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71.
Kyoko Nagao Thierry Morlet Elizabeth Haley Jennifer Padilla Julianne Nemith Robert W. Mason Shunji Tomatsu 《Molecular genetics and metabolism》2018,123(4):472-478
Background
Hearing impairment is a common problem in patients with mucopolysaccharidosis IV (MPS IV) throughout their life. Many of the adult patients with MPS IV exhibit permanent or severe hearing loss. However, there has been no systematic review of detailed audiological test results in MPS IV.Materials and methods
Fourteen individuals with MPS IV (13 MPS IVA and 1 MPS IVB; aged between 12 and 38?years old) participated in the current study. We obtained auditory neurophysiological responses (auditory brainstem responses and otoacoustic emissions test) in addition to pure-tone audiometry and middle ear function tests (tympanometry and acoustic reflexes).Results
The results indicated various levels and types of hearing loss with abnormal neurophysiological responses even in those patients with MPS IVA with normal pure tone thresholds. We also found a strong relationship between height (short stature is an indicator of skeletal severity) and hearing sensitivity as well as a strong relationship between height and outer hair cell function in the inner ear (measured by otoacoustic emissions) among MPS IVA patients.Conclusion
The strong correlation between reduced height and hearing loss indicates that patients with severe skeletal dysplasia may be at higher risk of developing more severe hearing loss. More importantly, the spectrum of hearing disorders indicates that MPS IV patients should have annual neurophysiological hearing tests in addition to audiometric testing from an early age regardless of their skeletal severity to more carefully monitor disease progression. 相似文献72.
Jeanna Wallenta Law Debanjali Mitra Henry G. Kaplan Tamuno Alfred Adam M. Brufsky Birol Emir Haley McCracken Xianchen Liu Ronda G. Broome Chenan Zhang Caroline DiCristo Connie Chen 《Current oncology (Toronto, Ont.)》2022,29(2):1047
This retrospective single-arm study assessed real-world treatment patterns and clinical outcomes in patients with hormone receptor—positive/human epidermal growth factor receptor 2—negative (HR+/HER2−) advanced/metastatic breast cancer (A/MBC) who received palbociclib plus an aromatase inhibitor as first-line therapy in US community health systems. Using electronic health records from the Syapse Learning Health Network, 242 patients were identified as having received first-line palbociclib plus an aromatase inhibitor between 3 February 2015, and 31 July 2019 (data cutoff 1 February 2020) resulting in a minimum potential 6-month follow-up period. In total, 56.6% of patients had de novo A/MBC at initial breast cancer diagnosis, 50.8% had bone-only disease, and 32.2% had visceral disease. Median follow-up was 22.4 months. Disease progression (26.4%) and intolerance/toxicity (14.9%) were the main reasons for treatment discontinuation. The median (95% CI) real-world progression-free survival was 31.7 (27.9—not estimable (NE)) months and 2-year estimated overall survival (OS) rate was 78.0%. In total, 25.6% of patients died; however, OS data are limited by the small population size and insufficient follow-up time. These real-world effectiveness outcomes complement findings from other real-world studies and randomized controlled trials and support palbociclib plus an aromatase inhibitor as first-line therapy for HR+/HER2− A/MBC. 相似文献
73.
Craig Barnes Binam Bajracharya Matthew Cannalte Zakir Gowani Will Haley Taha Kass-Hout Kyle Hernandez Michael Ingram Hara Prasad Juvvala Gina Kuffel Plamen Martinov J Montgomery Maxwell John McCann Ankit Malhotra Noah Metoki-Shlubsky Chris Meyer Andre Paredes Jawad Qureshi Xenia Ritter Philip Schumm Mingfei Shao Urvi Sheth Trevar Simmons Alexander VanTol Zhenyu Zhang Robert L Grossman 《J Am Med Inform Assoc》2022,29(4):619
ObjectiveThe objective was to develop and operate a cloud-based federated system for managing, analyzing, and sharing patient data for research purposes, while allowing each resource sharing patient data to operate their component based upon their own governance rules. The federated system is called the Biomedical Research Hub (BRH).Materials and MethodsThe BRH is a cloud-based federated system built over a core set of software services called framework services. BRH framework services include authentication and authorization, services for generating and assessing findable, accessible, interoperable, and reusable (FAIR) data, and services for importing and exporting bulk clinical data. The BRH includes data resources providing data operated by different entities and workspaces that can access and analyze data from one or more of the data resources in the BRH.ResultsThe BRH contains multiple data commons that in aggregate provide access to over 6 PB of research data from over 400 000 research participants.Discussion and conclusionWith the growing acceptance of using public cloud computing platforms for biomedical research, and the growing use of opaque persistent digital identifiers for datasets, data objects, and other entities, there is now a foundation for systems that federate data from multiple independently operated data resources that expose FAIR application programming interfaces, each using a separate data model. Applications can be built that access data from one or more of the data resources. 相似文献
74.
Alex Buga Gary L. Welton Katie E. Scott Adam D. Atwell Sarah J. Haley Noah J. Esbenshade Jacqueline Abraham Jeffrey D. Buxton Dana L. Ault Amy S. Raabe Timothy D. Noakes Parker N. Hyde Jeff S. Volek Philip J. Prins 《Nutrients》2022,14(6)
A growing number of endurance athletes have considered switching from a traditional high-carbohydrate/low-fat (HCLF) to a low-carbohydrate/high-fat (LCHF) eating pattern for health and performance reasons. However, few studies have examined how LCHF diets affect blood lipid profiles in highly-trained runners. In a randomized and counterbalanced, cross-over design, athletes (n = 7 men; VO2max: 61.9 ± 6.1 mL/kg/min) completed six weeks of two, ad libitum, LCHF (6/69/25% en carbohydrate/fat/protein) and HCLF (57/28/15% en carbohydrate/fat/protein) diets, separated by a two-week washout. Plasma was collected on days 4, 14, 28, and 42 during each condition and analyzed for: triglycerides (TG), LDL-C, HDL-C, total cholesterol (TC), VLDL, fasting glucose, and glycated hemoglobin (HbA1c). Capillary blood beta-hydroxybutyrate (BHB) was monitored during LCHF as a measure of ketosis. LCHF lowered plasma TG, VLDL, and TG/HDL-C (all p < 0.01). LCHF increased plasma TC, LDL-C, HDL-C, and TC/HDL-C (all p < 0.05). Plasma glucose and HbA1c were unaffected. Capillary BHB was modestly elevated throughout the LCHF condition (0.5 ± 0.05 mmol/L). Healthy, well-trained, normocholesterolemic runners consuming a LCHF diet demonstrated elevated circulating LDL-C and HDL-C concentrations, while concomitantly decreasing TG, VLDL, and TG/HDL-C ratio. The underlying mechanisms and implications of these adaptive responses in cholesterol should be explored. 相似文献
75.
Carol Dawson-Rose PhD Deena Shehadeh MSN RN-BC PHN Jennifer Hao BA Jasmine Barnard BA Ladan Khoddam-Khorasani MPH Adam Leonard MS MPH CPNP AAHIVS Kristen Clark MSN RN Eva Kersey BA Haley Mousseau MA Julie Frank LCSW MSW Aaron Miller PMHNP Adam Carrico PhD Amy Schustack EdD Yvette P. Cuca PhD 《Public health nursing (Boston, Mass.)》2020,37(3):363-370
76.
Haley J. Appaneal Aisling R. Caffrey Lan Jiang David Dosa Leonard A. Mermel Kerry L. LaPlante 《Diagnostic microbiology and infectious disease》2018,90(4):311-315
Pseudomonas aeruginosa is a major cause of healthcare-associated infections and resistance among isolates is an increasing burden. The study purpose was to describe national resistance rates for clinical P. aeruginosa respiratory and bloodstream cultures and the prevalence of multidrug-resistant (MDR) P. aeruginosa within the Veterans Affairs (VA). MDR was defined as non-susceptibility to at least one drug in at least 3 of the following 5 categories: carbapenems, extended-spectrum cephalosporins, aminoglycosides, and piperacillin/tazobactam. We reviewed 24,562 P. aeruginosa respiratory and bloodstream isolates across 126 VA facilities between 2009 and 2013. Most isolates were collected from inpatient settings (82%). Resistance was highest in fluoroquinolones (33%) and exceeded 20% for all classes assessed (carbapenems, extended-spectrum cephalosporins, aminoglycosides, and piperacillin/tazobactam). Resistance was higher in inpatient settings and in respiratory isolates. Prevalence of MDR was 20% overall (22% for inpatient isolates, 11% outpatient, 21% respiratory, 17% bloodstream). Our findings are consistent with previous surveillance reports. 相似文献
77.
The authors review widely accepted criteria for program accreditation and compare gerontology with well-established accredited fields including clinical psychology and social work. At present gerontology lacks many necessary elements for credible professional accreditation, including defined scope of practice, applied curriculum, faculty with applied professional credentials, and resources necessary to support professional credentialing review. Accreditation with weak requirements will be dismissed as "vanity" accreditation, and strict requirements will be impossible for many resource-poor programs to achieve, putting unaccredited programs at increased risk for elimination. Accreditation may be appropriate in the future, but it should be limited to professional or applied gerontology, perhaps for programs conferring bachelor's or master's degrees. Options other than accreditation to enhance professional skills and employability of gerontology graduates are discussed. 相似文献
78.
Xia Hong Shan Jiang Nancy Marmolejo Ramya Vangipuram Elmira Ramos‐Rojas Yulin Yuan Zuan‐Tao Lin Yaxi Li Jingyi Qiu Yikun Xing Christopher Haley Stephen K. Tyring Tianfu Wu 《Experimental dermatology》2018,27(9):1053-1057
To discover novel biomarkers of psoriasis, a target‐specific antibody array screening of serum samples from psoriasis patients was initially performed. The results revealed that vascular endothelial growth factor receptor 3 (VEGFR‐3) was significantly elevated in the sera of psoriasis patients, compared to healthy controls. Next, ELISA validation studies in a larger cohort of psoriasis patients (N = 73) were conducted, which confirmed that serum VEGFR‐3 was indeed significantly increased in patients with psoriasis compared to healthy controls (P < 0.001). Furthermore, receiver operating characteristic curve analysis demonstrated that serum VEGFR‐3 exhibited potential in distinguishing healthy controls from psoriasis patients: area under the curve = 0.85, P < 0.001. In addition, serum levels of VEGFR‐3 were correlated with Psoriasis Area Severity Index scores (R = 0.32, P = 0.008) in psoriasis patients. Interestingly, serum VEGFR‐3 levels were significantly elevated in psoriatic arthritis compared to non‐psoriatic arthritis (P = 0.026). A pilot longitudinal study demonstrated that serum levels of VEGFR‐3 could reflect disease progression in psoriasis. Collectively, serum VEGFR‐3 may have a clinical value in monitoring disease activity of psoriasis. 相似文献
79.
Hutchins SS Baughman AL Orr M Haley C Hadler S 《The Journal of infectious diseases》2004,189(Z1):S108-S115
Knowledge of the minimum level of vaccination capable of preventing measles transmission in an age group is helpful for establishing program targets for measles elimination. In 1990, during the measles resurgence in the United States, one-half of cases occurred in children aged <5 years. Although estimated population immunity among persons >or=6 years of age was 93%, immunity was lower and varied widely among preschool-aged children. To examine the association of vaccine coverage at 2 years of age and measles incidence among preschool-aged children, we analyzed ecological studies of measles incidence in Milwaukee (Wisconsin) census tracts, Dallas (Texas) ZIP code areas, and selected cities during the 1989-1991 measles resurgence. In each study area, measles incidence decreased rapidly with increasing measles vaccine coverage and became low or negligible when coverage was >or=80%. Regression analysis also suggested that measles would not be transmitted when vaccine coverage was at least 79%. A minimum vaccine coverage of approximately 80% at the second birthday in census tracts, ZIP code areas, and cities in the United States may be sufficient to prevent measles transmission among preschool-aged children if population immunity is >or=93% among persons >or=6 years of age. 相似文献
80.