bluuebunny/crossref_metadata_2025_split huggingface dataset:
Gene Therapy of the Hemoglobinopathies
Authors: Kunz Joachim B. , Kulozik Andreas E. 
Published: September 2020

AbstractSickle cell disease and the ß‐thalassemias are caused by mutations of the ß‐globin gene and represent the most frequent single gene disorders worldwide. Even in European countries with a previous low frequency of these conditions the prevalence has substantially increased following large scale migration from Africa and the Middle East to Europe. The hemoglobin diseases severely limit both, life expectancy and quality of life and require either life‐long supportive therapy if cure cannot be achieved by allogeneic stem cell transplantation. Strategies for ex vivo gene therapy aiming at either re‐establishing normal ß‐globin chain synthesis or at re‐activating fetal γ‐globin chain and HbF expression are currently in clinical development. The European Medicine Agency (EMA) conditionally licensed gene addition therapy based on lentiviral transduction of hematopoietic stem cells in 2019 for a selected group of patients with transfusion dependent non‐ß° thalassemia major without a suitable stem cell donor. Gene therapy thus offers a relevant chance to this group of patients for whom cure has previously not been on the horizon. In this review, we discuss the potential and the challenges of gene addition and gene editing strategies for the hemoglobin diseases.
ANGIOGRAPHIC SMOKESTACK LEAKAGE NOT ASSOCIATED WITH CENTRAL SEROUS CHORIORETINOPATHY
Authors: El Khatib Bahaeddin A. , Noble Carl W. , Chang Luke , Im Lily , Patronas Marena 
Published: September 2020

Purpose:
To report a series of cases with smokestack leakage on fundus fluorescein angiography outside the clinical setting of central serous chorioretinopathy.

Methods:
A multicenter, observational retrospective case series evaluating fundus fluorescein angiography on Topcon and Optos systems.

Results:
Seven patients with neovascularization due to ischemic retinopathy demonstrated a unique smokestack pattern of angiographic leakage. The patients' ages ranged between 44 and 71 years and were seen at 3 academic teaching hospitals in the Washington–Baltimore metropolitan area. Five patients had been diagnosed with proliferative diabetic retinopathy, one with sickle cell ischemic retinopathy, and one with branch retinal artery occlusion; none of the patients had a known history or clinical signs of current or past central serous chorioretinopathy.

Conclusion:
This is the first published case series to the author's knowledge of ischemic retinopathy displaying a smokestack leakage pattern on fundus fluorescein angiography that is classically described with idiopathic central serous chorioretinopathy.
Glaucoma Drainage Device Technique in a Cohort of Experienced Glaucoma Surgeons in Australia and New Zealand
Authors: Lee Graham A. , Craig Jamie E. , Liu Lance , Danesh-Meyer Helen V. 
Published: September 2020

Précis:
Glaucoma drainage devices (GDD) by Australian and New Zealand glaucomatologists are implanted superotemporally under a peribulbar anesthesia without the use of mitomycin C. Intraluminal stents and tube fenestration are utilized and covered with a scleral graft.

Purpose:
To evaluate current practice patterns of surgical techniques for GDD among Australia and New Zealand Glaucoma Society members routinely performing GDD surgery.

Methods:
Survey of surgeons who performed more than 20 GDD in past 5 years.

Results:
Surgeon participation rate was 31/32 (96.8%). The most common surgical techniques were Baerveldt GDD (24/32, 77.4%), superotemporal placement (31/31, 100%), and peribulbar anesthesia (21/31, 67.7%). Mitomycin C antimetabolite was used routinely by 9/31 surgeons (29.0%). Most surgeons employed intraluminal stents (23/31, 74.2%) with tube fenestrations (19/31, 61.3%). GDD was placed behind the recti muscles (27/31, 87.1%) and secured with nylon (8/0, 9/0 or 10/0) by 29/31 (93.6%). Most common sclerostomy techniques for tube insertion was a 23-G needle passed ab externo (18/31, 58.1%). Tube placement was in the sulcus (11/31, 35.5%) for pseudophakic patients. The external portion of the tube was most commonly covered with a full-thickness scleral patch graft (21/31, 67.7%). Majority of surgeons (21/31, 67.7%) reviewed patients 3 to 4 times in the first month.

Conclusions:
Although a wide range of practice patterns for GDD implantation exists among Australia and New Zealand Glaucoma Society surgeons, there are consistent techniques currently in use to optimize patient outcomes. This report can help surgeons seeking to improve outcomes and minimize complications when trialing the different surgical options.
Immune Response Persistence and Safety of a Booster Dose of the Tetravalent Dengue Vaccine in Adolescents and Adults Who Previously Completed the 3-dose Schedule 4–5 Years Earlier in Latin America
Authors: Coronel Diana , García-Rivera Enid J. , Rivera Doris Maribel , Arredondo-García José Luis , Dietze Reynaldo , Perroud Ana Paula , Cortés Margarita , Bonaparte Matthew , Wang Hao , Pagnon Anke , Jantet-Blaudez Frédérique , Peñalosa Luis Andrey Rojas , Dayan Gustavo , Zambrano Betzana , DiazGranados Carlos A. , Noriega Fernando 
Published: September 2020

Background:
We previously described an increased immune response 28 days after a booster dose of the live, attenuated, tetravalent dengue vaccine (CYD-TDV) in healthy adolescents and adults in Latin America (CYD64, NCT02623725). This follow-up study evaluated immune response persistence and safety of a CYD-TDV booster dose up to Month (M) 24 post-booster.

Methods:
This study included 250 participants who previously received 3 primary doses of CYD-TDV in the CYD13 (NCT00993447) and CYD30 (NCT01187433) studies, and who were randomized 4–5 years later to receive a CYD-TDV booster or placebo (3:1). Dengue neutralizing antibodies against the parental dengue virus strains were assessed using the plaque reduction neutralization test (PRNT50) at M6, M12, and M24 post-booster. Post-booster memory B-cell responses were assessed in a subset of participants using the FluoroSpot assay up to M12 post-booster.

Results:
In the CYD-TDV group (n = 187), dengue neutralizing antibody geometric mean titers (GMTs) declined from the peak at day 28 through to M24 for all serotypes. GMTs at M24 were similar to those at pre-booster among baseline dengue seropositives. A similar trend was observed for baseline dengue seronegatives, albeit at a lower magnitude. Previous vaccination-induced detectable B-cell memory responses in seropositives and seronegatives that decreased to pre-booster levels at M12 post-booster. The CYD-TDV booster dose was well-tolerated.

Conclusions:
In baseline dengue seropositives, following a CYD-TDV booster dose administered 4–5 years after primary immunization, dengue neutralizing antibody GMTs and B-cell memory responses peaked in the short-term before gradually decreasing over time. A CYD-TDV booster dose could improve protection against dengue during outbreak periods.
Better Together? An Examination of the Relationship Between Acute Care Hospital Mergers and Patient Experience
Authors: Attebery Timothy , Hearld Larry R. , Carroll Nathan , Szychowski Jeff , Weech-Maldonado Robert 
Published: September 2020

EXECUTIVE SUMMARYThe United States is experiencing another wave of hospital mergers. Whether patients benefit from these mergers, however, remains an open issue for many interested stakeholders. One measure of the potential benefit of hospital mergers is how they affect patient experience. This study used a quasi-experimental design to examine the relationship between hospital mergers and four different Hospital Consumer Assessment of Healthcare Providers and Systems (HCAHPS) ratings (i.e., overall, physician communication, nurse communication, and staff responsiveness). The study findings showed an association between hospitals that experienced a merger and slower growth in HCAHPS scores for two of the four HCAHPS domains (overall and nurse communication) when compared to matching hospitals that did not merge. Findings from this study can guide and inform hospital administrators, health system boards, state and federal government regulators and policymakers, and others across the spectrum of healthcare stakeholders.
The Relationship Between “Leader in LGBT Healthcare Equality” Designation and Hospitals’ Patient Experience Scores
Authors: DiLeo Rita , Borkowski Nancy , O’Connor Stephen J. , Datti Paul , Weech-Maldonado Robert 
Published: September 2020

EXECUTIVE SUMMARY
An increasing number of lesbian, gay, bisexual, and transgender (LGBT) individuals openly acknowledge their identity; however, the fear of discrimination prevents many from seeking healthcare—an issue challenged by a lack of culturally competent LGBT healthcare providers. With more than 4% of American adults identifying as LGBT individuals, greater attention to their needs is imperative to improve care and access for this population. This study examined organizational and market factors associated with hospitals achieving the “Leader in LGBT Healthcare Equality” (Healthcare Equality Index, HEI; HEI Leader) designation and reported patient experience scores. We found that system-affiliated hospitals have 4.16 greater odds and teaching hospitals have 2.86 greater odds of earning the HEI Leader designation compared to nonsystem and nonteaching hospitals, respectively. Governmental hospitals have 2.47 greater odds of achieving HEI Leader status, while for-profit hospitals have 86% lower odds of having HEI Leader status compared to not-for-profit hospitals. Hospitals located in a metropolitan area have 3.19 greater odds of being an HEI Leader. The percentage of minorities and per capita income in a county also demonstrated a positive association with being an HEI Leader, with odds ratios of 1.00 and 1.02, respectively, while lower education was associated with 4% lower odds of being an HEI Leader. The main finding of this study was that HEI Leader-designated hospitals reported significantly higher overall hospital rating patient experience scores (B = 1.785; p ≤ .001) as compared to non-HEI Leader hospitals. As such, participation in the HEI may be viewed as a motivation for hospitals attaining HEI Leader designation.
Monitoring early stage lung disease in cystic fibrosis
Authors: Nissenbaum Claire , Davies Gwyneth , Horsley Alexander , Davies Jane C. 
Published: September 2020

Purpose of review
Early stage lung disease has long been synonymous with infancy and childhood. As diagnosis happens earlier and conventional management improves, we are seeing larger proportions of people with cystic fibrosis (CF) in adolescence and even adulthood with well preserved lung health. The availability of highly effective cystic fibrosis transmembrane conductance regulator modulator drugs for a large proportion of the CF population will impact even further. Transitioning into adult care with ‘normal’ lung function will become more common. However, it is crucial that we are not blasé about this phase, which sets the scene for future lung health. It is well recognized that lung function assessed by spirometry is insensitive to ‘early’ changes occurring in the distal, small airways. Much of our learning has come from studies in infants and young children, which have allowed assessment and optimization of alternative forms of monitoring.

Recent findings
Here, as a group of paediatric and adult CF specialists, we review the evidence base for sensitive physiological testing based on multibreath washout, lung imaging, exercise and activity monitoring, assessment of infection and quality of life measures.

Summary
We seek to emphasise the importance of further work in these areas, as outcome measures become widely applicable to a growing CF population.
Opioids and sleep
Authors: Cutrufello Nicholas J. , Ianus Vlad D. , Rowley James A. 
Published: September 2020

Purpose of review
Summarize the effects of opioids on sleep including sleep architecture, sleep disordered breathing (SDB) and restless legs syndrome.

Recent findings
Opioids are associated with the development of central sleep apnea (CSA) and ataxic breathing. Recent reports suggest that adaptive servo-ventilation may be an effective treatment for CSA associated with opioids.

Summary
Opioids have multiple effects on sleep, sleep architecture and SDB. Although originally described with methadone use, most commonly used opioids have also been shown to affect sleep. In patients on chronic methadone, sleep architecture changes include decreases in N3 and REM sleep. However, in patients with chronic nonmalignant pain, opioids improve sleep quality and sleep time. Opioids, generally at a morphine equivalent dose more than 100 mg/day, are associated with an increased incidence of CSA and ataxic breathing as well as obstructive sleep apnea. Other risk factors may include concomitant use of other medications such as antidepressants, gabapentinoids and benzodiazepines. Opioid-induced CSA can be potentially treated with adaptive servo-ventilation. Finally, opioids are a potential therapeutic option for restless legs syndrome unresponsive to dopamine agonists and other medications. However, use in patients with restless legs syndrome should proceed with caution, taking into account the risk for dependence and development of SDB.
Synergic role of preoperative 18F-fluorodeoxyglucose PET and MRI parameters in predicting histopathological features of endometrial cancer
Authors: Mapelli Paola , Ironi Gabriele , Bergamini Alice , Mangili Giorgia , Rancoita Paola MV , Taccagni Gian Luca , Venturini Elena , Petrone Micaela , Nisi Valerio , Fallanca Federico , De Marzi Patrizia , Colarieti Anna , Candotti Giorgio , Bocciolone Luca , Candiani Massimo , Gianolli Luigi , Picchio Maria , De Cobelli Francesco 
Published: September 2020

Background
The aim of the present study is to explore the correlation between PET and MRI parameters of primary tumour and clinicopathological features and to determine their synergic predictive role in patients with endometrial cancer candidate to surgery.

Methods
Retrospective study including 27 patients with endometrial cancer and preoperative 18F-fluorodeoxyglucose (18F-FDG)-PET and MRI scan. The following parameters, calculated on the primary tumour, were used for analysis: maximum standardized uptake value (SUVmax), SUVmean, metabolic tumour volume (MTV) and total lesion glycolysis (TLG) for PET scans; mean apparent diffusion coefficient (ADCmean) and volume index for MRI scans. FIGO stage, grade, histotype, lymphovascular space invasion (LVSI) and myometrial invasion were the considered clinicopathological features.

Results
MRI volume index was a good predictor for deep myometrial invasion [area under the curve (AUC) = 0.85; P = 0.003] and for LVSI (AUC = 0.74; P = 0.039). A cutoff value of 9.555 for MRI volume index was predictive for deep myometrial invasion (sensitivity = 84.6%; specificity = 76.9%); a cutoff of 12.165 was predictive for LVSI (sensitivity = 69.2%; specificity = 83.3%). A TLG cutoff value of 26.03 was predictive for deep myometrial invasion (sensitivity = 84.6%; specificity = 76.9%). A high-direct correlation was found with MRI volume index (rho = 0.722; P < 0.001); low-direct correlation with SUVmax (rho = 0.484; P = 0.012), SUVmean (rho = 0.47; P = 0.015) and TLG (rho = 0.482; P = 0.013) were identified. The SUVmax/ADCmean ratio showed a low-direct correlation with percentage of myometrial invasion (rho = 0.467; P = 0.016).

Conclusion
Volume index, TLG and SUVmax/ADCmean ratio are associated with deep myometrial invasion. As myometrial invasion is the index used to predict lymph node involvement in endometrial cancer, the synergic use of these imaging parameters may be suggested to predict lymphnodal metastases.
Renography with a semiautomated algorithm for diuretic decision 7 min postradiopharmaceutical administration: a feasibility study
Authors: Bäck Anna-Karin , Savvopoulos Christos , Kero Tanja , Grybäck Per , Geijer Håkan 
Published: September 2020

Objective
The F+10 method for diuretic renography (diuretics given 10 min after the radiopharmaceutical) could be a time-conserving method. This method involves a 30-min dynamic acquisition where diuretics are administered only when necessary by the Nuclear Medicine technologist performing the examination. The purpose of this study was to assess the method’s performance and to discover the optimal threshold of residual activity for a diuretic administration 7 min into the F+10 renography by reprocessing raw data from prior performed examinations with 20-min acquisitions without diuretics.

Methods
Retrospectively, raw data from 320 original examinations of adult patients performed from 2013 to 2015 were reprocessed into 7-min series and categorized as requiring diuretic or not. The diuretic decisions made by an expert panel were used as a reference. A receiver-operating characteristic curve was drawn to assess the optimal cutoff value for the residual renal activity. Sensitivity, specificity, positive and negative predictive values, as well as the Youden J index were calculated.

Result
The experts classified 50% (160 examinations) as in need of diuretics. The receiver-operating characteristic curve demonstrated the theoretical optimal cutoff value at 7 min to be 94% of maximum activity (sensitivity 0.93, specificity 0.81, Youden J index 0.73). A clinically acceptable threshold is suggested to be 85% (sensitivity 0.99, specificity 0.59, Youden J index 0.58).

Conclusion

99mTc-mercaptoacetyltriglycine renography with the F+10 method and the threshold 85% for diuretic decision 7 min into the renography is a feasible and acceptable method in clinical practice.
Preliminary evaluation of 18F-FDG-PET/MRI for differentiation of serous from nonserous pancreatic cystic neoplasms: a pilot study
Authors: Sagheb Soodeh , Metser Ur , Razaz Suzan , Menezes Ravi , Gallinger Steven , Jhaveri Kartik S. 
Published: September 2020

Objectives
The aim of the study was to evaluate preliminary feasibility of 18F-FDG-PET/MRI in differentiation of pancreatic serous cystic neoplasms (SCNs) from non-SCNs.

Methods
From August 2017 to June 2019, 10 patients (3 men, 7 women; mean age, 63 years) previously diagnosed with pancreatic cystic neoplasm underwent simultaneous 18F-FDG-PET/MRI prospectively on an integrated 3-Tesla hybrid PET/MRI scanner. PET images were analyzed visually and semiquantitatively measuring standardized uptake values (SUV) including lesion SUVmax and SUVmean, lesion to pancreas and lesion to liver SUVmax and SUVmean ratio independent of MRI diagnosis. The reference standard for lesion diagnosis was by MRI features and interval follow-up.

Results
Visual assessment of PET images demonstrated uptake in 57% of SCNs. Lesion to liver SUVmax ratio of ≥0.5 showed the highest accuracy (90%) and area under the curve (0.9) followed by lesion SUVmax of ≥1.6 and lesion to pancreas SUVmax ratio of ≥0.77 for diagnosis of SCN. The sensitivity for lesion SUVmax of ≥1.6 was less than two other ones (71 versus 100%). All non-SCNs exhibited SUVmax value less than 1.6 while 33 and 66% demonstrated lesion to liver SUVmax ratio of >0.5 and lesion to pancreas SUVmax ratio of >0.77, respectively. PET/MRI specificity was 67, 100, 67 and 33% through lesion to liver SUVmax ratio, lesion SUVmax, lesion SUVmean and lesion to pancreas SUVmax ratio, respectively, for diagnosis of SCN.

Conclusion
Preliminary results show that PET/MRI utilization is promising for differentiation of pancreatic SCN from non-SCN lesions. This could reduce need for surveillance imaging or avoidance of unnecessary intervention in pancreatic cystic neoplasms with uncertain diagnoses.
A review of thulium-fiber laser in stone lithotripsy and soft tissue surgery
Authors: Enikeev Dmitry , Traxer Olivier , Taratkin Mark , Okhunov Zhamshid , Shariat Shahrokh 
Published: September 2020

Purpose of review
To evaluate emerging evidence and practical applications of thulium-fiber laser (TFL) for genitourinary soft tissue disease and urinary stone disease treatment.

Recent findings
A systematic review was developed using the PubMed, ScienceDirect, Wiley, SpringerLink and Mary Ann Liebert Scopus databases between 2012 and 2020 years, using the PRISMA statement. We analyzed recent publications including in vitro and in humans outcomes of surgery using TFL. This new laser technology can be used in soft tissue diseases and stones present in the genitourinary system. Most of the comparisons are made with the Ho:YAG laser, using completely different settings. Nevertheless, TFL is safe, feasible and effective in the management of urologic diseases, showing superiority to Ho:YAG even in some studies.

Summary
The introduction of pulsed TFL technology has enabled the ablation of stones at rates comparable to or better than currently existing lasers in vitro, while also potentially reducing stone retropulsion. TFL is effective in treating genitourinary soft tissue diseases, including benign prostatic hyperplasia, and early data indicate that it may be effective in the treatment of urinary stone disease. More clinical studies are needed to better understand the indications for this novel technology and clarify its position in the urologic endoscopy armamentarium.
Machine learning in the optimization of robotics in the operative field
Authors: Ma Runzhuo , Vanstrum Erik B. , Lee Ryan , Chen Jian , Hung Andrew J. 
Published: September 2020

Purpose of review
The increasing use of robotics in urologic surgery facilitates collection of ‘big data’. Machine learning enables computers to infer patterns from large datasets. This review aims to highlight recent findings and applications of machine learning in robotic-assisted urologic surgery.

Recent findings
Machine learning has been used in surgical performance assessment and skill training, surgical candidate selection, and autonomous surgery. Autonomous segmentation and classification of surgical data have been explored, which serves as the stepping-stone for providing real-time surgical assessment and ultimately, improve surgical safety and quality. Predictive machine learning models have been created to guide appropriate surgical candidate selection, whereas intraoperative machine learning algorithms have been designed to provide 3-D augmented reality and real-time surgical margin checks. Reinforcement-learning strategies have been utilized in autonomous robotic surgery, and the combination of expert demonstrations and trial-and-error learning by the robot itself is a promising approach towards autonomy.

Summary
Robot-assisted urologic surgery coupled with machine learning is a burgeoning area of study that demonstrates exciting potential. However, further validation and clinical trials are required to ensure the safety and efficacy of incorporating machine learning into surgical practice.
Relationship Between Symptomatic Dimensions and Global Functioning of Non–Help-Seeking Individuals at Risk for Psychosis
Authors: Ayoub Ibrahim Ali , Andrade Julio Cesar , Serpa Mauricio Henriques , Alves Tania Maria , Hortêncio Lucas , Freitas Elder Lanzani , van de Bilt Martinus Theodorus , Rössler Wulf , Gattaz Wagner Farid , Loch Alexandre Andrade 
Published: September 2020

Abstract
This study aims to analyze the relationship between the symptomatic dimensions of psychosis and functioning of individuals at risk for psychosis (ultrahigh risk [UHR]) in a non–help-seeking UHR sample from the general population. The sample is the same as the one used in the Brazilian Subclinical Symptoms and Prodromal Psychosis cohort study. We applied questionnaires of functioning (Global Assessment of Functioning Scale) and symptomatic dimensions (Scale of Prodromal Symptoms). Next, we correlated the symptomatic dimensions with functioning. We found a significant relationship between avolition and uncommon thought content with poor functioning, whereas the remaining symptoms were not as relevant. Poor functioning was most related to avolition, a negative symptom, followed by unusual thought content, a positive symptom.