bluuebunny/crossref_metadata_2025_split huggingface dataset:
Breaking barriers with tofersen: Enhancing therapeutic opportunities in amyotrophic lateral sclerosis
Authors: Saini Aniket , Chawla Pooja A. 
Published: November 2023

AbstractBackground and purposeAmyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease that primarily affects adults, characterized by muscle weakness resulting from the specific death of motor neurons in the spinal cord and brain. The pathogenesis of ALS is associated with the accumulation of mutant superoxide dismutase 1 (SOD1) proteins and neurofilaments in motor neurons, highlighting the critical need for disease‐modifying treatments. Current therapies, such as riluzole and edaravone, provide only symptomatic relief. Recently, tofersen gained approval from the US FDA under the brand name Qalsody as the first and only gene therapy for ALS, addressing a significant pathological aspect of the disease.MethodsWe carried out a literature survey using PubMed, Scopus, National Institutes of Health, and Biogen for articles published in the English language concerned with “amyotrophic lateral sclerosis”, pathophysiology, current treatment, treatment under clinical trial, and the newly approved drug “tofersen” and its detailed summary.ResultsA comprehensive review of the literature on the pathophysiology, available treatment, and newly approved drug for this condition revealed convincing evidence that we are now able to better monitor and treat ALS.ConclusionsAlthough treatment of ALS is difficult, the newly approved drug tofersen has emerged as a potential therapy to slow down the progression of ALS by targeting SOD1 mRNA, representing a significant advancement in the treatment of ALS.
Efficacy and safety of the implantable, magnetic resonance imaging‐compatible StimRouter neuromodulation system in the treatment of refractory lower urinary tract symptoms in multiple sclerosis patients
Authors: Sacco Rosaria , Maino Paolo , Koetsier Eva , Disanto Giulio , Renard Julien , Digesu Alex , Gobbi Claudio , Zecca Chiara 
Published: November 2023

AbstractBackground and purposeLower urinary tract symptoms (LUTS) significantly affect quality of life (QoL) of multiple sclerosis (MS) patients, and pharmacotherapy has limited efficacy. We investigated efficacy and safety of the implantable StimRouter neuromodulation system for treating refractory LUTS in MS.MethodsThis prospective, single‐center, clinical trial was conducted at the Multiple Sclerosis Center of Lugano, Switzerland, involving MS patients treated with self‐administered percutaneous tibial nerve stimulation delivered by StimRouter over 24 weeks. Changes in video‐urodynamic parameters as well as LUTS severity were measured by Overactive Bladder Questionnaire (OAB‐q), QoL using the Multiple Sclerosis Quality of Life (MSQoL‐54), and treatment satisfaction using a 1–10 visual analogue scale. Adverse events were also recorded.ResultsOf 23 MS patients recruited, six had neurogenic detrusor overactivity (NDO), five had detrusor sphincter dyssynergia (DSD), and 12 had both NDO and DSD. Of patients with NDO, median bladder volume at first uninhibited contraction significantly increased from baseline to week 24 (median = 136 mL, interquartile range [IQR] = 101–244 mL vs. 343 mL, IQR = 237–391 mL; β = 138.2, p = 0.001). No significant changes of urodynamic parameters were found in patients with DSD. OAB‐q symptom scores progressively decreased, and OAB‐q quality of life scores increased (β = −0.50, p < 0.001 and β = 0.47, p < 0.001, respectively), whereas MSQoL‐54 scores did not significantly change (β = 0.24, p = 0.084) in the overall population. Treatment satisfaction was overall high (median = 8, IQR = 6–9). No serious adverse events were recorded.ConclusionsStimRouter represents a minimally invasive, magnetic resonance imaging‐compatible, self‐administered neuromodulation device leading to objective and subjective improvements of OAB symptoms and related QoL in MS patients with refractory LUTS.
Caregivers' burden and deep brain stimulation for Parkinson disease: A systematic review of qualitative studies
Authors: Cavallieri Francesco , Ghirotto Luca , Sireci Francesca , Parmeggiani Margherita , Pedroni Cristina , Mardones Felipe Andres , Bassi Maria Chiara , Fioravanti Valentina , Fraix Valérie , Moro Elena , Valzania Franco 
Published: November 2023

AbstractBackground and purposeThe impact of subthalamic nucleus deep brain stimulation (STN‐DBS) on caregivers' burden is understudied. We perform a systematic review and meta‐synthesis aggregating qualitative studies involving partners of people with Parkinson disease (PwP) to explore their experiences and unmet needs.MethodsA systematic review for retrieving qualitative studies included six databases: MEDLINE, Embase, CINAHL, Cochrane, PsycInfo, and Scopus. Inclusion criteria were as follows: (i) studies on the experience of caregivers of PwP in the context of STN‐DBS, (ii) English peer‐reviewed articles, and (iii) qualitative or mixed methods studies reporting caregivers' quotations. After the appraisal of included studies, we performed meta‐synthesis of qualitative findings. Descriptive themes and conceptual elements related to PwP partners' experiences and unmet needs were generated.ResultsA total of 1108 articles were screened, and nine articles were included. Three categories were identified: (i) dealing with Parkinson disease (PD) every day (the starting situation characterized by the impact of PD on ordinary life; the limitations to partners' socialization; partners' efforts in stepping aside for love and care activities), (ii) facing life changes with STN‐DBS (the feeling of being unprepared for changes; the fear and concern due to loved ones' behavioral changes; struggling to find an explanation for those changes), and (iii) rebuilding the role of caregiver and partner after STN‐DBS.ConclusionsThis meta‐synthesis elucidates concerns, challenges, and unmet needs of partners of PwP who underwent STN‐DBS. It is important to provide them with information, education, and adequate support to face these challenges. Professionals need to involve partners in the care and decision process, because STN‐DBS‐related outcomes do not depend solely on the well‐being of PwP but also on the well‐being of individuals surrounding them.
Exosomes derived from human dermal fibroblasts (HDFn‐Ex) alleviate DNCB‐induced atopic dermatitis (AD) via PPARα
Authors: Jang You Na , Lee Jung Ok , Lee Jung Min , Park A. Yeon , Kim Yu Jin , Kim Su Young , Seok Joon , Yoo Kwang Ho , Kim Beom Joon 
Published: November 2023

AbstractAtopic dermatitis (AD) is a chronic inflammatory skin disease. Skin barrier dysfunction is the initial step in the development of AD. Recently, exosomes have been considered as potential cell‐free medicine for skin defects such as aging, psoriasis and wounds. The aim of this study was to investigate the effects of human dermal fibroblast‐neonatal‐derived exosome (HDFn‐Ex) on AD. HDFn‐Ex increased the expression of peroxisome proliferator activated receptor α (PPARα) and alleviated the 1‐chloro‐2,4‐dinitrobenzene (DNCB)‐mediated downregulation of filaggrin, involucrin, loricrin, hyaluronic acid synthase 1 (HAS1) and HAS2 in human keratinocyte HaCaT cells. However, these effects were inhibited by the PPARα antagonist GW6471. In the artificial skin model, HDFn‐Ex significantly inhibited DNCB‐induced epidermal hyperplasia and the decrease in filaggrin and HAS1 levels via a PPARα. In the DNCB‐induced AD‐like mouse model, HDFn‐Ex administration reduced epidermis thickening and mast cell infiltration into the dermis compared to DNCB treatment. Moreover, the decreases in PPARα, filaggrin and HAS1 expression, as well as the increases in IgE and IL4 levels induced by DNCB treatment were reversed by HDFn‐Ex. These effects were blocked by pre‐treatment with GW6471. Furthermore, HDFn‐Ex exhibited an anti‐inflammatory effect by inhibiting the DNCB‐induced increases in IκBα phosphorylation and TNF‐α expression. Collectively, HDFn‐Ex exhibited a protective effect on AD. Notably, these effects were regulated by PPARα. Based on our results, we suggest that HDFn‐Ex is a potential candidate for treating AD by recovering skin barrier dysfunction and exhibiting anti‐inflammatory activity.
Patient headache questionnaires can improve headache diagnosis and treatment in children
Authors: Szperka Christina L. , Witzman Stephanie , Ostapenko Svetlana , Farrar John T. , Hsu Jesse Yenchih , Malavolta Carrie P. , Bunney Janille D. , Bange Erin M. , Patterson Gentile Carlyn , Velasquez Gerardo , Marquez de Prado Blanca , Cosico Mahgenn , Lee Meyeon , Pojomovsky McDonnell Pamela , Prelack Marisa S. , Chadehumbe Madeline A. , Stephenson Donna J. , Kichula Elizabeth A. , Tomaine Scott C. , Hershey Andrew D. 
Published: November 2023

AbstractObjectiveTo examine trends in diagnosis of headache and migraine in a large pediatric neurology cohort, and test whether an electronic health record (EHR)‐integrated headache questionnaire can increase specificity of diagnosis and likelihood of prescribing migraine treatment.BackgroundUnder‐diagnosis of migraine contributes to the burden of disease. As we founded our Pediatric Headache Program in 2013, we recognized that the proportion of patients with headache who were given a diagnosis of migraine was much lower than expected.MethodsWe developed a patient headache questionnaire, initially on paper (2013–2014), then in an electronic database (2014–2016), and finally integrated into our electronic health record (pilot: 2016, full: May 2017). We compared diagnoses and prescribed treatments for new patients who were given a headache diagnosis, looking at trends in the proportion of patients given specific diagnoses (migraine, etc.) versus the non‐specific diagnosis, “headache.” Next, we conducted a prospective cohort study to test for association between provider use of the form and the presence of a specific diagnosis, then for an association between specific diagnosis and prescription of migraine treatment.ResultsBetween July 2011 and December 2022 the proportion of new headache patients who were given a diagnosis of migraine increased 9.7% and non‐specific headache diagnoses decreased 21.0%. In the EHR cohort (June 2017–December 2022, n = 15,122), use of the provider form increased the rate of specific diagnosis to 87.2% (1839/2109) compared to 75.5% (5708/7560) without a patient questionnaire, nearly doubling the odds of making a specific diagnosis (odds ratio [OR] 1.90, 95% confidence interval [CI]: 1.65–2.19). Compared to those given only a non‐specific headache diagnosis who were prescribed a migraine therapy 53.7% (1766/3286) of the time, 75.3% (8914/11836) of those given a specific diagnosis received a migraine therapy, more than doubling the odds of prescription (OR 2.39, 95% CI: 2.20–2.60).ConclusionsInterventions to improve specificity of diagnosis were effective and led to increased rates of prescription of migraine treatments. These results have been sustained over several years. This headache questionnaire was adapted into the Foundation system of EpicCare, so it is broadly available as a clinical and research tool for institutions that use this EHR software.
CARIN' about migrants through news? Linking migrant deservingness to traditional and digital media consumption
Authors: De Coninck David , Duque Maria , Schwartz Seth J. , d'Haenens Leen 
Published: November 2023

AbstractIn recent years, public discourse and political actors have increasingly used a deservingness rhetoric to refer to the arrival and permanent settlement of migrant groups. However, scholars have drawn on the concept of deservingness without developing a clear theoretical framework for it. Following our recent work on the migrant deservingness framework, in the present study we use the CARIN criteria (Control, Attitude, Reciprocity, Identity, Need) to establish the extent to which host nationals in eight nations impose conditions towards accepting permanent settlement among migrants. Specifically, we examine the links of these deservingness perceptions with news media consumption in seven European countries and Colombia using online survey panel data (N = 12,142). Our findings indicate that consuming news via commercial television and in popular newspapers, in particular, is linked to greater conditionality regarding migrant settlement. Consuming public television or quality news sources is only weakly linked to (reduced) conditionality. We discuss these findings using the migrant deservingness framework.
Recurrent Lupus Vulgaris Following Repeated BCG (Bacillus Calmette Guèrin) Vaccination
Authors: Şaşmaz Rüstem , Altinyazar H. Cevdet , Tatlican Semih , Eskioğlu Fatma , Yurtsever Pinar 
Published: November 2023

AbstractWe report a case of lupus vulgaris following BCG (Bacillus Calmette Guèrin) vaccination on the right shoulder. The patient had a history of lupus vulgaris on his left shoulder that developed following BCG vaccination and was treated successfully eight years ago.
Prognostic Value of Ki‐67, CD31 and Epidermal Growth Factor Receptor Expression in Basal Cell Carcinoma
Authors: Yerebakan Özlem , Çiftçiog͂lu Mehmet Akif , Akkaya Bahar Kiliçarslan , Yilmaz Ertan 
Published: November 2023

AbstractRecurrence of basal cell carcinoma following treatment is common, and the majority of recurrences appear in the first 3 years. We examined the original tumors of 26 basal cell carcinoma cases, 14 of whom had a recurrence after an average of 3.7 years, and 12 of whom had no recurrence during an average of 4.4 years follow‐up. Using immunohistochemistry, we tested for Ki‐67, CD31 and epidermal growth factor receptor expressions in the tumor tissue. The percentages of expression for Ki‐67, CD31 and epidermal growth factor receptor were significantly higher in the recurrent tumors than in the non‐recurrent ones. Expression of Ki‐67 and CD31 was 271.57 ± 17.91 and 58.1 ± 9.37 for the recurrent group and 187.08 ± 21.48 and 23.9 ± 5.45 for non‐recurrent group respectively (p<0.0001; p<0.0001). Expression of epidermal growth factor receptor was positive in all basal cell carcinoma cells. The staining intensity was strong in 57% of recurrent and 8.3% of non‐recurrent tumors (p=0.014). These results show that Ki‐67, CD31 and epidermal growth factor receptor expression differ between basal cell carcinomas which later recur and those that do not recur.
Bullous Pyoderma Gangrenosum Complicated by Disseminated Intravascular Coagulation with Subsequent Myelodysplastic Syndrome (Chronic Myelomonocytic Leukemia)
Authors: Rogalski Christina , Paasch Uwe , Glander Hans‐Jürgen , Haustein Uwe‐Frithjof 
Published: November 2023

AbstractA 33‐year‐old woman developed a bullous PG precursing a chronic myelomonocytic leukemia (CMML) complicated by life‐threatening, disseminated, intravascular coagulation after administration of systemic corticosteroids in combination with immunosuppressant and antibiotic agents. Although the association between PG and leukemia, as well as the coincidence of disseminated intravascular coagulation (DIC) and leukemia, is well known, a premonitoring effect of PG in combination with DIC preceding the diagnosis of chronic myelomonocytic leukemia in the same patient has not been reported recently.
The management of digital competence sharing in health care: A qualitative study of managers' and professionals' views
Authors: Hammarén Mira , Pölkki Tarja , Kanste Outi 
Published: November 2023

AbstractAimTo describe managers' and professionals' views on the management of digital competence sharing in health care.DesignA qualitative descriptive study.MethodsManagers (n = 22) and professionals (n = 12) from two public primary, one public special and one private health care organization in Finland participated in semi‐structured individual interviews between February and May 2022. Data were analysed using inductive content analysis.ResultsManagers' and professionals' views formed six main categories: providing resources and opportunities for digital competence sharing, creating methods and practices for digital competence sharing, managing digital competence, implementing intergenerational learning, creating a friendly and safe digital organizational atmosphere, and promoting digital competence sharing through leadership.ConclusionThe support of management is significant in promoting the sharing of digital competence in health care organizations. The management of digital competence sharing requires a both resources and a commitment to continuous training and development. It also requires the creation of a collaborative culture to promote mutual learning between professionals and people of different generations.ImpactDue to rapid technological advancements and the resulting load, it is important to focus on the development and sharing of digital competence among health care professionals. The study indicated that managers should have the ability to identify the strengths of professionals' digital competence and be able to utilize them in promoting digital competence sharing. It also highlighted the specific competency requirements for managers in this context. The results can be applied to the training of health care managers and professionals, particularly around digital competence.Patient or Public ContributionNo patient or public contribution.Reporting MethodThe Consolidated Criteria for Reporting Qualitative Research (COREQ) checklist was used in the reporting.
Pulsed‐field ablation does not induce esophageal and periesophageal injury—A new esophageal safety paradigm in catheter ablation of atrial fibrillation
Authors: Grosse Meininghaus Dirk , Freund Robert , Koerber Britta , Kleemann Tobias , Matthes Harald , Geller Johann Christoph 
Published: November 2023

AbstractIntroductionEsophageal injury is one of the most serious complications of pulmonary vein isolation (PVI) with thermic energy sources. Better tissue selectivity of primarily non‐thermic pulsed field ablation (PFA) may eliminate collateral injury, particularly the risk of atrio‐esophageal fistula (AEF).ObjectiveTo compare the incidence of any (peri)‐esophageal injury following PVI using PFA to thermic energy sources.MethodsUsing endoscopy, endoscopic ultrasound, and electrogastrography before and after PVI, esophageal and periesophageal injury (mucosal lesions, food retention, periesophageal edema, or vagal nerve injury) were assessed following PFA and radiofrequency (RF)‐ or cryoballoon (CB)‐PVI.ResultsBetween December 2022 and February 2023, 20 patients (67 ± 10 years, 53% male) undergoing PFA (Farapulse, Boston Scientific) for atrial fibrillation (AF) were studied and compared with a previous cohort of 57 patients who underwent thermic PVI (CB: n = 33; RF: n = 24). Following PFA‐PVI, none of the patients had mucosal lesions, food retention, or ablation‐induced vagal nerve injury; four patients showed periesophageal edema. Following thermic ablation, 33/57 patients (58%) showed esophageal and/or periesophageal injury (CB: 21/33 [64%], RF: 12/24 [50%]), in detail 4/57 mucosal lesions, 18/57 food retention, 17/57 vagal nerve injury, and 20/52 edema. Midterm success rates were similar for all energy sources.ConclusionIn contrast to thermic ablation tools, PFA is not associated with relevant esophageal and periesophageal injury, and might, therefore, reduce or eliminate the risk of potentially lethal AEF in interventional treatment of AF. The etiology of ablation‐induced periesophageal edema is unknown but has not been shown to be related to lesion progression.
Lysoglycosphingolipids have the ability to induce cell death through direct PI3K inhibition
Authors: Watanabe Ryosuke , Tsuji Daisuke , Tanaka Hiroki , Uno Michael Shintaro , Ohnishi Yukiya , Kitaguchi Shindai , Matsugu Tsuyoshi , Nakae Ryuto , Teramoto Hiromi , Yamamoto Kei , Shinohara Yasuo , Hirokawa Takatsugu , Okino Nozomu , Ito Makoto , Itoh Kohji 
Published: November 2023

AbstractSphingolipidoses are inherited metabolic disorders associated with glycosphingolipids accumulation, neurodegeneration, and neuroinflammation leading to severe neurological symptoms. Lysoglycosphingolipids (lysoGSLs), also known to accumulate in the tissues of sphingolipidosis patients, exhibit cytotoxicity. LysoGSLs are the possible pathogenic cause, but the mechanisms are still unknown in detail. Here, we first show that lysoGSLs are potential inhibitors of phosphoinositide 3‐kinase (PI3K) to reduce cell survival signaling. We found that phosphorylated Akt was commonly reduced in fibroblasts from patients with sphingolipidoses, including GM1/GM2 gangliosidoses and Gaucher's disease, suggesting the contribution of lysoGSLs to the pathogenesis. LysoGSLs caused cell death and decreased the level of phosphorylated Akt as in the patient fibroblasts. Extracellularly administered lysoGM1 permeated the cell membrane to diffusely distribute in the cytoplasm. LysoGM1 and lysoGM2 also inhibited the production of phosphatidylinositol‐(3,4,5)‐triphosphate and the translocation of Akt from the cytoplasm to the plasma membrane. We also predicted that lysoGSLs could directly bind to the catalytic domain of PI3K by in silico docking study, suggesting that lysoGSLs could inhibit PI3K by directly interacting with PI3K in the cytoplasm. Furthermore, we revealed that the increment of lysoGSLs amounts in the brain of sphingolipidosis model mice correlated with the neurodegenerative progression. Our findings suggest that the down‐regulation of PI3K/Akt signaling by direct interaction of lysoGSLs with PI3K in the brains is a neurodegenerative mechanism in sphingolipidoses. Moreover, we could propose the intracellular PI3K activation or inhibition of lysoGSLs biosynthesis as novel therapeutic approaches for sphingolipidoses because lysoGSLs should be cell death mediators by directly inhibiting PI3K, especially in neurons.
Nephrology nurses' perspectives working with patients experiencing needle‐related distress
Authors: Duncanson Emily , Le Leu Richard K. , Chur‐Hansen Anna , Masotti Tahlia , Collins Kathryn L. , Burke Anne L. J. , Macauley Luke P. , McDonald Stephen , Jesudason Shilpanjali 
Published: November 2023

AbstractBackgroundA key skill of nephrology nursing is cannulation of patients receiving haemodialysis. Traumatic and unsuccessful cannulation experiences, particularly in the initial weeks of haemodialysis, may contribute to the onset of needle distress for patients.ObjectivesTo identify the key knowledge, skills and attitudes of nephrology nurses working with haemodialysis patients and the competencies relevant to nephrology nursing working with patients with needle‐related distress.DesignA qualitative study involving semistructured interviews. Interviews were audio‐recorded, transcribed and deductive, and inductive thematic analysis applied.ParticipantsNephrology nurses (n = 17) were interviewed from a tertiary kidney service in South Australia. Nurses had varying roles and years of experience (range 1–30 years) working with dialysis patients within the service.ResultsTwo overarching themes, (1) Flexibility in Practice and Care and (2) Responsibility of Nephrology Nursing, were identified as relevant across all knowledge, skills and attitudes of nephrology nurses working with patients with needle‐related distress. Thirty‐six knowledge, skills and attitudes were identified; 12 related to knowledge, 14 related to skills and 10 were identified as attitudes and were summarised under seven broad competencies.ConclusionThis study identifies potential knowledge, skills and attitudes and competencies required for nephrology nurses working with patients with needle‐related distress. It highlights strategies that may prevent the onset and worsening of needle‐related distress, as well as reduce it. It also brings to light that nurses desire additional education regarding strategies to improve the patient experience of cannulation and nurse confidence and skill in this area.