bluuebunny/crossref_metadata_2025 huggingface dataset:
Facioscapulohumeral muscular dystrophy—Reproductive counseling, pregnancy, and delivery in a complex multigenetic disease
Authors: Vincenten Sanne C. C. , Van Der Stoep Nienke , Paulussen Aimée D. C. , Mul Karlien , Badrising Umesh A. , Kriek Marjolein , Van Der Heijden Olivier W. H. , Van Engelen Baziel G. M. , Voermans Nicol C. , De Die‐Smulders Christine E. M. , Lassche Saskia
Published: July 2021

Reproductive counseling in facioscapulohumeral muscular dystrophy (FSHD) can be challenging due to the complexity of its underlying genetic mechanisms and due to incomplete penetrance of the disease. Full understanding of the genetic causes and potential inheritance patterns of both distinct FSHD types is essential: FSHD1 is an autosomal dominantly inherited repeat disorder, whereas FSHD2 is a digenic disorder. This has become even more relevant now that prenatal diagnosis and preimplantation genetic diagnosis options are available for FSHD1. Pregnancy and delivery outcomes in FSHD are usually favorable, but clinicians should be aware of the risks. We aim to provide clinicians with case‐based strategies for reproductive counseling in FSHD, as well as recommendations for pregnancy and delivery.
Geriatric nutritional risk index as a prognostic indicator in elderly patients with early colorectal cancer undergoing endoscopic submucosal dissection
Authors: Kato Minoru , Hayashi Yoshito , Fukuda Hiromu , Yamaguchi Shinjiro , Inoue Takuya , Ogiyama Hideharu , Kitamura Shinji , Komori Masato , Yamamoto Katsumi , Yamamoto Masashi , Nagai Kengo , Nakahara Masanori , Egawa Satoshi , Yamada Takuya , Sasakawa Akira , Kizu Takashi , Nishiyama Osamu , Shichijo Satoki , Yoshii Shunsuke , Tsujii Yoshiki , Shinzaki Shinichiro , Iijima Hideki , Takehara Tetsuo
Published: July 2021

Objectives

Data on the long‐term outcomes of endoscopic submucosal dissection (ESD) performed in elderly patients with early colorectal cancer (CRC) are limited. We analyzed the prognosis of elderly CRC patients, not only from the viewpoint of treatment curability but also from the patients' baseline physical condition assessed by several indexes.

Methods

A retrospective analysis of 729 patients aged ≥75 years who underwent ESD for Tis/T1 CRC in 16 institutions was conducted. The patients were classified into three groups based on curability: curative ESD (Group A, n = 582), non‐curative ESD with additional surgery (Group B, n = 60), and non‐curative ESD without additional surgery (Group C, n = 87). Overall survival (OS) was compared among the groups, and factors associated with reduced OS were investigated.

Results

The median follow‐up periods in Groups A, B, and C were 41, 49, and 46 months, respectively (P = 0.62), during which 92 patients died. Two patients (0.3%) in Group A, none (0%) in Group B, and three (3.4%) in Group C died of CRC. Three‐year OS rates in Groups A, B, and C were 93.9%, 96.1%, and 90.1%, respectively, without a significant difference (P = 0.07). Multivariate analysis indicated low (<96.3) geriatric nutritional risk index (GNRI) as the sole independent predictor for reduced OS (hazard ratio 3.37; 95% confidence interval 2.18–5.22; P < 0.0001).

Conclusions

Low GNRI, but not the curability attained by ESD, was independently associated with reduced OS in patients with early CRC aged ≥75 years.
Estimating risk factor progression equations for the UKPDS Outcomes Model 2 (UKPDS 90)
Authors: Leal Jose , Alva Maria , Gregory Vanessa , Hayes Alison , Mihaylova Borislava , Gray Alastair M. , Holman Rury R. , Clarke Philip
Published: July 2021

Objectives

To estimate 13 equations that predict clinically plausible risk factor time paths to inform the United Kingdom Prospective Diabetes Study (UKPDS) Outcomes Model version 2 (UKPDS‐OM2).

Methods

Data from 5102 UKPDS participants from the 20‐year trial, and the 4031 survivors with 10 years further post‐trial follow‐up, were used to derive equations for the time paths of 13 clinical risk factors: HbA1c, systolic blood pressure, LDL‐cholesterol, HDL‐cholesterol, BMI, micro‐ or macro‐albuminuria, creatinine, heart rate, white blood cell count, haemoglobin, estimated glomerular filter rate, atrial fibrillation and peripheral vascular disease (PVD). The incidence of events and death predicted by the UKPDS‐OM2 when informed by the new risk factor equations was compared with the observed cumulative rates up to 25 years.

Results

The new equations were based on 24 years of follow‐up and up to 65,252 person‐years of data. Women were associated with higher values of all continuous risk factors except for haemoglobin. Older age and higher BMI at diagnosis were associated with higher rates of PVD (HR 1.06 and 1.02), atrial fibrillation (HR 1.10 and 1.08) and micro‐ or macro‐albuminuria (HR 1.01 and 1.18). Smoking was associated with higher rates of developing PVD (HR 2.38) and micro‐ and macro‐albuminuria (HR 1.39). The UKPDS‐OM2, informed by the new risk factor equations, predicted event rates for complications and death consistent with those observed.

Conclusions

The new equations allow risk factor time paths beyond observed data, which should improve modelling of long‐term health outcomes for people with type 2 diabetes when using the UKPDS‐OM2 or other models.
Effects of prebiotics on postprandial GLP‐1, GLP‐2 and glucose regulation in patients with type 2 diabetes: A randomised, double‐blind, placebo‐controlled crossover trial
Authors: Birkeland Eline , Gharagozlian Sedegheh , Gulseth Hanne L. , Birkeland Kåre I. , Hartmann Bolette , Holst Jens J. , Holst René , Aas Anne‐Marie
Published: July 2021

Aims

We aimed to investigate the effect of prebiotic inulin‐type fructans (ITF) versus a control supplement on postprandial levels of glucagon‐like peptide‐1 and ‐2 (GLP‐1 and ‐2), glucose and insulin in people with type 2 diabetes.

Methods

Adult men and women with type 2 diabetes were randomised in a double‐blind, placebo‐controlled crossover study. The study participants received 16 g/d ITF and 16 g/d control supplement (maltodextrin) for 6 weeks each in two phases separated by a 4‐week washout. A standardised mixed‐meal test was performed before and after each intake period. The primary end point was changes in the GLP‐1 response, and secondary end points were GLP‐2, glucose and insulin responses. Data were analysed using mixed‐model analysis.

Results

A total of 29 participants were included in the study. Differences between and within the two treatments in estimated area under the curves were not significant. Yet, the predicted means for meal‐induced GLP‐1 response in plasma showed a 4.8% decline after the prebiotic treatment and an 8.6% increase after the control treatment (difference in changes between the treatments, p < 0.001). Fasting or postprandial glucose, insulin or GLP‐2 levels were not changed.

Conclusions

Our findings do not support that ITF improve incretin responses or glucose regulations in this population.

Clinicaltrials.gov (NCT02569684).
Freedom as right
Authors: Rödl Sebastian
Published: July 2021

I want to expound the fundamental thought of Fichte's Grundlage des Naturrechts: a freely acting individual is her relation to every other freely acting individual. Their relation is this: they know each other to be free. This knowledge is practical, that is, it is activity, and it is a relation, that is, they who are so related act one toward the other. This knowledge, this activity, this relation, which the free individual is, is the relation of right.
Recognition and the moral nexus
Authors: Wallace R. Jay
Published: July 2021

A discussion of the account of recognition latent in the relational interpretation of moral requirements. I show how an appealing conception of recognition falls out of this interpretation, which treats moral norms as directed requirements that specify what we owe to others, and that correspond to claims on the part of those at whom they are directed. It is argued that relations of accountability, in which we demand of each other compliance with moral requirements, can be understood to involve demands for interpersonal recognition within the relational interpretation. The paper concludes with a discussion of the value of interpersonal recognition, understood in relational terms, considering the perspectives both of claimholders and of the agents who owe them moral consideration.
Parenting During COVID‐19: A Study of Parents' Experiences Across Gender and Income Levels
Authors: Kerr Margaret L. , Rasmussen Hannah F. , Fanning Kerrie A. , Braaten Sarah M.
Published: July 2021

Objective

This study describes parenting experiences at the beginning of the COVID‐19 pandemic and examines differences across parent gender and family income level.

Background

The COVID‐19 pandemic had unprecedented impacts on families. Many parents faced employment changes, including job loss, reduced pay, and working remotely, while simultaneously experiencing increased childcare responsibilities due to school and childcare closures. Research is needed to document the ongoing impact of these changes on parents and families.

Method

An online convenience sample of parents (N = 1,009) reported on their parenting experiences during the beginning of the COVID‐19 pandemic (April 2020) in an online survey.

Results

Parents reported high levels of depression, anxiety, and parental burnout. Further, many parents reported increased negative emotions, such as anger and worry, while simultaneously feeling closer to their children and offering more comfort and soothing. Differences across gender and income levels are presented.

Conclusion

These results align with other emerging findings of increased impacts to mental health and well‐being for parents and children and document the disproportionate effects on women and low‐income families.

Implications

Implications include needing additional support (e.g., financial, caregiving) for parents and families as we continue to face the impacts and consequences of COVID‐19.
Attachment Style Moderates the Relationship Between Solitude and Marital Satisfaction: The Cross‐Partner Effects
Authors: Zhang Shumin , Li Tianyuan
Published: July 2021

Objective

Considering the interdependence between close partners, the present study examined how the amount of one's alone time was related to both one's own and the partner's relationship satisfaction in married couples. It also tested how attachment style moderated the within‐ and cross‐partner associations between the experience of solitude and marital satisfaction.

Background

Solitude reflects a status of being alone without any active social interactions. It can be related to either positive or negative affects depending on the circumstances. The role that solitude plays in close relationships is understudied in previous research.

Method

Using data from 105 married couples from mainland China, both actor and partner effects and the moderating effect of attachment style were tested using the moderated actor–partner interdependence model (MAPIM).

Results

Two cross‐partner interactions between solitude and attachment style on husbands' marital satisfaction were identified. When wives reported high anxiety, the more husbands spent alone time, the lower was their reported marital satisfaction. When husbands reported high avoidance, the more wives spent time alone, the higher was their husbands reported marital satisfaction. No significant association between solitude and wives' marital satisfaction was found.

Conclusions and Implications

The results reveal the intricate role that solitude plays in marital relationships and highlight the importance to consider cross‐partner effects when studying dyadic interactions.
The geography of migrant workers' income in China: Evidence from a migrants dynamic survey in 2017
Authors: Wu Haijiang , Wu Qichao
Published: July 2021

Studying the spatial pattern and determinants of migrant workers’ income is vital for grasping their mobility tendencies to better administer to this urban floating population and propel urbanization. Using 2017 data from the China Migrants Dynamic Survey, this paper adopts trend surface, standard deviation ellipse, and spatial correlation methods to describe the spatial pattern of migrant workers' income in China. It then employs econometric models to examine the spatial effects and determinants of migrant workers' income. The spatial pattern demonstrates an obvious cluster phenomenon for the migrant workers' income in prefectural cities. Based on this social context, our empirical results further show that migrant workers' income has a spatial dependence effect. Migrant workers' income is positively affected by the migrant workers' individual characteristics (demographics, outflow characteristics, social integration) and negatively affected by the external factors (public social services, economic development environment) of the cities into which they flow. There is salient spatial variation in the determinants of migrant workers' income in prefectural cities, which are divided into multifactorial influence zones, work experience and house price influence zones, and education and family influence zones by cluster analysis, and into eastern and midwestern influence zones according to China's three economic areas.
Global histories of empire and climate in the Anthropocene
Authors: Gattey Emma
Published: July 2021

The scientific consensus on the causes of climate change has galvanised global history in the Anthropocene. Within this expanding subfield, however, many historians have afforded imperialism too little explanatory power. This reticence is partly attributable to the intellectual formation of the discipline itself, which long severed human from natural history. It is also due to the paleo‐biological scale of climate change and the related propagation of ‘species history’ by Dipesh Chakrabarty. Obscuring global asymmetries in responsibility for climate change, this approach has deflected attention from the intersections of imperialism and environmental degradation. This article surveys the historiography and methodological challenges of climate change, Chakrabarty's influence on Anthropocene scholarship, and critical responses by global historians. It also summarises recent global histories which have closely analysed the interconnections between empire and climate change, indicating a tipping point in global environmental historiography. These studies reveal intimate, necessarily longue durée linkages between the industrialisation, fossil‐fuel combustion, and exploitative socio‐political structures underpinning both imperialism and climate change.
Examining the influence of health on employment and work ability of Saudi haemodialysis patients: A mixed‐methods study
Authors: Alquwez Nahed , Ormandy Paula
Published: July 2021

Aims

To explore the employment, work productivity, activity impairment, and sustainability of work of Saudi patients with chronic kidney disease (CKD) on haemodialysis (HD).

Background

Failure to address CKD‐related challenges may cause HD patients to lose their job, create a lack of compassion from co‐workers and receive discrimination hindering progress in their profession.

Design

This is a cross‐sectional explanatory sequential mixed‐methods study.

Methods

A convenience sample of 130 CKD patients was surveyed in the quantitative phase using the ‘Short‐Form Health Survey’ and ‘Work Productivity and Activity Impairment Questionnaire.’ For the qualitative phase, face‐to‐face, semi‐structured interviews took place with 16 CKD patients. Data were collected in 2017.

Results

The respondents reported poor physical and mental health. Both employed and unemployed respondents reported moderate levels of work productivity. Better physical health was related to an increased likelihood of being employed. Physical health was negatively correlated with presenteeism, whereas mental health was negatively associated with activity impairment. Five key themes emerged from the qualitative data: work retention, sustaining employment, suitability of work, losing the work and unemployment.

Conclusions

Physical condition, personal distinctiveness, cultural and traditional aspects, and employment policies and approaches affect the employment status of CKD patients on HD.
The high normal ankle brachial index is associated with left ventricular hypertrophy in hypertension patients among the Han Chinese
Authors: Sun Jin , Wang Shuxia , Li Man , Su Yongkang , Ma Shouyuan , Zhang Yan , Zhang Anhang , Cai Shuang , Cheng Bokai , Bao Qiligeer , Zhu Ping
Published: July 2021

Left ventricular hypertrophy (LVH) is the most common target organs damage in the hypertension patients. Abnormal low (≤0.9) or high (≥1.40) ankle brachial index (ABI) are associated with an increased risk of cardiovascular events. However, the relationships between a high ABI in the normal range (0.9–1.4) and LVH in Han Chinese hypertension are not entirely elucidated. This study included 3953 hypertension patients aged 40–75 years among Han Chinese. Hypertension was defined as systolic blood pressure≥140 mm Hg, diastolic blood pressure≥90 mm Hg, or history of antihypertensive drug use. Left ventricular mass (LVM) was measured by transthoracic echocardiography. LVH was diagnosed by using the criteria of LVM ≥49.2 g/m2.7 for men and 46.7 g/m2.7 for women. Our study suggested that the ABI was higher in patients with LVH than in those without (1.13±0.11, 1.11±0.11, p < 0.001). The prevalence of LVH in patients with the lowest (0.9 < ABI≤1.03), second (1.04≤ABI≤1.11), the third (1.12≤ABI≤1.20), and the highest quartile (1.21≤ABI < 1.40) of ABI was 37.2%, 38.2%, 45.5%, 45.7%, respectively. Logistic regression analysis suggested that the highest and third quartile of ABI were significantly associated with increased LVH risk (multivariate‐adjusted OR of highest group:1.83; third group:1.61). The association of ABI at second quartile with LVH was nonsignificant. Similar results were observed in less than 60 years and without coronary heart disease or diabetes group. Our observations in Chinese patients with hypertension indicated high ABI may be an important risk factor for LVH in hypertension patients among Han Chinese, even in the normal range.
Metformin alleviates oxidative stress‐induced senescence of human lens epithelial cells via AMPK activation and autophagic flux restoration
Authors: Chen Mengmeng , Zhang Chunmei , Zhou Nan , Wang Xu , Su Dongmei , Qi Yanhua
Published: July 2021

Cataracts are the leading cause of blindness worldwide owing to the increasing proportion of elderly individuals in the population. The purpose of this study was to investigate whether metformin could alleviate the occurrence and development of age‐related cataract (ARC) and the underlying mechanism. In the present study, we established a senescence model induced by oxidative stress, which was confirmed by measuring β‐galactosidase activity, qRT‐PCR and Western blotting. In addition, we showed that metformin alleviated the oxidative stress‐induced senescence of HLE‐B3 cells via the activation of AMPK. Next, we provided evidence that oxidative stress impaired autophagic flux and induced lysosomal dysfunction. Subsequently, we found that metformin restored autophagic flux that had been impaired by oxidative stress by activating AMPK. Additionally, we found that metformin suppressed HLE‐B3 cell senescence by improving lysosomal function and inactivating mTOR. Furthermore, the inactivation of AMPK, impairment of autophagic flux and lysosomal dysfunction were observed in the human lens epithelium of ARC. In summary, our data suggest that the activation of AMPK may be a potential strategy for preventing ARC, and metformin may be an emerging candidate to alleviate the formation and development of ARC.
Periodontitis stage and grade are associated with poor oral‐health‐related quality of life: Findings from the Porto Alegre cohort study
Authors: Goergen Joseane , Albandar Jasim M. , Oppermann Rui Vicente , Rösing Cassiano Kuchenbecker , Susin Cristiano , Haas Alex Nogueira
Published: July 2021

Aim

To determine the association between periodontitis stage and grade with oral‐health‐related quality of life (OHRQoL).

Materials and methods

This cohort was derived from the Porto Alegre study. The original sample was representative of more than 3 million inhabitants of a Brazilian urban area. Full‐mouth periodontal examinations at six sites per tooth were performed at baseline and 5 years later. Periodontitis grade was determined by direct evidence of progression of attachment loss over the follow‐up. Stage of periodontitis and OHRQoL, determined by the oral health impact profile version 14 (OHIP‐14), were recorded at the follow‐up examination. Mean ratios (MRs) and 95% confidence intervals (95% CIs) were estimated adjusting for age, sex, smoking, systemic diseases, tooth loss, and baseline periodontitis diagnosis.

Results

Five‐hundred and ninety‐nine individuals were analysed. Individuals with periodontitis grade C + stage II (MR = 1.49; 95% CI = 1.08–2.04) and stages III/IV (MR = 1.83; 95% CI = 1.25–2.66) had significantly higher OHIP scores than those without periodontitis or with periodontitis stage I/grade B. Individuals with periodontitis stages II and III/IV + grade B did not differ from those without periodontitis or with periodontitis stage I/grade B.

Conclusion

Severity and progression rate of periodontitis are associated with poor OHRQoL.
Iron reduces the propagation of pathological α‐synuclein
Authors: Guo Yu‐jie , Ayton Scott , Lei Peng
Published: July 2021

Iron accumulation and α‐synuclein aggregates (e.g., Lewy bodies) have been linked with the pathogenesis of Parkinson's disease (PD), with yet‐to‐be‐determined interaction. Previous studies have indicated that iron binds to α‐synuclein and triggers its aggregation in vitro, and iron is found enriched in Lewy bodies. In the current study, Joppe et al. have found that the propagation of pathological α‐synuclein caused by intrastriatal α‐synuclein preformed fibrils (PFFs) injection was unexpectedly attenuated in rodent brains in a model of brain iron elevation (neonatal iron feeding). PFFs stimulated microglial activation was also reduced in mice with elevated iron. These results may provide new insight into the complex interaction between these two key pathologies of PD.

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NOTCH3 variant position is associated with NOTCH3 aggregation load in CADASIL vasculature
Authors: Gravesteijn Gido , Hack Remco J. , Mulder Aat A. , Cerfontaine Minne N. , van Doorn Remco , Hegeman Ingrid M. , Jost Carolina R. , Rutten Julie W. , Lesnik Oberstein Saskia A. J.
Published: July 2021

Aims

CADASIL, the most prevalent hereditary cerebral small vessel disease, is caused by cysteine‐altering NOTCH3 variants (NOTCH3cys) leading to vascular NOTCH3 protein aggregation. It has recently been shown that variants located in one of NOTCH3 protein epidermal growth‐factor like repeat (EGFr) domains 1–6, are associated with a more severe phenotype than variants located in one of the EGFr domains 7–34. The underlying mechanism for this genotype–phenotype correlation is unknown. The aim of this study was to analyse whether NOTCH3cys variant position is associated with NOTCH3 protein aggregation load.

Methods

We quantified vascular NOTCH3 aggregation in skin biopsies (n = 25) and brain tissue (n = 7) of CADASIL patients with a NOTCH3cys EGFr 1–6 variant or a EGFr 7–34 variant, using NOTCH3 immunohistochemistry (NOTCH3 score) and ultrastructural analysis of granular osmiophilic material (GOM count). Disease severity was assessed by neuroimaging (lacune count and white matter hyperintensity volume) and disability (modified Rankin scale).

Results

Patients with NOTCH3cys EGFr 7–34 variants had lower NOTCH3 scores (P = 1.3·10−5) and lower GOM counts (P = 8.2·10−5) than patients with NOTCH3cys EGFr 1–6 variants in skin vessels. A similar trend was observed in brain vasculature. In the EGFr 7–34 group, NOTCH3 aggregation levels were associated with lacune count (P = 0.03) and white matter hyperintensity volume (P = 0.02), but not with disability.

Conclusions

CADASIL patients with an EGFr 7–34 variant have significantly less vascular NOTCH3 aggregation than patients with an EGFr 1–6 variant. This may be one of the factors underlying the difference in disease severity between NOTCH3cys EGFr 7–34 and EGFr 1–6 variants.
The taxonomic relationship between Elaeagnus moorcroftii and E. angustifolia (Elaeagnaceae) based on morphological similarities and simple‐sequence repeat markers
Authors: Ma Weifang , Wang Panpan , Huang Junhua , Zhang Daoyuan , Yang Weikang , Pan Borong , Shi Wei
Published: July 2021

Elaeagnus L. (Elaeagnaceae) is an important plant genus widely used in erosion control due to its high wind breaking and soil binding capacity, but also possessing medicinal and ornamental values. The phenotypic similarity of fruits and flowers of E. moorcroftii and E. angustifolia has often led researchers to speculate on their close relationship and they have sometimes been considered conspecific. In this study, 16 morphological characters were selected and assigned for cluster analysis which divided the samples (n = 49) into six groups: three of E. moorcroftii and three of E. angustifolia. Simple‐sequence repeat (SSR) markers were used to discern the genetic diversity of the 49 individuals. Genetic diversity was found to be high (He = 288, I = 0.938) and there was a strong correlation between the morphological and SSR data. Thus, when combined, the phenotypic classification and SSR marker based evidence confirmed E. moorcroftii as a distinct species independent from E. angustifolia, a conclusion not in agreement with the treatment in recent floras.
Novelties among East African angraecoids (Orchidaceae, Angraecinae)
Authors: D'haijère Tania , Farminhão João N. M. , Stévart Tariq , Fischer Eberhard
Published: July 2021

Here we describe and illustrate two new species of angraecoid orchids from East Africa, Tridactyle delepierrei and Ypsilopus iversenii, respectively from Albertine Rift (Rwanda and Uganda) and west Usambaras (Tanzania). The first species is closely allied to the three species of Tridactyle sect. Eggelingiae and to Tridactyle nalaensis. The second species is closely related to Y. leedalii from the southern highlands of Tanzania, and the affinities of these two species to other Ypsilopus are discussed. Diagnostic and morphological characteristics of these new species along with identification keys are provided, including those for the species of Tridactyle from East Africa and the Albertine Rift, and for the species of Ypsilopus sect. Ypsilopus. These new species are threatened with extinction based on the categories of the IUCN Red List, with T. delepierrei suggested to be Endangered (EN B1), while Y. iversenii would be Critically Endangered (CR). Our findings highlight the need for further orchidological inventories and taxonomical studies to support evidence‐based conservation and management of the remaining montane forests of East Africa.
Adverse device‐device interaction between pacemaker and subcutaneous implantable cardiac defibrillator
Authors: Abbott Nicholas , Bender Aron , Henrikson Charles , Miller Jared , Nazer Babak , Balaji Seshadri
Published: July 2021

A 26‐year‐old patient with prior surgery for Ebstein's anomaly and a pacemaker (placed for post‐surgical heart block and poor underlying rhythm) underwent SICD was placement. During defibrillation testing, device‐device interaction led to undersensing of ventricular fibrillation with failure to shock. Increasing the pacemaker sensitivity resolved the problem but post shock pacing was unable to capture the heart after both shocks. The patient underwent removal of both the pacemaker and the SICD and placement of a transvenous ICD. Complex device‐device interactions can occur in patients who are pacemaker dependent and undergo placement of a SICD.
Heterogeneous outcomes of liver disease after heart transplantation for a failed Fontan procedure
Authors: Ybarra Aecha M , Khanna Geetika , Turmelle Yumirle P , Stoll Janis , Castleberry Chesney D , Scheel Janet , Ballweg Jean A , Ameduri Rebecca , Kimberling Matthew , Makil Elizabeth , Birnbaum Brian F , Exil Vernat , Canter Charles E , Simpson Kathleen E
Published: July 2021

Background

Fontan‐associated liver disease (FALD) uniformly affects patients with long‐term Fontan physiology. The effect of isolated heart transplant (HT) on the course of FALD post‐HT is not well understood.

Methods

We evaluated serial liver imaging pre‐ and post‐HT to assess liver changes over time in a single‐center retrospective analysis of Fontan HT recipients who had pre‐ and ≥1‐year post‐HT liver imaging. Available patient demographic and clinical data were reviewed, including available liver biopsy results.

Results

Serial liver imaging was available in 19 patients with a median age at HT of 12 years (range 3–23), the median age from Fontan to HT of 5.7 years (range 0.8–16), and the median time from imaging to follow up of 27 months (range 12–136 months). Pre‐HT liver imaging was classified as follows: normal (n=1), congested (n=9), fibrotic (n=7), and cirrhotic (n=2). The majority of transplanted patients (15/19) had improvement in their post‐HT liver imaging, including 13 patients with initially abnormal imaging pre‐HT having normal liver imaging at follow‐up. One patient had persistent cirrhosis at 26‐month follow‐up, one patient had unchanged fibrosis at 18‐month follow‐up, and one patient progressed from fibrosis pre‐HT to cirrhosis post‐HT at 136 months. No patients had overt isolated liver failure during pre‐ or post‐HT follow‐up. Liver biopsy did not consistently correlate with imaging findings.

Conclusions

Post‐HT liver imaging evaluation in Fontan patients reveals heterogeneous liver outcomes. These results not only provide evidence for the improvement of FALD post‐HT but also show the need for serial liver imaging follow‐up post‐HT.
Ethylene‐activated MdPUB24 mediates ubiquitination of MdBEL7 to promote chlorophyll degradation in apple fruit
Authors: Wei Yun , Jin Juntong , Xu Yaxiu , Liu Weiting , Yang Guangxin , Bu Haidong , Li Tong , Wang Aide
Published: July 2021

Chlorophyll (Chl) degradation is a natural phenomenon that occurs during ripening in many fleshy fruit species, and also during fruit storage. The plant hormone ethylene is a key factor in promoting Chl degradation during fruit storage, but the mechanisms involved in this induction are largely unknown. In this study, an apple (Malus domestica) BEL1‐LIKE HOMEODOMAIN transcription factor 7 (MdBEL7), potentially functioning as a transcriptional repressor of the Chl catabolic genes (CCGs), including MdCLH, MdPPH2 and MdRCCR2, was identified as a partner of the ethylene‐activated U‐box type E3 ubiquitin ligase MdPUB24 in a yeast library screen. Yeast‐two‐hybrid, co‐immunoprecipitation and luciferase complementation imaging assays were then used to verify the interaction between MdBEL7 and MdPUB24. In vitro and in vivo ubiquitination experiments revealed that MdPUB24 functions as an E3 ubiquitin ligase to ubiquitinate MdBEL7, thereby causing its degradation through the 26S proteasome pathway. Transient overexpression of MdPUB24 in apple fruit led to a decrease in MdBEL7 abundance and increased expression of CCG genes, including MdCLH, MdPPH2 and MdRCCR2, as well as greater Chl degradation. Taken together, the data indicated that an ethylene‐activated U‐box type E3 ubiquitin ligase MdPUB24 directly interacts with and ubiquitinates MdBEL7. Consequent degradation of MdBEL7 results in enhanced expression of MdCLH, MdPPH2 and MdRCCR2, and thus Chl degradation during apple fruit storage. Our results reveal that an ethylene‐MdPUB24‐MdBEL7 module regulates Chl degradation by post‐translational modification during apple fruit storage.
Extracorporeal photopheresis chargemasters show haphazard billing practices
Authors: Adkins Brian D. , Kneib Jessica , Plummer W. Dale , Dupont William D. , Booth Garrett S.
Published: July 2021

Background

United States healthcare spending continues to outpace other developed nations although efforts are being made to increase cost‐transparency. Recent legislation requires hospitals to publish a chargemaster, a list of all billable procedure codes together with prices. Chargemaster prices have been shown to be highly variable, if available, and are not typically paid, but contribute to negotiated rates. Extracorporeal photopheresis (ECP) is performed for a limited number of indications and could serve as a marker of this variability. We investigated the availability of chargemaster documentation for ECP procedures and the variability of pricing as assessed by institutional characteristics.

Study design and methods

A list of centers with photopheresis systems was obtained from the device manufacturer and the institutional websites were analyzed for chargemaster list prices. Multivariate linear regressions were performed to compare impact of facility variables on chargemaster pricing.

Results

There are 139 locations in the US which are listed as referral centers for ECP; and chargemaster prices were available in 66.2% of these centers. The range was $571.48–183,452.00, maximum price 321 times greater than minimum, and the median price, after outlier exclusion, was $8989.06 (SD = $4361.72). ECP cost did not correlate with hospital size, facility type, ownership, number of hospitals in the referral region, hospital care intensity index, academic status, or region (p ≥ .05).

Conclusions

Chargemaster costs for ECP procedures are highly variable and nonuniform, and the current data available for patients undergoing these specialized apheresis procedures is insufficient to afford patients the ability to compare prices.
Be good to thy neighbours: A spatial analysis of foreign direct investment and economic growth in sub‐Saharan Africa
Authors: Chih Yao‐Yu , Kishan Ruby P. , Ojede Andrew
Published: July 2021

This paper employs a spatial dependency framework to examine intermediary roles played by trade, institutional quality and natural resource endowment in determining direct and indirect (spillover) effects of inbound foreign direct investment (FDI) on economic growth. We develop a number of spatial mechanisms to assess how these intermediary factors limit and/or enhance the effect of FDI on economic growth. In particular, we test whether different levels of resource rents, severity of civil conflict and openness to international trade are statistically different in attenuating direct and indirect (spillover) effects of inbound FDI on growth. Last, we conduct a test of asymmetry to investigate whether the effect of FDI on growth is statistically different from zero in three democratic (autocratic) regimes, that is, low democratic (high autocratic), moderate democratic–autocratic and high democratic (low autocratic) societies. A number of insightful contributions to the economic growth‐FDI literature can be extracted from our analyses. First, results indicate that inbound FDIs on their own have positive and significant effect on economic growth. However, the positive FDI effect on growth is significantly dampened by the negative intermediary role of civil conflict. Second, we find that, the more opened sub‐Saharan African economies are to international trade, the greater is the impact of FDI on growth. However, trade among African countries as a share of their economies plays insignificant intermediation role on direct and indirect effects of FDI on growth. Third, we find that an increase in resource rents as a share of the economy significantly reduces direct and spillover effects of inbound FDI on economic growth. Last, our analysis indicates that both low democratic (high autocratic) and high democratic (low autocratic) regimes do accelerate the positive impact of FDI on growth. However, results indicate a slow‐down effect of FDI on growth under moderate democratic–autocratic regimes. This particular finding suggests that the intermediary role of democracy in determining the effect of FDI on economic growth is non‐linear. A number of policy implications can be extrapolated from our analysis. Most importantly, trade policies, which boost integration of sub‐Saharan African countries and open up the continent to the rest of the world, are likely to play major intermediation roles in accelerating the positive effect of FDI on growth.
The influence of a fast‐track service on case flow and client satisfaction in a high‐volume veterinary emergency department
Authors: Weltman Joel G. , Prittie Jennifer E.
Published: July 2021

Objective

To assess the benefit of a fast‐track service in the emergency department of a large, high‐volume veterinary hospital.

Design

Prospective, observational, clinical study.

Setting

Emergency department of an urban, tertiary referral veterinary hospital.

Animals

All animals presented to the emergency department between April 1 and April 30 in 2017 and 2018 were eligible for inclusion. Only patients seen on days in 2017 corresponding to those days of 2018 during which the fast‐track service was available were studied.

Measurement and Main Results

Triage case logs were collected and reviewed for April 2017 (prefast‐track) and 2018 (fast‐track). The fast‐track service was launched as a pilot program in April 2018 to provide expedited care to low acuity patients presented to the emergency department. The median number of daily emergency department cases did not differ between 2017 (45, range 26‐64) and 2018 (47, range 38‐64; P = 0.3). The median time from presentation until first discussion with a doctor for low acuity cases was lower in April 2017 (29 min, range 1‐163) than in April 2018 (24 min, range 1‐100; P < 0.001). This reduction in wait time was observed despite a 40% increase in low acuity case presentations in 2018. Wait times for high acuity patients did not differ between study periods. The number of cases that left without being seen was higher in April 2017 compared to April 2018 (77 and 45 cases, respectively P < 0.001).

Conclusions

Implementation of a fast‐track service reduced wait time for low acuity cases without adversely impacting wait times for sicker patients and led to a reduction in clients leaving without being seen. By introducing the fast‐track service in a large volume veterinary hospital, limited resources can be distributed to improve speed of care, case flow, and client satisfaction in the emergency department.
New leak elements for helium based on single-layer graphene composite membranes
Authors: Liu Zhaoxian , Meng Donghui , Ren Guohua , Zhang Xiao , Han Yan , Wang Lina , Sun Wei , Sun Lichen , Yan Rongxin
Published: July 2021

Graphene containing intrinsic pores with molecular dimensions is a highly promising material for standard leak elements because of its minimal and stable gas flow. However, the etching of polymethyl methacrylate (PMMA) during the typical graphene transfer process can cause the rupture of graphene on the porous substrate due to the surface tension as the etchant solution dries out. In this article, we simplified the typical graphene transfer process that enabled the transfer of a PMMA/graphene composite membrane onto a macroporous Cu gasket as the leak element and tested the leak rate, time stability, and response time of the composite membrane. The membrane permeation area depends on the pore size of the Cu substrate, which can be controlled by laser ablation or computer numerical control milling. To ensure the accuracy of time stability, the entire test lasted 60 days. The conductance results for two devices with a permeation size of 50 and 500 μm were about 10−17 and 10−14 m3 s−1, and the maximum variation of conductance in 60 days was 14% and 2.6%, respectively. Accordingly, the permeance of the composite membrane for helium can be calculated as 4.17 × 10−12–1.09 × 10−11 Pa m3 (cm2 s Pa)−1. Moreover, the composite membrane has been proven to have a rapid response of about 2 s to the upstream pressure.
Underwater noise from submarine turbidity currents
Authors: Hay Alex E. , Hatcher M. G. , Hughes Clarke J. E.
Published: July 2021

Noise from turbidity currents flowing down the face of a fjord delta was measured underwater at frequencies from 1 to 1200 kHz. The noise spectra are consistent with sound generation by collisions among sand-sized particles. The spectra from the leading head extend to higher frequencies than those from the trailing body, indicating that collisions were between finer-grained particles in the head and coarser-grained particles in the body. Noise intensity increased 100-fold for a two-fold increase in head speed, consistent with the expected collision rate for granular materials in the high-flow gas-like phase and highly turbulent particle-laden flows.
Intrasaccadic motion streaks jump-start gaze correction
Authors: Schweitzer Richard , Rolfs Martin
Published: July 2021

Rapid eye movements entail retinal motion streaks that provide spatiotemporal continuity of objects and guide gaze correction.
A malaria parasite phospholipid flippase safeguards midgut traversal of ookinetes for mosquito transmission
Authors: Yang Zhenke , Shi Yang , Cui Huiting , Yang Shuzhen , Gao Han , Yuan Jing
Published: July 2021

A malaria parasite plasma membrane phospholipid flippase protects ookinetes in mosquito midgut epithelium for transmission.
Fxr signaling and microbial metabolism of bile salts in the zebrafish intestine
Authors: Wen Jia , Mercado Gilberto Padilla , Volland Alyssa , Doden Heidi L. , Lickwar Colin R. , Crooks Taylor , Kakiyama Genta , Kelly Cecelia , Cocchiaro Jordan L. , Ridlon Jason M. , Rawls John F.
Published: July 2021

Genetic analysis in zebrafish reveals conserved and novel aspects of nuclear receptor Fxr function and bile salt signaling.
Dichotomous dopaminergic and noradrenergic neural states mediate distinct aspects of exploitative behavioral states
Authors: Koralek Aaron C. , Costa Rui M.
Published: July 2021

Sustained dopaminergic and noradrenergic dynamics independently modulate action drive and diversity during exploitative behavior.
New mechanism and exact theory of superconductivity from strong repulsive interaction
Authors: Crépel Valentin , Fu Liang
Published: July 2021

A mechanism for pairing in purely repulsive Fermi systems is developed, leading to unconventional superconducting behaviors.
X-ray videocystometry for high-speed monitoring of urinary tract function in mice
Authors: Franken Jan , De Bruyn Helene , Rietjens Roma , Segal Andrei , De Ridder Dirk , Everaerts Wouter , Voets Thomas , Vande Velde Greetje
Published: July 2021

Videocystometry, combining x-ray and cystometry, provides unprecedented details on urinary tract function in rodents.
A variable-gain stochastic pooling motif mediates information transfer from receptor assemblies into NF-κB
Authors: Cruz J. Agustin , Mokashi Chaitanya S. , Kowalczyk Gabriel J. , Guo Yue , Zhang Qiuhong , Gupta Sanjana , Schipper David L. , Smeal Steven W. , Lee Robin E. C.
Published: July 2021

A variable-gain stochastic pooling motif mediates robust and parsimonious information transmission from the extracellular milieu.
Subsetting the subsets: Heterogeneity and developmental relationships of T cells in human tumors
Authors: Alanio Cécile , Wherry E. John
Published: July 2021

High-dimensional profiling approaches inform developmental relationships between tumor-infiltrating lymphocyte
subpopulations.
Multiple melt source origin of the Line Islands (Pacific Ocean)
Authors: Pockalny Robert , Barth Ginger , Eakins Barry , Kelley Katherine A. , Wertman Christina
Published: July 2021

The Line Islands volcanic chain in the central Pacific Ocean exhibits many characteristics of a hotspot-generated seamount chain; however, the lack of a predictable age progression has stymied previous models for the origin of this feature. We combined plate-tectonic reconstructions with seamount age dates and available geochemistry to develop a new model that involves multiple melt regions and multiple melt delivery styles to explain the spatial and temporal history of the Line Islands system. Our model identifies a new melt source region (Larson melt region at ~17°S, ~125°W) that contributed to the formation of the Line Islands, as well as the Mid-Pacific Mountains and possibly the Pukapuka Ridge.
Familial giant cell arteritis
Authors: Hayreh Sohan Singh
Published: July 2021

This is a case report of ‘familial giant cell arteritis’ in three siblings from northwest India. This is the first case report of ‘familial giant cell arteritis’ in a non-Caucasian family.
Abdominal actinomycosis after reparative surgery for cholecystogastric fistula in an immunosuppressed patient
Authors: Fra Maria Carolina , Brenna Massimo , Di Benedetto Caroline , De Monti Marco
Published: July 2021

A male patient, aged over 75 years, was referred to the emergency room for pain in his right hypochondrium. This pain, which persisted for approximately 3 days, radiated to his right flank and right iliac quadrant and worsened after meals. The patient reported no nausea, vomiting, diarrhoea, fever or recent traumatic events. Blood laboratory tests, ultrasonography, an MRI and a CT scan were performed, leading to the diagnosis of a mass in the Morrison’s pouch with ultrasound features of adipose tissue compatible with a liposarcoma, infiltrating the liver and the right kidney. Subsequent CT-guided needle aspiration and a histological examination led to the diagnosis of actinomycosis. The patient was initially treated with a daily dose of 18 million IU of benzylpenicillin for 4 weeks and subsequently prescribed the oral administration of 3 g/day of amoxicillin for 11 months. A monitoring CT scan was performed after 1, 5, 8 and 12 months, and a CT scan re-evaluation confirmed that the mass had completely healed.
E-cigarette or vaping product use-associated lung injury (EVALI) masquerading as COVID-19
Authors: Ganne Nandita , Palraj Raj , Husted Elizabeth , Shah Ishan
Published: July 2021

SARS-CoV-2, the novel coronavirus causing COVID-19, has caused a global pandemic resulting in over 4 million deaths globally (data current as of 14 July 2021). E-cigarette or vaping product use-associated lung injury (EVALI) is a type of acute lung injury of unclear pathogenesis. The two pathologies present with overlapping clinical symptoms, laboratory values and imaging, making them difficult to distinguish, especially in the setting of a global COVID-19 pandemic. We present the case of a 32-year-old woman treated for COVID-19 despite multiple negative SARS CoV-2 PCR tests and nucleocapsid antibody test. On further investigation, she endorsed use of E-cigarettes and was subsequently diagnosed with EVALI. The patient was treated with oral and intravenous steroids, resulting in significant improvement in her symptoms. This case highlights the challenge of diagnosing rarer aetiologies of respiratory distress during the COVID-19 pandemic.
Slipped capital femoral epiphysis in a healed Perthes hip
Authors: Singh Kumar Amerendra , Madegowda Arkesh , Shah Hitesh
Published: July 2021

Perthes disease and slipped capital femoral epiphysis (SCFE) in the same child is a rare occurrence. A 7-year-old boy with the left hip Perthes’ disease was managed with femur osteotomy. The femur head healed with a spherical congruent hip. After 3 years, he developed right tibia vara which was treated with growth modulation. The deformity was corrected after one and a half years. At age of 15 years, he presented with left side painful limp and typical features of SCFE. The left hip was managed with modified Dunn’s osteotomy. The hip range of movements was painless and near-normal range at final follow-up. Though this is the first case of ipsilateral SCFE following healed Perthes in a non-syndromic child, one must always consider SCFE in adolescents with hip or knee pain or altered gait, even if they have had other conditions like Perthes disease in the past.