bluuebunny/crossref_metadata_2025_split huggingface dataset:
Pancreaticoduodenectomy for a primary duodenal capicua transcriptional repressor (CIC) -rearranged sarcoma with severe bleeding: a case report
Authors: Aoki Yuichi , Oshiro Hisashi , Yoshida Akihiko , Morishima Kazue , Miki Atsushi , Sasanuma Hideki , Sakuma Yasunaru , Lefor Alan Kawarai , Sata Naohiro 
Published: April 2020

Abstract
Background
Capicua transcriptional repressor (CIC) -rearranged sarcoma is characterized by small round cells, histologically similar to Ewing sarcoma. However, CIC-rearranged sarcoma has different clinical, histological, and immunohistochemical features from Ewing sarcoma. It is important to differentiate between these tumors.

Case presentation
The patient is a 44-year-old man with a duodenal tumor diagnosed in another hospital who presented with a history of melena. Laboratory studies showed anemia with a serum hemoglobin of 6.0 g/dL. He was hospitalized and gastrointestinal bleeding was controlled successfully with endoscopy. However, he suffered from appetite loss and vomiting and progression of anemia a few weeks after presentation. Upper gastrointestinal endoscopy showed a circumferential soft tumor in the second portion of the duodenum and the endoscope could not pass distally. Computed tomography scan showed a greater than 10 cm tumor in the duodenum, with compression of the inferior vena cava and infiltrating the ascending colon. A definitive pathologic diagnosis could not be established despite four biopsies from the tumor edge. Due to gastrointestinal obstruction and progression of anemia, a pylorus-preserving pancreaticoduodenectomy with partial resection of the inferior vena cava and right hemicolectomy was performed as a complete tumor resection. The tumor was diagnosed as a CIC-rearranged sarcoma, but 2 months postoperatively local recurrence and distant metastases to the liver and lung were found. The patient died 3 months after surgery.

Conclusions
Although the only definitive treatment for CIC-rearranged sarcoma is surgical resection, the CIC-rearranged sarcoma is highly malignant with a poor prognosis even after radical resection. More research is needed to establish optimal treatment strategies.
Combined effects of lifestyle risk factors on fatty liver index
Authors: Nivukoski Ulla , Niemelä Markus , Bloigu Aini , Bloigu Risto , Aalto Mauri , Laatikainen Tiina , Niemelä Onni 
Published: April 2020

Abstract
Background
Factors of lifestyle may have a major impact on liver-related morbidity and mortality.
We examined independent and joint effects of lifestyle risk factors on fatty liver index (FLI), a biomarker of hepatic steatosis, in a population-based cross-sectional national health survey.

Methods
The study included 12,368 participants (5784 men, 6584 women) aged 25–74 years. Quantitative estimates of alcohol use, smoking, adiposity and physical activity were used to establish a total score of risk factors, with higher scores indicating an unhealthier lifestyle. FLI was calculated based on an algorithm including body mass index, waist circumference, serum gamma-glutamyltransferase and triglycerides.

Results
The occurrence of FLI ≥ 60% indicating fatty liver increased from 2.4% in men with zero risk factors to 81.9% in those with a total risk score of 7–8 (p <  0.0005 for linear trend) and in women from 0 to 73.5% (p <  0.0005). The most striking individual impacts on the likelihood for FLI above 60% were observed for physical inactivity (p <  0.0005 for both genders) and alcohol consumption (p <  0.0005 for men). Interestingly, coffee consumption was also found to increase with increasing risk factor scores (p <  0.0005 for linear trend in both genders).

Conclusions
The data indicates that unfavorable combinations of lifestyle risk factors lead to a high likelihood of hepatic steatosis. Use of FLI as a diagnostic tool may benefit the assessment of interventions aimed at maintaining a healthy lifestyle and prevention of liver-related morbidity.
The impact of poverty transitions on frailty among older adults in South Korea: findings from the Korean longitudinal study of ageing
Authors: Youn Hin Moi , Lee Hyeon Ji , Lee Doo Woong , Park Eun-Cheol 
Published: April 2020

Abstract
Background
Frailty is an emerging public health concern among aging populations. Although socioeconomic status is a well-known contributor to frailty, there is limited research investigating the effects of poverty on frailty. This study aimed to examine the association between poverty transitions and frailty prevalence in older adults.

Methods
Data were collected from the six-wave Korean Longitudinal Study of Ageing (2006–2016). A total of 9263 middle-aged and older adults were included in the analysis. Poverty was defined as being below 50% of the median household income based on the equivalized household. Frailty was measured using an instrument comprising items on physical phenotype (grip strength) and psychological (exhaustion) and social aspects (isolation). Analyses using generalized estimating equations were conducted to estimate the relationship between poverty transition and frailty status.

Results
Among the 9263 respondents, 9.4% of the male respondents (n = 388) and 13.6% of the female respondents (n = 700) were frail. After controlling for covariates, female participants who transitioned into poverty (OR = 1.31, 95% CI: 1.02–1.69) and persistently remained in poverty (OR = 1.36, 95% CI: 1.10–1.68) showed increased odds of frailty in the follow-up year. We did not find significant results in the male participants.

Conclusions
The findings suggest that those who experience poverty transitions, enter poverty, and remain in poverty persistently are at higher risk of frailty. To improve age-related health status among the elderly, interventions aiming to prevent and reduce frailty among the elderly should target individuals who are more vulnerable to the negative effects of frailty.
Living arrangements and life satisfaction: mediation by social support and meaning in life
Authors: Lin Yan , Xiao Huimin , Lan Xiuyan , Wen Shuangshuang , Bao Shaoshao 
Published: April 2020

Abstract
Background
Living arrangements have impact on life satisfaction among older adults. However, the mechanism how it works has received less attention. This study aims to examine the mediating role of meaning in life and social support in the relationship between living arrangements and life satisfaction.

Methods

A total of 215 older adults from nine nursing homes and three communities were included in this study. The Social Support Rating Scale, Meaning in Life Questionnaire and Life Satisfaction Index A were adopted. Data were analyzed with Hayes’ s bias-corrected bootstrapping method.

Results
Both social support and presence of meaning in life had positive correlations with life satisfaction (p<0.001), and they were significant mediators between living arrangements and life satisfaction (p<0.01).

Conclusion
To improve the life satisfaction of nursing home residents, more emphasis should be placed on encouraging residents to seek or maintain a meaningful life and creating a more positive climate of social support.
The Residential Care Transition Module: a single-blinded randomized controlled evaluation of a telehealth support intervention for family caregivers of persons with dementia living in residential long-term care
Authors: Gaugler Joseph E. , Statz Tamara L. , Birkeland Robyn W. , Louwagie Katie W. , Peterson Colleen M. , Zmora Rachel , Emery Ann , McCarron Hayley R. , Hepburn Kenneth , Whitlatch Carol J. , Mittelman Mary S. , Roth David L. 
Published: April 2020

AbstractBackgroundFamilies do not fully disengage from care responsibilities following relatives’ admissions to residential long-term (RLTC) care settings such as nursing homes. Caregiver stress, depression, or other key outcomes remain stable or sometimes increase following a relative’s RLTC entry. Some interventions have attempted to increase family involvement after institutionalization, but few rigorous studies have demonstrated whether these interventions are effective in helping families navigate the potential emotional and psychological upheaval presented by relatives’ transitions to RLTC environments. The Residential Care Transition Module (RCTM) provides six formal sessions of consultation (one-to-one and family sessions) over a 4-month period to family caregivers who have admitted a relative to a RLTC setting.MethodsIn this embedded mixed methods randomized controlled evaluation, family members who have admitted a cognitively impaired relative to a RLTC setting are randomly assigned to the RCTM (n = 120) or a usual care control condition (n = 120). Primary outcomes include reductions in family members’ primary subjective stress and negative mental health outcomes; secondary role strains; and residential care stress. The mixed methods design will allow for an analysis of intervention action mechanisms by “embedding” qualitative components (up to 30 semi-structured interviews) at the conclusion of the 12-month evaluation.DiscussionThis evaluation will fill an important clinical and research gap by evaluating a psychosocial intervention designed for families following RLTC admission that determines whether and how the RCTM can help families better navigate the emotional and psychological challenges of residential care transitions.Trial registrationClinicalTrials.gov(NCT02915939, prospectively registered).
Community perceptions about dementia in southwestern Uganda
Authors: Owokuhaisa Judith , Rukundo Godfrey Zari , Wakida Edith , Obua Celestino , Buss Stephanie S. 
Published: April 2020

Abstract
Background
With the increasing number of people surviving into older age in Africa, dementia is becoming a public health concern. Understanding the social dynamics of dementia in resource-limited settings is critical for developing effective interventions. We explored community perceptions about people with dementia in southwestern Uganda.

Methods
Fifty-nine individuals (aged 19–85 years, 56% female) participated in seven focus group discussions. In addition, 22 individual in-depth interviews were conducted among individuals (aged 22–84 years, 36% female). Both interviews and focus group discussions were audio recorded, transcribed verbatim, and evaluated using a quantitative content analysis approach.

Results
Five themes were generated during content analysis: i) Labeling of the illness, ii) Presentation of the person with dementia, iii) Causation, iv) Impact of the disease on people with dementia and their caregivers and v) Views on how to address unmet needs in dementia care. Dementia was commonly referred to as “okuhuga”or “okwebwayebwa” (also, oruhuzyo/ empugye / akahuriko) which translates as “mental disorientation”. The participants reported that most people with dementia presented with forgetfulness, defecating and urinating on themselves, wandering away from home, going out naked, and picking up garbage. Some participants perceived memory problems as a normal part of the aging process, while others attributed the cause of dementia to syphilis, cancer, allergy, old age, satanic powers, witchcraft, poor nutrition, or life stress. Participants reported multiple sources of stress for caregivers of people with dementia, including financial, social, and emotional burdens. Finally, participants suggested that community and governmental organizations should be involved in meeting the needs of people with dementia and their caregivers.

Conclusions
Community members in southwestern Uganda largely identified dementia as a problem that comes with older age, and can identify key features of dementia presentation. Participants identified significant stressors affecting people with dementia and their caregivers, and reported that families and caregivers would benefit from education on the management of symptoms of dementia, and assistance in overcoming associated financial, social, and emotional burdens related to caretaking.
Burden of fatigue among adults living with HIV/AIDS attending antiretroviral therapy in Ethiopia
Authors: Gebreyesus Tsiwaye , Belay Addisalem , Berhe Gebretsadik , Haile Gebremedhin 
Published: April 2020

Abstract
Background
Fatigue is one of the most common and devastating Human Immuno-deficiency Virus (HIV) - related symptoms, with a varying prevalence in different study areas. In Ethiopia, there is a paucity of information on the magnitude and factors associated with fatigue among HIV/Acquired Immune Deficiency Syndrome (AIDS) patients. This may lead to under-diagnosis and eventually under-management of the symptom.

Methods
Institution based cross-sectional study design was conducted among 609 HIV/AIDS patients who were selected by using a systematic random sampling method. Data were collected by using interviewer administered structured questionnaire. Level of fatigue was measured by Fatigue Severity Scale.

Results
The prevalence of fatigue was found to be 51.7%. The factors associated with fatigue were: Parity [AOR = 2.01; 95% CI: 1.09–3.71], CD4 count 200–499 cells/mm3 [AOR = 2.81; 95% CI: 1.58–4.99], anemia [AOR = 4.90 95% CI: 2.40–9.97], co-morbidities [AOR = 3.65; 95% CI: 1.71–7.78], depression [AOR = 3.68 95% CI: 1.99–6.79], not being physically active [AOR = 3.20 95% CI: 1.50–6.81], clinical stage II or IV HIV [AOR = 3.11; 95% CI: 1.51–6.40] and [AOR = 4.08; 95% CI: 1.37–12.14], respectively.

Conclusion
The finding of this study revealed that fatigue is a common health problem among adult People Living with HIV (PLHIV). Factors associated with fatigue included: Parity, CD4 count 200–499 cells/mm3, Clinical Stage II or IV HIV, anemia, co-morbidities, depression, and not being physically active. The health care service needs to address the predisposing factors by provision integrated care including timely detection and treatment of comorbidities, mental health problems, and promote physical activity to slow down disease progression and then reduce exposure to fatigue.
Detection of viral RNA in diverse body fluids in an SFTS patient with encephalopathy, gastrointestinal bleeding and pneumonia: a case report and literature review
Authors: Akagi Kazumasa , Miyazaki Taiga , Oshima Kazuhiro , Umemura Asuka , Shimada Satoshi , Morita Kouichi , Senju Hiroaki , Tashiro Masato , Takazono Takahiro , Saijo Tomomi , Kurihara Shintaro , Sekino Motohiro , Yamamoto Kazuko , Imamura Yoshifumi , Izumikawa Koichi , Yanagihara Katsunori , Uda Akihiko , Morikawa Shigeru , Yoshikawa Tomoki , Kurosu Takeshi , Shimojima Masayuki , Saijo Masayuki , Mukae Hiroshi 
Published: April 2020

AbstractBackgroundSevere fever with thrombocytopenia syndrome (SFTS) is an emerging infectious disease that commonly has a lethal course caused by the tick-borne Huaiyangshan banyang virus [former SFTS virus (SFTSV)]. The viral load in various body fluids in SFTS patients and the best infection control measure for SFTS patients have not been fully established.Case presentationA 79-year-old man was bitten by a tick while working in the bamboo grove in Nagasaki Prefecture in the southwest part of Japan. Due to the occurrence of impaired consciousness, he was referred to Nagasaki University Hospital for treatment. The serum sample tested positive for SFTSV-RNA in the genome amplification assay, and he was diagnosed with SFTS. Furthermore, SFTSV-RNA was detected from the tick that had bitten the patient. He was treated with multimodal therapy, including platelet transfusion, antimicrobials, antifungals, steroids, and continuous hemodiafiltration. His respiration was assisted with mechanical ventilation. On day 5, taking the day on which he was hospitalized as day 0, serum SFTSV-RNA levels reached a peak and then decreased. However, the cerebrospinal fluid collected on day 13 was positive for SFTSV-RNA. In addition, although serum SFTSV-RNA levels decreased below the detectable level on day 16, he was diagnosed with pneumonia with computed tomography. SFTSV-RNA was detected in the bronchoalveolar lavage fluid on day 21. By day 31, he recovered consciousness completely. The pneumonia improved by day 51, but SFTSV-RNA in the sputum remained positive for approximately 4 months after disease onset. Strict countermeasures against droplet/contact infection were continuously conducted.ConclusionsEven when SFTSV genome levels become undetectable in the serum of SFTS patients in the convalescent phase, the virus genome remains in body fluids and tissues. It may be possible that body fluids such as respiratory excretions become a source of infection to others; thus, careful infection control management is needed.
Reporting a rare form of myopathy, myopathy with extrapyramidal signs, in an Iranian family using next generation sequencing: a case report
Authors: Mojbafan Marzieh , Nojehdeh Somayeh Takrim , Rahiminejad Faezeh , Nilipour Yalda , Tonekaboni Seyed Hasan , Zeinali Sirous 
Published: April 2020

Abstract
Background
Myopathy with extrapyramidal signs (MPXPS) is an autosomal recessive mitochondrial disorder which is caused by mutation in mitochondrial calcium uptake 1 (MICU1) gene located on chromosome 10q22.1. Next Generation Sequencing (NGS) technology is the most effective method for identification of pathogenic variants with the ability to overcome some limitations which Sanger sequencing may encountered. There are few reports on this rare disease around the world and here in this study we first revealed genetic identification of two affected individuals in an Iranian family with a novel mutation.

Case presentation
The proband was a 5-year-old girl from consanguenous parents. She was first clinically suspicious of affected with limb-girdle muscular dystrophy (LGMD). Muscle biopsy studies and autozygosity mapping, using four short tandem repeat (STR) markers linked to 6 genes of the most prevalent forms of LGMD, ruled out calpainopathy, dysferlinopathy, and sarcoglycanopathis. DNA sample of the proband was sent for NGS. Whole exome sequencing (WES) revealed a novel mutation c.1295delA in exon 13 of MICU1 gene. This homozygous deletion creates a frameshift and a premature stop codon downstream of canonical EF4 calcium binding motif of MICU1. According to the American College of Medical Genetics and Genomics (ACMG) guidline for sequence interpretation, this variant was a pathogenic one. Sanger sequencing in all family members confirmed the results of the WES.

Conclusions
This study was the first report of MPXPS in Iranian population which also revealed a novel mutation in the MICU1 gene.
Gastrointestinal symptoms as an extended clinical feature of Pierson syndrome: a case report and review of the literature
Authors: Nishiyama Kei , Kurokawa Mari , Torio Michiko , Sakai Yasunari , Arima Mitsuru , Tsukamoto Shoko , Obata Satoshi , Minamikawa Shogo , Nozu Kandai , Kaku Noriyuki , Maehara Yoshihiko , Sonoda Koh-Hei , Taguchi Tomoaki , Ohga Shouichi 
Published: April 2020

Abstract
Background
Pierson syndrome (PS) is a rare autosomal recessive disorder, characterized by congenital nephrotic syndrome and microcoria. Advances in renal replacement therapies have extended the lifespan of patients, whereas the full clinical spectrum of PS in infancy and beyond remains elusive.

Case presentation
We present the case of a 12-month-old boy with PS, manifesting as the bilateral microcoria and congenital nephrotic syndrome. He was born without asphyxia, and was neurologically intact from birth through the neonatal period. Generalized muscle weakness and hypotonia were recognized from 3 months of age. The infant showed recurrent vomiting at age 5 months of age, and was diagnosed with gastroesophageal reflux and intestinal malrotation. Despite the successful surgical treatment, vomiting persisted and led to severely impaired growth. Tulobuterol treatment was effective in reducing the frequency of vomiting. Targeted sequencing confirmed that he had a compound heterozygous mutation in LAMB2 (NM_002292.3: p.Arg550X and p.Glu1507X). A search of the relevant literature identified 19 patients with severe neuro-muscular phenotypes. Among these, only 8 survived the first 12 months of life, and one had feeding difficulty with similar gastrointestinal problems.

Conclusions
This report demonstrated that severe neurological deficits and gastrointestinal dysfunction may emerge in PS patients after the first few months of life.
Adapting wellbeing research tools for Aboriginal and Torres Strait Islander people with chronic kidney disease
Authors: Nagel Tricia , Sweet Michelle , Dingwall Kylie M. , Puszka Stefanie , Hughes Jaquelyne T. , Kavanagh David J. , Cass Alan , Howard Kirsten , Majoni Sandawana W. 
Published: April 2020

Abstract
Background
Chronic kidney disease is an increasingly common health problem for Aboriginal and Torres Strait Islander people. It is associated with multiple concurrent psychosocial stressors frequently resulting in negative impacts on emotional and social wellbeing. There is need for well-designed intervention studies to provide evidence of effective treatment for comorbid depression or other mental illness in this setting. Attention to early phase piloting and development work is recommended when testing complex interventions. This paper documents feasibility testing and adaptation of an existing culturally responsive brief wellbeing intervention, the Stay Strong App, and three commonly used wellbeing outcome measures, in preparation for a clinical trial testing effectiveness of the intervention.

Methods
The Stay Strong App, which has not been used in the setting of Chronic Kidney Disease before, is reviewed and adapted for people with comorbid wellbeing concerns through expert consensus between research team and an Expert Panel. The outcome measures (Kessler 10, Patient Health Questionnaire 9, and EuroQoL) are valid, reliable, and commonly used tools to assess various aspects of wellbeing, which have also not been used in this context before. Feasibility and acceptability are examined and developed through 3 stages: Pilot testing in a purposive sample of five haemodialysis patients and carers; translation of outcome measures through collaboration between the Aboriginal Interpreter Service, Aboriginal and Torres Strait Islander research officers and the research team; and conversion of translated outcome measures to electronic format.

Results
Research team and expert panel consensus led to adaptation of the Stay Strong App for renal patients through selective revision of words and images. Pilot testing identified challenges in delivery of the wellbeing measures leading to word changes and additional prompts, integration of audio translations in 11 local Indigenous languages within an interactive Outcome Measures App, and related research protocol changes.

Conclusion
Modelling the complex intervention prior to full-scale testing provided important information about the design of both the outcome measures and the intervention. These changes are likely to better support success in conduct of the clinical trial and future implementation of the intervention in clinical settings.