bluuebunny/crossref_metadata_2025_split huggingface dataset:
Genotype–phenotype correlation in two Polish neonates with alveolar capillary dysplasia
Authors: Kozłowska Zuzanna , Owsiańska Zuzanna , Wroblewska Joanna P. , Kałużna Apolonia , Marszałek Andrzej , Singh Yogen , Mroziński Bartłomiej , Liu Qian , Karolak Justyna A. , Stankiewicz Paweł , Deutsch Gail , Szymankiewicz-Bręborowicz Marta , Szczapa Tomasz 
Published: June 2020

AbstractBackgroundAlveolar capillary dysplasia (ACD) is a rare cause of severe pulmonary hypertension and respiratory failure in neonates. The onset of ACD is usually preceded by a short asymptomatic period. The condition is refractory to all available therapies as it irreversibly affects development of the capillary bed in the lungs. The diagnosis of ACD is based on histopathological evaluation of lung biopsy or autopsy tissue or genetic testing ofFOXF1on chromosome 16q24.1. Here, we describe the first two Polish patients with ACD confirmed by histopathological and genetic examination.Case presentationThe patients were term neonates with high Apgar scores in the first minutes of life. They both were diagnosed prenatally with heart defects. Additionally, the first patient presented with omphalocele. The neonate slightly deteriorated around 12thhour of life, but underwent surgical repair of omphalocele followed by mechanical ventilation. Due to further deterioration, therapy included inhaled nitric oxide (iNO), inotropes and surfactant administration. The second patient was treated with prostaglandin E1 since birth due to suspicion of aortic coarctation (CoA). After ruling out CoA in the 3rdday of life, infusion of prostaglandin E1 was discountinued and immediately patient’s condition worsened. Subsequent treatment included re-administration of prostaglandin E1, iNO and mechanical ventilation. Both patients presented with transient improvement after application of iNO, but died despite maximized therapy. They were histopathologically diagnosed post-mortem with ACD. Array comparative genomic hybridization in patient one and patient two revealed copy-number variant (CNV) deletions, respectively, ~ 1.45 Mb in size involvingFOXF1and an ~ 0.7 Mb in size involvingFOXF1enhancer and leavingFOXF1intact.ConclusionsBoth patients presented with a distinct course of ACD, extra-pulmonary manifestations and response to medications. Surgery and ceasing of prostaglandin E1 infusion should be considered as potential causes of this variability. We further highlight the necessity of thorough genetic testing and histopathological examination and propose immunostaining for CD31 and CD34 to facilitate the diagnostic process for better management of infants with ACD.
Unknown HIV status and the TB/HIV collaborative control program in Ethiopia: systematic review and meta-analysis
Authors: Arega Balew , Minda Abraham , Mengistu Getachew , Endale Mulugeta , Agunie Asnake 
Published: June 2020

Abstract
Background
Ethiopia has shown significant efforts to address the burden of TB/HIV comorbidity through the TB/HIV collaborative program. However, these diseases are still the highest cause of death in the country. Therefore, this systematic review and meta-analysis evaluated this program by investigating the overall proportion of unknown HIV status among TB patients using published studies in Ethiopia.

Methods
We conducted a systematic review and meta-analysis of published studies in Ethiopia. We identified the original studies using the databases MEDLINE/PubMed, and Google Scholar. The heterogeneity across studies was assessed using Cochran’s Q test and I 2 statistics. The Begg’s rank correlation and the Egger weighted regression tests were assessed for the publication bias. We estimated the pooled proportion of unknown HIV status among TB patients using the random-effects model.

Results
Overall, we included 47 studies with 347,896 TB patients eligible for HIV test. The pooled proportion of unknown HIV status among TB patients was 27%(95% CI; 21–34%) and with a substantial heterogeneity (I2 = 99.9%). In the subgroup analysis, the pooled proportion of unknown HIV status was 39% (95% CI; 25–54%) among children and 20% (95% CI; 11–30%) among adults. In the region based analysis, the highest pooled proportion of unknown HIV status was in Gambella, 38% (95% CI; 16–60%) followed by Addis Ababa, 34%(95% CI; 12–55%), Amhara,30%(95% CI; 21–40%),and Oromia, 23%(95% CI; 9–38%). Regarding the study facilities, the pooled proportion of unknown HIV status was 33% (95% CI; 23–43%) in the health centers and 26%(95% CI; 17–35%) in the hospitals. We could not identify the high heterogeneity observed in this review and readers should interpret the results of the pooled proportion analysis with caution.

Conclusion
In Ethiopia, about one-third of tuberculosis patients had unknown HIV status. This showed a gap to achieve the currently implemented 90–90-90 HIV/AIDS strategic plan in Ethiopia, by 2020. Therefore, Ethiopia should strengthen TB/HIV collaborative activities to mitigate the double burden of diseases.
Behavioral barriers to the use of modern methods of contraception among unmarried youth and adolescents in eastern Senegal: a qualitative study
Authors: Cohen Nicki , Mendy Finou Thérèse , Wesson Jennifer , Protti Amanda , Cissé Carol , Gueye Elhadji Babacar , Trupe Lydia , Floreak Rosii , Guichon Dana , Lorenzana Karina , Buttenheim Alison 
Published: June 2020

AbstractBackgroundMany unmarried young people in low- and middle-income countries (LMIC) want to avoid pregnancy but do not use modern methods of contraception—as a result, half of teen births in these countries are unintended. Researchers have identified numerous barriers that prevent youth from using contraception. However, much of the research in West Africa is narrowly focused on married women, and relatively little research has been done to understand the needs, preferences, barriers, and solution set for sexually active unmarried young people who would like to avoid pregnancy. The purpose of this study was to gain insight into the behavioral barriers that prevent unmarried young people in eastern Senegal from using modern methods of contraception.MethodsThis qualitative study conducted in 2017 in the Tambacounda and Kedougou regions in Senegal explores attitudes and beliefs relating to sex and contraception among unmarried young women and men through 48 in-depth individual interviews with young people aged 15–24 and parents of youth and 5 sex-segregated focus groups with 6–9 young people per group. The research team conducted a thematic content analysis and synthesized the findings by major theme following the behavioral diagnosis methodology.ResultsDrawing insights from behavioral science, the analysis yields five key findings: (1) unmarried young people avoid making a decision about contraception because thinking about contraceptive use provokes uncomfortable associations with a negative identity (i.e., being sexually active before marriage); (2) unmarried young people see modern methods as inappropriate for people like them; (3) unmarried young people are overconfident in their ability to prevent pregnancy through traditional and folk methods; (4) unmarried young people overestimate the social and health risks of modern contraceptive methods; and (5) unmarried young people fail to plan ahead and are not prepared to use modern contraceptive methods before every sexual encounter.ConclusionsInterventions aimed at increasing uptake of contraceptives among unmarried young people in eastern Senegal must address several significant behavioral barriers in addition to structural, informational, and socio-cultural barriers in order to be successful.
Association between osteoarthritis and unmet medical needs in Korea: limitations in activities as a mediator
Authors: Jo Hooin , Kim Eun-san , Jung Boyoung , Sung Soo-Hyun , Ha In-Hyuk 
Published: June 2020

Abstract
Background
The role of limitations in activities in relation to unmet needs is not clarified. This study aimed to analyze the effects of osteoarthritis on unmet medical needs and the mediating effects of limitations in activities.

Methods
A total number of 10,129 population aged ≥50 years were included using data from the Korean National Health and Nutrition Examination Survey from January 2010 to December 2013. Osteoarthritis was defined as Kellgren-Lawrence grade ≥ 2 in the knee, hip, and lumbar spine joints with pain reported to have lasted for ≥3 months. Limitations in activities were defined as currently experiencing restricted daily and social activities. Unmet medical needs were analyzed after they were further divided into availability, accessibility, and acceptability. Causal mediation analysis was employed to analyze mediating effects.

Results
The osteoarthritis group had a higher odds ratio (OR: 1.65; 95% confidence interval [CI], 1.56–1.75) for the total effects of osteoarthritis on unmet medical needs than the non-osteoarthritis group. Furthermore, the OR for the indirect effects mediated by limitations in activities was higher in the osteoarthritis group (OR: 1.07; 95% CI, 1.05–1.08), indicating that 13.2% of the total effect was mediated. When the analysis was further classified according to cause, the mediating effect of limitations in activities was the strongest at 23.9% for unmet medical needs due to lack of transportation accessibility.

Conclusions
Osteoarthritis exerts significant effects on the experience of unmet medical needs, and limitations in activities mediate such experiences of unmet medical needs in osteoarthritis patients.
Comparison of radiological and clinical outcomes, complications, and implant removals in anatomically pre-contoured clavicle plates versus reconstruction plates – a propensity score matched retrospective cohort study of 106 patients
Authors: Fang Christian X. , Liu Ruiping , Yee Dennis K. H. , Chau Jackie , Lau Tak-Wing , Chan Rebecca , Woo Siu-Bon , Wong Tak-Man , Fang Evan , Leung Frankie 
Published: June 2020

Abstract
Background
Plate fixation is frequently used to treat displaced midshaft clavicular fractures, however the ideal plate choice remains subject to discussion; reconstruction locking compression plates (RLCPs) are cheaper and can be easily contoured, whereas anatomically pre-contoured locking compression plates (ALCPs) are thought to provide better stability and therefore lower rates of mechanical failure.
To compare the incidence of mechanical failures, functional and radiological outcomes in patients with midshaft clavicular fractures treated with ALCPs versus RLCPs.

Methods
A propensity score matched retrospective cohort study was conducted across two centers. One hundred and six consecutively recruited patients with displaced midshaft clavicular fractures, who were treated with plate fixation and had a minimum follow-up of 6 months, were matched on gender, age, fracture grading, energy of injury, and fracture location. The resulting groups included 53 ALCP-treated fractures and 53 matched controls treated with RLCPs.

Results
During a mean follow-up of 20.5 months, there were no implant deformities in the ALCP group whereas the RLCP group had 6 patients (11.3%, p = 0.012) with implant deformities (5 occurrences of plate bending with fracture union, and 1 plate breakage with nonunion). Despite the higher rate of plate deformities in the RLCP group, there were no statistically significant differences in number of patients recovering full shoulder range of motion (ALCP 90.6%, RLCP 88.7%, p = 0.751), incidence of rest pain (ALCP 13.2%, RLCP 9.4%, p = 0.542), or implant removals (ALCP 49.1%, RLCP 56.6%, p = 0.439).

Conclusion
ALCPs may be superior to RLCPs in terms of implant stability but appear to produce similar clinical results.
Cerebrospinal fluid-cutaneous fistula associated with post-traumatic Charcot spinal arthropathy: a case report and review of literature
Authors: Ryu Ji Hyun , Lee Jun-Seok , Lim Chang-Rack , Cho Wan Jae , Kim Ki-Won 
Published: June 2020

AbstractBackgroundCharcot spinal arthropathy, also known as Charcot spine and neuropathic spinal arthropathy, is a progressive and destructive condition that affects an intervertebral disc and the adjacent vertebral bodies following loss of spinal joint innervation. We report the first case of Charcot spinal arthropathy (CSA) associated with cerebrospinal fluid (CSF)-cutaneous fistula.Case presentationA 54-year-old male who underwent T10-L2 posterior instrumented spinal fusion seven years prior for treatment of T11 burst fracture and accompanying T11 complete paraplegia visited our department complaining of leakage of clear fluid at his lower back. The patient had also undergone various types of skin graft and myocutaneous flap surgeries for treatment of repetitive pressure sores around his lumbosacral area. The patient presented with persistent CSF leakage from a cutaneous fistula (CSF-cutaneous fistula) formed in a lumbosacral pressure sore. The CSF-cutaneous fistula arose from the L5 post-traumatic CSA. Surgery was planned for management of CSF-cutaneous fistula and post-traumatic L5 CSA. We successfully treated the CSF-cutaneous fistula with ligation and transection of the dural sac and cauda equina at the L2-L3 level. In addition, the post-traumatic L5 CSA was successfully treated with a posterior four-rod spinopelvic fixation from T9 to ilium and S2 foramina. After surgery, the CSF leakage stopped and no other adverse neurological changes were found. The four-rod spinopelvic construct was well maintained five years later.ConclusionsCSA associated with CSF-cutaneous fistula is a very rare disorder. Only surgical treatment for both CSA and CSF-cutaneous fistula with ligation and transection of the dural sac and posterior four-rod spinopelvic fixation can bring satisfactory results.
Anti-rheumatic treatment and prosthetic joint infection: an observational study in 494 elective hip and knee arthroplasties
Authors: Borgas Ylva , Gülfe Anders , Kindt Mikael , Stefánsdóttir Anna 
Published: June 2020

Abstract
Background
Surgical site infections are more frequent among patients with rheumatic disease. To what extent this is related to immunosuppressive antirheumatic drugs is unclear, as is the value of discontinuing medication perioperatively. The aim of study was to assess the rate of surgical site infections after knee and hip replacement in patients with inflammatory joint disease, with an emphasis on periprosthetic joint infection, and to investigate the influence of treatment with disease-modifying antirheumatic drugs (DMARDs) in this regard.

Methods
Data were collected from 494 primary elective hip (51.4%) and knee arthroplasties, along with demographic and medication data. The primary outcome was surgical site infection during the first year after surgery.

Results
In 78% (n = 385) of the cases the patient used 1 to 3 disease-modifying antirheumatic drugs perioperatively. Thirty-two percent (n = 157) of patients used a TNF-alpha inhibitor. The rate of surgical site infection was 3.8% (n = 19). The rate of periprosthetic joint infection was 1.4% (n = 7), all of which occurred after knee arthroplasty. Periprosthetic joint infection occurred in only 1 patient medicating perioperatively with a TNF-alpha inhibitor.

Conclusion
Surgical site infections were not associated with ongoing medication with disease-modifying antirheumatic drugs. Due to the low event rate this should be interpreted with caution, but our center will maintain its routine of continuing treatment with TNF-alpha inhibitors perioperatively.
Orthotopic liver transplantation for Management of a Giant Liver Hemangioma: a case report and review of literature
Authors: Eghlimi Hesameddin , Arasteh Peyman , Azade Nazanin 
Published: June 2020

AbstractBackgroundHepatic hemangioma (HH) is the most common benign tumor of the liver. In special conditions such as rapidly growing tumors, persistent pain, hemorrhage and when pressure effect on adjacent organs exist treatment is indicated. Surgical management is the most common treatment for HH.Case presentationA 38-year-old male patient was diagnosed with HH for 7 years. The initial presentation of the mass was progressive abdominal distention causing early satiety, gastro-esophageal reflux disease, vomiting, dysphagia and weight loss. Later, the patient developed bilateral lower extremity edema. Imaging with computed tomography (CT scan) showed a large mass measuring 32.4*26*3.1 cm which was considered unresectable. The patient underwent a deceased donor liver transplantation. The excised mass was 9 kg. After nine days of hospitalization the patient was discharged in good condition. Three months later, the patient was admitted due to fever and cytomegalovirus infection for which he received intravenous ganciclovir and was discharged. In the latest follow-up the patient had no liver or kidney dysfunction eight months after the transplantation.ConclusionWith appropriate patient selection, liver transplantation can be considered as a treatment option for patients with huge HHs which are life-threatening and surgically unresectable.
Recurrent broad ligament leiomyosarcoma with pancreatic and thigh metastasis: a case report
Authors: Tian Xuan , Yan Xin , Wu Jun , Song Hongli , Shen Zhongyang 
Published: June 2020

Abstract
Background
Leiomyosarcoma (LMS) is an uncommon mesenchymal neoplasm, which infrequently metastasizes to pancreas and thigh. Clinical presentation and imaging findings of metastatic broad ligament LMS are often nonspecific. Complete excision plays an important role in treatment of patients with localized LMS.

Case presentation
Here, we report a case of a 33-year-old woman with recurrent broad ligament LMS metastasizing to pancreas and thigh. Previously, she was diagnosed with broad ligament LMS and underwent hysterectomy, bilateral salpingo-oophorectomy. The disease-free interval was 2.5 years until metastases were found. Computerized tomography (CT) of abdomen and thighs, magnetic resonance imaging (MRI) of thighs and whole-body 18-fluorodeoxyglucose positron emission tomography - computed tomography (PET-CT) performed, revealed pancreatic and thigh metastasis. Ultrasonography-guided biopsy and histological examinations confirmed LMS at both the sites. Pancreatic metastasis was completely resected first. Then the patient underwent surgical resection of thigh metastasis when both chemotherapy and radiotherapy failed. She recovered well and remained free of disease recurrence in the 2 years follow-up.

Conclusions
Though imaging lacks specificity, it is a valuable asset in assessing the burden of disease and characterizing lesions while histological examination with immunohistochemistry is helpful for the diagnosis of LMS. Complete surgical resection of all metastatic sites where-ever feasible should be strongly considered in a treated case of broad ligament LMS with a durable disease-free interval.
Functional expression of a novel methanol-stable esterase from Geobacillus subterraneus DSM13552 for biocatalytic synthesis of cinnamyl acetate in a solvent-free system
Authors: Cai Xianghai , Lin Lin , Shen Yaling , Wei Wei , Wei Dong-zhi 
Published: June 2020

Abstract
Background
Esterases are widely distributed in nature and have important applications in medical, industrial and physiological. Recently, the increased demand for flavor esters has prompted the search of catalysts like lipases and esterases. Esterases from thermophiles also show thermal stability at elevated temperatures and have become enzymes of special interest in biotechnological applications. Although most of esterases catalyzed reactions are carried out in toxic and inflammable organic solvents, the solvent-free system owning many advantages such as low cost and easy downstream processing.

Results
The gene estGSU753 from Geobacillus subterraneus DSM13552 was cloned, sequenced and overexpressed into Escherichia coli BL21 (DE3). The novel gene has an open reading frame of 753 bp and encodes 250-amino-acid esterase (EstGSU753). The sequence analysis showed that the protein contains a catalytic triad formed by Ser97, Asp196 and His226, and the Ser of the active site is located in the conserved motif Gly95-X-Ser97-X-Gly99 included in most esterases and lipases. The protein catalyzed the hydrolysis of pNP-esters of different acyl chain lengths, and the enzyme specific activity was 70 U/mg with the optimum substrate pNP-caprylate. The optimum pH and temperature of the recombinant enzyme were 8.0 and 60 °C respectively. The resulting EstGSU753 showed remarkable stability against methanol. After the incubation at 50% methanol for 9 days, EstGSU753 retained 50% of its original activity. Even incubation at 90% methanol for 35 h, EstGSU753 retained 50% of its original activity. Also, the preliminary study of the transesterification shows the potential value in synthesis of short-chain flavor esters in a solvent-free system, and more than 99% conversion was obtained in 6 h (substrate: cinnamyl alcohol, 1.0 M).

Conclusions
This is the first report of esterase gene cloning from Geobacillus subterraneus with detailed enzymatic properties. This methanol-stable esterase showed potential value in industrial applications especially in the perfume industry.
Eucalyptus essential oils inhibit the lipopolysaccharide-induced inflammatory response in RAW264.7 macrophages through reducing MAPK and NF-κB pathways
Authors: Ho Chen-Lung , Li Lan-Hui , Weng Yueh-Chun , Hua Kuo-Feng , Ju Tz-Chuen 
Published: June 2020

AbstractBackgroundEucalyptusessential oils have been used in traditional medicine for centuries. It was reported thatEucalyptusleaves possess antioxidant and antimicrobial effects. Here, we investigated the anti-inflammatory activity of the essential oils extracted from the leaves of four differentEucalyptusspecies in RAW264.7 macrophages.MethodsLipopolysaccharide (LPS)-activated RAW264.7 macrophages were used to evaluate the anti-inflammatory activity of the leaf essential oils ofEucalyptus. The cell survival was quantified by an Alamar Blue assay. Nitric oxide (NO) production was assessed by Griess reaction. TNF-α and IL-6 production were measured by enzyme-linked immunosorbent assay (ELISA). Nuclear factor-κB (NF-κB) transcriptional activity was measured by NF-κB reporter assay. Intracellular protein expression levels were determined by Western blot. The expression levels of inducible NO synthase (iNOS), cyclooxygenase-2 (COX-2), mitogen-activated protein kinase (MAPK), protein kinase C (PKC) and NF-κB pathway were measured by western blot in LPS-activated RAW 264.7 macrophage.ResultsThe essential oils extracted fromEucalyptus citriodoraleaf exert the best NO inhibitory activity in LPS-activated RAW264.7 macrophages. The essential oils were fractionated into fractions A-H, and fraction F has been demonstrated to inhibit the expression levels of TNF-α, IL-6, NO, iNOS and COX-2 in LPS-activated RAW264.7 macrophages. Mechanistic analysis revealed that fraction F reduced the phosphorylation levels of ERK1/2, p38, PKC-α, PKC-ε and PKC-δ, and inhibited the NF-κB transcriptional activity. The chemical composition of Fraction F was determined by GC-MS.ConclusionsThe discoveries made herein could help develop innovative nonsteroidal anti-inflammatory drugs with minimal side effects and strong efficacy. Clinical trials on theseEucalyptusleaf essential oils will help customize and optimize their therapeutic administration.