bluuebunny/crossref_metadata_2025_split huggingface dataset:
Bilateral Macular Dysplasia in Coffin-Siris Syndrome
Authors: Schumaier Nahrain Putris , Dodaballapur Sneha , Liu Tianyu , Drenser Kimberly , Capone Antonio 
Published: November 2024

Purpose:
To report a case of bilateral macular dysplasia without pigmentary retinopathy in a patient with genetically confirmed Coffin-Siris syndrome (CSS) and a co-existing, heterozygous CNGB1 variant.

Methods:
Case report.

Results:
A 7-year-old boy who failed his vision screening was referred for retinal consultation after his eye care provider identified bilateral macular deposits. Past medical history included developmental delay, atypical facial features, uteropelvic junction obstruction, koilonychia, and corpus callosum dysgenesis. The patient underwent whole exome sequencing, which revealed a pathogenic variant in the ARID1A gene consistent with a diagnosis of Coffin-Siris syndrome. Genetic testing also revealed a heterozygous, missense variant in the CNGB1 gene of unknown significance. Fundus examination revealed fine hypopigmented lesions in the macula with surrounding retinal pigment epithelial changes in both eyes. Spectral-domain ocular coherence tomography revealed abnormalities of the ellipsoid and interdigitation zones. The patient’s best-corrected visual acuity and clinical examination remained stable for a decade since his initial examination, with persistent macular dysplasia and no pigmentary retinopathy.

Conclusions:
It is possible that the ocular findings in this case may expand the phenotypes associated with CSS.
Postoperative rhinosinusitis and microbiological outcomes following endoscopic endonasal approaches: A retrospective analysis of 300 patients
Authors: Huang Yu-Wen , Wang Wei-Hsin , Lan Ming-Ying 
Published: November 2024

Background:
Endoscopic endonasal approaches (EEAs) are an efficient way to manage skull base lesions; however, studies regarding postoperative rhinosinusitis are limited. This study analyzed the incidence of postoperative rhinosinusitis, the associated risk factors, microbial species, and antibiotic usage in patients who underwent EEA.

Methods:
Patients who underwent EEAs at a tertiary referral hospital between July 2015 and May 2019 were retrospectively evaluated. Postoperative rhinosinusitis was defined as the presence of a purulent nasal discharge on postoperative endoscopic examination. The mucopus was aspirated and subjected to bacterial culture analysis. The culture results of various groups were analyzed.

Results:
This study included 300 patients, and the incidence of post-EEA rhinosinusitis was 59.7%. History of endonasal surgery was an independent predictive factor for post-EEA rhinosinusitis (odds ratio [OR]: 2.54 [1.28-5.05], p = 0.007). Lesions in the anterior skull base were associated with prolonged use of antibiotics for rhinosinusitis during postoperative care (OR: 3.91 [1.32-11.52], p = 0.013). Staphylococcus aureus was the most common causative pathogen (52.3%), followed by methicillin-resistant S. aureus (MRSA) (20.7%), Pantoea dispersa (14.4%), and Pseudomonas aeruginosa (52.3%). P. aeruginosa was more significant in the complex reconstruction group than in the control group. While most patients with post-EEA rhinosinusitis experienced a transient clinical course, one female patient underwent endoscopic sinus surgery (ESS) for prolonged rhinosinusitis.

Conclusion:
Rhinosinusitis is common in the postoperative stage of the EEA. As it usually lasts for a short duration and is treatable with proper medication, aggressive nasal care during the postoperative period is required to prevent related nasal morbidities.
Reflectance Confocal Microscopy and Dermoscopic Imaging Features in Extramammary Paget’s Disease: A Retrospective Study
Authors: Wang Wen-Ju , Liao Yan-Sen , Xu Jing-Kai , Zhou Pei-Mei , Feng Yan-Yan , Cui Yong 
Published: November 2024

Objective:
Extramammary Paget’s disease (EMPD) is often misdiagnosed as chronic dermatitis or eczema of the scrotum because of its clinical manifestations, leading to delayed diagnosis and treatment. Reflectance confocal microscopy (RCM) and dermoscopy are widely used in clinical practice, enabling early diagnosis and treatment assessment of skin diseases, particularly which are easily misdiagnosed. This study aimed to summarize the RCM and dermoscopic features of EMPD which may provide evidence for diagnosis and evaluation of treatment effects.

Methods:
A retrospective analysis was conducted on skin lesions from 34 patients diagnosed with EMPD, confirmed through histopathological examination at the dermatology departments of Chengdu Second People’s Hospital and the Japan-China Friendship Hospital between January 2022 and December 2023. The analysis focused on typical changes of lesions, such as blood vessel patterns and background color under dermoscopy, as well as cellular morphology under RCM. Count data were presented as constituent ratios.

Results:
Among the included 34 patients, 28 were male and 6 were female, and 104 skin lesions were identified. The lesions were classified into 3 types: 36 red patches, 41 dark red erosive patches, and 27 brown pigmented patches. Dermoscopic examination of EMPD revealed characteristic features, including milky-white globules (90.4%, 94/104), irregular shiny white streaks (91.3%, 95/104), dotted blood vessels (91.3%, 95/104), and glomerular-like vessels (88.5%, 92/104). RCM demonstrated epidermal structural disorganization (100.0%, 104/104), vacuolated cells in the epidermis (94.2%, 98/104), and Paget cells, either scattered or clustered (88.5%, 92/104). The red macular lesions predominantly contained isolated vacuolated cells in the epidermis, the dark red erosive lesions exhibited Paget cell nests in the basal layer, and the brown pigmented lesions contained numerous dendritic cells. All the patients underwent wide surgical excision followed by RCM and dermoscopic examinations postoperatively.

Conclusion:
This study identified characteristic dermoscopic and RCM features in patients with EMPD, providing a foundation for noninvasive auxiliary diagnosis and guiding postoperative follow-up. Additionally, RCM characteristics of different lesion morphologies can help physicians more accurately assess surgical margins, leading to more precise surgical treatment options.
Oncology Physician Turnover in the United States Based on Medicare Claims Data
Authors: Cornelius Sarah L. , Schaefer Andrew , Tosteson Anna N.A. , O’Malley Alistair James , Wong Sandra L. , Moen Erika L. 
Published: November 2024

Objective:
Physician turnover rates are rising in the United States. The cancer workforce, which relies heavily on clinical teamwork and care coordination, may be more greatly impacted by turnover. In this study, we aimed to characterize oncologists who move to identify targets for recruitment and retention efforts.

Methods:
We identified medical, radiation, and surgical oncologists who treated Medicare beneficiaries diagnosed with breast, colorectal, or lung cancer in 2016–2019. We used multivariable logistic regression to identify physician-level and multivariable multinomial regression to identify region-level characteristics associated with turnover. Measures included demographic, practice, and patient-sharing network characteristics.

Results:
Our cohort included 25,012 medical, radiation, and surgical oncologists, of which, 1448 (5.8%) moved. Women [vs men; odds ratio (OR): 1.46; 95% CI: 1.30–1.64] and surgeons (vs medical oncologists; OR: 1.17; 95% CI; 1.04–1.33) had higher odds of moving. Compared with oncologists with moderate patient-sharing ties, those with many ties had lower odds of moving (OR: 0.55; 95% CI: 0.43–0.70). Patient-sharing networks with low efficiency (vs moderate) were more likely to have a net loss in their oncology workforce (OR: 3.06; 95% CI: 1.12–8.35), whereas those with low specialist vulnerability (vs moderate) were less likely to have a net loss (OR: 0.32; 95% CI: 0.1–0.99).

Conclusions:
This study identified novel patient-sharing network characteristics associated with turnover, providing new insights into how the structural features of patient-sharing networks may be related to the recruitment and retention of oncologists.
Higher Percentage of Virtual Primary Care Associated With Minimal Differences in Achievement of Quality Metrics
Authors: Segal Jodi B. , Yanek Lisa , Jager Leah , Okoli Ebele , Hatef Elham , Dada Maqbool , Frick K. Davina 
Published: November 2024

Objective:
To test the impact of virtual care usage on quality metrics used for performance measurement.

Background:
Virtual care improves access to primary care; however, the quality of care must not be adversely impacted by its use.

Methods:
This is a mixed-design etiologic study using data from patients receiving primary care in a large, regional health system from January 2020 through December 2021. Eligible patients had at least one primary care contact. Eligible physicians had 10 or more patient contacts. The quartile of virtual visits per physician per month is calculated as the percentage of total visits conducted by phone or video (Q1 is the lowest). Six metrics used for value-based reimbursement were chosen for modeling with generalized linear mixed models.

Results:
The data included 200,090 patients of 683 physicians in 42 clinics over 24 months. Virtual care usage peaked in April 2020 at 78% and then stabilized at 18%. The blood pressure metric was met in 66% (95% CI: 63%–69%) of physician months in Q1 and 65% (95% CI: 63%–68%) in Q4 (P = 0.003). The hemoglobin A1c metric was met in 73% (95% CI: 70%–76%) of physician months in Q1 and 72% (95% CI: 69%–75%) in Q4, not a significant difference. Breast cancer screening completion and colon cancer screening completion did not differ across virtual care quartiles. Medicare annual wellness visits were completed in 55% (95% CI: 50%–60%) of Q1 physician months and 54% in each of Q2, Q3, and Q4 (P < 0.0001).

Conclusions:
Some quality metrics were modestly impacted by high virtual primary care usage; the absolute differences in rates were small. This may provide reassurance to physicians and their health systems that telemedicine use may not adversely impact quality metrics.
Elevated Interleukin-6 Levels as a Potential Marker of Neonatal Morbidity in Full-term Infants With Polycythemia: A Prospective Study
Authors: Tamer Reyhan , Ünal Şerife Suna Oğuz , Yozgat Can Yilmaz 
Published: November 2024

Objective:
To research and show that interleukin-6 (IL-6) and c-reactive protein (CRP), which can be used as infection markers, are also higher among newborns with polycythemia. The study took place in the neonatal intensive care unit of Zekai Tahir Burak Maternity Teaching and Research Hospital.

Patients and Methods:
Infants with a gestational age of >37 weeks were included in the study. Infants with chorioamnionitis, perinatal asphyxia, and positive blood culture were excluded from the study. Blood samples were obtained six hours after the delivery from the peripheral vein of the infants for measurements of central hematocrit, blood culture, IL-6, and CRP. Infants with a venous hematocrit value of >65% were grouped as the “polycythemia group,” and the ones with a venous hematocrit value of <65% were designated as the “control group.” Observation of significantly higher levels of CRP and IL-6 among newborns admitted to the neonatal intensive care unit due to different causes (such as respiratory distress, hypoglycemia, and feeding intolerance), but significantly higher IL-6 levels in newborns with polycythemia.

Results:
Thirty-five newborns (18 infants in the polycythemia group and 17 infants in the control group) were enrolled in the study. The IL-6 values for the polycythemia group were higher than the upper normal limits (mean ± 2SD, 37.6 ± 55 vs 12 ± 5 pg/dL, respectively; P = 0.00). The IL-6 values of the polycythemia group were found to be higher than the IL-6 values of the control group, with a mean ± 2SD of 37.6 ± 55 vs 6.3 ± 3.4 pg/dL, respectively; this was significant (P = 0.00). Although the CRP values of the polycythemia group were found to be slightly higher than those of the control group (a mean ± 2SD of 3.06 ± 4.07 vs 1.54 ± 2.21 mg/dL, respectively, P > 0.05), this was not significant.

Conclusions:
This study found a significant and robust statistical correlation between IL-6 and v. Hct values (P = 0.01, rs = 0.641). Contrary to IL-6 levels, however, a meaningful relationship was not found between CRP and v.htc values (P = 0.286; rs = 0.184).
Eltrombopag for Treatment of Thrombocytopenia After Autologous Stem Cell Transplantation in Children: Single Center Experience
Authors: Bayram Nihan , Yaman Yontem , Ozdilli Kursat , Nepesov Serdar , Al Isik Odaman , Elli Murat , Anak Sema 
Published: November 2024

Introduction:
Thrombocytopenia is a common clinical problem in cancer patients undergoing high-dose chemotherapy and autologous hematopoietic stem cell transplantation (HSCT). It can occur as prolonged isolated thrombocytopenia (PIT) or secondary failure of platelet recovery (SFPR) and may cause potentially fatal bleeding. However, data on the treatment of post-transplant thrombocytopenia is still lacking.

Methods:
We reported our practices involving 15 pediatric patients who received eltrombopag (ELT) treatment for PIT and SFPR after autologous HSCT.

Results:
The overall response was 78.5% (11/14), with 1 patient excluded due to noncompliance. The 12 surviving patients' median follow up was 699 days (range: 167 to 2250 d).

Conclusions:
Our study indicates the efficacy and safety of ELT for treating PIT and SFPR after autologous HSCT in pediatric patients. However, more studies are needed to confirm these findings in children.
Preparing PhD Students for Tenure-Track Faculty Positions
Authors: Killela Mary K. , Adynski Harry , Adynski Gillian , Myer Elizabeth Allen , Dictus Cassandra , Morgan Leah , Hmiel Hayden , Williams Jessica 
Published: November 2024

Abstract

Background
With an increasing number of nursing faculty approaching retirement, it is critical to prepare PhD students to take on tenure-track faculty roles. There are a multitude of competing time demands for nursing faculty, and one way that PhD students can prepare for faculty roles is leading team-based science with their peers during their predoctoral program.

Objectives
To describe the process of PhD students leading an independent research project, in addition to their dissertation work, which aimed to incorporate the tripartite mission- scholarship, teaching, and service.

Methods
This paper outlines the components of the PhD-student-led project, as well as provides recommendations for leadership and schools of nursing as well as sponsors interested in funding this type of work.

Results
Students were able to conduct independent research using diverse research approaches, practice mentoring skills, and provide service and helpful information about the nursing workforce to their academic clinical partner. A key component of this relationship building was incorporating a clinical fellowship during the PhD program for students that directly aided the transition from BSN to PhD programs.

Discussion
PhD students learned valuable lessons about time management and collaborating in a team setting with peers. We recommend school of nursing seek funding opportunities for PhD students to conduct independent team science, encourage academic partnerships with affiliated medical centers and public health settings, and facilitate peer mentorship across cohorts.
Nationwide Analysis of Variables Associated With Sarcoid Inpatient Mortality
Authors: Manansala Michael , Castellino Janelle , Arora Shilpa , Manadan Augustine M. 
Published: November 2024

Background
Sarcoidosis is a multisystem autoimmune disease that can result in significant morbidity and mortality. This study aims to identify factors associated with in-hospital death for sarcoid patients on a national level.

Methods
We performed a medical records review study of all adult sarcoid hospitalizations from 2016 to 2020 National Inpatient Sample database. A univariable screen followed by multivariable analysis was completed to identify predictors of in-hospital death among sarcoid patients.

Results
There were 405,650 admissions with a diagnosis of sarcoidosis, 10,210 of whom died. Multivariable analysis showed the following factors were independently associated with a higher odds of in-hospital death: age (odds ratio [OR], 1.03; 95% confidence interval [CI], 1.026–1.034), Charlson Comorbidity Index (OR, 1.09; 95% CI, 1.066–1.116), male sex (OR, 1.21; 95% CI, 1.101–1.331), other race (OR, 1.45; 95% CI, 1.073–1.954), arrhythmia/heart blocks (OR, 1.80; 95% CI, 1.617–1.995), cirrhosis/hepatic failure (OR, 8.26; 95% CI, 6.928–9.844), hemophagocytic lymphohistiocytosis (OR, 11.15; 95% CI, 4.172–29.802), infection (OR, 3.31; 95% CI, 3.007–3.633), interstitial lung disease (OR, 1.31; 95% CI, 1.193–1.438), heart failure/myocarditis (OR, 1.29; 95% CI, 1.157–1.436), neurologic diagnoses (OR, 1.37; 95% CI, 1.241–1.502), and pulmonary hypertension (OR, 1.47; 95% CI, 1.305–1.652).

Conclusions
Our multiyear national analysis showed that 2.5% of hospital admissions with a sarcoid diagnosis ended in death. The following factors were associated with death: age, Charlson Comorbidity Index, male sex, other race, arrhythmia/heart blocks, cirrhosis/hepatic failure, hemophagocytic lymphohistiocytosis, infection, interstitial lung disease, heart failure/myocarditis, neurologic diseases, and pulmonary hypertension. This information can help clinicians by improving awareness of these life-threatening complications because early recognition and intervention may improve inpatient sarcoid outcomes.
Scleroderma Renal Crisis and Musculoskeletal Corticosteroid Injections
Authors: Muruganandam Maheswari , Akpan Eyerusalem B. , McElwee Matthew K. , Emil N. Suzanne , Keller Meredith C. , Vangala Adarsh S. , Dihowm Fatmah , Nunez Sharon E. , Gibb James I. , O'Sullivan Frank X. , Fields Roderick A. , Sibbitt Wilmer L. 
Published: November 2024

Background/Objective
Inflammatory arthritis frequently affects patients with systemic sclerosis (SSc) but musculoskeletal corticosteroid (MSKC) injections are often avoided due to concerns of scleroderma renal crisis (SRC). This study investigated the incidence of SRC following MSKC injections.

Methods
In a 136-SSc cohort, 46 subjects underwent a total of 330 MSKC injections each receiving a significant dosage of triamcinolone acetonide (mean, 95.2 ± 44.2 mg per injection session). Data on blood pressure (BP), serum creatinine and glucose, urine protein, and complications were obtained before and after injection from the patients’ medical records.

Results
MSKC and control subjects were similar in age (MSKC: 58.9 ± 12.1 vs. 55.5 ± 14.9 years), female (MSKC: 97.8% [45/46] vs. 89.9% [81/90]), antinuclear antibody (MSKC: 71.7% [33/46] vs. 81.1% [73/90]), anti-centromere antibody (MSKC: 47.8% [22/46] vs. 37.8% [34/90]), anti-topoisomerase antibody (MSKC: 26.1% [12/46] vs. 26.7% [24/90]), and anti–RNA polymerase III antibody (MSKC: 17.4.1% [8/46] vs. 24.4% [22/90]) (all p > 0.05). Pre- and post-MSKC demonstrated nonsignificant changes in systolic BP (pre: 127 ± 22 vs. post: 127 ± 21 mm Hg, p = 1.0), diastolic BP (pre: 71 ± 13 vs. post: 71 ± 11 mm Hg, p = 1.0), creatinine (pre: 0.78 ± 0.56 vs. post: 0.76 ± 0.20 mg/dL, p = 0.64), glucose (pre: 100 ± 21 vs. post: 99 ± 24 mg/dL, p = 0.67), and urine protein-creatinine ratio (pre: 0.14 ± 0.12 vs. post: 0.12 ± 0.11 mg/mg, p = 0.41). One case of SRC with mortality occurred in the controls and none in the MSKC group. No infections, hematologic abnormalities, or tendon rupture were noted.

Conclusion
MSKC injections in established SSc are generally safe with low incidences of SRC and complications. However, it is still prudent to monitor high-risk individuals and recent-onset SSc post–MSKC injection.
TRPV4 calcium-permeable channel contributes to valve stiffening in aortic stenosis
Authors: Dutta Bidisha , Rahaman Suneha G. , Mukherjee Pritha , Rahaman Shaik O. 
Published: November 2024

AbstractAortic valve stenosis (AVS) is a progressive disease marked by fibrosis, inflammation, calcification, and stiffening of the aortic valve leaflets, leading to disrupted blood flow and left ventricular pressure overload. AVS can result in heart failure and death within 2 to 5 years if left untreated, highlighting its high mortality rate. Understanding the molecular mechanisms of AVS is essential for developing noninvasive treatments. Emerging data suggest that extracellular and intracellular matrix stiffness influences gene expression, inflammation, and cell differentiation. Myofibroblast activation of valvular interstitial cells (VICs) along with excess extracellular matrix (ECM) accumulation and remodeling are primary drivers of AVS progression. Inflammation also plays a critical role, with macrophages accumulating in valve leaflets from AVS patients, promoting inflammation, activating VICs, and synthesizing and remodeling the ECM.Our lab and others have reported that macrophage and fibroblast activities, including migration, inflammatory gene expression, and myofibroblast activation, are sensitive to matrix stiffness, indicating that valve leaflet stiffening may regulate AVS progression via a cellular stiffness sensor. Our published work shows that mechanosensitive Ca2+-permeable transient receptor potential vanilloid 4 (TRPV4) channels regulate fibrosis in other organs and control macrophage and fibroblast activation, suggesting TRPV4 as the potential stiffness sensor in AVS. This implies that fibrosis and tissue stiffening may reinforce each other, creating a vicious cycle in AVS development, with VICs and macrophages playing central roles. Here, we identify the cellular stiffness sensor mediating the link between stiffness and AVS development using human aortic valve tissues, a murine model of aortic valve stenosis, and atomic force microscopy analysis.
Neutrophil extracellular traps promote tumor chemoresistance to anthracyclines
Authors: Lin Cindy , Herlihy Sarah E. , Li Marina , Deng Hui , Kim Rina , Bernabei Luca , Rosenwasser Matthew , Gabrilovich Dmitry I. , Vogl Dan T. , Nefedova Yulia 
Published: November 2024

AbstractThe microenvironment plays an important role in promoting tumor cell chemoresistance, but the mechanisms responsible for this effect are not clear. Here, using models of multiple myeloma (MM) and solid cancers, we demonstrate a novel mechanism mediated by neutrophils, a major cell population in the bone marrow (BM), that protects cancer cells from chemotherapeutics. We show that in response to tumor-derived soluble factors, BM neutrophils release their DNA in the form of neutrophil extracellular traps (NETs). Cell-free DNA derived from NETs is then taken up by tumor cells via endocytosis and localizes to the cytoplasm. We found that both NETs and cell-free DNA taken up by tumor cells can bind anthracyclines, leading to tumor cell resistance to this class of chemotherapeutic agents. Targeting cell-free DNA with Pulmozyme or blocking NET formation with a PAD4 inhibitor abrogates the chemoprotective effect of neutrophils and restores sensitivity of tumor cells to anthracyclines.