bluuebunny/crossref_metadata_2025_split huggingface dataset:
Bilateral symmetrical deep gray matter involvement and leptomeningeal enhancement in a child with MOG-IgG-associated encephalomyelitis
Authors: Shen Weibing , Zhang Yaner , Zhou Chenguang , Shen Yaoyao 
Published: January 2021

Abstract
Background
Currently, myelin oligodendrocyte glycoprotein (MOG)-IgG-associated encephalomyelitis (MOG-EM) is regarded as an independent inflammatory demyelinating disease. Magnetic resonance imaging (MRI) abnormalities occur in 44.4% of patients with MOG-EM. However, symmetrical deep gray matter involvement with leptomeningeal enhancement is rarely described in the literature.

Case presentation
A 3-year-old boy was admitted to our hospital because of acute onset fever, headache, vomiting and disturbance of consciousness. Neurological examination showed somnolence, neck stiffness and positive Kernig’s sign. Brain MRI demonstrated bilateral symmetrical lesions in the basal ganglia and thalamus as well as diffuse leptomeningeal enhancement along the sulci of bilateral hemisphere. Cerebrospinal fluid analysis demonstrated increased cell count (7 cells/mm3, mononuclear cells dominant) and protein (1.17 g/L) without glucose and chloride abnormality. Work-up for infectious and autoimmune causes, serum MOG IgG was positive by cell based assay. Therefore, a diagnosis of MOG-EM was established according to the international recommendatory criteria in 2018. He was administrated with intravenous methylprednisolone followed by oral corticosteroids and had recovered completely within 1 week.

Conclusions
In the setting of meningoencephalitis-like clinical presentation with bilateral symmetrical deep gray matter involvement, MOG-EM should be distinguished from other infectious and autoimmune disorders, such as Epstein-Barr virus (EBV) encephalitis, Japanese encephalitis and Anti-NMDA receptor (NMDAR) encephalitis. Besides, aseptic meningitis associated with leptomeningeal enhancement may be an atypical phenotype of MOG-EM.
Bibliometric analysis of bacterial central nervous system infection research in Southeast Asia
Authors: Jalipa Francis Gerwin U. , Sy Marie Charmaine C. , Espiritu Adrian I. , Jamora Roland Dominic G. 
Published: January 2021

Abstract
Background
The status of research and scientific outputs with regards to bacterial central nervous system (CNS) infection in southeast Asia (SEA) is unknown. This study aimed to analyze and compare bacterial CNS infection research output of SEA countries in terms of bibliometric indices.

Methods
The major electronic databases (MEDLINE, Scopus, Embase, CENTRAL, Clinicaltrials.gov and WPRIM) were searched for studies on bacterial CNS infection in SEA until August 31, 2020. Frequencies, percentages and Spearman’s rho correlations were used.

Results
There was a total of 648 unique studies on bacterial CNS infection in SEA and it was 657 when double-counted (collaborative studies between SEA countries). Thailand (n=148, 22.5%) and Vietnam (n=142, 21.6%) had the highest number of publications. The most common type of research publication was the case report / case series (n=160, 24.7%). Tuberculous meningitis/tuberculoma (n=176, 26.7%) was the most common topic. This study showed that the %GDP for research and development (R&D) was associated with a higher number of research output. However, the GDP per capita was not associated with any of the bibliometric indices. The total number of neurologists was associated with all of the bibliometric analysis.

Conclusion
Bacterial CNS infection research output in SEA countries was low in terms of quantity. The %GDP for R&D was associated with the number of research publications. The total number of neurologists was associated with all of the bibliometric indices.
Implementation of a fetal ultrasound telemedicine service: women’s views and family costs
Authors: Smith V. J. , Marshall A. , Lie M.L.S , Bidmead E. , Beckwith B. , Van Oudgaarden E. , Robson S. C. 
Published: January 2021

AbstractBackgroundThe complexity of fetal medicine (FM) referrals that can be managed within obstetric units is dependent on the availability of specialist ultrasound expertise. Telemedicine can effectively transfer real-time ultrasound images via video-conferencing. We report the successful introduction of a fetal ultrasound telemedicine service linking a specialist fetal medicine (FM) centre and a remote obstetric unit.MethodsOver a four-year period from October 2015, all women referred for FM consultation from the obstetric unit were seen via telemedicine, excluding cases where invasive testing, intrauterine therapy or cardiac anomalies were anticipated. The outcomes measured included the indication for FM referral; scan duration and image and sound quality during the consultation. Women’s perceptions of the telemedicine consultation and estimated costs to attend the FM centre were measured by a structured questionnaire completed following the first telemedicine appointment during the Phase 1 of the project.ResultsOverall, 297 women had a telemedicine consultation during Phase 1 (pilot and evaluation) and Phase 2 (embedding and adoption) of the project, which covered a 4 year period 34 women completed questionnaires during the Phase 1 of the study. Travel to the telemedicine consultation took a median (range) time of 20 min (4150), in comparison to an estimated journey of 230 min (120,450) to the FM centre. On average, women would have spent approximately £28 to travel to the FM centre per visit. The overall costs for the woman and her partner/ friend to attend the FM centre was estimated to be £439.Women were generally satisfied with the service and valued the opportunity to have a FM consultation locally.ConclusionsWe have demonstrated that a fetal ultrasound telemedicine service can be successfully introduced to provide FM ultrasound of sufficient quality to allow fetal diagnosis and specialist consultation with parents. Furthermore, the service is acceptable to parents, has shown a reduction in family costs and journey times.
Clinical aspects for differential diagnosis of Kawasaki disease shock syndrome: a case control study
Authors: Park Woo Young , Lee Sang Yun , Kim Gi Beom , Song Mi Kyoung , Kwon Hye Won , Bae Eun Jung , Choi Eun hwa , Park June Dong 
Published: January 2021

Abstract
Background
Because of the absence of a specific diagnostic test and pathognomonic clinical features, physicians must rely on the presence of specific clinical criteria and laboratory data that support the diagnosis of KD. To help clinicians distinguish KD, KDSS, septic shock, and TSS earlier, we suggest differential diagnosis and treatment guideline.

Methods
Medical records of immunocompetent patients who were admitted to the pediatric department with a diagnosis of KDSS, septic shock or TSS (SS group) were retrospectively reviewed. In addition, KD patients were selected by seasonal matching to each case of KDSS patient by date of admission (± 2 weeks).

Results
There were 13 patients with KDSS, 35 patients with SS group, and 91 patients with KD. In comparison between KDSS and septic shock group, KDSS group had significantly higher rate of coronary aneurysm incidence, and higher left ventricle dysfunction rate. In comparison between KDSS and TSS, patients with KDSS had a significantly higher erythrocyte sedimentation rate (ESR) and significantly lower creatinine. Receiver operation characteristic curve revealed that the optimal ESR cut off value for determining the KDSS was 56.0 (sensitivity 75.0%, specificity of 100.0%) and the optimal creatinine cut off value for determining the TSS was 0.695 (sensitivity 76.9%, specificity 84.6%).

Conclusions
Clinical symptoms, laboratory finding, echocardiography, and culture studies can be used to differentiate KD, KDSS, septic shock and TSS.
Healthcare access barriers for FARC ex-combatants in Colombia: qualitative perspectives from healthcare providers and FARC health promoters
Authors: Reynolds Christopher W. , Aguiar Leonar G. , Arbelaez Christian , Restrepo Carlos Gómez , Patiño Andres , Carranza Heidy , Pileika Lindsey , Duarte Andrés 
Published: January 2021

Abstract
Background
Following the 2016 Peace Agreement with the Fuerzas Armadas Revolucionarias de Colombia (FARC), Colombia promised to reincorporate more than 13,000 guerrilla fighters into its healthcare system. Despite a subsidized healthcare insurance program and the establishment of 24 Espacios Territoriales de Capacitación y Reincorporación (ETCRs—Territorial Spaces for Training and Reintegration) to facilitate this transition, data has shown that FARC ex-combatants access care at disproportionately lower rates, and face barriers to healthcare services.

Methods
Semi-structured interviews were conducted with FARC health promoters and healthcare providers working in ETCRs to determine healthcare access barriers for FARC ex-combatants. Analysis was completed with a qualitative team-based coding method and barriers were categorized according to Julio Frenk’s Domains of Healthcare Access framework.

Results
Among 32 participants, 25 were healthcare providers and 7 self-identified as FARC health promoters. The sample was majority female (71.9%) and worked with the FARC for an average of 12 months in hospital, health center, medical brigade, and ETCR settings. Our sample had experiences with FARC across 16 ETCRs in 13 Departments of Colombia. Participants identified a total of 141 healthcare access barriers affecting FARC ex-combatants, which affected healthcare needs, desires, seeking, initiation and continuation. Significant barriers were related to a lack of resources in rural areas, limited knowledge of the Colombian health system, the health insurance program, perceived stigma, and transition process from the FARC health system.

Conclusions
FARC ex-combatants face significant healthcare access barriers, some of which are unique from other low-resource populations in Colombia. Potential solutions to these barriers included health insurance provider partnerships with health centers close to ETCRs, and training and contracting FARC health promoters to be primary healthcare providers in ETCRs. Future studies are needed to quantify the healthcare barriers affecting FARC ex-combatants, in order to implement targeted interventions to improve healthcare access.
Effectiveness of reverse total shoulder arthroplasty for primary and secondary fracture care: mid-term outcomes in a single-centre experience
Authors: Schwarz AM , Hohenberger GM , Sauerschnig M , Niks M , Lipnik G , Mattiassich G , Zacherl M , Seibert FJ , Plecko M 
Published: January 2021

Abstract
Background
The introduction of reverse total shoulder arthroplasty (RSA) as a treatment option in complex proximal humeral fractures, has significantly extended the surgical armamentarium.
The aim of this study was to investigate the mid-term outcome following fracture RSA in acute or sequelae, as well as salvage procedures. It was hypothesized that revision RSA (SRSA) leads to similar mid-term results as primary fracture treatment by RSA (PRSA).

Methods
This retrospective study describes the radiological and clinical mid-term outcomes in a standardized single-centre and Inlay design. Patients who underwent RSA in fracture care between 2008 and 2017 were included (minimum follow-up: 2 years, minimum age: 60 years).
The assessment tools used for functional findings were range of motion (ROM), Visual Analogue Scale, absolute (CS) plus normative Constant Score, QuickDASH, and Subjective Shoulder Value. All adverse events as well as the radiological results and their clinical correlations were statistically analysed (using p < .05and 95% confidence intervals).

Results
Following fracture RSA, 68 patients were included (mean age: 72.5 years, mean follow-up: 46 months). Forty-two underwent primary RSA (PRSA), and 26 underwent revision RSA (SRSA). Adverse advents were observed in 13% (n = 9/68).
No statistically significant results were found for the scores of the PRSA and SRSA groups, while the failed osteosynthesis SRSA subgroup obtained statistically significantly negative values for ROM subzones (flexion: p = .020, abduction: p = .020). Decreased instances of tubercle healing were observed for the in PRSA group relative to the SRSA group (p = .006). The absence of bony healing of the tubercles was related to significant negative clinical and subjective outcomes (all scores: p < .05, external rotation: p= .019). Significant postoperative improvements were evaluated in the SRSA group (CS: 23 to 56 at mean, p = .001), the time from index surgery to operative revision revealed no associations in functional findings.

Conclusions
RSA is an effective option in severe shoulder fracture management with predictable results for salvage as well as first-line treatment. Promising mid-term functional results, reasonable implant survival rates, and high patient satisfaction can be achieved.

Level of evidence
Level III.
Musculoskeletal pathology as an early warning sign of systemic amyloidosis: a systematic review of amyloid deposition and orthopedic surgery
Authors: Wininger Austin E. , Phelps Brian M. , Le Jessica T. , Harris Joshua D. , Trachtenberg Barry H. , Liberman Shari R. 
Published: January 2021

Abstract
Background
Transthyretin and immunoglobulin light-chain amyloidoses cause amyloid deposition throughout various organ systems. Recent evidence suggests that soft tissue amyloid deposits may lead to orthopedic conditions before cardiac manifestations occur. Pharmacologic treatments reduce further amyloid deposits in these patients. Thus, early diagnosis improves long term survival.

Questions/purposes
The primary purpose of this systematic review was to characterize the association between amyloid deposition and musculoskeletal pathology in patients with common orthopedic conditions. A secondary purpose was to determine the relationship between amyloid positive biopsy in musculoskeletal tissue and the eventual diagnosis of systemic amyloidosis.

Methods
We performed a systematic review using PRISMA guidelines. Inclusion criteria were level I-IV evidence articles that analyzed light-chain or transthyretin amyloid deposits in common orthopedic surgeries. Study methodological quality, risk of bias, and recommendation strength were assessed using MINORS, ROBINS-I, and SORT.

Results
This systematic review included 24 studies for final analysis (3606 subjects). Amyloid deposition was reported in five musculoskeletal pathologies, including carpal tunnel syndrome (transverse carpal ligament and flexor tenosynovium), hip and knee osteoarthritis (synovium and articular cartilage), lumbar spinal stenosis (ligamentum flavum), and rotator cuff tears (tendon). A majority of studies reported a mean age greater than 70 for patients with TTR or AL positive amyloid.

Conclusions
This systematic review has shown the presence of amyloid deposition detected at the time of common orthopedic surgeries, especially in patients ≥70 years old. Subtyping of the amyloid has been shown to enable diagnosis of systemic light-chain or transthyretin amyloidosis prior to cardiac manifestations.

Level of evidence
Level IV.
Efficacy of surgical repair for the functional restoration of injured facial nerve
Authors: Li Li , Fan Zhaomin , Wang Haibo , Han Yuechen 
Published: January 2021

Abstract
Background
Early surgical repair to restore nerve integrity has become the most commonly practiced method for managing facial nerve injury. However, the evidence for the efficacy of surgical repair for restoring the function of facial nerves remains deficient. This study evaluated the outcomes of surgical repair for facial nerve lesions.

Methods
This retrospective observational study recruited 28 patients with the diagnosis of facial nerve injury who consecutively underwent surgical repairs from September 2012 to May 2019. All related clinical data were retrospectively analyzed according to age, sex, location of the facial nerve lesion, size of the facial nerve defect, method of repair, facial electromyogram, and blink reflex. Facial function was then stratified with the House-Brackmann grading system pre-operation and 3, 9, 15, and 21 months after surgical repair.

Results
The 28 patients enrolled in this study included 17 male and 11 female patients with an average age of 34.3 ± 17.4 years. Three methods were applied for the repair of an injured facial nerve, including great auricular nerve transplantation in 15 patients, sural nerve grafting in 7 patients, and hypoglossal to facial nerve anastomosis in 6 patients. Facial nerve function was significantly improved at 21 months after surgery compared with pre-operative function (P = 0.008). Following surgical repair, a correlation was found between the amplitude of motor unit potential (MUP) and facial nerve function (r = -6.078, P = 0.02). Moreover, the extent of functional restoration of the facial nerve at 21 months after surgery depended on the location of the facial nerve lesion; lesions at either the horizontal or vertical segment showed significant improvement(P = 0.008 and 0.005), while no functional restoration was found for lesions at the labyrinthine segment (P = 0.26).

Conclusions
For surgical repair of facial nerve lesions, the sural nerve, great auricular nerve, and hypoglossal-facial nerve can be grafted effectively to store the function of a facial nerve, and MUP may provide an effective indicator for monitoring the recovery of the injured nerve.
Rationale and design of the cardiovascular status in patients with endogenous cortisol excess study (CV-CORT-EX): a prospective non-interventional follow-up study
Authors: Ehrlich Kristina , Morbach Caroline , Reiter Theresa , Heuschmann Peter Ulrich , Hannemann Anke , Fassnacht Martin , Störk Stefan , Hahner Stefanie , Deutschbein Timo 
Published: January 2021

Abstract
Background
Endogenous Cushing’s syndrome (CS) results in increased cardiovascular (CV) morbidity and mortality. So far, most studies focussed on distinct disease entities rather than the integrity of the CV system. We here describe the design of the Cardiovascular Status in Endogenous Cortisol Excess Study (CV-CORT-EX), a study aiming to comprehensively investigate the health status of patients with endogenous CS (with a particular focus on CV phenotypes, biochemical aspects, quality of life, and psychosocial status).

Method
A prospective non-interventional cohort study performed at a German tertiary referral centre. At the time of enrolment, patients will be categorised as: (1) newly diagnosed overt CS, (2) recurrent overt CS, (3) CS in remission, (4) presence of mild autonomous cortisol excess (MACE). The target cohorts will be n = 40 (groups 1 + 2), n = 80 (group 3), and n = 20 (group 4). Patients with overt CS at the time of enrolment will be followed for 12 months after remission (with re-evaluations after 6 and 12 months). At each visit, patients will undergo transthoracic echocardiography, cardiac magnetic resonance imaging, 24-h electrocardiogram, 24-h blood pressure measurement, and indirect evaluation of endothelial function. Furthermore, a standardised clinical investigation, an extensive biochemical workup, and a detailed assessment of quality of life and psychosocial status will be applied. Study results (e.g. cardiac morphology and function according to transthoracic echocardiography and cardiac magnetic resonance imaging; e.g. prevalence of CV risk factors) from patients with CS will be compared with matched controls without CS derived from two German population-based studies.

Discussion
CV-CORT-EX is designed to provide a comprehensive overview of the health status of patients with endogenous CS, mainly focussing on CV aspects, and the holistic changes following remission.

Trail registration
ClinicalTrials.gov (https://clinicaltrials.gov/) NCT03880513, registration date: 19 March 2019 (retrospectively registered). Protocol Date: 28 March 2014, Version 2.
Evaluating the impact of a medical school cohort sexual health course on knowledge, counseling skills and sexual attitude change
Authors: Ross Michael W. , Bayer Carey Roth , Shindel Alan , Coleman Eli 
Published: January 2021

Abstract
Background
Sexual health is generally considered an integral part of medical and allied healthcare professional training. However, many medical schools do not offer this as a mandatory curriculum, or minimize it. Sexual health as an academic area was introduced in the 1970s, but there have been few cohort evaluations of its impact. This was limited by the availability of few psychometric scales for evaluation. We evaluated the full, mandatory, sexual health course in year 1 medicine at a large state university in the Midwest US, including the course with lectures; panels and tutorials; a video app to give students feedback on their sexual history taking skills; and a 3-station sexual history OSCE at the end of the course.

Results
Seventy-four medical students (43% of the course cohort) volunteered, for an incentive, to complete evaluation materials pre- and post-course. We used the Sexual Health Education for Professionals Scale (SHEPS), designed and with appropriate psychometric standardization for such evaluation. The SHEPS data covers 7-point Likert scale ratings of 37 patient situations, asking first how well the student could communicate with such a patient, and on the second part how much knowledge they have to care for such a patient. The third subscale examines personal sexual attitudes and beliefs. Data indicated that the matched pretest-posttest ratings for skills and knowledge were all statistically significant and with very large effect sizes. Few of the attitude subscale items were significant and if so, had small effect sizes. Sexual attitudes and beliefs may be well-formed before entry into medical school, and sexual health teaching and learning has minimal effect on sexual attitudes in this US sample. However, using the 3 sexuality OSCE cases scores as outcomes, two of the 26 attitude-belief items predicted > 24% of the variance.

Conclusions
The sexual health course produced major changes in Communications with patients sexual health skills and Knowledge of sexual health, but little change in personal Attitudes about sexuality. These data suggest that personal attitude change is not essential for teaching US medical students to learn about sexual health and sexual function and dysfunction, and comfortably take a comprehensive sexual history.
Recurrent PALB2 mutations and the risk of cancers of bladder or kidney in Polish population
Authors: Złowocka-Perłowska Elżbieta , Dębniak Tadeusz , Słojewski Marcin , Lemiński Artur , Soczawa Michał , van de Wetering Thierry , Trubicka Joanna , Kluźniak Wojciech , Wokołorczyk Dominika , Cybulski Cezary , Lubiński Jan 
Published: January 2021

Introduction
The role of PALB2 in carcinogenesis remains to be clarified. Our main goal was to determine the prevalence of PALB2 (509_510delGA and 172_175delTTGT) mutations in bladder and kidney cancer patients from Polish population.

Materials and methods
1413 patients with bladder and 810 cases with kidney cancer and 4702 controls were genotyped for two PALB2 variants: 509_510delGA and 172_175delTTGT.

Results
Two mutations of PALB2 gene were detected in 5 of 1413 (0.35%) unselected bladder cases and in 10 of 4702 controls (odds ratio [OR], 1.7; 95% CI 0.56–4.88; p = 0.52). Among 810 unselected kidney cancer cases two PALB2 mutations were reported in two patients (0,24%) (odds ratio [OR], (OR = 1.2; 95% CI 0.25–5.13; p = 0.84). In cases with mutations in PALB2 gene cancer family history was negative.

Conclusion
We found no difference in the prevalence of recurrent PALB2 mutations between cases and healthy controls. The mutations in PALB2 gene seem not to play a major role in bladder and kidney cancer development in Polish patients.