bluuebunny/crossref_metadata_2025_split huggingface dataset:
Glucocorticoid‐related changes in body mass index among children and adolescents with rheumatic diseases
Authors: Shiff Natalie J. , Brant Rollin , Guzman Jaime , Cabral David A. , Huber Adam M. , Miettunen Paivi M. , Roth Johannes , Scuccimarri Rosie , Alos Nathalie , Atkinson Stephanie A. , Collet Jean Paul , Couch Robert , Cummings Elizabeth A. , Dent Peter B. , Ellsworth Janet , Hay John , Houghton Kristin , Jurencak Roman , Lang Bianca , Larche Maggie , LeBlanc Claire , Rodd Celia , Saint‐Cyr Claire , Stein Robert , Stephure David , Taback Shayne , Rauch Frank , Ward Leanne M. ,   and The Canadian Steroid‐associated Osteoporosis in the Pediatric Population Consortium
Published: November 2012

AbstractObjectiveTo examine the temporal and dose‐related effects of glucocorticoids (GCs) on body mass index (BMI) in children with rheumatic diseases.MethodsChildren initiating GCs for a rheumatic disease (n = 130) were assessed every 3 months for 18 months. BMI, weight, and height Z score trajectories were described according to GC starting dosage in prednisone equivalents: high (≥1.0 mg/kg/day), low (<0.2 mg/kg/day to a maximum of 7.5 mg/day), and moderate (between high and low) dosage. The impact of GC dosing, underlying diagnosis, pubertal status, physical activity, and disease activity on BMI Z scores and on percent body fat was assessed with longitudinal mixed‐effects growth curve models.ResultsThe GC starting dose was high in 59% and moderate in 39% of patients. The peak BMI Z score was +1.29 at 4 months with high‐dose GCs and +0.69 at 4.2 months with moderate‐dose GCs (P < 0.001). Overall, 50% (95% confidence interval 41–59%) of the children returned to within +0.25 SD of their baseline BMI Z score. Oral GC dose over the preceding 3 months was the most significant determinant of BMI Z score and percent body fat. The proportion of days in receipt of GCs, disease activity, and a diagnosis of systemic‐onset juvenile idiopathic arthritis were also associated with BMI Z scores. The correlation between changes in BMI and changes in percent body fat was 0.09.ConclusionIn children with rheumatic diseases starting moderate and high doses of GCs, BMI Z scores peaked at 4 months, and only half returned to within +0.25 SD of their baseline BMI Z score after 18 months.
Comprehensive tuberculosis screening program in patients with inflammatory arthritides treated with golimumab, a human anti–tumor necrosis factor antibody, in Phase III clinical trials
Authors: Hsia Elizabeth C. , Cush John J. , Matteson Eric L. , Beutler Anna , Doyle Mittie K. , Hsu Benjamin , Xu Stephen , Rahman Mahboob U. 
Published: November 2012

AbstractObjectiveReactivation of Mycobacterium tuberculosis infection is a major complication in patients treated with anti–tumor necrosis factor (anti‐TNF) agents. We report on the 5 cases of active tuberculosis (TB) that developed in the Golimumab Phase III Program (3 with rheumatoid arthritis, 1 with psoriatic arthritis, and 1 with ankylosing spondylitis) through 1 year among 2,210 patients receiving golimumab.MethodsData from global studies were used for an in‐depth evaluation of the 5 cases of TB through week 52. Integrated safety data were evaluated for potential hepatotoxicity in patients treated with anti‐TB therapy.ResultsNo active TB developed among 317 patients receiving golimumab and treated for latent TB with isoniazid. Active TB occurred in 5 patients not treated with isoniazid by week 52 (in 2 patients by week 24); all of the patients had negative TB screening tests (per the local guidelines) and resided in countries with high background rates of TB. No deaths were due to TB. Across all of the groups (placebo and golimumab), alanine aminotransferase and aspartate aminotransferase elevations occurred in greater proportions of patients treated for latent TB infection versus not treated; elevations were largely mild (<3 times the upper limit of normal).ConclusionComprehensive TB screening kept the number of active TB cases relatively low despite conducting the studies in TB‐endemic regions. Treatment for latent TB infection appeared effective, since no patients treated for latent TB had TB reactivation. Concurrent treatment with golimumab and anti‐TB medication was generally well tolerated. Clinicians should remain vigilant for development of active TB after initiation of TNF inhibitors, since prompt diagnosis and treatment can improve outcomes.
Body mass index, obesity, and prevalent gout in the United States in 1988–1994 and 2007–2010
Authors: Juraschek Stephen P. , Miller Edgar R. , Gelber Allan C. 
Published: November 2012

AbstractObjectiveTo determine the association and prevalence of gout among overweight, obese, and morbidly obese segments of the US population.MethodsAmong participants (age ≥20 years) of the National Health and Nutrition Examination Surveys in 1988–1994 and 2007–2010, gout status was ascertained by self‐report of a physician diagnosis. Body mass index (BMI) was examined in categories of <18.5 kg/m2, 18.5–24.9 kg/m2, 25–29.9 kg/m2, 30–34.9 kg/m2, and ≥35 kg/m2 and as a continuous variable. The cross‐sectional association of BMI category with gout status was adjusted for demographic and obesity‐related medical disorders.ResultsIn the US, the crude prevalence of gout was 1–2% among participants with a normal BMI (18.5–24.9 kg/m2), 3% among overweight participants, 4–5% with class I obesity, and 5–7% with class II or class III obesity. The adjusted prevalence ratio comparing the highest to a normal BMI category was 2.46 (95% confidence interval [95% CI] 1.44–4.21) in 1988–1994 and 2.21 (95% CI 1.50–3.26) in 2007–2010. Notably, there was a progressively greater prevalence ratio of gout associated with successively higher categories of BMI. In both survey periods, for an average American adult standing 1.76 meters (5 feet 9 inches), a 1‐unit higher BMI, corresponding to 3.1 kg (∼6.8 pounds) greater weight, was associated with a 5% greater prevalence of gout, even after adjusting for serum uric acid (P < 0.001).ConclusionHealth care providers should be aware of the elevated burden of gout among both overweight and obese adults, applicable to both women and men, and observed among non‐Hispanic whites, non‐Hispanic African Americans, and Mexican Americans in the US.
Contribution of assessing forefoot joints in early rheumatoid arthritis patients: Insights from item response theory
Authors: Siemons Liseth , ten Klooster Peter M. , Taal Erik , Kuper Ina H. , van Riel Piet L. C. M. , Glas Cees A. W. , van de Laar Mart A. F. J. 
Published: November 2012

AbstractObjectiveTo evaluate the contribution of assessing forefoot joints to the measurement range and measurement precision of joint counts in early rheumatoid arthritis (RA) using item response theory.MethodsBaseline measures of tender and swollen joint counts were analyzed in 459 early RA patients from the Dutch Rheumatoid Arthritis Monitoring remission induction cohort. The contribution of forefoot joints was studied by evaluating their effect on the measurement range and measurement precision of measures based on 28‐joint counts. In addition, the alignment between the patient and joint distributions was investigated to determine whether the forefoot joints were informative for measuring joint tenderness or swelling of an early RA patient.ResultsIn total, 233 patients (50.76%) experienced tenderness and 200 patients (43.57%) experienced swelling in ≥1 forefoot joint. Forefoot joints were more informative for measuring joint tenderness than joint swelling, but did not significantly improve the measurement range and measurement precision of the 28‐joint counts. Furthermore, including forefoot joints did not remove the existing discrepancy between the joint and patient distributions in both joint counts.ConclusionForefoot joints were frequently affected on an individual level, but did not significantly improve the measurement range or precision of 28‐joint counts in patients with early RA. From a measurement perspective, reduced joint counts are appropriate for use on a population level. The contribution of assessing forefoot joints on an individual level requires further investigation. Additionally, the results should be cross‐validated in patients with longer disease durations to determine whether the pattern of joint involvement is similar in later stages of RA.
Serious infections in a population‐based cohort of 86,039 seniors with rheumatoid arthritis
Authors: Widdifield Jessica , Bernatsky Sasha , Paterson J. Michael , Gunraj Nadia , Thorne J. Carter , Pope Janet , Cividino Alfred , Bombardier Claire 
Published: November 2012

AbstractObjectiveTo assess risk and risk factors for serious infections in seniors with rheumatoid arthritis (RA) using a case–control study nested within an RA cohort.MethodsWe assembled a retrospective RA cohort age ≥66 years from Ontario health administrative data across 1992–2010. Nested case–control analyses were done, comparing RA patients with a primary diagnosis of infection (based on hospital or emergency department records) to matched RA controls. We assessed independent effects of drugs, adjusting for demographics, comorbidity, and markers of RA severity.ResultsA total of 86,039 seniors with RA experienced 20,575 infections, for a rate of 46.4 events/1,000 person‐years. The most frequently occurring events included respiratory infections, herpes zoster, and skin/soft tissue infections. Factors associated with infection included higher comorbidity, rural residence, markers of disease severity, and history of previous infection. In addition, anti–tumor necrosis factor agents and disease‐modifying antirheumatic drugs were associated with a several‐fold increase in infections, with an adjusted odds ratio (OR) ranging from 1.2–3.5. The drug category with the greatest effect estimate was glucocorticoids, which exhibited a clear dose response with an OR ranging from 4.0 at low doses to 7.6 at high doses.ConclusionSeniors with RA have significant morbidity related to serious infections, which exceeds previous reports among younger RA populations. Rural residence, higher comorbidity, markers of disease severity, and previous infection were associated with serious infections in seniors with RA. Our results emphasize that many RA drugs may increase the risk of infection, but glucocorticoids appear to confer a particular risk.
Silent cardiovascular involvement in patients with diffuse systemic sclerosis: a controlled cross‐sectional study
Authors: Turiel Maurizio , Gianturco Luigi , Ricci Cristian , Sarzi‐Puttini Piercarlo , Tomasoni Livio , Colonna Vito de Gennaro , Ferrario Paolo , Epis Oscar , Atzeni Fabiola 
Published: November 2012

AbstractObjectiveAn association between systemic autoimmune diseases and atherosclerosis has been described in many connective tissue diseases, and this association is known to lead to increased cardiovascular morbidity and mortality. Systemic sclerosis (SSc) is characterized by multisystem organ inflammation, endothelial wall damage, and vasculopathy. There are many markers of endothelial dysfunction and/or atherosclerotic risk, such as asymmetric dimethylarginine (ADMA), arterial stiffness parameters, carotid intima‐media thickness (CIMT), and coronary flow reserve (CFR) assessed by transthoracic echocardiography. The aim of this pilot study was to use various endothelial and atherosclerosis markers to identify early cardiovascular involvement in a group of SSc patients.MethodsThe study involved 20 patients (2 men and 18 women with a mean ± SD age of 52.96 ± 12.51 years) with diffuse SSc who had no signs or symptoms of cardiovascular disease (CVD) and 20 age‐ and sex‐matched controls. All subjects underwent a dipyridamole echocardiographic stress test that included a determination of CFR and an evaluation of CIMT, arterial stiffness, and plasma ADMA levels.ResultsAll of the arterial wall measurements of the patients with diffuse SSc were significantly different from those of the controls, and both right and left CIMT, pulse wave velocity, and stiffness index (β) were significantly elevated in the SSc patients compared to the healthy controls. Moreover, in patients with diffuse SSc, CFR was significantly lower (P = 0.0033) and plasma ADMA levels were higher (P < 0.0001) than in healthy controls.ConclusionSSc patients without any clinical evidence of CVD seem to have had subclinical atherosclerosis, which was suggested by early impairment of coronary microcirculation and macrovascular involvement.
Abatacept and reduced immune response to pandemic 2009 influenza A/H1N1 vaccination in patients with rheumatoid arthritis
Authors: Ribeiro Ana C. , Laurindo Ieda M. , Guedes Lissiane K. , Saad Carla G. , Moraes Julio C. , Silva Clóvis A. , Bonfa Eloisa 
Published: November 2012

AbstractObjectiveTo evaluate the influence of abatacept (ABA) and associated contributing factors on pandemic 2009 influenza A/H1N1 vaccine immunogenicity in rheumatoid arthritis (RA) patients.MethodsThe response to a nonadjuvanted monovalent pandemic 2009 influenza A/H1N1 killed virus vaccine was analyzed in 11 RA patients using ABA (RA‐ABA), most with concomitant nonbiologic disease‐modifying antirheumatic drugs (DMARDS), and compared to 33 age‐matched RA patients on methotrexate (MTX) and 55 healthy controls, all without previous seroprotection. Clinical and laboratory evaluations were performed before and 21 days after vaccination. Anti‐influenza antibody titers were measured by hemagglutination inhibition assay. Seroprotection (antibody titers ≥1:40) and the factor increase (FI) in the geometric mean titers (GMTs) were calculated. Prevaccination lymphocyte counts and gammaglobulin levels were determined.ResultsSex distribution, disease duration, and the Disease Activity Score in 28 joints were similar in the RA groups (P > 0.05). After vaccination, seroprotection was significantly reduced in RA‐ABA patients compared to RA‐MTX patients (9% versus 58%; P = 0.006) and controls (69%; P ≤ 0.001). FI‐GMT was severely reduced in RA‐ABA patients compared to RA‐MTX patients (1.8 [1.4–2.3] versus 8.7 [5.2–17.4]; P < 0.001) and controls (11.5 [8.0–16.7]; P ≤ 0.001). Lymphocyte counts were comparable in RA groups (P > 0.05), but RA‐ABA patients had slightly lower gammaglobulin levels than RA‐MTX patients (0.9 gm/dl [0.6–1.8] versus 1.2 gm/dl [0.8–1.7]; P = 0.03), although almost all were within the normal range values.ConclusionThe current study established that ABA, in association with traditional DMARDs, significantly reduces the humoral response to pandemic 2009 influenza A/H1N1 vaccine in RA patients. The results suggest an influence of costimulatory modulation in humoral response to this vaccine.
Speech and hearing in adults with 22q11.2 deletion syndrome
Authors: Persson Christina , Friman Vanda , Óskarsdóttir Sólveig , Jönsson Radi 
Published: November 2012

AbstractThe purpose of the study was to investigate the prevalence of velopharyngeal impairment, compensatory articulation, reduced intelligibility, and to rate the general impression of speech in adults with 22q11.2 deletion syndrome. The second purpose was to study the prevalence and type of hearing impairment in these adults. A referred, consecutive series of 24 adults with confirmed 22q11.2 deletion, 16 female and 8 males, with a mean age of 25 years (19–38 years) was included in the study. A blind assessment of speech by three experienced speech‐ language pathologists was performed. Sixteen (66%) patients had a mild to severe velopharyngeal impairment. The most prevalent symptoms of velopharygeal impairment were hypernasality and audible nasal airflow. The mean nasalance score was 33% (6–66%). Only two patients had disordered articulation; one of these had glottal articulation. A mean of 96% (88–100%) of single words were rated to be intelligible. To achieve these results half of the patients previously had velopharyngeal flap surgery. Forty‐one percent (9/22) had mild–moderate hearing impairment; three had sensorineural type, four conductive and two had a mixed type. In conclusion the majority of the patients had no articulation errors and good intelligibility; while one‐third still had moderate to severe problems with velopharyngeal impairment. Around 40% still had some hearing impairment, in most cases with a mild to moderate conductive component. Thus, a high prevalence of speech and hearing problems seems to be a part of the phenotype in adults with 22q11.2DS. © 2012 Wiley Periodicals, Inc.
An emerging phenotype of interstitial 15q25.2 microdeletions: Clinical report and review
Authors: Palumbo Orazio , Palumbo Pietro , Palladino Teresa , Stallone Raffaella , Miroballo Mattia , Piemontese Maria Rosaria , Zelante Leopoldo , Carella Massimo 
Published: November 2012

AbstractInterstitial deletions of chromosome 15q25.2 are rare. To date, only nine patients with microdeletions within this chromosomal region have been described. Here, we report on a girl with severe speech and psychomotor delay, behavioral problems and mild dysmorphic features with a 1.6 Mb deletion in 15q25.2 region. In order to study the parental origin of the rearrangement, we analyzed selected SNPs in the deleted area in the patient and her parents, showing Mendelian incompatibilities suggesting a de novo deletion on the chromosome of maternal origin. By comparing the clinical and molecular features of our patient with five previously reported cases of an overlapping deletion, we suggest that 15q25.2 deletion is an emerging syndrome characterized by a distinct although variable spectrum of clinical manifestations, including mild dysmorphic features, neurodevelopmental delay, and a recognizable pattern of congenital malformation. Furthermore, our patient is the second one in which a behavioral phenotype characterized by hyperactivity, anxiety, and autistic features was reported, indicating that these features might be part of this new syndromic condition. Breakpoints of the deletion in the patient reported here are useful to better define the smallest region of overlap (SRO) among all the patients. Selected genes that are present in the hemizygous state and which might be important for the phenotype of these patients, are discussed in context of the clinical features. In conclusion, our patient increases the knowledge about the molecular and phenotypic consequences of interstitial 15q25.2 deletions, highlighting that deletions of this region may be responsible for a new microdeletion syndrome. © 2012 Wiley Periodicals, Inc.
A 0.7 Mb de novo duplication at 7q21.3 including the genes DLX5 and DLX6 in a patient with split‐hand/split‐foot malformation
Authors: Velinov Milen , Ahmad Ausaf , Brown‐Kipphut Brigette , Shafiq Mustafa , Blau Jonathan , Cooma Ruby , Roth Philip , Iqbal M. Anwar 
Published: November 2012

AbstractSplit‐hand/split‐foot malformation (SHFM1) has been reported to be caused by deletions, duplications or rearrangements involving the 7q21.3 region harboring DSS1, DLX5, and DLX6. We report on a female patient with unilateral syndactyly of the third and fourth fingers of the right hand and overgrowth and lateral deviation of the right great toe. There was a split foot malformation on the right, with absent fifth toe. The left hand was apparently normal and left foot was intact. The patient has no hearing loss. We performed conventional G‐banding karyotype analysis, array comparative genomic hybridization (aCGH) and fluorescence in situ hybridization (FISH). G‐banding karyotype result was normal 46,XX. However, a duplication of 719 kb (96,303,736–97,022,335; NCBI build36/hg18, March 2006) was identified at the 7q21.3 region by aCGH. The array result was also confirmed by FISH analysis. The duplicated region harbors only DLX5 and DLX6, which are known for their role in SHFM1. Additionally, FISH analysis of parental samples showed de novo origin of this abnormality in the patient. This is the first report that highlights the duplication of 719 kb at 7q21.3, harboring only DLX5 and DLX6 associated with the SHFM1 phenotype. © 2012 Wiley Periodicals, Inc.
Hypersociability in the behavioral phenotype of 17q21.31 microdeletion syndrome
Authors: Egger Jos I.M. , Wingbermühle Ellen , Verhoeven Willem M.A. , Dijkman Marije , Radke Sina , de Bruijn Ellen R.A. , de Vries Bert , Kessels Roy P.C. , Koolen David 
Published: November 2012

AbstractThe 17q21.31 microdeletion syndrome with its characteristic features including developmental delay, moderate intellectual disability, facial dysmorphisms, and anomalies of the brain and multiple organ systems was recently described. As to its behavioral profile, scarce data from clinical observations have suggested a remarkably amiable, friendly disposition, to some extent comparable to that observed in Angelman and Williams syndromes. The present study focuses on the various aspects of neurocognitive functioning, particularly social cognition, in patients with 17q21.31 microdeletion syndrome. Neuropsychological assessment was performed in three out of the four known Dutch patients with a genetically proven 17q21.31 microdeletion syndrome. Apart from developmental age, cognition and social‐emotional functioning was extensively assessed. In addition, data of three intellectually disabled physically healthy reference subjects, recruited from a small outpatient sample, were included. The general cognitive profile of all subjects was in accordance with their lowered intellectual capacities, albeit that in patients with the 17q21.31 microdeletion, a relatively strong memory for social‐contextual information was found. Basic emotion perception was intact, but patients with the 17q21.31 microdeletion syndrome showed less social fear and more approaching behavior. Interestingly, alexithymic traits, that is marked difficulties in the recognition and expression of emotions, were more prevalent in reference subjects. Despite the methodological limitations characteristic for research in people with intellectual disabilities, with a neuropsychological assessment strategy, in three patients with 17q21.31 microdeletion syndrome, preliminary evidence for hypersocial behavior with a high level of frustration tolerance was found that may be implicated in its behavioral phenotype. © 2012 Wiley Periodicals, Inc.
Externe Regulation kontrollierter Polymerisationen
Authors: Leibfarth Frank A. , Mattson Kaila M. , Fors Brett P. , Collins Hazel A. , Hawker Craig J. 
Published: November 2012

AbstractDurch Fortschritte bei Verfahren zur kontrollierten Polymerisation und die Entwicklung verlässlicher Postfunktionalisierungsmethoden stehen Chemikern heute Werkzeuge zur Verfügung, um Materialien nahezu jeglicher Art und Architektur herzustellen. Viele wichtige Herausforderungen in den Materialwissenschaften verlangen jedoch nicht nur die Herstellung funktioneller Polymere, sondern darüber hinaus auch einen gezielten Zugang zu spezifischen Eigenschaften dieser Materialien. Eine beeindruckende zeitliche und räumliche Polymerisationskontrolle beobachten wir in der Natur, wo die Bildung von Proteinen, Nukleinsäuren und Polysacchariden zur Regulation von Mehrkomponentensystemen beiträgt und die Homöostase aufrechterhält. Hier geben wir einen Überblick über vorhandene Strategien zur zeitlichen Kontrolle von Polymerisationen durch externe Stimuli, wie z. B. chemische Reagentien, elektrische Spannung, Licht und mechanische Kraft. Neueste Arbeiten veranschaulichen das beträchtliche Potential dieses noch jungen Gebiets und liefern einen Satz von Kriterien für zukünftige Strategien zur Regulation kontrollierter Polymerisationen.
An Adaptive Coverage Control for Deployment of Nonholonomic Mobile Sensor Networks Over Time‐Varying Sensory Functions
Authors: Luna José Marcio , Fierro Rafael , Abdallah Chaouki T. , Wood John 
Published: November 2012

AbstractIn this paper we show the Lyapunov stability of an adaptive and decentralized coverage control for a team of mobile sensors. This new coverage approach assumes nonholonomic sensors rather than the holonomic ones usually found in the literature. Furthermore, sufficient conditions are provided to guarantee an ultimate bound for the system when in presence of time‐varying sensory functions. The convergence and feasibility of the coverage control are verified through simulation and experimental results.