bluuebunny/crossref_metadata_2025_split huggingface dataset:
Exploring possible mechanisms of change in family‐based treatment for adolescent bulimia nervosa
Authors: Lock James , Le Grange Daniel , Crosby Ross 
Published: July 2008

Objective: Mediators of treatment for family‐based treatment are explored for outcomes among adolescents with bulimia nervosa (BN).Method: Using data from a recently completed randomized clinical trial (RCT) comparing family‐based treatment (FBT) to supportive psychotherapy (SPT) we examined possible mediators of treatment outcome.Results: Results suggest that FBT‐BN was more effective than SPT in producing remission by virtue of achieving greater reductions in eating disorder psychopathology by mid‐treatment. Specifically, reductions in cognitions related to BN as assessed by the EDE appeared to be the differentiating mechanism.Conclusion: It is reasonable to hypothesize that change in the EDE subscales mediates outcome for FBT‐BN. Future studies with sufficient power to examine this possibility as an a priori hypothesis would help to better understand how FBT‐BN works and may also help to guide future treatment refinement and development.
Yield assessment of integument‐led seed growth following targeted repair of auxin response factor 2
Authors: Hughes Rhiannon , Spielman Melissa , Schruff Marie C. , Larson Tony R. , Graham Ian A. , Scott Rod J. 
Published: July 2008

SummaryIt is becoming increasingly vital to improve the yield of seed crops to feed an expanding population and, more recently, for biofuel production. One strategy to increase the yield is to increase the seed size, provided that there is not a concomitant decrease in seed number. In a previous study, we described a mutant in the AUXIN RESPONSE FACTOR 2 (ARF2) gene which produced extra cells in the seed coat and, subsequently, enlarged seeds. However, arf2 mutant plants also show severely reduced self‐fertility caused, in part, by over‐elongated sepals that prevent flower opening. As a low seed set increases individual seed size, a meaningful comparison of the yield in arf2 and wild‐type plants could not be conducted. In this study, we show that targeted expression of wild‐type ARF2 in the sepals and petals of arf2‐9 mutant flowers restores flower opening and dramatically increases seed set. The restored plants retain both enlarged integuments and increased seed size, reinforcing previous evidence that arf2 mutations increase seed weight through their effect on integuments and not only via reduced fertility. We also show that the measurement of the harvest index in Arabidopsis is useful in assessing the impact of introduced traits on the yield.
Cerebrospinal fluid tau and amyloid β42 protein in patients with myotonic dystrophy type 1
Authors: Winblad S. , Månsson J. E. , Blennow K. , Jensen C. , Samuelsson L. , Lindberg C. 
Published: July 2008

Background:  Myotonic dystrophy type 1 (DM1) is associated with brain morphology changes including neurofibrillary degeneration.Methods:  We have examined cerebrospinal fluid (CSF) markers indicative of neuronal degeneration and amyloidogenesis; total tau (T‐tau), phosphorylated tau (P‐tau) and β amyloid1–42 (Aβ42), in 32 patients with DM1.Results and conclusions:  Associations between CSF markers and CTG repeat expansion size, brain MRI findings, and neuropsychological test results were analysed. As compared with matched controls Aβ42 was significantly decreased (P = 0.001), whilst levels of T‐tau were increased (P < 0.001). No difference was found between measures considering P‐tau levels. At present the clinical implications of these findings is unclear, because of an overlap between CSF values of DM1 patients and healthy controls, but also regarding modest associations between CSF markers and other measures. However notably, the Tau pathology, as seen in DM1, differs from Alzheimers disease, considering the lack of increased levels of P‐tau.
Size of PFO and amount of microembolic signals in patients with ischaemic stroke or TIA
Authors: Telman G. , Yalonetsky S. , Kouperberg E. , Sprecher E. , Lorber A. , Yarnitsky D. 
Published: July 2008

Background and purpose:  The inter‐relation between the size of patent foramen ovale (PFO) by transesophageal echocardiography (TEE) and the amount of microembolic signals (MES) on transcranial doppler (TCD) is still not determined.Methods:  The study group comprised of 104 patients with first‐ever ischaemic stroke or transient ischemic attack (TIA). Three groups were formed according to the amount of MES on TCD: a small amount of MES (0–10 MES); a moderate amount of MES (countable MES higher than 10); and multiple MES.Results:  According to TEE, there were 52 patients (50%) with a small PFO, 37 patients (35.6%) with a moderate PFO, and 15 patients (14.4%) with a large PFO. There were 48 patients (46.1%) with a small amount of MES, 34 patients (32.7%) with a moderate amount of MES, and 22 patients (21.1%) with multiple MES on TCD. A strong relationship between the size of the PFO on TEE and the amount of MES on contrast transcranial Doppler was found (P < 0.0001), such that the larger the PFO on TEE, the greater the amount of MES on TCD.Conclusions:  There is a high correlation between the size of the PFO on TEE and the amount of MES on TCD in stroke and TIA patients.
Human hair pigmentation – biological aspects
Authors: Tobin D. J. 
Published: July 2008

SynopsisSkin and hair colour contribute significantly to our overall visual appearance and to social/sexual communication. Despite their shared origins in the embryologic neural crest, the hair follicle and epidermal pigmentary units occupy distinct, although open, cutaneous compartments. They can be distinguished principally on the basis of the former’s stringent coupling to the hair growth cycle compared with the latter’s continuous melanogenesis. The biosynthesis of melanin and its subsequent transfer from melanocyte to hair bulb keratinocytes depend on the availability of melanin precursors and on a raft of signal transduction pathways that are both highly complex and commonly redundant. These signalling pathways can be both dependent and independent of receptors, act through auto‐, para‐ or intracrine mechanisms and can be modified by hormonal signals. Despite many shared features, follicular melanocytes appear to be more sensitive than epidermal melanocytes to ageing influences. This can be seen most dramatically in hair greying/canities and this is likely to reflect significant differences in the epidermal and follicular microenvironments. The hair follicle pigmentary unit may also serve as an important environmental sensor, whereby hair pigment contributes to the rapid excretion of heavy metals, chemicals and toxins from the body by their selective binding to melanin; rendering the hair fibre a useful barometer of exposures. The recent availability of advanced cell culture methodologies for isolated hair follicle melanocytes and for intact anagen hair follicle organ culture should provide the research tools necessary to elucidate the regulatory mechanisms of hair follicle pigmentation. In the longer term, it may be feasible to develop hair colour modifiers of a biological nature to accompany those based on chemicals.
Comparative in vitro study of interactions between particles and respiratory surface macrophages, erythrocytes, and epithelial cells of the chicken and the rat
Authors: Kiama S. G. , Adekunle J. S. , Maina J. N. 
Published: July 2008

AbstractIn mammals, surface macrophages (SMs) play a foremost role in protecting the respiratory system by engulfing and destroying inhaled pathogens and harmful particulates. However, in birds, the direct defense role(s) that SMs perform remains ambiguous. Paucity and even lack of SMs have been reported in the avian respiratory system. It has been speculated that the pulmonary defenses in birds are inadequate and that birds are exceptionally susceptible to pulmonary diseases. In an endeavour to resolve the existing controversy, the phagocytic capacities of the respiratory SMs of the domestic fowl and the rat were compared under similar experimental conditions by exposure to polystyrene particles. In cells of equivalent diameters (8.5 µm in the chicken and 9.0 µm in the rat) and hence volumes, with the volume density of the engulfed polystyrene particles, i.e. the volume of the particles per unit volume of the cell (SM) of 23% in the chicken and 5% in the rat cells, the avian cells engulfed substantially more particles. Furthermore, the avian SMs phagocytized the particles more efficiently, i.e. at a faster rate. The chicken erythrocytes and the epithelial cells of the airways showed noteworthy phagocytic activity. In contrast to the rat cells that did not, 22% of the chicken erythrocytes phagocytized one to six particles. In birds, the phagocytic efficiencies of the SMs, erythrocytes, and epithelial cells may consolidate pulmonary defense. The assorted cellular defenses may explain how and why scarcity of SMs may not directly lead to a weak pulmonary defense. The perceived susceptibility of birds to respiratory diseases may stem from the human interventions that have included extreme genetic manipulation and intensive management for maximum productivity. The stress involved and the structural–functional disequilibria that have occurred from a ‘directed evolutionary process’, rather than weak immunological and cellular immunity, may explain the alleged vulnerability of the avian gas exchanger to diseases.
Role of hippocampal sodium channel Nav1.6 in kindling epileptogenesis
Authors: Blumenfeld Hal , Lampert Angelika , Klein Joshua P. , Mission John , Chen Michael C. , Rivera Maritza , Dib‐Hajj Sulayman , Brennan Avis R. , Hains Bryan C. , Waxman Stephen G. 
Published: July 2008

SummaryPurpose:  Central nervous system plasticity is essential for normal function, but can also reinforce abnormal network behavior, leading to epilepsy and other disorders. The role of altered ion channel expression in abnormal plasticity has not been thoroughly investigated. Nav1.6 is the most abundantly expressed sodium channel in the nervous system. Because of its distribution in the cell body and axon initial segment, Nav1.6 is crucial for action potential generation. The goal of the present study was to investigate the possible role of changes in Nav1.6 expression in abnormal, activity‐dependent plasticity of hippocampal circuits.Methods:  We studied kindling, a form of abnormal activity‐dependent facilitation. We investigated: (1) sodium channel protein expression by immunocytochemistry and sodium channel messenger RNA (mRNA) by in situ hybridization, (2) sodium current by patch clamp recordings, and (3) rate of kindling by analysis of seizure behavior. The initiation, development, and expression of kindling in wild‐type mice were compared to Nav1.6 +/−medtg mice, which have reduced expression of Nav1.6.Results:  We found that kindling was associated with increased expression of Nav1.6 protein and mRNA, which occurred selectively in hippocampal CA3 neurons. Hippocampal CA3 neurons also showed increased persistent sodium current in kindled animals compared to sham‐kindled controls. Conversely, Nav1.6 +/−medtg mice resisted the initiation and development of kindling.Discussion:  These findings suggest an important mechanism for enhanced excitability, in which Nav1.6 may participate in a self‐reinforcing cycle of activity‐dependent facilitation in the hippocampus. This mechanism could contribute to both normal hippocampal function and to epilepsy and other common nervous system disorders.
Nodular heterotopia: A neuropathological study of 24 patients undergoing surgery for drug‐resistant epilepsy
Authors: Meroni Alessandra , Galli Carlo , Bramerio Manuela , Tassi Laura , Colombo Nadia , Cossu Massimo , Lo Russo Giorgio , Garbelli Rita , Spreafico Roberto 
Published: July 2008

SummaryPurpose:  Despite the availability of detailed electroclinical and imaging data, only a few neuropathological studies of nodular heterotopia have been published. The aim of this study was to describe the neuropathological features of various types of nodular heterotopia obtained from patients undergoing surgery for intractable epilepsy.Methods: Specimens of heterotopic nodules taken from 24 patients were neuropathologically investigated using routine and immunocytochemical procedures, and the data were compared with magnetic resonance imaging (MRI), electroclinical findings, and surgical outcomes.Results: The neuropathological data distinguished two groups. Group 1 (14 patients, 78% in Engel class 1) had similar characteristics regardless of the size, number, or location of the nodules, with both projecting and local circuit neurons in the nodules intermingled with glial cells. Thirteen patients had focal cortical dysplasia. The nodules were identified by MRI in all cases. In group 2 (10 patients, 90% in Engel class 1), all of the nodules were within the temporal lobe and associated with hippocampal sclerosis or gangliogliomas. They were very small (undetected by MRI) and mainly formed by projecting neurons with no evidence of glial cells. All of the patients had cortical dysplasia.Discussion: The distinctive neuropathological features of the nodules in the two groups suggest different etiopathogenetic mechanisms. In group 2, the presence of nodular formations in association with cortical dysplasia and either hippocampal sclerosis or ganglioglioma raises a question concerning so‐called dual pathology in the temporal lobe.
Focal decreases of cortical GABAA receptor binding remote from the primary seizure focus: What do they indicate?
Authors: Juhász Csaba , Asano Eishi , Shah Aashit , Chugani Diane C. , Batista Carlos E. A. , Muzik Otto , Sood Sandeep , Chugani Harry T. 
Published: July 2008

SummaryPurpose:  To determine the electroclinical significance and histopathological correlates of cortical γ‐aminobutyric acidA(GABAA) receptor abnormalities detected in and remote from human neocortical epileptic foci.Methods:  Cortical areas with decreased11C‐flumazenil (FMZ) binding were objectively identified on positron emission tomography (PET) images and correlated to intracranial electroencephalography (EEG) findings, clinical seizure variables, histology findings, and surgical outcome in 20 patients (mean age, 9.9 years) with intractable partial epilepsy of neocortical origin and nonlocalizing magnetic resonance imaging (MRI).Results:  Focal decrease of cortical FMZ binding was detected in the lobe of seizure onset in 17 (85%) patients. Eleven patients (55%) had 17 remote cortical areas with decreased FMZ binding outside the lobe of seizure onset. Thirteen of those 16 (81%) of the 17 remote cortical regions that were covered by subdural EEG were around cortex showing rapid seizure spread on intracranial EEG. Remote FMZ PET abnormalities were associated with high seizure frequency and, when resected, showed gliosis in all six cases where material was available. Higher number of unresected cortical regions with decreased FMZ binding was associated with poorer surgical outcome.Conclusions:  Focal decreases of cortical GABAA receptor binding on PET may include cortical regions remote from the primary focus, particularly in patients with high seizure frequency, and these regions are commonly involved in rapid seizure propagation. Although these regions may not always need to be resected to achieve seizure freedom, a careful evaluation of cortex with decreased GABAA receptor binding prior to resection using intracranial EEG may facilitate optimal surgical outcome in patients with intractable neocortical epilepsy.
Eicosapentaenoic and docosahexaenoic acids are not anticonvulsant or neuroprotective in acute mouse seizure models
Authors: Willis Sarah , Samala Ramakrishna , Rosenberger Thad A. , Borges Karin 
Published: July 2008

SummaryDocosahexaenoic acid (DHA), eicosapentaenoic acid (EPA), or vegetable oil (control) were added to standard rodent chow (6 g/kg) and fed to mice ad lib for 4 weeks to determine if polyunsaturated fatty acids (PUFA) are anticonvulsant or neuroprotective in mice. The seizure susceptibility of these mice was compared using the fluorothyl, pentylenetetrazole (PTZ), 6 Hz, and kainate models. We found that PUFA feeding significantly altered the fatty acid profile in both plasma and brain, but did not change seizure thresholds in the fluorothyl, PTZ, or 6 Hz models nor did it significantly alter seizure behavior or hippocampal damage following kainate injection. In conclusion, DHA or EPA feeding did not show anticonvulsant or neuroprotective activity in four acute seizure models. Chronic seizure models remain to be examined.
Usefulness of Nonlinear Analysis of ECG Signals for Prediction of Inducibility of Sustained Ventricular Tachycardia by Programmed Ventricular Stimulation in Patients with Complex Spontaneous Ventricular Arrhythmias
Authors: Durin Ornella , Pedrinazzi Claudio , Donato Giorgio , Pizzi Rita , Inama Giuseppe 
Published: July 2008

Introduction: The aim of our study was to assess the effectiveness of the nonlinear analysis (NLA) of ECG in predicting the results of invasive electrophysiologic study (EPS) in patients with ventricular arrhythmias. Methods: We evaluated 25 patients with history of cardiac arrest, syncope, sustained, or nonsustained ventricular tachycardia (VT). All patients underwent electrophysiologic study (EPS) and nonlinear analysis (NLA) of ECG. The study group was compared with a control group of 25 healthy subjects, in order to define the normal range of NLA. ECG was processed in order to obtain numerical values, which were analyzed by nonlinear mathematical functions. Patients were classified through the application of a clustering procedure to the whole set of functions, and the correlation between the results of nonlinear analysis of ECG and EPS was tested. Results: NLA assigned all patients with negative EPS to the same class of healthy subjects, whereas the patients in whom VT was inducible had been correctly and clearly isolated into a separate cluster. In our study, the result of NLA with application of the clustering technique was significantly correlated to that of EPS (P < 0.001), and was able to predict the result of EPS, with a negative predictive value of 100% and a positive predictive value of 100%. Conclusions: NLA can predict the results of EPS with good negative and positive predictive value. However, further studies are needed in order to verify the usefulness of this noninvasive tool for sudden death risk stratification in patients with ventricular arrhythmias.
Beat‐to‐Beat QT Interval Variability Is Primarily Affected by the Autonomic Nervous System
Authors: Mine Takanao , Shimizu Hiroki , Hiromoto Kenji , Furukawa Yoshio , Kanemori Tetsuzou , Nakamura Hiroaki , Masuyama Tohru , Ohyanagi Mitsumasa 
Published: July 2008

Background: Beat‐to‐beat QT interval variability is associated with life‐threatening arrhythmias and sudden death, however, its precious mechanism and the autonomic modulation on it remains unclear. The purpose of this study was to determine the effect of drugs that modulate the autonomic nervous system on beat‐to‐beat QT interval. Method: RR and QT intervals were determined for 512 consecutive beats during fixed atrial pacing with and without propranolol and automatic blockade (propranolol plus atropine) in 11 patients without structural heart disease. Studied parameters included: RR, QTpeak (QRS onset to the peak of T wave), QTend (QRS onset to the end of T wave) interval, standard deviation (SD) of the RR, QTpeak, and QTend (RR‐SD, QTpeak‐SD, and QTend‐SD), coefficients of variation (RR‐ CV, QTpeak‐CV, and QTend‐CV) from time domain analysis, total power (TP; RR‐TP, QTpeak‐TP, and QTend‐TP), and power spectral density of the low‐frequency band (LF; RR‐LF, QTpeak‐LF, and QTend‐LF) and the high‐frequency band (HF; RR‐HF, QTpeak‐HF and QTend‐HF). Results: Administration of propranolol and infusion of atropine resulted in the reduction of SD, CV, TP, and HF of the QTend interval when compared to controlled atrial pacing (3.7 ± 0.6 and 3.5 ± 0.5 vs 4.8 ± 1.4 ms, 0.9 ± 0.1 and 0.9 ± 0.1 vs 1.2 ± 0.3%, 7.0 ± 2.2 and 7.0 ± 2.2 vs 13.4 ± 8.1 ms2, 4.2 ± 1.4 and 4.2 ± 1.2 vs 8.4 ± 4.9 ms2, respectively). Administration of propranolol and atropine did not affect RR interval or QTpeak interval indices during controlled atrial pacing. Conclusions: Beat‐to‐beat QT interval variability is affected by drugs that modulate the autonomic nervous system.
Phenotypic Variability in Caucasian and Japanese Patients with Matched LQT1 Mutations
Authors: Liu Judy F. , Goldenberg Ilan , Moss Arthur J. , Shimizu Wataru , Wilde Arthur A. , Hofman Nynke , McNitt Scott , Zareba Wojciech , Miyamato Yoshihiro , Robinson Jennifer L. , Andrews Mark L. 
Published: July 2008

Background: Ethnic differences may affect the phenotypic expression of genetic disorders. However, data regarding the effect of ethnicity on outcome in patients with genetic cardiac disorders are limited. We compared the clinical course of Caucasian and Japanese long QT type‐1 (LQT1) patients who were matched for mutations in the KCNQ1 gene. Methods: The study population comprised 62 Caucasian and 38 Japanese LQT1 patients from the International LQTS Registry who were identified as having six identical KCNQ1 mutations. The biophysical function of the mutations was categorized into dominant‐negative (>50%) or haploinsufficiency (≤50%) reduction in cardiac repolarizing IKs potassium channel current. The primary end point of the study was the occurrence of a first cardiac event from birth through age 40 years. Results: Japanese patients had a significantly higher cumulative rate of cardiac events (67%) than Caucasian patients (39%; P = 0.01). The respective frequencies of dominant negative mutations in the two ethnic groups were 63% and 28% (P < 0.001). In multivariate analysis, Japanese patients had an 81% increase in the risk of cardiac events (P = 0.06) as compared with Caucasians. However, when the biophysical function of the mutations was included in the multivariate model, the risk associated with Japanese ethnicity was no longer evident (HR = 1.05; P = 0.89). Harboring a dominant negative mutation was shown to be the most powerful and significant predictor of outcome (HR = 3.78; P < 0.001). Conclusions: Our data indicate that ethnic differences in the clinical expression of LQTS can be attributed to the differences in frequencies of the specific mutations within the two populations.