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Clinical Practice Guideline for Evaluation and Management of Peripheral Nervous System Manifestations in Sjögren's Disease

Deboo, Anahita; Fox, Robert; Hammitt, Katherine M; Frantsve-Hawley, Julie; Baker, Matthew C; Danielides, Stamatina; De Sousa, Eduardo; Goodman, Brent P; King, Jennifer K; Mandel, Steven; Noaiseh, Ghaith; Pavlakis, Pantelis P; Sarka, George; Scofield, R Hal; Varadhachary, Arun; Wallace, Daniel J; Makara, Matt; Carteron, Nancy; Carsons, Steven; ,
OBJECTIVES/OBJECTIVE:Sjögren's disease is an autoimmune disorder that can impact multiple organ systems, including the peripheral nervous system (PNS). PNS manifestations, which can exist concurrently, include mononeuropathies, polyneuropathies, and autonomic nervous system neuropathies. To help patients and providers in the decision-making process, we developed an evidence-based clinical practice guideline for the evaluation and management of peripheral nervous system manifestations in patients with Sjögren's disease. METHODS:A Topic Review Group (TRG), comprised of experts in rheumatology, neurology, and guideline methodology, developed Patient, Intervention, Comparison, and Outcome (PICO) questions and conducted a systematic review to identify current best evidence on management of PNS manifestations of Sjögren's disease. PubMed and Embase were searched for evidence published up to July 22, 2025. Literature screening, data extraction, and critical appraisal were performed in duplicate. Six case series, one retrospective cohort, and two prospective cohort studies lacking a comparison group met the inclusion criteria. RESULTS:We developed an aligned nomenclature of PNS terms that can be used across disciplines, 31 good practices for evaluation of suspected PNS manifestations, and 20 evidence-based treatment recommendations, the latter of which were rated as conditional or strong based on Grading of Recommendations Assessment, Development and Evaluation (GRADE) methodology. Due to the scarcity of high-level evidence, this guideline predominantly derives from expert opinion. CONCLUSION/CONCLUSIONS:This clinical practice guideline on PNS manifestations of Sjögren's disease provides clinicians a rigorous, evidence-based resource, developed through an expert consensus-based process, for the assessment, diagnosis, and treatment of peripheral neuropathy in Sjögren's patients. Recommendations were rated as strong when the benefits significantly outweighed potential harms, creating a scenario in which the majority of patients would prefer the advised action.
PMID: 41327784
ISSN: 2151-4658
CID: 5974802

Danazol and cost-savings in immune thrombocytopenia and in immune thrombotic thrombocytopenic purpura

Varma, Mala; Shapira, Ilan; Friedman, Mark; Nakhoul, Ibrahim; Torri, Vamsee; Shulman, Jonah; Kozuch, Peter; Culliney, Bruce; Patel, Amit A; Yoe, Joseph; Shah, Vijay P; Mirzoyev, Tahir; Machuca, Maria
PMID: 41208120
ISSN: 1473-5733
CID: 5966392

Cost-Effectiveness of Expanding Home Cook Interventions for Salt Reduction in China

Li, Xuanjing; Lv, Yipeng; Gao, Xiang; Fan, Bonan; He, Yibei; Zhao, Shan; Liu, Fangchao; Li, Na; Wu, Qi; Li, Weixin; Zhang, Donglan; Marklund, Matti; Pagán, José A; Yan, Lijing L; Moran, Andrew E; Wu, Jing; Li, Yan
INTRODUCTION/BACKGROUND:Excessive salt intake is a major risk factor for cardiovascular disease (CVD) and premature mortality in China and globally. A recent cluster randomized controlled trial demonstrated the effectiveness of home cook interventions in reducing salt intake and blood pressure among participants from six provinces in China. Yet, it remains unclear whether expanding these interventions across China would be cost-effective. METHODS:The China CVD Prevention Model, a validated microsimulation model that captures the development and consequences of CVD among adults 35 years or older in China, was used to estimate lifetime averted CVD events and deaths, direct medical costs (2022 international dollar, Int$), quality-adjusted life years (QALYs) gained, and the incremental cost-effectiveness ratio (ICER) of home cook interventions versus the status quo. Costs and QALYs were discounted at 3%. RESULTS:Compared to the status quo, home cook interventions were projected to avert 1.97 million coronary heart disease (CHD) events, 3.69 million stroke events, 0.77 million deaths due to CHD, and 1.29 million deaths due to stroke in women. The interventions would also avert 1.62 million CHD events, 3.8 million stroke events, 0.6 million deaths due to CHD, and 1.15 million deaths due to stroke in men. The interventions resulted in an ICER of Int$3552/QALY in women and Int$5445/QALY in men and, thus, were cost-effective considering a willingness-to-pay threshold of Int$21,318 (one-time the gross domestic product per capita). CONCLUSIONS:Public health policymakers in China should consider widely adopting home cook interventions to better prevent CVD and reduce health care costs.
PMID: 41338536
ISSN: 1873-2607
CID: 5974992

Rising Exception Requests in the Current Heart Allocation System

Flattery, Erin; Patel, Suhani S; Golob, Stephanie; Massie, Allan B; Phillips, Katherine; Ali, Syed Zain; Singh, Arushi; Wayda, Brian; Rao, Shaline; Leacche, Marzia; Goldberg, Randal; Reyentovich, Alex; Moazami, Nader; Alam, Amit H
BACKGROUND:Despite the goal of the 2018 revision to the heart allocation policy to reduce reliance on exception requests through improved granularity in status criteria, there has been a dramatic rise in exception requests. OBJECTIVES/OBJECTIVE:This study evaluated trends in exception use over the first 6 years of the updated policy, assessing associated clinical factors, temporal changes, and impact on waitlist outcomes. METHODS:This retrospective transplant registry analysis included all adult isolated heart transplant candidates from October 18, 2018, to September 30, 2024. Candidates were stratified by exception use, listing era, and region. Exception use was compared using Wilcoxon rank-sum and chi-squared tests, with multilevel logistic regression assessing independent associations. Trends over time and across UNOS (United Network for Organ Sharing) regions were evaluated, and a competing risks framework examined time to transplant and waitlist mortality. RESULTS:Among 26,330 candidates, 38.6% used exception requests, with a statistically significant increase over time, particularly in higher priority statuses. Exception use was more common among Black, non-Hispanic candidates, and candidates with blood type O, and less likely for patients with blood type A (P < 0.001). Additionally, pretransplant isolated durable left ventricular assist devices were less common in candidates who requested exceptions (19.0% vs 31.6%; P < 0.001). Overall, 39.9% of exception candidates were listed at status 1 or 2 compared to 29% of nonexception candidates, and 69.2% of exception candidates were removed from the waitlist at status 1 or 2 compared with 37% of nonexception candidates. CONCLUSIONS:The rising use of exceptions underscores ongoing limitations in allocation criteria, and disparities suggesting inequities in access to higher listing status. Policy refinements are needed to ensure a balance between medical urgency and equitable allocation.
PMID: 41329111
ISSN: 2213-1787
CID: 5974852

Role of hypertension in the cardiovascular-kidney-metabolic syndrome among black adults: The Jackson Heart Study

Ghazi, Lama; Dubal, Medha; Bertoni, Alain; Carson, April; Young, Bessie A; Lewis, Cora E; Alanaeme, Chibuike J; Johnson, Dayna A; Shimbo, Daichi; Foti, Kathryn; Colantonio, Lisandro D; Arabadjian, Milla; Tanner, Rikki; Muntner, Paul
The cardiovascular-kidney-metabolic (CKM) syndrome consists of four progressive stages and is characterized by the interaction of metabolic risk factors, chronic kidney disease (CKD), and cardiovascular disease (CVD). We assessed the prevalence of hypertension in CKM and its role in progression to more advanced stages. We included 2118 Black adults from the Jackson Heart Study without a history of coronary heart disease, heart failure, stroke or stage 0 CKM (normal weight, no metabolic risk factors or CVD) at baseline. Participants were categorized into CKD stage: Stage 1: overweight/obesity, abdominal obesity or dysfunctional adipose tissue without metabolic risk factors or subclinical CVD; Stage 2: metabolic risk factors (hypertension, diabetes, hypertriglyceridemia, metabolic syndrome or CKD); or Stage 3: subclinical CVD. We used Cox proportional hazards regression to estimate the hazard ratio (HR) of developing stage 4 CKM, defined by a CVD event, in participants with hypertension and stages 2 and 3 CKM. At baseline, 20.2, 69.1 and 10.6% of participants had stage 1, 2 and 3 CKM, respectively. Hypertension was the most common metabolic risk factor in participants with stage 2 and 3 CKM with a prevalence of 80 and 95%, respectively. Incidence rates (95%CI) of stage 4 CKM per 1000 person-years were 1.4 (0.4, 2.4) for stage 1 CKM, 7.5 (6.1, 8.9) for stage 2 CKM with hypertension, and 26.6 (19.8, 33.3) for stage 3 CKM with hypertension. The HRs (95% CI) for developing stage 4 CKM were 3.25 (1.56, 6.80) and 5.11 (2.05,12.78) among participants with hypertension and stage 2 and 3 CKM versus stage 1 CKM, respectively. Hypertension was associated with an increased risk for progression to stage 4 CKM among Black adults.
PMCID:12685740
PMID: 41046247
ISSN: 1476-5527
CID: 5976992

Post-hurricane fluid conservation measures fail to reduce IV fluid use in critically ill children

Dixon, Celeste G; Odum, James D; Kothari, Ulka; Martin, Susan D; Fitzgerald, Julie C; Shah, Ami; Dapul, Heda; Braun, Chloe G; Barbera, Andrew; Terry, Nina; Weiss, Scott L; Hasson, Denise C; Dziorny, Adam C
BACKGROUND:There are risks associated with excessive intravenous fluid (IVF) administration in critically ill children. Previous efforts have described opportunities to reduce positive cumulative fluid balance (CFB) in this population but have not been widely implemented. In the wake of Hurricane Helene, a national IVF shortage led to the implementation of IVF conservation guidelines. We sought to determine if this was associated with a reduction in IVF use and CFB. METHODS:The present study is a four-site cohort study of critically ill children utilizing a federated data collection framework to extract patient age, sex, weight, and daily fluid intake/output for days 1-4 of all admissions 28 days prior to and 28 days after the implementation of IVF conservation guidelines. Guidelines were individualized per institution. Total fluid intake, total IVF intake, % intake from IVF, and % CFB were compared between pre- and post-IVF conservation groups. RESULTS:All sites had similar conservation recommendations. There were 633 patients admitted pre- and 619 patients admitted post-IVF conservation guideline implementation, with similar age and weight distributions. There was no significant difference in IVF use pre- and post-IVF conservation; 29-35% of patients had > 5% CFB on day 1 pre-IVF conservation while 27-39% did post-conservation, with increasing numbers on day 2. CONCLUSIONS:Even in the setting of a national IVF shortage, simple recommendations without structured change were insufficient to change IVF administration practices. This indicates additional practices will be needed to reduce IVF intake and % CFB in this vulnerable population.
PMID: 40828175
ISSN: 1432-198x
CID: 5908922

Application of Generative AI to enhance obstetrics and gynecology research

Kawakita, Tetsuya; Wong, Meilssa S; Gibson, Kelly S; Gupta, Megha; Gimovsky, Alexis; Moussa, Hind N; Heo, Hye
The rapid evolution of large-language models such as ChatGPT, Claude, and Gemini is reshaping the methodological landscape of obstetrics and gynecology (OBGYN) research. This narrative review provides a comprehensive account of generative AI capabilities, key use-cases, and recommended safeguards for investigators. First, generative AI expedites hypothesis generation, enabling researchers to interrogate vast corpora and surface plausible, overlooked questions. Second, it streamlines systematic reviews by composing optimized search strings, screening titles and abstracts, and identifying full-text discrepancies. Third, AI assistants can draft reproducible analytic code, perform preliminary descriptive or inferential analyses, and create publication-ready tables and figures. Fourth, the models support scholarly writing by suggesting journal-specific headings, refining prose, harmonizing references, and translating technical content for multidisciplinary audiences. Fifth, they augment peer-review and editorial workflows by delivering evidence-focused critiques. In educational settings, these models can create adaptive curricula and interactive simulations for trainees, fostering digital literacy and evidence-based practice early in professional development among clinicians. Integration into clinical decision-support pipelines is also foreseeable, warranting proactive governance. Notwithstanding these opportunities, responsible use demands vigilant oversight. Large-language models occasionally fabricate citations or misinterpret domain-specific data ("hallucinations"), potentially propagating misinformation. Outputs are highly prompt-dependent, creating a reliance on informed prompt engineering that may disadvantage less technical clinicians. Moreover, uploading protected health information or copyrighted text raises privacy, security, and intellectual-property concerns. We outline best-practice recommendations: maintain human verification of all AI-generated content; cross-validate references with primary databases; employ privacy-preserving, on-premises deployments for sensitive data; document prompts for reproducibility; and disclose AI involvement transparently. In summary, generative AI offers a powerful adjunct for OBGYN scientists by accelerating topic formulation, evidence synthesis, data analysis, manuscript preparation, and peer review. When coupled with rigorous oversight and ethical safeguards, these tools can enhance productivity without compromising scientific integrity. Future studies should quantify accuracy, bias, and downstream patient impact.
PMID: 40393680
ISSN: 1098-8785
CID: 5853042

Application of plasma cell-free metagenomic next-generation sequencing for the identification of Aspergillus fumigatus donor-derived infections among solid organ transplant recipients [Case Report]

Mah, Jordan K; Hogan, John I; Kothadia, Sonya; Keenan, Jeffrey E; Berger, Johnathan; Carugati, Manuela
A cluster of Aspergillus fumigatus donor-derived infections (DDI) was rapidly diagnosed using plasma metagenomic next-generation sequencing (mNGS) among solid organ transplant recipients. The heart recipient, experiencing marginal hemodynamics, underwent an endomyocardial biopsy, which was concerning for a fungal infection on histopathology. Plasma mNGS was performed, identifying A. fumigatus two days prior to conventional diagnostics. This timely diagnosis enabled prompt nephrectomies in the kidney recipients, who survived. This report represents the first published use of mNGS in the diagnosis of Aspergillus fumigatus DDI, highlighting the utility of this novel, underutilized assay for early diagnosis of donor-derived infections.
PMCID:12663023
PMID: 41324077
ISSN: 2211-7539
CID: 5974682

Implementing system-wide digital medical interpretation: a framework for healthcare organizations

Kothari, Ulka; Squires, Allison; Austrian, Jonathan; Feldman, Anatoly; Syed, Irfan; Jones, Simon
OBJECTIVES/UNASSIGNED:This study evaluates and enhances language access services for Limited English Proficiency (LEP) patients in a large urban health system by integrating interpreter services into the Electronic Health Record (EHR), aiming to reduce care disparities and improve the digital experience for both patients and clinicians. MATERIALS AND METHODS/UNASSIGNED:Using a descriptive evaluation approach, the project assessed barriers to interpreter service usage and developed solutions informed by stakeholder engagement. Emphasis was placed on interfacing the EHR with the vendor platform, using existing devices, tracking utilization, ensuring cost-effectiveness, and implementing the solution across multiple hospitals and outpatient settings. RESULTS DISCUSSION AND CONCLUSION/UNASSIGNED:After implementation, audio and video interpreter service use rose significantly, with calls increasing from an average of 9700 calls per month in 2022 to over 68 176 calls per month by the end of 2024. Over 14 000 clinicians used the service for more than 121 077 unique patients. Average waiting times for the top ten languages fell below 30 seconds, and user satisfaction was high, with an average interpreter rating of 4.9. Conclusion: The EHR-integrated interpreter service has improved language service access, reduced waiting times and enhanced user satisfaction, marking noteworthy progress in overcoming language barriers and potentially decreasing healthcare disparities for LEP populations. We plan to assess the impact on patient outcomes.
PMCID:12628185
PMID: 41267856
ISSN: 2574-2531
CID: 5976112

Adipose microsomal triglyceride transfer protein deficiency protects against hepatic steatosis by upregulating PPARα activity

Rajan, Sujith; Verano, Michael; Palaia, Thomas; Prakashmurthy, Chandana; Chung, Jay; Islam, Shahidul; Lee, Lili; James, Antonisamy William; Alemán, José O; Goldberg, Ira J; Fisher, Edward A; Hussain, M Mahmood
BACKGROUND & AIM/UNASSIGNED:Metabolic dysfunction-associated steatotic liver disease (MASLD) is a growing health issue. Identifying factors that prevent hepatic lipid accumulation could inform new MASLD prevention or treatment strategies. We previously demonstrated that adipocyte microsomal triglyceride transfer protein (MTP) regulates intracellular lipolysis by inhibiting adipose triglyceride lipase activity. The aim of this study was to investigate the impact of adipose MTP deficiency on MASLD. METHODS/UNASSIGNED: RESULTS/UNASSIGNED: CONCLUSION/UNASSIGNED:These findings highlight the importance of regulated FA flux from adipose tissue to the liver and the liver's adaptive capacity to utilize adipose-derived FAs in maintaining hepatic health. Modulation of adipocyte FA release may represent a therapeutic strategy to reduce hepatic steatosis. IMPACT AND IMPLICATIONS/UNASSIGNED:This study provides significant insights into the role of adipose-specific microsomal triglyceride transfer protein in regulating hepatic lipid metabolism and its potential implications for treating metabolic dysfunction-associated steatotic liver disease. By demonstrating that microsomal triglyceride transfer protein deficiency in adipose tissue leads to increased fatty acid oxidation and reduced hepatic steatosis through enhanced PPARα activation, the research underscores the importance of adipose-liver crosstalk in maintaining liver health. These findings suggest that targeting adipocyte fatty acid release could be a promising therapeutic strategy to mitigate hepatic lipid accumulation and combat metabolic dysfunction-associated steatotic liver disease, offering a novel approach to addressing this growing health issue.
PMCID:12657731
PMID: 41321937
ISSN: 2589-5559
CID: 5974542