bluuebunny/crossref_metadata_2025_split huggingface dataset:
La terapia enzimatica sostitutiva nella malattia di Fabry
Authors: Roggero Letizia , Auricchio Sara , Pieruzzi Federico 
Published: September 2019

Enzyme Replacement Therapy for Fabry Disease Anderson-Fabry disease (FD) is a X-linked lysosomal storage disorder, which involves glycosphingolipids metabolism. Specific treatment for FD has been available in the last two decades, after the development and commercialization of recombinant human alfa-galactosidase A. Since then enzyme replacement therapy (ERT) has changed the natural history of the disease. Two different enzymatic formulations are available: agalsidase alfa and agalsidase beta at different dosages. The safety and efficacy profiles are similar. ERT induces Gb3 deposits reduction in renal and cardiac biopsies, improves quality of life, reduces pain and GI symptoms, decreases left ventricular mass and slows down renal function decline. In case of organ involvement, clinical evidence confirms the need to treat all patients with enzyme therapy, both male and female. In all other clinical settings, the decision to start ERT is controversial, because of the extremely variable clinical manifestations of FD. However, data suggest a greater response to ERT if started as early as possible in any patients. Timely treatment appears to be effective in stabilizing and possibly delaying FD progression. ERT infusion reactions due to allergic hypersensitivity or IgG antibody development could occur but can be easily managed. In-hospital and at home infusions are possible. The wide genetic and phenotypic heterogeneity observed in all FD patients requires a tailored approach to treatment options. Patients should be referred to an expert multidisciplinary team for the long term management of this challenging disease.
A Puff of Smoke, a Hole in the Pocket, Fissure in the Lungs and Profit in Millions
Authors: Anand Manoj , Singh Jagandeep 
Published: September 2019

The idea that business is a medium to deliver goods and services in lieu of profits is outmoded. The triple bottom line (profit, people and planet) approach is no longer a buzzword. It is a reality and a pressing need. There is no denying the fact that ‘corporate India’ too has embraced the philosophy of ‘doing well by doing good’. All appears hunky-dory when cash rich and highly profitable firms are hailed for being socially responsible organizations. However, at times, the situation may be ironical. An organization, seen as an epitome of altruism, is engaged in the manufacturing of a legal, albeit potentially harmful product. Any organization in the cigarette industry would fit this bill to perfection. Business schools globally have revisited their course curriculum with increased focus on sustainability. Varun Chopra, on his day one of the MBA induction programme, has been confronted with a fundamental question: why do organizations exist? The answer to the complex question has to be examined through the lens of different stakeholders. Is there a simple answer as the interests of different stakeholders may be divergent? Scouting for an answer to this question in the cigarette industry draws attention to the need to juxtapose economic, social and environmental objectives.
Proactive risk assessment of vincristine use process in a teaching and referral hospital in Kenya and the implications
Authors: Kurgat Emmanuel K , Weru Irene , Wata David , Godman Brian , Kurdi Amanj , Guantai Anastasia N 
Published: September 2019

Introduction The chemotherapy use process is potentially risky for cancer patients. Vincristine, a “High Alert” medicine, has been associated with fatal but preventable medication errors. Consequently, there is a need to improve the use of vincristine especially in lower- and middle-income countries where there are constraints with resources and often a lack of trained personnel to administer cancer medicines. However, where there is a rising prevalence of cancer cases. These concerns can be addressed by performing proactive risk assessments using Healthcare Failure Mode Effect Analysis (HFMEA) and implementing the findings. Methods A multidisciplinary health team driven by pharmacists identified and evaluated potential failure modes based on a vincristine use process flow diagram using a hazard scoring matrix in a leading referral hospital in Kenya. Results The processes evaluated were: prescribing, preparation and dispensing, transportation and storage, administration and monitoring of the use of vincristine. Seventy-seven failure modes were identified over the three-month study period, of which 25 were classified as high risk. Thirteen were adequately covered by existing control measures while 12 including one combined mode required new strategies. Two of the failure modes were single-point weaknesses. Recommendations were subsequently made for improving the administration of vincristine. Conclusions HFMEA is a useful tool to identify improvements to medication safety and reduction of patient harm. The HFMEA process brings together the multidisciplinary team involved in patient care in actively identifying potential failure modes and owning the recommendations made, which are now being actively followed up in this hospital. Pharmacists are a key part of this process.
Facility and state-level factors associated with telemental health (TMH) adoption among mental health facilities in the United States
Authors: Zhao Xiaohui , Innes Kim E , Bhattacharjee Sandipan , Dwibedi Nilanjana , LeMasters Traci M , Sambamoorthi Usha 
Published: September 2019

Introduction Telemental health (TMH) is a promising approach to increase access to mental healthcare. This study examined the TMH adoption rates and associations with facility- and state-level factors among US mental health (MH) facilities. Methods This retrospective, cross-sectional study used linked data for 2016 from the National Mental Health Services Survey ( N = 11,833), Area Health Resources File, and national reports for broadband access and telehealth policies. The associations of facility and state-level characteristics with TMH adoption were examined with multi-level logistic regressions. Results Overall, 25.9% had used TMH. Having veteran affiliation [Adjusted Odds Ratio (AOR) = 18.53, 95% Confidence Interval (95%CI): 10.66–32.21] and greater Information Technology (IT) capacity [AOR(95%CI): 2.89(2.10–3.98)] were the strongest correlates of TMH adoption. Other facility characteristics associated with higher likelihood of TMH adoption were: public ownership, high patient volumes, having comprehensive MH treatments or Quality Improvement practices, having private or non-Medicaid public payers, and treating elderly patients (AORs: 1.16–2.41). TMH adoption was less likely among facilities treating more African Americans or patients with substance abuse disorders. TMH adoption varied substantially across states, with adoption more likely in states issuing special telehealth licences and those with more rural counties. Discussion One in four MH facilities adopted TMH in 2016. TMH adoption varied by multiple facility- and state-level factors. Our findings suggest that: legal/regulatory burden and lower facility IT capacity may discourage TMH adoption; significant racial disparities exist in TMH adoption; and there is a need to increase TMH use for substance abuse disorders.
Effect of a mindfulness intervention on patients admitted after multisystem trauma
Authors: Clement Elizabeth , Yoon Jenny , Sutherland Tala , Tsang Bonnie , Fawcett Vanessa , Kim Michael , Widder Sandy 
Published: September 2019

Background The incidence of depression, anxiety, and post-traumatic stress disorders is reported to be as high as 50% in trauma patients. The perpetual negative emotions and state of mind in these disorders predisposes patients to negative mental health outcomes. Mindfulness, on the other hand, helps people to process their experience and emotions in a non-judgmental manner, and recently, there has been increased utilization of mindfulness-based therapies for the treatment of mental health conditions. This proof-of-concept study evaluates the use of a mindfulness-based online application in patients admitted to the trauma service at a Level 1 Trauma Centre. Methods Trauma patients who were English speaking, over the age of 18, and without brain injury or pre-existing neurocognitive disorder were included. Participants completed the Depression Anxiety Stress Scale (DASS)-21 to assess level of depression, anxiety, and stress, and the Connor-Davidson Resilience Scale (CD-RISC) to assess level of resiliency. Then, after 28 consecutive days of practicing mindfulness using the app ‘Stop, Breathe, and Think,’ the questionnaires were repeated and an exit survey conducted. Results For this study, 13 participants were enrolled, 2 withdrew, and 5 were lost to follow-up. The mean DASS-21 score at time enrollment was 16.4 and was 11.2 at follow-up ( p = 0.10). There were no differences between the level of depression and stress from enrollment to follow-up, but there was significant decrease in anxiety symptoms from 7.2 to 3.0 (<0.05). CD-RISC scores at enrollment and follow-up were 77.8 and 81 ( p = 0.23), respectively. At the time of exit interview, 67% of patients continued to use the application three to four times a week and 67% responded they plan to continue using the application. In addition, 83% of patients always or often felt better after practicing mindfulness and stated they would recommend the application to others. Conclusions Mindfulness shows promising potential to decrease psychological distress in trauma patients.
Detection of additional abnormalities or co-morbidities in women with suspected intrahepatic cholestasis of pregnancy
Authors: Conti-Ramsden Frances , McEwan Michael , Hill Rachel , Wade Julie , Abraham Georgina , Buckeldee Olivia , Williamson Catherine , Knight Caroline L , Girling Joanna , Chappell Lucy C 
Published: September 2019

Background Current guidelines recommend viral, autoimmune, coagulation and liver ultrasound testing in intrahepatic cholestasis of pregnancy to exclude alternative diagnoses. Methods Electronic health records were searched for investigations and diagnoses in women with raised bile acid concentrations (>10 µmol/L) between January 2016 and December 2017 at two UK maternity units. Results Five hundred and thirty-one women had a raised bile acid concentration (median (IQR): 18 (13–32 µmol/L)) at a median gestation of 35.1 (IQR 31.8–37.0) weeks. Out of 531 women, 250 (47.1%) had full virology, autoimmune and ultrasound tests, and 348 (65.5%) had coagulation performed. Positive hepatitis B and C results were previously known. No new Epstein–Barr virus, cytomegalovirus or hepatitis A diagnoses were made. There were 11 positive autoimmune results, but no new diagnoses. No woman had an unexplained prolonged prothrombin time. No ultrasound liver ( n = 38) or gallbladder ( n = 85) abnormalities were of acute clinical significance. Conclusion Intrahepatic cholestasis of pregnancy investigations provided no new diagnoses that influenced clinical management during pregnancy.
Ghrelin-Induced Sodium Reabsorption Is Mediated by PKA and Microtubule-Dependent αENaC Translocation in Female Rats
Authors: Kemp Brandon A , Howell Nancy L , Gildea John J , Padia Shetal H 
Published: September 2019

Abstract
Intrarenal ghrelin infusion activates ghrelin receptors in the kidney collecting duct (CD) to increase α epithelial sodium (Na+) channel (αENaC)-dependent Na+ reabsorption in vivo, but the underlying mechanisms are unknown. Seventy-two hours following uninephrectomy, 12-week-old female Sprague-Dawley rats received the following renal interstitial (RI) infusions for 1 hour after a 1-hour control: vehicle (n = 10), ghrelin (3 μg/minute; n = 8), ghrelin + phosphatidylinositol 3-kinase (PI3K) inhibitor LY-294002 (0.1 μg/kg/minute; n = 7), ghrelin + protein kinase A (PKA) inhibitor adenosine 3′5′-cyclic monophosphorothioate, Rp-isomer (10 μg/kg/minute; n = 8), ghrelin + microtubule polymerization inhibitor nocodazole (0.3 μg/kg/minute; n = 7), or ghrelin + actin polymerization inhibitor cytochalasin D (0.3 μg/kg/minute; n = 6). Compared with vehicle infusion, RI ghrelin induced a significant anti-natriuresis (urine Na+ excretion was reduced by 53.7% ± 6.8%; P < 0.001). This effect was abolished during concomitant PKA or microtubule inhibition (106.4% ± 9.4% and 109.7% ± 10.6% of vehicle infusion, respectively; P < 0.01 from ghrelin) but not during concomitant PI3K or actin inhibition (reduced by 48.6% ± 3.9% and 52.8% ± 12.7%, respectively; P < 0.001 and P < 0.01 from vehicle, respectively; P = not significant from ghrelin). Infusions had no effect on mean arterial pressure. Western blot analysis demonstrated that CD membrane but not total αENaC expression increased in response to ghrelin infusion compared with vehicle, (0.39 ± 0.05 vs 0.12 ± 0.02 arbitrary units; P < 0.01). This effect was abolished during PKA or microtubule inhibition but persisted during PI3K or actin inhibition. Neural precursor cell expressed, developmentally down-regulated 4 isoform 2 (Nedd4-2) dependent internalization of αENaC was not affected by ghrelin, indicating that microtubule-dependent forward trafficking of αENaC is necessary for anti-natriuretic responses to ghrelin. Taken together, these studies highlight the importance of PKA and microtubule polymerization in ghrelin-induced αENaC-mediated Na+ reabsorption.
Case Report: A case of PAI-1 4G/5G heterozygosity causing Budd-Chiari Syndrome
Authors: Sousa Domingos , Antunes Silva Sergio , Jorge Catarina , Martins Fernandes Rita , Rodrigues Ana Isabel , Viana Coelho Margarida , Guimarães Joana Filipa , Osorio Rui Marques , Morais Juvenal , Ríos Elena , Figueiredo Armindo 
Published: September 2019

Budd-Chiari syndrome (BCS) is a hepatic venous outﬂow obstruction. A 36-year old Caucasian female was admitted with symptomatic hypoglycaemia. Lab tests revealed mild leucocytosis, thrombocytopenia and hepatic cytolysis. The abdominal ultrasound showed mild hepatomegaly due to hypertrophy of the left and caudate lobes, no blood flow on the right and medium hepatic veins and multiple intra-hepatic collateral vessels. Upper endoscopy showed grade I varicose veins. Further studies ruled out common prothrombotic disorders but identified an inherited thrombophilia: a plasminogen activator inhibitor 1 (PAI-1) 4G/5G heterozygous polymorphism. On presentation, this patient had signs of cirrhosis and secondary portal hypertension from imaging results at the time of diagnosis but no symptoms. Four years after the diagnosis the patient continues asymptomatic, which is very unusual. This patient's outcome will be favourable as long as their cirrhosis is compensated by the collateral vessels' permeability. Our case highlights a new association between primary BCS secondary to a prothrombotic inherit mutation: the PAI-1 4G/5G polymorphism.
Image – écrit – traduction
Authors: Bajda Justyna 
Published: September 2019

Painting – Text – Translations. Representation of the Colour Blue in Descriptions of Paintings in Art Books
Colour is a basic category for describing a painting. At the same time, it is one of the most problematic categories for an art historian who describes a real artefact as well as a translator who only works with a description of the artwork.The aim of this article is to present the terms used to refer to the colour blue that appear in descriptions of paintings reproduced in art books. The scope of the analysis was limited to French Impressionist paintings. The collected material was examined in terms of three fundamental issues:1. The functioning of two basic terms for the colour blue in the Polish language,2. The names of pigments used by Impressionist painters, and3. Descriptions of the intensity of the sky-blue colour in the French and Polish languages.
Cecal Bascule as a Rare Presentation of Cytomegalovirus Colitis in a Kidney Transplant Recipient
Authors: Shim Hong Gi , Huh Alex , Dickstein Aaron 
Published: September 2019

ABSTRACT
Cecal bascule is a rare type of volvulus of the colon and requires a mobile cecum and ascending colon, which could be due to congenital or acquired anatomic abnormalities. Inflammatory conditions that cause acute changes in colonic mobility or motility may contribute to development of volvulus, as described in other types of colonic obstruction. Patients with risk factors for a mobile proximal colon presenting with obstructive symptoms should undergo prompt diagnostic evaluation for volvulus to allow for timely intervention. We report an unusual case of invasive cytomegalovirus colitis presenting as cecal bascule in a kidney transplant recipient.
Syntheses, structures, and properties of zinc(II) and copper(II) coordination polymers with imidazole-containing ligands
Authors: Tang Long , Guo Ye , Xue Shili , Wang Jijiang , Hou Xiangyang , Wang Xiao 
Published: September 2019

Abstract
The reactions of Zn(NO3)2 · 6H2O/CuCl2 · 2H2O and 1,4-bis(1-imidazolyl)benzene (bib)/1,4-bis(1-imidazol-1-ylmethyl)benzene (biyb), carried out under hydrothermal conditions, produced two new coordination polymers [Zn(bib)(HCOO)2 · H2O] (1) and [Cu(biyb)Cl2] (2), respectively, which were characterized by elemental analysis, infrared spectroscopy, thermogravimetric analyses, and single-crystal X-ray diffraction. In complex 1, the ZnII centers are bridged by bib ligands to form a chain, where the adjacent chains are condensed by strong intermolecular O–H · · · O hydrogen-bonding and aromatic π-π stacking interactions to generate a 3D supramolecular structure. In 2, the CuII centers are bridged by biyb ligands to form, again, chains through C–H · · · Cl hydrogen bonds, and these adjacent chains with different orientations are joined to generate a 3D supramolecular structure.
Significant Neuroanatomical Variation Among Domestic Dog Breeds
Authors: Hecht Erin E. , Smaers Jeroen B. , Dunn William D. , Kent Marc , Preuss Todd M. , Gutman David A. 
Published: September 2019

Humans have bred different lineages of domestic dogs for different tasks such as hunting, herding, guarding, or companionship. These behavioral differences must be the result of underlying neural differences, but surprisingly, this topic has gone largely unexplored. The current study examined whether and how selective breeding by humans has altered the gross organization of the brain in dogs. We assessed regional volumetric variation in MRI studies of 62 male and female dogs of 33 breeds. Neuroanatomical variation is plainly visible across breeds. This variation is distributed nonrandomly across the brain. A whole-brain, data-driven independent components analysis established that specific regional subnetworks covary significantly with each other. Variation in these networks is not simply the result of variation in total brain size, total body size, or skull shape. Furthermore, the anatomy of these networks correlates significantly with different behavioral specialization(s) such as sight hunting, scent hunting, guarding, and companionship. Importantly, a phylogenetic analysis revealed that most change has occurred in the terminal branches of the dog phylogenetic tree, indicating strong, recent selection in individual breeds. Together, these results establish that brain anatomy varies significantly in dogs, likely due to human-applied selection for behavior.SIGNIFICANCE STATEMENTDog breeds are known to vary in cognition, temperament, and behavior, but the neural origins of this variation are unknown. In an MRI-based analysis, we found that brain anatomy covaries significantly with behavioral specializations such as sight hunting, scent hunting, guarding, and companionship. Neuroanatomical variation is not simply driven by brain size, body size, or skull shape, and is focused in specific networks of regions. Nearly all of the identified variation occurs in the terminal branches of the dog phylogenetic tree, indicating strong, recent selection in individual breeds. These results indicate that through selective breeding, humans have significantly altered the brains of different lineages of domestic dogs in different ways.
Epigenetic Regulation of HippocampalFosbExpression Controls Behavioral Responses to Cocaine
Authors: Gajewski Paula A. , Eagle Andrew L. , Williams Elizabeth S. , Manning Claire E. , Lynch Haley , McCornack Colin , Maze Ian , Heller Elizabeth A. , Robison A.J. 
Published: September 2019

Drug addiction results in part from maladaptive learning, including the formation of strong associations between the drug and the circumstances of consumption. However, drug-induced changes in gene expression underlying the saliency of these associations remain understudied. Consolidation of explicit memories occurs within the hippocampus, and we have shown that spatial learning induces expression of the transcription factor ΔFosB in hippocampus and that this induction is critical for learning. Drugs of abuse also upregulate ΔFosB in hippocampus, but the mechanism of its induction by cocaine and its role in hippocampus-dependent cocaine responses is unknown. We investigated differences in mouse dorsal and ventral hippocampal ΔFosB expression in response to chronic cocaine, because these regions appear to regulate distinct cocaine-related behaviors. We found that cocaine-mediated induction of ΔFosB was subregion-specific, and that ΔFosB transcriptional activity in both the dorsal and ventral hippocampus is necessary for cocaine conditioned place preference. Further, we characterize changes in histone modifications at theFosBpromoter in hippocampus in response to chronic cocaine and found that locus-specific epigenetic modification is essential forFosBinduction and multiple hippocampus-dependent behaviors, including cocaine place preference. Collectively, these findings suggest that exposure to cocaine induces histone modification at the hippocampalFosBgene promoter to cause ΔFosB induction critical for cocaine-related learning.SIGNIFICANCE STATEMENTAlthough cocaine addiction is driven in part by the formation of indelible associations between the drug and the environment, paraphernalia, and circumstances of use, and although this type of associative learning is dependent upon changes in gene expression in a brain region called the hippocampus, the mechanisms by which cocaine alters hippocampal gene expression to drive formation of these associations is poorly understood. Here, we demonstrate that chronic cocaine engages locus-specific changes in the epigenetic profile of theFosBgene in the hippocampus, and that these alterations are required for cocaine-dependent gene expression and cocaine–environment associations. This work provides novel insight into addiction etiology and potential inroads for therapeutic intervention in cocaine addiction.
THE EFFECTS OF MICROENCAPSULATED L. RHAMNOSUS AND STORAGE ON BIOGENIC AMINE AMOUNT OF SUCUK
Authors: ÜNAL TURHAN Emel , ERGİNKAYA Zerrin , BENLİ Hakan , Akın Mutlu Buket , AĞÇAM Erdal 
Published: September 2019

In this study, the effect of
microencapsulated L. rhamnosus was investigated on biogenic amine
amounts in sucuk during the 6 months storage. Traditional and probiotic sucuk
production were occurred with L. plantarum and L. rhamnosus.
Probiotic sucuk was produced with free or microencapsulated L. rhamnosus.
Microencapsulation protected survival of L. rhamnosus in sucuk. It was
observed that biogenic amine amounts of sucuk increased with storage.
Putrescine, cadaverine, histamine, tyramine and tryptamine were detected as the
most abundant biogenic amines in sucuk.
It was also determined that addition of L. rhamnosus plus L.
plantarum were reduced effectively biogenic amine amounts in sucuk
samples. Total biogenic amine amounts in
sucuk without probiotic L. rhamnosus was found higher than in sucuk with
probiotic L. rhamnosus. In conclusion, short storage period and the use
of probiotic culture are recommended for the production of sucuk with low
biogenic amine level.