bluuebunny/crossref_metadata_2025_split huggingface dataset:
What is in the Myopathy Literature?
Authors: Lacomis David 
Published: May 2018

Abstract
This update covers the results of a randomized, placebo-controlled study that provides evidence that lamotrigine is effective in treating nondystrophic myotonias. Next, an overview of adverse effects of immune checkpoint inhibitors is provided, and the association of autoimmune myopathy and these monoclonal antibody therapies is discussed in light of recent reports. Last, the utility of electrodiagnostic testing in patients with intensive care unit weakness is addressed with emphasis on the high sensitivity and specificity of prolonged compound muscle action potential amplitudes in diagnosing critical illness myopathy.
Fisher–Pharyngeal–Cervical–Brachial Overlap Syndrome With Novel Ganglioside Antibodies
Authors: Pedavally Swetha , Hernández Zulma M. , Zeidman Lawrence A. 
Published: May 2018

Abstract
Several variants of Guillain–Barré syndrome have been described. The Fisher syndrome (FS) presents with ataxia, areflexia, and ophthalmoparesis. The pharyngeal–cervical–brachial (PCB) variant presents with bulbar weakness, along with arm and neck weakness. The 2 variant syndromes can overlap. Both the isolated and overlap syndromes respond to immunomodulatory treatment, thus are important to recognize clinically. Ganglioside antibodies are detectable in the variant syndromes and may aid in their diagnosis. The FS typically is associated with anti-GQ1b antibodies, and PCB is typically associated with anti-GT1a antibodies, whereas the overlap syndrome may have both ganglioside antibody subtypes. We present a case of overlap FS–PCB syndrome with a novel ganglioside antibody profile of GM1 and GD1b antibodies, which typically are associated with other variant syndromes. This case suggests the need for all ganglioside antibodies to be tested in suspected variant Guillain–Barré syndromes. The antibodies may prove especially useful in cases in which the clinical diagnosis is ambiguous.
Case of Anti–Single Recognition Particle–Mediated Necrotizing Myopathy After Influenza Vaccination
Authors: Mamarabadi Mansoureh , Baisre Ada , Leitch Megan , Hsu Vivien , Kanduri Jaya S. , Chen Shan 
Published: May 2018

Abstract
Immune-mediated necrotizing myopathy is a very rare inflammatory disease affecting skeletal muscles. Immune-mediated necrotizing myopathy may be associated with myositis-specific autoantibodies including anti–single recognition particle and anti–3-hydroxy-3- methylglutaryl-coenzyme A reductase, infectious agents (HIV or hepatitis C), other connective tissue disorders (such as scleroderma), and malignancy. We reported a 28-year-old healthy woman presented with subacute onset ascending muscle weakness 2 weeks after an annual influenza vaccination. Cerebral Spinal Fluid study showed normal cell counts with elevated protein and nerve conduction study showed reduced diffuse compound muscle action potential amplitudes suggesting a diagnosis of Guillain–Barré syndrome. Despite treatment using intravenous immunoglobulin, her condition continued to get worse with new bulbar and respiratory muscle weakness. Eventually, the diagnosis of anti–single recognition particle–mediated necrotizing myopathy was made based on elevated creatine kinase, thigh magnetic resonance imaging, muscle biopsy, and positive antibody testing. Our patient responded to the combination of intravenous immunoglobulin, prednisone, and anti-CD20 monoclonal antibody, rituximab.
Lumbosacral Radiculoplexopathy as the Initial Presentation of Lymphoma: A Report of 4 Cases
Authors: Marquardt Robert J. , Li Yuebing 
Published: May 2018

Abstract

Objectives:
To evaluate the clinical, laboratory, and radiological features of 4 cases of biopsy-proven lymphomatous lumbosacral radiculoplexopathy.

Methods:
Retrospective chart review.

Results:
All patients suffered from diffuse large B-cell lymphoma. A mean diagnostic delay of 10 months was encountered. Presenting symptoms in all 4 patients included back pain, radicular leg pain, and leg weakness, similar to spondylotic radiculopathy. Electrodiagnostic study showed axon loss radiculoplexopathy and magnetic resonance imaging of the lumbar spine or pelvis demonstrated nerve or nerve root enhancement. Increased uptake by lumbosacral roots/plexus on fluorodeoxyglucose-positron emission tomography aided diagnosis in 3 cases. Cytology was positive in 1 of 10 cerebrospinal fluid samples. Combined chemotherapy and radiation treatment led to clinicoradiological improvement, with residual neurological symptoms in all patients.

Conclusions:
Lymphomatous lumbosacral radiculoplexopathy should be considered in patients with progressive lumbosacral radicular symptoms. Magnetic resonance imaging and fluorodeoxyglucose-positron emission tomography, but not cerebrospinal fluid, are helpful in achieving early diagnosis. Treatment responses seem favorable.
Dual-Positive CD4/CD8 Primary Cutaneous Peripheral T-Cell Lymphoma Previously Classified as Mycosis Fungoides a Tumor D'Emblée
Authors: Raychaudhuri Sreejata , Rahvar Maral , Jedrych Jaroslaw , Karunamurthy Arivarasan , Kruglov Oleg , Rakfal Susan , Kane Kevin , Akilov Oleg E. 
Published: May 2018

Abstract:
Cutaneous peripheral T-cell lymphoma, not otherwise specified represents a “waste basket” of all cases that cannot be put into another of the categories of mature cutaneous T-cell lymphoma. Previously, the sudden multifocal development of cutaneous CD4+ tumors without preceding a patch or plaque stage was classified as d'emblée form of mycosis fungoides (MF). Currently, the term “MF” reserved only for the classic Alibert–Bazin type characterized by the evolution of patches, plaques, and tumors or for variants showing a similar clinical course. The authors describe a 75-year-old white woman who presented with a solitary skin tumor in the right supraclavicular region, with no lymph node or systemic involvement. Local external beam radiation treatment resulted in a complete response. The patient relapsed after 5 months with new tumors in the left neck and left upper chest. Biopsy of the lesions showed a dermal infiltrate of atypical small- to medium-sized T-lymphocytes, and immunohistochemical staining showed coexpression of CD4/CD8 in a subset of these cells, which was confirmed with flow cytometry of the tumor. Although the patient had no preceding patch or plaque stage, the authors herein report this extremely rare case of CD4/CD8 dual-positive peripheral T-cell lymphoma, not otherwise specified presented as MF d'emblée and discuss the seldom similar cases published previously.
Effects of internet-based pain coping skills training before home exercise for individuals with hip osteoarthritis (HOPE trial): a randomised controlled trial
Authors: Bennell Kim L. , Nelligan Rachel K. , Rini Christine , Keefe Francis J. , Kasza Jessica , French Simon , Forbes Andrew , Dobson Fiona , Abbott J. Haxby , Dalwood Andrew , Harris Anthony , Vicenzino Bill , Hodges Paul W. , Hinman Rana S. 
Published: May 2018

Abstract
This assessor-, therapist-, and participant-blinded randomised controlled trial evaluated the effects of an automated internet-based pain coping skills training (PCST) program before home exercise for people with clinically diagnosed hip osteoarthritis. One hundred forty-four people were randomised to either the PCST group or the comparator group. In the first 8 weeks, the PCST group received online education and PCST, whereas the comparison group received online education only. From weeks 8 to 24, both groups visited a physiotherapist 5 times for home exercise prescription. Assessments were performed at baseline, 8, 24, and 52 weeks. Primary outcomes were hip pain on walking (11-point numerical rating scale) and physical function (Western Ontario and McMaster Universities Osteoarthritis Index [WOMAC]) at 24 weeks. Secondary outcomes were other measures of pain, quality-of-life, global change, self-efficacy, pain coping, pain catastrophizing, depression, anxiety, stress, physical activity, and adverse events. Primary outcomes were completed by 137 (95%), 131 (91%), and 127 (88%) participants at 8, 24, and 52 weeks, respectively. There were no significant between-group differences in primary outcomes at week 24 (change in: walking pain [mean difference 0.5 units; 95% confidence interval, −0.3 to 1.3] and function [−0.9 units; 95% confidence interval, −4.8 to 2.9]), with both groups showing clinically relevant improvements. At week 8, the PCST group had greater improvements in function, pain coping, and global improvement than comparison. Greater pain coping improvements persisted at 24 and 52 weeks. In summary, online PCST immediately improved pain coping and function but did not confer additional benefits to a subsequent exercise program, despite sustained pain coping improvements.
Predicting Performance and Non-Use in Prelingually Deaf and Late-Implanted Cochlear Implant Users
Authors: Lammers Marc J. W. , Versnel Huib , Topsakal Vedat , van Zanten Gijsbert A. , Grolman Wilko 
Published: May 2018

Objective:
To analyze postoperative hearing performance and independent predictors of speech perception and to assess cochlear implant nonuse in adults with prelingual deafness who received an implant during adulthood.

Design:
Retrospective cohort study including all prelingually deaf adults who received a cochlear implant at the University Medical Center Utrecht between 2000 and 2013.

Setting:
Tertiary referral center.

Patients:
Included were a total of 48 prelingually deaf patients diagnosed with severe to profound sensorineural hearing loss before the age of 2 years who received their implants during adulthood.

Main Outcome Measures:
Postoperative monosyllabic word and sentence recognition scores.

Results:
The average maximum postoperative monosyllabic word score for all subjects was 25% correct. Preoperative speech perception and the extent of preoperative residual hearing were both positive independent predictors of postoperative speech recognition, explaining 47% of the variance. Almost half of the population experienced only little (27%) or no benefit (21%) of their implants in daily communication. These patients were more likely to have either no or only low-frequency residual hearing. Eventually, all subjects without any benefit became non-user.

Conclusions:
Postoperative performance of adult, prelingually deaf cochlear implant candidates, with long-term deafness is dependent on their preoperative speech perception and residual hearing. Candidates who have negligible residual hearing are expected to have no or only limited postoperative benefit of their implants and are at risk of becoming nonusers.
Bone health in type 1 diabetes
Authors: Shah Viral N. , Carpenter R. Dana , Ferguson Virginia L. , Schwartz Ann V. 
Published: May 2018

Purpose of review
This article reviews recent publications on the effect of type 1 diabetes (T1D) on fracture risk, bone mineral density (BMD), bone structure, and bone tissue quality. Possible fracture prevention strategies for patients with T1D have also been reviewed.

Recent findings
T1D is associated with substantially elevated fracture risk and modestly low BMD at the femoral neck. However, BMD alone does not explain higher observed fracture risk in T1D. T1D also affects bone macro- and microstructure, characterized by thinner cortices and trabecular bone changes such as thinner and more widely spaced trabeculae. Structural bone deficit is pronounced in the presence of microvascular complications. Tissue-level changes, such as accumulation of advanced glycation endproducts, detrimental alterations of the mineral phase because of low bone turnover, and occlusion of vascular channels in bone by mineralized tissue, are implicated in pathophysiology of bone fragility in T1D. There are no guidelines on screening and prevention of osteoporotic fractures in T1D.

Summary
More studies are needed to understand the influence of T1D on structural bone quality and tissue material properties. There is a need for a prospective study to evaluate better screening strategies for diagnosis and treatment of osteoporosis in T1D.
SGLT inhibition: a possible adjunctive treatment for type 1 diabetes
Authors: Akturk Halis Kaan , Rewers Amanda , Garg Satish K. 
Published: May 2018

Purpose of review
To identify and evaluate the recent trials of sodium-glucose cotransporter 1 and 2 (SGLT1 and SGLT2, respectively) inhibitor use in patients with type 1 diabetes (T1D). SGLT-2 inhibitors have been approved by the Food and Drug Administration (FDA) and are effectively used in the treatment of type 2 diabetes (T2D). However, many studies (phase I–III) have validated their effects beyond improving glycemic control and have shown potential adjunctive use in adult patients with T1D treated with insulin therapy alone.

Recent findings
A review of the literature showed that there is a potential adjunctive role for the SGLT inhibitors with insulin in T1D for improving glycemic control. The inTandem3 (A phase III study to evaluate the safety of sotagliflozin in patients with type 1 diabetes who have inadequate glycemic control with insulin therapy alone) and the DEPICT-1 (Dapagliflozin evaluation in patients with inadequately controlled type 1 diabetes) trials demonstrated significant benefits in adult patients with T1D. The SGLT inhibitors may become the first oral medication to be approved for adjunctive use in T1D.

Summary
The risk of diabetic ketoacidosis still remains a concern, but considering additional benefits beyond glucose control, with proper counseling and education, these medications may allow a larger number of patients to achieve target glucose control without weight gain or increased risk of hypoglycemia.
Comparing Therapeutic versus Prophylactic Nipple-Sparing Mastectomy: Does Indication Inform Oncologic and Reconstructive Outcomes?
Authors: Frey Jordan D. , Salibian Ara A. , Karp Nolan S. , Choi Mihye 
Published: May 2018

Background:
Initially performed only in prophylactic cases, indications for nipple-sparing mastectomy have expanded. Trends and surgical outcomes stratified by nipple-sparing mastectomy indication have not yet been fully examined.

Methods:
Demographics and outcomes for all nipple-sparing mastectomies performed from 2006 to 2017 were compared by mastectomy indication.

Results:
A total of 1212 nipple-sparing mastectomies were performed: 496 (40.9 percent) for therapeutic and 716 (59.1 percent) for prophylactic indications. Follow-up time was similar between both the therapeutic and prophylactic nipple-sparing mastectomy groups (47.35 versus 46.83 months, respectively; p = 0.7942). Therapeutic nipple-sparing mastectomies experienced significantly greater rates of major (p = 0.0165) and minor (p = 0.0421) infection, implant loss (p = 0.0098), reconstructive failure (p = 0.0058), and seroma (p = 0.0043). Rates of major (p = 0.4461) and minor (p = 0.2673) mastectomy flap necrosis and complete (p = 0.3445) and partial (p = 0.7120) nipple necrosis were equivalent. The overall rate of locoregional recurrence/occurrence per nipple-sparing mastectomy was 0.9 percent: 2.0 percent in therapeutic nipple-sparing mastectomies and 0.1 percent in prophylactic nipple-sparing mastectomies (p < 0.0001).

Conclusions:
Approximately 40 percent of nipple-sparing mastectomies are currently performed for therapeutic indications. Therapeutic nipple-sparing mastectomies had higher rates of infectious complications and reconstructive failure. Rates of locoregional cancer recurrence/occurrence are low, but occur significantly more often after therapeutic nipple-sparing mastectomy.

CLINICAL QUESTION/LEVEL OF EVIDENCE:
Therapeutic, III.
Healthcare Professionals’ and Patients’ Views of Discussing Sexual Well-being Poststroke
Authors: Fox Siobhan , Antony Reema M. , Foley Mary J. , O’Sullivan Dawn , Timmons Suzanne 
Published: May 2018

Abstract

Purpose
Stroke can cause physical and emotional problems affecting sexual well-being; healthcare professionals (HCPs) are often uncomfortable discussing this topic with patients. We explored the perspectives of HCPs and stroke survivors about barriers to discussing sexual well-being poststroke.

Design
A mixed methodology was employed.

Methods
A postal survey of stroke survivors (n = 50), a focus group with HCPs on a stroke unit (n = 6), and a focus group with community-living stroke survivors (n = 6) were used in this study. Focus group data were analyzed thematically.

Findings
No patient surveyed (60% response rate) had discussed sexual well-being with an HCP. Focus groups revealed barriers on multiple levels: structural, HCP, patient, and professional–patient interface.

Conclusions
Healthcare professionals were poorly trained, adopted a passive role, and addressed sexual activity based on individual beliefs rather than having an agreed team approach.

Clinical Relevance
Relatively simple steps like inclusion in policy, training to empower HCPs, and the provision of written information for patients could help to improve practice.
Risk Factors, Depression, and Drugs Influencing Sexual Activity in Individuals With and Without Stroke
Authors: Dusenbury Wendy , Hill Twyla J. , Mosack Victoria , Steinke Elaine E. 
Published: May 2018

Purpose
The aim of the study was to analyze factors affecting sexual activity in individuals with and without stroke, ages 40–59 years, in a national, cross-sectional, population-based sample derived from the National Health and Nutrition Examination Survey (NHANES).

Design
Descriptive, cross-sectional survey.

Methods
Data were obtained from the NHANES (2011–2012) data set from individuals (N = 3,649) completing items related to cardiovascular risk factors, drugs, and sexual activity. Data were analyzed using chi-square, t tests, and logistic regression.

Findings
Overall, number of drugs, smoking, and depression significantly predicted sexual activity. When comparing sexually active to not sexually active, those with stroke had significantly less sexual activity (t = 2.822, p = .005) and reduced sexual activity per week or month (χ2 = 16.275, p = .005, df = 4). Those taking angiotensin-converting enzyme inhibitors and statins had reduced sexual activity.

Conclusions/Clinical Relevance
Findings illustrate the importance of risk factor modification and nurses engaging in sexual assessment, education, and counseling to support sexual quality of life in younger individuals with stroke.
Characterizing the genetic history of admixture across inner Eurasia
Authors: Jeong Choongwon , Balanovsky Oleg , Lukianova Elena , Kahbatkyzy Nurzhibek , Flegontov Pavel , Zaporozhchenko Valery , Immel Alexander , Wang Chuan-Chao , Ixan Olzhas , Khussainova Elmira , Bekmanov Bakhytzhan , Zaibert Victor , Lavryashina Maria , Pocheshkhova Elvira , Yusupov Yuldash , Agdzhoyan Anastasiya , Sergey Koshel , Bukin Andrei , Nymadawa Pagbajabyn , Churnosov Michail , Skhalyakho Roza , Daragan Denis , Bogunov Yuri , Bogunova Anna , Shtrunov Alexandr , Dubova Nadezda , Zhabagin Maxat , Yepiskoposyan Levon , Churakov Vladimir , Pislegin Nikolay , Damba Larissa , Saroyants Ludmila , Dibirova Khadizhat , Artamentova Lubov , Utevska Olga , Idrisov Eldar , Kamenshchikova Evgeniya , Evseeva Irina , Metspalu Mait , Robbeets Martine , Djansugurova Leyla , Balanovska Elena , Schiffels Stephan , Haak Wolfgang , Reich David , Krause Johannes 
Published: May 2018

AbstractThe indigenous populations of inner Eurasia, a huge geographic region covering the central Eurasian steppe and the northern Eurasian taiga and tundra, harbor tremendous diversity in their genes, cultures and languages. In this study, we report novel genome-wide data for 763 individuals from Armenia, Georgia, Kazakhstan, Moldova, Mongolia, Russia, Tajikistan, Ukraine, and Uzbekistan. We furthermore report genome-wide data of two Eneolithic individuals (∽5,400 years before present) associated with the Botai culture in northern Kazakhstan. We find that inner Eurasian populations are structured into three distinct admixture clines stretching between various western and eastern Eurasian ancestries. This genetic separation is well mirrored by geography. The ancient Botai genomes suggest yet another layer of admixture in inner Eurasia that involves Mesolithic hunter-gatherers in Europe, the Upper Paleolithic southern Siberians and East Asians. Admixture modeling of ancient and modern populations suggests an overwriting of this ancient structure in the Altai-Sayan region by migrations of western steppe herders, but partial retaining of this ancient North Eurasian-related cline further to the North. Finally, the genetic structure of Caucasus populations highlights a role of the Caucasus Mountains as a barrier to gene flow and suggests a post-Neolithic gene flow into North Caucasus populations from the steppe.
Characterization and identification of long non-coding RNAs based on feature relationship
Authors: Wang Guangyu , Yin Hongyan , Li Boyang , Yu Chunlei , Wang Fan , Xu Xingjian , Cao Jiabao , Bao Yiming , Wang Liguo , Abbasi Amir A. , Bajic Vladimir B. , Ma Lina , Zhang Zhang 
Published: May 2018

ABSTRACTThe significance of long non-coding RNAs (lncRNAs) in many biological processes and diseases has gained intense interests over the past several years. However, computational identification of lncRNAs in a wide range of species remains challenging; it requires prior knowledge of well-established sequences and annotations or species-specific training data, but the reality is that only a limited number of species have high-quality sequences and annotations. Here we first characterize lncRNAs by contrast to protein-coding RNAs based on feature relationship and find that the feature relationship between ORF (open reading frame) length and GC content presents universally substantial divergence in lncRNAs and protein-coding RNAs, as observed in a broad variety of species. Based on the feature relationship, accordingly, we further present LGC, a novel algorithm for identifying lncRNAs that is able to accurately distinguish lncRNAs from protein-coding RNAs in a cross-species manner without any prior knowledge. As validated on large-scale empirical datasets, comparative results show that LGC outperforms existing algorithms by achieving higher accuracy, well-balanced sensitivity and specificity, and is robustly effective (>90% accuracy) in discriminating lncRNAs from protein-coding RNAs across diverse species that range from plants to mammals. To our knowledge, this study, for the first time, differentially characterizes lncRNAs and protein-coding RNAs based on feature relationship, which is further applied in computational identification of lncRNAs. Taken together, our study represents a significant advance in characterization and identification of lncRNAs and LGC thus bears broad potential utility for computational analysis of lncRNAs in a wide range of species.