bluuebunny/crossref_metadata_2025_split huggingface dataset:
Use of Aromatase Inhibitors in Large Cell Calcifying Sertoli Cell Tumors: Effects on Gynecomastia, Growth Velocity, and Bone Age
Authors: Crocker Melissa K. , Gourgari Evgenia , Lodish Maya , Stratakis Constantine A. 
Published: September 2014

Context:
Large cell calcifying Sertoli cell tumors (LCCSCT) present in isolation or, especially in children, in association with Carney Complex (CNC) or Peutz-Jeghers Syndrome (PJS). These tumors overexpress aromatase (CYP19A1), which leads to increased conversion of delta-4-androstenedione to estrone and testosterone to estradiol. Prepubertal boys may present with growth acceleration, advanced bone age, and gynecomastia.

Objective:
To investigate the outcomes of aromatase inhibitor therapy (AIT) in prepubertal boys with LCCSCTs.

Design:
Case series of a very rare tumor and chart review of cases treated at other institutions.

Setting:
Tertiary care and referral center.

Patients:
Six boys, five with PJS and one with CNC, were referred to the National Institutes of Health for treatment of LCCSCT. All patients had gynecomastia, testicular enlargement, and advanced bone ages, and were being treated by their referring physicians with AIT.

Interventions:
Patients were treated for a total of 6–60 months on AIT.

Main Outcome Measures:
Height, breast tissue mass, and testicular size were all followed; physical examination, scrotal ultrasounds, and bone ages were obtained, and hormonal concentrations and tumor markers were measured.

Results:
Tumor markers were negative. All patients had decreases in breast tissue while on therapy. Height percentiles declined, and predicted adult height moved closer to midparental height as bone age advancement slowed. Testicular enlargement stabilized until entry into central puberty. Only one patient required unilateral orchiectomy.

Conclusions:
Patients with LCCSCT benefit from AIT with reduction and/or elimination of gynecomastia and slowing of linear growth and bone age advancement. Further study of long-term outcomes and safety monitoring are needed but these preliminary data suggest that mammoplasty and/or orchiectomy may be foregone in light of the availability of medical therapy.
Quantification of HDL Particle Concentration by Calibrated Ion Mobility Analysis
Authors: Hutchins Patrick M , Ronsein Graziella E , Monette Jeffrey S , Pamir Nathalie , Wimberger Jake , He Yi , Anantharamaiah G M , Kim Daniel Seung , Ranchalis Jane E , Jarvik Gail P , Vaisar Tomas , Heinecke Jay W 
Published: September 2014

AbstractBACKGROUNDIt is critical to develop new metrics to determine whether HDL is cardioprotective in humans. One promising approach is HDL particle concentration (HDL-P), the size and concentration of HDL in plasma. However, the 2 methods currently used to determine HDL-P yield concentrations that differ &gt;5-fold. We therefore developed and validated an improved approach to quantify HDL-P, termed calibrated ion mobility analysis (calibrated IMA).METHODSHDL was isolated from plasma by ultracentrifugation, introduced into the gas phase with electrospray ionization, separated by size, and quantified by particle counting. We used a calibration curve constructed with purified proteins to correct for the ionization efficiency of HDL particles.RESULTSThe concentrations of gold nanoparticles and reconstituted HDLs measured by calibrated IMA were indistinguishable from concentrations determined by orthogonal methods. In plasma of control (n = 40) and cerebrovascular disease (n = 40) participants, 3 subspecies of HDL were reproducibility measured, with an estimated total HDL-P of 13.4 (2.4) μmol/L. HDL-C accounted for 48% of the variance in HDL-P. HDL-P was significantly lower in participants with cerebrovascular disease (P = 0.002), and this difference remained significant after adjustment for HDL cholesterol concentrations (P = 0.02).CONCLUSIONSCalibrated IMA accurately determined the concentration of gold nanoparticles and synthetic HDL, strongly suggesting that the method could accurately quantify HDL particle concentration. The estimated stoichiometry of apolipoprotein A-I determined by calibrated IMA was 3–4 per HDL particle, in agreement with current structural models. Furthermore, HDL-P was associated with cardiovascular disease status in a clinical population independently of HDL cholesterol.
YKL-40 and Alcoholic Liver and Pancreas Damage and Disease in 86258 Individuals from the General Population: Cohort and Mendelian Randomization Studies
Authors: Kjaergaard Alisa D , Bojesen Stig E , Nordestgaard Børge G , Johansen Julia S 
Published: September 2014

Abstract

BACKGROUND
We tested the hypothesis that observationally and genetically increased YKL-40 concentrations are associated with alcoholic liver and pancreas damage and disease.

METHODS
We performed cohort and mendelian randomization in 86 258 individuals from the Danish general population, with measured concentrations of plasma YKL-40 (n = 21 646) and CHI3L1 rs4950928 genotype (n = 84 738).

RESULTS
Increased YKL-40 was associated with increased alanine aminotransferase, bilirubin, alkaline phosphatase, γ-glutamyl transferase, erythrocyte mean corpuscular volume, C-reactive protein, and fibrinogen and with decreased albumin; coagulation factors II, VII, and X; and pancreatic amylase. The multifactorially adjusted hazard ratio for alcoholic liver cirrhosis comparing the 96%–100% vs 0%–33% YKL-40 percentile categories was 41 (95% CI 14–118). Corresponding ratios were 7.9 (5.1–12) for any alcoholic liver disease, 4.1 (1.7–10) for alcoholic pancreatitis, and 3.4 (1.9–6.1) for any pancreatitis. CHI3L1 rs4950928 genotype explained 14% of the variation in plasma YKL-40 concentrations but was not associated with alcoholic liver and pancreas damage or disease. A doubling in YKL-40 concentrations was associated with a multifactorially adjusted observational hazard ratio of 2.8 (2.4–3.3) for alcoholic liver cirrhosis and a corresponding genetic odds ratio of 1.1 (0.7–1.5). Corresponding risk estimates were 2.0 (1.8–2.2) observationally and 1.0 (0.8–1.1) genetically for any alcoholic liver disease, 1.4 (1.1–1.9) observationally and 1.1 (0.8–1.5) genetically for alcoholic pancreatitis, and 1.3 (1.1–1.6) observationally and 1.0 (0.8–1.3) genetically for any pancreatitis. Excessive alcohol consumption combined with YKL-40 concentrations in the top 5% was associated with 10-year risk of alcoholic liver cirrhosis of up to 7% in ever-smokers and 2% in never-smokers.

CONCLUSIONS
YKL-40 concentration within the top 5% was a marker for alcoholic liver cirrhosis, with no evidence to support a causal relationship.
DNA-bound metal ions: recent developments
Authors: Morris Daniel L. 
Published: September 2014

AbstractThe affinity of metal ions for DNA is logical considering that the structure of DNA includes a phosphate backbone with a net-negative charge, a deoxyribose sugar with O atoms, and purine and pyrimidine bases that contain O and N atoms. DNA-metal ion interactions encompass a large area of research that ranges from the most fundamental characterization of DNA-metal ion binding to the role of DNA-bound metal ions in disease and human health. Alternative DNA base pairing mediated by metal binding is also being investigated and manipulated for applications in logic gates, molecular machines, and nanotechnology. This review highlights recent work aimed at understanding interactions of redox-active metal ions with DNA that provides a better understanding of the mechanisms by which various types of oxidative DNA damage (strand breakage and base modifications) occur. Antioxidants that mitigate oxidative DNA damage by coordinating metal ions that produce reactive oxygen species are addressed, as well as recent work on the effect of DNA-metal ion interactions and the efficacy of quinolone-based antibacterial drugs. Recent advances in metal-mediated base pairing that triggers conformational changes in DNA structure for use as selective metal ion sensors and novel nanotechnology applications are also included.
Recent trends in synthesis of five- and six-membered heterocycles using dimethyl N-cyanodithioiminocarbonate
Authors: Elgemeie Galal H. , Mohamed Reham A. 
Published: September 2014

Abstract
New approaches to synthesis of five- and six-membered heterocyclic compounds utilizing dimethyl N-cyanodithioiminocarbonate are surveyed. The scope and limitation of the most important approaches are discussed.
A Load Frequency Control in an Off-Grid Sustainable Power System Based on a Parameter Adaptive PID-Type Fuzzy Controller
Authors: Ronilaya Ferdian , Miyauchi Hajime 
Published: September 2014

Abstract
This paper presents a new implementation of a parameter adaptive PID-type fuzzy controller (PAPIDfc) for a grid-supporting inverter of battery to alleviate frequency fluctuations in a wind-diesel power system. A variable speed wind turbine that drives a permanent magnet synchronous generator is assumed for demonstrations. The PAPIDfc controller is built from a set of control rules that adopts the droop method and uses only locally measurable frequency signal. The output control signal is determined from the knowledge base and the fuzzy inference. The input-derivative gain and the output-integral gain of the PAPIDfc are tuned online. To ensure safe battery operating limits, we also propose a protection scheme called intelligent battery protection (IBP). Several simulation experiments are performed by using MATLAB®/SimPowersystems™. Next, to verify the scheme’s effectiveness, the simulation results are compared with the results of conventional controllers. The results demonstrate the effectiveness of the PAPIDfc scheme to control a grid-supporting inverter of the battery in the reduction of frequency fluctuations.
Sleep-related behaviors in Alzheimer’s disease and dementia with Lewy bodies
Authors: Raggi Alberto , Neri Walter , Ferri Raffaele 
Published: September 2014

AbstractPrevalence studies suggest that Alzheimer’s disease (AD) and dementia with Lewy bodies (DLB) are the most common dementing illnesses in the elderly. The aim of this narrative review was to provide data on sleep-related behaviors in AD and DLB. This paper contains arguments, with a clinical approach, on both circadian rhythm changes and dissociated states of wakefulness and sleep in these two conditions.
Ack kinase regulates CTP synthase filaments during Drosophila oogenesis
Authors: Strochlic Todd I , Stavrides Kevin P , Thomas Sam V , Nicolas Emmanuelle , O'Reilly Alana M , Peterson Jeffrey R 
Published: September 2014

AbstractThe enzyme CTP synthase (CTPS) dynamically assembles into macromolecular filaments in bacteria, yeast, Drosophila, and mammalian cells, but the role of this morphological reorganization in regulating CTPS activity is controversial. During Drosophila oogenesis, CTPS filaments are transiently apparent in ovarian germline cells during a period of intense genomic endoreplication and stockpiling of ribosomal RNA. Here, we demonstrate that CTPS filaments are catalytically active and that their assembly is regulated by the non‐receptor tyrosine kinase DAck, the Drosophila homologue of mammalian Ack1 (activated cdc42‐associated kinase 1), which we find also localizes to CTPS filaments. Egg chambers from flies deficient in DAck or lacking DAck catalytic activity exhibit disrupted CTPS filament architecture and morphological defects that correlate with reduced fertility. Furthermore, ovaries from these flies exhibit reduced levels of total RNA, suggesting that DAck may regulate CTP synthase activity. These findings highlight an unexpected function for DAck and provide insight into a novel pathway for the developmental control of an essential metabolic pathway governing nucleotide biosynthesis.
Cholesterol in brain disease: sometimes determinant and frequently implicated
Authors: Martín Mauricio G , Pfrieger Frank , Dotti Carlos G 
Published: September 2014

AbstractCholesterol is essential for neuronal physiology, both during development and in the adult life: as a major component of cell membranes and precursor of steroid hormones, it contributes to the regulation of ion permeability, cell shape, cell–cell interaction, and transmembrane signaling. Consistently, hereditary diseases with mutations in cholesterol‐related genes result in impaired brain function during early life. In addition, defects in brain cholesterol metabolism may contribute to neurological syndromes, such as Alzheimer's disease (AD), Huntington's disease (HD), and Parkinson's disease (PD), and even to the cognitive deficits typical of the old age. In these cases, brain cholesterol defects may be secondary to disease‐causing elements and contribute to the functional deficits by altering synaptic functions. In the first part of this review, we will describe hereditary and non‐hereditary causes of cholesterol dyshomeostasis and the relationship to brain diseases. In the second part, we will focus on the mechanisms by which perturbation of cholesterol metabolism can affect synaptic function.
Etiologies for Seizures Around the Time of Vaccination
Authors: Verbeek Nienke E. , Jansen Floor E. , Vermeer-de Bondt Patricia E. , de Kovel Carolien G. , van Kempen Marjan J.A. , Lindhout Dick , Knoers Nine V.A.M. , van der Maas Nicoline A.T. , Brilstra Eva H. 
Published: September 2014

OBJECTIVES:This study was an assessment of the incidence, course, and etiology of epilepsy with vaccination-related seizure onset in a population-based cohort of children.METHODS:The medical data of 990 children with seizures after vaccination in the first 2 years of life, reported to the National Institute for Public Health and Environment in the Netherlands in 1997 through 2006, were reviewed. Follow-up data were obtained of children who were subsequently diagnosed with epilepsy and had had seizure onset within 24 hours after administration of an inactivated vaccine or 5 to 12 days after a live attenuated vaccine.RESULTS:Follow-up was available for 23 of 26 children (median age: 10.6 years) with epilepsy onset after vaccination. Twelve children developed epileptic encephalopathy, 8 had benign epilepsy, and 3 had encephalopathy before seizure onset. Underlying causes were identified in 15 children (65%) and included SCN1A–related Dravet syndrome (formerly severe myoclonic epilepsy of infancy) or genetic epilepsy with febrile seizures plus syndrome (n = 8 and n = 1, respectively), a protocadherin 19 mutation, a 1qter microdeletion, neuronal migration disorders (n = 2), and other monogenic familial epilepsy (n = 2).CONCLUSIONS:Our results suggest that in most cases, genetic or structural defects are the underlying cause of epilepsy with onset after vaccination, including both cases with preexistent encephalopathy or benign epilepsy with good outcome. These results have significant added value in counseling of parents of children with vaccination-related first seizures, and they might help to support public faith in vaccination programs.
Safety of Live-Attenuated Influenza Vaccination in Cystic Fibrosis
Authors: Boikos Constantina , De Serres Gaston , Lands Larry C. , Boucher François D. , Tapiéro Bruce , Daigneault Patrick , Quach Caroline 
Published: September 2014

OBJECTIVES:
Given the improved efficacy of the nasal live-attenuated influenza virus vaccine (LAIV) compared with the injectable vaccine in children, we aimed to determine its safety in individuals with cystic fibrosis (CF).

METHODS:
A cohort of 168 study participants, aged 2 to 18 years with CF, vaccinated with LAIV between October 1, 2012, and January 30, 2013, was followed prospectively for 56 days after initial vaccination in 3 pediatric CF clinics across the province of Quebec. Days 0 to 28 post-LAIV were considered the at-risk period for all outcomes of interest, and days 29 to 56 post-LAIV were considered the non–at-risk period. Incident respiratory deteriorations were defined as an unscheduled medical visit, hospitalization, or a new course of oral antibiotics for respiratory complaints. Using a self-controlled design, incidence rate ratios (IRR) were used to compare at-risk and non–at-risk periods.

RESULTS:
Comparing at-risk to non–at-risk periods, there was no significant increase in the rate of incident respiratory deteriorations (IRR, 0.72; 95% confidence interval, 0.11–4.27) or all-cause hospitalizations (IRR, 1.16; 95% confidence interval, 0.30–4.81). A greater proportion of participants reported experiencing at least 1 minor respiratory and/or systemic adverse event after immunization during the at-risk period compared with the non–at-risk period (77% vs 54%, respectively). During the first week after LAIV, 13 of 168 (8%) children reported some wheezing, with the vast majority, 9 of 13 (69%), on the day of vaccination.

CONCLUSIONS:
There was no increased risk of respiratory deterioration or all-cause hospitalization associated with LAIV in our study population. LAIV seems well tolerated in children and adolescents with CF.
Coping with Cleft: A Conceptual Framework of Caregiver Responses to Nasoalveolar Molding
Authors: Sischo Lacey , Broder Hillary L. , Phillips Ceib 
Published: September 2014

Objective To present a conceptual framework of caregiver coping and adaptation to early cleft care using nasoalveolar molding. Design In-depth interviews were conducted at three time points with caregivers of infants with cleft lip or cleft lip and palate whose children had nasoalveolar molding to treat their cleft. Qualitative data were analyzed using modified grounded theory. Results Most caregivers expressed initial apprehension and anxiety about the responsibilities of care associated with nasoalveolar molding (e.g., changing and positioning tapes, cleaning the appliance). In subsequent interviews, caregivers often reported positive feelings related to their active participation in their child's treatment for cleft. These positive feelings were associated with increased self-esteem and feelings of empowerment for the caregivers. Although caregivers also identified burdens associated with nasoalveolar molding (e.g., stress related to lip taping, concerns about the appliance causing sores in their child's mouth, travel to weekly appointments), they tended to minimize the impact of these issues in comparison with the perceived benefits of nasoalveolar molding. Conclusions Despite the increased burden of care, many caregivers of infants with cleft used nasoalveolar molding as a problem-focused coping strategy to deal with their child's cleft. Completing nasoalveolar molding was often associated with positive factors such as increased empowerment, self-esteem, and bonding with their infant.
Lung Cancer Treatment Disparities in China: A Question in Need of an Answer
Authors: Yang Lu-Lu , Zhang Xu-Chao , Yang Xue-Ning , Yang Jin-Ji , Wang Zhen , Chen Hua-Jun , Yan Hong-Hong , Xu Chong-Rui , Guan Ji-Lin , He Yan-Yan , Zhong Wen-Zhao , An She-Juan , Wu Yi-Long 
Published: September 2014

Abstract

Background.
Substantial progress has been made in the treatment of malignancies in the People's Republic of China in recent years. The goal of this study was to identify the extent to which national treatment guidelines are being used to customize patient care in lung cancer and to analyze the reasons for treatment disparities.

Methods.
Patient characteristics and treatments were investigated retrospectively for the period from October 2004 to January 2013 using the outpatient database of the Guangdong Lung Cancer Institute (GLCI) in China.

Results.
A total of 2,535 outpatients with lung cancer were studied in this retrospective analysis. The treatment disparity was 45.3%. Overall, 20.6% of patients with stage I non-small cell lung cancer (NSCLC) were overtreated, and 20.1% of stage II patients were undertreated. Only 19.6% of stage IIIA patients and 30.7% of stage IIIB patients underwent the recommended combination of chemotherapy and radiotherapy, respectively. For advanced NSCLC, the greatest treatment disparity appeared in the second-line setting and beyond. Patients who were positive for epidermal growth factor receptor (EGFR) and receiving EGFR tyrosine kinase inhibitors experienced significant prolongation of survival compared with patients who were EGFR negative or whose EGFR mutation status was unknown (hazard ratio: 0.79; p = .037). The treatment disparities were significantly larger among patients aged younger than 65 years and in patients from developing regions compared with patients aged 65 years and older and from developed regions, respectively (p &lt; .001, p = .046). The difference in treatment disparity was statistically significant between GLCI and other hospitals (p &lt; .001).

Conclusion.
This retrospective study of a large number of patients from an outpatient oncology database demonstrated large disparities in the treatment of lung cancer in China. It is important to develop a new guideline for recommendations that are based on resource classification.
Temporal dynamics of a commensal network of cavity‐nesting vertebrates: increased diversity during an insect outbreak
Authors: Cockle Kristina L. , Martin Kathy 
Published: September 2014

Network analysis offers insight into the structure and function of ecological communities, but little is known about how empirical networks change over time during perturbations. “Nest webs” are commensal networks that link secondary cavity‐nesting vertebrates (e.g., bluebirds, ducks, and squirrels, which depend on tree cavities for nesting) with the excavators (e.g., woodpeckers) that produce cavities. In central British Columbia, Canada, Northern Flicker (Colaptes auratus) is considered a keystone excavator, providing most cavities for secondary cavity‐nesters. However, roles of species in the network, and overall network architecture, are expected to vary with population fluctuations. Many excavator species increased in abundance in association with a pulse of food (adult and larval beetles) during an outbreak of mountain pine beetle (Dendroctonus ponderosae), which peaked in 2003–2004. We studied nest‐web dynamics from 1998 to 2011 to determine how network architecture changed during this resource pulse.Cavity availability increased at the onset of the beetle outbreak and peaked in 2005. During and after the outbreak, secondary cavity‐nesters increased their use of cavities made by five species of beetle‐eating excavators, and decreased their use of flicker cavities. We found low link turnover, with 74% of links conserved from year to year. Nevertheless, the network increased in evenness and diversity of interactions, and declined slightly in nestedness and niche overlap. These patterns remained evident seven years after the beetle outbreak, suggesting a legacy effect.In contrast to previous snapshot studies of nest webs, our dynamic approach reveals how the role of each cavity producer, and thus quantitative network architecture, can vary over time. The increase in interaction diversity with the beetle outbreak adds to growing evidence that insect outbreaks can increase components of biodiversity in forest ecosystems at various temporal scales. The observed changes in (quantitative) network architecture contrast with the relatively stable (qualitative) architecture of empirical mutualistic networks that have been studied to date. However, they are consistent with recent theory on the importance of population fluctuations in driving network architecture. Our results support the view that models should allow for the possibility of rewiring (species switching partners) to avoid overestimation of secondary extinction risk.