bluuebunny/crossref_metadata_2025_split huggingface dataset:
Celiac disease and gluten-free diet
Authors: Hybenová Eva , Štofirová Julia , Mikulajová Anna 
Published: May 2013

Celiac disease is an autoimmunity inflammatory disorder of the small intestine caused by the ingestion of gluten in genetically predisposed individuals. The prevalence of the disorder is around 1 % of the Western population and is still increasing. The symptoms of celiac disease include chronic abdominal pain, diarrhoea, and growth retardation in children, and chronic fatigue and headache, bowel complaints, reduced fertility, dermatitis herpetiformis, osteoporosis, nerve and brain disorders, increasing risk of intestinal cancer. The clinical diagnosis of the disease is based on the serological tests and bowel biopsy. The treatment is a long-life gluten-free diet. It is necessary exclude from the diet wheat, rye, barley and probably oats and buckwheat and their products. The novel approaches for celiac disease are focused on the genetic manipulation of nontoxic gluten proteins, enzyme therapy, immune modulation, and induction of oral tolerance to gluten.
Insulin Independence after Islet Transplantation through an Indwelling Catheter in the Right Gastric Vein
Authors: Wu Zhixian , Luo Fang , Guo Junqi , Cai Jinquan , Yang Shunliang , Wu Weizhen , Jiang Yanyan , Liao Lianming , Wu Zhifen , Tan Jianming 
Published: May 2013

Background Current islet transplantation approaches have various defects. This study was to investigate the feasibility and safety of an indwelling catheter in the right gastric vein for intra-hepatic islet transplantation. Methods Twenty patients had islet transplants through the indwelling catheter in the right gastric vein. The catheters were placed into the portal vein trunk using open surgery. While monitored with Doppler ultrasound, the islet suspensions were infused after the catheter location was confirmed in the trunk of the portal vein. The catheter was kept indwelling and secured to the skin for optional subsequent infusions and flushed with heparinized saline once per day to avoid peri-catheter thrombosis. After one month, the catheter was removed. Adverse effects and transplant efficacy parameters were observed. Results Insulin independence was finally achieved in 17 patients; 11 patients received a second infusion. The mean surgical duration was 55 ∓ 7 minutes and the hemorrhage volume was approximately 40 ∓ 11 mL. No significant change in portal pressure was observed (before infusion 2.9 ∓ 1.5 cm H2O, after infusion 2.6 ∓ 1.7 cm H2O, p>0.05). However, peak systolic velocity (PRV) of the hepatic artery after infusion was markedly higher than that before infusion (35.1 ∓ 10.7 cm/s vs. 68.5 ∓ 46.2 cm/s, p<0.01). Neither infection nor severe hemorrhage was found after surgery. Conclusions It is feasible, convenient, and safe to use an indwelling catheter in the right gastric vein for islet transplantation.
Low Estimated Glomerular Filtration Rate and Chronic Kidney Failure following Liver Transplant: A Retrospective Cohort Study
Authors: Narciso Roberto C. , Ferraz Leonardo R. , Rodrigues Cassio J. O. , Monte Júlio C. M. , Mies Sérgio , dos Santos Oscar F. P. , Paes Ângela T. , Cendoroglo Miguel , Jaber Bertrand L. , Durão Marcelino S. , Batista Marcelo C. 
Published: May 2013

Background Patients undergoing orthotropic liver transplant (LTx) often present with chronic kidney disease (CKD). Identification of patients who will progress to end-stage renal disease (ESRD) might allow not only the implementation of kidney protective measures but also simultaneous kidney transplant. Study Design Retrospective cohort study in adults who underwent LTx at a single center. ESRD, death, and composite of ESRD or death were studied outcomes. Results 331 patients, who underwent LTx, were followed up for 2.6 ± 1.4 years; 31 (10%) developed ESRD, 6 (2%) underwent kidney transplant after LTx and 25 (8%) remained on chronic hemodialysis. Patients with pre-operative eGFR lesser than 60 ml/min per 1.73 m2 had a 4-fold increased risk of developing ESRD after adjustment for sex, diabetes mellitus, APACHE II score, use of nephrotoxic drugs, and severe liver graft failure (HR = 3.95, 95% CI 1.73, 9.01; p = 0.001). Other independent risk factors for ESRD were pre-operative diabetes mellitus and post-operative severe liver graft dysfunction. Conclusion These findings emphasize low eGFR prior to LTx as a predictor for ESRD or death. The consideration for kidney after liver transplant as a treatment modality should be taken into account for those who develop chronic kidney failure after LTx.
Non-invasive evaluation of electrical brain activity: effects of non-medicative treatment and subject’s state
Authors: Griškova-Bulanova Inga 
Published: May 2013

Introduction. The electroencephalogram (EEG) and evoked potentials (EPs) are widely used in psychiatry. Thus, it is important to standardize recording conditions according to instructions, arousal and attention levels of the subject. Also, it is important to evaluate the effect of nonpharmacologic treatment (electroconvulsive therapy (ECT) and transcranial magnetic stimulation (TMS)) on EEG and EPs. In this paper, an overview of researches, conducted in the above mentioned directions in Lithuania, is given.
Materials and methods. EEG was recorded using standard protocols; P300, P50 and auditory steady-state responses (ASSR) were elicited. 44 schizophrenic patients, 25 depressive patients and 136 healthy subjects were investigated. Effects of ECT treatment, 10 Hz TMS procedure, and varying attention and arousal levels on EEG/EPs were investigated.
Results. ECT had no negative impact on P300; alongside with clinical response, normalization of P300 parameters occurred. It was related to subject’s age and autonomic nervous system response during the treatment. A single 10 Hz TMS procedure produced a profound increase in the delta power of background EEG. In lower arousal level conditions, higher and more precise P50 potential, ASSR and background alpha power were observed. During distraction task performance, reduced gamma band response and ASSR, and increased background gamma power were observed.
Conclusions. During the treatment course of ECT it is recommended to evaluate P300 potential changes and to monitor subject’s indices of the autonomic nervous system function, as these are related to clinical efficiency. Passively elicited auditory responses should be registered during low arousal unfocused attention conditions when the most precise responses of the highest amplitude are registered.
Effect of harvest date on barley grain contamination with Fusarium spp. and deoxynivalenol in northeastern Ontario
Authors: Xue Allen G. , Rowsell John , Ho Keh Ming , Chen Yuanhong , Chi Dawn T. , Manceur Ameur , Zhang Shuzhen , Ren Changzhong 
Published: May 2013

The effect of harvest date on the incidence of seed-borne Fusarium spp. and deoxynivalenol (DON) concentration in barley (Hordeum vulgare L.) was studied using three cultivars at three locations in Ontario in both 2004 and 2005. The profile of seed-borne Fusarium spp. was dominated by F. equiseti (Corda) Sacc., F. sporotrichioides Sherb., and F. poae (Peck) Wollenw., isolated from 4.4%, 3.3%, and 1.6% of the kernels, representing 39.3%, 29.4%, and 14.2% of the Fusarium pathogen population, respectively. Fusarium graminearum Schwabe and F. avenaceum (Fr.) Sacc. were each recovered from <1% of the kernels and represented 8.3% and 6.6% of the pathogen population, respectively. Other species, including F. acuminatum Ellis & Everh., F. culmorum (W.G. Sm.) Sacc., and F. semitectum Berk. & Rav., collectively occurred only on 0.2% of all kernels and represented <2% of the population. The incidence level of all Fusarium spp. increased from 6.9 to 13.9% when harvest was delayed. Of the commonly recovered species, only F. avenaceum and F. sporotrichioides levels increased with the delayed harvest, while other species did not follow a clear pattern. DON concentration in the harvested grain ranged from 0.20 to 0.28 mg kg‑1 with the five harvest dates, and was not statistically different. Significant differences in the incidence of all Fusarium spp. and in DON concentration were observed among cultivars, locations, and between the 2 yr of the study. The highest DON concentration observed in this study was 0.5 mg kg‑1, which is below the Canadian tolerance level of 1.0 mg kg‑1.
Antioxidant response of the invasive herb Ambrosia artemisiifolia L. to different irradiance levels
Authors: Qin Zhong , Mao DanJuan , Quan GuoMing , Zhang Jia-En , Xie JunFang , DiTommaso Antonio 
Published: May 2013

The exotic invasive Ambrosia artemisiifolia L. and a native co-occurring species in southern China, Urena lobata L., were compared to investigate the possible protective role of leaf antioxidant systems in the acclimation of invasive plants to different irradiance levels. Antioxidant activities of superoxide dismutase (SOD), peroxidase (POD), and catalase (CAT) were examined under four irradiance regimes: 10% (dense shade), 30% (low irradiance), 55% (medium irradiance) and 100% (full irradiance). Free proline (Pro) content and the rate of lipid peroxidation in terms of malondialdehyde (MDA) content, glutathione reductase (GR), and tea polyphenols (TP) were also assessed under the different irradiance regimes. Antioxidant enzyme activity of SOD and CAT and the MDA, GR and TP contents for the two species increased with increasing irradiance levels. Invasive A. artemisiifolia was able to scavenge oxygen radicals more efficiently at higher irradiance levels by enhancing CAT activity and GR and TP contents although leaf SOD activity was not greatly enhanced. This exotic species also maintained normal physiological functions when subjected to low irradiance, which might be attributed to the increase in POD activity with decreasing irradiance levels. The higher efficiency of adaptive responses of antioxidant enzymes may protect plants from irradiance-induced stress and may contribute to the invasiveness of A. artemisiifolia in subtropical and tropical regions.
High Heritability of Hallux Valgus and Lesser Toe Deformities in Adult Men and Women
Authors: Hannan Marian T. , Menz Hylton B. , Jordan Joanne M. , Cupples L. Adrienne , Cheng Chia‐Ho , Hsu Yi‐Hsiang 
Published: May 2013

ObjectiveTo estimate the heritability of 3 common disorders affecting the forefoot, i.e., hallux valgus, lesser toe deformities, and plantar forefoot soft tissue atrophy, in white adult men and women.MethodsBetween 2002 and 2008, a trained examiner used a validated foot examination to document the presence of hallux valgus, lesser toe deformities, and plantar soft tissue atrophy in 2,446 adults from the Framingham Foot Study. Among these, 1,370 participants with an available pedigree structure were included. Heritability was estimated using pedigree structures by the Sequential Oligogenic Linkage Analysis Routines package. Results were adjusted for age, sex, and body mass index.ResultsThe mean age of the participants was 66 years (range 39–99 years) and 57% were women. The prevalence of hallux valgus, lesser toe deformities, and plantar soft tissue atrophy was 31%, 29.6%, and 28.4%, respectively. Significant heritability was found for hallux valgus (range 0.29–0.89, depending on age and sex) and lesser toe deformity (range 0.49–0.90, depending on age and sex). The heritability for lesser toe deformity in men and women ages >70 years was 0.65 (P = 9 × 10−7). Significant heritability was found for plantar soft tissue atrophy in men and women ages >70 years (H2 = 0.37, P = 3.8 × 10−3).ConclusionTo our knowledge, these are the first findings of heritability of foot disorders in humans, and they confirm the widely‐held view that hallux valgus and lesser toe deformities are highly heritable in white men and women of European descent, underscoring the importance of future work to identify genetic determinants of the underlying genetic susceptibility to these common foot disorders.
The natural history and clinical characteristics of paranasal sinus mucoceles: a clinical review
Authors: Scangas George A. , Gudis David A. , Kennedy David W. 
Published: May 2013

BackgroundA retrospective data analysis at a university tertiary referral center was conducted to characterize the natural history, clinical characteristics, management principles, and outcomes of paranasal sinus mucoceles.MethodsA chart review was performed on 102 patients with a total of 133 paranasal sinus mucoceles who were treated between 1987 and 2011 at the Hospital of the University of Pennsylvania.ResultsThe study population included patients with a mean age of 53.1 years (range, 22‐82 years). Patients were diagnosed with a mucocele on average 5.3 years following prior functional endoscopic sinus surgery (FESS), 17.7 years following prior paranasal sinus trauma, and 18.1 years following prior open sinus surgery. The most common presenting symptoms were headache (42.1%) and maxillofacial pressure (28.6%). The most common sites were the frontal, frontoethmoidal, and ethmoid sinuses. Fifty‐seven mucoceles (44.9%) had intraorbital extension, intracranial extension, or both. Out of 133 mucoceles, 114 underwent ESS without complication.ConclusionThe length of time between prior surgery or trauma and mucocele presentation highlights the importance of long‐term follow‐up in both patient care and in the understanding and reporting of surgical outcomes. In this study, most patients exhibited nonspecific symptomatology despite extensive mucoceles and a significant incidence of orbital and skull‐base erosion. The endoscopic approach can be safely used for the management of such lesions.
Functional and structural brain changes in anti–N‐methyl‐D‐aspartate receptor encephalitis
Authors: Finke Carsten , Kopp Ute A. , Scheel Michael , Pech Luisa‐Maria , Soemmer Carina , Schlichting Jeremias , Leypoldt Frank , Brandt Alexander U. , Wuerfel Jens , Probst Christian , Ploner Christoph J. , Prüss Harald , Paul Friedemann 
Published: May 2013

ObjectiveAnti–N‐methyl‐D‐aspartate receptor (NMDAR) encephalitis is an autoimmune encephalitis with a characteristic neuropsychiatric syndrome and severe and prolonged clinical courses. In contrast, standard clinical magnetic resonance imaging (MRI) remains normal in the majority of patients. Here, we investigated structural and functional brain changes in a cohort of patients with anti‐NMDAR encephalitis.MethodsTwenty‐four patients with established diagnosis of anti‐NMDAR encephalitis and age‐ and gender‐matched controls underwent neuropsychological testing and multimodal MRI, including T1w/T2w structural imaging, analysis of resting state functional connectivity, analysis of white matter using diffusion tensor imaging, and analysis of gray matter using voxel‐based morphometry.ResultsPatients showed significantly reduced functional connectivity of the left and right hippocampus with the anterior default mode network. Connectivity of both hippocampi predicted memory performance in patients. Diffusion tensor imaging revealed extensive white matter changes, which were most prominent in the cingulum and which correlated with disease severity. In contrast, no differences in T1w/T2w structural imaging and gray matter morphology were observed between patients and controls.InterpretationAnti‐NMDAR encephalitis is associated with characteristic alterations of functional connectivity and widespread changes of white matter integrity despite normal findings in routine clinical MRI. These results may help to explain the clinicoradiological paradox in anti‐NMDAR encephalitis and advance the pathophysiological understanding of the disease. Correlation of imaging abnormalities with disease symptoms and severity suggests that these changes play an important role in the symptomatology of anti‐NMDAR encephalitis. Ann Neurol 2013;74:257–265
Phenotypes of hypofrontality in older female fragile X premutation carriers
Authors: Yang Jin‐Chen , Simon Christa , Niu Yu‐Qiong , Bogost Mark , Schneider Andrea , Tassone Flora , Seritan Andreea , Grigsby Jim , Hagerman Paul J. , Hagerman Randi J. , Olichney John M. 
Published: May 2013

ObjectiveTo investigate the nature of cognitive impairments and underlying brain mechanisms in older female fragile X premutation carriers with and without fragile X‐associated tremor/ataxia syndrome (FXTAS).MethodsExtensive neuropsychological testing and cognitive event‐related brain potentials (ERPs; particularly, the auditory P300) were examined in 84 female participants: 33 fragile X premutation carriers with FXTAS (mean age = 62.8 years), 25 premutation carriers without FXTAS (mean age = 55.4 years), and 26 normal healthy controls (mean age = 59.3 years).ResultsBoth premutation groups exhibited executive dysfunction on the Behavioral Dyscontrol Scale, with subtle impairments in inhibition and performance monitoring in female carriers without FXTAS, and more substantial deficits in FXTAS women. However, the female carrier group without FXTAS showed more pronounced deficiencies in working memory. Abnormal ERPs were recorded over the frontal lobes, where FXTAS patients showed both P300 amplitude reduction and latency prolongation, whereas only decreased frontal P300 amplitudes were found in carriers without FXTAS. These frontal P300 measures correlated with executive function and information processing speed.InterpretationThe neuropsychological testing and ERP results of the present study provide support for the hypothesis that executive dysfunction is the primary cognitive impairment among older female premutation carriers both with and without FXTAS, although these deficits are relatively mild compared to those in FXTAS males. These findings are consistent with a synergistic effect of the premutation and aging on cognitive impairment among older female fragile X premutation carriers, even in those without FXTAS symptoms. Ann Neurol 2013;74:275–283
Stages of pTDP‐43 pathology in amyotrophic lateral sclerosis
Authors: Brettschneider Johannes , Del Tredici Kelly , Toledo Jon B. , Robinson John L. , Irwin David J. , Grossman Murray , Suh EunRan , Van Deerlin Vivianna M. , Wood Elisabeth M. , Baek Young , Kwong Linda , Lee Edward B. , Elman Lauren , McCluskey Leo , Fang Lubin , Feldengut Simone , Ludolph Albert C. , Lee Virginia M.‐Y. , Braak Heiko , Trojanowski John Q. 
Published: May 2013

ObjectiveTo see whether the distribution patterns of phosphorylated 43kDa TAR DNA‐binding protein (pTDP‐43) intraneuronal inclusions in amyotrophic lateral sclerosis (ALS) permit recognition of neuropathological stages.MethodspTDP‐43 immunohistochemistry was performed on 70μm sections from ALS autopsy cases (N = 76) classified by clinical phenotype and genetic background.ResultsALS cases with the lowest burden of pTDP‐43 pathology were characterized by lesions in the agranular motor cortex, brainstem motor nuclei of cranial nerves V, VII, and X–XII, and spinal cord α‐motoneurons (stage 1). Increasing burdens of pathology showed involvement of the prefrontal neocortex (middle frontal gyrus), brainstem reticular formation, precerebellar nuclei, and the red nucleus (stage 2). In stage 3, pTDP‐43 pathology involved the prefrontal (gyrus rectus and orbital gyri) and then postcentral neocortex and striatum. Cases with the greatest burden of pTDP‐43 lesions showed pTDP‐43 inclusions in anteromedial portions of the temporal lobe, including the hippocampus (stage 4). At all stages, these lesions were accompanied by pTDP‐43 oligodendroglial aggregates. Ten cases with C9orf72 repeat expansion displayed the same sequential spreading pattern as nonexpansion cases but a greater regional burden of lesions, indicating a more fulminant dissemination of pTDP‐43 pathology.InterpretationpTDP‐43 pathology in ALS possibly disseminates in a sequential pattern that permits recognition of 4 neuropathological stages consistent with the hypothesis that pTDP‐43 pathology is propagated along axonal pathways. Moreover, the finding that pTDP‐43 pathology develops in the prefrontal cortex as part of an ongoing disease process could account for the development of executive cognitive deficits in ALS. Ann Neurol 2013;74:20–38
Three times weekly glatiramer acetate in relapsing–remitting multiple sclerosis
Authors: Khan Omar , Rieckmann Peter , Boyko Alexey , Selmaj Krzysztof , Zivadinov Robert ,   GALA Study Group
Published: May 2013

ObjectiveTo assess the efficacy and safety of glatiramer acetate (GA) 40mg administered 3× weekly (tiw) compared with placebo in patients with relapsing–remitting multiple sclerosis (RRMS).MethodsThis randomized, double‐blind study was conducted in 142 sites in 17 countries. Patients with RRMS with at least 1 documented relapse in the 12 months before screening, or at least 2 documented relapses in the 24 months before screening, and an Expanded Disability Status Scale score ≤ 5.5, were randomized 2:1 to receive either subcutaneous (sc) GA 40mg tiw (1ml) or placebo for 12 months.ResultsOf 1,524 patients screened, 1,404 were randomized to receive GA 40mg sc tiw (n = 943) or placebo (n = 461). Ninety‐three percent and 91% of patients in the placebo and GA groups, respectively, completed the 12‐month study. GA 40mg tiw was associated with a 34.0% reduction in risk of confirmed relapses compared with placebo (mean annualized relapse rate = 0.331 vs 0.505; p < 0.0001). Patients who received GA 40mg tiw experienced highly significant reduction (p < 0.0001) in the cumulative number of gadolinium‐enhancing T1 (44.8%) and new or newly enlarging T2 lesions (34.7%) at months 6 and 12. GA 40mg tiw was safe and well tolerated. The most common adverse events in the GA group were injection site reactions (35.5% with GA vs 5.0% with placebo).InterpretationGA 40mg sc tiw is a safe and effective regimen for the treatment of RRMS, providing the convenience of fewer sc injections per week. ANN NEUROL 2013;73:705–713
Validation of midbrain positron emission tomography measures for nigrostriatal neurons in macaques
Authors: Brown Christopher A. , Karimi Morvarid K. , Tian LinLin , Flores Hugh , Su Yi , Tabbal Samer D. , Loftin Susan K. , Moerlein Stephen M. , Perlmutter Joel S. 
Published: May 2013

ObjectiveDevelopment of an effective therapy to slow the inexorable progression of Parkinson disease requires a reliable, objective measurement of disease severity. In the present study, we compare presynaptic positron emission tomography (PET) tracer uptake in the substantia nigra (SN) to cell loss and motor impairment in 1‐methyl‐4‐phenyl‐1,2,3,6‐tetrahydropyridine (MPTP)‐treated nonhuman primates.MethodsPresynaptic PET tracers 6‐[18F]‐fluorodopa (FD), [11C]‐2β‐methoxy‐3β‐4‐fluorophenyltropane (CFT), and [11C]‐dihydrotetrabenazine (DTBZ) were used to measure specific uptake in the SN and striatum before and after a variable dose of MPTP in nonhuman primates. These in vivo PET‐based measures were compared with motor impairment, as well as postmortem tyrosine hydroxylase‐positive cell counts and striatal dopamine concentration.ResultsWe found the specific uptake of both CFT and DTBZ in the SN had a strong, significant correlation with dopaminergic cell counts in the SN (R2 = 0.77, 0.53, respectively, p < 0.001), but uptake of FD did not. Additionally, both CFT and DTBZ specific uptake in the SN had a linear relationship with motor impairment (rs = −0.77, −0.71, respectively, p < 0.001), but FD uptake did not.InterpretationOur findings demonstrate that PET‐measured binding potentials for CFT and DTBZ for a midbrain volume of interest targeted at the SN provide faithful correlates of nigral neuronal counts across a full range of lesion severity. Because these measures correlate with both nigral cell counts and parkinsonian ratings, we suggest that these SN PET measures are relevant biomarkers of nigrostriatal function. Ann Neurol 2013;74:602–610