bluuebunny/crossref_metadata_2025_split huggingface dataset:
Evaluation of Malignant Hyperthermia Features in Patients with Pathogenic or Likely Pathogenic RYR1 Variants Disclosed through a Population Genomic Screening Program
Authors: Yu Kristen D. , Betts Megan N. , Urban Gretchen M. , Schwartz Marci L. B. , Robinson Tanisha O. , Moyer Robert J. , Taddonio Scott W. , Vasudevan Anasuya , Johns Alicia , Sturm Amy C. , Kelly Melissa A. , Williams Marc S. , Poler S. Mark , Buchanan Adam H. 
Published: October 2023

Background
Malignant hyperthermia (MH) susceptibility is a heritable musculoskeletal disorder that can present as a potentially fatal hypermetabolic response to triggering anesthesia agents. Genomic screening for variants in MH-associated genes RYR1 and CACNA1S provides an opportunity to prevent morbidity and mortality. There are limited outcomes data from disclosing variants in RYR1, the most common MH susceptibility gene, in unselected populations. The authors sought to identify the rate of MH features or fulminant episodes after triggering agent exposure in an unselected population undergoing genomic screening including actionable RYR1 variants.

Methods
The MyCode Community Health Initiative by Geisinger (USA) is an electronic health record–linked biobank that discloses pathogenic and likely pathogenic variants in clinically actionable genes to patient-participants. Available electronic anesthesia and ambulatory records for participants with actionable RYR1 results returned through December 2020 were evaluated for pertinent findings via double-coded chart reviews and reconciliation. Descriptive statistics for observed phenotypes were calculated.

Results
One hundred fifty-two participants had an actionable RYR1 variant disclosed during the study period. None had previous documented genetic testing for MH susceptibility; one had previous contracture testing diagnosing MH susceptibility. Sixty-eight participants (44.7%) had anesthesia records documenting triggering agent exposure during at least one procedure. None received dantrolene treatment or had documented muscle rigidity, myoglobinuria, hyperkalemia, elevated creatine kinase, severe myalgia, or tea-colored urine. Of 120 possibly MH-related findings (postoperative intensive care unit admissions, hyperthermia, arterial blood gas evaluation, hypercapnia, or tachycardia), 112 (93.3%) were deemed unlikely to be MH events; 8 (6.7%) had insufficient records to determine etiology.

Conclusions
Results demonstrate a low frequency of classic intraanesthetic hypermetabolic phenotypes in an unselected population with actionable RYR1 variants. Further research on the actionability of screening for MH susceptibility in unselected populations, including economic impact, predictors of MH episodes, and expanded clinical phenotypes, is necessary.

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A Triple Combination Therapy Using 2-mm Biopsy Punch for the Treatment of Multifocal Keloids
Authors: Park Tae Hwan 
Published: October 2023

BACKGROUNDKeloid treatment is challenging. The surgical approach can be divided into complete excision versus partial excision.OBJECTIVEThe current study aims to introduce our novel surgical approach of partial excision using a 2-mm punch biopsy device to treat refractory multifocal keloids in the trunk.MATERIALS AND METHODSThis is a case series of 30 patients with refractory multifocal keloids treated with a triple combination therapy consisting of a punch-assisted partial excision and intralesional triamcinolone injections followed by immediate single fractional electron beam radiotherapy within 8 hours, postoperatively. The follow-up period was 12 months. The primary outcome was recorded asrecurrenceversusnonrecurrenceoraggravationversusremission. The secondary outcome was patient satisfaction as assessed by the POSAS.RESULTSThe recurrence or aggravation of keloid was not found without complications. Scores obtained from the POSAS patient scale showed that pain, itchiness, color, stiffness, thickness, and irregularity significantly improved.CONCLUSIONOur novel surgical approach using a 2-mm punch biopsy device effectively treats refractory multifocal keloids once considered intractable. Triple combination therapy of partial excision using a 2-mm punch biopsy device, intralesional triamcinolone injections, followed by immediate single fractional electron beam radiotherapy, is a safe, efficacious, and more convenient protocol to treat this condition.
Mechanisms of improved body composition among perimenopausal women practicing Meditative Movement: a proposed biobehavioral model
Authors: James Dara L. , Larkey Linda K. , Evans Bronwynne , Sebren Ann , Goldsmith Kimberley , Ahlich Erica , Hawley Nanako A. , Kechter Afton , Sears Dorothy D. 
Published: October 2023

Abstract

Objectives
Weight gain and unfavorable body composition are prevalent among midlife/older women throughout menopause. These shifts may negatively impact health, well-being, and longevity. Efforts to attenuate weight and body composition changes are traditionally driven by manipulation of diet and/or exercise; however, sustained results are limited, possibly because the full spectrum of biobehavioral systems is not addressed by diet and exercise alone. We propose a biobehavioral model detailing mechanisms of body composition decline among perimenopausal women and the associated components of Meditative Movement (ie, tai chi, qigong, yoga) that address each of these factors.

Methods
Based on our previous work and extensive review of the literature, we developed a multifactorial and multidimensional biobehavioral model including factors that most directly relate to body composition among perimenopausal women: 1) psychological (ie, stress and mood, mindfulness and self-compassion, body awareness), 2) behavioral (ie, sleep, physical activity, eating behaviors), and 3) physiological (ie, cortisol, estrogen). Relationships between each factor, Meditative Movement practice components, and predicted effects on body composition were explored in detail.

Results
Our model describes select psychological, behavioral, and physiological factors, and potential mechanistic pathways of Meditative Movement practice driving improved changes in body composition and weight outcomes for perimenopausal women.

Conclusions
The proposed model details a novel, evidence-supported means to reduce the risk of deleterious shifts in body composition throughout perimenopause and menopause thereafter. We suggest that these changes may occur directly and/or indirectly through psychological, behavioral, and physiological mechanisms that facilitate the desired changes in body composition.
Health Literacy and Perceived Control
Authors: Miller Jennifer L. , Chung Misook , Williams Lovoria B. , Connell Alison , Saleh Zyad T. , Alhurani Abdullah , Bailey Alison , Rayens Mary Kay , Moser Debra K. 
Published: October 2023

Background
Black race, inadequate health literacy, and poor perceived control are predictors of increased cardiovascular disease (CVD) risk. The purpose of this study was to explore the relationships among race, health literacy, perceived control, and CVD risk while controlling for known risk factors in incarcerated men.

Methods
We included data from 349 incarcerated men to examine race and CVD risk (Framingham Risk Score) using a serial mediation model with health literacy and perceived control using 95% confidence intervals (CIs) from 5000 bootstrap samples.

Results
Of the participants (age, 36 ± 10; education, 12 ± 2; body mass index, 28.3 ± 5.0), 64.2% were White and 35.8% were Black. Black incarcerated men were younger (P = .047) with lower levels of health literacy (P < .001). All 3 indirect effects of race on CVD were significant, whereas the direct effect of race was not. Black incarcerated men had higher levels of CVD risk through health literacy (a
1
b
1 = 0.3571; 95% CI, 0.0948–0.7162) and lower levels of CVD risk through perceived control (a
2
b
2 = −0.1855; 95% CI, −0.4388 to −0.0077). Black incarcerated men had higher levels of CVD risk through health literacy influenced by perceived control (a
1
b
2
d
21 = 0.0627; 95% CI, 0.0028–0.1409), indicating that despite the protective effect of higher levels of perceived control in Black incarcerated men, CVD risk remained higher compared with their White counterparts.

Conclusion
Future CVD risk reduction interventions in incarcerated men, specifically Black incarcerated men, should include goals of improving health literacy and perceived control as modifiable risk factors.
Agreement of Computerized QT and QTc Interval Measurements Between Both Bedside and Expert Nurses Using Electronic Calipers
Authors: Ho Karolina , Ganesh Gopika K. , Prasad Shelvin , Hoffmann Thomas J. , Larsen Amy , Sandoval Cass , Berger Sarah , Schell-Chaple Hildy , Badilini Fabio , Mackin Lynda A. , Pelter Michele M. 
Published: October 2023

Background
In hospitalized patients, QT/QTc (heart rate corrected) prolongation on the electrocardiogram (ECG) increases the risk of torsade de pointes. Manual measurements are time-consuming and often inaccurate. Some bedside monitors automatically and continuously measure QT/QTc; however, the agreement between computerized versus nurse-measured values has not been evaluated.

Objective
The aim of this study was to examine the agreement between computerized QT/QTc and bedside and expert nurses who used electronic calipers.

Methods
This was a prospective observational study in 3 intensive care units. Up to 2 QT/QTc measurements (milliseconds) per patient were collected. Bland-Altman test was used to analyze measurement agreement.

Results
A total of 54 QT/QTc measurements from 34 patients admitted to the ICU were included. The mean difference (bias) for QT comparisons was as follows: computerized versus expert nurses, −11.04 ± 4.45 milliseconds (95% confidence interval [CI], −2.3 to −19.8; P = .016), and computerized versus bedside nurses, −13.72 ± 6.70 (95% CI, −0.70 to −26.8; P = .044). The mean bias for QTc comparisons was as follows: computerized versus expert nurses, −12.46 ± 5.80 (95% CI, −1.1 to −23.8; P = .035), and computerized versus bedside nurses, −18.49 ± 7.90 (95% CI, −3.0 to −33.9; P = .022).

Conclusion
Computerized QT/QTc measurements calculated by bedside monitor software and measurements performed by nurses were in close agreement; statistically significant differences were found, but differences were less than 20 milliseconds (on-half of a small box), indicating no clinical significance. Computerized measurements may be a suitable alternative to nurse-measured QT/QTc. This could reduce inaccuracies and nurse burden while increasing adherence to practice recommendations. Further research comparing computerized QT/QTc from bedside monitoring to standard 12-lead electrocardiogram in a larger sample, including non-ICU patients, is needed.
Autoimmune hepatitis recurrence after liver transplantation: “Les jeux sont faits”
Authors: Chouik Yasmina , Corpechot Christophe , Francoz Claire , De Martin Eleonora , Guillaud Olivier , Abergel Armand , Altieri Mario , Barbier Louise , Besch Camille , Chazouillères Olivier , Conti Filomena , Dharancy Sébastien , Durand François , Duvoux Christophe , Gugenheim Jean , Hardwigsen Jean , Hilleret Marie-Noëlle , Houssel-Debry Pauline , Kamar Nassim , Minello Anne , Neau-Cransac Martine , Pageaux Georges-Philippe , Radenne Sylvie , Roux Olivier , Saliba Faouzi , Samuel Didier , Vanlemmens Claire , Woehl-Jaegle Marie-Lorraine , Leroy Vincent , Duclos-Vallée Jean-Charles , Dumortier Jérôme 
Published: October 2023

Autoimmune hepatitis (AIH) may recur after liver transplantation (LT). The aims of this study were to evaluate the incidence and risk factors for recurrent autoimmune hepatitis (rAIH). A multicenter retrospective French nationwide study, including all patients aged ≥16 transplanted for AIH, with at least 1 liver biopsy 1 year after LT, was conducted between 1985 and 2018. Risk factors for rAIH were identified using a multivariate Cox regression model. Three hundred and forty-four patients were included (78.8% women) with a median age at LT of 43.6 years. Seventy-six patients (22.1%) developed recurrence in a median time of 53.6 months (IQR, 14.1–93.2). Actuarial risk for developing rAIH was 41.3% 20 years after LT. In multivariate analysis, the strongest risk factor for rAIH was cytomegalovirus D+/R− mismatch status (HR=2.0; 95% CI: 1.1–3.6; p=0.03), followed by associated autoimmune condition. Twenty-one patients (27.6% of rAIH patients) developed liver graft cirrhosis after rAIH. Independent risk factors for these severe forms of rAIH were young age at LT, IgG levels >20.7 g/L, and LT in the context of (sub)fulminant hepatitis. Immunosuppression, especially long-term maintenance of corticosteroid therapy, was not significantly associated with rAIH. Recurrence of AIH after LT is frequent and may lead to graft loss. Recurrence is more frequent in young patients with active disease at the time of LT, yet systematic corticosteroid therapy does not prevent it.
The Kvetch
Authors: Yager Joel , Kay Jerald 
Published: October 2023

Abstract
Clinicians often encounter patients whose presentations are characterized by long lists of complaints about their biological, psychological, interpersonal, and social conditions. The problems on which the complaints are based are variably reality-based and variably modifiable. Some of these patients display chronic complaining as a core, distinguishing feature. Accordingly, the aims of this article are to consider excesses of chronic complaining as psychiatric phenomena, explore possible pathogenetic contributions, describe approaches for treating conditions marked by clinically pertinent chronic complaining, and suggest areas for future research. Based on clinical observations enhanced by selective narrative literature review, we delineate and differentiate four groups of patients: 1) situational complainers; 2) chronic complainers due to unidentified medical problems; 3) mood-induced chronic complainers; and 4) personality-driven pan-dimensional chronic complainers. The last-mentioned group consists of help-seeking versus help-rejecting subtypes, the latter including a subset we designate as malignant chronic complainers. Strategies for managing these patients begin with detailed assessment of all complaints, ascertaining reality-based contributions to the complaints, including those initiated by patients themselves. Management approaches use specific biopsychosocial techniques based on patient-centered particulars. Psychotherapeutic strategies center on compassionate, empathic witnessing. Specific tactics include attending to unresolved grief and trauma, behavioral activation, cognitive and narrative restructuring and reframing methods, mentalizing and imaginal approaches, and psychodynamic methods including attention to attachment issues and transference. Sources of countertransference reactions to these patients should be identified and can be addressed. The many questions raised by these patients' presentations merit further research.
Continuity Strategies for Long-Stay PICU Patients: Consensus Statements From the Lucile Packard Foundation PICU Continuity Panel
Authors: Edwards Jeffrey D. , Wocial Lucia D. , Madrigal Vanessa N. , Moon Michelle M. , Ramey-Hunt Cheryl , Walter Jennifer K. , Baird Jennifer D. , Leland Brian D. 
Published: October 2023

OBJECTIVES:To develop consensus statements on continuity strategies using primary intensivists, primary nurses, and recurring multidisciplinary team meetings for long-stay patients (LSPs) in PICUs.PARTICIPANTS:The multidisciplinary Lucile Packard Foundation PICU Continuity Panel comprising parents of children who had prolonged PICU stays and experts in several specialties/professions that care for children with medical complexity in and out of PICUs.DESIGN/METHODS:We used modified RAND Delphi methodology, with a comprehensive literature review, Delphi surveys, and a conference, to reach consensus. The literature review resulted in a synthesized bibliography, which was provided to panelists. We used an iterative process to generate draft statements following panelists’ completion of four online surveys with open-ended questions on implementing and sustaining continuity strategies. Panelists were anonymous when they voted on revised draft statements. Agreement of 80% constituted consensus. At a 3-day virtual conference, we discussed, revised, and re-voted on statements not reaching or barely reaching consensus. We used Grading of Recommendations Assessment, Development, and Evaluation to assess the quality of the evidence and rate the statements’ strength. The Panel also generated outcome, process, and balancing metrics to evaluate continuity strategies.RESULTS:The Panel endorsed 17 consensus statements in five focus areas of continuity strategies (Eligibility Criteria, Initiation, Standard Responsibilities, Resources Needed to Implement, Resources Needed to Sustain). The quality of evidence of the statements was low to very low, highlighting the limited evidence and the importance of panelists’ experiences/expertise. The strength of the statements was conditional. An extensive list of potential evaluation metrics was generated.CONCLUSIONS:These expert/parent-developed consensus statements provide PICUs with novel summaries on how to operationalize, implement, and sustain continuity strategies for LSP, a rapidly growing, vulnerable, resource-intensive population in PICUs.
Proprotein convertase subtisilin/kexin 9 levels decline with hepatitis C virus therapy in people with HIV/hepatitis C virus and correlate with inflammation
Authors: Gandhi Malini M. , Nguyen Kim-Lien , Lake Jordan E. , Liao Diana , Khodabakhshian Aleen , Guerrero Mario , Shufelt Chrisandra L. , Bairey Merz C. Noel , Jordan Wilbert C. , Daar Eric S. , Bhattacharya Debika , Chew Kara W. 
Published: October 2023

Background:
Proprotein convertase subtisilin/kexin 9 (PCSK9) raises low-density lipoprotein cholesterol (LDL-C) levels and is associated with inflammation, which is elevated in HIV and hepatitis C virus (HCV) infection. We compared PCSK9 levels in people with co-occurring HIV and HCV (HIV/HCV) vs. HIV alone, and evaluated the impact of HCV direct-acting antiviral (DAA) therapy on PCSK9.

Design:
A prospective, observational cohort study.

Methods:
Thirty-five adults with HIV/HCV and 37 with HIV alone were evaluated, all with HIV virologic suppression and without documented cardiovascular disease. Circulating PCSK9 and inflammatory biomarkers were measured at baseline and following HCV treatment or at week 52 (for HIV alone) and compared using Wilcoxon tests and Spearman correlations.

Results:
At baseline, PCSK9 trended higher in HIV/HCV vs. HIV alone (307 vs. 284 ng/ml, P = 0.06). Twenty-nine participants with HIV/HCV completed DAA therapy with sustained virologic response. PCSK9 declined from baseline to posttreatment 1 (median 7.3 weeks after end of therapy [EOT]) and posttreatment 2 (median 43.5 weeks after EOT), reaching levels similar to HIV alone; median within-person reduction was -60.5 ng/ml (P = 0.003) and -55.6 ng/ml (P = 0.02), respectively. Decline in PCSK9 correlated with decline in soluble (s)E-selectin and sCD163 (r = 0.64, P = 0.002; r = 0.58, P = 0.008, respectively), but not with changes in LDL-C or other biomarkers. No significant change in PCSK9 occurred in the HIV alone group over 52 weeks.

Conclusion:
PCSK9 declined with DAA therapy in participants with HIV/HCV, correlating with declines in several inflammatory biomarkers but not LDL-C. Elevated PCSK9 with HCV may be linked to particular HCV-associated inflammatory pathways more so than cholesterol homeostasis.
Lower endoscopy, early-onset, and average-onset colon cancer among Medicaid beneficiaries with and without HIV
Authors: Joshu Corinne E. , Calkins Keri L. , Rudolph Jacqueline E. , Xu Xiaoqiang , Wentz Eryka , Coburn Sally B. , Kaur Maneet , Pirsl Filip , Moore Richard D. , Lau Bryan 
Published: October 2023

Background:
Studies suggest a lower colorectal cancer (CRC) risk and lower or similar CRC screening among people with HIV (PWH) compared with the general population. We evaluated the incidence of lower endoscopy and average-onset (diagnosed at ≥50) and early-onset (diagnosed at <50) colon cancer by HIV status among Medicaid beneficiares with comparable sociodemographic factors and access to care.

Methods:
We obtained Medicaid Analytic eXtract (MAX) data from 2001 to 2015 for 14 states. We included 41 727 243 and 42 062 552 unique individuals with at least 7 months of continuous eligibility for the endoscopy and colon cancer analysis, respectively. HIV and colon cancer diagnoses and endoscopy procedures were identified from inpatient and other nondrug claims. We used Cox proportional hazards regression models to assess endoscopy and colon cancer incidence, controlling for age, sex, race/ethnicity, calendar year and state of enrollment, and comorbidities conditions.

Results:
Endoscopy and colon cancer incidence increased with age in both groups. Compared with beneficiaries without HIV, PWH had an increased hazard of endoscopy; this association was strongest among those 18–39 years [hazard ratio: 1.85, 95% confidence interval (95% CI) 1.77–1.92] and attenuated with age. PWH 18–39 years also had increased hazard of early-onset colon cancer (hazard ratio: 1.66, 95% CI:1.05–2.62); this association was attenuated after comorbidity adjustment. Hazard ratios were null among all beneficiaries less than 50 years of age. PWH had a lower hazard of average-onset colon cancer compared with those without HIV (hazard ratio: 0.79, 95% CI: 0.66–0.94).

Conclusion:
PWH had a higher hazard of endoscopy, particularly at younger ages. PWH had a lower hazard of average-onset colon cancer. Early-onset colon cancer was higher among the youngest PWH but not associated with HIV overall.
Factors associated with insulin resistance in a longitudinal study of Ugandan youth with and without HIV
Authors: Dirajlal-Fargo Sahera , Strah Monika , Ailstock Kate , Sattar Abdus , Karungi Christine , Nazzinda Rashidah , Funderburg Nicholas , Kityo Cissy , Musiime Victor , McComsey Grace A. 
Published: October 2023

Prospective investigations from sub-Saharan Africa on metabolic complications in youth with perinatally acquired HIV (PHIV) are lacking. We investigated the changes in insulin resistance in Ugandan PHIV on ART and uninfected controls and their relationship with inflammation, HIV, and cardiovascular disease (CVD) risk factors.
Participants 10–18 years of age were included in a prospective study performed in Kampala, Uganda. We compared baseline and changes in insulin resistance (by HOMA-IR) and in markers of inflammation at baseline and 96 weeks. PHIVs were on ART with HIV-1 RNA level 400 copies/ml or less. Generalized Estimating Equation models were used to assess associations between HOMA-IR, and demographic as well as inflammatory markers.
Of the 197 participants recruited at baseline (101 PHIV, 96 HIV-negative), 168 (89 PHIV, 79 HIV-negative) had measurements at 96 weeks. At baseline, median (Q1, Q3) age was 13 years (11,15), 53.5% were women, median CD4+ cell counts were 988 cells/μl (631, 1310). At baseline, HOMA-IR was significantly higher in PHIV than in controls (P = 0.03). HOMA-IR did not significantly change by week 96 in either group, and at 96 weeks, was similar between groups (P = 0.15). HOMA-IR was not associated with any inflammatory markers, or any specific ART. In longitudinal analysis, age and Tanner stage remained associated with higher HOMA-IR throughout the study period, after adjusting for HIV status.
In this longitudinal cohort of virally suppressed PHIV in Uganda, PHIV have decreased insulin sensitivity compared to controls, however this difference does not persist through adolescence. ART and immune activation do not appear to affect glucose homeostasis in this population.