bluuebunny/crossref_metadata_2025_split huggingface dataset:
Pediatric Inflammatory and Autoimmune Neurologic Disorders at a Tertiary Medical Center
Authors: Curcio Angela M. , Bain Jennifer M. , Beck Erin S. , Vargas Wendy S. 
Published: July 2020

Objectives: To describe the spectrum of pediatric inflammatory neurologic diseases and compare the sensitivity of ancillary testing for these diagnoses. Methods: We analyzed clinical features and outcomes of 98 children with an immune-mediated central nervous system disorder. We compared sensitivities of each diagnostic modality. Results: We identified the following diagnoses: acute cerebellar ataxia (n = 14; 14.3%), acute demyelinating encephalomyelitis (n = 13; 13.3%), multiple sclerosis (MS) (n = 18; 18.4%), anti- N-methyl-d-aspartate receptor encephalitis (anti-NMDAR encephalitis) (n = 15; 15.3%), encephalitis not otherwise specified (n = 12; 12.2%), and “Other” (n = 26; 26.5%). “Other” included acute transverse myelitis, neuromyelitis optica, central nervous system lupus, primary central nervous system vasculitis, Rasmussen encephalitis, opsoclonus myoclonus ataxia syndrome, and clinically isolated syndrome. The mean age of onset of all diagnoses was 7.9 ± 5.5 years. The diagnostic sensitivity of magnetic resonance imaging (MRI) for acute demyelinating encephalomyelitis and multiple sclerosis was 92.3% and 94.4%, respectively. Cerebrospinal fluid was sensitive for multiple sclerosis in 92.3%, where 75% of patients had cerebrospinal fluid oligoclonal bands. Electroencephalogram (EEG) coupled with cerebrospinal fluid studies was highly sensitive for anti-NMDAR encephalitis (100%). EEG was sensitive for acute demyelinating encephalomyelitis and encephalitis not otherwise specified (77.8% and 80%). No diagnostic studies were sensitive for acute cerebellar ataxia. Seventy-three percent of patients with multiple sclerosis had residual deficits. Thirty-six percent of anti-NMDAR encephalitis patients were nonverbal and wheel-chair bound. Conclusions: We found that MRI is useful for detecting multiple sclerosis and acute demyelinating encephalomyelitis, cerebrospinal fluid is helpful in diagnosing multiple sclerosis and anti-NMDAR encephalitis, and EEG is often abnormal in suspected anti-NMDAR encephalitis, acute demyelinating encephalomyelitis, and encephalitis not otherwise specified. Neurologic outcome at follow-up was unfavorable in patients with multiple sclerosis and anti-NMDAR encephalitis.
Morning glory optic nerve in Aicardi syndrome: Report of a case with fluorescein angiography
Authors: Al-Moujahed Ahmad , Callaway Natalia F , Ludwig Cassie A , Rayess Nadim , Wood Edward , Moshfeghi Darius M 
Published: July 2020

Background: Aicardi syndrome is an X-linked condition that is associated with multiple ophthalmic malformations. Here, we report the first published fluorescein angiography (FA) study of a morning glory optic nerve in a patient with Aicardi syndrome and contralateral persistent fetal vasculature (PFV). Case description: A 12-day old full-term baby girl with a normal neurological exam was referred for evaluation of microphthalmia. The posterior segment of the right eye demonstrated chorioretinal lacunae typical of Aicardi syndrome and microphthalmos with a stalk consistent with PFV. The right eye imaging could not be captured due to the severe microphthalmos and cataract, however, fluorescein angioscopy was performed. The left eye demonstrated a morning glory appearing optic disc with peripapillary chorioretinal lacunae. Fluorescein angiography of the eye showed and late staining in the areas of ellipsoid chorioretinal lacunae emanating from the optic nerve and extensive peripapillary staining and late leakage of the optic nerve. Conclusion: Patients with Aicardi syndrome can have morning glory optic nerve anomaly and PFV. Using FA under anesthesia to detect these abnormalities help in estimating the extend of the disease and its complications, which allows for better management of the complications.
Acetabular reconstruction using a composite layer of impacted cancellous allograft bone and cement: minimum 5-year follow-up study
Authors: Garala Kanai , Boutefnouchet Tarek , Amblawaner Rishanthanan , Lawrence Trevor 
Published: July 2020

Background: Revision total hip arthroplasty (rTHA) is often complex and has a less reliable outcome than primary total hip arthroplasty. Acetabular reconstruction remains a challenge because of the variable amount of bone loss. This study describes and evaluates a novel technique of acetabular reconstruction using composite bone grafting. Patients and methods: Between June 2005 and January 2012, 108 consecutive patients underwent revision hip arthroplasty in which the acetabular component was revised using composite bone grafting. Of these, 24 were lost to follow-up leaving 84 in the study with a minimum 5-year follow-up. There were 54 women and 30 men with a mean age of 70.1 (31–91) years. All patients were assessed clinically and radiologically after a minimum of 5 years. The primary outcome measures were rate of re-revision, patient-reported outcomes and radiological evidence of loosening. Results: The mean follow-up after revision surgery was 6.9 years (5–10). At operation, 60 patients had an acetabular defect of AAOS stage 3 or more. There was no evidence of loosening in 69 revision acetabular components; 12 showed evidence of non-progressive loosening and 3 patients underwent a re-revision procedure (2 for infection, 1 recurrent dislocation). The mean postoperative Hip Disability and Osteoarthritis Outcome Score (HOOS) was 89.11/100 (95% CI, 87.8–90.5). Scores were significantly improved in patients with a more extensive preoperative defect ( p  = 0.006). The overall patient satisfaction rate at final follow-up was very high. Conclusions: Composite impaction grafting using a layered cement and bone graft technique can give satisfactory clinical and radiological outcomes in the medium- to long-term.
A single educational intervention on heart failure self-care: Extended follow-up from a multisite randomized controlled trial
Authors: Deek Hiba , Noureddine Samar , Allam Dalia , Newton Phillip J , Davidson Patricia M 
Published: July 2020

Abstract

Background
Heart failure outcomes remain poor, and little is known about the causes and predictors of these outcomes in Lebanon.

Aim
The purpose of this article is to report the causes and predictors of the 6- and 12-month readmission and mortality of previously recruited patients to the Family focused Approach to iMprove Heart Failure care In LebanonQualitY intervention (FAMILY) study.

Methods
A multi-site block randomized controlled trial in three tertiary medical centers in Beirut. Initially, participants were randomized to either the control or the intervention group. The latter group, with their family caregivers, received heart failure self-care resources and an educational intervention on self-care and symptom management during their index admission. Participants from the FAMILY study were followed up with through phone calls for readmission and mortality at 6 and 12 months following their hospital discharge.

Results
A total of 218 (85%) patients were followed up with for this evaluation. There was a significant difference between the intervention group and the control group in terms of mortality at 6 months (n=18 (16%) versus n=36 (33%); p&lt;0.05) and 12 months (n=29 (26%) versus n=45 (42%); p&lt;0.05) post the index discharge. Mortality at 6 and 12 months was associated with aging, lower body mass index scores and readmission at 30 days post the index admission. Results of a logistic regression for mortality at 6 months showed hypertensive etiology of heart failure and 30-day readmission to be the only significant predictors.

Conclusion
A single session intervention was associated with lower mortality, even after an extended period of time, possibly mediated by other variables. Future studies should be powered for such outcomes while also addressing the cultural needs and literacy levels of the patients using multi-session trials and more frequent follow-ups.
Intracranial EEG in the 21st Century
Authors: Jobst Barbara C. , Bartolomei Fabrice , Diehl Beate , Frauscher Birgit , Kahane Philippe , Minotti Lorella , Sharan Ashwini , Tardy Nastasia , Worrell Gregory , Gotman Jean 
Published: July 2020

Intracranial electroencephalography (iEEG) has been the mainstay of identifying the seizure onset zone (SOZ), a key diagnostic procedure in addition to neuroimaging when considering epilepsy surgery. In many patients, iEEG has been the basis for resective epilepsy surgery, to date still the most successful treatment for drug-resistant epilepsy. Intracranial EEG determines the location and resectability of the SOZ. Advances in recording and implantation of iEEG provide multiple options in the 21st century. This not only includes the choice between subdural electrodes (SDE) and stereoelectroencephalography (SEEG) but also includes the implantation and recordings from microelectrodes. Before iEEG implantation, especially in magnetic resonance imaging -negative epilepsy, a clear hypothesis for seizure generation and propagation should be based on noninvasive methods. Intracranial EEG implantation should be planned by a multidisciplinary team considering epileptic networks. Recordings from SDE and SEEG have both their advantages and disadvantages. Stereo-EEG seems to have a lower rate of complications that are clinically significant, but has limitations in spatial sampling of the cortical surface. Stereo-EEG can sample deeper areas of the brain including deep sulci and hard to reach areas such as the insula.  To determine the epileptogenic zone, interictal and ictal information should be taken into consideration. Interictal spiking, low frequency slowing, as well as high frequency oscillations may inform about the epileptogenic zone. Ictally, high frequency onsets in the beta/gamma range are usually associated with the SOZ, but specialized recordings with combined macro and microelectrodes may in the future educate us about onset in higher frequency bands. Stimulation of intracranial electrodes triggering habitual seizures can assist in identifying the SOZ. Advanced computational methods such as determining the epileptogenicity index and similar measures may enhance standard clinical interpretation. Improved techniques to record and interpret iEEG may in the future lead to a greater proportion of patients being seizure free after epilepsy surgery.
Endpoints for randomized controlled clinical trials for COVID-19 treatments
Authors: Dodd Lori E , Follmann Dean , Wang Jing , Koenig Franz , Korn Lisa L , Schoergenhofer Christian , Proschan Michael , Hunsberger Sally , Bonnett Tyler , Makowski Mat , Belhadi Drifa , Wang Yeming , Cao Bin , Mentre France , Jaki Thomas 
Published: July 2020

Background: Endpoint choice for randomized controlled trials of treatments for novel coronavirus-induced disease (COVID-19) is complex. Trials must start rapidly to identify treatments that can be used as part of the outbreak response, in the midst of considerable uncertainty and limited information. COVID-19 presentation is heterogeneous, ranging from mild disease that improves within days to critical disease that can last weeks to over a month and can end in death. While improvement in mortality would provide unquestionable evidence about the clinical significance of a treatment, sample sizes for a study evaluating mortality are large and may be impractical, particularly given a multitude of putative therapies to evaluate. Furthermore, patient states in between “cure” and “death” represent meaningful distinctions. Clinical severity scores have been proposed as an alternative. However, the appropriate summary measure for severity scores has been the subject of debate, particularly given the variable time course of COVID-19. Outcomes measured at fixed time points, such as a comparison of severity scores between treatment and control at day 14, may risk missing the time of clinical benefit. An endpoint such as time to improvement (or recovery) avoids the timing problem. However, some have argued that power losses will result from reducing the ordinal scale to a binary state of “recovered” versus “not recovered.” Methods: We evaluate statistical power for possible trial endpoints for COVID-19 treatment trials using simulation models and data from two recent COVID-19 treatment trials. Results: Power for fixed time-point methods depends heavily on the time selected for evaluation. Time-to-event approaches have reasonable statistical power, even when compared with a fixed time-point method evaluated at the optimal time. Discussion: Time-to-event analysis methods have advantages in the COVID-19 setting, unless the optimal time for evaluating treatment effect is known in advance. Even when the optimal time is known, a time-to-event approach may increase power for interim analyses.
Consumer perception and experience regarding medicine packaging systems: A survey of medicine dispensing for outpatients
Authors: Wiedyaningsih Chairun , Rahmawati Fita , Lukitaningsih Endang 
Published: July 2020

Background Prescription unit packaging (PUP) and unit-of-dose packaging (UDP) are two types of medicine packaging systems utilized in hospital pharmacies across Indonesia. However, UDP is generally not used for outpatients. Objectives The purpose of this study was to investigate consumer perception and experience of the use of medicines in a packaging system for outpatients. Methods A cross-sectional descriptive study with quantitative and qualitative components was performed. A questionnaire about medicine distribution systems, involving packaging and usage was distributed from August to November 2018. During the same time frame, interviews were performed to identify participants’ experiences when using drugs that were distributed and packaged using the PUP system. Key findings: A total of 104 participants completed the questionnaire. Although 52.9% of the participants did not experience difficulties when using medicines distributed in PUP, 68.2% of participants preferred to use medicines in UDP. The reason for their choice of UDP was its practical use (80.8%), particularly if the medicine was taken when traveling (78.9%). Participants reported that UDP was very practical and helpful for geriatric patients, as UDP enables elderly people to use their medicine appropriately. Furthermore, they reported that UDP reminded consumers of the time to take their medicine. The problems with UDP were the number of packages and unfamiliarity. Conclusions Medicine was preferred in UDP by consumers but the model needs to be adapted to outpatients.
The effect of social deprivation on the incidence rate of carpal and cubital tunnel syndrome surgery
Authors: Johnson Nick A. , Darwin Oliver , Chasiouras Dimitrious , Selby Anna , Bainbridge Chris 
Published: July 2020

We sought to establish whether carpal and cubital tunnel syndrome requiring surgery is associated with deprivation in England. Data from 10,496 adult patients who were treated in our hand unit over a 20-year period were reviewed. The Index of Multiple Deprivation was used to measure deprivation from the patients’ postcode. The mean age at surgery in the most deprived three quintiles was significantly lower than in the least deprived two quintiles for carpal tunnel release (55 vs 59 years, respectively) and cubital tunnel release (52 vs 57 years, respectively). The incidence rate was significantly lower for the three least deprived quintiles when compared with the most deprived quintile for both conditions. The incidence rate ratio of the least deprived quintile compared with the most deprived quintile for carpal tunnel release was 0.70 for men and 0.76 for women. The incidence rate ratio of the least deprived quintile compared with the most deprived quintile for cubital tunnel release was 0.79 for men and 0.49 for women. Carpal tunnel and cubital tunnel syndrome requiring surgery is more common in deprived patients and occurs at an earlier age. Level of evidence: IV
Early Failures After Lumbar Discectomy Surgery: An Analysis of 62 690 Patients
Authors: Samuel Andre M. , Morse Kyle , Lovecchio Francis , Maza Noor , Vaishnav Avani S. , Katsuura Yoshihiro , Iyer Sravisht , McAnany Steven J. , Albert Todd J. , Gang Catherine Himo , Qureshi Sheeraz A. 
Published: July 2020

Study Design: Retrospective cohort study. Objective: To determine the rate of early failures (readmission or reoperation for new or recurrent pain/neurological symptoms) within 30 days after lumbar discectomy and identify associated risk factors. Methods: A retrospective cohort study was conducted of patients undergoing lumbar discectomy in the National Surgical Quality Improvement Program database between 2013 and 2017. Rates of readmission for new or recurrent symptoms or reoperation for revision discectomy or fusion within 30 days postoperatively were measured and correlated with risk factors. Results: In total 62 690 patients were identified; overall rate of readmission within 30 days was 3.3%, including 1.2% for pain or neurological symptoms. Populations at increased risk of readmission were those with 3 or more levels of treatment (2.0%, odds ratio [OR] 2.8%, P < .01), age >70 years (1.8%, OR 1.6, P < .01), class 3 obesity (1.5%, OR 1.4, P = .04), and female gender (1.4%, OR 1.2, P = .02). The overall rate of reoperation within 30 days was 2.2%, including 1.2% for revision decompression or lumbar fusion surgery. Populations at increased risk of reoperation were revision discectomies (1.4%, OR 1.7, P < .01) and females (1.1%, OR 1.4, P < 0.01). Extraforaminal discectomies were associated with lower rates of readmission (0.7%, OR 0.6, P = 0.02) and reoperation (0.4%, OR 0.4, P = .01). Conclusions: Early failures after lumbar discectomy surgery are rare. However, certain subpopulations are associated with increased rates of early failure: obesity, multilevel surgery, females, and revision discectomies.
Factor VIII–driven changes in activated factor IX explored by hydrogen-deuterium exchange mass spectrometry
Authors: Freato Nadia , Ebberink Eduard H. T. M. , van Galen Josse , Fribourg Caroline , Boon-Spijker Mariëtte , van Alphen Floris P. J. , Meijer Alexander B. , van den Biggelaar Maartje , Mertens Koen 
Published: July 2020

AbstractThe assembly of the enzyme-activated factor IX (FIXa) with its cofactor, activated factor VIII (FVIIIa) is a crucial event in the coagulation cascade. The absence or dysfunction of either enzyme or cofactor severely compromises hemostasis and causes hemophilia. FIXa is a notoriously inefficient enzyme that needs FVIIIa to drive its hemostatic potential, by a mechanism that has remained largely elusive to date. In this study, we employed hydrogen–deuterium exchange-mass spectrometry (HDX-MS) to investigate how FIXa responds to assembly with FVIIIa in the presence of phospholipids. This revealed a complex pattern of changes that partially overlaps with those changes that occur upon occupation of the substrate-binding site by an active site-directed inhibitor. Among the changes driven by both cofactor and substrate, HDX-MS highlighted several surface loops that have been implicated in allosteric networks in related coagulation enzymes. Inspection of FVIIIa-specific changes indicated that 3 helices are involved in FIXa–FVIIIa assembly. These are part of a basic interface that is also known as exosite II. Mutagenesis of basic residues herein, followed by functional studies, identified this interface as an extended FVIIIa-interactive patch. HDX-MS was also applied to recombinant FIXa variants that are associated with severe hemophilia B. This revealed that single amino acid substitutions can silence the extended network of FVIIIa-driven allosteric changes. We conclude that HDX-MS has the potential to visualize the functional impact of disease-associated mutations on enzyme–cofactor complexes in the hemostatic system.
Methylmercury toxic mechanism related to protein degradation and chemokine transcription
Authors: Lee Jin-Yong , Hwang Gi-Wook , Naganuma Akira , Satoh Masahiko 
Published: July 2020

AbstractMethylmercury is an environmental pollutant that causes neurotoxicity. Recent studies have reported that the ubiquitin-proteasome system is involved in defense against methylmercury toxicity through the degradation of proteins synthesizing the pyruvate. Mitochondrial accumulation of pyruvate can enhance methylmercury toxicity. In addition, methylmercury exposure induces several immune-related chemokines, specifically in the brain, and may cause neurotoxicity. This summary highlights several molecular mechanisms of methylmercury-induced neurotoxicity.
Isolation of TSCD11 Gene for Early Chloroplast Development under High Temperature in Rice
Authors: Fang Guonan , Yang Shenglong , Ruan Banpu , Liu Chaolei , Zhang Anpeng , Jiang Hongzhen , Ding Shilin , Tian Biao , Zhang Yu , Jahan Noushin , Zhu Li , Zhang Guangheng , Dong Guojun , Zhang Qiang , Zeng Dali , Guo Longbiao , Gao Zhenyu , Qian Qian 
Published: July 2020

Abstract
Background
Chloroplasts are essential for photosynthesis and play key roles in plant development. High temperature affects structure of chloroplasts and metabolism in plants. The seryl-tRNA synthetase plays an important role in translation of proteins. Although seryl-tRNA synthetase has been widely studied in microbes and animals, few studies have reported about its role in chloroplast development under high temperature in rice.

Results
In this study, we isolated a novel temperature-sensitive chlorophyll-deficient 11 (tscd11) mutant by ethyl methane sulfonate (EMS) mutagenesis of japonica variety Wuyujing7. The tscd11 mutant developed albino leaves at the 3-leaf stage under high temperature (35 °C), but had normal green leaves under low temperature (25 °C). Consistent with the albino phenotype, impaired chloroplasts, decreased chlorophyll content and increased ROS accumulation were found in the tscd11 mutant at 35 °C. Fine mapping and DNA sequencing of tscd11 revealed a missense mutation (G to A) in the eighth exon of LOC_Os11g39670 resulted in amino acid change (Glu374 to Lys374). The TSCD11 gene encodes a seryl-tRNA synthetase localized to chloroplast. Complementation test confirmed that the point mutation in TSCD11 is responsible for the phenotype of tscd11. TSCD11 is highly expressed in leaves. Compared with the wild type (WT), mutation in TSCD11 led to significant alteration in expression levels of genes associated with chlorophyll biosynthesis, photosynthesis and chloroplast development under high temperature.

Conclusions
TSCD11, encoding a seryl-tRNA synthetase localized to chloroplast, is vital to early chloroplast development at high temperature in rice, which help to further study on the molecular mechanism of chloroplast development under high temperature.