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Amit Thakral, Marisa S Klein-Gitelman
Systemic lupus erythematosus (SLE) is a chronic multisystem autoimmune disorder in which 20 % of patients are diagnosed in childhood. Childhood-onset SLE is associated with higher morbidity and mortality than adult-onset SLE. The aims of disease management with early immunosuppression are to decrease disease activity and improve quality of life. A multidisciplinary approach is necessary due to the complexity of lupus in pediatric patients. It is important to provide patients with high quality of care and to instill ownership of their disease process from a young age to prepare them to manage this life-long illness...
September 30, 2016: Rheumatol Ther
Marisa S Klein-Gitelman
Neonatal lupus results from the passive transfer of autoantibodies; however, this transfer is not sufficient to cause disease. This article reviews clinical presentation with a focus on autoimmune-mediated congenital heart disease. Recent data looking for additional disease mechanisms and biomarkers as well as latest information on interventions will be reviewed. Our understanding of this rare disease is often dependent on patient participation in disease registries and biorepositories. Future participation in registries including descriptive as well as biophysical data is critical to our knowledge...
September 2016: Current Rheumatology Reports
Amer Khojah, Marisa Klein-Gitelman
Investigators from University of California Los Angeles studied the presence of different T cell subset population in the brain tissue of 7 patients with Rasmussen encephalitis, a rare neuroinflammatory disorder characterized by intractable seizures and usually associated with progressive hemi cerebral atrophy, who underwent brain surgery and compared them to patients with focal cortical dysplasia.
March 2016: Pediatric neurology briefs
Anne M Stevens, Sami B Kanaan, Kathryn S Torok, Thomas A Medsger, Maureen D Mayes, John D Reveille, Marisa Klein-Gitelman, Ann M Reed, Tzielan Lee, Suzanne C Li, Gretchen Henstorf, Christine Luu, Tessa Aydelotte, J Lee Nelson
OBJECTIVE: Systemic sclerosis (SSc) is a rare disease that is particularly uncommon in children. Specific HLA alleles have been associated with SSc in adults. This study investigated HLA class II alleles in juvenile onset SSc (jSSc). METHODS: DRB1, DQA1 and DQB1 alleles were determined by DNA-based HLA typing. Analyses were conducted comparing Caucasians with jSSc (n=76) to healthy Caucasians (n=581). RESULTS: Initial analyses focused on HLA class II associations previously reported in adult Caucasians with SSc...
May 23, 2016: Arthritis & Rheumatology
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
Hermine I Brunner, Michael R Bennett, Khalid Abulaban, Marisa S Klein-Gitelman, Kathleen M O'Neil, Lori Tucker, Stacy P Ardoin, Kelly A Rouster-Stevens, Karen B Onel, Nora G Singer, B Anne Eberhard, Lawrence K Jung, Lisa Imundo, Tracey B Wright, David Witte, Brad H Rovin, Jun Ying, Prasad Devarajan
OBJECTIVE: Noninvasive estimation of the degree of inflammation seen on kidney biopsy with lupus nephritis (LN) remains difficult. The objective of this study was to develop a Renal Activity Index for Lupus (RAIL) that, based solely on laboratory measures, accurately reflects histologic LN activity. METHODS: We assayed traditional LN laboratory tests and 16 urine biomarkers (UBMs) in children (n = 47) at the time of kidney biopsy. Histologic LN activity was measured by the National Institutes of Health activity index (NIH-AI) and the tubulointerstitial activity index (TIAI)...
July 2016: Arthritis Care & Research
Marisa S Klein-Gitelman, Megan L Curran
No abstract text is available yet for this article.
December 2015: Journal of Pediatrics
Lakshmi Nandini Moorthy, Eyal Muscal, Meredith Riebschleger, Marisa Klein-Gitelman, Lise E Nigrovic, Jeffrey R Horon, Kelly Rouster-Stevens, Polly J Ferguson, B Anne Eberhard, Hermine I Brunner, Sampath Prahalad, Rayfel Schneider, Peter A Nigrovic
OBJECTIVE: The small size of many pediatric rheumatology programs translates into limited mentoring options for early career physicians. To address this problem, the American College of Rheumatology (ACR) and the Childhood Arthritis and Rheumatology Research Alliance (CARRA) developed a subspecialty-wide interinstitutional mentoring program, the ACR/CARRA Mentoring Interest Group (AMIGO). We sought to assess the impact of this program on mentoring within pediatric rheumatology. METHODS: In a longitudinal 3-year study, participant ratings from the AMIGO pilot program were compared with those after the program was opened to general enrollment...
May 2016: Arthritis Care & Research
Rina Mina, Julia G Harris, Marisa S Klein-Gitelman, Simone Appenzeller, Maraisa Centeville, Diane Eskra, Jennifer L Huggins, Anne L Johnson, Raju Khubchandani, Prachi Khandekar, Jiha Lee, Hai Mei Liu, Joshua D Pendl, Clovis A Silva, Marco F Silva, Ahmad I Zaal, Esi Morgan DeWitt, Stacy P Ardoin, Hermine I Brunner
OBJECTIVE: To assess the quality of medical care in childhood-onset systemic lupus erythematosus (SLE) at tertiary pediatric rheumatology centers as measured by observance of SLE quality indicators (SLE-QIs). METHODS: International consensus has been achieved for childhood-onset SLE-QIs capturing medical care provision in 9 domains: diagnostic testing, education of cardiovascular (CV) risk and lifestyles, lupus nephritis (LN), medication management, bone health, ophthalmologic surveillance, transition, pregnancy, and vaccination...
February 2016: Arthritis Care & Research
Rina Mina, Khalid Abulaban, Marisa S Klein-Gitelman, Barbara A Eberhard, Stacy P Ardoin, Nora Singer, Karen Onel, Lori Tucker, Kathleen O'neil, Tracey Wright, Elizabeth Brooks, Kelly Rouster-Stevens, Lawrence Jung, Lisa Imundo, Brad Rovin, David Witte, Jun Ying, Hermine I Brunner
OBJECTIVE: To validate clinical indices of lupus nephritis activity and damage when used in children against the criterion standard of kidney biopsy findings. METHODS: In 83 children requiring kidney biopsy, the Systemic Lupus Erythematosus Disease Activity Index renal domain (SLEDAI-R), British Isles Lupus Assessment Group index renal domain (BILAG-R), Systemic Lupus International Collaborating Clinics (SLICC) renal activity score (SLICC-RAS), and SLICC Damage Index renal domain (SDI-R) were measured...
February 2016: Arthritis Care & Research
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
J T Jones, M DiFrancesco, A I Zaal, M S Klein-Gitelman, D Gitelman, J Ying, H I Brunner
OBJECTIVES: The objective of this report is to use diffusion-tensor imaging (DTI) for investigating white-matter connectivity changes associated with neurocognitive dysfunction in childhood-onset lupus (cSLE-NCD) as measured by formal neuropsychological testing. METHODS: DTI was performed in six individuals with (cSLE-NCD) and nine without neurocognitive dysfunction (cSLE-noNCD) as well as 14 healthy controls. Presence of neurocognitive deficits was identified by formal neuropsychological testing...
September 2015: Lupus
Hermine I Brunner, Clovis A Silva, Andreas Reiff, Gloria C Higgins, Lisa Imundo, Calvin B Williams, Carol A Wallace, Nadia E Aikawa, Shannen Nelson, Marisa S Klein-Gitelman, Susan R Rose
OBJECTIVE: To determine the dose of triptorelin that is sufficient to maintain complete ovarian suppression in female patients with childhood-onset systemic lupus erythematosus (SLE) who require cyclophosphamide therapy, to determine the length of time needed to achieve ovarian suppression after initiation of triptorelin treatment, and to investigate the safety of triptorelin. METHODS: In this randomized, double-blind, placebo-controlled, dose-escalation study, female patients ages <21 years were randomized 4:1 to receive triptorelin (n = 25) or placebo (n = 6)...
May 2015: Arthritis & Rheumatology
Lisa M Arkin, Leah Ansell, Alfred Rademaker, Megan L Curran, Michael L Miller, Annette Wagner, Brandi M Kenner-Bell, Sarah L Chamlin, Anthony J Mancini, Marisa Klein-Gitelman, Amy S Paller
BACKGROUND: Pediatric discoid lupus erythematosus (DLE) is rare. The risk of progression to systemic lupus erythematosus (SLE) is uncertain. OBJECTIVE: We sought to determine the risk of progression of pediatric DLE to SLE and to characterize its phenotype. METHODS: This was a retrospective review of 40 patients with DLE. RESULTS: Six (15%) of 40 patients presented with DLE as a manifestation of concurrent SLE. Of the remaining 34, 9 (26%) eventually met SLE criteria and 15 (44%) developed laboratory abnormalities without meeting SLE criteria...
April 2015: Journal of the American Academy of Dermatology
Hermine I Brunner, Marisa S Klein-Gitelman, Frank Zelko, Dean W Beebe, Dirk Foell, Jiha Lee, Ahmad Zaal, Jordan Jones, Tresa Roebuck-Spencer, Jun Ying
OBJECTIVE: To examine select brain-reactive proteins for their usefulness to serve as blood-based biomarkers in the screening for neurocognitive deficits in childhood-onset systemic lupus erythematosus (cSLE-NCD). METHODS: Patients withcSLE (n=40) were studied longitudinally (month 1; month 18): working memory, psychomotor speed and visuoconstructional ability were assessed using formal neurocognitive testing to determine the presence of cSLE-NCD. Patients also completed the computerised Paediatric Automated Neuropsychological Assessment Metrics...
2014: Lupus Science & Medicine
R Mina, M S Klein-Gitelman, S Nelson, B A Eberhard, G Higgins, N G Singer, K Onel, L Tucker, K M O'Neil, M Punaro, D M Levy, K Haines, J Ying, H I Brunner
OBJECTIVE: This study evaluated the effects of obesity on health-related quality of life (HRQOL) measures in juvenile-onset systemic lupus erythematosus (jSLE). METHODS: Obesity was defined as a body mass index (BMI) ≥ 95 th percentile according to the Sex-specific Center for Disease Control BMI-For-Age Charts and determined in a multicenter cohort of jSLE patients. In this secondary analysis, the domain and summary scores of the Pediatric Quality of Life (PedsQL) Inventory and the Child Health Questionnaire (CHQ) of obese jSLE patients were compared to those of non-obese jSLE patients as well as historical obese and non-obese healthy controls...
February 2015: Lupus
Sarah Ringold, Audrey Hendrickson, Leslie Abramson, Timothy Beukelman, Peter R Blier, John Bohnsack, Elizabeth C Chalom, Harry L Gewanter, Beth Gottlieb, Roger Hollister, Joyce Hsu, Andrea Hudgins, Norman T Ilowite, Marisa Klein-Gitelman, Carol Lindsley, Jorge M Lopez Benitez, Daniel J Lovell, Tom Mason, Diana Milojevic, Lakshmi N Moorthy, Kabita Nanda, Karen Onel, Sampath Prahalad, C Egla Rabinovich, Linda Ray, Kelly Rouster-Stevens, Natasha Ruth, Michael Shishov, Steven Spalding, Reema Syed, Matthew Stoll, Richard K Vehe, Jennifer E Weiss, Andrew J White, Carol A Wallace, Rachel E Sobel
OBJECTIVE: Few data are available regarding the rates of serious adverse events (SAEs) and important medical events (IMEs) outside of product-based registries and clinical trials for juvenile idiopathic arthritis (JIA). The Enhanced Drug Safety Surveillance Project (EDSSP) was developed to pilot a novel system to collect SAEs/IMEs in children with JIA. This analysis reports the results from this 4-year (2008-2012) EDSSP. METHODS: Participating physicians were surveyed monthly to ascertain whether their JIA patients experienced an SAE or IME...
April 2015: Arthritis Care & Research
Marek Bohm, Maria Isabel Gonzalez Fernandez, Seza Ozen, Angela Pistorio, Pavla Dolezalova, Paul Brogan, Giancarlo Barbano, Claudia Sengler, Marisa Klein-Gitelman, Pierre Quartier, Anders Fasth, Troels Herlin, Maria Teresa R A Terreri, Susan Nielsen, Marion A J van Rossum, Tadej Avcin, Esteban Rodolfo Castell, Ivan Foeldvari, Dirk Foell, Anuela Kondi, Isabelle Koné-Paut, Rolf-Michael Kuester, Hartmut Michels, Nico Wulffraat, Halima Ben Amer, Clara Malattia, Alberto Martini, Nicolino Ruperto
BACKGROUND: Granulomatosis with polyangiitis (GPA), formerly known as Wegener's granulomatosis (WG), belongs to the group of ANCA-associated necrotizing vasculitides. This study describes the clinical picture of the disease in a large cohort of GPA paediatric patients. Children with age at diagnosis ≤ 18 years, fulfilling the EULAR/PRINTO/PRES GPA/WG classification criteria were extracted from the PRINTO vasculitis database. The clinical signs/symptoms and laboratory features were analysed before or at the time of diagnosis and at least 3 months thereafter and compared with other paediatric and adult case series (>50 patients) derived from the literature...
2014: Pediatric Rheumatology Online Journal
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
Patricia Vega-Fernandez, Frank A Zelko, Marisa Klein-Gitelman, Jiha Lee, Jessica Hummel, Shannen Nelson, Erin C Thomas, Jun Ying, Dean W Beebe, Hermine I Brunner
OBJECTIVE: To investigate the utility of questionnaire-based assessment of cognitive function and behavioral/emotional symptoms to screen for neurocognitive dysfunction in childhood-onset systemic lupus erythematosus (cSLE). METHODS: Forty children with cSLE and 24 healthy controls ages 10–16 years were enrolled. Formal neurocognitive testing (FNCT) was done to determine cognitive performance in 4 key areas that appear to be sensitive to the adverse effects of cSLE: attention, working memory, psychomotor speed, and visuoconstructional ability...
June 2014: Arthritis Care & Research
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