bluuebunny/crossref_metadata_2025_split huggingface dataset:
Pediatric Wilson's Disease: Phenotypic, Genetic Characterization and Outcome of 182 Children in France
Authors: Couchonnal Eduardo , Lion-François Laurence , Guillaud Olivier , Habes Dalila , Debray Dominique , Lamireau Thierry , Broué Pierre , Fabre Alexandre , Vanlemmens Claire , Sobesky Rodolphe , Gottrand Frederic , Bridoux-Henno Laure , Dumortier Jérôme , Belmalih Abdelouahed , Poujois Aurelia , Jacquemin Emmanuel , Brunet Anne Sophie , Bost Muriel , Lachaux Alain 
Published: June 2021

ABSTRACT

Objectives:
To describe a cohort of Wilson disease (WD) pediatric cases, and to point out the diagnostic particularities of this age group and the long-term outcome.

Methods:
Clinical data of 182 pediatric patients included in the French WD national registry from 01/03/1995 to 01/06/2019 were gathered.

Results:
Diagnosis of WD was made at a mean age of 10.7 ± 4.2 years (range 1–18 years). At diagnosis, 154 patients (84.6%) had hepatic manifestations, 19 (10.4%) had neurological manifestations, and 9 patients (4.9%) were asymptomatic. The p.His1069Gln mutation was the most frequently encountered (14% of patients).
Neurological patients were diagnosed at least 1 year after they presented their first symptoms. At diagnosis, the median urinary copper excretion (UCE) was 4.2 μmol/24 hours (0.2–253). The first-line treatment was d-penicillamine (DP) for 131 (72%) patients, zinc salts for 24 (13%) patients, and Trientine for 17 (9%) patients. Liver transplantation was performed in 39 (21.4%) patients, for hepatic indications in 33 of 39 patients or for neurological deterioration in 6 of 39 patients, mean Unified Wilson's Disease Rating Scale of the latter went from 90 ± 23.1 before liver transplantation (LT) to 26.8 ± 14.1 (P < 0.01) after a mean follow-up of 4.3 ± 2.5 years. Overall survival rate at 20 years of follow-up was 98%, patient and transplant-free combined survival was 84% at 20 years.

Conclusion:
Diagnosis of WD can be challenging in children, particularly at the early stages of liver disease and in case of neurological presentation; hence the support of clinical scores and genetic testing is essential. Diagnosis at early stages and proper treatment ensure excellent outcomes, subject to good long-term treatment compliance. LT is a valid option for end-stage liver disease not responding to treatment and can be discussed for selected cases of neurological deterioration.
Are Mobile Persons With Parkinson Disease Necessarily More Active?
Authors: Zajac Jenna A. , Cavanaugh James T. , Baker Teresa , Colón-Semenza Cristina , DeAngelis Tamara R. , Duncan Ryan P. , Fulford Daniel , LaValley Michael , Nordahl Timothy , Rawson Kerri S. , Saint-Hilaire Marie , Thomas Cathi A. , Earhart Gammon M. , Ellis Terry D. 
Published: June 2021

Background and Purpose:
Walking activity in persons with Parkinson disease (PD) is important for preventing functional decline. The contribution of walking activity to home and community mobility in PD is poorly understood.

Methods:
Cross-sectional baseline data (N = 69) were analyzed from a randomized controlled PD trial. The Life-Space Assessment (LSA) quantified the extent, frequency, and independence across 5 expanding levels of home and community mobility, producing individual subscores and a total score. Two additional summed scores were used to represent mobility within (Levels 1-3) and beyond (Levels 4-5) neighborhood limits. An accelerometer measured walking activity for 7 days. Regression and correlation analyses evaluated relationships between daily steps and mobility scores. Mann-Whitney U tests secondarily compared differences in mobility scores between the active and sedentary groups.

Results:
Walking activity contributed significantly to the summed Level 1-3 score (β = 0.001, P = 0.004) but not to the summed Level 4-5 (β = 0.001, P = 0.33) or total (β = 0.002, P = 0.07) scores. Walking activity was significantly related to Level 1 (ρ = 0.336, P = 0.005), Level 2 (ρ = 0.307, P = 0.010), and Level 3 (ρ = 0.314, P = 0.009) subscores. Only the summed Level 1-3 score (P = 0.030) was significantly different between the active and sedentary groups.

Discussion and Conclusions:
Persons with PD who demonstrated greater mobility beyond the neighborhood were not necessarily more active; walking activity contributed more so to home and neighborhood mobility. Compared with LSA total score, the Level 1-3 summed score may be a more useful participation-level measure for assessing the impact of changes in walking activity.

Video Abstract available for more insights from the authors (see the Video, Supplemental Digital Content 1 available at: http://links.lww.com/JNPT/A349)
Effects of Condom Use on Human Immunodeficiency Virus Transmission Among Adolescent Sexual Minority Males in the United States: A Mixed Epidemiology and Epidemic Modeling Study
Authors: Katz David A. , Hamilton Deven T. , Rosenthal Elizabeth M. , Wang Li Yan , Dunville Richard L. , Aslam Maria , Barrios Lisa C. , Zlotorzynska Maria , Sanchez Travis H. , Sullivan Patrick S. , Rosenberg Eli S. , Goodreau Steven M. 
Published: June 2021

Background
We examined condom use patterns and potential population-level effects of a hypothetical condom intervention on human immunodeficiency virus (HIV) transmission among adolescent sexual minority males (ASMM).

Methods
Using 3 data sets: national Youth Risk Behavior Survey 2015 to 2017 (YRBS-National), local YRBS data from 8 jurisdictions with sex of partner questions from 2011 to 2017 (YRBS-Trends), and American Men's Internet Survey (AMIS) 2014 to 2017, we assessed associations of condom use with year, age, and race/ethnicity among sexually active ASMM. Using a stochastic agent-based network epidemic model, structured and parameterized based on the above analyses, we calculated the percent of HIV infections averted over 10 years among ASMM ages 13 to 18 years by an intervention that increased condom use by 37% for 5 years and was delivered to 62% of ASMM at age 14 years.

Results
In YRBS, 51.8% (95% confidence interval [CI], 41.3–62.3%) and 37.9% (95% CI, 32.7–42.3%) reported condom use at last sexual intercourse in national and trend data sets, respectively. In AMIS, 47.3% (95% CI, 44.6–49.9%) reported condom use at last anal sex with a male partner. Temporal trends were not observed in any data set (P > 0.1). Condom use varied significantly by age in YRBS-National (P < 0.0001) and YRBS-Trends (P = 0.032) with 13- to 15-year-olds reporting the lowest use in both; age differences were not significant in AMIS (P = 0.919). Our hypothetical intervention averted a mean of 9.0% (95% simulation interval, −5.4% to 21.2%) of infections among ASMM.

Conclusions
Condom use among ASMM is low and appears to have remained stable during 2011 to 2017. Modeling suggests that condom use increases, consistent with previous interventions, have potential to avert 1 in 11 new HIV infections among ASMM.
An Automated Syphilis Serology Record Search and Review Algorithm to Prioritize Investigations by Health Departments
Authors: Karki Saugat , Peterman Thomas A. , Johnson Kimberly , Hennessy Robin R. , Matthias James , Wilson Craig , Mishra Ninad , Weinstock Hillard 
Published: June 2021

Background
Reactive syphilis serologies are investigated by health departments to determine if they represent new infection, reinfection, or treatment failure. Serologies prioritized for investigation based on nontreponemal test titer and age (using a “reactor grid”) undergo manual record search and review. We developed a computerized algorithm that automates the record search and review.

Methods
We developed and tested the algorithm using a Florida Department of Health data set containing serologies reported January 2016 to December 2018 and previous records linked to each individual. The algorithm was based on the syphilis case definition, which requires (except primary cases with signs and symptoms) (1) a positive treponemal test result and a newly positive nontreponemal test result or (2) a 4-fold increase in nontreponemal test titer. Two additional steps were added to avoid missing cases. New York City Department of Health and Mental Hygiene validated this algorithm.

Results
The algorithm closed more investigations (49.9%) than the reactor grid (27.0%). The algorithm opened 99.4% of the individuals investigated and labeled as cases by the health department; it missed 75 cases. Many investigations opened by the algorithm were closed by the reactor grid; we could not assess how many would have been cases. In New York City, the algorithm closed 70.9% of investigations, likely because more individuals had previous test in the database (88.2%) compared with Florida (56.5%).

Conclusions
The automated algorithm successfully searched and reviewed records to help identify cases of syphilis. We estimate the algorithm would have saved Florida 590 workdays for 3 years.
Reported Chlamydia and Gonorrhea Are Decreasing Among Young Black Women: Good News or Bad News? A Narrative Review
Authors: Diesel Jill , Peterson Amy , Peterman Thomas 
Published: June 2021

Abstract
Since 2010, reported chlamydia and gonorrhea rates decreased among Black women aged 15 to 19 years and were stable for Black women aged 20 to 24 years in the United States. Rates increased for older Black women aged 25 to 39 years and all White women. The Black/White rate ratio decreased across age groups. We examined whether trends in reported rates reflected changing prevalence or changing screening. We analyzed trends in reported chlamydia and gonorrhea rates from 2010 to 2018 among women in the United States aged 15 to 39 years by age and race/ethnicity subgroup, state, and reporting source. Most jurisdictions reported decreased chlamydia and gonorrhea rates among Black teens and increased rates among White teens and older women. Between 2010 and 2018, public clinics reported fewer cases, especially among young Black women, that were not restored by increases elsewhere. We reviewed literature on trends in screening, prevalence, and sequelae. Family planning clinics annual reports showed chlamydia tests among women younger than 25 years decreased by 541,573 tests (−38%) in 2018 compared with 2010 and the number of women visiting sexually transmitted disease clinics had decreased 50% by 2016 compared with 2010. Prevalence of chlamydia in a sentinel population (Job Corps) was unchanged for Black women younger than 25 years and increased for Whites aged 20 to 24 years. Sequelae trends using data from a large all-payer emergency department database were mixed: pelvic inflammatory disease decreased, whereas ectopic pregnancy increased. Decreases in testing at public clinics likely missed diagnoses among young Black women, a group traditionally at highest risk and in need of more testing. Innovative approaches to screening are needed.
A Single-center Retrospective Trial of a Blink-assisted Eyelid Device in Treating the Signs and Symptoms of Dry Eye
Authors: Chester Thomas 
Published: June 2021

SIGNIFICANCE
The clinical features of meibomian gland disease include altered tear film stability, damage to the ocular surface, symptoms of ocular surface irritation, and visual fluctuations. Finding an adequate treatment to alleviate a patient's signs and symptoms is vital to caring for those with dry eye disease resulting from meibomian gland disease.

PURPOSE
The purpose of this analysis was to determine whether the controlled heating of meibomian glands with the SmartLid devices (TearCare) combined with evacuation of the liquefied meibum using a handheld clearance assistant would improve a patient's dry eye symptoms (as measured by the Standardized Patient Evaluation of Eye Dryness [SPEED] questionnaire) and signs (as measured by meibomian gland expression [MGE] scores).

METHODS
This study involved a retrospective analysis of data gathered in a single-center ophthalmology/optometry practice. The symptom frequency and severity were assessed using the SPEED questionnaire, and the signs were assessed via MGE scores before and after (8 to 12 weeks) treatment. A further analysis evaluating efficacy in subgroups based on age, race, and sex was performed. A statistical analysis was performed with t tests for group comparisons.

RESULTS
A SPEED questionnaire was answered by 92 patients with dry eye disease. In addition, each patient's meibomian gland function was recorded as MGE scores for each eye (176 eyes). These procedures were completed before and approximately 8 weeks after a single bilateral TearCare treatment. The median total SPEED score was reduced from 16 to 9, and the total MGE scores improved from 5.0 to 9.0 in the right eye and 4.0 to 9.0 in the left eye after a single TearCare treatment.

CONCLUSIONS
A single TearCare treatment was effective in reducing both the signs and symptoms of dry eye in all subjects.
Updates in Forensic Pathology Education
Authors:   Forensic Pathology Working Group
Published: June 2021

Abstract
The Accreditation Council for Graduate Medical Education Milestones offer a framework for competency-based assessment of forensic pathology fellows. The initial forensic pathology–specific milestones (Milestones 1.0) were developed in 2014. Although a great foundation, this first iteration of the milestones was somewhat convoluted and exhibited areas of discrepancy. A Forensic Pathology Milestone 2.0 Working Group of 12 volunteers, with various levels of forensic pathology expertise representing a broad array of institutions across the United States, sought to reduce milestone complexity and modify the cross-specialty “harmonized” milestones to ensure they fit within the context of forensic pathology training. The group also created a supplemental guide to provide additional clarification for each milestone. Overall, 5 specialty-specific subcompetencies were drafted, and the language in the 14 harmonized subcompetencies was adjusted to reflect the unique population of patients and multidisciplinary medicolegal systems inherent to forensic pathology. Once the initial development was complete, the Milestones 2.0 draft was made available for review by the greater forensic pathology community, with the aim of creating a shared mental model for forensic pathology education and the trajectory of the field. Additional changes were made based on the survey results and Milestones 2.0 will be implemented during the 2021–2022 academic year.
The Pediatric Emergency Research Network
Authors: Klassen Terry , Dalziel Stuart R. , Babl Franz E. , Benito Javier , Bressan Silvia , Chamberlain James , Chang Todd P. , Freedman Stephen B. , Kohn-Loncarica Guillermo , Lyttle Mark D. , Mintegi Santiago , Mistry Rakesh D. , Nigrovic Lise E. , Oostenbrink Rianne , Plint Amy C. , Rino Pedro , Roland Damian , Van De Mosselaer Gregory , Kuppermann Nathan 
Published: June 2021

ObjectivesThe Pediatric Emergency Research Network (PERN) was launched in 2009 with the intent for existing national and regional research networks in pediatric emergency care to organize globally for the conduct of collaborative research across networks.MethodsThe Pediatric Emergency Research Network has grown from 5- to 8-member networks over the past decade. With an executive committee comprising representatives from all member networks, PERN plays a supportive and collaborative rather than governing role. The full impact of PERN's facilitation of international collaborative research, although somewhat difficult to quantify empirically, can be measured indirectly by the observed growth of the field, the nature of the increasingly challenging research questions now being addressed, and the collective capacity to generate and implement new knowledge in treating acutely ill and injured children.ResultsBeginning as a pandemic response with a high-quality retrospective case-controlled study of H1N1 influenza risk factors, PERN research has progressed to multiple observational studies and ongoing global randomized controlled trials. As a recent example, PERN has developed sufficient network infrastructure to enable the rapid initiation of a prospective observational study in response to the current coronavirus disease 2019 pandemic. In light of the ongoing need for translation of research knowledge into equitable clinical practice and to promote health equity, PERN is committed to a coordinated international effort to increase the uptake of evidence-based management of common and treatable acute conditions in all emergency department settings.ConclusionsThe Pediatric Emergency Research Network's successes with global research, measured by prospective observational and interventional studies, mean that the network can now move to improve its ability to promote the implementation of scientific advances into everyday clinical practice. Achieving this goal will involve focus in 4 areas: (1) expanding the capacity for global randomized controlled trials; (2) deepening the focus on implementation science; (3) increasing attention to healthcare disparities and their origins, with growing momentum toward equity; and (4) expanding PERN's global reach through addition of sites and networks from resource-restricted regions. Through these actions, PERN will be able to build on successes to face the challenges ahead and meet the needs of acutely ill and injured children throughout the world.
Understanding Clinical Significance in Rehabilitation
Authors: Armijo-Olivo Susan , de Castro-Carletti Ester Moreira , Calixtre Letícia Bojikian , de Oliveira-Souza Ana Izabela Sobral , Mohamad Norazlin , Fuentes Jorge 
Published: June 2021

Abstract
The objective of this review was to summarize the concept of clinical significance and associated methods focusing on the area of rehabilitation to provide a resource to rehabilitation researchers and clinicians. Studies were searched on electronic databases from inception until July 28, 2020, with no date or language limits. Manual searches as well as Scopus forward citation for relevant references were performed. Narrative synthesis of study results was performed. Definitions of the concepts related to clinical significance, ways of calculating, and interpreting each method were provided using rehabilitation examples. An explanation of methods to evaluate clinical significance (distribution, anchor, and opinion-based methods) and their advantages and disadvantages were also provided. Considering the limitations of statistical significance in assuring meaningfulness of results, clinical interpretation of research outcomes and the report of clinical significance in intervention trials should be a priority in rehabilitation research. When possible, the use of multiple methods (distribution, anchor, and opinion based) is recommended. Thus, clinical researchers are encouraged to present results in a manner that allow the rehabilitation professionals to easily interpret and implement those results in their clinical practice.
Comparison of Extracorporeal Shockwave Therapy and Blind Steroid Injection in Patients With Coccydynia
Authors: Ahadi Tannaz , Hosseinverdi Sima , Raissi Gholamreza , Sajadi Simin , Forogh Bijan 
Published: June 2021

Objective
Extracorporeal shockwave therapy (ESWT) emerged recently for the treatment of musculoskeletal conditions owing to its regenerative and anti-inflammatory effects. In this study, the aim was to compare the efficacies of ESWT and steroid injection in patients with coccydynia.

Design
Thirty-four patients with coccydynia were randomized into two groups. In the ESWT group, patients received radial shockwave for three weekly sessions. The second treatment group received an injection of steroid in the tip of the coccyx or sacrococcygeal junction. Outcome measures were visual analog scale, Short-Form Health Survey, and Dallas Pain Questionnaire. Visual analog scale was measured at baseline and 1 wk, 1 mo, 2 mos, and 6 mos after the intervention. Short-Form Health Survey and Dallas Pain Questionnaire were assessed before and 1 mo after treatment.

Results
The visual analog scale score was significantly decreased in all follow-ups in both groups. There was also a significant decrease between 1 wk and three other posttreatment visits in the ESWT treatment group. Time and group interaction effect was significant on the visual analog scale (F
2.64, 84.63 = 2.95, P = 0.043), in favor of ESWT. Concerning the Short-Form Health Survey and Dallas Pain Questionnaire, only some dimensions improved.

Conclusions
ESWT is an effective intervention in patients with coccydynia and had more long-lasting efficacy than steroid in terms of pain relief over 6 mos of observation.
Long-Term Decreased Exercise Capacity of COVID-19 Patients Who Received Mechanical Ventilation in Japan
Authors: Saeki Takuya , Ogawa Fumihiro , Matsumiya Mina , Yamamura Mei , Oritsu Hideyuki , Nonogaki Manabu , Uesugi Jo , Takeuchi Ichiro , Nakamura Takeshi 
Published: June 2021

Abstract
The long-term exercise capacity of coronavirus disease 2019 patients with acute respiratory distress syndrome is not clear. The 6-min walking distance of four patients with coronavirus disease 2019–associated acute respiratory distress syndrome was followed for 6 mos after admission to the hospital. These four patients were admitted to the intensive care unit of our hospital and received mechanical ventilation. Rehabilitation therapy (positioning, postural drainage, and passive range-of-motion exercises) was started after intensive care unit admission. Mobilization therapy, including muscle power training, sitting on the edge of the bed, and endurance training, was performed after the end of sedation. The Medical Research Council sum scores and Barthel Indexes for the patients improved after intensive care unit discharge and completely recovered 6 mos after admission to the hospital. However, the 6-min walking distance of the four patients remained shorter than those of healthy persons of the same age at 6 mos after admission to the hospital. Furthermore, the minimum Spo
2 during the 6-min walking test remained less than 96%. It is possible that patients who receive mechanical ventilation due to coronavirus disease 2019–associated acute respiratory distress syndrome have decreased long-term exercise capacity, despite muscle power and activities of daily living recovering completely.