bluuebunny/crossref_metadata_2025_split huggingface dataset:
Enhancing disease awareness for tuberous sclerosis complex in patients with radiologic diagnosis of renal angiomyolipoma: an observational study
Authors: Bausch Kathrin , Wetterauer Christian , Diethelm Julian , Ebbing Jan , Boll Daniel T. , Dill Patricia , Rentsch Cyrill A. , Seifert Hans-H. 
Published: January 2021

Abstract
Background
Tuberous Sclerosis Complex (TSC) is a genetic disorder, with renal manifestations like angiomyolipoma (AML) occurring in 70–80% of patients. AML usually cause more complications in TCS patients than in non-TSC patients. However, AML patients are not routinely investigated for TSC. Our aim was to retrospectively assess the correlation between radiologically diagnosed AML and TSC.

Methods
All patients were stratified into AML related vs. unrelated to TSC. Correlations were calculated to determine the association between age, AML, and TSC.

Results
Complete data were available for 521 patients with renal AML, in 7 of which the concurrent diagnosis of TSC was found. Younger age significantly positively correlated with the prevalence of TSC in AML patients (p <  0.01). 37 (7%) of the 521 patients were within the age-range of 18–40 years, in which TSC occurred in 6 cases, 4 (66.7%) of which presented with multiple, bilateral renal AML (p <  0.05), and 2 (33.3%) of which with a single, unilateral AML (p <  0.05). In patients with AML but without TSC, unilateral AML was found in 83.9% and bilateral AML in 16.1% (p <  0.05). Simple binary logistic regression analysis revealed bilateral AML (OR 33.0; 95% CI 3.2–344.0; p = 0.003) (but not unilateral AML (OR 0.09; 95% CI 0.01–0.88; p = 0.04)) to be a risk factor for TSC.

Conclusions
The presence of bilateral AML in patients within the age-range of 18–40 years should raise suspicion for TSC as the underlying cause. Therefore, our advice is to refer patients with multiple bilateral renal AML for further investigations regarding TSC.
Analgesic purchases among older adults – a population-based study
Authors: Marttinen Maiju K. , Kautiainen Hannu , Haanpää Maija , Pohjankoski Heini , Hintikka Jukka , Kauppi Markku J. 
Published: January 2021

Abstract
Background
Pain is a frequent and inevitable factor affecting the quality of life among older people. Several studies have highlighted the ineffectiveness of treating chronic pain among the aged population, and little is known about the prevalence of analgesics administration among community-dwelling older adults. The objective was to examine older adults’ prescription analgesic purchases in relation to SF-36 pain in a population-based setting.

Methods
One thousand four hundred twenty community-dwelling citizens aged 62–86 years self-reported SF-36 bodily pain (pain intensity and pain-related interference) scores for the previous 4 weeks. The Social Insurance Institution of Finland register data on analgesic purchases for 6 months prior to and 6 months after the questionnaire data collection were considered. Special interest was focused on factors related to opioid purchases.

Results
Of all participants, 84% had purchased prescription analgesics during 1 year. NSAIDs were most frequently purchased (77%), while 41% had purchased paracetamol, 32% opioids, 17% gabapentinoids, and 7% tricyclic antidepressants. Age made no marked difference in purchasing prevalence. The number of morbidities was independently associated with analgesic purchases in all subjects and metabolic syndrome also with opioid purchases in subjects who had not reported any pain.

Discussion
Substantial NSAID and opioid purchases emerged. The importance of proper pain assessment and individual deliberation in terms of analgesic contraindications and pain quality, as well as non-pharmacological pain management, need to be highlighted in order to optimize older adults’ pain management.
Effects of tourniquet use on clinical outcomes and cement penetration in TKA when tranexamic acid administrated: a randomized controlled trial
Authors: Yi Zeng , Yan Li , Haibo Si , Yuangang Wu , Mingyang Li , Yuan Liu , Bin Shen 
Published: January 2021

Abstract
Background
The role of a tourniquet is still controversial for patients undergoing total knee arthroplasty (TKA). Our current study was performed to determine whether the nonuse of the tourniquet combine with tranexamic acid (TXA) application in TKA patients with end-stage osteoarthritis would accelerate the perioperative recovery rate and provide enough cement mantle thickness for implant fixation.

Methods
In this prospective, randomized controlled trial, 150 end-stage knee osteoarthritis patients receiving TKA were divided into three groups: group A (tourniquet group), group B (non-tourniquet group), and group C (tourniquet in cementation group). All enrolled patients received 3 g of intravenous TXA and 1 g topical TXA. The primary outcomes included blood loss variables and transfusion values. The secondary outcomes included VAS pain score, inflammatory factors level, range of motion, HSS score, postoperative hospital stay, and complication. Furthermore, by using a digital linear tomosynthesis technique, tibial baseplate bone cement mantle thickness was measured in four zones based on the knee society scoring system.

Results
No significant difference was found among the three groups with regards to total blood loss, transfusion, and complication. However, patients in group B showed lower inflammatory factors levels, shorter length of hospital stay, better range of motion, and lower postoperative pain. No significant difference was found among the three groups in four zones in terms of bone cement mantle thickness.

Conclusions
For end-stage knee osteoarthritis patients, the absence of tourniquet did not appear to affect blood loss and cement penetration in TKA patients. Furthermore, less inflammation reaction and better knee function can be achieved without a tourniquet. We recommend no longer use a tourniquet in primary TKA for patients with end-stage osteoarthritis when TXA is administrated.

Trial registration
ChiCTR-INR-16009026.

Level of evidence
Therapeutic Level I.
Long term disease burden post-transplantation: three decades of observations in 25 Hurler patients successfully treated with hematopoietic stem cell transplantation (HSCT)
Authors: Guffon N. , Pettazzoni M. , Pangaud N. , Garin C. , Lina-Granade G. , Plault C. , Mottolese C. , Froissart R. , Fouilhoux A. 
Published: January 2021

Abstract
Background
Mucopolysaccharidosis type I-Hurler syndrome (MPSI-H) is a lysosomal storage disease characterized by severe physical symptoms and cognitive decline. Early treatment with hematopoietic cell transplant (HSCT) is critical to the survival of these patients. While survival rates and short-term outcomes are known to be improved by HSCT, the long-term cognitive, adaptive and psychosocial functional outcomes of children with (MPSI-H) post-HSCT are not well documented. This manuscript focuses on retrospective long-term follow-up (7–33 years) of 25 MPSI-H patients, transplanted between 1986 and 2011.

Results
The median age at transplantation was 21 months (range 12–57 months). Except for one death, all successfully transplanted MPSI-H patients surviving at least 1 year after HSCT are alive to-date, with a median age of 21 years (range 8–36 years) at the last follow-up evaluation. A majority of HSCT grafts were bone marrow transplants (BMT), resulting in durable full chimerism in 18 (72%). Pre-HSCT, the onset of first symptoms occurred very early, at a median age of 3 months (range birth-16 months). The most prevalent symptoms before MPSI-H diagnosis involved progressive dysostosis multiplex; almost all patients suffered from hip dysplasia and thoracolumbar spine Kyphosis. Despite HSCT, considerable residual disease burden and ensuing corrective surgical interventions were observed in all, and at every decade of follow-up post HSCT. Late-onset psychiatric manifestations were significant (n = 17 patients; 68%), including depression in 13 patients at a median onset age of 18 years (range 13–31 years), hyperactivity and attention deficit disorder (n = 4), and multiple acute psychotic episodes (APE), independent of depression observed (n = 3) at a median onset age of 18 years (range 17–31 years). The adult Welscher Intelligence Scale results (n = 16) were heterogenous across the four scale dimensions; overall lower scores were observed on both working memory index (median WMI = 69.5) and processing speed index (median PSI = 65), whereas verbal comprehension index (median VCI = 79) and perceptual reasoning index (median PRI = 74) were higher.

Conclusion
With advanced treatment options, MPSI-H are living into 3rd and 4th decades of life, however not disease free and with poor adaptation. Residual disease (loss of mobility, limited gross and fine motor skills; low cognitive ability; suboptimal cardiopulmonary function, vision and hearing) negatively impacts the quality of life and psychosocial functioning of affected individuals.
Clusterin secreted from astrocyte promotes excitatory synaptic transmission and ameliorates Alzheimer’s disease neuropathology
Authors: Chen Fading , Swartzlander Dan B. , Ghosh Anamitra , Fryer John D. , Wang Baiping , Zheng Hui 
Published: January 2021

Abstract
Background
Genome-wide association studies have established clusterin (CLU) as a genetic modifier for late-onset Alzheimer’s disease (AD). Both protective and risk alleles have been identified which may be associated with its expression levels. However, the physiological function of clusterin in the central nervous system remains largely unknown.

Methods
We examined Clu expression in mouse brains by immunohistochemistry and high-resolution imaging. We performed electrophysiological recordings and morphological analysis of dendritic spines in wild-type and Clu knockout mice. We tested synaptic function of astrocytic Clu using neuron-glia co-cultures and by AAV-mediated astroglial Clu expression in vivo. Finally, we investigated the role of astrocytic Clu on synaptic properties and amyloid pathology in 5xFAD transgenic mouse model of AD.

Results
We show that astrocyte secreted Clu co-localizes with presynaptic puncta of excitatory neurons. Loss of Clu led to impaired presynaptic function and reduced spine density in vivo. Neurons co-cultured with Clu-overexpressing astrocytes or treated with conditioned media from HEK293 cells transfected with Clu displayed enhanced excitatory neurotransmission. AAV-mediated astroglial Clu expression promoted excitatory neurotransmission in wild-type mice and rescued synaptic deficits in Clu knockout mice. Overexpression of Clu in the astrocytes of 5xFAD mice led to reduced Aβ pathology and fully rescued the synaptic deficits.

Conclusion
We identify Clu as an astrocyte-derived synaptogenic and anti-amyloid factor; the combination of these activities may influence the progression of late-onset AD.
Effects of neoadjuvant chemotherapy on the contralateral non-tumor-bearing breast assessed by diffuse optical tomography
Authors: Altoe Mirella L. , Kalinsky Kevin , Marone Alessandro , Kim Hyun K. , Guo Hua , Hibshoosh Hanina , Tejada Mariella , Crew Katherine D. , Accordino Melissa K. , Trivedi Meghna S. , Hershman Dawn L. , Hielscher Andreas H. 
Published: January 2021

Abstract
Background
The purpose of this study is to evaluate whether the changes in optically derived parameters acquired with a diffuse optical tomography breast imager system (DOTBIS) in the contralateral non-tumor-bearing breast in patients administered neoadjuvant chemotherapy (NAC) for breast cancer are associated with pathologic complete response (pCR).

Methods
In this retrospective evaluation of 105 patients with stage II–III breast cancer, oxy-hemoglobin (ctO2Hb) from the contralateral non-tumor-bearing breast was collected and analyzed at different time points during NAC. The earliest monitoring imaging time point was after 2–3 weeks receiving taxane. Longitudinal data were analyzed using linear mixed-effects modeling to evaluate the contralateral breast ctO2Hb changes across chemotherapy when corrected for pCR status, age, and BMI.

Results
Patients who achieved pCR to NAC had an overall decrease of 3.88 μM for ctO2Hb (95% CI, 1.39 to 6.37 μM), p = .004, after 2–3 weeks. On the other hand, non-pCR subjects had a non-significant mean reduction of 0.14 μM (95% CI, − 1.30 to 1.58 μM), p > .05. Mixed-effect model results indicated a statistically significant negative relationship of ctO2Hb levels with BMI and age.

Conclusions
This study demonstrates that the contralateral normal breast tissue assessed by DOTBIS is modifiable after NAC, with changes associated with pCR after only 2–3 weeks of chemotherapy.
Primary intratesticular rhabdomyosarcoma in children: a case report and review of the literature
Authors: Yahaya James J. , Mremi Alex 
Published: January 2021

Abstract
Background
The importance of this paper is to help to emphasize the importance of chemotherapy for children with pure intratesticular rhabdomyosarcoma after radical inguinal orchiectomy is done as first treatment of rhabdomyosarcoma. The information provided in this paper about the follow-up outcomes of the patient described in this paper, it highlights that, recurrence and even metastasis of intratesticular rhabdomyosarcoma in children are more likely to occur if surgery it not combined with chemotherapy.

Case presentation
Herein, we present a 6-year old African male child with a 3 months history of a painless right intratesticular tumour. The tumour was poorly vascularized and was in continuity with the spermatic cord. Pelvic computer tomography (CT) scan showed a heterogeneous mass with well-defined margins without microcalcification and multiple bilateral inguinal enlarged lymph nodes were noticed without pelvic lymphadenopathy. The tumour measured 3.8 × 2.8 × 3.9 cm. The tumour marker panel showed: lactate dehydrogenase of (472 UI/l), alpha-fetoprotein (1.43 UI/ml) and human chorionic gonadotrophin beta (2.9 mUI/ml). Microscopically, the tumour was composed of small to medium size undifferentiated cells. These were oval to spindle, hyperchromatic cells to stromal myxoid degeneration were noted. Tunica albuginea and rete testis both were infiltrated by tumour. The tumour showed high mitotic count which measured 50 mitoses per 10 High Power Field (HPF). The diagnosis of rhabdomyosarcoma (RMS) was confirmed by immunohistochemistry (IHC) testing using myoD antibody which showed strong and diffuse intranuclear staining of the tumour cells. Currently, he is on cyclophosphamide and vincristine chemotherapy regime and his condition has improved much.

Conclusions
The experience obtained from the index case is crucial for the management of patients with intratesticular rhabdomyosarcoma which should always make sure that radical inguinal orchiectomy is covered by chemotherapy and/or radiotherapy. This will potentially lower the possibilities of recurrence and/or metastasis of the tumour, hence improving the prognosis of the patients. We report the clinical, radiological, and laboratory characteristics as well as the outcome of the patient.
Design of running training assistance system based on blockchain technology in wireless network
Authors: Ma Fuxing 
Published: January 2021

AbstractRunning exercise can increase the basal metabolic rate and increase the time of aerobic exercise. Based on the current needs of the general public for running auxiliary training, this paper combines wireless sensing and blockchain technology in the design scheme, and designs and implements a running training auxiliary technology. First, it obtains the user's gait information and other related parameters in the process through the wireless sensor network, and optimize the calculation gait in different states through the noise processing algorithm. Then, we use the blockchain technology to design a data transmission and storage plan for the protection and analysis of the user's personal privacy data. The proposed method builds a new type of sports training assistance system for the masses of modern society and contributes to the masses' physical exercise.
Mediating role diet self-efficacy plays in the relationship between social support and diet self-management for patients with type 2 diabetes
Authors: Yang Li , Li Kun , Liang Yan , Zhao Qiuli , Cui Dan , Zhu Xuemei 
Published: January 2021

Abstract
Background
It has previously been established that patients who have strong barriers to their diet self-management are more likely to have weak social support; however, the key mechanisms underlying the association between these two variables have not yet been established. This study aims to examine the potential role that diet self-efficacy plays in the relationship between social support and diet behavior in patients with type 2 diabetes mellitus (T2DM).

Methods
It was a cross-sectional survey. Three hundred-eighty patients diagnosed with T2DM were recruited for this study from five community health centers in China. The Chronic Disease Resource Scale (CIRS), Cardiac Diet Self-efficacy Scale (CDSE), and Food Control Behavior Scale (FCBS) were used to estimate participants’ utilization of social resources, diet self-efficacy, and diet self-management, respectively. The data were analyzed utilizing structural equation modelling.

Results
The results suggest that both higher levels of social support and diet self-efficacy are related to higher levels of diet self-management. The mediating effect that diet self-efficacy has on the relationship between social support and diet self-management was significant (β = .30, p < .05), explaining 55.68% of the total effect of social support on diet self-management.

Conclusions
Diet self-efficacy plays a mediating role in the association between social support and diet behavior in patients with type 2 diabetes mellitus.
Toothbrush microbiomes feature a meeting ground for human oral and environmental microbiota
Authors: Blaustein Ryan A. , Michelitsch Lisa-Marie , Glawe Adam J. , Lee Hansung , Huttelmaier Stefanie , Hellgeth Nancy , Ben Maamar Sarah , Hartmann Erica M. 
Published: January 2021

Abstract
Background
While indoor microbiomes impact our health and well-being, much remains unknown about taxonomic and functional transitions that occur in human-derived microbial communities once they are transferred away from human hosts. Toothbrushes are a model to investigate the potential response of oral-derived microbiota to conditions of the built environment. Here, we characterize metagenomes of toothbrushes from 34 subjects to define the toothbrush microbiome and resistome and possible influential factors.

Results
Toothbrush microbiomes often comprised a dominant subset of human oral taxa and less abundant or site-specific environmental strains. Although toothbrushes contained lower taxonomic diversity than oral-associated counterparts (determined by comparison with the Human Microbiome Project), they had relatively broader antimicrobial resistance gene (ARG) profiles. Toothbrush resistomes were enriched with a variety of ARGs, notably those conferring multidrug efflux and putative resistance to triclosan, which were primarily attributable to versatile environmental taxa. Toothbrush microbial communities and resistomes correlated with a variety of factors linked to personal health, dental hygiene, and bathroom features.

Conclusions
Selective pressures in the built environment may shape the dynamic mixture of human (primarily oral-associated) and environmental microbiota that encounter each other on toothbrushes. Harboring a microbial diversity and resistome distinct from human-associated counterparts suggests toothbrushes could potentially serve as a reservoir that may enable the transfer of ARGs.
Gut microbiota-derived propionate mediates the neuroprotective effect of osteocalcin in a mouse model of Parkinson’s disease
Authors: Hou Yan-fang , Shan Chang , Zhuang Si-yue , Zhuang Qian-qian , Ghosh Arijit , Zhu Ke-cheng , Kong Xiao-ke , Wang Shu-min , Gong Yan-ling , Yang Yu-ying , Tao Bei , Sun Li-hao , Zhao Hong-Yan , Guo Xing-zhi , Wang Wei-qing , Ning Guang , Gu Yan-yun , Li Sheng-tian , Liu Jian-min 
Published: January 2021

Abstract
Background
Parkinson’s disease (PD) is a neurodegenerative disorder with no absolute cure. The evidence of the involvement of gut microbiota in PD pathogenesis suggests the need to identify certain molecule(s) derived from the gut microbiota, which has the potential to manage PD. Osteocalcin (OCN), an osteoblast-secreted protein, has been shown to modulate brain function. Thus, it is of interest to investigate whether OCN could exert protective effect on PD and, if yes, whether the underlying mechanism lies in the subsequent changes in gut microbiota.

Results
The intraperitoneal injection of OCN can effectively ameliorate the motor deficits and dopaminergic neuronal loss in a 6-hydroxydopamine-induced PD mouse model. The further antibiotics treatment and fecal microbiota transplantation experiments confirmed that the gut microbiota was required for OCN-induced protection in PD mice. OCN elevated Bacteroidetes and depleted Firmicutes phyla in the gut microbiota of PD mice with elevated potential of microbial propionate production and was confirmed by fecal propionate levels. Two months of orally administered propionate successfully rescued motor deficits and dopaminergic neuronal loss in PD mice. Furthermore, AR420626, the agonist of FFAR3, which is the receptor of propionate, mimicked the neuroprotective effects of propionate and the ablation of enteric neurons blocked the prevention of dopaminergic neuronal loss by propionate in PD mice.

Conclusions
Together, our results demonstrate that OCN ameliorates motor deficits and dopaminergic neuronal loss in PD mice, modulating gut microbiome and increasing propionate level might be an underlying mechanism responsible for the neuroprotective effects of OCN on PD, and the FFAR3, expressed in enteric nervous system, might be the main action site of propionate.
The lift snorkel technique for type Ia endoleak after fenestrated endovascular aneurysm repair of a juxtarenal abdominal aortic aneurysm
Authors: Ito Eisaku , Ohki Takao , Toya Naoki , Nakagawa Hikaru , Nishide Ryou , Okazaki Kohei , Akiba Tadashi 
Published: January 2021

Abstract
Background
The snorkel technique for a juxtarenal abdominal aortic aneurysm (JAAA) is an important treatment option for high-risk patients. We report the lift snorkel technique through the trans-femoral access for a type Ia endoleak after fenestrated endovascular aneurysm repair (FEVAR) in a case of difficult trans-brachial access.

Case presentation
A 76-year-old woman who had JAAA presented with a type Ia endoleak and sac expansion after FEVAR. We planned for proximal additional stentgraft with the bilateral renal artery snorkel technique. However, during the secondary intervention, it was difficult to cannulate to the left renal artery through the trans-brachial access due to interference of the supra-renal stent. Stentgraft was eventually delivered into the left renal artery via the trans-femoral access with a 5 Fr sheath. A plain angioplasty balloon was inserted coaxially through the sheath. The balloon was inflated in the proximal end of the stentgraft and then pushed up to replace the proximal end from down to up. The additional aortic cuff was deployed parallel to the snorkel stentgraft. One year after the additional treatment, computed tomography (CT) revealed aneurysm sac shrinkage.

Conclusion
The lift snorkel technique is a unique method converting the retrograde approach to antegrade renal artery stenting and would be an effective option for difficult trans-brachial cases for a type Ia endoleak after FEVAR of a JAAA.