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    Imaging findings in systemic childhood diseases presenting with dermatologic manifestations.
    Clin Imaging 2017 Oct 31;49:17-36. Epub 2017 Oct 31.
    Department of Radiology, Division of Pediatric Radiology, Montefiore Medical Center, Bronx, NY, United States. Electronic address:
    Purpose: Many childhood diseases often present with skin abnormalities with which radiologists are largely unfamiliar. Knowledge of associated dermatologic manifestations may aid the radiologist in confirming the diagnosis and recommending targeted imaging of affected organs.

    Methods: We review the imaging findings in childhood diseases associated with dermatologic manifestations. Read More

    Effect of intravenous immunoglobulin administration on erythrocyte and leukocyte parameters.
    J Eur Acad Dermatol Venereol 2017 Nov 8. Epub 2017 Nov 8.
    Department of Dermatology, Medical University of Vienna, Vienna, Austria.
    Background: Intravenous immunoglobulins (IVIG) are an attractive therapeutic tool for therapy of toxic epidermal necrolysis and severe forms of certain autoimmune diseases, including dermatomyositis, autoimmune blistering diseases, systemic vasculitis, and lupus erythematodes.

    Objective: Prompted by a case of IVIG-associated haemolytic anaemia, the effects of IVIG administrations on haematological parameters in patients with dermatological conditions were investigated.

    Methods: Erythrocyte and leucocyte parameters were retrospectively analysed in 16 patients who had received IVIG at doses from 1-3g per kg body weight (n=35 cycles). Read More

    Factors Associated With Clinical Remission of Skin Disease in Dermatomyositis.
    JAMA Dermatol 2017 Nov 7. Epub 2017 Nov 7.
    Stanford University School of Medicine, Department of Dermatology, Stanford, California.
    Importance: Cutaneous disease represents a significant burden for patients with dermatomyositis. However, quantitative estimates of the probability of skin disease remission and clinical factors associated with skin outcomes are lacking.

    Objective: To characterize cutaneous disease course in adult patients with dermatomyositis. Read More

    2017 European League Against Rheumatism/American College of Rheumatology Classification Criteria for Adult and Juvenile Idiopathic Inflammatory Myopathies and Their Major Subgroups.
    Arthritis Rheumatol 2017 Oct 27. Epub 2017 Oct 27.
    National Institute of Environmental Health Sciences, NIH, Bethesda, Maryland.
    Objective: To develop and validate new classification criteria for adult and juvenile idiopathic inflammatory myopathies (IIM) and their major subgroups.

    Methods: Candidate variables were assembled from published criteria and expert opinion using consensus methodology. Data were collected from 47 rheumatology, dermatology, neurology, and pediatric clinics worldwide. Read More

    Genetic Association of HLA-DRB1 Multiple Polymorphisms with Dermatomyositis in Chinese Population.
    HLA 2017 Nov 6. Epub 2017 Nov 6.
    Department of Rheumatology, China-Japan Friendship Hospital, Beijing, 100029, P. R. China.
    Genetic variation in human leukocyte antigen (HLA) plays an important role in the pathogenesis of dermatomyositis (DM). The aim of this study was to investigate the association of HLA class II with DM in China. 224 DM patients and 300 healthy controls were randomly enrolled at China-Japan Friendship Hospital. Read More

    Reexamining mechanic's hands as a characteristic skin finding in dermatomyositis.
    J Am Acad Dermatol 2017 Nov 1. Epub 2017 Nov 1.
    Corporal Michael J. Crescenz VAMC, Philadelphia, Pennsylvania, USA; Department of Dermatology, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA. Electronic address:
    Mechanic's hands is a poorly defined clinical finding that has been reported in a variety of rheumatologic diseases. Morphologic descriptions include hyperkeratosis on the sides of the digits that sometimes extends to the distal tips, diffuse palmar scale, and, more recently observed, linear discrete scaly papules in a similar lateral distribution. The association of mechanic's hands with dermatomyositis, although recognized, is still debatable. Read More

    Assessment of anti-MDA5 antibody as a diagnostic biomarker in patients with dermatomyositis-associated interstitial lung disease or rapidly progressive interstitial lung disease.
    Oncotarget 2017 Sep 6;8(44):76129-76140. Epub 2017 Jul 6.
    Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing, China.
    Anti-melanoma differentiation-associated protein 5 (MDA5) antibody have been found in dermatomyositis (DM)-associated interstitial lung disease (DM-ILD) and DM-associated rapidly progressive ILD (DM-RPILD). Due to the conflicting results regarding the association between anti-MDA5 antibody and DM-ILD or DM-RPILD and the diagnostic value of this antibody for DM-ILD and DM-RPILD, we performed this meta-analysis. A systematic search was performed to identify studies published to January 14, 2017. Read More

    The Serum Ferritin Level is Associated with the Treatment Responsivity for Rapidly Progressive Interstitial Lung Disease with Amyopathic Dermatomyositis, Irrespective of the Anti-MDA5 Antibody Level: A Case Report.
    Intern Med 2017 Nov 1. Epub 2017 Nov 1.
    Department of Respiratory Medicine, Fukujuji Hospital, Japan Anti-Tuberculosis Association, Japan.
    We herein report he case of a 61-year-old woman with rapidly progressive interstitial lung disease caused by clinically amyopathic dermatomyositis. Both the serum ferritin and anti-MDA5 antibody levels were elevated at the time of admission. Despite intensive treatment with corticosteroids, immunosuppressants, immunoglobulins and polymyxin B direct hemoperfusion, the patient died 75 days after symptom onset. Read More

    Cutaneous Manifestations of Dermatomyositis: a Comprehensive Review.
    Clin Rev Allergy Immunol 2017 Oct 31. Epub 2017 Oct 31.
    Division of Rheumatology and Clinical Immunology, Humanitas Research Hospital, Rozzano, Milan, Italy.
    Dermatomyositis (DM) is an idiopathic inflammatory myopathy characterized by the presence of skin lesions and inflammation of skeletal muscles; however, this feature may be absent in amyopathic DM. DM is a rare disease, occurring at any age, and has two peaks of incidence: one in childhood between 5 and 15 years of age and one in adulthood between 40 and 60 years, with a female preponderance. DM has been associated with malignancy; therefore, every newly diagnosed patient should undergo screening investigations, but evidence-based guidelines on their extension are lacking. Read More

    Breast cancer and dermatomyositis: a case study and literature review.
    Curr Oncol 2017 Oct 25;24(5):e429-e433. Epub 2017 Oct 25.
    Division of Hematology Oncology, Department of Medicine, St. Michael's Hospital, Toronto, ON.
    A 49-year-old woman presents with an extensive violaceous rash, rapidly progressive proximal muscle weakness, and dysphagia to solids, consistent with a diagnosis of dermatomyositis. Two weeks later, she palpates a mass in her left breast and is diagnosed with her2-positive metastatic invasive ductal carcinoma of the breast. There is a well-established association between dermatomyositis and malignancy. Read More

    Effective induction therapy for anti-SRP associated myositis in childhood: A small case series and review of the literature.
    Pediatr Rheumatol Online J 2017 Oct 31;15(1):77. Epub 2017 Oct 31.
    Rheumatology Unit, Great Ormond Street Hospital for Children, London, UK.
    Background: Anti-Signal Recognition Particle associated myopathy is a clinically and histopathologically distinct subgroup of Juvenile Idiopathic Inflammatory Myositis, which is under-recognised in children and fails to respond to conventional first line therapies. We present three cases where remission was successfully induced using combination therapy with intensive rehabilitation.

    Case Presentations: Three new patients are reported. Read More

    Productivity Losses and Costs in the Less-Common Systemic Autoimmune Rheumatic Diseases.
    Curr Rheumatol Rep 2017 Oct 30;19(11):72. Epub 2017 Oct 30.
    Arthritis Research Canada, Richmond, BC, Canada.
    Purpose Of Review: We synthesised the literature on productivity losses and costs in the less-common systemic autoimmune rheumatic diseases: Sjogren's syndrome (SjS), systemic sclerosis (SSc), poly/dermatomyositis (PM/DM), and systemic vasculitides (SV).

    Recent Findings: Of 29 studies located, 12 were published 2012 onwards (SSc = 6, SjS = 2, PM/DM = 2, SV = 2). In these, 25% of PM/DM, and 21-26% of SV, were work disabled, 22% of SSc stopped work within 3 years of diagnosis, and annual costs of absenteeism in SSc averaged $12,024 2017 USD. Read More

    Development of a consensus core dataset in juvenile dermatomyositis for clinical use to inform research.
    Ann Rheum Dis 2017 Oct 30. Epub 2017 Oct 30.
    Department of Paediatric Rheumatology, Alder Hey Children's NHS Foundation Trust, Liverpool, UK.
    Objectives: This study aimed to develop consensus on an internationally agreed dataset for juvenile dermatomyositis (JDM), designed for clinical use, to enhance collaborative research and allow integration of data between centres.

    Methods: A prototype dataset was developed through a formal process that included analysing items within existing databases of patients with idiopathic inflammatory myopathies. This template was used to aid a structured multistage consensus process. Read More

    2017 European League Against Rheumatism/American College of Rheumatology classification criteria for adult and juvenile idiopathic inflammatory myopathies and their major subgroups.
    Ann Rheum Dis 2017 12 27;76(12):1955-1964. Epub 2017 Oct 27.
    US Department of Health and Human Services, Environmental Autoimmunity Group, Clinical Research Branch, National Institute of Environmental Health Sciences, National Institutes of Health, Bethesda, Maryland, USA.
    Objective: To develop and validate new classification criteria for adult and juvenile idiopathic inflammatory myopathies (IIM) and their major subgroups.

    Methods: Candidate variables were assembled from published criteria and expert opinion using consensus methodology. Data were collected from 47 rheumatology, dermatology, neurology and paediatric clinics worldwide. Read More

    A Retrospective Medical Record Review of Utilization Patterns and Medical Resource Use Associated with Repository Corticotropin Injection among Patients with Rheumatologic Diseases in the United States.
    Rheumatol Ther 2017 Oct 25. Epub 2017 Oct 25.
    Mallinckrodt Pharmaceuticals, Bedminster, NJ, USA.
    Introduction: Repository corticotropin injection (RCI) has anti-inflammatory and immune-modulatory effects and is approved for multiple indications, including several rheumatologic conditions. The aims of this nationally representative, retrospective, observational study were to describe patient characteristics, RCI treatment patterns, and barriers to RCI use in patients with rheumatologic disease, and to compare medical resource use (MRU) before and after RCI therapy.

    Methods: A random sample of US physicians was recruited to abstract the medical records of deidentified patients with a diagnosis of rheumatoid arthritis (RA), psoriatic arthritis (PsA), dermatomyositis/polymyositis (DM/PM), or systemic lupus erythematosus (SLE) who had been treated with RCI in the previous 24 months. Read More

    Cutaneous dermatomyositis disease course followed over time using the Cutaneous Dermatomyositis Disease Area and Severity Index (CDASI).
    J Am Acad Dermatol 2017 Oct 21. Epub 2017 Oct 21.
    Background: Limited studies describe the longitudinal course of cutaneous dermatomyositis (DM).

    Objective: To characterize disease course in treated cutaneous DM using the Cutaneous Dermatomyositis Disease Area and Severity Index (CDASI), a validated outcome instrument.

    Methods: A retrospective cohort included patients with DM who had the CDASI activity subscore recorded for at least 2 years. Read More

    Clinical effects of direct hemoperfusion using a polymyxin B-immobilized fiber column in clinically amyopathic dermatomyositis-associated rapidly progressive interstitial pneumonias.
    BMC Pulm Med 2017 Oct 24;17(1):134. Epub 2017 Oct 24.
    Department of Respiratory Medicine, Kumamoto University Hospital, Faculty of Life Sciences, Kumamoto University, 1-1-1 Honjo, Chuo-ku, Kumamoto, 860-8556, Japan.
    Background: Rapidly progressive interstitial pneumonias (RPIPs) associated with clinically amyopathic dermatomyositis (CADM) are highly resistant to therapy and have a poor prognosis. Multimodal therapies, including direct hemoperfusion using a polymyxin B-immobilized fiber column (PMX-DHP), have a protective effect on RPIPs. We evaluated the effects of PMX-DHP on CADM-associated RPIPs. Read More

    The involvement of galectin-3 in skin injury in systemic lupus erythematosus patients.
    Lupus 2017 Jan 1:961203317736144. Epub 2017 Jan 1.
    1 Department of Dermatology, 56713 Sun Yat-sen Memorial Hospital , Guangzhou, China.
    Objective Our previous research suggested that anti-galectin-3 antibody was highly associated with the development of lupus skin lesions in systemic lupus erythematosus (SLE). In this study we aimed to investigate the involvement of galectin-3 in SLE skin damage. Methods The study consisted of 49 patients with SLE, 16 with dermatomyositis and 11 with systemic scleroderma and 20 healthy controls. Read More

    [Spontaneous pneumomediastinum: A rare complication of dermatomyositis].
    Rev Pneumol Clin 2017 Oct 18;73(5):258-262. Epub 2017 Oct 18.
    Service de médecine interne, CHU Ibn Rochd, FMPC-université Hassan II, Casablanca, Maroc.
    Dermatomyositis is a rare connective tissue disease of unknown origin, including inflammatory myopathy and cutaneous manifestations. Several pulmonary complications associated to dermatomyositis were described; especially interstitial lung disease. Some rare and particular pulmonary complications were reported in the literature such as pneumodiastinum and pneumothorax. Read More

    [Lung transplantation for connective tissue disease-associated interstitial lung disease recipient].
    Zhonghua Jie He He Hu Xi Za Zhi 2017 Oct;40(10):744-748
    Transplantation Center of Wuxi People's Hospital Affiliated to Nanjing Medical University, Wuxi 214023, China.
    Objective: To investigate the timing and complications of lung transplantation for the treatment of connective tissue disease-associated interstitial lung disease (CTD-ILD). Method: The clinical data of connective tissue-associated disease lung transplantation recipients from September, 2015 to February, 2017 were collected. Results: 11 patients with CTD-ILD were evaluated by lung transplantation and were treated with lung transplantation. Read More

    [Changes of serum Krebs von den Lungen-6 levels in interstitial lung disease associated with dermatomyositis and secondary Sjögren's syndrome: a case report].
    Beijing Da Xue Xue Bao Yi Xue Ban 2017 Oct;49(5):910-914
    Department of Rheumatology and Immunology, Peking University People's Hospital, Beijing 100044, China.
    Interstitial lung diseases (ILDs) are a diverse group of pulmonary disorders characterized by various patterns of inflammation and fibrosis in the interstitium of the lung. The underlying pathogenesis of ILDs is complex and associated with multiple rheumatologic conditions, such as systemic sclerosis, rheumatoid arthritis, pollymyositis and dermatomyositis, Sjögren's syndrome, and systemic lupus erythematosus. As the disease progresses, excessive pulmonary fibrosis impairs alveolar gas exchange and damages pulmonary function. Read More

    Trajectories of cardiorespiratory fitness in patients with juvenile dermatomyositis.
    Rheumatology (Oxford) 2017 Sep 27. Epub 2017 Sep 27.
    Child Development and Exercise Centre, Wilhelmina Children's Hospital, University Medical Centre Utrecht.
    Objectives: Previous research demonstrated decreased cardiorespiratory fitness (CRF) in patients with JDM during active disease and remission. However, longitudinal data regarding trajectories of CRF are currently lacking. The objective of this study was to determine trajectories of CRF in patients with both monocyclic and chronic JDM, and to identify potential predictors of these trajectories. Read More

    Lipid-lowering agent-triggered dermatomyositis and polymyositis: a case series and literature review.
    Rheumatol Int 2017 Oct 12. Epub 2017 Oct 12.
    Division of Rheumatology, Faculdade de Medicina FMUSP, Universidade de Sao Paulo, Sao Paulo, SP, Brazil.
    Lipid-lowering agent-triggered dermatomyositis (DM) or polymyositis (PM) is a rare event. Therefore, the aim of the present study was to describe a series of such cases. A retrospective cohort study of 5 DM and 4 PM cases triggered by prior exposure to lipid-lowering agents between 2001 and 2017 was carried out. Read More

    Cardiopulmonary Manifestations of Collagen Vascular Diseases.
    Curr Rheumatol Rep 2017 Oct 9;19(11):71. Epub 2017 Oct 9.
    Division of Diagnostic Radiology, Cardiothoracic Imaging Section, Mallinckrodt Institute of Radiology, Washington University in St. Louis, Campus Box 8131, 510 S Kingshighway Blvd, St. Louis, MO, USA.
    Purpose Of Review: The study aimed to illustrate the cardiopulmonary findings of the following collagen vascular diseases on cross-sectional imaging: rheumatoid arthritis, scleroderma (progressive systemic sclerosis), systemic lupus erythematosus, the inflammatory myopathies (polymyositis/dermatomyositis), and Sjögren's syndrome.

    Recent Findings: Although collagen vascular diseases can affect any part of the body, interstitial lung disease and pulmonary hypertension are the two most important cardiopulmonary complications and are responsible for the majority of morbidity and mortality in this patient population. Interstitial pneumonia with autoimmune features (IPAF) is a newly described entity that encompasses interstitial lung disease in patients with clinical, serologic, or morphologic features suggestive of but not diagnostic of collagen vascular disease; these patients are thought to have better outcomes than idiopathic interstitial pneumonias. Read More

    Abatacept in the treatment of adult dermatomyositis and polymyositis: a randomised, phase IIb treatment delayed-start trial.
    Ann Rheum Dis 2017 Oct 9. Epub 2017 Oct 9.
    Rheumatology Unit, Department of Medicine, Karolinska University Hospital Solna, Karolinska Institutet, Stockholm, Sweden.
    Objectives: To study the effects of abatacept on disease activity and on muscle biopsy features of adult patients with dermatomyositis (DM) or polymyositis (PM).

    Methods: Twenty patients with DM (n=9) or PM (n=11) with refractory disease were enrolled in a randomised treatment delayed-start trial to receive either immediate active treatment with intravenous abatacept or a 3 month delayed-start. The primary endpoint was number of responders, defined by the International Myositis Assessment and Clinical Studies Group definition of improvement (DOI), after 6 months of treatment. Read More

    Antimalarial drug toxicities in patients with cutaneous lupus and dermatomyositis: A retrospective cohort study.
    J Am Acad Dermatol 2017 Oct 6. Epub 2017 Oct 6.
    Corporal Michael J. Crescenz Veterans Affairs Medical Center, Philadelphia, Pennsylvania; Department of Dermatology, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania. Electronic address:
    Background: Although existing evidence demonstrates the efficacy of antimalarials for rheumatic skin disease, the safety of these medications, and particularly quinacrine, remains debated.

    Objective: We investigated the toxicity risk associated with antimalarials in patients with cutaneous lupus erythematosus and dermatomyositis.

    Methods: A total of 532 patients (mean age, 52. Read More

    Associations between anti-melanoma differentiation-associated gene 5 antibody and demographics, clinical characteristics and laboratory results of patients with dermatomyositis: A systematic meta-analysis.
    J Dermatol 2017 Oct 6. Epub 2017 Oct 6.
    Department of Rheumatology, Huai'an First People's Hospital, Nanjing Medical University, Jiangsu, China.
    Anti-melanoma differentiation-associated gene 5 (MDA5) antibody is a specific biomarker in patients with dermatomyositis (DM). Results from several studies that examined the relationship between anti-MDA5 antibody and the demographics, clinical characteristics and laboratory results of DM patients have been conflicting. The purpose of this study was to identify the relationship, if any, of anti-MDA5 antibody with demographics, clinical characteristics and laboratory results of DM patients. Read More

    2016 ACR-EULAR adult dermatomyositis and polymyositis and juvenile dermatomyositis response criteria-methodological aspects.
    Rheumatology (Oxford) 2017 Nov;56(11):1884-1893
    Department of Medicine, Division of Rheumatology and Clinical Immunology, University of Pittsburgh, Pittsburgh, PA, USA.
    Objective: The objective was to describe the methodology used to develop new response criteria for adult DM/PM and JDM.

    Methods: Patient profiles from prospective natural history data and clinical trials were rated by myositis specialists to develop consensus gold-standard ratings of minimal, moderate and major improvement. Experts completed a survey regarding clinically meaningful improvement in the core set measures (CSM) and a conjoint-analysis survey (using 1000Minds software) to derive relative weights of CSM and candidate definitions. Read More

    Risk factors for the recurrence of interstitial lung disease in patients with polymyositis and dermatomyositis: a retrospective cohort study.
    Clin Rheumatol 2017 Oct 3. Epub 2017 Oct 3.
    Division of Rheumatology, Department of Internal Medicine, Keio University School of Medicine, 35 Shinanomachi, Shinjuku-ku, Tokyo, Japan.
    To identify risk factors for the recurrence of interstitial lung disease (ILD) in patients with polymyositis (PM)/dermatomyositis (DM). Forty-four PM/DM-ILD patients who had been treated with glucocorticoid and/or immunosuppressive agents as a remission induction therapy were enrolled. The patients were first classified into two groups: the early recurrence group that recurred within 52 weeks, and the non-early recurrence group, which was further classified into the late recurrence group that recurred after 52 weeks, and the non-recurrence group. Read More

    Safety of Intraventricular rt-PA for Pan-Ventricular IVH Caused by a Ruptured AVM: A Case Report.
    Neurohospitalist 2017 Oct 31;7(4):NP5-NP8. Epub 2017 Jan 31.
    Department of Neurology, University of Miami Miller School of Medicine, Miami, FL, USA.
    Intraventricular recombinant tissue plasminogen activator (IVT rt-PA) has improved outcomes for intraventricular hemorrhage (IVH). Patients with suspected or untreated arteriovenous malformations (AVMs) have been excluded from clinical trials. We present a patient with IVH secondary to a ruptured AVM safely treated with IVT rt-PA. Read More

    A case of clinically amyopathic dermatomyositis that developed during anti-TNF-α therapy for rheumatoid arthritis.
    Allergol Int 2017 Sep 27. Epub 2017 Sep 27.
    Division of Medical Oncology and Molecular Respirology, Department of Multidisciplinary Internal Medicine, Faculty of Medicine, Tottori University, Yonago, Japan.

    Amyopathic dermatomyositis with plantar keratoderma responding to methotrexate therapy.
    Cutis 2017 Aug;100(2):E20-E24
    Department of Dermatology, Loma Linda University, California, USA.
    Amyopathic dermatomyositis (ADM) represents a substantial subset of dermatomyositis (DM). Patients with this symptom of the disorder may present initially to a dermatologist. Amyopathic dermatomyositis shares cutaneous findings with DM and both overlap and differ with respect to other key disease characteristics, including autoantibody profile, associated lung disease, and malignancy risk. Read More

    Shiitake Dermatitis-like Eruption Due to Tegafur/Gimeracil/Oteracil Combination Usage
    Yonago Acta Med 2017 09 15;60(3):179-181. Epub 2017 Sep 15.
    Division of Dermatology, Department of Medicine of Sensory and Motor Organs, School of Medicine, Tottori University Faculty of Medicine, Yonago 683-8504, Japan.
    S-1 is a combination drug of tegafur, gimeracil and oteracil potassium that is designed on the basis of 5- fluorouracil. We report here for the first time that S-1 is a causative agent of drug eruption mimicking shiitake dermatitis. A 58-year-old Japanese man presented with pruritic erythemas arranged in a linear fashion. Read More

    Presentations and outcomes of juvenile dermatomyositis patients admitted to intensive care units.
    Rheumatology (Oxford) 2017 Oct;56(10):1814-1816
    Service d'Immunologie, Hématologie et Rhumatologie Pédiatrique, Centre de Référence National des Maladies Rhumatologiques et Inflammatoires Pédiatriques (CERHUMIP), Hôpital Necker - Enfants Malades, AP-HP Paris.

    Bilateral leg ulcers secondary to dystrophic calcinosis in a patient with rheumatoid arthritis.
    J Med Invest 2017 ;64(3.4):308-310
    Department of Dermatology, Tokushima University Graduate School of Medical Science.
    Calcinosis cutis can be classified into four subtypes: dystrophic, metastatic, idiopathic, and iatrogenic. Of these subtypes, dystrophic calcinosis (DC) is the most common, and is most frequently associated with connective tissue disease, particularly dermatomyositis and systemic sclerosis, and less commonly with systemic lupus erythematosus. However, DC associated with rheumatoid arthritis (RA) is extremely rare. Read More

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