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Muscal, eyal

Saman K Hashmi, Katie Bergstrom, Alison A Bertuch, Jenny M Despotovic, Eyal Muscal, Fan Xia, Weimin Bi, Andrea Marcogliese, Rosa Diaz
Neutropenia in pediatric patients can be due to a variety of disorders. We describe two patients who underwent extensive evaluation over many years for arthralgias and moderate neutropenia of unclear etiology. Genetic testing identified a pathogenic variant in PSTPIP1 (proline-serine-threonine phosphatase-interacting protein 1) in both patients. Markedly elevated inflammatory markers and zinc levels confirmed the rare diagnosis of PSTPIP1-associated myeloid-related proteinemia inflammatory (PAMI) syndrome, tailoring treatment...
September 10, 2018: Pediatric Blood & Cancer
Andrea Knight, Michelle Vickery, Lauren Faust, Eyal Muscal, Alaina Davis, Julia Harris, Aimee O Hersh, Martha Rodriguez, Karen Onel, Tamar Rubinstein, Nina Washington, Elissa R Weitzman, Hana Conlon, Jennifer M P Woo, Dana Gerstbacher, Emily von Scheven
OBJECTIVE: To identify behavioral health provider perspectives on gaps in mental health care for youth with rheumatologic conditions. METHODS: Social workers (n=34) and psychologists (n=8) at pediatric rheumatology centers in the Childhood Arthritis and Rheumatology Research Alliance (CARRA) completed an online survey assessing current practices and mental health care needs of youth with rheumatologic conditions. Responses were compared to a published survey of CARRA rheumatologists (n=119)...
June 28, 2018: Arthritis Care & Research
Sonika Agarwal, Jake R Keller, Chloe E Nunneley, Eyal Muscal, Michael C Braun, Poyyapakkam Srivaths, Timothy E Lotze
Pediatric neurologic conditions requiring therapeutic plasma exchange are rare in children and literature is sparse. The study aims to determine the outcomes, safety, and feasibility of therapeutic plasma exchange treatment in pediatric neurologic disorders. This retrospective analysis looked at the outcomes and safety of therapeutic plasma exchange in children (n = 50) with neurologic conditions. Patient age ranged <1 to 19 years old with a mean of 10.35 years. Of the 50 children treated with plasmapheresis, 26 patients received inpatient rehabilitation...
February 2018: Journal of Child Neurology
Natalie C Pon, Kimberly M Houck, Eyal Muscal, Sindhu A Idicula
Antibody-mediated encephalopathies associated with serum or cerebrospinal fluid antibodies directed against neuronal structures may present with a multitude of neuropsychiatric syndromes. Although some of the antibody-driven conditions are now well recognized in adults (eg, N-methyl-D-aspartate receptor antibody encephalitis), the spectrum of neuropsychiatric manifestations in the pediatric population is less clear. Psychosis, confusion, catatonia, and additional behavioral changes, along with seizures, encephalopathy, and movement disorders, may be initial manifestations or concurrent features in all age groups...
November 2017: Journal of Psychiatric Practice
Maria Pereira, Eyal Muscal, Karen Eldin, M John Hicks, Anna Carmela P Sagcal-Gironella, Marietta DeGuzman, Scott E Wenderfer
BACKGROUND: Best practices for managing childhood-onset membranous lupus nephritis (MLN) are not yet established. Most studies involve primarily or exclusively adult cohorts or pediatric cohorts with combinations of pure or mixed membranous and proliferative nephritis. METHODS: We performed a single-center cohort study of consecutively diagnosed children with pure MLN from 1990 and 2016. Patients received care in Houston, Texas, one of the most diverse metropolitan areas in North America...
December 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
Saimun Singla, Scott E Wenderfer, Eyal Muscal, Anna Carmela P Sagcal-Gironella, Jordan S Orange, George Makedonas
BACKGROUND: Several studies suggest that defects of regulatory T-cells (Tregs) and impaired cellular immunity are secondary to an imbalance between auto-aggressive T-cells and Tregs in lupus patients. Discrepancies in Tregs and effector T-cells (Teff) in active lupus patients are shown to be restored in patients upon receiving immunosuppressive therapy. Therefore, our main aim was to observe frequencies of these CD4(+) T-cell subsets and Tregs/Teff ratio in a new diagnosis of childhood-onset systemic lupus erythematous (cSLE) before and after initiation of therapy...
2017: Frontiers in Pediatrics
Luca Bartolini, Eyal Muscal
The objective of the study was to identify differences in treatment strategies for anti-NMDA receptor encephalitis based on specialty of treating physicians, geographic location, and years in practice. We conducted an anonymous worldwide electronic survey through the Practice Current section of Neurology® Clinical Practice to appraise differences in decisions about first- and second-line treatment and timing for initiation of second-line treatment for anti-NMDA receptor encephalitis. 399 participants answered all questions of the survey and were included in the analysis...
April 2017: Journal of Neurology
Ashwaq AlE'ed, Patricia Vega-Fernandez, Eyal Muscal, Claas H Hinze, Lori B Tucker, Simone Appenzeller, Brigitte Bader-Meunier, Johannes Roth, Vicenç Torrente-Segarra, Marisa S Klein-Gitelman, Deborah M Levy, Tresa Roebuck-Spencer, Hermine I Brunner
No abstract text is available yet for this article.
October 2017: Arthritis Care & Research
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
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
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
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
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
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
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
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
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
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
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
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
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