20 results on '"R. Trachtman"'
Search Results
2. Mapping Systemic Inflammation and Antibody Responses in Multisystem Inflammatory Syndrome in Children (MIS-C).
- Author
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Gruber CN, Patel RS, Trachtman R, Lepow L, Amanat F, Krammer F, Wilson KM, Onel K, Geanon D, Tuballes K, Patel M, Mouskas K, O'Donnell T, Merritt E, Simons NW, Barcessat V, Del Valle DM, Udondem S, Kang G, Agashe C, Karekar N, Grabowska J, Nie K, Le Berichel J, Xie H, Beckmann N, Gangadharan S, Ofori-Amanfo G, Laserson U, Rahman A, Kim-Schulze S, Charney AW, Gnjatic S, Gelb BD, Merad M, and Bogunovic D
- Published
- 2023
- Full Text
- View/download PDF
3. Resilience and its associations in children with Systemic Lupus Erythematosus and Juvenile Idiopathic Arthritis.
- Author
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Trachtman R, Samuels J, Wojtal E, and Feldman BM
- Subjects
- Humans, Child, Quality of Life, Longitudinal Studies, Surveys and Questionnaires, Arthritis, Juvenile diagnosis, Lupus Erythematosus, Systemic complications
- Abstract
Background: Resilience has been shown to be associated with better psychological outcomes and ability to cope with negative and traumatic events in the healthcare setting. Therefore, in this study, we aimed to evaluate resilience and its association with disease activity and health-related quality of life (HRQOL) in children with Systemic Lupus Erythematosus (SLE) and Juvenile Idiopathic Arthritis (JIA)., Findings: Patients with diagnoses of SLE or JIA were recruited. We collected: demographic data, medical history and physical examination, physician and patient global health assessments, Patient Reported Outcome Measurement Information System questionnaires, Connor Davidson Resilience Scale 10 (CD-RISC 10), Systemic Lupus Erythematosus Disease Activity Index, and clinical Juvenile Arthritis Disease Activity Score 10. Descriptive statistics were calculated, and PROMIS raw scores were converted to T-scores. Spearman's correlations were performed, with statistical significance set to p < 0.05. 47 study subjects were recruited. The average CD-RISC 10 score in SLE was 24.4, and in JIA was 25.2. In children with SLE, CD-RISC 10 was correlated with disease activity and inversely correlated with anxiety. In children with JIA, resilience was inversely associated with fatigue, and positively correlated with mobility and peer relationships., Conclusions: In children with SLE and JIA, resilience is lower than in the general population. Further, our results suggest that interventions to increase resilience may improve the HRQOL of children with rheumatic disease. Ongoing study of the importance of resilience in this population, as well as interventions to increase resilience, will be an important area of future research in children with SLE and JIA., (© 2023. The Author(s).)
- Published
- 2023
- Full Text
- View/download PDF
4. Autoimmunity in Down's syndrome via cytokines, CD4 T cells and CD11c + B cells.
- Author
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Malle L, Patel RS, Martin-Fernandez M, Stewart OJ, Philippot Q, Buta S, Richardson A, Barcessat V, Taft J, Bastard P, Samuels J, Mircher C, Rebillat AS, Maillebouis L, Vilaire-Meunier M, Tuballes K, Rosenberg BR, Trachtman R, Casanova JL, Notarangelo LD, Gnjatic S, Bush D, and Bogunovic D
- Subjects
- Humans, Autoantibodies immunology, B-Lymphocytes cytology, B-Lymphocytes immunology, B-Lymphocytes metabolism, Disease Susceptibility, Interleukin-6 immunology, Receptors, Complement 3d, Autoimmunity, CD4-Positive T-Lymphocytes cytology, CD4-Positive T-Lymphocytes immunology, Cytokines analysis, Cytokines immunology, Down Syndrome immunology, Down Syndrome physiopathology
- Abstract
Down's syndrome (DS) presents with a constellation of cardiac, neurocognitive and growth impairments. Individuals with DS are also prone to severe infections and autoimmunity including thyroiditis, type 1 diabetes, coeliac disease and alopecia areata
1,2 . Here, to investigate the mechanisms underlying autoimmune susceptibility, we mapped the soluble and cellular immune landscape of individuals with DS. We found a persistent elevation of up to 22 cytokines at steady state (at levels often exceeding those in patients with acute infection) and detected basal cellular activation: chronic IL-6 signalling in CD4 T cells and a high proportion of plasmablasts and CD11c+ Tbethigh CD21low B cells (Tbet is also known as TBX21). This subset is known to be autoimmune-prone and displayed even greater autoreactive features in DS including receptors with fewer non-reference nucleotides and higher IGHV4-34 utilization. In vitro, incubation of naive B cells in the plasma of individuals with DS or with IL-6-activated T cells resulted in increased plasmablast differentiation compared with control plasma or unstimulated T cells, respectively. Finally, we detected 365 auto-antibodies in the plasma of individuals with DS, which targeted the gastrointestinal tract, the pancreas, the thyroid, the central nervous system, and the immune system itself. Together, these data point to an autoimmunity-prone state in DS, in which a steady-state cytokinopathy, hyperactivated CD4 T cells and ongoing B cell activation all contribute to a breach in immune tolerance. Our findings also open therapeutic paths, as we demonstrate that T cell activation is resolved not only with broad immunosuppressants such as Jak inhibitors, but also with the more tailored approach of IL-6 inhibition., (© 2023. The Author(s), under exclusive licence to Springer Nature Limited.)- Published
- 2023
- Full Text
- View/download PDF
5. 2021 American College of Rheumatology Guideline for the Treatment of Juvenile Idiopathic Arthritis: Recommendations for Nonpharmacologic Therapies, Medication Monitoring, Immunizations, and Imaging.
- Author
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Onel KB, Horton DB, Lovell DJ, Shenoi S, Cuello CA, Angeles-Han ST, Becker ML, Cron RQ, Feldman BM, Ferguson PJ, Gewanter H, Guzman J, Kimura Y, Lee T, Murphy K, Nigrovic PA, Ombrello MJ, Rabinovich CE, Tesher M, Twilt M, Klein-Gitelman M, Barbar-Smiley F, Cooper AM, Edelheit B, Gillispie-Taylor M, Hays K, Mannion ML, Peterson R, Flanagan E, Saad N, Sullivan N, Szymanski AM, Trachtman R, Turgunbaev M, Veiga K, Turner AS, and Reston JT
- Subjects
- Glucocorticoids therapeutic use, Humans, Immunization, Quality of Life, United States, Antirheumatic Agents therapeutic use, Arthritis, Juvenile drug therapy, Arthritis, Juvenile therapy, Rheumatology, Uveitis drug therapy
- Abstract
Objective: To provide recommendations for the management of juvenile idiopathic arthritis (JIA) with a focus on nonpharmacologic therapies, medication monitoring, immunizations, and imaging, irrespective of JIA phenotype., Methods: We developed clinically relevant Patient/Population, Intervention, Comparison, and Outcomes questions. After conducting a systematic literature review, the Grading of Recommendations Assessment, Development and Evaluation approach was used to rate the quality of evidence (high, moderate, low, or very low). A Voting Panel including clinicians and patients/caregivers achieved consensus on the direction (for or against) and strength (strong or conditional) of recommendations., Results: Recommendations in this guideline include the use of physical therapy and occupational therapy interventions; a healthy, well-balanced, age-appropriate diet; specific laboratory monitoring for medications; widespread use of immunizations; and shared decision-making with patients/caregivers. Disease management for all patients with JIA is addressed with respect to nonpharmacologic therapies, medication monitoring, immunizations, and imaging. Evidence for all recommendations was graded as low or very low in quality. For that reason, more than half of the recommendations are conditional., Conclusion: This clinical practice guideline complements the 2019 American College of Rheumatology JIA and uveitis guidelines, which addressed polyarthritis, sacroiliitis, enthesitis, and uveitis, and a concurrent 2021 guideline on oligoarthritis, temporomandibular arthritis, and systemic JIA. It serves as a tool to support clinicians, patients, and caregivers in decision-making. The recommendations take into consideration the severity of both articular and nonarticular manifestations as well as patient quality of life. Although evidence is generally low quality and many recommendations are conditional, the inclusion of caregivers and patients in the decision-making process strengthens the relevance and applicability of the guideline. It is important to remember that these are recommendations. Clinical decisions, as always, should be made by the treating clinician and patient/caregiver., (© 2022 American College of Rheumatology.)
- Published
- 2022
- Full Text
- View/download PDF
6. 2021 American College of Rheumatology Guideline for the Treatment of Juvenile Idiopathic Arthritis: Therapeutic Approaches for Oligoarthritis, Temporomandibular Joint Arthritis, and Systemic Juvenile Idiopathic Arthritis.
- Author
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Onel KB, Horton DB, Lovell DJ, Shenoi S, Cuello CA, Angeles-Han ST, Becker ML, Cron RQ, Feldman BM, Ferguson PJ, Gewanter H, Guzman J, Kimura Y, Lee T, Murphy K, Nigrovic PA, Ombrello MJ, Rabinovich CE, Tesher M, Twilt M, Klein-Gitelman M, Barbar-Smiley F, Cooper AM, Edelheit B, Gillispie-Taylor M, Hays K, Mannion ML, Peterson R, Flanagan E, Saad N, Sullivan N, Szymanski AM, Trachtman R, Turgunbaev M, Veiga K, Turner AS, and Reston JT
- Subjects
- Glucocorticoids therapeutic use, Humans, Quality of Life, Temporomandibular Joint, United States, Antirheumatic Agents therapeutic use, Arthritis, Juvenile diagnosis, Arthritis, Juvenile drug therapy, Rheumatology, Temporomandibular Joint Disorders diagnosis, Temporomandibular Joint Disorders drug therapy, Uveitis drug therapy
- Abstract
Objective: To provide updated guidelines for pharmacologic management of juvenile idiopathic arthritis (JIA), focusing on treatment of oligoarthritis, temporomandibular joint (TMJ) arthritis, and systemic JIA with and without macrophage activation syndrome. Recommendations regarding tapering and discontinuing treatment in inactive systemic JIA are also provided., Methods: We developed clinically relevant Patient/Population, Intervention, Comparison, and Outcomes questions. After conducting a systematic literature review, the Grading of Recommendations Assessment, Development and Evaluation approach was used to rate the quality of evidence (high, moderate, low, or very low). A Voting Panel including clinicians and patients/caregivers achieved consensus on the direction (for or against) and strength (strong or conditional) of recommendations., Results: Similar to those published in 2019, these JIA recommendations are based on clinical phenotypes of JIA, rather than a specific classification schema. This guideline provides recommendations for initial and subsequent treatment of JIA with oligoarthritis, TMJ arthritis, and systemic JIA as well as for tapering and discontinuing treatment in subjects with inactive systemic JIA. Other aspects of disease management, including factors that influence treatment choice and medication tapering, are discussed. Evidence for all recommendations was graded as low or very low in quality. For that reason, more than half of the recommendations are conditional., Conclusion: This clinical practice guideline complements the 2019 American College of Rheumatology JIA and uveitis guidelines, which addressed polyarthritis, sacroiliitis, enthesitis, and uveitis. It serves as a tool to support clinicians, patients, and caregivers in decision-making. The recommendations take into consideration the severity of both articular and nonarticular manifestations as well as patient quality of life. Although evidence is generally low quality and many recommendations are conditional, the inclusion of caregivers and patients in the decision-making process strengthens the relevance and applicability of the guideline. It is important to remember that these are recommendations. Clinical decisions, as always, should be made by the treating clinician and patient/caregiver., (© 2022 American College of Rheumatology.)
- Published
- 2022
- Full Text
- View/download PDF
7. 2021 American College of Rheumatology Guideline for the Treatment of Juvenile Idiopathic Arthritis: Recommendations for Nonpharmacologic Therapies, Medication Monitoring, Immunizations, and Imaging.
- Author
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Onel KB, Horton DB, Lovell DJ, Shenoi S, Cuello CA, Angeles-Han ST, Becker ML, Cron RQ, Feldman BM, Ferguson PJ, Gewanter H, Guzman J, Kimura Y, Lee T, Murphy K, Nigrovic PA, Ombrello MJ, Rabinovich CE, Tesher M, Twilt M, Klein-Gitelman M, Barbar-Smiley F, Cooper AM, Edelheit B, Gillispie-Taylor M, Hays K, Mannion ML, Peterson R, Flanagan E, Saad N, Sullivan N, Szymanski AM, Trachtman R, Turgunbaev M, Veiga K, Turner AS, and Reston JT
- Subjects
- Glucocorticoids therapeutic use, Humans, Immunization, Quality of Life, United States, Antirheumatic Agents therapeutic use, Arthritis, Juvenile drug therapy, Arthritis, Juvenile therapy, Rheumatology, Uveitis drug therapy
- Abstract
Objective: To provide recommendations for the management of juvenile idiopathic arthritis (JIA) with a focus on nonpharmacologic therapies, medication monitoring, immunizations, and imaging, irrespective of JIA phenotype., Methods: We developed clinically relevant Patient/Population, Intervention, Comparison, and Outcomes questions. After conducting a systematic literature review, the Grading of Recommendations Assessment, Development and Evaluation approach was used to rate the quality of evidence (high, moderate, low, or very low). A Voting Panel including clinicians and patients/caregivers achieved consensus on the direction (for or against) and strength (strong or conditional) of recommendations., Results: Recommendations in this guideline include the use of physical therapy and occupational therapy interventions; a healthy, well-balanced, age-appropriate diet; specific laboratory monitoring for medications; widespread use of immunizations; and shared decision-making with patients/caregivers. Disease management for all patients with JIA is addressed with respect to nonpharmacologic therapies, medication monitoring, immunizations, and imaging. Evidence for all recommendations was graded as low or very low in quality. For that reason, more than half of the recommendations are conditional., Conclusion: This clinical practice guideline complements the 2019 American College of Rheumatology JIA and uveitis guidelines, which addressed polyarthritis, sacroiliitis, enthesitis, and uveitis, and a concurrent 2021 guideline on oligoarthritis, temporomandibular arthritis, and systemic JIA. It serves as a tool to support clinicians, patients, and caregivers in decision-making. The recommendations take into consideration the severity of both articular and nonarticular manifestations as well as patient quality of life. Although evidence is generally low quality and many recommendations are conditional, the inclusion of caregivers and patients in the decision-making process strengthens the relevance and applicability of the guideline. It is important to remember that these are recommendations. Clinical decisions, as always, should be made by the treating clinician and patient/caregiver., (© 2022 American College of Rheumatology.)
- Published
- 2022
- Full Text
- View/download PDF
8. 2021 American College of Rheumatology Guideline for the Treatment of Juvenile Idiopathic Arthritis: Therapeutic Approaches for Oligoarthritis, Temporomandibular Joint Arthritis, and Systemic Juvenile Idiopathic Arthritis.
- Author
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Onel KB, Horton DB, Lovell DJ, Shenoi S, Cuello CA, Angeles-Han ST, Becker ML, Cron RQ, Feldman BM, Ferguson PJ, Gewanter H, Guzman J, Kimura Y, Lee T, Murphy K, Nigrovic PA, Ombrello MJ, Rabinovich CE, Tesher M, Twilt M, Klein-Gitelman M, Barbar-Smiley F, Cooper AM, Edelheit B, Gillispie-Taylor M, Hays K, Mannion ML, Peterson R, Flanagan E, Saad N, Sullivan N, Szymanski AM, Trachtman R, Turgunbaev M, Veiga K, Turner AS, and Reston JT
- Subjects
- Humans, Quality of Life, Temporomandibular Joint, United States, Arthritis, Juvenile drug therapy, Rheumatology, Temporomandibular Joint Disorders drug therapy, Uveitis drug therapy
- Abstract
Objective: To provide updated guidelines for pharmacologic management of juvenile idiopathic arthritis (JIA), focusing on treatment of oligoarthritis, temporomandibular joint (TMJ) arthritis, and systemic JIA with and without macrophage activation syndrome. Recommendations regarding tapering and discontinuing treatment in inactive systemic JIA are also provided., Methods: We developed clinically relevant Patient/Population, Intervention, Comparison, and Outcomes questions. After conducting a systematic literature review, the Grading of Recommendations Assessment, Development and Evaluation approach was used to rate the quality of evidence (high, moderate, low, or very low). A Voting Panel including clinicians and patients/caregivers achieved consensus on the direction (for or against) and strength (strong or conditional) of recommendations., Results: Similar to those published in 2019, these JIA recommendations are based on clinical phenotypes of JIA, rather than a specific classification schema. This guideline provides recommendations for initial and subsequent treatment of JIA with oligoarthritis, TMJ arthritis, and systemic JIA as well as for tapering and discontinuing treatment in subjects with inactive systemic JIA. Other aspects of disease management, including factors that influence treatment choice and medication tapering, are discussed. Evidence for all recommendations was graded as low or very low in quality. For that reason, more than half of the recommendations are conditional., Conclusion: This clinical practice guideline complements the 2019 American College of Rheumatology JIA and uveitis guidelines, which addressed polyarthritis, sacroiliitis, enthesitis, and uveitis. It serves as a tool to support clinicians, patients, and caregivers in decision-making. The recommendations take into consideration the severity of both articular and nonarticular manifestations as well as patient quality of life. Although evidence is generally low quality and many recommendations are conditional, the inclusion of caregivers and patients in the decision-making process strengthens the relevance and applicability of the guideline. It is important to remember that these are recommendations. Clinical decisions, as always, should be made by the treating clinician and patient/caregiver., (© 2022 American College of Rheumatology.)
- Published
- 2022
- Full Text
- View/download PDF
9. Response to a Letter to the Editor.
- Author
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Trachtman R
- Abstract
Competing Interests: The authors declare no conflict of interest.
- Published
- 2021
- Full Text
- View/download PDF
10. Review article: Emerging issues in pediatric skin of color, part 1.
- Author
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Mitchell KN, Tay YK, Heath CR, Trachtman R, and Silverberg NB
- Subjects
- Child, Humans, Alopecia, Hair, Skin, Racial Groups, Ethnicity, Skin Diseases diagnosis, Skin Pigmentation
- Abstract
Dermatology for the pediatric skin of color population is the application of dermatology to the genetically diverse and distinctive segment of the pediatric population that includes children of non-White racial and ethnic groups with increased pigmentation including individuals of Asian, LatinX, African, Native American, Pacific Island descent, Indigenous Peoples, among others, with overlap in particular individuals, and mixtures thereof. Treating children of color is a unique skill set within the field of pediatric dermatology, requiring knowledge and sensitivity. The discipline of pediatric skin of color can be challenging. Difficulty in diagnosis of common conditions stems from underlying pigmentation, variations in common hairstyling practices, and differences in demographics of cutaneous disease, whereas some conditions are more common in children of color, other conditions have nuances in clinical appearance and/or therapeutics with regard to skin color. This article is the first in a series of two articles looking at recently published skin-related issues of high concern in children of color. Conditions reviewed in Part 1 include (1) hairstyling hair-related concerns (traction alopecia, central centrifugal cicatricial alopecia, endocrine disruption), (2) autoimmune concerns (cutaneous lupus, vitiligo), and (3) infections (tinea capitis, progressive macular hypomelanosis)., (© 2021 Wiley Periodicals LLC.)
- Published
- 2021
- Full Text
- View/download PDF
11. The value of the patient global health assessment in polyarticular juvenile idiopathic arthritis: a nested cohort study.
- Author
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Trachtman R, Issa R, Pan S, Wilson KM, Lovell DJ, and Onel KB
- Abstract
Objective: The objectives were: 1) to explore the discordance between the Patient Global Health Assessment (PtGA) scores, the Physician Global Health Assessment (PhGA) scores, and Pain scores; and 2) to explore whether the PtGA during disease remission is associated with future disease flare in pJIA., Methods: Data from an NIH funded clinical trial (NCT00792233) evaluating flare were used (N = 137). PtGA, PhGA, and Pain scores were assessed. Flare was defined as any active arthritis. Spearman's correlation coefficients were calculated, and multivariable logistic regression was performed., Results: 122 patients had records of flare status, of which 63 developed flare, and 42 of these patients had a visit immediately prior to flare. For study subjects with a visit immediately prior to flare, the PtGA, pain scores, and PhGA all increased at time of flare. For every unit increase in PtGA and Pain scores, there was a 9% and 23% higher odds of developing flare, respectively (p = 0.76, p = 0.40). For every unit increase in the PhGA score, there was a substantially lower odds of developing flare (p = 0.05)., Conclusion: Our results demonstrate that the PtGA and Pain scores are strongly correlated with each other and increased at the visit prior to flare, while the PhGA scores are not. Further, the PtGA and Pain score have some predictive value for flare, while the PhGA does not. These findings highlight the value of patient input in medical care and decision-making, and support the development and use of more sophisticated PROs in the care of JIA patients.
- Published
- 2021
- Full Text
- View/download PDF
12. PROMIS Computer Adaptive Tests and Their Correlation With Disease Activity in Juvenile Idiopathic Arthritis.
- Author
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Trachtman R, Wang CM, Murray E, Szymonifka J, Pan N, Adams AB, Taber SF, Onel KB, and Mandl LA
- Subjects
- Child, Computers, Cross-Sectional Studies, Disability Evaluation, Humans, Patient Reported Outcome Measures, Quality of Life, Surveys and Questionnaires, Arthritis, Juvenile diagnosis
- Abstract
Background/objective: The importance of patient-reported outcomes, like the Patient-Reported Outcomes Measurement Information System (PROMIS) measures, is increasingly recognized both in clinical care and in research. While "short forms" have been studied in juvenile idiopathic arthritis (JIA), study of PROMIS computer adaptive tests (CATs) in JIA is limited. This cross-sectional study evaluates whether PROMIS CATs correlate with disease activity in patients with JIA., Methods: A convenience sample of patients with JIA (N = 44) was recruited from a single center. Patients and parents completed pediatric and parent proxy PROMIS CATs. Disease activity was evaluated using the Juvenile Arthritis Disease Activity Score in 71 joints (JADAS-71) and the Childhood Health Assessment Questionnaire (CHAQ). Correlation of the CAT T scores with disease activity was assessed using Spearman correlation coefficients., Results: Forty-four of 80 eligible subjects (29 patients and 15 parents) completed all or some PROMIS CATs. Pain interference and mobility CATs correlated moderately with JADAS-71. Nearly all correlations with the JADAS-71 were weakened when the patient global was removed. Pain interference, mobility, and fatigue were strongly correlated with the CHAQ. Among parent proxy CATs, only mobility and depressive symptoms correlated strongly with the CHAQ., Conclusions: Only pain interference and mobility PROMIS CATs showed strong correlation with standard disease activity measures in JIA, and nearly all correlations were weakened when the patient global was removed. Correlations of the CATs with the CHAQ were stronger than correlations with the JADAS-71, indicating that although the CHAQ is no longer routinely used it may be a better measure of health-related quality of life in routine clinical care., Competing Interests: The authors declare no conflict of interest., (Copyright © 2019 Wolters Kluwer Health, Inc. All rights reserved.)
- Published
- 2021
- Full Text
- View/download PDF
13. Procalcitonin Differs in Children With Infection and Children With Disease Flares in Juvenile Idiopathic Arthritis.
- Author
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Trachtman R, Murray E, Wang CM, Szymonifka J, Toussi SS, Walters H, Nellis ME, Onel KB, and Mandl LA
- Subjects
- Biomarkers, Blood Sedimentation, Child, Cohort Studies, Humans, Symptom Flare Up, Arthritis, Juvenile diagnosis, Procalcitonin
- Abstract
Background/objective: Patients with juvenile idiopathic arthritis (JIA) often present with signs and symptoms suggestive of serious bacterial infection (SBI). Procalcitonin (PCT) is a biomarker that is elevated in SBI. We conducted a comparative cohort study to test the hypothesis that PCT levels will differ between active JIA, quiescent JIA, and bacteremic patients and healthy controls., Methods: From October 2016 to May2018, consecutive children 6 months to 18 years of age with (a) active untreated JIA, (b) quiescent JIA, and (c) healthy elective presurgical candidates were recruited from clinics at a musculoskeletal specialty hospital. Juvenile idiopathic arthritis was defined according to the International League of Associations for Rheumatology criteria. Clinical data and serum samples meeting the same criteria were included from a prior study. Consecutive bacteremic patients were identified over the same period. Procalcitonin and other common measures of inflammation were measured. Descriptive statistics and univariate logistic analyses were performed., Results: Ninety-two study subjects were recruited. Erythrocyte sedimentation rate, C-reactive protein (CRP), and PCT levels were all elevated in bacteremic patients in comparison to the other groups. Erythrocyte sedimentation rate and CRP both had wide ranges that overlapped between groups; however, the PCT concentration was 0.15 μg/mL or greater in 1 of 59 patients with JIA, whereas it was 0.15 μg/mL or less in only 1 bacteremic patient., Conclusions: Our study indicates that serum erythrocyte sedimentation rate, CRP, and PCT levels are all biomarkers that can be used to distinguish SBI versus active JIA at presentation. However, PCT is the most accurate, with the least overlap between patients with infection and noninfectious inflammatory arthritis. This finding can help clinicians direct therapy., Competing Interests: The authors declare no conflict of interest., (Copyright © 2019 Wolters Kluwer Health, Inc. All rights reserved.)
- Published
- 2021
- Full Text
- View/download PDF
14. Multisystem inflammatory syndrome in children related to COVID-19: A New York City experience.
- Author
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Riollano-Cruz M, Akkoyun E, Briceno-Brito E, Kowalsky S, Reed J, Posada R, Sordillo EM, Tosi M, Trachtman R, and Paniz-Mondolfi A
- Subjects
- Adolescent, COVID-19 epidemiology, COVID-19 immunology, Child, Child, Preschool, Female, Humans, Male, New York City epidemiology, Retrospective Studies, Tertiary Care Centers, Young Adult, COVID-19 complications, Cytokines immunology, Systemic Inflammatory Response Syndrome epidemiology
- Abstract
In December 2019, the 2019, a novel coronavirus disease (COVID-19) caused by severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) first emerged in Wuhan, China. This has now spread worldwide and was declared a pandemic by March 2020. Initially, the pediatric population was described as a low risk for severe COVID-19. However, reports have emerged recently of cases of COVID-19 in children with a systemic inflammatory disease, with features that overlap with Kawasaki disease (KD). We describe the first 15 cases with the multi-systeminflammatory syndrome in children (MIS-C), temporally related to COVID-19, who presented for care to a tertiary pediatric referral center in New York City. We discuss the disproportionate burden of disease among Hispanic/Latino and Black/African American ancestry, the distinct cytokine signature across the disease spectrum (IL-1/IL-6), and the potential role and pathogenesis of SARS-CoV-2 in this new clinical entity., (© 2020 Wiley Periodicals LLC.)
- Published
- 2021
- Full Text
- View/download PDF
15. Longitudinal Echocardiographic Assessment of Coronary Arteries and Left Ventricular Function following Multisystem Inflammatory Syndrome in Children.
- Author
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Jhaveri S, Ahluwalia N, Kaushik S, Trachtman R, Kowalsky S, Aydin S, and Stern K
- Subjects
- Adolescent, COVID-19 diagnostic imaging, Child, Child, Preschool, Coronary Vessels physiopathology, Echocardiography, Female, Follow-Up Studies, Humans, Inflammation, Longitudinal Studies, Male, Retrospective Studies, Systole, Ventricular Dysfunction, Left, Young Adult, COVID-19 complications, Coronary Vessels diagnostic imaging, Systemic Inflammatory Response Syndrome complications, Systemic Inflammatory Response Syndrome diagnostic imaging, Ventricular Function, Left
- Abstract
Myocardial dysfunction and coronary artery dilation have been reported in the acute setting of severe acute respiratory syndrome coronavirus disease-2-related multisystem inflammatory syndrome in children. Through a longitudinal echocardiographic single-center study of 15 children, we report the short-term outcomes of cardiac dysfunction and coronary artery dilation in severe acute respiratory syndrome coronavirus disease-2-related multisystem inflammatory syndrome in children., (Copyright © 2020 Elsevier Inc. All rights reserved.)
- Published
- 2021
- Full Text
- View/download PDF
16. Mapping Systemic Inflammation and Antibody Responses in Multisystem Inflammatory Syndrome in Children (MIS-C).
- Author
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Gruber CN, Patel RS, Trachtman R, Lepow L, Amanat F, Krammer F, Wilson KM, Onel K, Geanon D, Tuballes K, Patel M, Mouskas K, O'Donnell T, Merritt E, Simons NW, Barcessat V, Del Valle DM, Udondem S, Kang G, Gangadharan S, Ofori-Amanfo G, Laserson U, Rahman A, Kim-Schulze S, Charney AW, Gnjatic S, Gelb BD, Merad M, and Bogunovic D
- Subjects
- Adolescent, Antibodies, Viral blood, Autoantibodies blood, Betacoronavirus immunology, Betacoronavirus isolation & purification, COVID-19, Chemokine CCL3 metabolism, Child, Child, Preschool, Coronavirus Infections complications, Coronavirus Infections pathology, Coronavirus Infections virology, Female, Humans, Immunity, Humoral, Infant, Infant, Newborn, Inflammation metabolism, Interleukin-17 metabolism, Interleukin-18 metabolism, Killer Cells, Natural cytology, Killer Cells, Natural metabolism, Male, Pandemics, Pneumonia, Viral complications, Pneumonia, Viral pathology, Pneumonia, Viral virology, SARS-CoV-2, Systemic Inflammatory Response Syndrome immunology, Systemic Inflammatory Response Syndrome metabolism, T-Lymphocytes cytology, T-Lymphocytes metabolism, Young Adult, Inflammation pathology, Systemic Inflammatory Response Syndrome pathology
- Abstract
Initially, children were thought to be spared from disease caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). However, a month into the epidemic, a novel multisystem inflammatory syndrome in children (MIS-C) emerged. Herein, we report on the immune profiles of nine MIS-C cases. All MIS-C patients had evidence of prior SARS-CoV-2 exposure, mounting an antibody response with intact neutralization capability. Cytokine profiling identified elevated signatures of inflammation (IL-18 and IL-6), lymphocytic and myeloid chemotaxis and activation (CCL3, CCL4, and CDCP1), and mucosal immune dysregulation (IL-17A, CCL20, and CCL28). Immunophenotyping of peripheral blood revealed reductions of non-classical monocytes, and subsets of NK and T lymphocytes, suggesting extravasation to affected tissues. Finally, profiling the autoantigen reactivity of MIS-C plasma revealed both known disease-associated autoantibodies (anti-La) and novel candidates that recognize endothelial, gastrointestinal, and immune-cell antigens. All patients were treated with anti-IL-6R antibody and/or IVIG, which led to rapid disease resolution., Competing Interests: Declaration of Interests DB reports ownership in Lab11 Therapeutics. S. Gnjatic reports consultancy and/or advisory roles for Merck, Neon Therapeutics and OncoMed and research funding from Bristol-Myers Squibb, Genentech, Immune Design, Agenus, Janssen R&D, Pfizer, Takeda, and Regeneron., (Published by Elsevier Inc.)
- Published
- 2020
- Full Text
- View/download PDF
17. Multisystem Inflammatory Syndrome in Children Associated with Severe Acute Respiratory Syndrome Coronavirus 2 Infection (MIS-C): A Multi-institutional Study from New York City.
- Author
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Kaushik S, Aydin SI, Derespina KR, Bansal PB, Kowalsky S, Trachtman R, Gillen JK, Perez MM, Soshnick SH, Conway EE Jr, Bercow A, Seiden HS, Pass RH, Ushay HM, Ofori-Amanfo G, and Medar SS
- Subjects
- Adolescent, Betacoronavirus, C-Reactive Protein analysis, COVID-19, Child, Child, Preschool, Coronavirus Infections drug therapy, Female, Fibrin Fibrinogen Degradation Products analysis, Humans, Infant, Intensive Care Units, Pediatric, Male, Natriuretic Peptide, Brain blood, New York City, Pandemics, Procalcitonin analysis, Retrospective Studies, SARS-CoV-2, Systemic Inflammatory Response Syndrome therapy, Treatment Outcome, Ventricular Function, Left, Young Adult, COVID-19 Drug Treatment, Coronavirus Infections complications, Pneumonia, Viral complications, Systemic Inflammatory Response Syndrome diagnosis
- Abstract
Objective: To assess clinical characteristics and outcomes of severe acute respiratory syndrome coronavirus 2-associated multisystem inflammatory syndrome in children (MIS-C)., Study Design: Children with MIS-C admitted to pediatric intensive care units in New York City between April 23 and May 23, 2020, were included. Demographic and clinical data were collected., Results: Of 33 children with MIS-C, the median age was 10 years; 61% were male; 45% were Hispanic/Latino; and 39% were black. Comorbidities were present in 45%. Fever (93%) and vomiting (69%) were the most common presenting symptoms. Depressed left ventricular ejection fraction was found in 63% of patients with median ejection fraction of 46.6% (IQR, 39.5-52.8). C-reactive protein, procalcitonin, d-dimer, and pro-B-type natriuretic peptide levels were elevated in all patients. For treatment, intravenous immunoglobulin was used in 18 (54%), corticosteroids in 17 (51%), tocilizumab in 12 (36%), remdesivir in 7 (21%), vasopressors in 17 (51%), mechanical ventilation in 5 (15%), extracorporeal membrane oxygenation in 1 (3%), and intra-aortic balloon pump in 1 (3%). The left ventricular ejection fraction normalized in 95% of those with a depressed ejection fraction. All patients were discharged home with median duration of pediatric intensive care unit stay of 4.7 days (IQR, 4-8 days) and a hospital stay of 7.8 days (IQR, 6.0-10.1 days). One patient (3%) died after withdrawal of care secondary to stroke while on extracorporeal membrane oxygenation., Conclusions: Critically ill children with coronavirus disease-2019-associated MIS-C have a spectrum of severity broader than described previously but still require careful supportive intensive care. Rapid, complete clinical and myocardial recovery was almost universal., (Copyright © 2020 Elsevier Inc. All rights reserved.)
- Published
- 2020
- Full Text
- View/download PDF
18. Recurrent focal segmental glomerulosclerosis after kidney transplantation: response to comments by Straatmann and Vehaskari.
- Author
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Trachtman H and Trachtman R
- Subjects
- Humans, Plasmapheresis, Recurrence, Glomerulosclerosis, Focal Segmental, Kidney Transplantation
- Published
- 2016
- Full Text
- View/download PDF
19. Recurrent focal segmental glomerulosclerosis after kidney transplantation.
- Author
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Trachtman R, Sran SS, and Trachtman H
- Subjects
- Humans, Incidence, Recurrence, Risk Factors, Glomerulosclerosis, Focal Segmental epidemiology, Glomerulosclerosis, Focal Segmental etiology, Glomerulosclerosis, Focal Segmental therapy, Kidney Failure, Chronic surgery, Kidney Transplantation adverse effects, Plasmapheresis methods
- Abstract
Focal segmental glomerulosclerosis (FSGS) is an important cause of glomerular disease in children and adolescents and nearly 50 % of affected patients will progress to end-stage kidney disease over a 5 to 10-year period. Unfortunately, there is no established treatment for disease in the native kidney. Moreover, up to 55 % of patients develop recurrent disease after receiving a kidney transplant, with a substantially higher risk in patients who have already experienced recurrent disease in a prior transplant. A number of clinical and laboratory factors have been identified as risk factors for this complication. In addition, new investigations into podocyte biology and circulating permeability factors have shed light on the cause of recurrent the disease. While a number of novel therapeutic agents have been applied in the management of this problem, there still is no proven treatment. In this review, we summarize recent advances in the epidemiology, pathophysiology, and treatment of recurrent FSGS in pediatric patients who have received a kidney transplant.
- Published
- 2015
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20. Parental and other factors associated with hydroxyurea use for pediatric sickle cell disease.
- Author
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Oyeku SO, Driscoll MC, Cohen HW, Trachtman R, Pashankar F, Mullen C, Giardina PJ, Velazco N, Racine AD, and Green NS
- Subjects
- Adolescent, Child, Child, Preschool, Cross-Sectional Studies, Female, Humans, Male, Anemia, Sickle Cell drug therapy, Antisickling Agents therapeutic use, Health Knowledge, Attitudes, Practice, Hydroxyurea therapeutic use, Parents
- Abstract
Background: Hydroxyurea (HU) is highly effective treatment for sickle cell disease (SCD). While pediatric use of HU is accepted clinical practice, barriers to use may impede its potential benefit., Procedure: A survey of parents of children ages 5-17 years with SCD was performed across five institutions to assess factors associated with HU use., Results: Of the 173 parent responses, 65 (38%) had children currently taking HU. Among parents of children not taking HU, the most commonly cited reasons were that their hematology provider had not offered it, their child was not sufficiently symptomatic and concerns about potential side effects. Even parents of HU users reported widespread concern about effectiveness, long-term safety, and off-label use. In bivariate analyses, children's ages, parental demographics such as education level, or travel time to their hematology provider were not correlated with HU use. Bivariate analysis and multivariate logistic regression revealed three significant factors associated with current HU use: better parental knowledge about its major therapeutic effects (P < 0.001), sickle genotype (P = 0.005), and institution of clinical care (P = 0.04)., Conclusions: Pervasive concerns about HU safety exist, even among parents of current users. Varying knowledge among parents appears to be independent of their demographics, and is associated with HU use. Inter-institutional variability in parental knowledge and drug uptake highlights potentially potent site-specific influences on likelihood of HU use. Overall, these survey data underscore the need for strategies to bolster parental understanding about benefits of HU and address concerns about its safety., (Copyright © 2012 Wiley Periodicals, Inc.)
- Published
- 2013
- Full Text
- View/download PDF
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