bluuebunny/crossref_metadata_2025_split huggingface dataset:
Hypothalamic hamartomas in adulthood: Clinical spectrum and treatment outcome—A unicenter experience
Authors: Conde Blanco Estefanía , Anciones Martín Carla , Manzanares Isabel , Gil López Francisco , Roldán Pedro , Donaire Antonio , Rumiá Jordi , Carreño Mar 
Published: October 2019

AbstractIntroductionClinical manifestations of the hypothalamic hamartoma‐epilepsy syndrome (HH‐ES) in adulthood are variable. Efficacy of therapeutic options and outcome are diverse.MethodsRetrospective study of adult patients diagnosed with a HH in magnetic resonance imaging and epilepsy who attended our tertiary Epilepsy Unit between 2003 and 2018. We report the clinical and electroencephalographic features of a series of adult patients with HH and related epilepsy seen in our center together with the treatments and seizure outcome.ResultsWe describe a series of eight patients. Five males (62.5%), median age at evaluation was 28.5 years (IQR: 15.5). Clinical manifestations included focal with preserved and impaired awareness emotional seizures (gelastic seizures [GS]) in six patients (75%), focal tonic, atonic with impaired awareness and focal to bilateral tonic–clonic seizures. Mild GS were the only symptom in one patient. Three patients (37.5%) had endocrinological disturbances such as obesity and hypothyroidism. Fifty percent of the patients showed psychiatric comorbidity such as anxiety disorder and aggressiveness, and two patients had psychomotor delay. Seven patients (87.7%) had drug‐resistant seizures and three of them were treated with radiosurgery. Out of the treated group, only one (33.3%) became seizure‐free 2 years after surgery but developed psychiatric problems. The other two patients had an Engel IV outcome and received a vagal nerve stimulation (VNS) implant. VNS did not lead to changes either in intensity nor in seizure frequency.ConclusionsHypothalamic hamartoma‐epilepsy syndrome clinical manifestations in adult patients are as variable as at pediatric age. Outcome of therapeutic options such as radiosurgery or VNS may be poorer at this stage.
Yeast surface display of full‐length human microtubule‐associated protein tau
Authors: Wang Shiyao , Cho Yong Ku 
Published: October 2019

AbstractMicrotubule‐associated protein tau is an intrinsically disordered, highly soluble protein found primarily in neurons. Under normal conditions, tau regulates the stability of axonal microtubules and intracellular vesicle transport. However, in patients of neurodegeneration such as Alzheimer's disease (AD), tau forms neurofibrillary deposits, which correlates well with the disease progression. Identifying molecular signatures in tau, such as posttranslational modification, truncation, and conformational change has great potential to detect earliest signs of neurodegeneration and develop therapeutic strategies. Here, we show that full‐length human tau, including the longest isoform found in the adult brain, can be robustly displayed on the surface of yeast Saccharomyces cerevisiae. Yeast‐displayed tau binds to anti‐tau antibodies that cover epitopes ranging from the N‐terminus to the 4R repeat region. Unlike tau expressed in the yeast cytosol, surface‐displayed tau was not phosphorylated at sites found in AD patients (probed by antibodies AT8, AT270, AT180, and PHF‐1). However, yeast‐displayed tau showed clear binding to paired helical filament (PHF) tau conformation‐specific antibodies Alz‐50, MC‐1, and Tau‐2. Although the tau possessed a conformation found in PHFs, oligomerization or aggregation into larger filaments was undetected. Taken together, yeast‐displayed tau enables robust measurement of protein interactions and is of particular interest for characterizing conformational change.
Genistein protects ovarian granulosa cells from oxidative stress via cAMP‐PKA signaling
Authors: Luo Man , Yang Zhan‐Qing , Huang Ji‐Cheng , Wang Yu‐Si , Guo Bin , Yue Zhan‐Peng 
Published: October 2019

AbstractGenistein is an isoflavone that has estrogen (E2)‐like activity and is beneficial for follicular development, but little is known regarding its function in oxidative stress (OS)‐mediated granulosa cell (GC) injury. Here, we found that after exposure to H2O2, Genistein weakened the elevated levels of intracellular reactive oxygen species (ROS) and malondialdehyde (MDA), which were regarded as the biomarkers for OS, and rescued glutathione (GSH) content and GSH/GSSG ratio accompanying with a simultaneous increase in cyclic adenosine monophosphate (cAMP) level, whereas addition of protein kinase A (PKA) inhibitor H89 impeded the effects of Genistein on the levels of ROS and MDA. Further analysis evidenced that Genistein enhanced the activities of antioxidant enzymes superoxide dismutase (SOD), GSH‐peroxidase (GSH‐Px), and catalase (CAT) in H2O2‐treated GCs, but this enhancement was attenuated by H89. Under OS, Genistein improved cell viability and lessened the apoptotic rate of GCs along with a reduction in the activity of Casp3 and levels of Bax and Bad messenger RNA (mRNA), while H89 reversed the above effects. Moreover, Genistein treatment caused an obvious elevation in mitochondrial membrane potential (MMP) followed by a decline in the levels of intracellular mitochondrial superoxide, but H89 inhibited the regulation of Genistein on MMP and mitochondrial superoxide. Supplementation of Genistein promoted the secretion of E2 and increased the expression of Star and Cyp19a1 mRNA, whereas suppressed the level of progesterone (P4) accompanied with a decline in the level of Hsd3b1 mRNA expression. H89 blocked the regulation of Genistein on the secretion of E2 and P4, and alleviated the ascending of Star and Cyp19a1 elicited by Genistein. Collectively, Genistein protects GCs from OS via cAMP‐PKA signaling.
Modified kissing balloon inflation associated with better results after Culotte stenting for bifurcation lesions: A bench test
Authors: Liu Jia , Li Li , Chen Can , Wei Jianrui , Chen Xiwei , Li Biao , Chen Yaogui , Luo Jingyun , Chen Shao‐Liang 
Published: October 2019

AbstractBackgroundMain vessel (MV) stent deformation and overstretch caused by classical kissing balloon inflation (C‐KBI) using two balloons with a longer overlapping in the MV for bifurcation lesions has caused a widespread concern.PurposeThis bench study tested our hypothesis that mini‐KBI (M‐KBI) with a shorter protrusion of side branch (SB) balloon would ascertain a better result after Culotte stenting.MethodsTwenty‐four coronary stents were deployed using Culotte approach in twelve bifurcation models with a bifurcation angle of 45°, 3.5 mm in MV diameter, and 3.0 mm in SB diameter. After stent implantation, the final KBI were assigned to C‐KBI (two kissing balloons juxtaposed within the MV stent, at least overlap for 3 mm; n = 6) and M‐KBI (the proximal marker of SB balloon just sited at the level of upper edge of SB ostium; n = 6). Proximal optimization technique (POT) was performed after KBI. Stent geometry was visually evaluated based on bench photos, microscopy, videoscopy, micro‐CT, and scanning electron microscopy. Stent deformation index, minimal lumen diameter, and cross‐sectional area at either carina level of MV and ostium of SB were measured from optical coherence tomography (OCT).ResultsIn Culotte technique, C‐KBI was associated with visually significant stent deformation, overexpansion and the “bottleneck” effect of the MV stent, which could not be effectively rectified by POT, while M‐KBI could keep the circle shape of MV stent with good stent apposition in both MV and SB stent. By quantitative measurements, deformation index of MV was 0.06 ± 0.01 after M‐KBI, significantly lower than 0.25 ± 0.02 if C‐KBI was performed. In the line in carina, compared to C‐KBI, M‐KBI has smaller CSA‐stent/CSA‐reference, which indicated a less overstretch of MV stent. However, minimal lumen diameter and cross‐sectional area of SB ostium was not different in the mini‐KBI group (3.0958 ± 0.0285 mm and 7.9667 ± 0.1741 mm), when compared those after C‐KBI (3.1217 ± 0.0772 mm and 7.9083 ± 0.3115 mm, p > .05).ConclusionsFollowed by POT, M‐KBI is preferable than C‐KBI in preventing stent deformation, overexpansion in MV stent and could get well apposed of MV stent and well‐opened SB stent as expected in a Culotte technique.
Encephalitis as a complication of a spinal‐esophageal fistula due to discitis
Authors: Lee Jae Hee , Kim Eun , Choi Yoon Hee 
Published: October 2019

AbstractIn patients with back or scapular pain, if there is evidence of neurological symptoms, fever, chills, malaise, or other signs of infection, the work‐up should consider the possibility of a spinal‐esophageal fistula.
The elusive nature of mucormycosis in an immunocompetent host and the role of a dermatology consult
Authors: Badin Dylan J. , Baker Catherine , Simmons Brian J. , Yan Shaofeng , Zug Kathryn A. 
Published: October 2019

Key Clinical MessageMucormycosis infection in the immunocompetent host typically occurs in the setting of trauma and presents a diagnostic challenge. The earliest signs of infection are often cutaneous, but can easily be misinterpreted, which can be a fatal mistake. Dermatology has tools to help recognize these infections and initiate earlier therapy.
A contemporary analysis of racial disparities in recommended and received treatment for head and neck cancer
Authors: Nocon Cheryl C. , Ajmani Gaurav S. , Bhayani Mihir K. 
Published: October 2019

BackgroundRacial disparities in squamous cell carcinoma of the head and neck (HNSCC) negatively affect non‐Hispanic black (NHB) patients. This study was aimed at understanding how treatment is prescribed and received across all HNSCC subsites.MethodsWith the National Cancer Database, patients from 2004 to 2014 with surgically resectable HNSCCs, including tumors of the oral cavity (OC), oropharynx (OP), hypopharynx (HP), and larynx (LX), were studied. The treatment received was either upfront surgery or nonsurgical treatment. Treatment patterns were compared according to race and subsite, and how these differences changed over time was evaluated.ResultsNHB patients were less likely than non‐Hispanic white (NHW) patients to receive surgery across all subsites (relative risk [RR] for OC, 0.87; RR for OP, 0.75; RR for HP, 0.73; RR for LX, 0.87; all P values <.05). They were also more likely to refuse a recommended surgery (RR for OC, 1.50; RR for OP, 1.23; RR for HP, 1.23; RR for LX, 1.34), and this difference was significant except for HP. NHB patients were more likely to not be offered surgery across all subsites (RR for OC, 1.38; RR for OP, 1.07; RR for HP, 1.05; RR for LX, 1.03; all P values <.05). Rates of surgery increased and rates of not being offered surgery declined for both NHB and NHW patients from 2004 to 2014, but the absolute disparities persisted in 2014.ConclusionsAcross all HNSCC subsites, NHB patients were less likely than NHW patients to be recommended for and receive surgery and were more likely to refuse surgery. These differences have closed over time but persist. Enhanced shared decision making may improve these disparities.
Changes in internet use and wishes of cancer survivors: A comparison between 2005 and 2017
Authors: van Eenbergen Mies C. H. J. , Vromans Ruben D. , Boll Dorry , Kil Paul J. M. , Vos Caroline M. , Krahmer Emiel J. , Mols Floortje , van de Poll‐Franse Lonneke V. 
Published: October 2019

BackgroundGiven the major changes in internet use for health communication, the objective of the current study was to compare the internet use and wishes of cancer survivors between 2005 and 2017.MethodsThe authors drew a sample of 390 patients in 2005 and 539 patients in 2017 who were diagnosed with breast (128 patients in 2005 and 143 patients in 2017), prostate (96 patients in 2005 and 126 patients in 2017), or gynecologic (89 patients in 2005 and 188 patients in 2017) cancer or lymphoma (77 patients in 2005 and 82 patients in 2017) in 4 different hospitals for the periods 2002 through 2004 and 2014 through 2016. These patients were sent a paper‐based questionnaire that contained 45 questions regarding demographics and 4 functions of internet use: content, communication, community, and e‐health.ResultsThe response in 2017 (53%) was lower than that in 2005 (75%). Survivors browsed the internet most frequently to search for information regarding cancer shortly after being diagnosed and while waiting for treatment. There was little change noted with regard to the relative importance attached to the various subjects. In 2017, significant increases were evident with regard to finances (+33%), health care insurance (+29%), and genetics and/or heritability (+27%). The wishes expressed in 2005 by patients were realized in part in 2017.ConclusionsA significant sample of cancer survivors in the Netherlands have indicated that the internet is an important source of information regarding their illness. However, little change was evident over the past 15 years with regard to patients' priorities regarding their wishes for internet use. The wishes of users in 2005 were found to accurately reflect the internet use of the majority of patients in 2017. The results of the current study support the belief that health care professionals should expand their online services and tailor them toward the needs and wishes of their patients.
Patterns of recurrence and metastasis in BRCA1/BRCA2‐associated breast cancers
Authors: Song Yun , Barry William T. , Seah Davinia S. , Tung Nadine M. , Garber Judy E. , Lin Nancy U. 
Published: October 2019

BackgroundBreast cancer subtypes are associated with distinct metastatic patterns. Whether germline BRCA1/BRCA2 mutation status is independently associated with central nervous system (CNS) relapse, controlling for tumor subtype, is unknown.MethodsPatients who were treated at Dana‐Farber Cancer Institute and diagnosed with a first locoregional recurrence (LRR) or metastasis between 1981 and 2014 were identified using 2 institutional registries: 1) patients treated for recurrent breast cancer and 2) patients who underwent BRCA testing. The frequencies of LRR, sites of metastasis, and breast cancer‐specific survival from LRR or metastasis were calculated, and the factors associated with CNS recurrence were evaluated using multivariable logistic regression models.ResultsThe final study cohort included 30 BRCA1 mutation carriers, 32 BRCA2 mutation carriers, and 270 noncarriers. Most BRCA1 carriers (73%) had triple‐negative breast cancer; whereas most BRCA2 carriers (72%) had hormone receptor‐positive tumors. BRCA1 carriers frequently experienced lung and distant lymph node metastasis, whereas BRCA2 carriers and noncarriers most often experienced bone metastasis. Although CNS disease occurred frequently in both BRCA1 and BRCA2 carriers (53% BRCA1, 50% BRCA2, 25% noncarriers; P < .001), only BRCA2 mutation (P = .006) was significantly associated with CNS metastasis in multivariable analysis controlling for tumor subtype. BRCA2 mutation (P = .01), triple‐negative subtype (P < .001), and the involvement of CNS (P < .001) and other non‐CNS distant sites (relative to locoregional recurrence or contralateral disease; P < .001) at presentation of recurrent breast cancer were associated with risk for mortality.ConclusionsCNS involvement is frequent in women with germline BRCA1/BRCA2 mutations who have metastatic breast cancer. BRCA2 mutation carriers had a significantly higher frequency of CNS metastasis than noncarriers when controlling for breast cancer subtype.
Pancreatoblastoma: Cytologic and histologic analysis of 12 adult cases reveals helpful criteria in their diagnosis and distinction from common mimics
Authors: Reid Michelle D. , Bhattarai Shristi , Graham Rondell P. , Pehlivanoglu Burcin , Sigel Carlie S. , Shi Jiaqi , Saqi Anjali , Shirazi Maryam , Xue Yue , Basturk Olca , Adsay Volkan 
Published: October 2019

BackgroundPancreatoblastoma (PBL) is a rare malignant pancreatic tumor seen predominantly in childhood, and its cytologic diagnosis remains challenging.MethodsTwelve fine‐needle‐aspirations from 11 adults were analyzed.ResultsIn total, 6 men and 5 women (median age, 45 years; age range, 32‐60 years) had tumors measuring a median 5.6 cm (range, 2.5‐12 cm) located in the pancreatic head (n = 7) or tail (n = 4), including 3 with familial adenomatous polyposis (FAP)/FAP‐related syndromes and 4 with metastasis at diagnosis. The median follow‐up was 39.8 months (range, 0.8‐348 months), and 5 patients died of disease. The original cytology diagnoses were: PBL (n = 2), neuroendocrine neoplasm (n = 2), poorly differentiated neuroendocrine carcinoma (n = 2), well differentiated neuroendocrine tumor (n = 1), poorly differentiated carcinoma (n = 2), “positive for malignancy” (n = 1), acinar cell carcinoma (n = 1), and epithelioid neoplasm with endocrine and acinar differentiation versus PBL (n = 1). Universal cytopathologic findings included hypercellularity; 3‐dimensional clusters; and single, monotonous, blast‐like cells that were from 1.5 to 2.0 times the size of red blood cells with high nuclear‐to‐cytoplasmic ratio, fine chromatin, small, distinct nucleoli, and a resemblance to well differentiated neuroendocrine tumor and poorly differentiated neuroendocrine carcinoma. Branching pseudopapillae (n = 7) and grooved nuclei (n = 3) raised the differential diagnosis of solid‐pseudopapillary neoplasm, but with more atypia. Uncommon features included pleomorphism (n = 4) and numerous mitoses (n = 1). Squamoid morules were seen on smears (n = 5) or cell blocks (n = 6) in 70% of patients and were characterized by epithelioid cells with elongated, streaming nuclei, fine chromatin, absent nucleoli, and positive nuclear β‐catenin (n = 6 of 8). The median Ki‐67 index was 21% (range, 2%‐70%), and neuroendocrine marker expression was common (100%), but acinar markers were variable (63%).ConclusionsA combination of cytologic findings in PBL, including a predominant population of primitive blast‐like cells, subtle squamoid morules, frequent neuroendocrine and variable acinar phenotype, should facilitate accurate cytologic diagnosis and distinction from common mimics.
Precise color capture using custom color targets
Authors: Olejnik‐Krugly Agnieszka , Korytkowski Przemyslaw 
Published: October 2019

AbstractWe have developed a procedure to achieve a high accuracy of color capture for digital still cameras for cultural heritage digitization. The procedure uses a standard color target and additional spectral measurements directly on the artwork. A set of complementary colors is determined thanks to an initial analysis of the digital image and a comparison with the standard color target. The developed procedure results in a digital copy that better represents the colors of the original than using only standard color targets.
Autonomic nervous system functioning in early childhood: Responses to cognitive and negatively valenced emotional challenges
Authors: Zeytinoglu Selin , Calkins Susan D. , Leerkes Esther M. 
Published: October 2019

AbstractAlthough autonomic nervous system (ANS) functioning is “context‐dependent,” few studies examined children's normative sympathetic and parasympathetic autonomic responses to distinct challenges in early childhood years. Examining children's ANS responsivity to distinct challenges is important for understanding normative autonomic responses toward everyday life stressors and identifying paradigms that effectively elicit a “stress response.” We examined children's (N = 278) sympathetic (preejection period [PEP]) and parasympathetic (respiratory sinus arrhythmia [RSA]) responses to cognitive (i.e., problem‐solving and cognitive control) and negatively valenced emotional (i.e., blocked goal and unfairness) challenges in preschool, kindergarten, and grade 1. Children, on average, demonstrated parasympathetic inhibition (RSA withdrawal) in response to all challenges but the magnitude of these responses depended on the task. Children showed sympathetic activation (PEP shortening) toward the problem‐solving task at each assessment and there was no sample‐level change in the magnitude of this response over time. Children showed greater sympathetic responsivity toward the cognitive control task over time, with evidence for a sympathetic activation response only in grade 1. Children experienced sympathetic inhibition (PEP lengthening) toward the unfairness tasks but did not experience significant sympathetic responsivity toward the blocked goal tasks. Parasympathetic responsivity to most challenges were modestly stable but there was no stability in sympathetic responsivity across time.
Extramacrochaetae promotes branch and bouton number via the sequestration of daughterless in the cytoplasm of neurons
Authors: Waddell Edward A. , Viveiros Jennifer M. , Robinson Erin L. , Sharoni Michal A. , Latcheva Nina K. , Marenda Daniel R. 
Published: October 2019

AbstractThe Class I basic helix–loop–helix (bHLH) proteins are highly conserved transcription factors that are ubiquitously expressed. A wealth of literature on Class I bHLH proteins has shown that these proteins must homodimerize or heterodimerize with tissue‐specific HLH proteins in order to bind DNA at E‐box consensus sequences to control tissue‐specific transcription. Due to its ubiquitous expression, Class I bHLH proteins are also extensively regulated posttranslationally, mostly through dimerization. Previously, we reported that in addition to its role in promoting neurogenesis, the Class I bHLH protein daughterless also functions in mature neurons to restrict axon branching and synapse number. Here, we show that part of the molecular logic that specifies how daughterless functions in neurogenesis is also conserved in neurons. We show that the Type V HLH protein extramacrochaetae (Emc) binds to and represses daughterless function by sequestering daughterless to the cytoplasm. This work provides initial insights into the mechanisms underlying the function of daughterless and Emc in neurons while providing a novel understanding of how Emc functions to restrict daughterless activity within the cell.