bluuebunny/crossref_metadata_2025_split huggingface dataset:
Congenital Refractory Glaucoma: A New Ophthalmic Association of Kabuki Syndrome and its Management With Glaucoma Drainage Devices
Authors: Bravetti Giorgio E. , Gillmann Kevin , Mansouri Kaweh , Mermoud André 
Published: January 2019

Introduction:
Kabuki syndrome (KS) is a rare congenital and polymalformative condition, traditionally associated with mental retardation, unusual facial features, and skeletal abnormalities. We hereby describe a case of bilateral congenital glaucoma associated with MLL2-mutation KS. To the best of our knowledge, this is the first association of KS with congenital glaucoma.

Case Report:
The patient was a 3-year-old male of North African ethnicity diagnosed with KS and bilateral congenital glaucoma at the age of 3 months and the first child of a nonconsanguineous healthy couple, with no known genetic conditions within the family. The patient was referred to our tertiary glaucoma center with uncontrollable intraocular pressures between 50 and 60 mm Hg in both eyes. Past ophthalmic history included bilateral trabeculectomies and right retinal detachment with phthisis bulbi. We performed left lensectomy combined with Baerveldt tube insertion in the vitreous cavity, revealing extensive posterior synechiae. Postoperatively, he developed intense inflammation with fibrin and vitreous strand formation, requiring subsequent surgeries and subconjunctival injections of betamethasone. Despite these complications, IOP stabilized between 3 and 6 mm Hg.

Discussion:
This case confirms congenital glaucoma as a rare association of KS, and highlights the potential high-risk nature of such cases. In our opinion, the presence of high levels of inflammation perioperatively and postoperatively is an indication for primary glaucoma drainage device surgery given the high risk of bleb scarring resulting in recurrent surgical revisions. In view of these observations, we also advocate the use of intensive postoperative anti-inflammatory therapy.
Adenosine A3 receptor activation inhibits pronociceptive N-type Ca2+ currents and cell excitability in dorsal root ganglion neurons
Authors: Coppi Elisabetta , Cherchi Federica , Fusco Irene , Failli Paola , Vona Alessia , Dettori Ilaria , Gaviano Lisa , Lucarini Elena , Jacobson Kenneth A. , Tosh Dilip K. , Salvemini Daniela , Ghelardini Carla , Pedata Felicita , Di Cesare Mannelli Lorenzo , Pugliese Anna Maria 
Published: January 2019

Abstract
Recently, studies have focused on the antihyperalgesic activity of the A3 adenosine receptor (A3AR) in several chronic pain models, but the cellular and molecular basis of this effect is still unknown. Here, we investigated the expression and functional effects of A3AR on the excitability of small- to medium-sized, capsaicin-sensitive, dorsal root ganglion (DRG) neurons isolated from 3- to 4-week-old rats. Real-time quantitative polymerase chain reaction experiments and immunofluorescence analysis revealed A3AR expression in DRG neurons. Patch-clamp experiments demonstrated that 2 distinct A3AR agonists, Cl-IB-MECA and the highly selective MRS5980, inhibited Ca2+-activated K+ (KCa) currents evoked by a voltage-ramp protocol. This effect was dependent on a reduction in Ca2+ influx via N-type voltage-dependent Ca2+ channels, as Cl-IB-MECA–induced inhibition was sensitive to the N-type blocker PD173212 but not to the L-type blocker, lacidipine. The endogenous agonist adenosine also reduced N-type Ca2+ currents, and its effect was inhibited by 56% in the presence of A3AR antagonist MRS1523, demonstrating that the majority of adenosine's effect is mediated by this receptor subtype. Current-clamp recordings demonstrated that neuronal firing of rat DRG neurons was also significantly reduced by A3AR activation in a MRS1523-sensitive but PD173212-insensitive manner. Intracellular Ca2+ measurements confirmed the inhibitory role of A3AR on DRG neuronal firing. We conclude that pain-relieving effects observed on A3AR activation could be mediated through N-type Ca2+ channel block and action potential inhibition as independent mechanisms in isolated rat DRG neurons. These findings support A3AR-based therapy as a viable approach to alleviate pain in different pathologies.
Pain-related nucleus accumbens function: modulation by reward and sleep disruption
Authors: Seminowicz David A. , Remeniuk Bethany , Krimmel Samuel R. , Smith Michael T. , Barrett Frederick S. , Wulff Andreas B. , Furman Andrew J. , Geuter Stephan , Lindquist Martin A. , Irwin Michael R. , Finan Patrick H. 
Published: January 2019

AbstractThe nucleus accumbens (NAc) has been implicated in sleep, reward, and pain modulation, but the relationship between these functional roles is unclear. This study aimed to determine whether NAc function at the onset and offset of a noxious thermal stimulus is enhanced by rewarding music, and whether that effect is reversed by experimental sleep disruption. Twenty-one healthy subjects underwent functional magnetic resonance imaging scans on 2 separate days after both uninterrupted sleep and experimental sleep disruption. During functional magnetic resonance imaging scans, participants experienced noxious stimulation while listening to individualized rewarding or neutral music. Behavioral results revealed that rewarding music significantly reduced pain intensity compared with neutral music, and disrupted sleep was associated with decreased pain intensity in the context of listening to music. In whole-brain family-wise error cluster-corrected analysis, the NAc was activated at pain onset, but not during tonic pain or at pain offset. Sleep disruption attenuated NAc activation at pain onset and during tonic pain. Rewarding music altered NAc connectivity with key nodes of the corticostriatal circuits during pain onset. Sleep disruption increased reward-related connectivity between the NAc and the anterior midcingulate cortex at pain onset. This study thus indicates that experimental sleep disruption modulates NAc function during the onset of pain in a manner that may be conditional on the presence of competing reward-related stimuli. These findings point to potential mechanisms for the interaction between sleep, reward, and pain, and suggest that sleep disruption affects both the detection and processing of aversive stimuli that may have important implications for chronic pain.
Trajectories of sickness absence and disability pension before and after opioid initiation for noncancer pain: a 10-year population-based study
Authors: Lalic Samanta , Bell J. Simon , Gyllensten Hanna , Gisev Natasa , Friberg Emilie , Ilomaki Jenni , Sluggett Janet K. , Mittendorfer-Rutz Ellenor , Alexanderson Kristina 
Published: January 2019

Abstract
Chronic noncancer pain is a leading cause of sickness absence (SA) and disability pension (DP). The objectives of this study were to identify trajectories of SA/DP before and after strong and weak opioid initiation for noncancer pain and the factors associated with these trajectories. A longitudinal population-based study of 201,641 people (24-59 years) without cancer who initiated opioid analgesics in 2009 in Sweden was conducted. Trajectories of net annual SA/DP days in the 5 years before/after opioid initiation were estimated with group-based trajectory modelling. Multinomial logistic regression was used to estimate odds ratios (ORs) and 95% confidence intervals (CIs) for factors associated with trajectory groups. Among the 6.9% of people initiating strong opioids, 12.5% had persistent high SA/DP (estimated 320 days/year) before and after opioid initiation and 72.9% had persistent low/minimum SA/DP (estimated 30 days/year). Approximately 8.6% of people had increasing SA/DP, and 6.1% had decreasing SA/DP after opioid initiation, although this seemed to reflect continuation of preinitiation patterns. Trajectories were similar at lower SA/DP days/year among those initiating weak opioids. Persistent high SA/DP among strong opioid initiators were associated with ≥5 comorbidities (OR = 8.72, 95% CI 5.61-13.56), ≤9 years of education (OR = 5.83, 95% CI 4.84-7.03), and previous use of antidepressants (OR = 4.57, 95% CI 3.89-5.37) and antipsychotics (OR = 4.49, 95% CI 2.93-6.88). Three-quarters of people initiating opioids for noncancer pain had persistent low/minimum levels of SA/DP 5 years before and after initiation. Increasing and decreasing SA/DP after opioid initiation seemed to reflect a continuation of preinitiation patterns. Our findings highlight the complex range of sociodemographic and medication-related factors associated with persistent SA/DP.
Olfactory Dysfunction in Aging African American and Caucasian Men With HIV: A Pilot Study
Authors: Vance David E. , Cody Shameka L. , Nicholson Chance , Cheatwood John , Morrison Shannon , Fazeli Pariya L. 
Published: January 2019

Abstract
As people age with HIV, olfactory dysfunctions may become more pronounced, especially for African Americans who are predisposed to declines in olfaction. In this cross-sectional study, 34 middle-age and older African American and 17 Caucasian men living with HIV were administered two objective olfactory measures (UPSIT). In the Smell Threshold Test, compared to the HIV-negative age norms, adults living with HIV were significantly less able to detect a lower threshold smell. In the University of Pennsylvania Smell Identification Test, compared to the HIV-negative age norms, adults living with HIV were significantly less able to correctly identify odors; furthermore, using such norms, African Americans with HIV were less likely to correctly identify odors compared to Caucasians with HIV. Since the literature indicates that African Americans have a stronger attraction for salty and sweet foods, such olfactory dysfunction may contribute to poor eating habits, potentially predisposing this population to additional health problems.
Insurance Status and Access to Sexual Health Services Among At-Risk Men: A Qualitative Study
Authors: Sowemimo-Coker Genoviva , Lee Alice J. , Montgomery Madeline C. , Nunn Amy S. , van den Berg Jacob J. , Chan Philip A. 
Published: January 2019

Abstract
Our objective was to evaluate the impact of insurance coverage on access to sexual health services among at-risk men. Data were collected from Hispanic/Latino and non-Hispanic White male patients at a publicly funded sexually transmitted disease clinic in a Medicaid expansion state from February to July 2017, using in-depth, semistructured interviews. A coding scheme was applied to interview transcripts with iterative revisions until a final coding scheme was achieved. Data were analyzed using Nvivo 10 software. Three key themes emerged from qualitative analysis: Most participants reported (a) financial barriers, (b) fluctuations in insurance status and challenges with insurance re-enrollment, and (c) lack of access to a provider and discomfort discussing sexual health as barriers to accessing HIV/sexually transmitted disease care in primary care settings. Hispanic/Latino men more frequently cited these barriers compared with non-Hispanic White men. Insurance status and out-of-pocket costs are barriers to sexual health care for at-risk men.
Hearing Preservation in Cochlear Implant Surgery: A Meta-Analysis
Authors: Snels Chantal , IntHout Joanna , Mylanus Emmanuel , Huinck Wendy , Dhooge Ingeborg 
Published: January 2019

Objective(s):
The aim of the present meta-analysis is to assess the effects of hearing preservation (HP) methods on residual hearing in patients undergoing cochlear implant (CI) surgery and to look at the effect of follow-up time on HP outcome.

Data Sources:
A systematic search was conducted in PubMed, Embase, and Cochrane Library. Only articles in English were included.

Study Selection:
Prospective studies published until January 2018 on hearing preservation methods were included.

Data Extraction:
Studies were assessed on unaided pre- and postoperative hearing thresholds, follow up time, and methodological quality.

Data Synthesis:
A random-effects meta-regression was performed for the HP outcome in relation to surgical technique, electrode array design, inserted electrode length, insertion speed, and corticosteroid use for different follow up times (1 month, 6 months, and 12 months or more postoperatively).

Conclusion:
Hearing preservation in cochlear implant surgery is feasible. A statistically significant difference was found between the round window procedure and cochleostomy approach, in favor of the round window procedure at 6 months postoperatively (p = 0.001). A statistically significant difference was found between the straight and the perimodiolar electrode array at 1 month postoperatively in favor of the straight electrode array (p < 0.001). No statistically significant difference was found between the other HP methods. The round window approach with the straight electrode array might result in a better HP outcome at 1 month and 6 months postoperatively compared with the cochleostomy approach with the perimodiolar electrode array. A declining trend in HP outcome in both combinations was seen over time.
Factors Influencing Access to Cochlear Implantation in Deaf and Hard-of-Hearing Children in Southern California
Authors: Moshtaghi Omid , Kuan Edward C. , Mahboubi Hossein , Khoshab Nima , Zardouz Shawn , Yau Amy Yuk-Yee , Rothholtz Vanessa S. , Lin Harrison W. , Djalilian Hamid R. 
Published: January 2019

Objective:
To determine the epidemiologic relationship of family demographics and educational resources with parental knowledge of and willingness for their children to receive cochlear implantation (CI) for deaf and hard-of-hearing (DHH) children.

Methods:
A total of 213 parents of DHH children were surveyed at local schools, specialized camps, and clinics in Southern California. Data on parents were solicited, including income, insurance status, education level, hearing status, primary language, and motivations towards CI.

Results:
Sixty-six surveys were included in the analysis. Three of these patients had already undergone CI, thus of the 63 children without CI, 59% had been presented with the option of CI by a healthcare professional and 27% were willing to have their child undergo CI. Willingness for children to undergo CI was statistically higher in families with an annual income less than $15,000 or more than $75,000 (p = 0.02), and children enrolled in specialized schools for DHH (p = 0.02). The leading reasons for unwillingness to undergo CI were risks of surgery (17%) and discouragement from others (14%).

Conclusion:
A significant gap exists between the number of CI candidates and families and willingness to undergo CI. The difference could be related to socioeconomic status and the patient's school type. This underscores the importance of parental education through the use of a multi-disciplinary team to ensure all hearing rehabilitation options are explained.
Jugular Bulb Resurfacing With Bone Cement for Patients With High Dehiscent Jugular Bulb and Ipsilateral Pulsatile Tinnitus
Authors: Lee Sang-Yeon , Song Seul-Ki , Park Sung Joon , Park Han Gyeol , Choi Byung Yoon , Koo Ja-Won , Song Jae-Jin 
Published: January 2019

Objective:To discuss the possible pathophysiologic mechanism of pulsatile tinnitus (PT) perception due to high jugular bulb with bony dehiscence (HJBD) and its improvement after the dehiscent jugular bulb (JB) resurfacing using bone cement, and to describe the efficacy of an objective measure of PT using transcanal sound recording and spectro-temporal analysis (TSR/STA).Study Design:Retrospective case series study.Setting:A university-based, tertiary care cancer center.Patients:Three PT patients with HJBD.Main Outcome Measure(s):All patients underwent transtympanic resurfacing after the source of PT was confirmed by temporal bone imaging and TSR/STA. Improvement of symptom and the changes in the TSR/STA were analyzed.Results:In the first case, a revision operation was performed due to slightly improved but persistent PT after initial resurfacing with bone pate and a piece of conchal cartilage. Revision transtympanic JB resurfacing was performed in this case using bone cement, and PT resolved immediately after the surgery. In the second and third cases, PT resolved completely, or was much abated, immediately after transtympanic resurfacing with bone cement. The TSR/STA also revealed improvement of PT. The median follow-up duration was 28 months, and all three patients remained asymptomatic or much improved compared with their preoperative status.Conclusions:Transtympanic resurfacing with bone cement, reinforcing the dehiscent JB to reduce focal turbulent flow, is a simple and effective surgical treatment option in patients with PT due to HJBD. In patients with HJBD, the objective measurement of PT by TSR/STA may be of help in selecting appropriate surgical candidates and objective evaluation of the treatment outcome.
Fracture of the Incus Caused by Digital Manipulation of the Ear Canal and its Diagnosis Using Wideband Acoustic Immittance
Authors: Masud Salwa F. , Knudson Inge M. , Stankovic Konstantina M. , Nakajima Hideko Heidi 
Published: January 2019

Objective:
To describe the first reported case of a fracture of the long process of the incus due to digital manipulation of the ear canal and to discuss diagnostic markers for ossicular fractures.

Patient:
A 46-year-old woman with incessant clicking and crunching in her left ear, and hearing loss after digital manipulation of the ear canal.

Intervention:
Diagnostic evaluation and therapeutic ossiculoplasty.

Main Outcome Measure(s):
Audiometric and wideband acoustic immittance (WAI) measurements were made before surgery to investigate the cause of clicking sounds and mild conductive hearing loss (CHL).

Results:
The clinical suspicion of a loose ossicular chain was confirmed by a large narrow-band decrease in power reflectance (calculated from WAI) at frequencies between 600 and 700 Hz, and a mid- to high-frequency air-bone gap. Exploratory tympanotomy revealed an ossicular fracture of the distal aspect of the long process of the incus. Ossiculoplasty with bone cement resolved bothersome clicking sounds.

Conclusion:
A finger inserted into the ear canal can produce an air seal, and subsequent quick removal of the finger can result in the fracture of an ossicle. Clinicians should be cognizant of this form of trauma because insertion of a finger, ear plug, and earphone into the ear canal are common. Ossicular fractures can result in high-frequency CHL, and can be misdiagnosed as sensorineural loss because bone conduction thresholds are not measured above 4 kHz. As in this case, an ossicular fracture may be misdiagnosed and result in inappropriate treatment. Here, WAI, a non-invasive measure of ear mechanics, diagnosed a loose ossicular chain.
Audiometric Results of Stapedotomy Surgery for Otoscelorsis: Influence of the Radiological Stage
Authors: Meranger Antoine , David Alina , Beigner Baptiste Marcel , Charpiot Anne , Tavernier Laurent 
Published: January 2019

Background:
The objective of this study was to identify a correlation between the radiological stage of otosclerosis and the pre- and postoperative audiometric results of patients who underwent a stapedotomy.

Methods:
Ninety-three patients with radiologically and surgically confirmed otosclerosis who underwent stapedotomy surgery and CT scanning within 18 months before the operation were included. The CT scans were interpreted by an otologist and a specialised radiologist to determine their radiological stage according to the classification of Veillon and Fraysse. The patients received a pre- and postoperative audiogram in the short and long term.

Results:
The preoperative bone conduction thresholds were higher in patients who presented with an advanced radiological stage of otosclerosis: 32.7 dB ± 12.4 compared with those who presented with a less advanced radiological stage: 24.3 dB ± 10.0. The preoperative air conduction thresholds were higher in patients who presented with an impairment of the round window: 58.1 dB ± 13.5 compared with those who presented with no impairment of the round window: 48.7 dB ± 14.5. The postoperative improvement in the air-bone gap was significantly higher for the localised foci: 16.9 dB ± 8.6 versus 11.0 dB ± 9.2, but only in the short term.

Conclusion:
There was a clinical radiological correlation with the preoperative results: In BC, there was a correlation with the radiological stage of Veillon and in AC, there was a correlation with impairment of the round window. The link between the radiological stage of otosclerosis and the postoperative audiometric results is less obvious. In the short term, the audiometric improvements in the air-bone gap were greater in patients in the early stages according to the Veillon classification, but this result was not sustained in the long-term.
Objective Measures of Tinnitus: a Systematic Review
Authors: Jackson Richard , Vijendren Ananth , Phillips John 
Published: January 2019

Background:
The purpose of this review is to systematically appraise the English-language literature to identify methods of objectively diagnosing and measuring the severity of subjective idiopathic nonpulsatile tinnitus.

Materials and Methods:
A systematic literature search was undertaken from the databases of MEDLINE (1946–May 2018), EMBASE (1980–May 2018), CINAHL (1981–May 2018), and BNI (1992–May 2018) according to predefined inclusion and exclusion criteria.

Results:
We identified 21 articles in this field. Reported objective tests include blood tests, electrophysiology measures, radiological measures, and balance tests. The overall quality of evidence was low, with most studies being low-powered.

Discussion:
Our review has not identified any reliable or reproducible objective measures of tinnitus. However, this piece of work has highlighted emerging areas where further high quality research may lead to the development of an effective method for objectively identifying and measuring the severity of tinnitus.
Update on the management of acute liver failure
Authors: Trovato Francesca M. , Rabinowich Liane , McPhail Mark J.W. 
Published: January 2019

Purpose of review
This review describes the current intensive care management of acute liver failure (ALF) and the latest evidence for emerging therapies.

Recent findings
Mortality from ALF continues to improve and in some cases, medical therapy can negate the need for liver transplantation because of protocolized management in specialist centres. Liver transplantation remains the cornerstone of management for poor prognosis ALF. The reduced use of blood products in ALF reflects growing evidence of balanced haemostasis in severe liver disease. Prophylactic therapeutic hypothermia is no longer recommended for neuroprotection. In cases not suitable for liver transplantation, high-volume plasma exchange (HVP) has potential benefit, although further research on the optimal timing and dosing is needed. Although sepsis remains an important complication in ALF, the use of prophylactic antimicrobials is being questioned in the era of emerging bacterial resistance.

Summary
ICU management of ALF has improved such that liver transplantation is not required in some cases. HVP has emerged as a potential therapy for patients who may not be good liver transplantation candidates. Nevertheless in suitable patients with poor prognosis liver transplantation remains the optimal therapy.