bluuebunny/crossref_metadata_2025_split huggingface dataset:
Growth hormone treatment for adults with Prader-Willi syndrome: another point of view
Authors: Hirsch Harry J. , Gross-Tsur Varda 
Published: August 2021

AbstractGrowth hormone treatment for children with Prader Willi syndrome (PWS) has shown proven benefits not only in increasing final height but also with positive effects on body composition and motor development. In a recent letter to the editor, Hoybye and colleagues recommend growth hormone treatment for adults with PWS based exclusively on the genetic diagnosis and without regard for growth hormone secretory status. We question whether the benefits of growth hormone treatment in PWS adults, mainly improvement in body composition, are significant enough to justify the as yet unkown consequences of long-term treatment in an adult population. Morbidity and mortality in PWS are mainly due to complications of obesity, and growth hormone treatment does not result in a decrease in BMI or waist circumference. Increases in insulin-like factor-1 as a result of growth hormone treatment over the course of several decades in PWS adults raises concern over possible increase risk of cancer. Compliance with daily injections is likely to be poor. We suggest that efforts to provide appropriate dietary and exercise regimens may be more beneficial and cost-effective than advocating for growth hormone treatment for adults with PWS.
Supported online self-management versus care as usual for symptoms of fatigue, pain and urgency/incontinence in adults with inflammatory bowel disease (IBD-BOOST): study protocol for a randomised controlled trial
Authors: Norton Christine , Syred Jonathan , Kerry Sally , Artom Micol , Sweeney Louise , Hart Ailsa , Czuber-Dochan Wladyslawa , Taylor Stephanie J. C. , Mihaylova Borislava , Roukas Chris , Aziz Qasim , Miller Laura , Pollok Richard , Saxena Sonia , Stagg Imogen , Terry Helen , Zenasni Zohra , Dibley Lesley , Moss-Morris Rona 
Published: August 2021

Abstract
Background
Despite being in clinical remission, many people with inflammatory bowel disease (IBD) live with fatigue, chronic abdominal pain and bowel urgency or incontinence that limit their quality of life. We aim to test the effectiveness of an online self-management programme (BOOST), developed using cognitive behavioural principles and a theoretically informed logic model, and delivered with facilitator support.

Primary research question
In people with IBD who report symptoms of fatigue, pain or urgency and express a desire for intervention, does a facilitator-supported tailored (to patient needs) online self-management programme for fatigue, pain and faecal urgency/incontinence improve IBD-related quality of life (measured using the UK-IBDQ) and global rating of symptom relief (0–10 scale) compared with care as usual?

Methods
A pragmatic two-arm, parallel group randomised controlled trial (RCT), of a 12-session facilitator-supported online cognitive behavioural self-management programme versus care as usual to manage symptoms of fatigue, pain and faecal urgency/incontinence in IBD. Patients will be recruited through a previous large-scale survey of unselected people with inflammatory bowel disease. The UK Inflammatory Bowel Disease Questionnaire and global rating of symptom relief at 6 months are the co-primary outcomes, with multiple secondary outcomes measured also at 6 and 12 months post randomisation to assess maintenance. The RCT has an embedded pilot study, health economics evaluation and process evaluation.
We will randomise 680 patients, 340 in each group. Demographic characteristics and outcome measures will be presented for both study groups at baseline. The UK-IBDQ and global rating of symptom relief at 6 and 12 months post randomisation will be compared between the study groups.

Discussion
The BOOST online self-management programme for people with IBD-related symptoms of fatigue, pain and urgency has been designed to be easily scalable and implemented. If it is shown to improve patients’ quality of life, this trial will enable clinicians and patients to make informed management decisions. This is the first trial, to our knowledge, focused on multiple symptoms prioritised by both people with IBD and health professionals.

Trial registration
ISRCTN71618461. Registered on 9 September 2019.
Do biologic therapies reduce aortic inflammation in rheumatoid arthritis patients?
Authors: Trang D. A. M. Thuy , Okamura Koichi , Suto Takahito , Sakane Hideo , Yonemoto Yukio , Nakajima Takahito , Tsushima Yoshito , Chikuda Hirotaka 
Published: August 2021

Abstract
Objectives
Rheumatoid arthritis (RA) patients have an increased risk of cardiovascular disease (CVD). In the present study, we evaluated the inflammatory activity of the ascending aorta in RA patients who received biological treatment.

Methods
We assessed the aortic wall inflammation of RA patients using 18F-fluorodeoxyglucose (FDG) positron emission tomography/computed tomography before and after 6 months of biologic therapies. We also compared the inflammatory activity at the aortic wall in RA patients with remission or low disease activity (RLDA) and those with moderate or high disease activity (MHDA). The aortic uptake was measured by the standardized uptake value (SUV) and the target-to-background ratio (TBR).

Results
A total of 64 patients were included in the analysis (mean age, 58.4 ± 13.8 years old; female, 77%). The Disease Activity Score for 28 joints (DAS28) erythrocyte sedimentation rate (ESR) had significantly decreased after 6 months: from 5.0 ± 1.2 to 3.3 ± 1.2 (p < 0.001). The FDG uptake in the ascending aorta changed from baseline to 6 months, showing a maximum SUV (SUVmax) of 1.83 ± 0.34 to 1.90 ± 0.34 (p = 0.059) and TBR of 1.71 ± 0.23 to 1.75 ± 0.24 (p = 0.222). The SUVmax and TBR after 6 months were significantly higher in the RLDA group than in the MHDA group (2.05 ± 0.32 vs. 1.79 ± 0.33 (p = 0.002) and 1.89 ± 0.33 vs. 1.65 ± 0.20 (p = 0.001), respectively). The percentage of monocytes also significantly increased from baseline to 6 months: from 5.9 ± 1.6 to 6.9 ± 2.6 (p = 0.032).

Conclusion
The inflammation activity at the ascending aorta in RA patients did not change significantly after 6 months of biological treatment. RA patients with a low disease activity or in clinical remission after 6 months of biological treatment still had an increased inflammatory activity at the aortic wall.
Development of the external and internal shame scale: Japanese version
Authors: Hiramatsu Yoichi , Asano Kenichi , Kotera Yasuhiro , Endo Ayumu , Shimizu Eiji , Matos Marcela 
Published: August 2021

AbstractObjectiveShame contains external and internal aspects. However, a Japanese language scale for simultaneously assessing both aspects of shame has not been developed to date. This study aimed to standardize the Japanese version of the External and Internal Shame Scale (EISS-J). An online survey was conducted among university students (N = 203) at six universities in Japan (Study 1). A retest questionnaire was sent to the participants by email three weeks after the first survey (Study 2). Study 1 examined the internal consistency, factor structure, and criterion-related validity of the EISS-J, while Study 2 examined its test-retest reliability. Moreover, an additional study was conducted to examine the criterion-related validity of the scale.ResultsStudy 1 demonstrated the high internal consistency of the EISS-J. Moreover, confirmatory factor analysis indicated a two-factor model: external and internal shame. However, exploratory factor analysis indicated a three-factor structure. Study 2 confirmed the test-retest reliability of the scale. Furthermore, both studies indicated correlations between the EISS-J and fear of compassion, anger, humiliation, depression, anxiety, and stress. In addition, the study established the criterion-related validity of the scale. These results confirmed adequate reliability and validity of the EISS-J.
Polycystic liver disease with lethal abdominal wall rupture: a case report
Authors: Akuzawa Daichi , Uchida Yoichiro , Ishimura Takuya , Kakita Hiroko , Endo Tomomi , Matsuzaki Naomi , Terajima Hiroaki , Tsukamoto Tatsuo 
Published: August 2021

Abstract
Background
Polycystic liver disease is a clinical feature of autosomal dominant polycystic kidney disease, and it can sometimes cause health damage more serious than polycystic kidney. Dialysis therapy can be used for renal failure, but liver transplantation is the only method available for liver failure. Thus, giant and multiple hepatic cysts may affect mortality. However, liver transplantation is not indicated in many cases because of the preserved liver function.

Case presentation
A 54-year-old Japanese woman with polycystic liver disease was transferred back to our hospital for abdominal pain caused by liver cyst infection with abdominal wall herniation. She had been diagnosed with polycystic liver disease associated with sporadic autosomal dominant polycystic kidney disease 25 years earlier. Although she had several surgical interventions to reduce her liver volume, including right hepatic lobectomy and fenestration for liver cysts in another hospital, she needed further repair of the recurrent incisional herniation with patch graft surgery using fascia lata to cover the herniation site. However, new herniation sites reemerged in the fragile abdominal wall area around the patch, and therefore, she reduced the recurrent abdominal wall herniation by herself. Recurrent intestinal obstructions were luckily released by fasting with decompression treatment via nasogastric tube insertion, but multiple skin ulcers around the enlarged hernia sac gradually developed, and ascites was extremely difficult to control with any medication. At final admission, her abdominal wall was even more prominent, causing shortness of breath, and it spontaneously ruptured many times, which was accompanied by discharge of around 5 liters of ascites each time. She died from sepsis caused by drug-resistant Enterococcus.

Conclusions
We report a case of autosomal dominant polycystic kidney disease with ruptured abdominal wall resulting from a hepatic cyst enlargement despite multiple laparotomy operations. Throughout the entire disease course, her liver volume increased rapidly, and her quality of life was severely impaired, but she could not undergo liver transplantation after readmission to our hospital. We will discuss the therapeutic strategy for this patient, including the timing and indication for liver transplantation.
Coincidental light chain induced proximal tubulopathy with lupus nephritis: a case report and review of the literature
Authors: Hamza Wael Mostafa , AlEssa Ahmed Mohammed 
Published: August 2021

Abstract
Background
We report a case of light chain proximal tubulopathy associated with lupus nephritis in a patient known to have systemic lupus erythematosus. The kidney can be injured in several ways in any of these disorders. Light chain proximal tubulopathy is a rare form of renal tubular injury that may occur in and complicate plasma cell dyscrasia, characterized by cytoplasmic inclusions of the monoclonal light chain within proximal tubular cells. Lupus nephritis is a common form of renal injury as it occurs in about 25–50% of adult patients with systemic lupus erythematosus.

Case presentation
We present a 57-year-old African patient known to have systemic lupus erythematosus and hypertension presented with a new complaint of microscopic hematuria. A renal biopsy was performed and revealed lupus nephritis class II concurrently associated with light chain induced proximal tubulopathy. A subsequent bone marrow biopsy was performed, which revealed multiple myeloma.

Conclusions
We report a case of coincidental lupus nephritis and proximal tubulopathy featuring a combined constellation of rare histopathological features that might add to the relationship between systemic lupus and paraproteinemia.
Key residues of Bacillus thuringiensis Cry2Ab for oligomerization and pore-formation activity
Authors: Pan Zhi-Zhen , Xu Lian , Liu Bo , Chen Qing-Xi , Zhu Yu-Jing 
Published: July 2021

AbstractAs a pore-forming toxin, activation, oligomerization and pore-formation were both required for the mode of action of Cry toxins. Previous results revealed that the helices α4–α5 of Domain I were involved in the oligomerization of Cry2Ab, however, the key residues for Cry2Ab aggregation remained ambiguous. In present studies, we built 20 Cry2Ab alanine mutants site-directed in the helices α4–α5 of Domain I and demonstrated that mutants N151A, T152A, F157A, L183A, L185A and I188A could reduce the assembly of the 250 kDa oligomers, suggesting that these mutation residues might be essential for Cry2Ab oligomerization. As expected, all of these variants showed lower insecticidal activity against P. xylostella. Furthermore, we found that the pore-forming activities of these mutants also decreased when compared to wild-type Cry2Ab. Taken together, our data identified key residues for Cry2Ab oligomerization and emphasized that oligomerization was closely related to the insecticidal activity and pore-forming activity of Cry2Ab.
Effects of low lead exposure on sperm quality and sperm DNA methylation in adult men
Authors: Zhang Tiancheng , Ru Yan Fei , Wu Bin , Dong Haiyan , Chen Liang , Zheng Jufen , Li Jianhui , Wang Xin , Wang Zhikai , Wang Xuemei , Shen Xiaorong , Wu Jun , Qian Jun , Miao Maohua , Gu Yihua , Shi Huijuan 
Published: August 2021

Abstract
Instruction
Lead (Pb) exposure is a risk factor for male infertility, but the epigenetic changes in sperm DNAattributable to lead exposure is poorly defined.

Methods
In this study, we investigated whether low Pb exposure (<  10 µg/dL) affects the sperm quality. Blood, urine, and semen samples of 297 men of childbearing age were analyzed for all relevant parameters. Based on the blood Pb level (BLL), participants were allocated to RL (0–2.5 µg/dL), RM (2.5–5 µg/dL), and RH (5–10 µg/dL) groups. The 5-methylcytosine and 5-hydroxymethylcytosine patterns in the sperm DNA were identified using methylated DNA immunoprecipitation and hydroxymethylated DNA immunoprecipitation sequencing.

Results
The non-progressive motility (NP) was significantly increased and associated with global hypomethylation of sperm DNA in the RH group compared with the RL group, indicating that aberrant sperm methylation due to low Pb exposure is possibly associated with reduced sperm motility. The hypomethylated promoter regions were primarily enriched in the calcium (Ca) homeostasis pathway. Further, the interaction between Ca and Pb was associated with sperm rapid progressive motility and asthenospermia risk, although no significant methylation abnormality was observed in those with BLL  <  5 µg/dL. When BLL was  >  5 µg/dL or when predicting NP, no significant Pb–Ca interaction was observed.

Discussion
Overall, our results indicate that aberrant DNA methylation of the Ca homeostasis pathway, induced by low Pb exposure, is the potential cause for reduced sperm velocity.
Predictive performance of dynamic arterial elastance for arterial pressure response to fluid expansion in mechanically ventilated hypotensive adults: a systematic review and meta-analysis of observational studies
Authors: Zhou Xiaoyang , Pan Weihao , Chen Bixin , Xu Zhaojun , Pan Jianneng 
Published: July 2021

Abstract
Background
Dynamic arterial elastance (Eadyn) has been extensively considered as a functional parameter of arterial load. However, conflicting evidence has been obtained on the ability of Eadyn to predict mean arterial pressure (MAP) changes after fluid expansion. This meta-analysis sought to assess the predictive performance of Eadyn for the MAP response to fluid expansion in mechanically ventilated hypotensive patients.

Methods
We systematically searched electronic databases through November 28, 2020, to retrieve studies that evaluated the association between Eadyn and fluid expansion-induced MAP increases in mechanically ventilated hypotensive adults. Given the diverse threshold value of Eadyn among the studies, we only reported the area under the hierarchical summary receiver operating characteristic curve (AUHSROC) as the primary measure of diagnostic accuracy.

Results
Eight observational studies that included 323 patients with 361 fluid expansions met the eligibility criteria. The results showed that Eadyn was a good predictor of MAP increases in response to fluid expansion, with an AUHSROC of 0.92 [95% confidence interval (CI) 0.89 to 0.94]. Six studies reported the cut-off value of Eadyn, which ranged from 0.65 to 0.89. The cut-off value of Eadyn was nearly conically symmetrical, most data were centred between 0.7 and 0.8, and the mean and median values were 0.77 and 0.75, respectively. The subgroup analyses indicated that the AUHSROC was slightly higher in the intensive care unit (ICU) patients (0.96; 95% CI 0.94 to 0.98) but lower in the surgical patients in the operating room (0.72; 95% CI 0.67 to 0.75). The results indicated that the fluid type and measurement technique might not affect the diagnostic accuracy of Eadyn. Moreover, the AUHSROC for the sensitivity analysis of prospective studies was comparable to that in the primary analysis.

Conclusions
Eadyn exhibits good performance for predicting MAP increases in response to fluid expansion in mechanically ventilated hypotensive adults, especially in the ICU setting.
N2O emission and its influencing factors in subtropical streams, China
Authors: Zhao Binjie , Zhang Quanfa 
Published: August 2021

Abstract
Background
Rivers and streams are one of the primary sources of nitrous oxide (N2O) which is an important greenhouse gas with great global warming potential. Yet, over the past century, human activities have dramatically increased reactive nitrogen loadings into and consequently led to increased N2O emission from the river ecosystems. Here, we carried out a study in two subtropical rivers, i.e., Jinshui River and Qi River with slight and intense human disturbance in their respective catchments in China. The study intended to explore spatial variability and seasonality in N2O emissions, and the relative importance of physicochemical variables, nitrification and denitrification potentials, and functional genes abundance influencing N2O emissions.

Results
N2O concentration, N2O saturation, and N2O flux of Jinshui River peaked in high flow season. N2O concentration, N2O saturations, and N2O flux in Qi River and downstream of Jinshui River were significantly higher than that in other areas in normal and low flow seasons. N2O concentration was positively correlated with water temperature, water NO3−, and DOC, negatively correlated with water NH4+ and DOC/NO3− (the ratio of dissolved organic carbon to NO3− in water), and positively correlated with potential nitrification rate in high flow season, but not correlated with functional genes abundance. Both rivers had lower N2O saturation and flux than many freshwater systems, and their EFr-5 (N2O emission factor for river) was lower than the recommended values of IPCC.

Conclusions
While the two rivers were moderate sources of N2O and N2O emissions in river systems were normally elevated in the summer, areas with intense human disturbance had higher N2O concentration, N2O saturations, and N2O flux than those with slight human disturbance. Physicochemical variables were good indicators of N2O emissions in the river ecosystems.
Rural households’ livelihood vulnerability to climate variability and extremes: a livelihood zone-based approach in the Northeastern Highlands of Ethiopia
Authors: Mekonen Abebe Arega , Berlie Arega Bazezew 
Published: August 2021

AbstractBackgroundThe adverse effects of climate variability and extremes exert increasing pressure on rural farm households whose livelihoods are dependent on nature. However, integrated and area-specific vulnerability assessments in Ethiopia in general and the study area, in particular, are scarce and insufficient for policy implications. Therefore, this study aims to quantify, map, classify, and prioritize the level of vulnerability in terms of the components of exposure, sensitivity, and adaptive capacity in the Northeastern Highlands of Ethiopia. The study area is divided into six livelihood zones, namely, Abay-Beshilo Basin (ABB), South Wollo and Oromia eastern lowland sorghum and cattle (SWS), Chefa Valley (CHV),Meher-Belg,Belg, andMeher.A total of 361 sample households were selected using proportional probability sampling techniques. Survey questionnaire, key informant interview, and focus group discussions were used to collect the necessary data. Rainfall and temperature data were also used. Following the IPCC’s climate change vulnerability assessment approach, the climate vulnerability index (CVI) framework of Sullivan and Meigh’s model was used to assess the relative vulnerability of livelihoods of rural households. Twenty-four vulnerability indicators were identified for exposure, sensitivity, and adaptive capacity components. In this regard, Iyengar and Sudarshan’s unequal weighting system was applied to assign a weight to indicators.ResultsThe results revealed thatBelgandMeherwere found to be the highest exposure livelihood zones to vulnerability with an aggregated value of 0.71. Equally, SWS, ABB,Belg, and CHV livelihood zones showed moderate level of sensitivity to vulnerability with an aggregated value between 0.45 and 0.60. The study noted that livelihood zone ofBelg(0.75) was found to be at high level of livelihood vulnerability. ABB (0.57) and CHV (0.45) were at a moderate level of livelihood vulnerability whileMeher-Belg(0.22) was the least vulnerable livelihood zone due to a high level of adaptive capacity such as infrastructure, asset accumulation, and social networks.ConclusionIt was identified that disparities of livelihood vulnerability levels of rural households were detected across the study livelihood zones due to differences in the interaction of exposure, sensitivity, and adaptive capacity components. The highest levels of exposure and sensitivity combined with low level of adaptive capacity have increased households’ livelihood vulnerability. More importantly, the biophysical and socioeconomic sensitivity to livelihood vulnerability were exacerbated by slope/topography, soil erodibility, and population pressure. Therefore, designing livelihood zone-based identifiable adaptation strategies are essential to reduce the exposure and sensitivity of crop-livestock mixed agricultural systems to climate calamity.
A case report of brain abscess caused by Nocardia farcinica
Authors: Song Jiangqin , Dong Lian , Ding Yan , Zhou Junyang 
Published: August 2021

Abstract
Background
Brain abscess due to the Nocardia genus is rarely reported and it is usually found in immunocompromised patients. Treatment of Nocardia brain abscess is troublesome and requires consideration of the severity of the underlying systemic disease. The difficulties in identifying the bacterium and the frequent delay in initiating adequate therapy often influence the prognosis of patients.

Case presentation
Here, we report a rare case of brain abscess caused by Nocardia farcinica. The patient’s medical history was complicated: he was hospitalized several times, but no pathogens were found. At last, bacteria were found in the culture of brain abscess puncture fluid; the colony was identified as Nocardia farcinica by mass spectrometry. Targeted antibiotic treatment was implemented, brain abscess tended to alleviate, but the patient eventually developed fungal pneumonia and died of acute respiratory distress syndrome (ARDS).

Conclusion
Brain abscess caused by Nocardia farcinica can appear in non-immunocompromised individuals. Early diagnosis, reasonable surgical intervention, and targeted antibiotic treatment are critical for Nocardia brain abscess treatment. In the treatment of Nocardia brain abscess, attention should paid be to the changes in patients’ immunity and infection with other pathogens, especially fungi, avoided.