bluuebunny/crossref_metadata_2025_split huggingface dataset:
Mastocytosis and Mast Cells
Authors: Horny Hans‐Peter , Sotlar Karl , Reiter Andreas , Valent Peter 
Published: September 2015

Abstract

Mastocytosis is defined by abnormal accumulations of mast cells in various tissue sites. The clinical features of this multifaceted disorder originate from mast cell infiltration leading to local and systemic effects induced by numerous pharmacological mediators. As mastocytosis is an unusual disorder and presents a multiplicity of symptoms, it is an often overlooked consideration in a differential diagnosis that includes not only chronic diarrhoea, urticaria, syncope and peptic ulceration but also various haematological neoplasms. Diagnosis of mastocytosis can be confirmed only by the pathologist who should use a combined immunohistochemical and molecular approach. Recognition and subtyping of its rare, life‐threatening high‐grade disease variants including aggressive systemic mastocytosis and mast cell leukaemia is based on the thorough investigation of tissue samples (usually bone marrow) but also smear preparations of blood and bone marrow.

Key Concepts

Mastocytosis is an unusual haematopoietic disorder derived from transformed bone marrow progenitor cells.

Clinically and histologically, mastocytosis presents an extremely broad spectrum of subvariants ranging from a benign, sometimes even regressive, cutaneous disease to the progressively fatal mast cell leukaemia.

Mastocytosis is a histological diagnosis established by the pathologist and cannot be confirmed by clinical findings alone.

Most patients with systemic mastocytosis carry the activating point mutation
KIT
‐D816V.

The presence of
KIT
‐D816V enables targeting therapy using some of the recently developed tyrosine kinase inhibitors.

Although stated in the recent WHO classification system on haematopoietic neoplasms, mastocytosis should not be grouped among myeloproliferative neoplasms.

Mastocytosis rather should be considered as a distinct group of disorders among haematological tumours/neoplasms.

Mastocytosis must be separated from a variety of reactive (mast cell activation syndrome) and neoplastic (basophilic and myelomastocytic leukaemias) disease states.

In more than 80% of patients with advanced systemic mastocytosis (aggressive and leukaemic SM and SM‐AHNMD) mutations other than KIT‐D816V are detected.
Molecular Genetics of the
IPEX
Syndrome
Authors: Bin Dhuban Khalid , Piccirillo Ciriaco A 
Published: September 2015

Abstract

IPEX syndrome is a rare X‐linked syndrome caused by mutations in the
FOXP3
gene, which encodes the FOXP3 protein. FOXP3 is an essential transcription factor for the development and function of regulatory T (Treg) cells, a subset of CD4
+
T cells responsible for the maintenance of immune tolerance. IPEX presents early in life with severe generalised autoimmunity and failure to thrive. Unless promptly diagnosed and properly treated, most affected children die within the first 2 years of life. Although several immunosuppressive regimens have been used to control IPEX, hematopoietic stem cell transplantation remains the only curative option. Inspired by the clinical heterogeneity observed in IPEX patients, valuable progress has been made towards a better understanding of the complex interactions of FOXP3 and its multi‐faceted role in orchestrating the different aspects of Treg functions. Such advances will aid in the development of sensitive diagnostic tools and targeted therapeutic strategies with potential applications extending beyond IPEX.

Key Concepts

IPEX is an X‐linked primary immunodeficiency syndrome in which Treg cells are unable to control auto‐reactive immune cells

IPEX may be significantly under‐diagnosed

More than 60
FOXP3
mutations reported to cause IPEX with various degrees of severity

IPEX research has contributed significantly to our knowledge of Treg biology

Efforts are focused on understanding the specific processes affected by individual mutation in order to develop mutation‐specific therapeutic strategies as well as more generalisable strategies for modulation of Treg activity
Copper‐Catalyzed Aerobic Oxidative C(aryl)OH Bond Functionalization of Catechols with Amines Affording Benzoxazoles
Authors: Chen Xiuling , Ji Fangyan , Zhao Yalei , Liu Yanxi , Zhou Yongbo , Chen Tieqiao , Yin Shuang‐Feng 
Published: September 2015

AbstractThe first copper‐catalyzed aerobic oxidative C(aryl)OH bond functionalization of catechols is reported. Under air, at room temperature, in the presence of a copper catalyst, catechols react with amines to produce the corresponding benzoxazoles in high yields. Mechanistic studies suggest that the reaction proceeds via an intriguing competitive oxidation and a synergetic catalysis between catechol and amine.magnified image
Wheezing in tobacco farm workers in Southern Brazil
Authors: Fiori Nadia Spada , Fassa Anaclaudia Gastal , Faria Neice Muller Xavier , Meucci Rodrigo Dalke , Miranda Vanessa Iribarrem , Christiani David C. 
Published: September 2015

BackgroundTobacco workers are exposed to several respiratory occupational sensitizers.MethodsA representative cross‐sectional study was carried out on 2469 tobacco family farming growers. Gender‐stratified multivariate analyses evaluated the association between wheezing and socio‐demographic, behavioral, and occupational variables.ResultsWheezing prevalence was 11.0% with no difference between genders. Among men, age, smoking, strenuous work, pesticide use, contact with vegetable dust and dried tobacco dust, lifting sticks with tobacco leaves to the curing barns, and green tobacco sickness (GTS) were risk factors for wheezing. Among women, family history of asthma, tying hands of tobacco, strenuous work, contact with chemical disinfectants, and GTS were positively associated with wheezing. Harvesting lower tobacco leaves was a protective factor for the outcome in both genders.ConclusionsPesticides, dusts exposure, and GTS were risk factors for wheezing. The synergic effect of these factors needs to be better evaluated to improve prevention. Am. J. Ind. Med. 58:1217–1228, 2015. © 2015 Wiley Periodicals, Inc.
Undocumented status as a social determinant of occupational safety and health: The workers’ perspective
Authors: Flynn Michael A. , Eggerth Donald E. , Jacobson C. Jeffrey 
Published: September 2015

BackgroundUndocumented immigration to the United States has grown dramatically over the past 25 years. This study explores undocumented status as a social determinant of occupational health by examining its perceived consequences on workplace safety of Latino immigrants.MethodsGuided by the Theory of Work Adjustment, qualitative analysis was conducted on transcripts from focus groups and individual interviews conducted with a convenience sample of Latino immigrant workers.ResultsParticipants reported that unauthorized status negatively impacted their safety at work and resulted in a degree of alienation that exceeded the specific proscriptions of the law. Participants overwhelming used a strategy of disengagement to cope with the challenges they face as undocumented immigrants.ConclusionThis study describes the complex web of consequences resulting from undocumented status and its impact on occupational health. This study presents a framework connecting the daily work experiences of immigrants, the coping strategy of disengagement, and efforts to minimize the impact of structural violence. Am. J. Ind. Med. 58:1127–1137, 2015. © 2015 Wiley Periodicals, Inc.
Assessment of the hybridization between rhesus (Macaca mulatta) and long‐tailed macaques (M. fascicularis) based on morphological characters
Authors: Hamada Yuzuru , San Aye Mi , Malaivijitnond Suchinda 
Published: September 2015

ABSTRACTObjectivesHybridization between rhesus (Macaca mulatta) and long‐tailed (M. fascicularis) macaques has become a focal point of interest. The majority of such studies have evaluated their genetics, but not their morphological characters.Materials and MethodsWe analyzed morphological characters of eight free‐ranging populations of Indochinese rhesus and long‐tailed macaques distributed at the proposed hybrid zone (15.75–21.58° N) in comparison with one population each of Chinese and Indian‐derived rhesus macaques and three populations of Sundaic long‐tailed macaques.ResultsChinese and Indian rhesus macaques had a heavier body mass, longer crown‐rump length, shorter relative facial length and relative tail length, and a greater contrast of reddish and yellowish dorsal pelage color than the Sundaic long‐tailed macaques for which the latter three parameters could be used to visually discriminate between the two species. Although the morphological characters of Indochinese rhesus and long‐tailed macaques were intermediate between the Chinese/Indian rhesus and Sundaic long‐tailed macaques, they were more similar to their respective conspecifics. The species‐specific characters of a shorter tail (<70%) and a bipartite pelage color pattern were retained in the Indochinese rhesus macaques while the longer tail (>90%) and no bipartite pattern was found in the Indochinese long‐tailed macaques. No morphological cline was observed across the species and the variations were abrupt to some extent.DiscussionThe hybridization between rhesus and long‐tailed macaques may be results of multiple contacts and isolations over a long period of time, thus their evolutionary history should not be drawn solely by genetic or morphological analysis. Am J Phys Anthropol 159:189–198, 2016. © 2015 Wiley Periodicals, Inc.
A physiologically based pharmacokinetic modeling approach to predict drug–drug interactions between domperidone and inhibitors of CYP3A4
Authors: Templeton Ian , Ravenstijn Paulien , Sensenhauser Carlo , Snoeys Jan 
Published: September 2015

AbstractDomperidone is a dopamine receptor antagonist and a substrate of CYP3A4, hence there is a potential for CYP3A inhibition‐based drug–drug interactions (DDI). A physiologically based pharmacokinetic model was developed to describe DDIs between domperidone and three different inhibitors of CYP3A4. Simcyp V13.1 was used to simulate human domperidone pharmacokinetics and DDIs. Inputs included domperidone chemical and physical properties (LogP, pKa, etc.), in vitro human liver microsomal data and pharmacokinetic parameters from single‐dose intravenous clinical studies in healthy participants. The simulated mean maximum domperidone plasma concentration and AUC after single‐ and multiple‐oral doses under diverse conditions were within 1.1–1.4 fold of the observed values. The simulated intestinal availability, hepatic availability and the fraction absorbed were 0.45 ± 0.14, 0.31 ± 0.10 and 0.89 ± 0.11, respectively, and comparable to observed in vivo values. The simulated ratios of AUC and Cmax in the presence of ketoconazole, erythromycin or itraconazole to baseline were consistent with the observed ratios. Simulated ketoconazole, erythromycin, itraconazole and Cmax,ss and AUCss were within 1.5‐fold of the observed values. Copyright © 2016 John Wiley & Sons, Ltd.
Synaptic input changes to spinal cord motoneurons correlate with motor control impairments in a type 1 diabetes mellitus model
Authors: Benitez Suzana Ulian , Carneiro Everardo Magalhães , de Oliveira Alexandre Leite Rodrigues 
Published: September 2015

AbstractIntroductionHyperglycemia is the main cause of diabetic complications, contributing to a widespread degeneration of the nervous system. Nevertheless, the main focus has been the sensory neurons because of neuropathic pain, while the impairments associated with the spinal cord and motor deficits, mostly of those initiated at early stages of the disease, have been poorly investigated. In this way, the present study used the nonobese diabetic mouse model to evaluate the microenvironment around motoneurons at ventral horn of the spinal cord, following prolonged hyperglycemia.MethodsAdult female mice were divided into two groups: spontaneously diabetic (n = 33) and nondiabetic (n = 26). Mice were considered hyperglycemic when blood glucose surpassed 400 mg/dL. Following 2 weeks from that stage, part of the animals was euthanized and the lumbar intumescences were obtained and processed for immunohistochemistry and transmission electron microscopy. For immunohistochemistry, the antibodies used for integrated density of pixels quantification were anti‐synaptophysin, anti‐GFAP, and anti‐Iba1. The functional analysis was monitored with the walking track test (CatWalk system) during 4 weeks.ResultsThe results revealed significant motor impairment in diabetic animals in comparison to the control group. Such loss of motor control correlated with a significant reduction in presynaptic terminals apposed to the motoneurons. Nevertheless, there were no significant changes in glial reaction in the spinal cord.ConclusionOverall, the results herein revealed central nervous system changes at early stages of the disease that may in turn contribute to the motor deficit. Such changes open a new window of investigation in early stages of diabetes to better comprehend motor impairment as a long‐term complication of the disease.
Potent and Selective Modulation of the RhlR Quorum Sensing Receptor by Using Non‐native Ligands: An Emerging Target for Virulence Control in Pseudomonas aeruginosa
Authors: Eibergen Nora R. , Moore Joseph D. , Mattmann Margrith E. , Blackwell Helen E. 
Published: September 2015

AbstractPseudomonas aeruginosa uses N‐acylated l‐homoserine lactone signals and a triumvirate of LuxR‐type receptor proteins—LasR, RhlR, and QscR—for quorum sensing (QS). Each of these receptors can contribute to QS activation or repression and, thereby, the control of myriad virulence phenotypes in this pathogen. LasR has traditionally been considered to be at the top of the QS receptor hierarchy in P. aeruginosa; however, recent reports suggest that RhlR plays a more prominent role in infection than originally predicted, in some circumstances superseding that of LasR. Herein, we report the characterization of a set of synthetic, small‐molecule agonists and antagonists of RhlR. Using E. coli reporter strains, we demonstrated that many of these compounds can selectively activate or inhibit RhlR instead of LasR and QscR. Moreover, several molecules maintain their activities in P. aeruginosa at concentrations analogous to native RhlR signal levels. These compounds represent useful chemical probes to study the role of RhlR in the complex QS circuitry of P. aeruginosa, its direct (and indirect) effects on virulence, and its overall merit as a target for anti‐infective therapy.
Highly Efficient Asymmetric Aziridination of Unsaturated Aldehydes Promoted by Chiral Hetero‐organic Catalysts
Authors: Rachwalski Michał , Wujkowska Zuzanna , Leśniak Stanisław , Kiełbasiński Piotr 
Published: September 2015

AbstractOptically pure hetero‐organic catalysts that bear a hydroxyl moiety, a stereogenic sulfinyl group, and a chiral amine moiety, are highly efficient in the asymmetric aziridination of unsaturated aldehydes and lead to the desired chiral products in high yields (up to 93 %) with enantiomeric excess values up to 92 %. The influence of the stereogenic centers at the sulfinyl sulfur atom and in the amine moiety on the stereochemical outcome of the title reaction is discussed.
Efficiency Factors in Photocatalytic Reactors: Quantum Yield and Photochemical Thermodynamic Efficiency Factor
Authors: Lasa Hugo de , Rosales Benito Serrano , Moreira Jesus , Valades‐Pelayo Patricio 
Published: September 2015

AbstractPhotocatalytic efficiency is evaluated using quantum yields (QYs) and the photochemical thermodynamic efficiency factor (PTEF). The PTEF allows establishing reactor efficiency as the ratio of utilized enthalpy for the formation of consumed OH• free radicals over the absorbed photon energy. A key consideration for the evaluation of efficiency factors is the establishment of macroscopic energy balances together with an accurate assessment of evolved and absorbed photons. Of considerable help are the experimental devices developed at the Chemical Reactor Engineering Centre (CREC)/University of Western Ontario (UWO) laboratories. Photoconversion kinetics is required for calculation of the OH• consumption rates and establishment of the related kinetic parameters. PTEFs and QYs have been applied by CREC‐UWO researchers for efficiency calculations in photocatalytic reactors for the decontamination of air, water, and hydrogen production.
High‐Power Supercapacitive Properties of Graphene Oxide Hybrid Films with Highly Conductive Molybdenum Disulfide Nanosheets
Authors: Byun Segi , Sim Dong Min , Yu Jin , Yoo Jung Joon 
Published: September 2015

AbstractBy introducing a graphene oxide (GO) sheet as a phase stabilizer and a mechanical supporter, MoS2/GO hybrid films consisting of a high concentration of distorted 1T‐MoS2 are successfully fabricated through a post‐annealing process in air with the form of a restacked film. After the annealing process, the MoS2 nanosheets undergo direct binding with the oxygen functional groups of GO, maintaining their highly conducting 1T phase and leading to distorted 1T‐MoS2 or 1T′‐MoS2. A hybrid film‐based supercapacitor reveals significantly enhanced electrochemical properties, such as an ultrahigh volumetric power density (≈207 W cm−3), high volumetric capacitance (≈380 F cm−3 at 10 mV s−1), superior rate capability, and a semi‐permanent cycle life (95 % retention after 10 000 cycles) in an aqueous electrolyte.
Selective Sensing of Fe3+ and Al3+ Ions and Detection of 2,4,6‐Trinitrophenol by a Water‐Stable Terbium‐Based Metal–Organic Framework
Authors: Cao Li‐Hui , Shi Fang , Zhang Wen‐Min , Zang Shuang‐Quan , Mak Thomas C. W. 
Published: September 2015

AbstractA water‐stable luminescent terbium‐based metal–organic framework (MOF), {[Tb(L1)1.5(H2O)]⋅3 H2O}n (Tb‐MOF), with rod‐shaped secondary building units (SBUs) and honeycomb‐type tubular channels has been synthesized and structurally characterized by single‐crystal X‐ray diffraction. The high green emission intensity and the microporous nature of the Tb‐MOF indicate that it can potentially be used as a luminescent sensor. In this work, we show that Tb‐MOF can selectively sense Fe3+ and Al3+ ions from mixed metal ions in water through different detection mechanisms. In addition, it also exhibits high sensitivity for 2,4,6‐trinitrophenol (TNP) in the presence of other nitro aromatic compounds in aqueous solution by luminescence quenching experiments.
A meta‐analytic review of the Bethesda System for Reporting Thyroid Cytopathology: Has the rate of malignancy in indeterminate lesions been underestimated?
Authors: Straccia Patrizia , Rossi Esther Diana , Bizzarro Tommaso , Brunelli Chiara , Cianfrini Federica , Damiani Domenico , Fadda Guido 
Published: September 2015

BACKGROUNDThe Bethesda System for Reporting Thyroid Cytopathology (TBSRTC) provides a 6‐tier diagnostic framework using uniform criteria in reports of thyroid aspirates. One of the major advantages of this framework is its association with defined risks of malignancy, allowing standardized management algorithms for each diagnosis. The objective of the current meta‐analysis was to demonstrate the feasibility of using TBSRTC among specimens in the atypia of undetermined significance or follicular lesion of undetermined significance (AUS/FLUS) and follicular neoplasm or suspicious for neoplasm (FN/SFN) categories. The authors also evaluated both the morphologic features and the risk of malignancy in the presence of Hurthle cells.METHODSA literature search was performed of the PubMed, Scopus, and Web of Science databases for English‐language studies published from January 2008 to December2014. Studies were considered eligible only if they evaluated the risk of malignancy for specimens in the AUS/FLUS and/or FN/SFN categories and included surgical follow‐up.RESULTSIn total, 51 articles were identified that used TBSRTC criteria and provided data for a total of 145,928 fine‐needle aspiration (FNA) specimens. Of these, FNAs that had surgical follow‐up were selected among the AUS/FLUS (N = 4475) and FN/SFN (N = 3202) specimens. The overall rate of malignancy was 27% for the AUS/FLUS category and 31% for the FN/SFN category.CONCLUSIONSThe AUS category was characterized by limited reported follow‐up and surgical outcome. The data demonstrated that FNAs with an AUS diagnosis had a higher risk of malignancy than the risk according to published TBSRTC criteria, whereas the percentage of malignancy in FNAs with an FN/SFN diagnosis did not differ from that according to TBSRTC. Hurthle cell lesions represent a challenging category, underlying the importance of further studies to define whether they can be diagnosed in the AUS/FLUS category rather than the FN/SFN category. Cancer (Cancer Cytopathol) 2015;123:713–722. © 2015 American Cancer Society.
Vagal Intramuscular Arrays: The Specialized Mechanoreceptor Arbors That Innervate the Smooth Muscle Layers of the Stomach Examined in the Rat
Authors: Powley Terry L. , Hudson Cherie N. , McAdams Jennifer L. , Baronowsky Elizabeth A. , Phillips Robert J. 
Published: September 2015

ABSTRACTThe fundamental roles that the stomach plays in ingestion and digestion notwithstanding, little morphological information is available on vagal intramuscular arrays (IMAs), the afferents that innervate gastric smooth muscle. To characterize IMAs better, rats were given injections of dextran biotin in the nodose ganglia, and, after tracer transport, stomach whole mounts were collected. Specimens were processed for avidin–biotin permanent labeling, and subsets of the whole mounts were immunohistochemically processed for c‐Kit or stained with cuprolinic blue. IMAs (n = 184) were digitized for morphometry and mapping. Throughout the gastric muscle wall, IMAs possessed common phenotypic features. Each IMA was generated by a parent neurite arborizing extensively, forming an array of multiple (mean = 212) branches averaging 193 µm in length. These branches paralleled, and coursed in apposition with, bundles of muscle fibers and interstitial cells of Cajal. Individual arrays averaged 4.3 mm in length and innervated volumes of muscle sheet, presumptive receptive fields, averaging 0.1 mm3. Evaluated by region and by muscle sheet, IMAs displayed architectural adaptations to the different loci. A subset (32%) of circular muscle IMAs issued specialized polymorphic collaterals to myenteric ganglia, and a subset (41%) of antral longitudinal muscle IMAs formed specialized net endings associated with the serosal boundary. IMAs were concentrated in regional patterns that correlated with the unique biomechanical adaptations of the stomach, specifically proximal stomach reservoir functions and antral emptying operations. Overall, the structural adaptations and distributions of the IMAs were consonant with the hypothesized stretch receptor roles of the afferents. J. Comp. Neurol. 524:713–737, 2016. © 2015 Wiley Periodicals, Inc.
Unique hue data and CIECAM02: A comment on Xiao et al
Authors: Hunt R. W. G. 
Published: September 2015

AbstractThis letter to the editor notes the offsets from the origin of unique hue loci in the recent article, Unique Hue Data for Colour Appearance Models. Part III: Comparison with NCS Unique Hues by Xiao et al. [Color Res Appl 2015;40:256–263] and suggests that modifications to the cone response function in CIECAM02 be explored. © 2015 Wiley Periodicals, Inc. Col Res Appl, 2015