bluuebunny/crossref_metadata_2025_split huggingface dataset:
Acute limb ischaemia due to thrombosed persistent sciatic artery aneurysm presents a surgical challenge in a frail octogenarian
Authors: Power Foley Megan , Alawy Mahmoud , Tubassam Muhammad 
Published: June 2022

Objectives Persistent sciatic artery (PSA) is a rare congenital anomaly, whereby the embryonic sciatic artery remains patent with associated degrees of femoral axis hypoplasia. Aneurysmal degeneration and distal ischaemia from thromboembolic complications are common. Revascularisation strategies include embolectomy, bypass or interposition grafting and catheter-directed thrombolysis. Methods We describe a sedentary 88-year-old woman with right acute limb ischaemia secondary to a thrombosed PSA aneurysm and concurrent occlusive thrombus at the femoral bifurcation. Results The patient presented with a 3-day history of a cold, painful right foot. Examination revealed Rutherford IIb ischaemia. CT-angiography demonstrated no continuity between the hypoplastic superficial femoral and popliteal arteries, complete occlusion of the right PSA distal to the thrombosed aneurysm and occlusive thrombus in the right profunda. As she was too frail for femoral-distal bypass, we restored femoral axis inflow via profunda embolectomy. Her prognosis remained guarded as we deliberately did not reconstruct the PSA. However, she was discharged pain-free and mobilising with aids 2 weeks later. Conclusion Limb ischaemia in frail, high-risk patients is an ever-increasing challenge for vascular surgeons and requires complex decision-making, balancing comorbidities against desired outcomes. This case illustrates that a selective approach can be sufficient to maintain function despite complex anatomy.
Assessment of systemic and gastrointestinal tissue damage biomarkers for GVHD risk stratification
Authors: Etra Aaron , Gergoudis Stephanie , Morales George , Spyrou Nikolaos , Shah Jay , Kowalyk Steven , Ayuk Francis , Baez Janna , Chanswangphuwana Chantiya , Chen Yi-Bin , Choe Hannah , DeFilipp Zachariah , Gandhi Isha , Hexner Elizabeth , Hogan William J. , Holler Ernst , Kapoor Urvi , Kitko Carrie L. , Kraus Sabrina , Lin Jung-Yi , Al Malki Monzr , Merli Pietro , Pawarode Attaphol , Pulsipher Michael A. , Qayed Muna , Reshef Ran , Rösler Wolf , Schechter Tal , Van Hyfte Grace , Weber Daniela , Wölfl Matthias , Young Rachel , Özbek Umut , Ferrara James L. M. , Levine John E. 
Published: June 2022

Abstract
We used a rigorous PRoBE (prospective-specimen collection, retrospective-blinded-evaluation) study design to compare the ability of biomarkers of systemic inflammation and biomarkers of gastrointestinal (GI) tissue damage to predict response to corticosteroid treatment, the incidence of clinically severe disease, 6-month nonrelapse mortality (NRM), and overall survival in patients with acute graft-versus-host disease (GVHD). We prospectively collected serum samples of newly diagnosed GVHD patients (n = 730) from 19 centers, divided them into training (n = 352) and validation (n = 378) cohorts, and measured TNFR1, TIM3, IL6, ST2, and REG3α via enzyme-linked immunosorbent assay. Performances of the 4 strongest algorithms from the training cohort (TNFR1 + TIM3, TNFR1 + ST2, TNFR1 + REG3α, and ST2 + REG3α) were evaluated in the validation cohort. The algorithm that included only biomarkers of systemic inflammation (TNFR1 + TIM3) had a significantly smaller area under the curve (AUC; 0.57) than the AUCs of algorithms that contained ≥1 GI damage biomarker (TNFR1 + ST2, 0.70; TNFR1 + REG3α, 0.73; ST2 + REG3α, 0.79; all P &lt; .001). All 4 algorithms were able to predict short-term outcomes such as response to systemic corticosteroids and severe GVHD, but the inclusion of a GI damage biomarker was needed to predict long-term outcomes such as 6-month NRM and survival. The algorithm that included 2 GI damage biomarkers was the most accurate of the 4 algorithms for all endpoints.
Addition of romiplostim to conditioning prior to HSCT allows chemotherapy reduction while maintaining engraftment levels
Authors: Wilk C. Matthias , Kovtonyuk Larisa V. , Manz Markus G. 
Published: June 2022

Abstract
Allogeneic hematopoietic stem cell transplantation (HSCT) offers a curative treatment approach for certain benign and malignant hematologic diseases. The actual HSCT is preceded by a conditioning therapy that reduces host-vs-HSCT graft rejection and creates niche space for transplanted hematopoietic stem and progenitor cells (HSPCs). Conditioning consists of chemotherapy with or without irradiation and is a major cause of side effects in HSCT. However, reduction of the intensity of cytotoxic conditioning leads to higher rates of engraftment failure and increased rates of relapse. We here tested if the addition of an HSC cycling inducing agent during conditioning allows to diminish the dose of conditioning drugs without reducing subsequent transplanted HSC engraftment in a mouse HSCT model. The thrombopoietin receptor agonist romiplostim was shown to induce cell cycling activity in hematopoietic stem cells (HSCs). We thus tested if the addition of romiplostim to the clinically applied conditioning chemotherapy regimen cyclophosphamide and busulfan leads to increased efficacy of the chemotherapeutic regimen. We found that romiplostim not only sensitizes HSCs to chemotherapy but also enables a reduction of the main chemotherapeutic component busulfan by half while HSC engraftment levels are maintained in long-term, serial transplantation assays.
Phase 2 trial of ixazomib, cyclophosphamide, and dexamethasone for previously untreated light chain amyloidosis
Authors: Muchtar Eli , Gertz Morie A. , LaPlant Betsy R. , Buadi Francis K. , Leung Nelson , O’Brien Patrick , Bergsagel P. Leif , Fonder Amie , Hwa Yi Lisa , Hobbs Miriam , Helgeson Dania K. , Bradt Erin E. , Gonsalves Wilson , Lacy Martha Q. , Kapoor Prashant , Siddiqui Mustaqueem , Larsen Jeremy T. , Warsame Rahma , Hayman Suzanne R. , Go Ronald S. , Dingli David , Kourelis Taxiarchis V. , Dispenzieri Angela , Rajkumar S. Vincent , Kumar Shaji K. 
Published: June 2022

Abstract
Bortezomib, a proteasome inhibitor (PI), has shown efficacy in the treatment of newly diagnosed and relapsed light chain (AL) amyloidosis, and is often used in combination with cyclophosphamide and dexamethasone. Ixazomib is the first oral PI to be approved in routine practice but has not yet been evaluated in the upfront treatment setting. Newly diagnosed AL amyloidosis patients with measurable disease and adequate organ function were enrolled. The primary objective was to determine the hematologic response rate of ixazomib in combination with cyclophosphamide and dexamethasone. Treatment was given for 12 cycles, followed by ixazomib maintenance until progression. Thirty-five patients were included; their median age was 67 years, and 69% were male. Major organ involvement included heart (66%) and kidneys (54%). A median of 4 induction cycles (range, 1-12) were administered. The overall hematologic response to induction was 63% and included complete response in 11.4% and very good partial response in 37.1% of patients. One patient was upstaged to complete response during maintenance. The most common reason for going off study was the institution of alternate therapy (61%). With a median follow-up of 29.7 months for the living patients, the 2-year progression-free survival and overall survival were 74% and 78%, respectively. The median time to alternate therapy was 7.5 months. Grade ≥3 hematologic and nonhematologic adverse events occurred in 23% and 49% of patients. Given ixazomib’s favorable toxicity profile, which is an important advantage for the typically frail AL population, further evaluation of ixazomib in other combinations in the upfront setting is warranted. This trial was registered at www.clinicaltrials.gov as #NCT01864018.
Hypercontractile esophagus responsive to potassium-competitive acid blockers: a case report
Authors: Shao Yuyang , Xie Chen , Feng Huang , Yan Donglin , Chen Weichang 
Published: June 2022

Abstract
Background
Hypercontractile esophagus is a rare hypercontractile esophageal motility disorder. The etiology of hypercontractile esophagus is unknown but an association between acid reflux and hypercontractile esophagus has been suggested. We present the first report on the use of potassium-competitive acid blockers in the treatment of hypercontractile esophagus.

Case presentation
A 43-year-old man presented with dysphagia, chest pain and regurgitation for a period of 1 year. Initial workup showed a twisted lumen with abnormal contractions in the distal esophagus during upper gastrointestinal endoscopy and abnormal acid exposure under 24-h esophageal pH monitoring. The use of standard-dose proton pump inhibitors didn’t relieve his symptoms. Subsequent high-resolution esophageal manometry made a diagnosis of hypercontractile esophagus. Treatment with vonoprazan resulted in symptomatic resolution and abnormal contractions were no longer detected on follow-up high-resolution manometry.

Conclusions
Potassium-competitive acid blockers like vonoprazan offer an alternative therapeutic method for patients with hypercontractile esophagus who are refractory to proton pump inhibitor therapy. The use of potassium-competitive acid blockers in hypercontractile esophagus warrants further research and may provide evidence for an acid-related etiology of hypercontractile esophagus.
Pre-diagnostic C-reactive protein concentrations, CRP genetic variation and mortality among individuals with colorectal cancer in Western European populations
Authors: Nimptsch Katharina , Aleksandrova Krasimira , Fedirko Veronika , Jenab Mazda , Gunter Marc J. , Siersema Peter D. , Wu Kana , Katzke Verena , Kaaks Rudolf , Panico Salvatore , Palli Domenico , May Anne M , Sieri Sabina , Bueno-de-Mesquita Bas , Standahl Karina , Sánchez Maria-Jose , Perez-Cornago Aurora , Olsen Anja , Tjønneland Anne , Bonet Catalina Bonet , Dahm Christina C. , Chirlaque María-Dolores , Fiano Valentina , Tumino Rosario , Gurrea Aurelio Barricarte , Boutron-Ruault Marie-Christine , Menegaux Florence , Severi Gianluca , van Guelpen Bethany , Lee Young-Ae , Pischon Tobias 
Published: June 2022

Abstract
Background
The role of elevated pre-diagnostic C-reactive protein (CRP) concentrations on mortality in individuals with colorectal cancer (CRC) remains unclear.

Methods
We investigated the association between pre-diagnostic high-sensitivity CRP concentrations and CRP genetic variation associated with circulating CRP and CRC-specific and all-cause mortality based on data from 1,235 individuals with CRC within the European Prospective Investigation into Cancer and Nutrition cohort using multivariable-adjusted Cox proportional hazards regression.

Results
During a median follow-up of 9.3 years, 455 CRC-specific deaths were recorded, out of 590 deaths from all causes. Pre-diagnostic CRP concentrations were not associated with CRC-specific (hazard ratio, HR highest versus lowest quintile 0.92, 95% confidence interval, CI 0.66, 1.28) or all-cause mortality (HR 0.91, 95% CI 0.68, 1.21). Genetic predisposition to higher CRP (weighted score based on alleles of four CRP SNPs associated with higher circulating CRP) was not significantly associated with CRC-specific mortality (HR per CRP-score unit 0.95, 95% CI 0.86, 1.05) or all-cause mortality (HR 0.98, 95% CI 0.90, 1.07). Among four investigated CRP genetic variants, only SNP rs1205 was significantly associated with CRC-specific (comparing the CT and CC genotypes with TT genotype, HR 0.54, 95% CI 0.35, 0.83 and HR 0.58, 95% CI 0.38, 0.88, respectively) and all-cause mortality (HR 0.58, 95% CI 0.40, 0.85 and 0.64, 95% CI 0.44, 0.92, respectively).

Conclusions
The results of this prospective cohort study do not support a role of pre-diagnostic CRP concentrations on mortality in individuals with CRC. The observed associations with rs1205 deserve further scientific attention.
Long anterior lens zonules with retinal stripes: a case report
Authors: Cao Xiaolu , Qin Jiayin , Li Zhiyong , Jia Peipei , Gao Beibei , Feng Lin , Wang Wei 
Published: June 2022

Abstract
Background
Long anterior lens zonules   (LAZs) is a rare disease that was mostly conducted among African Americans. Through the observation of a Chinese patient, we discoverd that the disease may show different characteristics in Asians.

Case presentation
A patient with vision loss due to a macular hole was found to have several special clinical signs during vitrectomy combined with phacoemulsification and intraocular lens implantation surgery in our hospital, including radially oriented lines on the anterior capsule with pigment, a shallow anterior chamber, slightly high intraocular pressure, and radial retinal stripes in the peripheral retina. Finally, he was diagnosed with long anterior lens zonule syndrome.

Conclusion
Clinicians need to pay more attention to the rare disease LAZs. It is important to tear the appropriate size of the anterior capsule so as to avoid radial capsular tearing and intraocular lens dislocation.
Development and validation of the ND10 to measure neck-related functional disability
Authors: MacDermid Joy C. , Walton David M. 
Published: June 2022

Abstract
Background
Previous neck-specific patient-reported outcome measures (PROMs) have tended to measure both symptoms and disability. This multi-staged study developed and evaluated a neck-specific PROM focusing on functional disability.

Methods
This study integrated findings from systematic reviews on neck-specific outcome measures, patient interviews, qualitative studies on neck disability, and iterative item testing to develop a 10-item measure of neck-related disability (ND10). Content validity was assessed by classifying items using the International Classification of Functioning, Disability and Health (ICF) and perspective linking. Patients (n = 78) with neck pain completed cognitive interviews, exploring items of the Neck Disability Index (NDI) and ND10, and completed structured questions related to literacy and relevance. Test–retest reliability and internal consistency were evaluated using intraclass correlation coefficients, Bland Altman graphs, and Cronbach’s alpha. Concurrent convergent validity was evaluated by comparing the ND10 to the NDI, Single Assessment Numeric Evaluation (SANE), and Disabilities of the Arm, Shoulder and Hand (DASH). Known group validity was determined by comparing ND10 scores from patients, who rated their neck as more or less than 1/2 of “normal” on the SANE, using t-tests.

Results
The ND10 requires respondents to make rational judgements about their neck-related body function and disability. It has high internal consistency (0.94) and re-test reliability (0.87; SEM = 3.2/100; MDC = 7.5); and no re-test bias (mean re-test difference of 0.6). It followed expected correlation patterns, being highly correlated with related multi-item PROMs (r = 0.85–0.91), and moderately correlated to the single-item SANE. More patients agreed that the ND10 was easily readable than did so for the NDI (84% vs 68%; p < 0.05). All the PROMs distinguished the patients who perceived themselves as being abnormal/normal defined by a dichotomized SANE (p < 0.01).

Conclusion
The ND10 is reliable and valid for measuring neck-related functional disability. Longitudinal and cross-cultural translation studies are needed to support future use.
Providing palliative care for residents in LTC facilities: an analysis of routine data of LTC facilities in Lower Saxony, Germany
Authors: Walther Wenke , Müller-Mundt Gabriele , Wiese Birgitt , Schneider Nils , Stiel Stephanie 
Published: June 2022

Abstract
Background
Demographic trends show an increasing number of elderly people and thus a growing need for palliative care (PC). Such care is increasingly being provided by long-term care (LTC) facilities. The present study aimed at exploring PC indicators of residents at LTC facilities belonging to a non-profit provider in Lower Saxony, Germany, in order to identify potential improvements.

Methods
A descriptive cross-sectional study was conducted, drawing on routine nursing chart data. Structural data from 16 participating LTC facilities and the care data of all residents who died in 2019 (N = 471) were collected anonymously between March and May 2020. Based on key literature on quality indicators of PC in LTC facilities in Germany, a structured survey was developed by a multidisciplinary research team. The descriptive, comparative and inferential data analysis was conducted using the SPSS software package.

Results
In total, the complete records of 363 (77%) residents who died in the participating LTC facilities in 2019 were retrieved. The records reflected that 45% of the residents had been hospitalized at least once during the last 6 months of their lives, and 19% had died in hospital. Advance care planning (ACP) consultation was offered to 168 (46%) residents, and 64 (38%) declined this offer. A written advance directive was available for 47% of the residents. A specialized PC team and hospice service volunteers were involved in caring for 6% and 14% of the residents, respectively. Cancer patients received support from external services significantly more frequently (p < .001) than did non-cancer patients. Differences emerged in the distribution of PC indicators between LTC facilities. Facilities that have more PC trained staff offered more ACP, supported by more specialized PC teams and hospice services, and had fewer hospitalizations. In addition, more volunteer hospice services were offered in urban facilities.

Conclusions
Overall, a rather positive picture of PC in participating LTC facilities in Germany emerged, although there were differences in the expression of certain indicators between facilities. ACP consultation, volunteer hospice services, and hospital admissions appeared to be superior in LTC facilities with more trained PC staff. Therefore, PC training for staff should be further promoted.
Components of case management in caring for patients with dementia: a mixed-methods study
Authors: Jerez-Barranco Desirée , Gutiérrez-Rodríguez Laura , Morilla-Herrera Juan Carlos , Cuevas Fernandez-Gallego Magdalena , Rojano-Perez Remedios , Camuñez-Gomez María Dolores , Sanchez-Del Campo José Luis , García-Mayor Silvia 
Published: June 2022

Abstract
Background
Case management has shown improvements in some health outcomes for dementia patients and their families. However, despite its benefits the components of case management in order to provide effective patient and family care remain unknown at present. Thus, the aim of this study is to identify the specific components of case management in caring for patients with dementia and to determine the necessary intensity of its deployment to enhance outcomes for these patients and their caregivers.

Methods
Mixed-methods study with a qualitative phase to characterise forms of service provision, according to the case management components involved, followed by a quantitative phase to analyse the correlations between different patterns of service provision, adverse events in patients and caregiver overload. This study will be based on the variables described in the RANGE.COM register.

Discussion
This research is expected to achieve a reproducible, evaluable set of interventions that can be modelled to optimise case management effectiveness for patients with dementia. Interactions between patients with dementia, their family caregivers and case management healthcare services, the components of these interactions and their association with the conditions of the individuals concerned are issues of great interest in the field of case management, which is constantly evolving.
Identification of novel proteins for lacunar stroke by integrating genome-wide association data and human brain proteomes
Authors: Zhang Chengcheng , Qin Fengqin , Li Xiaojing , Du Xiangdong , Li Tao 
Published: June 2022

AbstractBackgroundPrevious genome-wide association studies (GWAS) have identified numerous risk genes for lacunar stroke, but it is challenging to decipher how they confer risk for the disease. We employed an integrative analytical pipeline to efficiently transform genetic associations to identify novel proteins for lacunar stroke.MethodsWe systematically integrated lacunar stroke genome-wide association study (GWAS) (N=7338) with human brain proteomes (N=376) to perform proteome-wide association studies (PWAS), Mendelian randomization (MR), and Bayesian colocalization. We also used an independent human brain proteomic dataset (N=152) to annotate the new genes.ResultsWe found that the protein abundance of seven genes (ICA1L,CAND2,ALDH2,MADD,MRVI1,CSPG4, andPTPN11) in the brain was associated with lacunar stroke. These seven genes were mainly expressed on the surface of glutamatergic neurons, GABAergic neurons, and astrocytes. Three genes (ICA1L,CAND2,ALDH2) were causal in lacunar stroke (P< 0.05/proteins identified for PWAS; posterior probability of hypothesis 4 ≥ 75 % for Bayesian colocalization), and they were linked with lacunar stroke in confirmatory PWAS and independent MR. We also found thatICA1Lis related to lacunar stroke at the brain transcriptome level.ConclusionsOur present proteomic findings have identifiedICA1L,CAND2, andALDH2as compelling genes that may give key hints for future functional research and possible therapeutic targets for lacunar stroke.
An experimental model to induce digital dermatitis in beef calves
Authors: Thomas Anice D. , Pajor Edmond A. , Caddey Benjamin , Goldhawk Christy , Martins Larissa , Orsel Karin 
Published: June 2022

AbstractBackgroundDigital dermatitis (DD) is a multifactorial infectious disease affecting the skin on feet of cattle causing erosion and inflammation above the heel bulbs. Some cases of DD cause lameness and significantly impact animal welfare and productivity. While DD has emerged as a concern for the beef industry, key information regarding early detection and its impact on cattle behaviour is lacking. The primary objective of this study was to determine if an established DD experimental model for dairy calves could be used to induce DD lesions in beef calves. A secondary objective was to describe changes in behaviour and pain associated with induction of DD lesions. Eight beef calves acquired from a single cow-calf operator were enrolled in the study. Upon enrolment, calves were evaluated and determined to be free of foot lesions. Within the experimental environment, calves were housed in individual pens and assigned to two groups (mock-inoculated and inoculated). Both hind feet of each calf were enrolled. Within calf, inoculation protocol was consistent, and a 28-day experimental protocol was employed. Two days prior to inoculation, both hind feet of each calf were abraded (area above the heel bulbs and below the dewclaws), moistened, and wrapped to facilitate an anaerobic condition. Feet were inoculated with macerated DD lesion material or mock inoculum and remained wrapped until clinical signs of DD or protocol endpoint.ResultsAfter a period of 14 to 18 days post inoculation, three of five inoculated calves developed clinical signs (lameness), and upon close inspection, DD lesions were present on at least one hind foot. Two of five inoculated calves did not develop lesions within 28 days. Zero of three mock-inoculated calves developed DD.Treponemaspp. were detected by quantitative polymerase chain reaction from biopsies of induced lesions. Measurements of behaviour prior to disease induction were numerically different between DD affected and mock-inoculated calves.ConclusionsAn experimental infection model established for dairy cattle was used to successfully induce acute DD lesions in three of five inoculated beef calves. This model can provide a framework to study intervention protocols and to evaluate the impact of DD on behaviour and pain.