bluuebunny/crossref_metadata_2025_split huggingface dataset:
Non‐paraneoplastic Lambert–Eaton myasthenic syndrome presenting dropped head and respiratory failure with normoreflexia
Authors: Ikeda Kensuke , Kuroda Hiroshi , Ono Hiroya , Miura Emiko , Suzuki Naoki , Misu Tatsuro , Motomura Masakatsu , Aoki Masashi 
Published: February 2015

AbstractA 57‐year‐old woman developed insidious dropped head, respiratory failure and autonomic dysfunction with inconspicuous limb weakness, but without diminished deep tendon reflexes. Progressive myopathy affecting truncal muscles was initially suspected, but non‐paraneoplastic Lambert–Eaton myasthenic syndrome was finally diagnosed based on the results of electrophysiological studies and positivity for anti‐P/Q‐type voltage‐gated calcium channel antibody without any evidence of malignancy. In general, most patients with Lambert–Eaton myasthenic syndrome have more than one of the core manifestations of Lambert–Eaton myasthenic syndrome including proximal‐dominant limb weakness, diminished or absent deep tendon reflexes and autonomic dysfunction. However, this patient presented atypical manifestations, and lacked two core manifestations except for autonomic dysfunction. It is important to take Lambert–Eaton myasthenic syndrome into consideration in the differential diagnosis of patients showing atypical presentation, such as dropped head or respiratory failure, when they have only one core manifestation of Lambert–Eaton myasthenic syndrome.
DEFORMED FLORAL ORGAN1 (DFO1) regulates floral organ identity by epigenetically repressing the expression of OsMADS58 in rice (Oryza sativa)
Authors: Zheng Ming , Wang Yihua , Wang Yunlong , Wang Chunming , Ren Yulong , Lv Jia , Peng Cheng , Wu Tao , Liu Kai , Zhao Shaolu , Liu Xi , Guo Xiuping , Jiang Ling , Terzaghi William , Wan Jianmin 
Published: February 2015

Summary

Floral organ identity in plants is controlled by floral homeotic A/B/C/D/E‐class genes. In Arabidopsis thaliana, several epigenetic repressors that regulate these floral organ identity genes have been characterized. However, the roles of epigenetic factors in rice floral development have not been explored in detail. Here, we report the identification and functional characterization of a rice epigenetic repressor, DEFORMED FLORAL ORGAN1 (DFO1) gene, which causes abnormal floral morphology when mutated.

We isolated dfo1 by mapping, and confirmed its function by rescue experiments, combined with genetic, cytological and molecular biological analysis.

We showed that DFO1 is constitutively expressed and encodes a nuclear‐localized protein. Mutation of DFO1 causes the ectopic expression of C‐class genes in the dfo1‐1 mutant, and overexpression of OsMADS58, a C‐class gene, phenocopies the dfo1 mutants. In vitro and in vivo experiments demonstrated that DFO1 interacts with the rice polycomb group (PcG) proteins (OsMSI1 and OsiEZ1). Remarkably, trimethylation of histone H3 lysine 27, a mark of epigenetic repression, is significantly reduced on OsMADS58 chromatin in the dfo1‐1 mutant.

Our results suggest that DFO1 functions in maintaining rice floral organ identity by cooperating with PcG proteins to regulate the H3K27me3‐mediated epigenetic repression on OsMADS58.
AN INDIVIDUAL‐BASED MODEL FOR FERAL HOGS IN GREAT SMOKY MOUNTAINS NATIONAL PARK
Authors: SALINAS RENÉ A. , STIVER WILLIAM H. , CORN JOSEPH L. , LENHART SUZANNE , COLLINS CHARLES , MADDEN MARGUERITE , VERCAUTEREN KURT C. , SCHMIT BRANDON B. , KASARI ELLEN , ODOI AGRICOLA , HICKLING GRAHAM , MCCALLUM HAMISH 
Published: February 2015

AbstractThe expansion of feral hog (Sus scrofa) populations in the United States has resulted in increased efforts to develop and implement control strategies designed to minimize the impacts done by this invasive species. We describe an individual‐based model for feral hogs in Great Smoky Mountains National Park (GSMNP). The objectives of the model are to provide an understanding of the population dynamics of this feral hog population and to determine the efficacy of the annual harvest as a population control method. Results suggest that the dynamics of the population are driven by fall hard mast production and the GSMNP harvests currently limit growth of the population, but these control efforts have not reduced the population.
The CRISPR/Cas system inhibited the pro‐oncogenic effects of alternatively spliced fibronectin extra domain A via editing the genome in salivary adenoid cystic carcinoma cells
Authors: Wang H‐C , Yang Y , Xu S‐Y , Peng J , Jiang J‐H , Li C‐Y 
Published: February 2015

ObjectivesTo identify the association of fibronectin (FN) extra domain A (EDA) with the progression of salivary adenoid cystic carcinoma (SACC). Accordingly, the exclusion of EDA exon through the CRISPR/Cas9 system was investigated as the rescue for such pro‐oncogenic splicing.Materials and methodsSACC‐83 cells were transiently transfected with plasmids containing recombinant EDA, and the cellular growth and motility were then accessed in vitro. Epithelial–mesenchymal transition (EMT) was investigated with immunohistochemistry, Western blot, and real‐time PCR analysis. SACC tissues from 81 patients were used to access the associations between EDA+FN and clinical–pathological parameters. CRISPR/Cas9 plasmids containing sgRNA were designed and co‐transfected into SACC‐83 cells; the effects of EDA knockout on cellular growth and motility were then accessed.ResultsThe recombinant EDA exhibited little effect on the proliferation of SACC cells, but significantly promoted the migration and invasion of the cells (P < 0.05), accompanied with upregulated EMT (P < 0.05); consistently, the expression of EDA+FN was positively associated with the metastasis, nerve invasion and recurrence of SACC (P < 0.05). Furthermore, the EDA knockout from the FN gene in most SACC cells resulted in a decrease in cell motility and invasion, as well as prolonged population doubling time, compared with untreated SACC‐83 cells (P < 0.05).ConclusionThe EDA domain significantly promoted the motility of SACC cells, and positively associated with the tumor progression in patients with SACC. Thus, it is a potential risk factor and also a therapeutic target for SACC. The CRISPR/Cas9 system may control a pro‐oncogenic splicing process through the exclusion of EDA exon from the FN gene, leading to inhibition of motility, invasion and proliferation of cancer cells.
Visual discomfort while watching stereoscopic three‐dimensional movies at the cinema
Authors: Zeri Fabrizio , Livi Stefano 
Published: February 2015

AbstractPurposeThis study investigates discomfort symptoms while watching Stereoscopic three‐dimensional (S3D) movies in the ‘real’ condition of a cinema. In particular, it had two main objectives: to evaluate the presence and nature of visual discomfort while watching S3D movies, and to compare visual symptoms during S3D and 2D viewing.MethodCinema spectators of S3D or 2D films were interviewed by questionnaire at the theatre exit of different multiplex cinemas immediately after viewing a movie.ResultsA total of 854 subjects were interviewed (mean age 23.7 ± 10.9 years; range 8–81 years; 392 females and 462 males). Five hundred and ninety‐nine of them viewed different S3D movies, and 255 subjects viewed a 2D version of a film seen in S3D by 251 subjects from the S3D group for a between‐subjects design for that comparison. Exploratory factor analysis revealed two factors underlying symptoms: External Symptoms Factors (ESF) with a mean ± S.D. symptom score of 1.51 ± 0.58 comprised of eye burning, eye ache, eye strain, eye irritation and tearing; and Internal Symptoms Factors (ISF) with a mean ± S.D. symptom score of 1.38 ± 0.51 comprised of blur, double vision, headache, dizziness and nausea. ISF and ESF were significantly correlated (Spearman r = 0.55; p = 0.001) but with external symptoms significantly higher than internal ones (Wilcoxon Signed‐ranks test; p = 0.001). The age of participants did not significantly affect symptoms. However, females had higher scores than males for both ESF and ISF, and myopes had higher ISF scores than hyperopes. Newly released movies provided lower ESF scores than older movies, while the seat position of spectators had minimal effect. Symptoms while viewing S3D movies were significantly and negatively correlated to the duration of wearing S3D glasses. Kruskal–Wallis results showed that symptoms were significantly greater for S3D compared to those of 2D movies, both for ISF (p = 0.001) and for ESF (p = 0.001).ConclusionsIn short, the analysis of the symptoms experienced by S3D movie spectators based on retrospective visual comfort assessments, showed a higher level of external symptoms (eye burning, eye ache, tearing, etc.) when compared to the internal ones that are typically more perceptual (blurred vision, double vision, headache, etc.). Furthermore, spectators of S3D movies reported statistically higher symptoms when compared to 2D spectators.
Intestinal lymphoid nodular hyperplasia in children: The relationship to food allergy
Authors: Lucarelli Sandra , Lastrucci Ginevra , Di Nardo Giovanni , D'Alfonso Ylenia , Aloi Marina , Oliva Salvatore , Frediani Simone , Rossetti Danilo , Frediani Tullio 
Published: February 2015

AbstractBackgroundLymphoid nodular hyperplasia (LNH) of the lower gastrointestinal tract is a common finding during paediatric colonoscopies, and its clinical significance has not yet been clearly established.ObjectiveThe purpose of this prospective, parallel multi‐arm, randomized clinical trial was to study relationship between food allergy and LNH.MethodsWe recruited 268 children who had undergone a diagnostic colonoscopy between 1 January 2009 and 30 September 2011. The inclusion criteria were the following: (i) demonstration of LNH; (ii) no concomitant inflammatory or immune disease; (iii) no treatment since the clinical onset. The patients were assigned 1:1:1 to elimination diet (Group A), mesalamine (Group B) or symptomatic treatment with antispasmodics or antidiarrhoeal drugs (Group C) for an 8‐wk period. Patients were followed for 24 months.ResultsWe enrolled 72 of 97 children with intestinal LNH who were referred for haematochezia (76%), recurrent abdominal pain (68%) and/or chronic diarrhoea (32%). Clinical improvement was observed in 75%, 83% and 79% of the patients in Groups A, B and C, respectively. The difference in the response to treatment among the groups and the association to the clinical, endoscopic and allergic features of the patients were not statistically significant.ConclusionsIntestinal LNH should be considered a benign finding in children without red flags, because the symptoms are largely self‐limiting. The use of mesalamine or elimination diet does not modify the clinical outcome compared to symptomatic therapy. The presence of food allergy was not predicted by allergy skin testing and was found in a minority of patients.
Dermatologic Features of Smith–Magenis Syndrome
Authors: Guérin‐Moreau Morgane , Colin Estelle , Nguyen Sylvie , Andrieux Joris , de Leersnyder Hélène , Bonneau Dominique , Martin Ludovic 
Published: February 2015

AbstractSmith–Magenis syndrome (SMS) is characterized by distinctive facial and skeletal features, developmental delay, cognitive impairment, and behavioral abnormalities, including self‐injurious behaviors. We aimed to investigate whether cutaneous features are common in SMS. We performed a complete skin examination in 20 young SMS patients. Skin features secondary to self‐injurious behavior, such as bites, abrasions, dystrophic scars, limited spots of hyperkeratosis, anomalies of the nails, and whitlows, were found in the majority of patients. Acral pachydermia and fissured plantar keratoderma were common. Xerosis was constant and associated with extensive keratosis pilaris in the majority of patients. Dermatofibromas were frequent in older patients. The hair was dense and shiny, with an unusual hairline. Eyelash trichomegaly and heavy brows were common, as well as folliculitis on the back. The skin features of SMS have rarely been reported in the literature. Some of these are the consequence of neurobehavioral features, but some cutaneous features and abnormalities of appendages have not been reported in other related syndromes. Skin manifestations of SMS are varied, sometimes induced by self‐injurious behavior and sometimes more specific. It remains to be determined whether the combination of the two kinds of signs could contribute to early diagnosis of the syndrome.
Prognostic significance of aminopeptidase‐N (CD13) in hepatoblastoma
Authors: Saida Satoshi , Watanabe Ken‐ichiro , Kato Itaru , Fujino Hisanori , Umeda Katsutsugu , Okamoto Shinya , Uemoto Shinji , Hishiki Tomoro , Yoshida Hideo , Tanaka Shiro , Adachi Souichi , Niwa Akira , Nakahata Tatsutoshi , Heike Toshio 
Published: February 2015

AbstractBackgroundHepatoblastoma is a rare childhood malignant tumor that originates from immature hepatic cells. Aminopeptidase‐N(CD13), an ectopeptidase that promotes tumor invasion and metastasis, is expressed in fetal stage hepatic progenitor cells, although its role in hepatoblastoma remains unclear.MethodsThe expression pattern of CD13 was investigated on immunohistochemistry in 30 tissue samples from 27 hepatoblastoma patients (16 with predominantly embryonal [pE] histology and 14 with predominantly fetal [pF] histology). Immunoreactive score (IRS) was used to quantify staining data, and the relationship between CD13 expression, clinicopathological factors, and clinical outcome was investigated. The biological function of CD13 was also examined in the hepatoblastoma cell lines Huh6 and HepG2.ResultsAll specimens stained positive for CD13, with higher CD13 expression in pE than in pF hepatoblastoma samples (median IRS, 4; range, 2–9 vs 2; range, 1–4). Strong CD13 expression was correlated with vascular invasion. Five year event‐free survival and overall survival were better in patients with CD13low than in those with CD13high tumors (100% vs 51.0%, P = 0.026; and 100% vs 74.0%, P = 0.114, respectively). A CD13‐neutralizing antibody and the potent CD13 inhibitor, Ubenimex, suppressed invasive activity in HepG2 cells in vitro.ConclusionsCD13 expression is associated with hepatoblastoma invasiveness and could be a novel prognostic marker for hepatoblastoma.
Risk factors for seizure recurrence in children after first unprovoked seizure
Authors: Mizorogi Sonoko , Kanemura Hideaki , Sano Fumikazu , Sugita Kanji , Aihara Masao 
Published: February 2015

AbstractBackgroundThe question of whether to treat a patient after a first unprovoked seizure is controversial. This prospective study assessed the time to recurrence and risk factors for seizure recurrence after a first unprovoked seizure in children.MethodsParticipants were recruited between 1 July 1997, and 30 June 2009. Eligible candidates were children between 1 month and 15 years old who presented with their first unprovoked afebrile seizure. After enrollment, recurrence of seizures was investigated. All participants were followed for at least 2 years. Log‐rank test was used for bivariate analysis to check associations, and hazard ratios were used to analyze variables and clinical outcome (recurrence) during follow‐up.ResultsOf 73 subjects, 42 (57.5%) experienced recurrence. The overall product‐limit estimate of recurrence was 61.9% at 6 months, 85.7% at 1 year, and 95.2% at 2 years after seizure onset, respectively. Incidence of recurrence with partial and generalized seizures was 69.0% and 31.0%, respectively. Children with partial seizures had recurrence significantly more often than those with generalized seizures (P < 0.001). Recurrent seizures occurred after normal findings on electroencephalogram (EEG) in 21.4%, after generalized spike‐and‐wave complexes in 16.7%, and after focal epileptic discharge in 61.9%. Children with focal epileptic discharge had recurrence significantly more often than children with normal EEG findings (P < 0.001).ConclusionThe time to seizure recurrence after first unprovoked seizure may be within 1 year, and particularly within 6 months; and partial seizure and abnormal EEG with focal epileptic discharge may be risk factors for seizure recurrence.
Clinical features, outcomes, and genetic analysis in Korean children with Alagille syndrome
Authors: Cho Jin Min , Oh Seak Hee , Kim Hyun Jin , Kim Joon Sung , Kim Kyung Mo , Kim Gu‐Hwan , Yu Eunsil , Lee Beom Hee , Yoo Han‐Wook 
Published: February 2015

AbstractBackgroundAlagille syndrome (AGS) is a multisystem autosomal dominant disorder that affects the liver, heart, eyes, face, bone, and other organs. AGS is caused by mutations in one of two genes, JAG1 or NOTCH2. We evaluated clinical features, outcomes, and the presence of JAG1 and NOTCH2 mutations in Korean children with AGS.MethodsBetween January 1997 and December 2013, 19 children were diagnosed with AGS at Asan Medical Center, Seoul, Korea. Their clinical features, outcomes, and JAG1 and NOTCH2 mutation status were retrospectively analyzed.ResultsThe prevalence of clinical features in the 19 patients was as follows: dysmorphic facial features, 100% (n = 19); liver symptoms, 89% (n = 17); cardiac symptoms, 95% (n = 18); ophthalmologic symptoms, 67% (n = 10); skeletal deformities, 47% (n = 9); and renal symptoms, 21% (n = 4). JAG1 mutations were identified in 14 patients. The 13 different JAG1 mutations, seven of which were novel, included four deletions, three insertions, two missense mutations, three nonsense mutations, and one indel mutation. No NOTCH2 mutations were found. Two patients who received liver transplantation due to liver failure were still alive. Two patients died of comorbidities related to AGS: one of cardiac failure and one of hepatic failure.Conclusion This study describes the clinical characteristics of 19 Korean AGS patients with seven novel JAG1 mutations. Neonatal cholestatic jaundice was the most common initial presenting symptom; thus the presence of neonatal cholestasis warrants screening for syndromic features of AGS. Complex heart anomalies and progressive liver dysfunction resulted in significant morbidity and mortality in AGS.
Sirolimus‐induced interstitial lung disease following pediatric stem cell transplantation
Authors: Garrod Andrea S. , Goyal Rakesh K. , Weiner Daniel J. 
Published: February 2015

AbstractSirolimus‐induced ILD is a known but rare complication in adults who have undergone SOT. However, little is known about this adverse effect in children. Diagnosis of sirolimus‐induced ILD can be challenging, especially in patients who have difficulty participating in lung function testing. We present a case of presumed sirolimus‐induced ILD in a pediatric stem cell transplant patient who developed polycythemia and hypoxemia. To our knowledge, no other cases of sirolimus‐induced pulmonary toxicity in children after HCT have been reported.
Oral mucositis in pediatric patients undergoing hematopoietic stem cell transplantation: Clinical outcomes in a context of specialized oral care using low‐level laser therapy
Authors: Eduardo Fernanda de Paula , Bezinelli Leticia Mello , de Carvalho Danielle Lima Corrêa , Lopes Roberta Marques da Graça , Fernandes Juliana Folloni , Brumatti Melina , Vince Carolina Sgaroni Camargo , de Azambuja Alessandra Milani Prandini , Vogel Cristina , Hamerschlak Nelson , Correa Luciana 
Published: February 2015

AbstractOM is a painful inflammatory condition of the oral mucosa, derived from the toxic effects of chemotherapy and radiotherapy. High OM severity is frequently present in HSCT pediatric patients, who exhibit multiple painful ulcers that limit their mastication and swallowing, leading to poor nutritional status. Few studies have demonstrated OM clinical outcomes in young patients undergoing HSCT. Feasibility of oral care and LLLT on OM prophylaxis and treatment is also poorly discussed. The aim of this study was to describe a specialized oral care protocol that included LLLT for pediatric patients undergoing transplantation and to demonstrate the clinical outcomes after OM prevention and treatment. Data from OM‐related morbidity were collected from 51 HSCT pediatric patients treated daily with LLLT, followed by standard oral care protocols. All the patients, even infants and young children, accepted the daily oral care and LLLT well. The majority (80.0%) only exhibited erythema in the oral mucosa, and the maximum OM degree was WHO II. Patients who had undergone autologous and HLA‐haploidentical transplants showed OM with the lowest severity. The frequency of total body irradiation and methotrexate prescriptions was higher in adolescents when compared with infants (p = 0.044), and adolescents also exhibited OM more severely than infants and young children. We found that good clinical outcomes were obtained using this therapy, mainly in regard to the control of OM severity and pain reduction in the oral cavity. Specialized oral care, including LLLT, is feasible and affordable for HSCT pediatric patients, although some adaptation in the patient's oral hygiene routine must be adopted with help from parents/companions and clinical staff.
Low rates of iridoid glycoside hydrolysis in twoLongitarsusleaf beetles with different feeding specialization confer tolerance to iridoid glycoside containing host plants
Authors: Pankoke Helga , Dobler Susanne 
Published: February 2015

AbstractIridoid glycosides are plant defence compounds that are deterrent and/or toxic for unadapted herbivores but are readily sequestered by dietary specialists of different insect orders. Hydrolysis of iridoid glycosides by β‐glucosidase leads to protein denaturation. Insect digestive β‐glucosidases thus have the potential to mediate plant–insect interactions. In the present study, mechanisms associated with iridoid glycoside tolerance are investigated in two closely‐related leaf beetle species (Coleoptera:Chrysomelidae) that feed on iridoid glycoside containing host plants. The polyphagousLongitarsus luridusScopoli does not sequester iridoid glycosides, whereas the specialistLongitarsus tabidusFabricius sequesters these compounds from its host plants. To study whether the biochemical properties of their β‐glucosidases correspond to the differences in feeding specialization, the number of β‐glucosidase isoforms and their kinetic properties are compared between the two beetle species. To examine the impact of iridoid glycosides on the β‐glucosidase activity of the generalist,L. luridusbeetles are kept on host plants with or without iridoid glycosides. Furthermore, β‐glucosidase activities of both species are examined using an artificial β‐glucosidase substrate and the iridoid glycoside aucubin present in their host plants. Both species have one or two β‐glucosidases with different substrate affinities. Interestingly, host plant use does not influence the specific β‐glucosidase activities of the generalist. Both species hydrolyse aucubin with a much lower affinity than the standard substrate. The neutralpHreduces the β‐glucosidase activity of the specialist beetles by approximately 60% relative to itspHoptimum. These low rates of aucubin hydrolysis suggest that the ability to sequester iridoid glycosides has evolved as a key to potentially preventing iridoid glycoside hydrolysis by plant‐derived β‐glucosidases.
Automated 3d feature matching
Authors: Chuang Tzu‐Yi , Jaw Jen‐Jer 
Published: February 2015

AbstractThree‐dimensional (3D) feature‐matching techniques, which are essential for progress towards an automated feature‐based procedure, have attracted considerable research attention in both the photogrammetry and computer vision communities. This study introduces a novel matching approach, called RSTG, that comprises four major phases: rotation alignment; scale estimation; translation alignment; and geometry checks. These steps efficiently determine a feature‐based correspondence and frame transformation between datasets. RSTG analyses the similarity and relative geometry of features by employing feature observations and their uncertainty; this allows different types of features to be matched exclusively or simultaneously. This study validates the proposed method with both simulated and real datasets, demonstrating its effectiveness with satisfactory matching rates in a diverse range of feature‐based point cloud registration tasks.