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https://www.readbyqxmd.com/read/27390368/diffuse-alveolar-hemorrhage-as-a-manifestation-of-childhood-onset-systemic-lupus-erythematosus
#1
Saimun Singla, Debra L Canter, Timothy J Vece, Eyal Muscal, Marietta DeGuzman
BACKGROUND: Diffuse alveolar hemorrhage (DAH) is a devastating clinical syndrome characterized by a falling hematocrit, respiratory insufficiency, and radiographic evidence of pulmonary infiltrates. Literature regarding management of DAH in childhood-onset SLE (cSLE) is limited. METHODS: We reviewed the presentation, management, and outcome of DAH in a pediatric tertiary medical center with one of the largest cSLE cohorts in North America. During a 10 year period 7 of 410 children with cSLE had DAH...
August 2016: Hospital Pediatrics
https://www.readbyqxmd.com/read/27111558/comparing-presenting-clinical-features-in-48-children-with-microscopic-polyangiitis-to-183-children-who-have-granulomatosis-with-polyangiitis-wegener-s-an-archive-cohort-study
#2
David A Cabral, Debra L Canter, Eyal Muscal, Kabita Nanda, Dawn M Wahezi, Steven J Spalding, Marinka Twilt, Susanne M Benseler, Sarah Campillo, Sirirat Charuvanij, Paul Dancey, Barbara A Eberhard, Melissa E Elder, Aimee Hersh, Gloria C Higgins, Adam M Huber, Raju Khubchandani, Susan Kim, Marisa Klein-Gitelman, Mikhail M Kostik, Erica F Lawson, Tzielan Lee, Joanna M Lubieniecka, Deborah McCurdy, Lakshmi N Moorthy, Kimberly A Morishita, Susan M Nielsen, Kathleen M O'Neil, Andreas Reiff, Goran Ristic, Angela B Robinson, Angelyne Sarmiento, Susan Shenoi, Mary B Toth, Heather A Van Mater, Linda Wagner-Weiner, Jennifer E Weiss, Andrew J White, Rae S M Yeung
OBJECTIVE: To uniquely classify children with microscopic polyangiitis (MPA), to describe their demographic characteristics, presenting clinical features, and initial treatments in comparison to patients with granulomatosis with polyangiitis (Wegener's) (GPA). METHODS: The European Medicines Agency (EMA) classification algorithm was applied by computation to categorical data from patients recruited to the ARChiVe (A Registry for Childhood Vasculitis: e-entry) cohort, with the data censored to November 2015...
October 2016: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/27036378/identifying-targets-for-improving-mental-healthcare-of-adolescents-with-systemic-lupus-erythematosus-perspectives-from-pediatric-rheumatology-clinicians-in-the-united-states-and-canada
#3
Andrea M Knight, Michelle E Vickery, Eyal Muscal, Alaina M Davis, Julia G Harris, Arzu Soybilgic, Karen B Onel, Laura E Schanberg, Tamar Rubinstein, Beth S Gottlieb, David S Mandell, Emily von Scheven
OBJECTIVE: To identify targets for improving mental healthcare of adolescents with systemic lupus erythematosus (SLE) by assessing current practices and perceived barriers for mental health intervention by pediatric rheumatology clinicians. METHODS: Members of the Childhood Arthritis and Rheumatology Research Alliance (CARRA) completed a Web-based survey assessing current mental health practices, beliefs, and barriers. We examined associations between provider characteristics and the frequency of barriers to mental health screening and treatment using multivariable linear regression...
June 2016: Journal of Rheumatology
https://www.readbyqxmd.com/read/26236759/earlier-treatment-of-nmdar-antibody-encephalitis-in-children-results-in-a-better-outcome
#4
Susan Byrne, Cathal Walsh, Yael Hacohen, Eyal Muscal, Joseph Jankovic, Amber Stocco, Russell C Dale, Angela Vincent, Ming Lim, Mary King
No abstract text is available yet for this article.
August 2015: Neurology® Neuroimmunology & Neuroinflammation
https://www.readbyqxmd.com/read/26220353/autoimmune-encephalitis-following-bone-marrow-transplantation
#5
Geetanjali S Rathore, Kathryn S Leung, Eyal Muscal
BACKGROUND: Neurological complications, especially encephalopathy and seizures, are commonly seen in bone marrow transplant patients. Infections, chemotoxicity, graft versus host disease, or secondary central nervous system malignancies are the most common underlying etiologies. There is increased awareness that autoimmune encephalitis may cause neurological dysfunction in immunocompetent children. The potential role of such a mechanism in children undergoing bone marrow transplantation is unknown...
September 2015: Pediatric Neurology
https://www.readbyqxmd.com/read/25926050/high-titer-anti-basement-membrane-antibodies-in-a-subset-of-patients-with-pediatric-systemic-lupus-erythematosus
#6
Alvaro Orjuela, Adisak Suwanichkul, Debra Canter, Charles G Minard, Sridevi Devaraj, M John Hicks, Eyal Muscal, Scott E Wenderfer
BACKGROUND/AIMS: There is a critical need for more noninvasive biomarkers to identify nephritis in patients with systemic lupus erythematosus (SLE). Recent studies in a model mouse and an adult SLE patient cohort suggest that anti-basement membrane antibody levels correlate well with lupus activity and kidney injury. The purpose of this study was to assess the anti-basement membrane reactivity in pediatric SLE (pSLE) patients with or without nephritis. METHODS: Auto-antibodies to basement membrane antigens were assessed using an anti-matrigel ELISA...
2015: American Journal of Nephrology
https://www.readbyqxmd.com/read/25708844/cognitive-performance-scores-for-the-pediatric-automated-neuropsychological-assessment-metrics-in-childhood-onset-systemic-lupus-erythematosus
#7
Patricia Vega-Fernandez, Shana Vanderburgh White, Frank Zelko, Natasha M Ruth, Deborah M Levy, Eyal Muscal, Marisa S Klein-Gitelman, Adam M Huber, Lori B Tucker, Tresa Roebuck-Spencer, Jun Ying, Hermine I Brunner
OBJECTIVE: To develop and initially validate a global cognitive performance score (CPS) for the Pediatric Automated Neuropsychological Assessment Metrics (PedANAM) to serve as a screening tool of cognition in childhood lupus. METHODS: Patients (n = 166) completed the 9 subtests of the PedANAM battery, each of which provides 3 principal performance parameters (accuracy, mean reaction time for correct responses, and throughput). Cognitive ability was measured by formal neurocognitive testing or estimated by the Pediatric Perceived Cognitive Function Questionnaire-43 to determine the presence or absence of neurocognitive dysfunction (NCD)...
August 2015: Arthritis Care & Research
https://www.readbyqxmd.com/read/25708626/barriers-to-and-facilitators-of-a-career-as-a-physician-scientist-among-rheumatologists-in-the-us
#8
Alexis Ogdie, Ami A Shah, Una E Makris, Yihui Jiang, Amanda E Nelson, Alfred H J Kim, Sheila T Angeles-Han, Flavia V Castelino, Amit Golding, Eyal Muscal, J Michelle Kahlenberg, Frances K Barg
OBJECTIVE: To determine perceived barriers to and facilitators of a career in rheumatology research, examine factors leading rheumatologists to leave an academic research career, and solicit ways to best support young physician-scientists. METHODS: A web-based survey was conducted among the domestic American College of Rheumatology (ACR) membership from January through March 2014. Inclusion criteria were ACR membership and an available e-mail address. Non-rheumatologists were excluded...
September 2015: Arthritis Care & Research
https://www.readbyqxmd.com/read/24920861/utility-and-safety-of-rituximab-in-pediatric-autoimmune-and-inflammatory-cns-disease
#9
MULTICENTER STUDY
Russell C Dale, Fabienne Brilot, Lisa V Duffy, Marinka Twilt, Amy T Waldman, Sona Narula, Eyal Muscal, Kumaran Deiva, Erik Andersen, Michael R Eyre, Despina Eleftheriou, Paul A Brogan, Rachel Kneen, Gulay Alper, Banu Anlar, Evangeline Wassmer, Kirsten Heineman, Cheryl Hemingway, Catherine J Riney, Andrew Kornberg, Marc Tardieu, Amber Stocco, Brenda Banwell, Mark P Gorman, Susanne M Benseler, Ming Lim
OBJECTIVE: To assess the utility and safety of rituximab in pediatric autoimmune and inflammatory disorders of the CNS. METHODS: Multicenter retrospective study. RESULTS: A total of 144 children and adolescents (median age 8 years, range 0.7-17; 103 female) with NMDA receptor (NMDAR) encephalitis (n = 39), opsoclonus myoclonus ataxia syndrome (n = 32), neuromyelitis optica spectrum disorders (n = 20), neuropsychiatric systemic lupus erythematosus (n = 18), and other neuroinflammatory disorders (n = 35) were studied...
July 8, 2014: Neurology
https://www.readbyqxmd.com/read/24677983/a53-natural-history-of-pediatric-class-v-membranous-lupus-nephritis-a-serial-biopsy-study
#10
Debra Canter, Karen Eldin, M John Hicks, Eyal Muscal, Marietta Deguzman, Scott E Wenderfer
BACKGROUND/PURPOSE: In 1998, Sorof et al. reported 28% of 60 pediatric SLE patients had class V lesions on their first biopsy and 37% had class V lesions on their most recent biopsy. Although 19% of patients with LN transformed from other classes to class V, there were no patients with repeat biopsies who showed complete resolution of their class V lesions. The prognosis of the patients with class V LN was better than those with non-class V LN (only 5% vs. 21% developed renal dysfunction or died)...
March 2014: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/24677962/a33-cognitive-performance-scores-for-the-pediatric-automated-neuropsychological-assessment-metrics-in-childhood-onset-systemic-lupus-erythematosus
#11
Patricia Vega-Fernandez, Shana Vanderburgh, Natasha M Ruth, Deborah M Levy, Frank A Zelko, Eyal Muscal, Marisa S Klein-Gitelman, Adam Huber, Kasha Wiley, Erin C Thomas, Lori B Tucker, Tresa Roebuck-Spencer, Jun Ying, Hermine Brunner
BACKGROUND/PURPOSE: Children with SLE (cSLE) can experience neuropsychiatric SLE (NPSLE), commonly manifesting as neurocognitive dysfunction which can interfere with normal development. Formal neurocognitive testing (FNCT) is the most accepted test for diagnosing neurocognitive deficits (NCD). Access to it is limited and costly, and it is time-consuming. The Pediatric Automated Neurophysiological Assessments Metrics (PedANAM) is a computerized battery of 10 subtests measuring various aspects of cognitive ability...
March 2014: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/24677934/a177-program-evaluation-of-the-acr-carra-inter-institutional-mentoring-program-amigo-in-pediatric-rheumatology
#12
Eyal Muscal, Lakshmi N Moorthy, Meredith P Riebschleger, Anne Eberhard, Anna Huttenlocher, Marisa S Klein-Gitelman, Sampath Prahalad, Kelly Rouster Stevens, Rayfel Schneider, Peter A Nigrovic
BACKGROUND/PURPOSE: In pediatric rheumatology, the small size of many academic programs translates into limited mentoring options for early career physicians. To address this "mentorship gap," in 2011 the American College of Rheumatology (ACR) and the Childhood Arthritis and Rheumatology Research Alliance (CARRA) joined together to develop AMIGO, the ACR/CARRA Mentoring Interest Group, that now includes greater than half of pediatric rheumatology fellows and junior faculty in the US and Canada...
March 2014: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/24677865/a10-younger-age-and-severity-of-renal-presentation-distinguishes-microscopic-polyangiitis-from-granulomatosis-with-polyangiitis-in-children-an-archive-study
#13
Debra Bingham, Eyal Muscal, Kabita Nanda, Dawn M Wahezi, Steven J Spalding, Marinka Twilt, Susa Benseler, David A Cabral
BACKGROUND/PURPOSE: Comparisons of pediatric ANCA-associated vasculitis subtypes (AAV) are limited by the paucity of reported cases, standardized definitions, and overlapping classification criteria. Published work from ARChiVe (A Registry for Childhood Vasculitis) demonstrated modifications of validated classification algorithms applied to pediatric patients with AAV can classify each patient to mutually exclusive diagnostic categories. We compared presenting features of children with microscopic polyangiitis (MPA) and granulomatosis with polyangiitis (GPA) classified according to this methodology...
March 2014: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/24677828/a79-pulmonary-hemorrhage-in-pediatric-systemic-lupus-erythematous-clinical-course-and-outcomes
#14
Saimun Singla, Eyal Muscal, Debra Canter, Marietta Deguzman, Timothy Vece
BACKGROUND/PURPOSE: Pulmonary hemorrhage (PH) is a rare and devastating manifestation of SLE, occurring in 1-5% of adult cohorts with mortality rates ranging from 70%-90%. The majority of PH reported in SLE refers to the adult population, and there is a paucity of data in pediatrics. Therefore, we describe our experience with PH in pediatric SLE, outlining disease presentation, treatment, and outcomes. METHODS: We reviewed records of pediatric SLE patients who presented with pulmonary involvement diagnosed between January 2000 and August 2010...
March 2014: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/24677818/a69-lupus-nephritis-and-autoantibody-characteristics-of-a-single-center-cohort-of-male-pediatric-sle-patients
#15
Scott E Wenderfer, Debra Canter, Marietta Deguzman, Eyal Muscal
BACKGROUND/PURPOSE: Males represent 4-22% of all SLE patients, but it is unclear if gender should influence management. A number of studies of mostly adult patients suggest genetic, hormonal and environmental aspects of gender differences contribute to differences in presentation, clinical course and outcome, including a positive association between male gender and nephritis and a negative association with disease activity and outcome. While differences may exist, these findings are supported by limited evidence, particularly for children and adolescents with SLE...
March 2014: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/24461154/amigo-a-novel-approach-to-the-mentorship-gap-in-pediatric-rheumatology
#16
EDITORIAL
Peter A Nigrovic, Eyal Muscal, Meredith Riebschleger, L Nandini Moorthy, Hermine I Brunner, Barbara A Eberhard, Marisa Klein-Gitelman, Sampath Prahalad, Rayfel Schneider
No abstract text is available yet for this article.
February 2014: Journal of Pediatrics
https://www.readbyqxmd.com/read/23400857/the-spectrum-of-movement-disorders-in-children-with-anti-nmda-receptor-encephalitis
#17
José Fidel Baizabal-Carvallo, Amber Stocco, Eyal Muscal, Joseph Jankovic
BACKGROUND: Movement disorders are frequent but difficult to characterize in patients with anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis. METHODS: The phenomenology of movement disorders was characterized after a detailed examination of children with anti-NMDAR-encephalitis. RESULTS: We studied 9 children (5 females), ages 3-14 years, with confirmed anti-NMDAR-encephalitis. All patients presented with at least 1 movement disorder, including chorea (n=4), stereotypic movements (n=4), ataxia (n=3), limb dystonia (n=2), limb myorhythmia (n=2), oromandibular dystonia (n=2), facial myorhythmia, blepharospasm, opisthotonus, athetosis, and tremor (n=1, each)...
April 2013: Movement Disorders: Official Journal of the Movement Disorder Society
https://www.readbyqxmd.com/read/23155205/childhood-polyarteritis-nodosa-presenting-with-central-nervous-system-manifestations-and-the-posterior-reversible-encephalopathy-syndrome
#18
Ricardo Guirola, Jill V Hunter, Maria Perez, Eyal Muscal
Polyarteritis nodosa is a systemic necrotizing vasculitis involving medium-sized muscular arteries. Polyneuropathy is the only neurologic manifestation included in the pediatric classification schema. Central nervous system manifestations include infarction, hemorrhage, and encephalitis. We report on a 13-year-old female whose initial presentation of polyarteritis nodosa included hypertension, seizures, and neuroimaging findings of vasogenic edema and posterior reversible encephalopathy syndrome. Posterior reversible encephalopathy syndrome has been reported in association with renal disease, transplantation, autoimmunity, and cytotoxic medications...
January 2014: Journal of Child Neurology
https://www.readbyqxmd.com/read/22859342/do-adult-disease-severity-subclassifications-predict-use-of-cyclophosphamide-in-children-with-anca-associated-vasculitis-an-analysis-of-archive-study-treatment-decisions
#19
Kimberly Morishita, Jaime Guzman, Peter Chira, Eyal Muscal, Andrew Zeft, Marisa Klein-Gitelman, America G Uribe, Leslie Abramson, Susanne M Benseler, Anne Eberhard, Kaleo Ede, Philip J Hashkes, Aimee O Hersh, Gloria Higgins, Lisa F Imundo, Lawrence Jung, Susan Kim, Daniel J Kingsbury, Erica F Lawson, Tzielan Lee, Suzanne C Li, Daniel J Lovell, Thomas Mason, Deborah McCurdy, Kathleen M O'Neil, Marilynn Punaro, Suzanne E Ramsey, Andreas Reiff, Margalit Rosenkranz, Kenneth N Schikler, Rosie Scuccimarri, Nora G Singer, Anne M Stevens, Heather van Mater, Dawn M Wahezi, Andrew J White, David A Cabral
OBJECTIVE: To determine whether adult disease severity subclassification systems for antineutrophil cytoplasmic antibody-associated vasculitis (AAV) are concordant with the decision to treat pediatric patients with cyclophosphamide (CYC). METHODS: We applied the European Vasculitis Study (EUVAS) and Wegener's Granulomatosis Etanercept Trial (WGET) disease severity subclassification systems to pediatric patients with AAV in A Registry for Childhood Vasculitis (ARChiVe)...
October 2012: Journal of Rheumatology
https://www.readbyqxmd.com/read/22589257/increased-sensitivity-of-the-european-medicines-agency-algorithm-for-classification-of-childhood-granulomatosis-with-polyangiitis
#20
América G Uribe, Adam M Huber, Susan Kim, Kathleen M O'Neil, Dawn M Wahezi, Leslie Abramson, Kevin Baszis, Susanne M Benseler, Suzanne L Bowyer, Sarah Campillo, Peter Chira, Aimee O Hersh, Gloria C Higgins, Anne Eberhard, Kaleo Ede, Lisa F Imundo, Lawrence Jung, Daniel J Kingsbury, Marisa Klein-Gitelman, Erica F Lawson, Suzanne C Li, Daniel J Lovell, Thomas Mason, Deborah McCurdy, Eyal Muscal, Lorien Nassi, Egla Rabinovich, Andreas Reiff, Margalit Rosenkranz, Kenneth N Schikler, Nora G Singer, Steven Spalding, Anne M Stevens, David A Cabral
OBJECTIVE: Granulomatosis with polyangiitis (Wegener's; GPA) and other antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) are rare in childhood and are sometimes difficult to discriminate. We compared use of adult-derived classification schemes for GPA against validated pediatric criteria in the ARChiVe (A Registry for Childhood Vasculitis e-entry) cohort, a Childhood Arthritis and Rheumatology Research Alliance initiative. METHODS: Time-of-diagnosis data for children with physician (MD) diagnosis of AAV and unclassified vasculitis (UCV) from 33 US/Canadian centers were analyzed...
August 2012: Journal of Rheumatology
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