bluuebunny/crossref_metadata_2025_split huggingface dataset:
The Possible Relationship Between Familial Mediterranean Fever and Chronic Nonbacterial Osteomyelitis
Authors: Çiçek Sümeyra Özdemir , Şahin Nihal , Karaman Zehra Filiz , Taşkın Sema Nur , Kısaarslan Ayşenur Paç , Gündüz Zübeyde , Poyrazoğlu Muammer Hakan , Düşünsel Ruhan 
Published: June 2020

Background/Objectives
Chronic nonbacterial osteomyelitis (CNO) is an inflammatory disease characterized by recurrent attacks and remissions due to sterile bone inflammation. The CNO may be accompanied by various inflammatory diseases. The aims of our study were to determine the clinical, laboratory, and radiological characteristics of children with CNO, and to investigate the possible effect of concomitant diseases on the course of CNO.

Methods
Twenty-three patients who were diagnosed with CNO between 2012 and 2019 were analyzed. Demographic characteristics, clinical courses, laboratory and imaging findings, and concomitant diseases were recorded. The characteristics of the CNO patients with and without concomitant diseases were compared.

Results
The mean ± SD age of patients at the time of diagnosis and the last follow-up was 10.46 ± 4.1 and 12.47 ± 4.47 years, respectively. The median (range) time interval between disease onset and diagnosis was 5.33 (1–55) months. The mean ± SD duration of disease was 24.71 ± 16.76 months. Twelve patients (52.2%) were male. The most commonly affected areas were femur (74%), tibia/fibula (74%), and pelvis (52.2%). Age at symptom onset, age at diagnosis, mean number of lesions, presence of sacroiliitis, acute phase reactants at the start of disease, clinical and radiological remission rates, and treatment responses were not significantly different between the 13 patients with concomitant diseases and those without. Eight patients (34.8%) had familial Mediterranean fever (FMF), and all of them had exon 10 mutations. Four patients (17.4%) had juvenile spondylarthritis, one had inflammatory bowel disease, and one had psoriatic arthritis as concomitant diseases. Clinical remission was achieved in 19 patients (82.6%) and complete remission in 11 patients (47.8%) at the time of follow-up.

Conclusions
In our cohort, half of the patients with CNO had concomitant diseases, with FMF being the most common. We think that the coexistence of FMF and CNO is not a coincidental one and that both may result due to an abnormality of a common pathogenetic pathway.
Temporal Fat Grafting in Children With Craniofacial Anomalies
Authors: Fahradyan Artur , Goel Pedram , Williams Madeline , Liu Alice , Gould Daniel G. , Urata Mark M. 
Published: June 2020

Background
Fat grafting is a common adjunct procedure used to treat temporal fat hollowing in children with craniofacial anomalies. The goal of this study was to assess the surgical and aesthetic outcomes of this procedure.

Methods
We retrospectively reviewed patients who underwent temporal fat grafting at a single tertiary pediatric craniofacial center. The surgical outcome was assessed based on intraoperativeand 30-day postoperative complication rates. The aesthetic outcome was assessed by 3 independent investigators using a 3-point grading scale for preoperative temporal hollowing severity (1 = mild, 2 = moderate, 3 = severe) and a 5-point scale for postoperative improvement (0 = no, 1 = mild, 2 = moderate, 3 = significant, and 4 = complete improvement).

Results
Forty-three patients met inclusion criteria. Twenty-seven (63%) were male, 39 (91%) had a history of craniosynostosis, and 18 (42%) had associated syndromes. The mean age at fat grafting was 9.9 years (2.7–20.4, SD = 5.5) with an average follow-up time of 1.6 years (0–5.8, SD = 1.8). The average volume of fat grafted was 8.6 mL (0–30, SD = 5.9) to the right temporal region and 8.6 mL (0–30, SD = 5.8) to the left. There were no intraoperative or postoperative complications. The mean improvement score was 2.9 (1–4, SD = 0.7), demonstrating that most patients experienced moderate to significant improvement. Multiple linear regression analysis demonstrated that syndromic status had a negative impact on the aesthetic outcome (P < 0.001).

Conclusions
These findings demonstrate that fat grafting is an effective method to treat temporal hollowing in children with craniofacial anomalies with no perioperative complications.
Facial Plastic and Reconstructive Surgery During the COVID-19 Pandemic
Authors: Shokri Tom , Saadi Robert A. , Liaw Jeffrey , Bann Darrin V. , Patel Vijay A. , Goyal Neerav , Lighthall Jessyka G. 
Published: June 2020

BackgroundThe global COVID-19 pandemic has had a profound impact on facial plastic and reconstructive surgery. Our review serves as a safety resource based on the current literature and is aimed at providing best-practice recommendations. Specifically, this article is focused on considerations in the management of craniomaxillofacial trauma as well as reconstructive procedures after head and neck oncologic resection.MethodsRelevant clinical data were obtained from peer-reviewed journal articles, task force recommendations, and published guidelines from multiple medical organizations utilizing data sources including PubMed, Google Scholar, MEDLINE, and Google search queries. Relevant publications were utilized to develop practice guidelines and recommendations.ConclusionsThe global COVID-19 pandemic has placed a significant strain on health care resources with resultant impacts on patient care. Surgeons operating in the head and neck are particularly at risk of occupational COVID-19 exposure during diagnostic and therapeutic procedures and must therefore be cognizant of protocols in place to mitigate exposure risk and optimize patient care.
Early Diagnosis and Intervention for Airway-Obstructing Neonatal Plexiform Neurofirbomatosis
Authors: Day Kristopher M. , Roward Simin , Gillispie Alexandra , Attra James , Kelley Patrick 
Published: June 2020

Background:Respiratory distress is a frequent occurrence in neonates, typically caused by a variety of pulmonary conditions. Accurate diagnosis of the cause is vital to appropriately treat neonates and prevent long-term complications. Neck masses rarely cause respiratory distress in this setting but should be considered when clinical signs indicate.Methods:The authors present the patient with a neonate born at term who developed stertor, respiratory distress requiring intubation, and repeated failure to extubate.Results:Physical examination showed right-sided lower and midface enlargement with a firm mass mostly over the parotid and right neck. Both computerized and magnetic resonance tomography demonstrated a right-sided neck mass. Surgical exploration revealed extensive tumor burden emanating from the great auricular, hypoglossal, and other nerves of the neck, including invasion of the carotid sheath encasing the artery. Excisional biopsy showed plexiform neurofibroma, and pathognomonic for neurofibromatosis type 1. The decision was made to pursue medical management, as complete excision would have resulted in increased morbidity due to the involvement of multiple cranial nerves. The patient underwent microlaryngoscopy, bronchoscopy, and tracheostomy and was started on Trametinib chemotherapy.Conclusion:Neonatal airway obstruction can rarely be caused by unanticipated mass lesion, such as plexiform neurofibroma. A high index of suspicion must be maintained for early onset mass lesions causing respiratory obstruction to inhibit early disease progression and avoid potentially fatal sequelae.
Lingual Lateral Canal Mimicking Mandible Fracture
Authors: Zambrana Jéssica Rabelo Mina , Carneiro Ana Luiza Esteves , Zambrana Nataly Rabelo Mina , Neto Henrique Tuzzolo , Salgado Daniela Miranda Richarte de Andrade , Ribeiro Rodrigo Alves , Costa Claudio 
Published: June 2020

Abstract
Lingual lateral canal is a rare variation in the trajectory of the mandibular canal, and is usually detected as an incidental finding on radiographic exams, especially on cone-beam computed tomography. Due to its radiographic characteristics, this anatomical variation might be confused with a mandible fracture. Therefore, the knowledge of its presence is essential not only to differentiate it from a fracture, but also for the success of surgical procedures performed in the mandible, making it easier to avoid clinical complications such as swelling, bruising, bleeding, and neurovascular disorders. This report shows the case of a lingual lateral canal mimicking a fracture on the mandibular body.
Optical Coherence Tomography Findings Following Orbital Fractures
Authors: De Cillà Stefano , Muraca Andrea , Brucoli Matteo , Boffano Paolo , Benech Arnaldo 
Published: June 2020

Introduction:
A closed globe trauma is often associated with orbital wall fractures. In addition to diplopia, further eye disturbances can be observed. The aim of this preliminary prospective study was to investigate the optical coherence tomography (OCT) findings in patients that presented with orbital fractures in association with diplopia but without decreased visual acuity or further ocular symptoms.

Methods:
Only patients who were admitted and surgically treated for orbital wall fractures and that presented diplopia were included in this study. Patients with post traumatic ocular symptoms were excluded. All the patients that were diagnosed with an orbital fracture underwent an ophthalmological assessment, including best-corrected visual acuity (BCVA), examination of fundus oculi, Hess Scheme, OCT, and OCT angiography.

Results:
Five patients met the inclusion criteria and were enrolled. Hess Scheme examinations confirmed the presence of diplopia in all the included patients. The examination of fundus oculi did not reveal any pathology in 4 patients, whereas in a patient a commotio retinae (Berlin's Edema) was diagnosed in the infero-papillary field. Following OCT assessment, a pachycoroid was observed in 4 patients.

Conclusions:
A thorough ophthalmological assessment in patients that have suffered from orbital fractures is fundamental. In particular, OCT examinations seem to provide more insight into the detection and monitoring of choroidal changes after ocular trauma without visible macular changes.
Increased Intraoperative Faculty Entrustment and Resident Entrustability Does Not Compromise Patient Outcomes After General Surgery Procedures
Authors: Williams Aaron M. , Karmakar Monita , Thompson-Burdine Julie , Matusko Niki , Ji Sunjong , Kamdar Neil , Seiler Kristian , Minter Rebecca M. , Sandhu Gurjit 
Published: June 2020

Background:
Intraoperative resident autonomy has been compromised secondary to expectations for increased supervision without defined parameters for safe progressive independence, diffusion of training experience, and more to learn with less time. Surgical residents who are insufficiently entrusted during training attain less autonomy, confidence, and even clinical competency, potentially affecting future patient outcomes.

Objective:
To determine if OpTrust, an educational intervention for increasing intraoperative faculty entrustment and resident entrustability, negatively impacts patient outcomes after general surgery procedures.

Methods:
Surgical faculty and residents received OpTrust training and instruction to promote intraoperative faculty entrustment and resident entrustability. A post-intervention OpTrust cohort was compared to historical and pre-intervention OpTrust cohorts. Multivariable logistic and negative binomial regression was used to evaluate the impact of the OpTrust intervention and time on patient outcomes.

Setting:
Single tertiary academic center.

Participants:
General surgery faculty and residents.

Main Outcome(s) and Measure(s):
Thirty-day postoperative outcomes, including mortality, any complication, reoperation, readmission, and length of stay.

Results:
A total of 8890 surgical procedures were included. After risk adjustment, overall patient outcomes were similar. Multivariable regression estimating the effect of the OpTrust intervention and time revealed similar patient outcomes with no increased risk (P > 0.05) of mortality {odds ratio (OR), 2.23 [95% confidence interval (CI), 0.87–5.6]}, any complication [OR, 0.98 (95% CI, 0.76–1.3)], reoperation [OR, 0.65 (95% CI, 0.42–1.0)], readmission [OR, 0.82 (95% CI, 0.57–1.2)], and length of stay [OR, 0.99 (95% CI, 0.86–1.1)] compared to the historic and pre-intervention OpTrust cohorts.

Conclusions:
OpTrust, an educational intervention to increase faculty entrustment and resident entrustability, does not compromise postoperative patient outcomes. Integrating faculty and resident development to further enhance entrustment and entrustability through OpTrust may help facilitate increased resident autonomy within the safety net of surgical training without negatively impacting clinical outcomes.
Strategy to Enable and Accelerate Kidney Transplant in Small Children and Results of the First 130 Transplants in Children ≤15 kg in a Single Center
Authors: Feltran Luciana de Santis , Genzani Camila Penteado , Fonseca Mariana Janiques Barcia Magalhaes , da Silva Erica Francisco , Baptista José Carlos , de Carvalho Maria Fernanda Camargo , Koch-Nogueira Paulo Cesar 
Published: June 2020

Background.
Proper care of young children in need of kidney transplant (KT) requires many skilled professionals and an expensive hospital structure. Small children have lesser access to KT.

Methods.
We describe a strategy performed in Brazil to enable and accelerate KT in children ≤15 kg based on the establishment of one specialized transplant center, focused on small children, and cooperating with distant centers throughout the country. Actions on 3 fronts were implemented: (a) providing excellent medical assistance, (b) coordinating educational activities to disseminate expertise and establish a professional network, and (c) fostering research to promote scientific knowledge. We presented the number and outcomes of small children KT as a result of this strategy.

Results.
Three hundred forty-six pediatric KTs were performed in the specialized center from 2009 to 2017, being 130 in children ≤15 kg (38%, being 41 children ≤10 kg) and 216 in >15 kg (62%). Patient survival after 1 and 5 years of the transplant was 97% and 95% in the “small children” group, whereas, in the “heavier children” group, it was 99% and 96% (P = 0.923). Regarding graft survival, we observed in the “small children” group, 91% and 87%, whereas in the “heavier children” group, 94% and 87% (P = 0.873). These results are comparable to the literature data. Groups were similar in the incidence of reoperation, vascular thrombosis, posttransplant lymphoproliferative disease, and estimated glomerular filtration rate.

Conclusions.
The strategy allowed an improvement in the number of KT in small children with excellent results. We believe this experience may be useful in other locations.
Socioeconomic Deprivation Does Not Impact Liver Transplantation Outcome for HCC: A Survival Analysis From a National Database
Authors: Menahem Benjamin , Dejardin Olivier , Alves Arnaud , Launay Ludivine , Lubrano Jean , Duvoux Christophe , Laurent Alexis , Launoy and Guy 
Published: June 2020

Background.
To investigate the value of European deprivation index (EDI) and hepatocellular carcinoma (HCC) characteristics and their relationships with outcome after liver transplantation (LT).

Methods.
Patients undergoing LT for HCC were included from a national database (from “Agence de la Biomédecine” between 2006 and 2016. Characteristics of the patients were blindly extracted from the database. Thus, EDI was calculated in 5 quintiles and prognosis factors of survival were determined according to a Cox model.

Results.
Among the 3865 included patients, 33.9% were in the fifth quintile (quintile 1, N = 562 [14.5%]; quintile 2, N = 647 [16.7%]; quintile 3, N = 654 [16.9%]; quintile 4, N = 688 [17.8%]). Patients in each quintile were comparable regarding HCC history, especially median size of HCC, number of nodules of HCC and alpha-fetoprotein score. In the univariate analysis of the crude survival, having >2 nodules of HCC before LT and time on waiting list were associated with a higher risk of death (P < 0.0001 and P = 0.03, respectively). EDI, size of HCC, model for end-stage liver disease score, Child-Pugh score were not statistically significant in the crude and net survival. In both survival, time on waiting list and number of HCC ≥2 were independent factor of mortality after LT for HCC (P = 0.009 and 0.001, respectively, and P = 0.03 and 0.02, respectively).

Conclusions.
EDI does not impact overall survival after LT for HCC. Number of HCC and time on waiting list are independent prognostic factors of survival after LT for HCC.
Hidden in plain sight: The effects of BCG vaccination in COVID-19 pandemic
Authors: Toraih Eman Ali , Sedhom Jessica Ashraf , Dokunmu Titilope Modupe , Hussein Mohammad Hosny , Ruiz Emmanuelle ML , Muthusamy Kunnimalaiyaan , Zerfaoui Mourad , Kandil Emad 
Published: June 2020

AbstractTo investigate the relationship between BCG vaccination and SARS-CoV-2 by bioinformatic approach. Two datasets for Sars-CoV-2 infection group and BCG-vaccinated group were downloaded. Differentially Expressed Genes were identified. Gene ontology and pathways were functionally enriched, and networking was constructed in NetworkAnalyst. Lastly, correlation between post-BCG vaccination and COVID-19 transcriptome signatures were established. A total of 161 DEGs (113 upregulated DEGs and 48 downregulated genes) were identified in the Sars-CoV-2 group. In the pathway enrichment analysis, cross-reference of upregulated KEGG pathways in Sars-CoV-2 with downregulated counterparts in the BCG-vaccinated group, resulted in the intersection of 45 common pathways, accounting for 86.5% of SARS-CoV-2 upregulated pathways. Of these intersecting pathways, a vast majority were immune and inflammatory pathways with top significance in IL-17, TNF, NOD-like receptors, and NF-κB signaling pathways. Our data suggests BCG-vaccination may incur a protective role in COVID-19 patients until a targeted vaccine is developed.Supplementary Materials(https://drive.google.com/open?id=15Na738L282XNaQAJUh0cZf1WoG9jJfzJ)
Phase separation induced by cohesin SMC protein complexes
Authors: Ryu Je-Kyung , Bouchoux Celine , Liu Hon Wing , Kim Eugene , Minamino Masashi , de Groot Ralph , Katan Allard J. , Bonato Andrea , Marenduzzo Davide , Michieletto Davide , Uhlmann Frank , Dekker Cees 
Published: June 2020

AbstractCohesin is a key protein complex that organizes the spatial structure of chromosomes during interphase. Here, we show that yeast cohesin shows pronounced clustering on DNA in an ATP-independent manner, exhibiting all the hallmarks of phase separation.In vitrovisualization of cohesin on DNA shows DNA-cohesin clusters that exhibit liquid-like behavior. This includes mutual fusion and reversible dissociation upon depleting the cohesin concentration, increasing the ionic strength, or adding 1,6-hexanediol, conditions that disrupt weak interactions. We discuss how bridging-induced phase separation can explain the DNA-cohesin clustering through DNA-cohesin-DNA bridges. We confirm that,in vivo,a fraction of cohesin associates with chromatin in yeast cells in a manner consistent with phase separation. Our findings establish that SMC proteins can exhibit phase separation, which has potential to clarify previously unexplained aspects ofin vivoSMC behavior and constitute an additional principle by which SMC complexes impact genome organization.One sentence summaryYeast cohesin complex is observed to phase separate with DNA into liquid droplets, which it accomplishes by ATP-independent DNA bridging.
Pathways of inter- and intrasubunit allosteric signaling in the C-linker disk and cyclic nucleotide-binding domain of HCN2 channels
Authors: Pfleger Christopher , Kusch Jana , Kondapuram Mahesh , Schwabe Tina , Sattler Christian , Benndorf Klaus , Gohlke Holger 
Published: June 2020

AbstractOpening of hyperpolarization-activated cyclic nucleotide-modulated (HCN) channels is controlled by membrane hyperpolarization and binding of cyclic nucleotides to the tetrameric cyclic nucleotide-binding domain (CNBD), attached to the C-linker disk (CL). Confocal patch-clamp fluorometry revealed a pronounced cooperativity of ligand binding among protomers. However, by which pathways allosteric signal transmission occurs remained elusive. Here, we investigate how changes in the structural dynamics of the CL- CNBD of mouse HCN2 upon cAMP binding relate to inter- and intrasubunit signal transmission. Applying a rigidity theory-based approach, we identify two intersubunit and one intrasubunit pathways that differ in allosteric coupling strength between cAMP binding sites or towards the CL. These predictions agree with results from electrophysiological and patch-clamp fluorometry experiments. Our results map out distinct routes within the CL-CNBD that modulate different cAMP binding responses in HCN2 channels. They signify that functionally relevant submodules may exist within and across structurally discernable subunits in HCN channels.
Differential effect of anesthesia on visual cortex neurons with diverse population coupling
Authors: Lee Heonsoo , Tanabe Sean , Wang Shiyong , Hudetz Anthony G. 
Published: June 2020

AbstractIntroductionFiring rate (FR) and population coupling (PC) are intrinsic properties of cortical neurons. Neurons with different FR and PC have diverse excitability to stimulation, tuning curve, and synaptic plasticity. Therefore, investigation of the effect of anesthesia on neurons with different FR and PC would be important to understand state-dependent information processing in neuronal circuits.MethodsTo test how anesthesia affects neurons with diverse PC and FR, we measured single-unit activities in deep layers of primary visual cortex at three levels of anesthesia with desflurane and in wakefulness. Based on PC and FR in wakefulness, neurons were classified into three distinct groups: high PC-high FR (HPHF), low PC-high FR (LPHF), and low PC-low FR (LPLF) neurons.ResultsApplying repeated light flashes as visual stimuli, HPHF neurons showed the strongest early response (FR at 20-150ms post-stimulus) among the three groups, whereas the response of LPHF neurons persisted longest (up to 440ms). Anesthesia profoundly altered PC and FR, and differently affected the three neuron groups: (i) PC and FR became strongly correlated suppressing population-independent spike activity; (ii) Pairwise correlation of spikes between neurons could be predicted by a PC-based raster model suggesting uniform neuron-to-neuron coupling; (iii) Contrary to evoked-potential studies under anesthesia, the flash-induced early response of HPHF neurons was attenuated, and their spike timing was split and delayed; (iv) Late response (FR at 200-400ms post-stimulus) was suppressed both in HPHF and LPHF neurons.ConclusionsAnesthetic-induced association between PC and FR suggests reduced information content in the neural circuit. Altered response of HPHF neurons to visual stimuli suggests that anesthesia interferes with conscious sensory processing in primary sensory cortex.