This has stimulated much interest in a search for additional functions of FSAP in the cardiovascular system. FSAP is a potent inhibitor of vascular smooth muscle cell proliferation and migration in vitro and local application of FSAP (but not Marburg I variant) in animal models reduces neointima formation. This is due to a reduced proteolytic activity of the variant isoform towards platelet derived growth factor-BB, a key mediator of neointima development. Moreover, appreciable quantities of FSAP are localized to unstable atherosclerotic plaques and may contribute to plaque instability. These data indicate that the cellular regulatory effects of FSAP may be more important than its influence on haemostasis. In this review the contribution of FSAP to vascular fibroproliferative inflammatory diseases in the context of pericellular proteolysis of the extracellular matrix, growth factor activity and haemostasis will be highlighted.
Meeting George Bush versus Meeting Cinderella: The Neural Response When Telling Apart What is Real from What is Fictional in the Context of Our Reality
Authors: Abraham Anna , von Cramon D. Yves , Schubotz Ricarda I. 
Published: January 2008

AbstractA considerable part of our lives is spent engaging in the entertaining worlds of fiction that are accessible through media such as books and television. Little is known, however, about how we are able to readily understand that fictional events are distinct from those occurring within our real world. The present functional imaging study explored the brain correlates underlying such abilities by having participants make judgments about the possibility of different scenarios involving either real or fictional characters being true, given the reality of our world. The processing of real and fictional scenarios activated a common set of regions including medial-temporal lobe structures. When the scenarios involved real people, brain regions associated with episodic memory retrieval and self-referential thinking, the anterior prefrontal cortex and the precuneus/posterior cingulate, were more active. In contrast, areas along the left lateral inferior frontal gyrus, associated with semantic memory retrieval, were implicated for scenarios with fictional characters. This implies that there is a fine distinction in the manner in which conceptual information concerning real persons in contrast to fictional characters is represented. In general terms, the findings suggest that fiction relative to reality tends to be represented in more factual terms, whereas our representations of reality relative to fiction are colored by personal subjectivity. What modulates our understanding of the relative difference between reality and fiction seems to be whether such character-type information is coded in self-relevant terms or not.
Item-specific Training Reduces Prefrontal Cortical Involvement in Perceptual Awareness
Authors: Eriksson Johan , Larsson Anne , Nyberg Lars 
Published: January 2008

Abstract
Previous studies on the neural correlates of perceptual awareness implicate sensory-specific regions and higher cortical regions such as the prefrontal cortex (PFC) in this process. The specific role of PFC regions is, however, unknown. PFC activity could be bottom-up driven, integrating signals from sensory regions. Alternatively, PFC regions could serve more active top-down processes that help to define the content of consciousness. To compare these alternative views of PFC function, we used functional magnetic resonance imaging and measured brain activity specifically related to conscious perception of items that varied in ease of identification (by being presented 0, 12, or 60 times previously). A bottom-up account predicts that PFC activity would be largely insensitive to stimulus difficulty, whereas a top-down account predicts reduced PFC activity as identification becomes easier. The results supported the latter prediction by showing reduced activity for previously presented compared to novel items in the PFC and several other regions. This was further confirmed by a functional connectivity analysis showing that the interaction between frontal and visual sensory regions declined as a function of ease of identification. Given the attribution of top-down processing to PFC regions in combination with the marked decline in PFC activity for easy items, these findings challenge the prevailing notion that the PFC is necessary for consciousness.
An Agent Harms a Victim: A Functional Magnetic Resonance Imaging Study on Specific Moral Emotions
Authors: Kédia Gayannée , Berthoz Sylvie , Wessa Michele , Hilton Denis , Martinot Jean-Luc 
Published: January 2008

Abstract
The statement: “An agent harms a victim,” depicts a situation that triggers moral emotions. Depending on whether the agent and the victim are the self or someone else, it can lead to four different moral emotions: self-anger (“I harm myself”), guilt (“I harm someone”), other-anger (“someone harms me”), and compassion (“someone harms someone”). In order to investigate the neural correlates of these emotions, we examined brain activation patterns elicited by variations in the agent (self vs. other) and the victim (self vs. other) of a harmful action. Twenty-nine healthy participants underwent functional magnetic resonance imaging while imagining being in situations in which they or someone else harmed themselves or someone else. Results indicated that the three emotional conditions associated with the involvement of other, either as agent or victim (guilt, other-anger, and compassion conditions), all activated structures that have been previously associated with the Theory of Mind (ToM, the attribution of mental states to others), namely, the dorsal medial prefrontal cortex, the precuneus, and the bilateral temporo-parietal junction. Moreover, the two conditions in which both the self and other were concerned by the harmful action (guilt and other-anger conditions) recruited emotional structures (i.e., the bilateral amygdala, anterior cingulate, and basal ganglia). These results suggest that specific moral emotions induce different neural activity depending on the extent to which they involve the self and other.
Working Memory Load for Faces Modulates P300, N170, and N250r
Authors: Morgan Helen M. , Klein Christoph , Boehm Stephan G. , Shapiro Kimron L. , Linden David E. J. 
Published: January 2008

Abstract
We used event-related potential (ERP) methodology to examine neural activity associated with visual working memory (WM) for faces. There were two main goals. First, to extend previous findings of P300 load modulation to WM for faces. Second, to examine whether N170 and N250r are also influenced by WM load. Between one and four unfamiliar faces were simultaneously presented for memory encoding. After a 1-sec delay, a target face appeared, and participants had to judge whether this face was part of the previous face array. P300 amplitude decreased as WM load increased, and this P300 suppression was observed at both encoding and retrieval. WM load was also found to modulate other ERPs. The amplitude of the N170 elicited by the target face decreased with load, and this N170 decrease leveled off at load 2, reflecting the behavioral WM capacity of around two faces. In addition, the N250r, observed as an ERP difference for target faces that were present in the encoding array relative to target faces that were absent, was also reduced for higher WM loads. These findings extend previous work by showing that P300 modulation by WM load also occurs for faces. Furthermore, we show, for the first time, that WM load affects the N250r and the early visual N170 component. This suggests that higher visual areas play an important role in WM for faces.
Involvement of the Supplementary Eye Field in Oculomotor Predictive Behavior
Authors: Nyffeler Thomas , Rivaud-Pechoux Sophie , Wattiez Nicolas , Gaymard Bertrand 
Published: January 2008

Abstract
The presentation of saccadic and smooth pursuit eye movements as two separate systems has recently been reconsidered: The two subsystems share a number of anatomical structures, and recent data suggest that this sharing also extends to physiological processes. The aim of our study was first to test whether these two subsystems share a common predictive process. We designed a new predictive smooth pursuit paradigm that requires the triggering of unpredictable saccades, performed either during low (ongoing pursuit) or high (pursuit direction reversal) predictive behavior. Saccade latency was used as a probe to reveal a possible sharing of prediction between the two subsystems. The main finding was that saccade latencies were markedly decreased when triggered around pursuit direction reversal and performed in the direction of the predicted pursuit. The aim of the second part of this study was to determine the neural substrate of this common predictive process. According to previous studies, the supplementary eye field (SEF) would be involved in the control of predictive pursuit. The same subjects therefore performed the same tasks, and transcranial magnetic stimulation (TMS) was applied over this area: Decreased saccade latencies were no longer observed, whereas it continued to be observed when applied over the occipital cortex. These results are consistent with (1) The existence of a common predictive process shared by both oculomotor subsystems; (2) The view of the SEF not as a primary oculomotor area but as a higher order structure able to elaborate complex processes, such as prediction, independently of the oculomotor output.