bluuebunny/crossref_metadata_2025_split huggingface dataset:
Factors Associated With Long-term Intraocular Pressure Fluctuation in Primary Angle Closure Disease: The CUHK PACG Longitudinal (CUPAL) Study
Authors: Cheung Carol Y. , Li Sophia Ling , Chan Noel , Wong Mandy Oi-Man , Chan Poemen Pui-Man , Lai Isabel , Baig Nafees , Tan Shaoying , Man Xiaofei , Tang Fangyao , Wang Yu Meng , Tham Clement C. 
Published: June 2018

Purpose:
To determine the demographic, ocular, and systemic factors associated with long-term intraocular pressure (IOP) fluctuation in primary angle closure disease (PACD).

Methods:
This prospective cohort study included 422 PACD eyes from 269 Chinese patients, including 274 primary angle closure glaucoma (PACG) eyes and 152 primary angle closure/primary angle closure suspect (PAC/PACS) eyes. Long-term IOP fluctuation defined as the SD of all IOP measurements over 2 years (at least 5 measurements in total). Chinese patients with PACD were recruited and followed up 3 monthly. Eyes with IOP-lowering surgery or lens extraction performed within the 2-year study period were excluded. Patient demographics, received treatments, ocular biometry, retinal nerve fiber layer thickness, and systemic factors (eg, hypertension, smoking) were evaluated. Generalized estimating equations adjusting for inter-eye correlation were used to determine the associations.

Results:
Eyes with PACG had significantly higher IOP fluctuation than PAC/PACS (2.4±1.2 versus 2.1±0.9 mm Hg; P=0.04). In the multivariate analysis with PACG eyes, higher baseline IOP (P<0.001), greater number of IOP-lowering medications (P<0.001), previous trabeculectomy (P=0.002), and current smoking (P=0.03) were significantly associated with larger IOP fluctuation, whereas diabetes mellitus was associated with lower IOP fluctuation (P=0.03). Among PAC/PACS eyes, younger age group (P<0.001), male sex (P=0.002), and higher baseline IOP (P<0.001) were significantly associated with larger IOP fluctuation.

Conclusions:
PACG eyes have greater IOP fluctuation than PAC/PACS eyes. Certain demographic, ocular, and systemic factors are associated with IOP fluctuation in PACD eyes.
Trabeculectomy Following Failed Ab Interno Gelatin Microstent: Case Series
Authors: Gizzi Corrado , Mohamed-Noriega Jibran , Elkarmouty Ahmed , Scott Andrew 
Published: June 2018

Purpose:
The purpose of this study is to describe the outcomes of mitomycin C (MMC)-augmented trabeculectomy performed following a failed MMC-augmented XEN-45 implantation.

Design:
Retrospective case series.

Methods:
All consecutive cases of MMC-augmented trabeculectomy performed after MMC-augmented XEN-45 in the Glaucoma Service at Moorfields Eye Hospital between April 2015 and June 2017 were retrospectively reviewed.

Results:
We identified 8 patients who had a trabeculectomy following a failed XEN-45 implantation. The median intraocular pressure (IOP) before and after trabeculectomy was 29.0 mm Hg (12 to 45 mm Hg) and 7.5 mm Hg (2 to 12 mm Hg), respectively. At the last follow-up, 6 cases had a single-digit IOP and 2 of them developed hypotony-related complications (1 case of late choroidal detachment and 1 case of hypotony maculopathy). The median number of glaucoma drops and the median visual acuity (logMAR) before and after trabeculectomy were 2.5 (0 to 5), and 0 and 0.17 (0 to 0.3), and 0.3 (0.17 to 0.47), respectively. At the last follow-up, 3 cases showed an avascular bleb.

Conclusions:
Trabeculectomy following a failed XEN-45 implantation seems technically feasible; however, higher than expected number of cases with low IOP and avascular blebs were observed. This might be related to the cumulative effect of MMC in these cases.
Study of the influence of heme oxygenase 1 gene single nucleotide polymorphism (rs2071746) on esophageal varices among patients with cirrhosis
Authors: Ellakany Walid Ismail , Mahmoud MoheyEldin Khaled , Invernizzi Pietro , Mahmoud ElKady Ali , Eldin Fathy Abou Elkheir Hossam , Abdel Haleem Abo Elwafa Reham , Ellakany Ahmed 
Published: June 2018

Introduction
Cirrhosis as a pathological term has some criteria known to be common in all cases of liver cirrhosis. Esophageal varices are portosystemic collaterals arising in the submucosa of the lower esophagus because of portal hypertension. Portal hypertension is defined as hepatic venous pressure gradient greater than 5 mmHg that arises often as a sequelae of mesenchymal dysfunction in a cirrhotic liver. This study was carried out on 120 personnel divided into three groups: group A included 50 cases of liver cirrhosis with esophageal varices, group B consisted of 50 cases of cirrhosis without esophageal varices, and group C had 20 healthy volunteers a control group.

Patients and methods
DNA was extracted from the peripheral blood of the study participants. Genotyping of the HO1 413A>T promoter SNP (rs2071746) was performed using TaqMan SNP genotyping assay according to the manufacturer’s protocol by RQ-PCR.

Results
Patients carrying T allele of HO1 promoter were found to have 5.46-fold increased risk of esophageal varices development than patients with cirrhosis carrying A allele. T allele was significantly higher in cirrhotics with esophageal varices compared with those without esophageal varices (P<0.001). Rates of esophageal varices development in patients with cirrhotic liver were 52, 40, and 8% for genotypes TT, AT, and AA, respectively.

Conclusion
The T allele of heme oxygenase 1 gene SNP polymorphism (rs2071746) is a risk factor for esophageal varices development in cirrhotics.
Approaches to Address Premature Death of Patients When Assessing Patterns of Use of Health Care Services After an Index Event
Authors: Bush Montika , Simpson Ross J. , Kucharska-Newton Anna , Fang Gang , Stürmer Til , Brookhart M. Alan 
Published: June 2018

Background:
Studies of the use of health care after the onset of disease are important for assessing quality of care, treatment disparities, and guideline compliance. Cohort definition and analysis method are important considerations for the generalizability and validity of study results. We compared different approaches for cohort definition (restriction by survival time vs. comorbidity score) and analysis method [Kaplan-Meier (KM) vs. competing risk] when assessing patterns of guideline adoption in elderly patients.

Methods:
Medicare beneficiaries aged 65–95 years old who had an acute myocardial infarction (AMI) in 2008 were eligible for this study. Beneficiaries with substantial frailty or an AMI in the prior year were excluded. We compared KM with competing risk estimates of guideline adoption during the first year post-AMI.

Results:
At 1-year post-AMI, 14.2% [95% confidence interval (CI), 14.0%–14.5%) of beneficiaries overall initiated cardiac rehabilitation when using competing risk analysis and 15.1% (95% CI, 14.8%–15.3%) from the KM analysis. Guideline medication adoption was estimated as 52.3% (95% CI, 52.0%–52.7%) and 53.4% (95% CI, 53.1%–53.8%) for competing risk and KM methods, respectively. Mortality was 17.0% (95%CI, 16.8%–17.3%) at 1 year post-AMI. The difference in cardiac rehabilitation initiation at 1-year post-AMI from the overall population was 0.1%, 1.7%, and 1.9% compared with 30-day survivor, 1-year survivor, and comorbidity-score restricted populations, respectively.

Conclusions:
In this study, the KM method consistently overestimated the competing risk method. Competing risk approaches avoid unrealistic mortality assumptions and lead to interpretations of estimates that are more meaningful.
The Role of Inflammation on Vitamin D Levels in a Cohort of Pediatric Patients With Inflammatory Bowel Disease
Authors: Strisciuglio Caterina , Cenni Sabrina , Giugliano Francesca Paola , Miele Erasmo , Cirillo Grazia , Martinelli Massimo , Vitale Alessandra , Tolone Carlo , Staiano Annamaria , Miraglia Del Giudice Emanuele , Perrone Laura 
Published: June 2018

ABSTRACTObjectives:Existing studies usually do not measure the free vitamin D in pediatric patients with inflammatory bowel disease (IBD) and not consider the effect of inflammation on vitamin D levels. The aim of our study was to evaluate the concentrations of vitamin D–binding protein (VDBP), total and free 25‐hydroxyvitamin‐D (25(OH)D), and to correlate these values with the disease activity markers.Methods:Newly diagnosed children with IBD and a group of healthy controls (HCs) were enrolled. VDBP and total and free 25(OH)D levels were measured by enzyme‐linked immunosorbent assay and compared using the Student t test. In each patient with IBD, the activity scores of disease and the main inflammation markers were correlated to total and free 25(OH)D levels. C‐reactive protein was also measured in the control group, and it was related to VDBP by a linear regression test for all the groups.Results:Fifty‐one consecutive children were enrolled: IBD = 33, HC = 18. Levels of total 25(OH)D were higher in HC than in patients with IBD (P = 0.01). The free/total 25(OH)D ratio was, however, higher in patients with IBD compared to HC (P < 0.001). Finally, levels of VDBP were lower in patients with IBD than in HC (P = 0.001). A significant direct correlation was found between the free/total 25(OH)D ratio and the activity index of disease (r2: 0.17; P = 0.01). Moreover, in patients with IBD and controls we found a significant indirect correlation between VDBP and C‐reactive protein (r2: 0.12; P = 0.01).Conclusions:Inflammation inversely correlates to VDBP concentrations and patients with IBD, despite their deficiency in total 25(OH)D, have normal or even higher levels of free 25(OH)D.
Economic Assessment of Reverse Algorithm Syphilis Screening in a High Prevalence Population
Authors: Buono Sean A. , Basurto-Davila Ricardo , Godwin Hilary A. , Green Nicole M. 
Published: June 2018

Background
More laboratories are screening for syphilis with automated treponemal immunoassays. We compared direct costs and downstream consequences when a local public health laboratory switches from a traditional algorithm (nontreponemal screening) to a reverse algorithm (treponemal screening).

Methods
We created a decision analysis model based on laboratory and surveillance data to estimate the cost-effectiveness of a reverse syphilis-screening algorithm from the perspectives of the Los Angeles County Public Health Laboratory and the Los Angeles County Department of Public Health (laboratory + STD Program costs) in 2015 US dollars.

Results
The estimated total costs for the Department (Public Health Laboratories) were $2,153,225 ($367,119) for the traditional algorithm and $2,197,478 ($239,855) for the reverse algorithm. Reverse algorithm screening was estimated to detect an additional 626 cases of syphilis, 9.7% more than the traditional algorithm. The incremental cost-effectiveness ratio for the reverse algorithm from the Public Health Department's perspective was $39 per additional syphilis case detected. Cost of follow-up, screening test costs, positivity rates, and frequency of repeat infections most affected the cost-effectiveness of reverse algorithm. Costs were significantly higher for the reverse algorithm when the enzyme Immunoassay/chemiluminescence immunoassay screening test cost was the same as the published Centers for Medicaid Services treponemal test cost.

Conclusions
Using the reverse algorithm would have been slightly more expensive for the Los Angeles County Department of Public Health, but would have identified more syphilis cases and would have resulted in lower laboratory costs.
Counterclockwise Craniofacial Distraction Osteogenesis for Tracheostomy-Dependent Children with Treacher Collins Syndrome
Authors: Hopper Richard A. , Kapadia Hitesh , Susarla Srinivas , Bly Randall , Johnson Kaalan 
Published: June 2018

Background:
The craniofacial rotation deformity in Treacher Collins syndrome results in airway compression that is not addressed by isolated mandibular distraction osteogenesis. Our purpose is to present a surgical technique—counterclockwise craniofacial distraction osteogenesis—that improves airway morphology and occlusal rotation in tracheostomy-dependent patients with this condition.

Methods:
All patients underwent subcranial Le Fort II osteotomies with simultaneous mandibular osteotomies, followed by coordinated maxillomandibular distraction with counterclockwise rotation. We reviewed pretreatment, posttreatment, and end-treatment cephalograms. Airway changes were assessed using polysomnography, sleep endoscopy, and direct laryngoscopy. Bivariate statistics were computed to compare pretreatment and posttreatment measures.

Results:
Five subjects (age range, 4.5 to 12.1 years) underwent this new procedure; three had previously undergone mandibular distraction. The average palatal plane rotation was 17 degrees, the effective mandible length increase was 18 mm, and the facial plane relative to skull base rotation was 14 degrees. There was a symmetric 30 percent relapse of rotation with maintained occlusion in the first 9 months of follow-up that then stabilized. Four patients were successfully decannulated following counterclockwise craniofacial distraction osteogenesis following polysomnography. Sleep endoscopy available on two patients demonstrated resolution of the upper airway obstruction.

Conclusions:
Counterclockwise craniofacial distraction osteogenesis provided greater palatal rotation than previous techniques. The resulting improvement in airway anatomy allowed for decannulation in four of five tracheotomized patients. Stability of the counterclockwise rotation is comparable to that of related orthognathic operations, despite substantially greater magnitude.

CLINICAL QUESTION/LEVEL OF EVIDENCE:
Therapeutic, IV.
18F-FDG PET Brain in a Patient With Fatal Familial Insomnia
Authors: Tham Wei Ying , Thian Yee-Liang , Ratnagopal Pavanni , Xie Wanying 
Published: June 2018

Abstract
A 57-year-old woman presented with a 3-month history of cognitive impairment, daytime somnolence, and violent sleep behavior. Her first- and second-degree relatives had similar symptoms prior to their premature deaths. Her MRI scan of the brain showed no significant abnormality. Electroencephalogram showed loss of slow-wave activity. Functional brain imaging performed with 18F-FDG PET was fused with her MRI scans. This demonstrated profound hypometabolism in bilateral thalami and the posterior cingulate cortex, which is pathognomonic for familial fatal insomnia. Hypometabolism in the temporal lobes suggests a long-standing course of the disease. Genetic testing confirmed a mutation of the prion-protein gene (PRNP).
Prognostic Significance of Interim 11C-Methionine PET/CT in Primary Central Nervous System Lymphoma
Authors: Ahn Seo-Yeon , Kwon Seong Young , Jung Sung-Hoon , Ahn Jae-Sook , Yoo Su Woong , Min Jung-Joon , Bom Hee-Seung , Ki So Yeon , Kim Hyeoung-Joon , Lee Je-Jung , Song Sang Yun , Yang Deok-Hwan 
Published: June 2018

Purpose
Primary central nervous system lymphoma (PCNSL) has a poor prognosis. There has been limited study evaluating the role of interim PET/CT in PCNSL. This prospective study investigated the interim response using sequential brain PET/CT with 11C-methionine (11C-MET) to provide prognostic information during the treatment of PCNSL.

Materials and Methods
A total of 26 immunocompetent patients recently diagnosed with PCNSL were evaluated. Brain MRI and 11C-MET PET/CT were performed at the time of diagnosis and after 4 cycles of high-dose methotrexate-based induction chemotherapy. Tumor-to-normal tissue (T/N) ratio and MTV were used to assess the interim response.

Results
All patients had diffuse large B-cell lymphoma. No differences were observed in initial tumor volume or quantitative uptake among the International Extranodal Lymphoma Study Group groups. Higher International Extranodal Lymphoma Study Group risk scores were associated with higher median values for interim MTV and T/N ratios, as well as poor outcomes. After a median follow-up of 21 months, interim 11C-MET PET/CT assessments based on the quantitative T/N ratio and MTV predicted progression-free survival and overall survival, respectively. A high interim T/N ratio was significantly associated with decreased progression-free survival (hazards ratio, 3.68; P = 0.044).

Conclusions
Response assessments based on interim 11C-MET PET/CT could predict the therapeutic outcome of PCNSL.
Multidisciplinary Diagnostic and Surgical Management of Adenocarcinoma Gingival Metastases
Authors: Di Stasio Dario , Montella Marco , Cozzolino Immacolata , Cicciù Marco , Cervino Gabriele , Paparella Renato Silvio , Serpico Rosario , Laino Luigi 
Published: June 2018

Abstract
Colon cancer is the third leading cause of cancer-related mortality worldwide and colon cancer metastases in rare sites, such as the oral cavity, lead to a worse prognosis. Oral metastasis is a rare clinical condition and it represents only the 1% among all oral cavity neoplasms. A multidisciplinary approach is recommended to carry out a correct diagnostic procedure that allows distinguishing between metastatic and primitive lesions of the oral cavity. Quick diagnosis and management are fundamental to take an appropriate action as early as possible, as usually the prognosis in patients with oral metastases of colon carcinoma is poor. Aim of this brief clinical report is to underline how the quick diagnosis and management of gingival lesions can be crucial for the correct management of those uncommon oral diseases and for having a better prognosis of the primary cancer.
Changes in Cervical Lordosis After Orthognathic Surgery in Skeletal Class III Patients
Authors: Andriola Fernando de Oliveira , Kulczynski Fernando Zugno , Deon Pedro Henrique , Melo Denizar Alberto da Silva , Zanettini Leonardo Matos Santolim , Pagnoncelli Rogério Miranda 
Published: June 2018

Abstract
The purpose of this study was to evaluate cervical lordosis and head posture changes using lateral cephalographs after bimaxillary orthognathic surgery for mandibular prognatism by null hypothesis. Twenty-five patients with skeletal class III dentofacial deformities (10 men, 15 women; mean age, 29.28 ± 8.23 years; range 18–48 years) were included in this prospective clinical study. Lateral cephalographs were taken in natural head position (NHP) 2 weeks before and 6 months after orthognathic surgery. The reproducibility of the radiographer's technique of taking radiographs in NHP was investigated using a photographic method and found to be acceptable. All measurements for cervical lordosis (CV1/CV2; CV3/CV7; CV1/CV7), head posture (NSL/OPT; NSL/VER), and other cephalometric values (NSL/Go-Gn; NSL/OCL; Overjet) were repeated 3 times by the same blinded investigator at 2-week intervals and the average values of the 3 measurements were calculated to use in statistical analysis. Intraclass correlation coefficients (ICC) ranged between 0.996 and 1.000, demonstrating a high reliability of the measures. Statistically significant differences were found for CV3/CV7 (P = 0.006) and CV1/CV7 (P = 0.005) and no significant differences were identified in head posture for both cranio-cervical and cranio-vertical angles. The null hypothesis was rejected. Orthognathic surgery resulted in significant cervical lordosis extension, and a tendency for head extension could also be observed.
Premise for Standardized Sepsis Models
Authors: Remick Daniel G. , Ayala Alfred , Chaudry Irshad H. , Coopersmith Craig M. , Deutschman Clifford , Hellman Judith , Moldawer Lyle , Osuchowski Marcin F. 
Published: June 2018

ABSTRACT
Sepsis morbidity and mortality exacts a toll on patients and contributes significantly to healthcare costs. Preclinical models of sepsis have been used to study disease pathogenesis and test new therapies, but divergent outcomes have been observed with the same treatment even when using the same sepsis model. Other disorders such as diabetes, cancer, malaria, obesity, and cardiovascular diseases have used standardized, preclinical models that allow laboratories to compare results. Standardized models accelerate the pace of research and such models have been used to test new therapies or changes in treatment guidelines. The National Institutes of Health mandated that investigators increase data reproducibility and the rigor of scientific experiments and has also issued research funding announcements about the development and refinement of standardized models. Our premise is that refinement and standardization of preclinical sepsis models may accelerate the development and testing of potential therapeutics for human sepsis, as has been the case with preclinical models for other disorders. As a first step toward creating standardized models, we suggest standardizing the technical standards of the widely used cecal ligation and puncture model and creating a list of appropriate organ injury and immune dysfunction parameters. Standardized sepsis models could enhance reproducibility and allow comparison of results between laboratories and may accelerate our understanding of the pathogenesis of sepsis.
BIOLOGICAL SCIENCES: Neuroscience, cell biology MTSS1/Src family kinase Dysregulation Underlies Multiple Inherited Ataxias
Authors: Brown Alexander S. , Meera Pratap , Altindag Banu , Chopra Ravi , Perkins Emma , Paul Sharan , Scoles Daniel R. , Tarapore Eric , Jackson Mandy , Shakkottai Vikram G. , Otis Thomas S. , Pulst Stefan M. , Atwood Scott X. , Oro Anthony E. 
Published: June 2018

AbstractThe genetically heterogeneous Spinocerebellar ataxias (SCAs) are caused by Purkinje neuron dysfunction and degeneration, but their underlying pathological mechanisms remain elusive. The Src family of non-receptor tyrosine kinases (SFK) are essential for nervous system homeostasis and are increasingly implicated in degenerative disease. Here we reveal that the SFK suppressor Missing-in-Metastasis (MTSS1) is a novel ataxia locus that links multiple SCAs. MTSS1 loss results in increased SFK activity, reduced Purkinje neuron arborization, and low basal firing rates, followed by cell death. Surprisingly, mouse models for SCA1, SCA2, and SCA5 show elevated SFK activity, with SCA1 and SCA2 displaying dramatically reduced MTSS1 protein levels through reduced gene expression and protein translation, respectively. Treatment of each SCA model with a clinically-approved Src inhibitor corrects Purkinje basal firing, and delays ataxia progression in MTSS1 mutants. Our results identify a common SCA therapeutic target and demonstrate a key role for MTSS1/SFK in Purkinje neuron survival and ataxia progression.Significance StatementThe Src family of non-receptor tyrosine kinases (SFK) are essential for nervous system function, and may contribute to neurodegeneration. Spinocerebellar ataxias (SCAs) are neurodegenerative diseases where Purkinje neurons fire irregularly and degenerate leading to motor problems. We show that the SFK suppressor Missing-in-Metastasis (MTSS1) is a novel ataxia gene that links multiple SCAs. MTSS1 loss results in increased SFK activity, degenerating Purkinje neurons with low basal firing rates, and cell death. Surprisingly, mouse models for three different SCAs show elevated SFK activity, with SCA1 and SCA2 models displaying dramatically reduced MTSS1 protein levels. Treatment of each SCA model with SFK inhibitor corrects Purkinje basal firing, and delays ataxia progression in MTSS1 mutants. Our results identify a common link among disparate neurodegenerative diseases.