bluuebunny/crossref_metadata_2025_split huggingface dataset:
Lymphangioleiomyomatosis
Authors: Xu Kai-Feng , Xu Wenshuai , Liu Song , Yu Jane , Tian Xinlun , Yang Yanli , Wang Shao-Ting , Zhang Weihong , Feng Ruie , Zhang Tengyue 
Published: April 2020

AbstractLymphangioleiomyomatosis (LAM) is a slow albeit progressive rare neoplastic disease featured with diffuse thin-walled cysts in lungs and angiomyolipomas in kidneys. LAM affects almost exclusively women and has one of the strongest gender predispositions of any extragenital human disease. Two forms of LAM present clinically, sporadic (S-LAM) and tuberous sclerosis complex-associated (TSC-LAM). TSC is an autosomal dominant genetic multisystems neoplastic disease. A high prevalence of LAM can be detected in adult female TSC patients. Tremendous progress has been made in our understanding and management of this rare disease. Both LAM and TSC are TSC2 or TSC1 mutated diseases that result in overactivation of the mechanistic target of rapamycin (mTOR) pathway. Sirolimus, an mTOR inhibitor, has been approved for LAM treatment in the United States and many other countries. Therapies targeting female sex hormones have shown preclinical efficacy in animal and cell culture-based experiments, but have not been properly investigated clinically. In this review, we summarize current recommendations in the diagnosis and treatment of LAM.
Pulmonary Alveolar Microlithiasis
Authors: Shaw Brian M. , Shaw Steven D. , McCormack Francis X. 
Published: April 2020

AbstractPulmonary alveolar microlithiasis (PAM) is a rare, autosomal recessive disorder that is caused by mutations in SCL34A2 that encodes for the type IIb sodium-dependent phosphate cotransporter (Npt2b). The loss of Npt2b transporter function from alveolar epithelial cells results in failure to export inorganic phosphate from the alveolar lining fluid, which then accumulates, binds to calcium, and forms hydroxyapatite microliths. Radiographs and computed tomography of the chest demonstrate hyperdense infiltrates that are often quite dramatic and distinctive, and in many cases, the diagnosis can be made without invasive measures. The most common presenting symptom of PAM is dyspnea on exertion, but the disease is frequently first noted as an incidental finding in asymptomatic patients who have chest films performed for unrelated reasons. Pulmonary fibrosis, pulmonary hypertension, and respiratory failure can develop as the disease progresses, and treatment remains supportive. Lung transplantation is an option for those with end stage disease.
Successful Strategy for the Conservative Management of Acquired Tracheoesophageal Fistula Due to Lithium Button Battery Ingestion
Authors: Shibuya Soichi , Azuma Takahiro , Lane Geoffrey J. , Okawada Manabu , Yamataka Atsuyuki 
Published: April 2020

AbstractA 16-month-old boy was referred to our hospital for the management of suspected lithium button battery (LBB) ingestion. He had been previously well, but became febrile with a persistent cough resistant to oral antibiotics and dysphagia for 5 days. Radiography identified an LBB lodged in the upper esophagus. The LBB was retrieved under direct visualization with rigid laryngoscopy. He was sedated for 5 days and enteral feeding was commenced through a nasojejunal tube on the next day after procedure. On day 8 after retrieval, endoscopy and fluoroscopy identified a tracheoesophageal fistula (TEF), 6 mm in diameter. Conservative management was conducted with periodic follow-up endoscopies, which showed signs of healing in the esophagus. Following continuous antibiotics and proactive nutritional support, the TEF was found to have closed spontaneously by day 28 after the LBB removal. We present our experience of the successful nonsurgical management of acquired TEF secondary to LBB ingestion and aim to establish a protocol for managing it conservatively by reviewing the relevant literature.
Familial Interstitial Lung Disease
Authors: Kropski Jonathan A. 
Published: April 2020

AbstractThe interstitial lung diseases (ILDs) are a group of progressive disorders characterized by chronic inflammation and/or fibrosis in the lung. While some ILDs can be linked to specific environmental causes (i.e., asbestosis, silicosis), in many individuals, no culprit exposure can be identified; these patients are deemed to have “idiopathic interstitial pneumonia” (IIP). Family history is now recognized as the strongest risk factor for IIP, and IIP cases that run in families comprise a syndrome termed “familial interstitial pneumonia” (FIP). Mutations in more than 10 different genes have been implicated as responsible for disease in FIP families. Diverse ILD clinical phenotypes can be seen within a family, and available evidence suggests underlying genetic risk is the primary determinant of disease outcomes. Together, these FIP studies have provided unique insights into the pathobiology of ILDs, and brought focus on the unique issues that arise in the care of patients with FIP.
Amyloid in the Lung
Authors: Baqir Misbah , Roden Anja C. , Moua Teng 
Published: April 2020

AbstractAmyloidosis is the term given to abnormal deposition of misfolded precursor proteins at single or multiple sites, leading to organ dysfunction or clinical signs and symptoms. Pulmonary manifestations are nonspecific and may be associated with several amyloid protein subtypes, commonly AL (light chain) and AA (autoimmune) amyloids. Signs or symptoms of amyloid disease may often involve more of the clinical abnormalities of other affected organs than the lungs themselves. Radiologic pulmonary findings include septal and parenchymal ground glass or nodular infiltrates, multiple nodules, cysts, and focal tracheobronchial abnormalities. Lymphadenopathy with or without calcification and pleural effusions has also been reported. Directed therapy is initiated in response to clinical signs or symptoms often as a result of systemic or secondary diseases or conditions. Long-term prognosis is more dependent on the extent of organ involvement where morbidity is often the highest in those with multisystemic disease.
Surgical Thoracoabdominal Aortic Aneurysm Repair in a Non-High-Volume Institution
Authors: Kunihara Takashi , Vukic Claudia , Sata Fumihiro , Schäfers Hans-Jaochim 
Published: April 2020

Abstract
Background Surgical thoracoabdominal aortic aneurysm (TAAA) repair remains challenging. Apart from mortality, spinal cord injury (SCI) is a dreaded complication. We analyzed our experience to identify predictors for SCI in a nonhigh-volume institution.
Patients and Methods All patients who underwent TAAA repair between February 1996 and November 2016 (n = 182) were enrolled. Most were male (n = 121; 66.4%), median age was 68 years (range: 21–84). Elective operations were performed in 153 instances (84.1%). Our approach to minimize SCI includes distal aortic perfusion, mild hypothermia, identification of the Adamkiewicz artery, and sequential aortic clamping. Cerebrospinal fluid drainage was introduced in 2001 and liberal use of selective visceral perfusion in 2006.
Results Early mortality was 12.1%; it was 8.5% after elective procedures. Reduced left ventricular function, nonelective setting, older age, and longer bypass time were identified as independent predictors for mortality in multivariable logistic regression model. Permanent SCI was observed in nine patients (4.9%), of whom seven (3.8%) developed paraplegia. In a multivariable logistic regression model for paraplegia, peripheral arterial disease (PAD), Crawford type II repair, smaller body surface area, and era before 2001 were identified as independent predictors, whereas only PAD was significant for SCI. The incidence of paraplegia was 13.8% in extensive repair out of the first 91 cases, whereas it was improved up to 2.7% thereafter.
Conclusion Using an integrated approach, acceptable outcome of TAAA repair can be achieved, even in a nonhigh-volume center. PAD and extensive involvement of the aorta are strong independent predictors for spinal cord deficit after TAAA repair.
Nonspecific Interstitial Pneumonia
Authors: Teoh Alan K. Y. , Corte Tamera J. 
Published: April 2020

AbstractNonspecific interstitial pneumonia (NSIP) is a complex disorder commonly associated with other conditions such as connective tissue diseases (CTDs) and environmental exposures. Although idiopathic NSIP has been recognized as a separate clinical entity, recent studies have suggested that a proportion of these cases have autoimmune features suggestive of underlying CTDs. The diagnosis of NSIP usually carries a better prognosis compared with idiopathic pulmonary fibrosis but has an unpredictable natural history. Its pathogenesis is thought to be an inflammatory-driven process involving multiple pathways, including a genetic predisposition. The lack of specific clinical features often makes the diagnosis of NSIP difficult. The huge variability of radiological and histological features seen in NSIP adds to the complexity of achieving an accurate diagnosis of NSIP and a multidisciplinary approach is often required. There is a lack of consensus on the optimal management strategy of NSIP. Early clarification of the goals of therapy and close monitoring for the progression of disease is important across the spectrum of NSIP irrespective of its etiology. Although immunosuppressive and immunomodulatory agents are commonly used for severe and progressive disease, the therapeutic landscape of NSIP is constantly evolving as the role of newer agents such as antifibrotic therapies is being explored.
A Champion of Host Defense: A Generic Large-Scale Cause for Platelet Dysfunction and Depletion in Infection
Authors: Page Martin J. , Pretorius Etheresia 
Published: April 2020

AbstractThrombocytopenia is commonly associated with sepsis and infections, which in turn are characterized by a profound immune reaction to the invading pathogen. Platelets are one of the cellular entities that exert considerable immune, antibacterial, and antiviral actions, and are therefore active participants in the host response. Platelets are sensitive to surrounding inflammatory stimuli and contribute to the immune response by multiple mechanisms, including endowing the endothelium with a proinflammatory phenotype, enhancing and amplifying leukocyte recruitment and inflammation, promoting the effector functions of immune cells, and ensuring an optimal adaptive immune response. During infection, pathogens and their products influence the platelet response and can even be toxic. However, platelets are able to sense and engage bacteria and viruses to assist in their removal and destruction. Platelets greatly contribute to host defense by multiple mechanisms, including forming immune complexes and aggregates, shedding their granular content, and internalizing pathogens and subsequently being marked for removal. These processes, and the nature of platelet function in general, cause the platelet to be irreversibly consumed in the execution of its duty. An exaggerated systemic inflammatory response to infection can drive platelet dysfunction, where platelets are inappropriately activated and face immunological destruction. While thrombocytopenia may arise by condition-specific mechanisms that cause an imbalance between platelet production and removal, this review evaluates a generic large-scale mechanism for platelet depletion as a repercussion of its involvement at the nexus of responses to infection.
Comparison of the prebiotic properties of native chicory and synthetic inulins using swine fecal cultures
Authors: Nakayama Yasunori , Kawasaki Naoko , Tamiya Taiga , Anzai Sayuri , Toyohara Kiyotsuna , Nishiyama Akira , Kitazono Eiichi 
Published: April 2020

Abstract
Inulin-type fructans are known to exert different effects on the fermentation profile depending on the average and range of the degree of polymerization (DP). Here, swine fecal cultures were used to investigate the prebiotic properties of native chicory inulin (NIN), extracted from the chicory root, and synthetic inulin (SIN), which has a narrower DP distribution than NIN. Both NIN and SIN showed prebiotic effects, but NIN exhibited a significant decrease in pH and increase in the production of propionate and butyrate compared to SIN. There were also differences in the production of succinate and lactate, the precursors of propionate and butyrate, and the relative abundance of associated genes. Furthermore, NIN induced the growth of certain species of Bifidobacterium and Lactobacillus more strongly than SIN. These results suggest that NIN and SIN exhibit different prebiotic properties due to differences in DP, and that NIN might be more beneficial to host health.
Characterization of plant immunity-activating mechanism by a pyrazole derivative
Authors: Kusajima Miyuki , Fujita Moeka , Yamakawa Hiromoto , Ushiwatari Tsukasa , Mori Takamasa , Tsukamoto Kazuki , Hayashi Hiroshi , Maruyama-Nakashita Akiko , Che Fang-Sik , Nakashita Hideo 
Published: April 2020

Abstract
A newly identified chemical, 4-{3-[(3,5-dichloro-2-hydroxybenzylidene)amino]propyl}-4,5-dihydro-1H-pyrazol-5-one (BAPP) was characterized as a plant immunity activator. BAPP enhanced disease resistance in rice against rice blast disease and expression of a defense-related gene without growth inhibition. Moreover, BAPP was able to enhance disease resistance in dicotyledonous tomato and Arabidopsis plants against bacterial pathogen without growth inhibition, suggesting that BAPP could be a candidate as an effective plant activator. Analysis using Arabidopsis sid2-1 and npr1-2 mutants suggested that BAPP induced systemic acquired resistance (SAR) by stimulating between salicylic acid biosynthesis and NPR1, the SA receptor protein, in the SAR signaling pathway.
Purification and characterization of formaldehyde dismutases of Methylobacterium sp. FD1
Authors: Imoto Seiji , Yonemitsu Hiroshi , Totsui Noriki , Kishimoto Noboru 
Published: April 2020

Abstract
In the present study, we purified and characterized three formaldehyde dismutases (Fdms) (EC 1.2.98.1) (Fdm1, Fdm2, and Fdm3) of Methylobacterium sp. FD1. These Fdms (with His-tag) were produced in the recombinant E. coli and purified by immobilized metal affinity chromatography from the E. coli extracts. In each of the three Fdms, the enzyme-bound coenzyme was nicotinamide adenine dinucleotide (NAD(H)) and the enzyme-bound metal was zinc. The quaternary structures of these Fdms were estimated as homotetrameric. The optimal pHs and temperatures of Fdm1, Fdm2, and Fdm3 were approximately 6.5, 6.0, and 6.0, and 35°C, 25°C, and 30°C, respectively. The Km values of Fdm1, Fdm2, and Fdm3 were 621, 865, and 414 mM, respectively. These results were similar to the properties of already-known Fdms. However, each of the Fdms of FD1 had methanol:p-nitroso-N,N-dimethylaniline oxidoreductase activity that is not found in already-known Fdms.
Three new late-type hypervelocity star candidates from Gaia DR2 with refined selection criteria
Authors: Li Jiao , Jia Shi , Gao Yan , Jiang Deng-Kai , Kupfer Thomas , Heber Ulrich , Liu Chao , Chen Xue-Fei , Han Zhan-Wen 
Published: April 2020

Abstract
Several dozen hypervelocity star (HVS) candidates have been reported based on the second data release of Gaia (Gaia DR2). However, it has been proven that the radial velocities of some Gaia HVS candidates are not reliable. In this paper, we employ refined astrometric criteria to re-examine Gaia DR2, arriving at a more reliable sample of HVS and high velocity star candidates than those found by previous authors. We develop a method called Binary Escape Probability Analysis to identify some HVS candidates. This method allows us to work with stars having only two epochs of measured radial velocity. These stars were usually discarded in previous similar studies. A scrutiny of our final results sheds light on selection effects present in our studies, which we propose to be the focus of future studies. In total, we find three late-type (2 G-type and 1 K-type) HVS and 21 high velocity star candidates, 3 and 11 of which are new, respectively. Judging by their historical trajectories, which we calculate, all three HVS candidates could not have had Galactic center origins. Further monitoring is required to confirm their status.
Engrailed Homeobox 1 and Cytokeratin 19 Are Independent Diagnostic Markers of Eccrine Porocarcinoma and Distinguish It From Squamous Cell Carcinoma
Authors: Miura Keiko , Akashi Takumi , Namiki Takeshi , Hishima Tsunekazu , Bae Yuan , Sakurai Urara , Murano Keimei , Shiraishi Junichi , Warabi Masahiro , Tanizawa Toru , Tanaka Michio , Bhunchet Ekapot , Kumagai Jiro , Ayabe Shinya , Sekiya Takahiro , Ando Noboru , Shintaku Hiroshi , Kinowaki Yuko , Tomii Shohei , Kirimura Susumu , Kayamori Kou , Yamamoto Kurara , Ito Takashi , Eishi Yoshinobu 
Published: April 2020

AbstractObjectivesThe diagnostic utility of En1 in the histopathologic differentiation of eccrine porocarcinoma (EPC) from invasive squamous cell carcinoma (SCC) was investigated.MethodsExpression of En1 and CK19 in 16 cases of EPC was immunohistochemically examined and compared with that in 32 cases of SCC.ResultsIn all 16 EPCs, En1 was expressed in 3% to 100% of tumor cells. In 20 of the 32 SCCs, En1 was expressed in 3% to 90% of tumor cells. A total of 13 of the 16 EPCs and five of the 32 SCCs were judged as En1 positive, with a cutoff value of 25%. In addition, 11 of the 16 EPCs and four of the 32 SCCs were CK19 positive. The frequencies of En1- and CK19-positive cases were significantly higher in EPCs than in SCCs. In a logistic regression analysis for predicting EPC, En1 and CK19 were independent markers. When expression patterns of En1 and CK19 were combined, none of the 32 SCCs was both positive. In contrast, 15 of the 16 EPCs were positive for either En1 or CK19.ConclusionsA combination of En1 and CK19 expression can improve the accuracy of histologic diagnosis of EPC.
A global challenge and a legal requirement: new findings can help the Welsh response and put sustainable development into practice
Authors: Weatherup Cathy , Azam Sumina 
Published: April 2020

Abstract
Sustainable development legislation in Wales requires us to think and act differently to contribute to seven well being goals, namely; prosperity, resilience, health, equity, cohesive communities, globally responsible and a vibrant culture and thriving welsh language, now and over the longer term. Findings from a Literature Review, commissioned by the Health and Sustainability Hub, Public Health Wales, identify approaches and recommendations to help implement the legislation; aimed at people, policies and practice.
Uncovering TAPS strategies of the tobacco industry at PoS in low- and middle-income countries: the case of Turkey
Authors: Uznay Fatih , Gumus Sevtap 
Published: April 2020

Abstract

Background
This study aims to uncover strategies executed by the tobacco industry against tobacco advertising, promotion and sponsorship (TAPS) bans at points of sale (PoS) in Turkey.

Methods
The data are based on a field study conducted in the city of Izmir. There are 10 750 PoS in all of Izmir; the 6200 PoS in the 11 central districts were considered as potential subjects. Using a proportional sampling method (95% confidence interval, 5% margin of error), it was calculated that 384 PoS could adequately represent the whole. Face-to-face interviews were conducted with PoS administrators, using survey form consisting of 53 questions. Descriptive statistics methods have been used to analyze the data.

Results
Of a total of 384 PoS visited; 96.4% featured at least one type of display/TAPS ban violation, 94.5% featured display settings creating brand dominance on display units, 76.7% received promotional payments and 14.8% received free tobacco products for featured display settings. Additionally, 29.2% of PoS had tobacco product displays visible from outside, 26.8% sold tobacco products on the counter, 24.7% featured advertising items on display units, 19.3% sold tobacco products as single stick and 11.7% had price tags with fonts larger than the legally fixed 20 pt.

Conclusions
An official institution specializing in tobacco control must be established to continually inspect the tobacco market. Tobacco products must stay hidden, in closed cupboards or under the counter, and communication between PoS and tobacco industry must be limited. In addition, tobacco products must be distributed by government institutions rather than the industry.
Measles outbreak in Romania: understanding factors related to suboptimal vaccination uptake
Authors: Habersaat Katrine Bach , Pistol Adriana , Stanescu Aurora , Hewitt Catherine , Grbic Miljana , Butu Cassandra , Jackson Cath 
Published: April 2020

Abstract

Background
A large measles outbreak started in Romania in 2016. Current study aimed to (i) clarify who was affected by the outbreak, (ii) identify their barriers and drivers to vaccination and (iii) explore variation by population group.

Methods
This was a two-component study. Outbreak surveillance data for 6743 measles cases were reviewed to identify key characteristics. A survey was administered via telephone to 704 caregivers of measles cases (520 respondents) to explore capability, opportunity and motivation barriers to vaccination. Data were summarized descriptively for respondent characteristics and statements. Differences by population group (education, household income, ethnicity, setting and mobility) were explored using χ2 tests, Fisher’s exact tests or regression models.

Results
Most cases were unvaccinated and lived in low coverage areas. Ethnic minorities were disproportionally affected. Most caregivers felt welcome at health facilities. Some were less satisfied with the waiting time and had found the vaccine out of stock. Not everybody knew that vaccines were free of charge. Less than half knew the child’s next vaccination date, some had not been informed and did not know where to seek this information. Some said their peers did not vaccinate. Beliefs were generally supportive of vaccination; but many were concerned about vaccine safety and found they had not received good information about this. Conclusions varied greatly between minorities and less educated groups, compared with people with higher education levels.

Conclusions
Identifying characteristics of the population affected and underlying factors can inform a strategy to avoid future outbreaks and further research to obtain deeper insights.
BRAF and KRAS mutations in metastatic colorectal cancer: future perspectives for personalized therapy
Authors: Li Zi-Nan , Zhao Lin , Yu Li-Feng , Wei Min-Jie 
Published: April 2020

Abstract
Colorectal cancer (CRC) is one of the most commonly diagnosed cancers worldwide and 30% of patients with CRC experience metastasis. Patients with metastatic colorectal cancer (mCRC) have a 5-year overall survival rate of &lt;10%. V-raf murine sarcoma viral oncogene homolog B1 (BRAF) and V-Ki-ras2 Kirsten ratsarcoma viral oncogene homolog (KRAS) mutations are mostly studied in mCRC, as clinical trials found that first-line chemotherapy with anti-epidermal growth factor receptor agent confers limited efficacy for mCRC. Treatment decisions for early-stage mCRC do not consider BRAF or KRAS mutations, given the dramatically poor prognosis conferred by these mutations in clinical trials. Thus, it is necessary to identify patients with mCRC harboring BRAF or KRAS mutations to formulate rational therapeutic strategies to improve prognosis and survival. BRAF and KRAS mutations occur in ∼10% and ∼44% of patients with mCRC, respectively. Although the survival rate of patients with mCRC has improved in recent years, the response and prognosis of patients with the aforementioned mutations are still poor. There is a substantial unmet need for prospective personalized therapies for patients with BRAF- or KRAS-mutant mCRC. In this review, we focus on BRAF and KRAS mutations to understand the mechanisms underlying resistance and improving the response rate, outcomes, and prognosis of patients with mCRC bearing these mutations and to discuss prospective personalized therapies for BRAF- and KRAS-mutant mCRC.