bluuebunny/crossref_metadata_2025_split huggingface dataset:
Higher Waitlist Mortality in Pediatric Acute‐on‐chronic Liver Failure in the UNOS Database
Authors: Godfrey Elizabeth , Desai Moreshwar , Lam Fong , Goss John , Rana Abbas , Miloh Tamir 
Published: August 2020

ABSTRACTObjectives:Acute‐on‐chronic liver failure (ACLF), whereas increasingly well‐defined in adults, has been poorly characterized in pediatric patients other than having a poor prognosis. This study aimed to identify ACLF and evaluate prognosis in the American pediatric population.Methods:Modified ACLF definitions (p‐CLIF) were applied to 11,300 children listed for liver transplantation from March 2002 through 2017 in the Organ Procurement and Transplantation Network (OPTN) database.Results:Pediatric ACLF patients have greater mortality within 90 days from listing (46.6% by p‐CLIF) than other types of failure (<30%), including acute liver failure, as well as greater mortality within the first 30 and 90 days after transplantation than all other types of liver failure, but do not have increased mortality rates relative to other groups between 90 and 365 days from transplant. Although some ACLF listings also received 1B status, ACLF mortality at 90 days was greater than the general 1B population (50 vs 29.4%). Model for End‐Stage Liver Disease/Pediatric End‐Stage Liver Disease scores of ACLF patients are lower than 1B listings, and do not predict waitlist or posttransplant death. Greater number of organ failures does correlate with increased mortality. Biliary atresia is the leading etiology of pediatric chronic liver disease, accounting for over 30% of chronic and 45% of ACLF listings, yet is protective against mortality (hazard ratio [HR] = 0.142 for ACLF). Receiving exception approval is independently but similarly protective in ACLF (HR = 0.145).Conclusions:These findings pose a challenge for allocation decisions but indicate greater attention to ACLF is needed, as scoring systems may not capture these children's risk of early death, which appears to currently be mitigated by exceptions. Multicenter, clinical, preferably prospective study of ACLF is necessary to determine how to prioritize ACLF relative to other liver failure types to address its relatively higher early mortality.
Positivity of Stool Pathogen Sampling in Pediatric Inflammatory Bowel Disease Flares and Its Association With Disease Course
Authors: Melnik Pesah , Soffair Niv , Matar Manar , Shamir Raanan , Assa Amit 
Published: August 2020

ABSTRACTObjectives:Acute exacerbations of inflammatory bowel disease (IBD) may involve enteric pathogen. We aimed to assess the frequency and outcomes of Clostridium difficille toxin (CDT) and non‐CDT enteric infections in symptomatic pediatric patients with IBD.Methods:Patients’ records were retrospectively searched for disease flares in which stool samples were collected for enteric pathogens. Each patient with a positive sample was matched with a patient with IBD flare and negative samples for analyzing 1‐year outcomes following sampling.Results:A total of 618 pediatric patients with IBD [Crohn's disease, n = 439 (71%), mean age at diagnosis 13.0 ± 3.4 years, girls, n = 264 (42.7%)] had 1048 stool samples during the study period (2001–2018). Of 914 bacterial cultures, 40 (4.3%) were positive, 30 (75%) of which, positive for Campylobacter jejuni. Of 393 samples for CDT, 28 (7.1%) were positive while parasitic infection rate was 21/529 (3.9%).Overall, 19 positive C jejuni cases and 19 positive CDT cases with matching controls were examined. During 12 months of follow‐up, the mean number of disease flares and emergency room visits was higher among patients with positive CDT (1.5 ± 1.4 vs 0.5 ± 0.9, P = 0.019, 1.3 ± 1.5 vs 0.4 ± 0.8, P = 0.05, respectively) with a numeric increase of surgical interventions (3 vs 0, P = 0.08). There were no significant differences in disease outcomes between patients with C jejuni infections and matched controls.Conclusions:C difficile and C jejuni are the most common enteric infections among pediatric patients with IBD but only clostridial infection was associated with a more severe disease course within 12 months.
Impaired Bone Health in Children With Biliary Atresia
Authors: Ruuska Satu , Laakso Saila , Leskinen Outi , Hagfors Anna , Jalanko Hannu , Kolho Kaija‐Leena , Pakarinen Mikko P. 
Published: August 2020

ABSTRACTObjectives:The aim of the study was to examine the frequency of rickets and bone fractures and to assess areal bone mineral density (aBMD) in childhood among patients with biliary atresia (BA).Methods:We gathered data on all patients diagnosed with BA in Finland that survived to ≥1 year of age between 1 January 2000 to 30 June 2018. Data on gestational age, birth weight, postsurgical medications, and history of rickets and bone fractures were collected retrospectively. Serum levels of 25‐hydroxyvitamin D [25(OH)D] postportoenterostomy (PE) were collected. Plain radiographs and dual energy X‐ray absorptiometry (DXA) measurements of study subjects were reviewed.Results:Out of 49 patients, 7 (14%) were diagnosed with rickets during infancy. Clearance of jaundice [odds ratio 0.055, 95% confidence interval [CI] 0.00266–0.393; P < 0.01] was a protective factor against rickets. Sufficient 25(OH)D levels were reached 3 months post‐PE. Eleven (22%) patients suffered at least one bone fracture (range 1–9) during childhood and adolescence. In DXA measurements, median lumbar spine aBMD anthropometrically adjusted z‐scores were as follows: in native liver survivors 0.8 (interquartile range [IQR] −1.9 to 1.4) at 5 and −0.3 (IQR −1.3 to 0.8) at 10 years and for liver transplanted patients 0.4 (IQR −0.2 to 1.1) at 5 and 0.6 (IQR −0.1 to 1.3) at 10 years.Conclusions:BA patients have an increased risk for rickets and bone fractures compared with the normal population. Most BA patients have aBMD within normal range between 5 and 10 years of age irrespective of liver transplantation status.
The Umbrella Technique for the Closure of Cerebrospinal Fluid Fistula
Authors: Maggiore Giandomenico , Locatello Luca Giovanni , Cannavicci Angelo , Bruno Chiara , Gallo Oreste 
Published: August 2020

AbstractThe diagnosis and management of cerebrospinal fluid (CSF) rhinorrhea remains an important challenge in the field of rhinology. In this study, the authors want to propose a technique for transnasal endoscopic closure of CSF fistulas, tested for the first time on 2 adult fresh cadaveric specimens. The authors think that the use of a device similar to the umbrella device, used to close cardiac atrial defects, may also be valid for the closure of defects at the level of the anterior skull base.
Advanced Simultaneous Isolation, Culture, and Identification of Myoblasts and Fibroblasts From Sternocleidomastoid Muscle of Congenital Muscular Torticollis
Authors: Qiu Xin , Zeng Shuaidan , Xiong Zhu , Tang Shengping , Tang Gen , Li Shicheng 
Published: August 2020

Background:
Congenital muscular torticollis (CMT) is a common pediatric disease caused by contracture of sternocleidomastoid muscle (SCM) that leads to neck stiffness and deformity. Based on the adhesion characteristics of different cells in affected SCM of CMT, myoblasts and fibroblasts can be isolated simultaneously by advanced culture conditions. Our study aimed to explore and optimize the isolation, culture, and identification of myoblasts and fibroblasts in SCM of CMT.

Methods:
Myoblasts and fibroblasts were separated by combined digestion with trypsin and collagenase. With this improved method, the morphology of isolated myoblasts and fibroblasts was observed under the microscope, the cell growth curve was drawn, and the purity of myoblasts and fibroblasts was determined by immunofluorescence.

Results:
The method allowed to satisfactorily culture myoblasts and fibroblasts. The cells could stably grow and be passaged, provided they were at least 80% confluent. Immunofluorescence of myoblasts and fibroblasts showed high rate of positive staining, and cell count showed excellent growth state. Moreover, according to the growth curve, fibroblasts grew at a higher rate than myoblasts.

Conclusions:
The isolated myoblasts and fibroblasts have high purity, intact structure, and relatively high vitality. This method can be used to establish a cell model with myoblasts and fibroblasts, which can be applied to investigate etiology of CMT or mechanisms of drug action.
Long-Term Outcomes of Zygomaticomaxillary Reconstruction With Autologous Bone Grafts Supported by Pedicled Buccal Fat Pad Flap
Authors: Chu Hanwen , Huang Linjian , Xu Feifei , Chen Guanfu , Xu Xin 
Published: August 2020

Purpose:The purpose of this study was to investigate the effectiveness of the pedicle buccal fat pad flap combined with autogenous bone grafts to reconstruct zygomaticomaxillary defects after tumor resection.Materials and Methods:A retrospective case series analysis of 11 patients that underwent zygomaticomaxillary reconstruction after tumor resection with autologous bone grafts supported by pedicle buccal fat pad flap as the lining of the maxillary sinus from January 2009 to December 2015 was performed. All the patients underwent computed tomography to measure the aesthetic appearance, bone graft formation, mucosa of the maxillary sinus, and their response to treatment, including complications.Results:With a mean follow-up period of 4.7 years, all patients were pleased with the symmetrical contours of zygomaticomaxillary and symptom improvements of the maxillary sinus except for one patient who complained of transient infraorbital numbness. Recurrence, bone infection or necrosis, and other complications were not observed in the follow-up period.Conclusion:Pedicled buccal fat pad flap promotes wound healing and prevents the exposure of bone grafts to the maxillary sinus. This technique may be a promising treatment option when reconstructing complicated zygomaticomaxillary defects.
First Report of Pai Syndrome With Upper Alveolar Cavernous Hemangioma: Diagnosis and Surgical Treatment
Authors: Demir Can Ilker , Yaşar Emrah Kağan , Bayrak Büşra Yaprak , Işik Halil , Alagöz Murat Şahin 
Published: August 2020

Abstract
This case describes the surgical methodology and the procedure for follow-up in a patient who had midline cleft of the upper lip, mid-alveolar cleft, double frenulum, and alveolar mass. Our patient is the first who meets the criteria for Pai syndrome with cavernous hemangioma and is also the fourth case with concomitant double frenulum.
Correction of Temporal Hollowing With the Superior Gluteal Artery Perforator Free Flap
Authors: Lee Yoon Jae , Lee Chae Rim , Moon Suk-Ho , Jun Young Joon , Oh Deuk Young 
Published: August 2020

Abstract
Temporal hollowing is most frequently an acquired deformity and can be caused by volume defects of bone, soft tissue, or both. Recently, surgical reconstruction with autogenous fat or allografts has been introduced, but these materials can be resorbed over time and may also lead to infection. Herein, the authors present 2 cases demonstrating the treatment of temporal hollowing with a superior gluteal artery perforator (SGAP) free flap. Both patients had a history of traffic accidents and subsequent craniectomy because of subdural hemorrhage, followed by cranioplasty. An SGAP free flap was selected to reconstruct the soft tissue temporal defect in both patients. Pre-operatively, computed tomography (CT) angiography was used to locate the superior temporal artery (STA). A preauricular incision provided surgical access to the STA, and a subcutaneous pocket was created. The STA and SGAP artery and vein anastomoses were performed. Before flap insetting, it was de-epithelized, and its volume and shape trimmed to fit into the temporal defect. The patients’ post-operative courses were without complications, and facial symmetry was achieved. This is the first case report describing the microvascular reconstruction of temporal hollowing with an SGAP free flap. The SGAP free flap is permanent and highly vascularized with no risk of infection. These case reports illustrate a unique reconstruction technique that resulted in a satisfactory outcome for the patients.
Current trends in the clinical trial landscape for amyotrophic lateral sclerosis
Authors: van Eijk Ruben P.A. , Kliest Tessa , van den Berg Leonard H. 
Published: August 2020

Purpose of review
To review the current developments in the design and conduct of clinical trials for amyotrophic lateral sclerosis (ALS), illustrated by a critical appraisal of ClinicalTrials.gov.

Recent findings
In total, 63 clinical trials were included in the analysis, of which 13 phase 1, 35 phase 2 and 15 phase 3. Virtually all phase 3 clinical trials can be classified as randomized, placebo controlled, whereas this is only true for 57% of the phase 2 clinical trials. There are promising developments in the routes of drug administration, eligibility criteria, efficacy endpoints and overall trial design. Some of these innovative approaches may, however, not fulfil clinical trial guidelines or regulatory requirements. This could delay the development of effective therapy or hamper our ability to determine whether a treatment is truly (in)effective. The initiation of trial consortia comprising patient organizations, academia, industry and funding bodies may significantly strengthen the future clinical trial landscape for ALS.

Summary
The ALS clinical trial landscape is currently highly active with several promising innovative developments and therapeutic options. By further refinement of evidence-based guidelines, and alignment of our current endeavours, we may soon be able to positively impact the lives of people living with ALS.
Factors Associated with Disease Severity and Mortality among Patients with Coronavirus Disease 2019: A Systematic Review and Meta-Analysis
Authors: Chidambaram Vignesh , Tun Nyan Lynn , Haque Waqas Z , Majella Marie Gilbert , Sivakumar Ranjith Kumar , Kumar Amudha , Hsu Angela Ting-Wei , Ishak Izza A , Nur Aqsha A , Ayeh Samuel K , Salia Emmanuella L , Zil-E-Ali Ahsan , Saeed Muhammad A , Sarena Ayu PB , Seth Bhavna , Ahmadzada Muzzammil , Haque Eman F , Neupane Pranita , Wang Kuang-Heng , Pu Tzu-Miao , Ali Syed MH , Arshad Muhammad A , Wang Lin , Baksh Sheriza , Karakousis Petros C , Galiatsatos Panagis 
Published: August 2020

ABSTRACTBackgroundUnderstanding the factors associated with disease severity and mortality in Coronavirus disease (COVID-19) is imperative to effectively triage patients. We performed a systematic review to determine the demographic, clinical, laboratory and radiological factors associated with severity and mortality in COVID-19.MethodsWe searched PubMed, Embase and WHO database for English language articles from inception until May 8, 2020. We included Observational studies with direct comparison of clinical characteristics between a) patients who died and those who survived or b) patients with severe disease and those without severe disease. Data extraction and quality assessment were performed by two authors independently.ResultsAmong 15680 articles from the literature search, 109 articles were included in the analysis. The risk of mortality was higher in patients with increasing age, male gender (RR 1.45, 95%CI 1.23–1.71), dyspnea (RR 2.55, 95%CI 1.88–2.46), diabetes (RR 1.59, 95%CI 1.41–1.78), hypertension (RR 1.90, 95%CI 1.69–2.15). Congestive heart failure (OR 4.76, 95%CI 1.34–16.97), hilar lymphadenopathy (OR 8.34, 95%CI 2.57–27.08), bilateral lung involvement (OR 4.86, 95%CI 3.19–7.39) and reticular pattern (OR 5.54, 95%CI 1.24–24.67) were associated with severe disease. Clinically relevant cut-offs for leukocytosis(>10.0 ×109/L), lymphopenia(< 1.1 ×109/L), elevated C-reactive protein(>100mg/L), LDH(>250U/L) and D-dimer(>1mg/L) had higher odds of severe disease and greater risk of mortality.ConclusionKnowledge of the factors associated of disease severity and mortality identified in our study may assist in clinical decision-making and critical-care resource allocation for patients with COVID-19.Primary Funding SourceNone.
Face masks release water vapour but where does it go? An early observational study
Authors: Stubington T.J. , Sahota R.S. , Mottacki N. , Johnston M.N. , Judd O. 
Published: August 2020

AbstractObjectivesThe aim of this observational study was to demonstrate the behaviour and trajectory of exhaled material from an individual wearing an FFP3 mask. Valves allow material release, but we theorised that valve design may direct material downwards towards patient and surrounding environment.Limiting transmission of diseases with aerosolised spread is a current and serious concern within healthcare worldwide. Filtering face piece masks (FFP) are an essential piece of protective equipment when treating patients with ongoing infection. However, valved masks in other settings such as elective theatre and by the general public may have unforeseen negative effects.DesignA heating coil-based vaporiser was used to produce visible water vapour. A healthy test subject was filmed wearing a variety of different masks and exhaling the water vapour.ResultsFlexible pleated and solid-shell FFP masks direct exhaled material downwards in plumes exceeding 25 cm. Duckbill-shaped masks appear to direct exhaled vapour laterally, with a smaller plume. The effect is influenced by mask design and type of valve. Fluid repellent surgical masks reduce material directed downwards, and when used in conjunction with an FFP3 mask, appear to reduce the size and density of the exhaled vapour plume. The use of a visor was ineffective in reducing plume expulsion.InterpretationA properly fit-tested FFP3-rated protective mask may only moderately limit expulsion of aerosolised particles from asymptomatic healthcare workers to patients, particularly in cases where procedures are being performed in close proximity to patients or in cases where mucosal surfaces are exposed. Further research in this area is needed.
A city-level analysis of air pollution, climate and COVID-19 early spread during the Spanish lockdown
Authors: Briz-Redón Álvaro , Belenguer-Sapiña Carolina , Serrano-Aroca Ángel 
Published: August 2020

AbstractThe COVID-19 outbreak has escalated into the worse pandemic of the present century. The fast spread of the new SARS-CoV-2 coronavirus has caused devastating health and economic crises all over the world, with Spain being one of the worst affected countries in terms of confirmed COVID-19 cases and deaths per inhabitant. In this situation, the Spanish Government declared the lockdown of the country. The variations of air pollution in terms of fine particulate matter (PM2.5) levels in seven cities of Spain are analyzed here considering the effect of meteorology during the national lockdown. The possible associations of PM2.5pollution and climate with COVID-19 accumulated cases were also analyzed. While the epidemic curve was flattened, the results of the analysis show that the 4-week Spanish lockdown significantly reduced the PM2.5levels in only one of the cities despite the drastically reduced human activity in good agreement with our previous study of changes in air quality in terms of CO, SO2, PM10, O3and NO2levels. Furthermore, no associations between either PM2.5exposure or environmental conditions and COVID-19 transmission were found during the early spread of the pandemic.
Transmission heterogeneities, kinetics, and controllability of SARS-CoV-2
Authors: Sun Kaiyuan , Wang Wei , Gao Lidong , Wang Yan , Luo Kaiwei , Ren Lingshuang , Zhan Zhifei , Chen Xinghui , Zhao Shanlu , Huang Yiwei , Sun Qianlai , Liu Ziyan , Litvinova Maria , Vespignani Alessandro , Ajelli Marco , Viboud Cécile , Yu Hongjie 
Published: August 2020

AbstractA long-standing question in infectious disease dynamics is the role of transmission heterogeneities, particularly those driven by demography, behavior and interventions. Here we characterize transmission risk between 1,178 SARS-CoV-2 infected individuals and their 15,648 close contacts based on detailed contact tracing data from Hunan, China. We find that 80% of secondary transmissions can be traced back to 14% of SARS-CoV-2 infections, indicating substantial transmission heterogeneities. Regression analysis suggests a marked gradient of transmission risk scales positively with the duration of exposure and the closeness of social interactions, after adjusted for demographic and clinical factors. Population-level physical distancing measures confine transmission to families and households; while case isolation and contact quarantine reduce transmission in all settings. Adjusted for interventions, the reconstructed infectiousness profile of a typical SARS-CoV-2 infection peaks just before symptom presentation, with ~50% of transmission occurring in the pre-symptomatic phase. Modelling results indicate that achieving SARS-CoV-2 control would require the synergistic efforts of case isolation, contact quarantine, and population-level physical distancing measures, owing to the particular transmission kinetics of this virus.
Germline mutations in hereditary breast cancer genes are associated with early age at diagnosis and family history in Guatemalan breast cancer
Authors: Ren Megan , Orozco Anali , Shao Kang , Albanez Anaseidy , Ortiz Jeremy , Cao Boyang , Wang Lusheng , Barreda Lilian , Alvarez Christian S. , Garland Lisa , Wu Dongjing , Chung Charles , Wang Jiahui , Frone Megan , Ralon Sergio , Argueta Victor , Orozco Roberto , Gharzouzi Eduardo , Dean Michael 
Published: August 2020

AbstractBackgroundMutations in hereditary breast cancer genes play an important role in the risk for cancer, however, little is known of the type and frequency of mutations in Central American populations, including Guatemala.MethodsTwo separate panels of known cancer susceptibility genes were used to sequence blood DNA from 664 unselected breast cancer cases from two large hospitals in Guatemala. Variants were annotated with ClinVar and VarSome. Data from a structured questionnaire was used to compare mutation carriers of medium and high penetrance genes.ResultsA total of 73 out of 664 subjects (11%) had a variant classified as pathogenic in a gene with known high or medium penetrance for inherited breast cancer. The most frequently mutated genes were BRCA1 (37/664, 5.6%) followed by BRCA2 (15/664, 2.3%), PALB2 (5/664, 0.8%) and TP53 (5/664, 0.8%). Pathogenic variants were also detected in the moderate penetrance genes ATM, BARD1, CHEK2, and MSH6, and rare pathogenic variants detected in the low penetrance genes AXIN2, FH, MLH1, MSH2, MUTYH, NF1, and SDHB. The high ratio of BRCA1/BRCA2 mutations is due to the presence of two potential founder mutations, BRCA1 c.212+1G>A splice mutation (15 cases) and BRCA1 c.799delT (9 cases). Compared to all others, cases with pathogenic mutations had a significantly earlier age at diagnosis (45 vs 51 years, P<0.001), more likely to have had diagnosis before menopause, and a higher percentage had a relative with any cancer (51% vs 37%, P=0.038) or breast cancer (33% vs 15%, P<0.001). Mammography usage was less frequent in lower SES women indicating this group is less likely to be screened for breast cancer (p < 0.001).ConclusionsGuatemalan women have rates of hereditary breast cancer mutations similar to other populations, and these women are more likely to have early age at diagnosis and family history. This data supports the use of genetic testing in breast cancer patients and those at high risk as part of a strategy to reduce breast cancer mortality in Guatemala.