bluuebunny/crossref_metadata_2025_split huggingface dataset:
ENHANCED S-CONE SYNDROME: VISUAL FUNCTION, CROSS-SECTIONAL IMAGING, AND CELLULAR STRUCTURE WITH ADAPTIVE OPTICS OPHTHALMOSCOPY
Authors: Ammar Michael J. , Scavelli Kurt T. , Uyhazi Katherine E. , Bedoukian Emma C. , Serrano Leona W. , Edelstein Ilaina D. , Vergilio Grace , Cooper Robert F. , Morgan Jessica I. W. , Kumar Priyanka , Aleman Tomas S. 
Published: July 2019

Purpose:
To describe in detail the phenotype of a patient with enhanced S-cone syndrome.

Methods:
We describe a 13-year-old boy who presented with blurred vision, vitreous cells, cystoid macular edema refractory to steroid treatment, and a negative uveitic workup. The patient underwent a complete ophthalmic examination, full-field electroretinograms (ffERG), automatic static perimetry and multimodal imaging with spectral domain optical coherence tomography, and adaptive optics scanning laser ophthalmoscopy (AOSLO).

Results:
Spectral domain optical coherence tomography demonstrated cystoid macular edema and a hyperthick, delaminated midperipheral retina. Fluorescein angiography did not demonstrate macular leakage. Rod-mediated ffERGs were undetectable, and there was a supernormal response to short-wavelength stimuli compared with photopically matched longer wavelengths of light consistent with enhanced S-cone syndrome. Gene screening was positive for compound heterozygous mutations NR2E3: a known (c.119-2 A>C) and a novel (c.119-1G>A) mutation. By perimetry, sensitivities were normal or above normal for short-wavelength stimuli; there was no detectable rod-mediated vision. AOSLO demonstrated higher than normal cone densities in the perifoveal retina and evidence for smaller outer segment cone diameters.

Conclusion:
Evidence for supernumerary cones (at least twice the normal complement) by AOSLO and spectral domain optical coherence tomography was associated with supernormal S-cone sensitivities and electroretinogram responses confirming previous in vivo findings in postmortem human specimens. Smaller than normal cones in enhanced S-cone syndrome may represent “hybrid” photoreceptors analogous to the rd7/rd7 murine model of the disease.
Spatial Analysis of Corneal Densitometry, Thickness Profile, and Volume Distribution After Uneventful Descemet Membrane Endothelial Keratoplasty
Authors: Lazaridis Apostolos , Giallouros Elefterios , Sekundo Walter , Schroeder Frank Michael , Sklavos Sokratis , Droutsas Konstantinos 
Published: July 2019

Purpose:
To present normative corneal densitometry (CD), thickness (CT), and volume (CV) after uneventful Descemet membrane endothelial keratoplasty (DMEK).

Methods:
Medical records of 212 consecutive surgeries were reviewed. One hundred cases without intraoperative and postoperative complications were included (group 1). Scheimpflug CD of the optically relevant 0- to 2-mm zone and 2- to 6-mm annulus, CT at the 2-, 4-, and 6-mm rings, and CV at the 3-, 5-, and 7-mm zones were evaluated preoperatively and at 3, 6, 12, and 24 months postoperatively. The 24-month results were compared with an age-matched group of uncomplicated pseudophakic eyes (group 2; n = 20) and a group of healthy, young subjects (group 3; n = 30).

Results:
Total CD at 0 to 2 mm was 33.9 ± 10.7 grayscale units preoperatively and decreased to 20.3 ± 4.7 at 24 months (P < 0.001), being significantly higher compared with both control groups (P ≤ 0.026). Total CD at 2 to 6 mm was 27.9 ± 8.1 preoperatively and decreased to 20.2 ± 4.6 at 24 months (P < 0.001), showing no statistically significant difference compared with group 2 (P = 0.061). At 24 months, group 1 showed a similar central CT, CT at the 2-mm and 4-mm rings compared with group 2 (P ≥ 0.440); however, CT at 6 mm increased significantly (P = 0.008). CV at 3, 5, and 7 m decreased significantly at 24 months (P < 0.001), showing no significant differences compared with both control groups (P ≥ 0.122).

Conclusions:
Twenty four months after uneventful DMEK, CT within the central 4-mm zone and CV at the 3-, 5-, and 7-mm zones were similar to uncomplicated pseudophakic eyes. The total CD at the 0- to 2-mm zone and midperipheral CT at the 6-mm ring were higher compared with the pseudophakic controls.
Clinical Outcomes of a New Asymmetric Intracorneal Ring Segment for the Treatment of Keratoconus
Authors: Vega-Estrada Alfredo , Chorro Elisabet , Sewelam Ashraf , Alio Jorge L. 
Published: July 2019

Purpose:
To report visual, refractive, and corneal aberrations after implantation of a new asymmetric intracorneal ring segment (ICRS).

Methods:
This was a prospective, multicenter clinical study including 30 eyes of 26 patients with keratoconus. All cases were implanted with an ICRS, named the VISUMRING (VR), which had an arc length of 353 degrees and 2 asymmetric sections that can be customized in base width, length, and thickness. Ophthalmic evaluation included uncorrected distance visual acuity (UDVA), corrected distance visual acuity (CDVA), refraction, topography, and anterior corneal aberrations using the Sirius System (CSO, Firenze, Italy). Mean follow-up period was 14.7 ± 7.9 months.

Results:
Significant improvement of both UDVA and CDVA was observed after 1 year. UDVA improved from 0.08 ± 0.22 to 0.22 ± 0.16 and CDVA from 0.24 ± 0.29 to 0.43 ± 0.18 (P = 0.01). A significant reduction of more than 7 D in the spherical equivalent from −12.38 ± 3.77 D to −5.00 ± 3.26 D (P < 0.05) was noted 1 year after the procedure. In terms of higher-order aberrations, a slight reduction that was not statistically significant in the higher-order and coma-like aberrations was noted at 1 year from 4.32 and 3.82, to 4.12 and 3.55, respectively. Regarding complications, 5 of the 30 cases needed to have the VR explanted throughout the follow-up period due to severe corneal melting.

Conclusions:
VR ICRS improves vision and refraction and induces major corneal flattening in patients with keratoconus. Further design enhancement is needed to increase the reduction of the asymmetric corneal aberrations and reduce the extrusion rate.
Role of Pattern Electroretinogram in Ocular Hypertension and Early Glaucoma
Authors: Turkey Eman , Elsanabary Zeinab S.E. , Elshazly Laila Hassan M. , Osman Moataz H. 
Published: July 2019

Purpose:
To test whether pattern electroretinogram (PERG) can early detect retinal ganglion cells dysfunction in ocular hypertension.

Design:
Cross-sectional observational study.

Participants:
The study included 3 groups: control, primary open-angle glaucoma (POAG) and ocular hypertension (OHT) groups with 30 eyes in each group.

Materials and Methods:
Visual fields were examined using automated perimetry with central 24-2 program. Optical coherent tomography (OCT) was done to assess the neuroretinal rim area, vertical cup/disc ratio, and average superior and inferior retinal nerve fiber layer thickness. PERG was recorded using skin electrodes. Amplitude and latency of P50 and N95 were documented.

Results:
PERG data: the mean P50 and N95 latency were significantly higher in the POAG group and the OHT group compared with the control group (P<0.001, <0.001, respectively). Also, the mean P50 and N95 amplitude were significantly lower in the POAG group and the OHT group compared with the control group (P<0.001,<0.001, respectively). In the POAG group, there was a significant negative correlation between PSD on one hand and P50 amplitude (r=−0.620, P=0.001) and N95 amplitude (r=−0.61, P<0.001) on the other hand. Also, the mean deviation was positively correlated with P50 amplitude (r=0.51, P=0.007) and N95 amplitudes (r=0.50, P=0.002). However, there was no significant correlation between PERG parameters and OCT parameters. In the OHT group, PERG parameters did not correlate with visual field and OCT parameters.

Conclusions:
PERG can detect the dysfunctional, but still live retinal ganglion cells earlier than OCT in OHT cases, allowing the early start of treatment that can restore the ganglion cell function before irreversible damage occurs.
High-dose Corticosteroids for Adult Bell's Palsy: Systematic Review and Meta-analysis
Authors: Fujiwara Takashi , Namekawa Motoki , Kuriyama Akira , Tamaki Hisanobu 
Published: July 2019

Objectives:
To compare the efficacy and safety of high-dose corticosteroids (initial prednisolone [PSL] of 100 mg or more daily) and standard-dose corticosteroids (initial PSL of 50–60 mg) in patients with Bell's palsy.

Study Design:
A systematic review and meta-analysis.

Data Sources:
Medline, Embase, Cochrane Central Register of Controlled Trials, Ichushi-Web, Web of Science, and CINAHL, combined with data from ClinicalTrials.gov.

Study Selection:
Published and unpublished cohort studies comparing high- and standard-dose corticosteroids in adult patients with Bell's palsy were included.

Data Extraction:
Study characteristics (study design, patient's number), patient characteristics (sex, age, disease severity, prescription of antivirals), and outcomes (nonrecovery, any adverse effects).

Data Synthesis:
From the 1,974 identified articles, 8 studies were met eligible criteria. Of the included studies, the initial dose in high-dose corticosteroids regimens varied from 120 mg to 200 mg PSL daily. Compared with standard-dose corticosteroids, high-dose corticosteroids were associated with a significantly decreased nonrecovery at 6 months after disease onset (odds ratio 0.42, 95% confidence interval 0.22–0.80; very low quality) in patients with Bell's palsy. No severe adverse effects were observed in patients receiving high- or standard-dose corticosteroids.

Conclusion:
High-dose corticosteroids reduce nonrecovery in patients with Bell's palsy. The dose of high-dose corticosteroids was varied and further prospective study is needed to identify an adequate dose of corticosteroids in these patients.
Serum Levels of Chemerin, Apelin, and Adiponectin in Relation to Clinical Symptoms, Quality of Life, and Psychological Factors in Irritable Bowel Syndrome
Authors: Baram Mohammad A. , Abbasnezhad Amir , Ghanadi Koroush , Anbari Khatereh , Choghakhori Razieh , Ahmadvand Hassan 
Published: July 2019

Background:
Adipokines have endocrine roles in metabolism and immunity. Dysregulation of adipokine levels is associated with several diseases with chronic inflammation. We aimed to assess the serum concentrations of chemerin, apelin, and adiponectin in irritable bowel syndrome (IBS). Furthermore, we evaluated the possible association of these adipokines with clinical symptoms, quality of life (QoL), and psychological factors.

Materials and Methods:
In this case-control study, 114 male and female IBS patients were recruited from outpatient clinics. Along with the IBS patients, 114 sex and age-matched healthy volunteers were recruited. Patients filled in the questionnaires of the IBS severity scoring system (IBSSS), gastrointestinal (GI) and somatic symptoms, IBS specific QoL (IBS-QoL), and psychological disorders, and went to the lab for blood sampling.

Results:
Serum levels of both adiponectin and apelin were significantly (P=0.04, 0.03, respectively) lower, whereas chemerin was significantly (P=0.01) higher in IBS patients. Chemerin was higher in IBS-D compared with both IBS-C and IBS-A, while apelin and adiponectin were not different between subtypes. After adjustments for confounders only, chemerin had a positive association with IB severity scoring system and GI symptoms. Furthermore, chemerin had positive associations, whereas apelin and adiponectin had inverse associations with somatic symptoms and psychological factors. There were no significant associations between adipokines including chemerin, apelin, and adiponectin, and IBS-QoL.

Conclusions:
Chemerin had significant associations with both the severity of clinical symptoms and psychological factors in IBS; thus, it could be considered as a potential therapeutic target in these patients; however, further studies are needed.
Obesity Does Not Impact Outcomes or Rates of Gastroesophageal Reflux After Peroral Endoscopic Myotomy in Achalasia
Authors: Sanaka Madhusudhan R. , Parikh Malav P. , Subramanium Subanandhini , Thota Prashanthi N. , Gupta Niyati M. , Lopez Rocio , Gabbard Scott , Murthy Sudish , Raja Siva 
Published: July 2019

Background:
Outcomes of laparoscopic Heller myotomy in obese patients with achalasia are suboptimal along with the increased risk of gastroesophageal reflux disease (GERD). The impact of obesity on treatment success and GERD after peroral endoscopic myotomy (POEM) are not well known. Hence, our study aims were to compare the clinical outcomes and rates of GERD after POEM in nonobese versus obese patients with achalasia.

Methods:
Chart review of all achalasia patients who underwent POEM at our institution between April 2014 and June 2018. Patients with timed barium esophagram (TBE) and high-resolution esophageal manometry (HREM) before POEM along with post-POEM TBE, HREM, and esophageal pH study were included. Patients were categorized into 2 groups, nonobese (body mass index <30 kg/m2) and obese (body mass index ≥30 kg/m2). Patient demographics, TBE, HREM, pH study findings, and Eckardt scores were compared between the 2 groups.

Results:
A total of 89 patients (46 nonobese; 43 obese) met the study criteria. There were no significant differences in age, gender, achalasia subtype, operative time, length of stay and complication rates between the 2 groups. Treatment success (Eckardt score ≤3) was similar in both groups (97.7% nonobese vs. 92.7% obese, P=0.35). Abnormal DeMeester scores on pH study (>14.72) were similar in nonobese and obese patients (58.7% vs. 46.5%, P=0.25). Symptomatic GERD was also similar in both groups (17.8% in nonobese vs. 20% in obese, P=0.79).

Conclusions:
POEM is an equally safe and effective treatment option for both nonobese and obese patients with achalasia in the short-term. Interestingly, POEM does not lead to higher rates of GERD in obese compared with nonobese patients.
CHD is Associated With Higher Grades of NAFLD Predicted by Liver Stiffness
Authors: Song Yan , Dang Ying , Wang Ping , Tian Gang , Ruan Litao 
Published: July 2019

Background and Aim:
Accumulating clinical and epidemiologic evidence indicates that nonalcoholic fatty liver disease (NAFLD) is not only associated with liver-related morbidity and mortality, but also with a greater risk of coronary heart disease (CHD). However, there is currently no diagnostic parameter for NAFLD that has been determined to reliably indicate the presence of CHD as a co-morbidity. We evaluated the liver stiffness and visceral fat thickness of NAFLD patients ultrasonographically to explore the relationship between liver stiffness, visceral fat thickness, and CHD, aiming to find explore the relationship between the liver stiffness and CHD.

Methods:
We enrolled 120 consecutive patients who had been initially diagnosed with CHD on the basis of their symptoms. All patients underwent coronary angiography or computed tomography angiography, and were classified into a CHD group and a non-CHD group on the basis of the results. All patients underwent liver ultrasonography, shear-wave elastography, and visceral fat thickness measurement.

Results:
NAFLD and visceral fat thickness were significantly positively correlated with CHD and Gensini score (P<0.001). Multivariate regression showed that age, male, cholesterol, liver stiffness, and visceral fat thickness were determinants of CHD. Age, cholesterol, liver stiffness, and visceral fat thickness cut-off points for the prediction of CHD were above 50 years old [area under the curve (AUC): 0.678; sensitivity, 87%; specificity, 42.6%], >3.76 mmol/L (AUC: 0.687; sensitivity, 68.4%; specificity, 64.8%), >6.1 kPa (AUC: 0.798; sensitivity, 50%; specificity, 92.6%), and >7.41 cm (AUC: 0.694; sensitivity, 52.6%; specificity, 87%), respectively. Compared with the use of age, gender, and cholesterol (model 1), the addition of the liver stiffness cut-off to model 1 resulted in a stronger predictive value (P=0.005).

Conclusions:
High-grade NAFLD is more present in symptomatic CHD. The higher degree of liver stiffness in patients with NAFLD, the higher risk of CHD in these NAFLD patients.
Symptomatic posterior fossa and supratentorial subdural hygromas as a rare complication following transarticular screw fixation with posterior wiring for atlantoaxial instability
Authors: Han In-Bo , Choi Un Yong , Shin Dong-Eun , Ropper Alexander E. , Choi Dae-Sung , Ahn Tae-Keun 
Published: July 2019

Abstract

Rationale:
Atlantoaxial transarticular screw fixation has been an effective and appealing method for inducing fusion of the C1-C2 complex. This technique is usually performed with Gallie fusion. In performing Gallie fusion using sublaminar wiring, a major concern is the risk of dural tear associated with passing sublaminar wires through the epidural space. We present the first report on symptomatic symptomatic subdural hygroma (SDH) due to transarticular screw fixation with posterior wiring.

Patients concerns:
A 50-year-old man had sustained dens fracture 20 years ago and presented with severe neck pain following a recent traffic accident. The images showed atlantoaxial instability due to nonunion of the dens fracture and the patient underwent transarticular screw fixation with posterior sublaminar wiring using Gallie technique. When the U-shaped wire was passed under the arch of C1 from inferior to superior, a dural tear and cerebrospinal fluid (CSF) leak occurred. The site of dural tear was repaired by direct application of sutures. The patient was discharged in good condition. Fifteen day after surgery, the patient was readmitted with a history of a progressive headache associated with vomiting and vertigo.

Diagnonsis:
Brain CT and MRI showed bilateral posterior fossa and a right-sided supratentorial SDH.

Interventions:
The patient underwent right occipital burr hole and evacuation of posterior fossa SDH due to deteriorating neurological status.

Outcomes:
The patient's condition gradually improved after the operation and became asymptomatic at 3-year follow-up.

Lessons:
Posterior fossa and supratentorial SDH could occur resulting from any intraoperative dural tear and CSF leakage during posterior cervical spinal surgery. Symptomatic SDH after posterior cervical spinal surgery should be cautiously assessed and treated.

Level of Evidence: 5
Prognostic significance of polyvascular disease in heart failure with preserved left ventricular ejection fraction
Authors: Fujisue Koichiro , Tokitsu Takanori , Yamamoto Eiichiro , Sueta Daisuke , Takae Masafumi , Nishihara Taiki , Oike Fumi , Usuku Hiroki , Ito Miwa , Motozato Kota , Kanazawa Hisanori , Araki Satoshi , Arima Yuichiro , Takashio Seiji , Izumiya Yasuhiro , Suzuki Satoru , Sakamoto Kenji , Kaikita Koichi , Tsujita Kenichi 
Published: July 2019

Abstract
The prognostic significance of systemic atherothrombosis in heart failure (HF) with preserved ejection fraction (HFpEF) remains unclear. This study aimed to investigate the relation between the presence of polyvascular disease (PVD) and cardiovascular outcomes in HFpEF patients.
A total of 510 consecutive HFpEF patients were prospectively observed for up to 1500 days or until occurrence of cardiovascular events. PVD was defined as ≥2 coexistence of coronary artery disease, peripheral arterial disease, and cerebrovascular disease.
Overall, 124 cardiovascular events were observed during follow-up (median: 1430 days). Kaplan–Meier curve showed HFpEF with PVD (n = 84) experienced more cardiovascular events than did those without PVD patients (44.0% vs 20.4%, log-rank: P < .001). Multivariable Cox proportional hazards analysis with significant factors from univariate analysis showed the presence of PVD (hazard ratio [HR]: 2.875, 95% [CI]: 1.894–4.365, P < .001), previous HF hospitalization (HR: 1.578, 95% CI: 1.031–2.414, P = .036), hemoglobin (HR: 0.889, 95% CI: 0.805–0.983, P = .021), serum sodium (HR: 0.946, 95% CI 0.896–1.000, P = .048), ln-BNP (per 1.0, HR: 1.255, 95% CI: 1.055–1.494, P = .010), and E/e’ (HR: 1.047, 95% CI: 1.020–1.075, P < .001) significantly predicted future cardiovascular events. Multivariable Cox hazard analysis with 4 established factors (age, BNP, diabetes mellitus, and previous HF hospitalization) from the I-PRESERVE (Irbesartan in HFpEF) study showed PVD was independently associated with cardiovascular events in HFpEF patients (HR: 2.562, 95% CI: 1.715–3.827, P < .001).
The presence of PVD is significantly associated with cardiovascular events in HFpEF, suggesting the importance of screening PVD in HFpEF.
Disappearance of the blood supply to a giant uterine myoma due to childbirth
Authors: Li Lu-Jing , Liang Xuan-Kun , Wang Xian-Xiang , Zeng Dan , Xu Zuo-Feng 
Published: July 2019

Abstract

Rationale:
Giant uterine myomas may be life-threatening due to pressure effects on the lungs and other contiguous organs.

Patient concerns:
A 32-year-old pregnant Asian woman was admitted to our hospital early in her pregnancy with a pre-pregnancy history of multiple uterine myomas.

Diagnosis:
She was diagnosed with multiple giant uterine myomas in pregnancy.

Interventions:
No intervention was performed on the woman.

Outcomes:
A reduction in tumor size and disappearance of tumor blood supply were seen on conventional and contrast-enhanced ultrasounds (CEUS) on postpartum day 34. Mass volume gradually decreased and no blood flow signals were seen on CEUS during postpartum follow-up.

Lessons:
Childbirth can block the blood supply of giant uterine myomas and reduce mass size. In such cases, childbirth may be considered therapeutic.
The mediating and moderating roles of self-acceptance and self-reported health in the relationship between self-worth and subjective well-being among elderly Chinese rural empty-nester
Authors: Su Hong , Wang Lina , Li Yang , Yu Hong , Zhang Jiayuan 
Published: July 2019

Abstract
This study aims to test the moderation and mediation effects of self-acceptance and self-reported health on self-worth's impact on subjective well-being among elderly Chinese rural empty-nester elderly, and confirm whether self-report health is a moderating variable between self-worth and subjective well-being.
This cross-sectional study was performed from May 2017 to April 2018; the participants were 365 empty-nest elderly adults from rural areas of Chifeng City in Inner Mongolia.
Data were collected with the General information questionnaire, Self-worth questionnaire for adults, Self-acceptance Questionnaire, and Memorial University of New Found land Scale of Happiness. For the analyses, correlations, regressions, and structural equation models were used. Bootstrapping was performed to confirm the mediation effect. Multiple regression analysis was performed to confirm the moderation effect.
Self-worth showed significant correlations with self-acceptance and subjective well-being (all P < .01). Bootstrapping indicated that the mediating role of self-acceptance was statistically significant. And self-reported health moderated the self-worth and subjective well-being association.
Self-acceptance partially mediated the relationship between self-worth and subjective well-being of the rural empty-nest elderly and self-reported health moderated self-worth and subjective well-being association. Consequently, to improve the subjective well-being of the rural empty-nest elderly, self-acceptance and personal health should be the focus.