bluuebunny/crossref_metadata_2025_split huggingface dataset:
A thrombophilia family with protein S deficiency due to protein translation disorders caused by a Leu607Ser heterozygous mutation in PROS1
Authors: Zhang Yan-ping , Lin Bin , Ji Yuan-yuan , Hu Ya-nan , Lin Xin-fu , Tang Yi , Zhang Jian-hui , Wu Shao-jie , Cai Sen-lin , Zhou Yan-feng , Chen Ting , Fang Zhu-ting , Luo Jie-wei 
Published: September 2021

AbstractBackgroundProtein S deficiency (PSD) is an autosomal dominant hereditary disease. In 1984, familial PSD was reported to be prone to recurrent thrombosis. Follow-up studies have shown that heterozygous protein S (PROS1) mutations increase the risk of thrombosis. More than 300PROS1mutations have been identified; among them, only a small number of mutations have been reported its possible mechanism to reduce plasma protein S (PS) levels. However, whetherPROS1mutations affect protein structure and why it can induce PSD remains unknown.MethodsThe clinical phenotypes of the members of a family with thrombosis were collected. Their PS activity was measured using the coagulation method, whereas their protein C and antithrombin III activities were measured using methods such as the chromogenic substrate method. The proband and her parents were screened for the responsible mutation using second-generation whole exon sequencing, and the members of the family were verified for suspected mutations using Sanger sequencing. Mutant and wild type plasmids were constructed and transfected into HEK293T cells to detect the mRNA and protein expression ofPROS1.ResultsIn this family, the proband with venous thrombosis of both lower extremities, the proband’s mother with pulmonary embolism and venous thrombosis of both lower extremities, and the proband’s younger brother had significantly lower PS activity and carried aPROS1c. 1820 T > C:p.Leu607Ser heterozygous mutation (NM_000313.3). However, no such mutations were found in family members with normal PS activity. The PS expression in the cell lysate and supernatant of the Leu607Ser mutant cells decreased, while mRNA expression increased. Immunofluorescence localization showed that there was no significant difference in protein localization before and after mutation.ConclusionsThe analysis of family phenotype, gene association, and cell function tests suggest that thePROS1Leu607Ser heterozygous mutation may be a pathogenic mutation. Serine substitution causes structural instability of the entire protein. These data indicate that impaired PS translation and synthesis or possible secretion impairment is the main pathogenesis of this family with hereditary PSD and thrombophilia.
Successful venous thromboprophylaxis in a patient with vaccine-induced immune thrombotic thrombocytopenia (VITT): a case report of the first reported case in Thailand
Authors: Khuhapinant Archrob , Rungjirajittranon Tarinee , Suwanawiboon Bundarika , Chinthammitr Yingyong , Ruchutrakool Theera 
Published: September 2021

Abstract
Background
Vaccine-induced immune thrombotic thrombocytopenia (VITT) is a rare but fatal complication of the Coronavirus Disease 2019 vaccine. The many reports of VITT have mostly been in the Caucasian population. Here, we present the first reported case in an Asian population.

Case presentation
A 26-year-old female had severe headache and severe thrombocytopenia 8 days after administration of the ChAdOx1 nCoV-19 vaccine developed by AstraZeneca. Although no thrombosis was demonstrated by imaging studies, she had very highly elevated d-dimer levels during hospitalization. Serology for antibodies against platelet factor 4 was positive on several days with very high optical density readings. We found that the antibody could induce spontaneous platelet aggregation without the presence of heparin. We decided to treat her with intravenous immunoglobulin, high-dose dexamethasone, and a prophylactic dose of apixaban. She improved rapidly and was discharged from the hospital 6 days after admission. Neither thrombocytopenia nor thrombosis was subsequently detected at the three-week follow-up.

Conclusions
Despite the lower rate of thrombosis, VITT can occur in the Asian population. Early detection and prompt treatment of VITT can improve the patient’s clinical outcome. Thromboprophylaxis with nonheparin anticoagulants also prevents clot formation.
Testing front-of-package warnings to discourage red meat consumption: a randomized experiment with US meat consumers
Authors: Taillie Lindsey Smith , Chauvenet Christina , Grummon Anna H. , Hall Marissa G. , Waterlander Wilma , Prestemon Carmen E. , Jaacks Lindsay M. 
Published: September 2021

AbstractBackgroundReducing red meat is a strategy to improve public health and mitigate climate change in the United States and other high-income countries. Policies requiring warnings on the front of red meat packages are a promising intervention to shift consumers towards healthier and more sustainable food choices. We aimed to explore participants’ reactions to health and environmental warning messages about red meat.MethodsBetween June and July 2020, we recruited a national convenience sample of US red meat consumers (n = 1,235; mean age 44 years) for an online survey. Participants were randomly assigned to one of four label conditions: no-label control, health warning, environment warning, and combined health and environment warning (both warnings shown side-by-side). Participants viewed three types of burritos (red meat [steak], chicken, and vegetarian) and selected their preferred item (primary outcome), the item they perceived to be most damaging to health, and the item they perceived to be most damaging to the environment (secondary outcomes). Participants then viewed their assigned warning on a series of other red meat products (no-label control participants were randomly re-assigned to one of the warning conditions) and rated the warnings on perceived message effectiveness, believability, negative emotions, perceived risk, attention, and learning something new. Finally, participants reported their intentions to reduce red meat consumption.ResultsThere were no significant differences in selection of the steak burrito between label conditions or in selection of the item most damaging to the environment. Those exposed to the health warning were more likely to select the steak burrito as most damaging to health compared to those exposed to other label conditions (health 73 %, combined 64 %, environment 60 %, no-label control 63 %,p < 0.05). The combined and health warnings elicited higher perceived message effectiveness ratings than the environment warning (combined mean 2.91, health 2.84, environment 2.61,p < 0.05).ConclusionsWarnings did not have a significant effect on item preference in the choice experiment. However, combined and health warnings performed better than the environment warning across a variety of warning label reaction measures. More research will be needed to understand whether warnings elicit behavioral change in real-world environments.Trial registrationAnalyses and hypotheses were preregistered onhttps://aspredicted.org/ph7mb.pdfon 23 June 2020.
Increased sensitivity to SMAC mimetic LCL161 identified by longitudinal ex vivo pharmacogenomics of recurrent, KRAS mutated rectal cancer liver metastases
Authors: Kryeziu Kushtrim , Moosavi Seyed H. , Bergsland Christian H. , Guren Marianne G. , Eide Peter W. , Totland Max Z. , Lassen Kristoffer , Abildgaard Andreas , Nesbakken Arild , Sveen Anita , Lothe Ragnhild A. 
Published: September 2021

AbstractTumor heterogeneity is a primary cause of treatment failure. However, changes in drug sensitivity over time are not well mapped in cancer. Patient-derived organoids (PDOs) may predict clinical drug responses ex vivo and offer an opportunity to evaluate novel treatment strategies in a personalized fashion. Here we have evaluated spatio-temporal functional and molecular dynamics of five PDO models established after hepatic re-resections and neoadjuvant combination chemotherapies in a patient with microsatellite stable and KRAS mutated metastatic rectal cancer. Histopathological differentiation phenotypes of the PDOs corresponded with the liver metastases, and ex vivo drug sensitivities generally reflected clinical responses and selection pressure, assessed in comparison to a reference data set of PDOs from metastatic colorectal cancers. PDOs from the initial versus the two recurrent metastatic settings showed heterogeneous cell morphologies, protein marker expression, and drug sensitivities. Exploratory analyses of a drug screen library of 33 investigational anticancer agents showed the strongest ex vivo sensitivity to the SMAC mimetic LCL161 in PDOs of recurrent disease compared to those of the initial metastasis. Functional analyses confirmed target inhibition and apoptosis induction in the LCL161 sensitive PDOs from the recurrent metastases. Gene expression analyses indicated an association between LCL161 sensitivity and tumor necrosis factor alpha signaling and RIPK1 gene expression. In conclusion, LCL161 was identified as a possible experimental therapy of a metastatic rectal cancer that relapsed after hepatic resection and standard systemic treatment.
Clinicians’ perceptions of a potential wearable device for capturing upper limb activity post-stroke: a qualitative focus group study
Authors: Simpson Lisa A. , Menon Carlo , Hodgson Antony J. , Ben Mortenson W. , Eng Janice J. 
Published: September 2021

Abstract
Background
There is growing interest in the use of wearable devices that track upper limb activity after stroke to help determine and motivate the optimal dose of upper limb practice. The purpose of this study was to explore clinicians’ perceptions of a prospective wearable device that captures upper limb activity to assist in the design of devices for use in rehabilitation practice.

Methods
Four focus groups with 18 clinicians (occupational and physical therapists with stroke practice experience from a hospital or private practice setting) were conducted. Data were analyzed thematically.

Results
Our analysis revealed three themes: (1) “Quantity and quality is ideal” emphasized how an ideal device would capture both quantity and quality of movement; (2) “Most useful outside therapy sessions” described how therapists foresaw using the device outside of therapy sessions to monitor homework adherence, provide self-monitoring of use, motivate greater use and provide biofeedback on movement quality; (3) “User-friendly please” advocated for the creation of a device that was easy to use and customizable, which reflected the client-centered nature of their treatment.

Conclusions
Findings from this study suggest that clinicians support the development of wearable devices that capture upper limb activity outside of therapy for individuals with some reach to grasp ability. Devices that are easy to use and capture both quality and quantity may result in greater uptake in the clinical setting. Future studies examining acceptability of wearable devices for tracking upper limb activity from the perspective of individuals with stroke are needed.
Pulmonary delivery of the broad-spectrum matrix metalloproteinase inhibitor marimastat diminishes multiwalled carbon nanotube-induced circulating bioactivity without reducing pulmonary inflammation
Authors: Young Tamara L. , Mostovenko Ekaterina , Denson Jesse L. , Begay Jessica G. , Lucas Selita N. , Herbert Guy , Zychowski Katherine , Hunter Russell , Salazar Raul , Wang Ting , Fraser Kelly , Erdely Aaron , Ottens Andrew K. , Campen Matthew J. 
Published: September 2021

Abstract
Background
Multiwalled carbon nanotubes (MWCNT) are an increasingly utilized engineered nanomaterial that pose the potential for significant risk of exposure-related health outcomes. The mechanism(s) underlying MWCNT-induced toxicity to extrapulmonary sites are still being defined. MWCNT-induced serum-borne bioactivity appears to dysregulate systemic endothelial cell function. The serum compositional changes after MWCNT exposure have been identified as a surge of fragmented endogenous peptides, likely derived from matrix metalloproteinase (MMP) activity. In the present study, we utilize a broad-spectrum MMP inhibitor, Marimastat, along with a previously described oropharyngeal aspiration model of MWCNT administration to investigate the role of MMPs in MWCNT-derived serum peptide generation and endothelial bioactivity.

Results
C57BL/6 mice were treated with Marimastat or vehicle by oropharyngeal aspiration 1 h prior to MWCNT treatment. Pulmonary neutrophil infiltration and total bronchoalveolar lavage fluid protein increased independent of MMP blockade. The lung cytokine profile similarly increased following MWCNT exposure for major inflammatory markers (IL-1β, IL-6, and TNF-α), with minimal impact from MMP inhibition. However, serum peptidomic analysis revealed differential peptide compositional profiles, with MMP blockade abrogating MWCNT-derived serum peptide fragments. The serum, in turn, exhibited differential potency in terms of inflammatory bioactivity when incubated with primary murine cerebrovascular endothelial cells. Serum from MWCNT-treated mice led to inflammatory responses in endothelial cells that were significantly blunted with serum from Marimastat-treated mice.

Conclusions
Thus, MWCNT exposure induced pulmonary inflammation that was largely independent of MMP activity but generated circulating bioactive peptides through predominantly MMP-dependent pathways. This MWCNT-induced lung-derived bioactivity caused pathological consequences of endothelial inflammation and barrier disruption.
Depression and anxiety symptoms in young adults before and during the COVID-19 pandemic: evidence from a Canadian population-based cohort
Authors: Watkins-Martin Kia , Orri Massimiliano , Pennestri Marie-Hélène , Castellanos-Ryan Natalie , Larose Simon , Gouin Jean-Philippe , Ouellet-Morin Isabelle , Chadi Nicholas , Philippe Frederick , Boivin Michel , Tremblay Richard E. , Côté Sylvana , Geoffroy Marie-Claude 
Published: September 2021

Abstract
Background
Concerns have been raised that the COVID-19 pandemic could increase risk for adverse mental health outcomes, especially in young adults, a vulnerable age group. We investigated changes in depression and anxiety symptoms (overall and severe) from before to during the pandemic, as well as whether these changes are linked to COVID-19-related stressors and pre-existing vulnerabilities in young adults followed in the context of a population-based cohort.

Method
Participants (n = 1039) from the Quebec Longitudinal Study of Child Development reported on their depression (Centre for Epidemiological Studies Depression Scale, short form) and anxiety (General Anxiety Disorder-7 Scale) symptoms and completed a COVID-19 questionnaire during the first wave of the COVID-19 pandemic in the summer of 2020 (age 22 years). Assessments at age 20 (2018) were used to estimate pre-pandemic depression and anxiety symptom severity.

Results
While mean levels of depression and anxiety symptoms did not change from before to during the first wave of the COVID-19 pandemic (e.g., the mean of depressive symptoms was 9.30 in 2018 and 9.59 in 2020), we observed a slight increase in rates of severe depression (scores ≥ 21) from before (6.1%) to during (8.2%) the pandemic. Most COVID-19-related variables (e.g., loss of education/occupation, frequent news-seeking) – except living alone – and most pre-existing vulnerabilities (e.g., low SES, low social support) were not associated with changes in depression or anxiety symptoms. However, results varied as a function of pre-pandemic levels of depression and anxiety: depression and anxiety symptoms increased among adults with the lowest levels of symptoms before the pandemic, while they decreased among those with the highest levels of symptoms, possibly reflecting a regression to the mean.

Conclusions
Depression and anxiety symptoms in young adults from Québec in Summer 2020 were mostly comparable to symptoms reported in 2018. Most COVID-19-related stressors and pre-existing vulnerabilities were not associated with changes in symptoms, except living alone and pre-existing symptoms of depression and anxiety. However, the increased rate of severe depression warrants further investigation.
In situ conservation of traditional vegetable diversity in Wa homegardens in southwestern Yunnan, China
Authors: Shao Hua , Hill Rosemary , Xue Dayuan , Yang Jingbiao 
Published: September 2021

Abstract
Background
Homegardens are in situ conservation sources of germplasm diversity for overcoming homogenous germplasm problems in industrial agricultural systems. The Wa people constitute a long-dwelling ethnic group mainly in southwestern Yunnan with a unique culture and rich knowledge of traditional vegetables. We hypothesized that traditional vegetable varieties are well conserved in Wa homegardens because Wa culture promotes the preservation of traditional vegetables. We surveyed vegetable varieties and the practices that are involved in the conservation of traditional vegetables in Wa homegardens, which could form the basis for in situ conservation.

Methods
The methods were used including questionnaires and semi-structured interviews. Sixty homegardens were surveyed through purposive sampling in 6 Wa villages. We documented ethnobotanical information about vegetables in homegardens. Plant species were identified according to the Flora of China. And thematic analyses were conducted for in-depth interviews to identify the conservation factors for traditional vegetables.

Results
Fifty-two vegetable species belonging to 16 families and 41 genera were recorded from 60 Wa homegardens. Fifty-five traditional vegetable varieties and thirty-six hybrids were recorded. Among all the villages, 23 ± 6 (average ± SD) traditional vegetable varieties per homegarden and 9 ± 3 (average ± SD) introduced varieties per homegarden were recorded. Local seeds were stored in 78% of households, with an additional 9% of households’ seed supplies coming from neighbors and relatives; the other 13% of households purchased local seeds from markets. In 83% of families, the female head was mainly responsible for the decision-making concerning traditional vegetables in homegardens; in 10% of families, the male head was responsible for decision-making, and a small percentage (2%) was determined by elderly people. Five percent of families made decisions jointly between male and female household heads.

Conclusions
This study demonstrated that rich traditional germplasm diversity is harbored in Wa homegardens because of the unique culture and traditional knowledge of Wa communities, which are practiced daily with homegrown food plants. Local vegetable seed conservation and sharing systems help maintain germplasm diversity in the Wa community homegardens. Wa homegardens constitute a practical solution for protecting traditional germplasm diversity and maintaining traditional lifestyles.
Subtotal cholecystectomy for difficult acute cholecystitis: how to finalize safely by laparoscopy—a systematic review
Authors: Toro Adriana , Teodoro Michele , Khan Mansoor , Schembari Elena , Di Saverio Salomone , Catena Fausto , Di Carlo Isidoro 
Published: September 2021

Abstract
Background
Aim of this study was to clarify the best laparoscopic subtotal cholecystectomy (LSTC) technique for finalizing a difficult cholecystectomy.

Patients and methods
A review was performed (1987–2021) searching "difficulty cholecystectomy" AND/OR "subtotal cholecystectomy". The LSTC techniques considered were as follows: type A, leaving posterior wall attached to the liver and the remainder of the gallbladder stump open; type B, like type A but with the stump closed; type C, resection of both the anterior and posterior gallbladder walls and the stump closed; type D, like type C but with the stump open. Morbidity (including mortality) was analysed with Dindo–Clavien classification.

Results
Nineteen articles were included. Of the 13,340 patients screened, 678 (8.2%) had cholecystectomy finalized by LSTC: 346 patients (51.0%) had type A LSTC, 134 patients (19.8%) had type B LSTC, 198 patients (29.2%) had type C LSTC, and 198 patients (0%) had type D LSTC. Bile leakage was found in 83 patients (12.2%), and recorded in 58 patients (69.9%) treated by type A. Twenty-three patients (3.4%) developed a subhepatic collection, 19 of whom (82.6%) were treated by type A. Other complications were reported in 72 patients (10.6%). The Dindo–Clavien classification was four for grade I, 27 for grade II, 126 for grade IIIa, 18 for grade IIIb, zero for grade IV and three for grade V.

Conclusion
In the case of LSTC, closure of the gallbladder stump represents the best method to avoid complications. Careful exploration of the gallbladder stump is mandatory, washing the abdominal cavity and leaving drainage.
Trampoline-related fractures of the proximal tibia in children
Authors: Jeong Changhoon , Lee Sang Uk , Kim Hyun Gyun , Joo Sun Young 
Published: September 2021

Abstract
Background
Trampoline-related fractures of the proximal tibial metaphysis are common in children and have been linked to subsequent valgus deformity of the tibia. The purpose of this study was to investigate the characteristics of trampoline-related proximal tibial fractures in young children.

Methods
We evaluated 40 patients with proximal tibial fracture after trampolining between 2013 and 2019. The median duration of follow-up was 18 months. Standing long leg radiographs were obtained at the last follow-up to evaluate angular deformity and limb length inequality in the patients. The measurements recorded include the lower limb length, mechanical lateral distal femoral angle (mLDFA), medial proximal tibial angle (MPTA), mechanical axis deviation (MAD), and anatomical tibio-femoral angle (aTFA). The anterior tilt angle (ATA) was measured using a lateral radiograph of the tibia.

Results
The median age at injury was 40.0 months. Using trampoline with a heavier person was the most common mechanism of injury. aTFA and MAD were found to be increased towards the valgus at the last follow-up in our patient; however, the increase was not statistically significant (p = 0.692 and p = 0.973, respectively). The anterior tilt angle was increased in the injured leg at the last follow-up. But the change was not statistically significant (p = 0.09).

Conclusions
Using trampoline with a heavier person carries the risk of trampoline-related proximal tibial fracture in young children. We did not find a significant change in limb alignment at a minimum of one year of follow-up.