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1,044 results on '"Laxer, Ronald M."'

2. Paediatric inflammatory multisystem syndrome in Canada: population-based surveillance and role of SARS-CoV-2 linkage

Author

El Tal, Tala, Morin, Marie-Paule, Morris, Shaun K., Farrar, Daniel S., Berard, Roberta A., Kakkar, Fatima, Moore Hepburn, Charlotte, Baerg, Krista, Beaufils, Camille, Bennett, Terri-Lyn, Benseler, Susanne M., Beaudoin-Bussières, Guillaume, Chan, Kevin, Cyr, Claude, Dahdah, Nagib, Donner, Elizabeth J., Drouin, Olivier, Edjoc, Rojiemiahd, Eljaouhari, Maryem, Embree, Joanne E., Farrell, Catherine, Finzi, Andrés, Forgie, Sarah, Giroux, Ryan, Kang, Kristopher T., King, Melanie, Laffin Thibodeau, Melanie, Lang, Bianca, Laxer, Ronald M., Luu, Thuy Mai, McCrindle, Brian W., Orkin, Julia, Papenburg, Jesse, Pound, Catherine M., Price, Victoria E., Proulx-Gauthier, Jean-Philippe, Purewal, Rupeena, Sadarangani, Manish, Salvadori, Marina I., Thibeault, Roseline, Top, Karina A., Viel-Thériault, Isabelle, Haddad, Elie, Scuccimarri, Rosie, and Yeung, Rae S. M.

Published
2023
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3. SARS-CoV-2 infection in technology-dependent children: a multicenter case series

Author

Robinson, Joan, Dewan, Tammie, Morris, Shaun K., Bitnun, Ari, Gill, Peter, Tal, Tala El, Laxer, Ronald M., Yeh, E. Ann, Yea, Carmen, Ulloa-Gutierrez, Rolando, Brenes-Chacon, Helena, Yock-Corrales, Adriana, Ivankovich-Escoto, Gabriela, Soriano-Fallas, Alejandra, Mezerville, Marcela Hernandez-de, Papenburg, Jesse, Lefebvre, Marie-Astrid, Nateghian, Alireza, Aski, Behzad Haghighi, Manafi, Ali, Dwilow, Rachel, Bullard, Jared, Cooke, Suzette, Restivo, Lea, Lopez, Alison, Sadarangani, Manish, Roberts, Ashley, Le Saux, Nicole, Bowes, Jennifer, Purewal, Rupeena, Lautermilch, Janell, Wong, Jacqueline K., Piche, Dominique, Top, Karina A., Foo, Cheryl, Panetta, Luc, Merckx, Joanna, and Barton, Michelle

Published
2023
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4. Capturing the Range of Disease Involvement in Localized Scleroderma: The Localized Scleroderma Total Severity Scale

Author

Li, Suzanne C., Rabinovich, C. Egla, Becker, Mara L., Torok, Kathryn S., Ferguson, Polly J., Dedeoglu, Fatma, Hong, Sandy, Sivaraman, Vidya, Laxer, Ronald M., Stewart, Katie, Ibarra, Maria F., Mason, Thomas, II, Higgins, Gloria, Pope, Elena, Li, Xiaohu, Lozy, Tara, Fuhlbrigge, Robert C., Anderson, E., Benham, H., Feldman, B., Francis, K., Goh, I., Jaquith, J., SchollaertFitch, K., Smith, C., Weiss, J., and Wooton, J.

Published
2024
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6. Pediatric Rheumatology Care and Outcomes Improvement Networkʼs Quality Measure Set to Improve Care of Children With Juvenile Idiopathic Arthritis

Author

Bingham, Catherine A., Harris, Julia G., Qiu, Tingting, Gilbert, Mileka, Vora, Sheetal S., Yildirim‐Toruner, Cagri, Ferraro, Kerry, Lovell, Daniel J., Taylor, Janalee, Mannion, Melissa L., Weiss, Jennifer E., Laxer, Ronald M., Shishov, Michael, Oberle, Edward J., Gottlieb, Beth S., Lee, Tzielan C., Pan, Nancy, Burnham, Jon M., Fair, Danielle C., Batthish, Michelle, Hazen, Melissa M., Spencer, Charles H., and Morgan, Esi M.

Published
2023
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7. Distinct interferon signatures and cytokine patterns define additional systemic autoinflammatory diseases

Author

de Jesus, Adriana A, Hou, Yangfeng, Brooks, Stephen, Malle, Louise, Biancotto, Angelique, Huang, Yan, Calvo, Katherine R, Marrero, Bernadette, Moir, Susan, Oler, Andrew J, Deng, Zuoming, Montealegre Sanchez, Gina A, Ahmed, Amina, Allenspach, Eric, Arabshahi, Bita, Behrens, Edward, Benseler, Susanne, Bezrodnik, Liliana, Bout-Tabaku, Sharon, Brescia, AnneMarie C, Brown, Diane, Burnham, Jon M, Caldirola, Maria Soledad, Carrasco, Ruy, Chan, Alice Y, Cimaz, Rolando, Dancey, Paul, Dare, Jason, DeGuzman, Marietta, Dimitriades, Victoria, Ferguson, Ian, Ferguson, Polly, Finn, Laura, Gattorno, Marco, Grom, Alexei A, Hanson, Eric P, Hashkes, Philip J, Hedrich, Christian M, Herzog, Ronit, Horneff, Gerd, Jerath, Rita, Kessler, Elizabeth, Kim, Hanna, Kingsbury, Daniel J, Laxer, Ronald M, Lee, Pui Y, Lee-Kirsch, Min Ae, Lewandowski, Laura, Li, Suzanne, Lilleby, Vibke, Mammadova, Vafa, Moorthy, Lakshmi N, Nasrullayeva, Gulnara, O'Neil, Kathleen M, Onel, Karen, Ozen, Seza, Pan, Nancy, Pillet, Pascal, Piotto, Daniela Gp, Punaro, Marilynn G, Reiff, Andreas, Reinhardt, Adam, Rider, Lisa G, Rivas-Chacon, Rafael, Ronis, Tova, Rösen-Wolff, Angela, Roth, Johannes, Ruth, Natasha Mckerran, Rygg, Marite, Schmeling, Heinrike, Schulert, Grant, Scott, Christiaan, Seminario, Gisella, Shulman, Andrew, Sivaraman, Vidya, Son, Mary Beth, Stepanovskiy, Yuriy, Stringer, Elizabeth, Taber, Sara, Terreri, Maria Teresa, Tifft, Cynthia, Torgerson, Troy, Tosi, Laura, Van Royen-Kerkhof, Annet, Wampler Muskardin, Theresa, Canna, Scott W, and Goldbach-Mansky, Raphaela

Subjects
Genetics, Clinical Research, Rare Diseases, 2.1 Biological and endogenous factors, Aetiology, Autoimmune Diseases, Female, Humans, Interferon Type I, Interleukin-18, Macrophage Activation Syndrome, Male, Mutation, Panniculitis, Pulmonary Alveolar Proteinosis, Genetic diseases, Immunology, Inflammation, Innate immunity, Monogenic diseases, Medical and Health Sciences
Abstract

BACKGROUNDUndifferentiated systemic autoinflammatory diseases (USAIDs) present diagnostic and therapeutic challenges. Chronic interferon (IFN) signaling and cytokine dysregulation may identify diseases with available targeted treatments.METHODSSixty-six consecutively referred USAID patients underwent underwent screening for the presence of an interferon signature using a standardized type-I IFN-response-gene score (IRG-S), cytokine profiling, and genetic evaluation by next-generation sequencing.RESULTSThirty-six USAID patients (55%) had elevated IRG-S. Neutrophilic panniculitis (40% vs. 0%), basal ganglia calcifications (46% vs. 0%), interstitial lung disease (47% vs. 5%), and myositis (60% vs. 10%) were more prevalent in patients with elevated IRG-S. Moderate IRG-S elevation and highly elevated serum IL-18 distinguished 8 patients with pulmonary alveolar proteinosis (PAP) and recurrent macrophage activation syndrome (MAS). Among patients with panniculitis and progressive cytopenias, 2 patients were compound heterozygous for potentially novel LRBA mutations, 4 patients harbored potentially novel splice variants in IKBKG (which encodes NF-κB essential modulator [NEMO]), and 6 patients had de novo frameshift mutations in SAMD9L. Of additional 12 patients with elevated IRG-S and CANDLE-, SAVI- or Aicardi-Goutières syndrome-like (AGS-like) phenotypes, 5 patients carried mutations in either SAMHD1, TREX1, PSMB8, or PSMG2. Two patients had anti-MDA5 autoantibody-positive juvenile dermatomyositis, and 7 could not be classified. Patients with LRBA, IKBKG, and SAMD9L mutations showed a pattern of IRG elevation that suggests prominent NF-κB activation different from the canonical interferonopathies CANDLE, SAVI, and AGS.CONCLUSIONSIn patients with elevated IRG-S, we identified characteristic clinical features and 3 additional autoinflammatory diseases: IL-18-mediated PAP and recurrent MAS (IL-18PAP-MAS), NEMO deleted exon 5-autoinflammatory syndrome (NEMO-NDAS), and SAMD9L-associated autoinflammatory disease (SAMD9L-SAAD). The IRG-S expands the diagnostic armamentarium in evaluating USAIDs and points to different pathways regulating IRG expression.TRIAL REGISTRATIONClinicalTrials.gov NCT02974595.FUNDINGThe Intramural Research Program of the NIH, NIAID, NIAMS, and the Clinical Center.

Published
2020

8. Infants hospitalized for acute COVID-19: disease severity in a multicenter cohort study

Author

Merckx, Joanna, Morris, Shaun K., Bitnun, Ari, Gill, Peter, El Tal, Tala, Laxer, Ronald M., Yeh, Ann, Yea, Carmen, Ulloa-Gutierrez, Rolando, Brenes-Chacon, Helena, Yock-Corrales, Adriana, Ivankovich-Escoto, Gabriela, Soriano-Fallas, Alejandra, Hernandez-de Mezerville, Marcela, Papenburg, Jesse, Lefebvre, Marie-Astrid, Nateghian, Alireza, Haghighi Aski, Behzad, Manafi, Ali, Dwilow, Rachel, Bullard, Jared, Cooke, Suzette, Dewan, Tammie, Restivo, Lea, Lopez, Alison, Sadarangani, Manish, Roberts, Ashley, Barton, Michelle, Petel, Dara, Le Saux, Nicole, Bowes, Jennifer, Purewal, Rupeena, Lautermilch, Janell, Tehseen, Sarah, Bayliss, Ann, Wong, Jacqueline K., Viel-Thériault, Isabelle, Piche, Dominique, Top, Karina A., Leifso, Kirk, Foo, Cheryl, Panetta, Luc, and Robinson, Joan

Published
2022
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12. Gain-of-function variants in SYK cause immune dysregulation and systemic inflammation in humans and mice

Author

Wang, Lin, Aschenbrenner, Dominik, Zeng, Zhiyang, Cao, Xiya, Mayr, Daniel, Mehta, Meera, Capitani, Melania, Warner, Neil, Pan, Jie, Wang, Liren, Li, Qi, Zuo, Tao, Cohen-Kedar, Sarit, Lu, Jiawei, Ardy, Rico Chandra, Mulder, Daniel J., Dissanayake, Dilan, Peng, Kaiyue, Huang, Zhiheng, Li, Xiaoqin, Wang, Yuesheng, Wang, Xiaobing, Li, Shuchao, Bullers, Samuel, Gammage, Anís N., Warnatz, Klaus, Schiefer, Ana-Iris, Krivan, Gergely, Goda, Vera, Kahr, Walter H. A., Lemaire, Mathieu, Lu, Chien-Yi, Siddiqui, Iram, Surette, Michael G., Kotlarz, Daniel, Engelhardt, Karin R., Griffin, Helen R., Rottapel, Robert, Decaluwe, Hélène, Laxer, Ronald M., Proietti, Michele, Hambleton, Sophie, Elcombe, Suzanne, Guo, Cong-Hui, Grimbacher, Bodo, Dotan, Iris, Ng, Siew C., Freeman, Spencer A., Snapper, Scott B., Klein, Christoph, Boztug, Kaan, Huang, Ying, Li, Dali, Uhlig, Holm H., and Muise, Aleixo M.

Published
2021
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13. Using a collaborative learning health system approach to improve disease activity outcomes in children with juvenile idiopathic arthritis in the Pediatric Rheumatology Care and Outcomes Improvement Network.

Author

Harris, Julia G., Bingham, Catherine A., Vora, Sheetal S., Yildirim-Toruner, Cagri, Batthish, Michelle, Bullock, Danielle R., Burnham, Jon M., Fair, Danielle C., Ferraro, Kerry, Ganguli, Suhas, Gilbert, Mileka, Gottlieb, Beth S., Halyabar, Olha, Hazen, Melissa M., Laxer, Ronald M., Lee, Tzielan C., Liu, Alice, Lovell, Daniel J., Mannion, Melissa L., and Oberle, Edward J.

Published
2024
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24. Overlap Syndromes

Author

Laxer, Ronald M., Sherry, David D., Hashkes, Philip J., Laxer, Ronald M., Sherry, David D., and Hashkes, Philip J.

Published
2016
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25. Lyme Arthritis

Author

Laxer, Ronald M., Sherry, David D., Hashkes, Philip J., Laxer, Ronald M., Sherry, David D., and Hashkes, Philip J.

Published
2016
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26. Vasculitis

Author

Laxer, Ronald M., Sherry, David D., Hashkes, Philip J., Laxer, Ronald M., Sherry, David D., and Hashkes, Philip J.

Published
2016
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38. Organizational Learning in the Morbidity and Mortality Conference.

Author

Batthish, Michelle, Kuper, Ayelet, Fine, Claire, Laxer, Ronald M., and Baker, G. Ross

Abstract

Introduction: The focus of morbidity and mortality conferences (M&MCs) has shifted to emphasize quality improvement and systems-level care. However, quality improvement initiatives targeting systems-level errors are challenged by learning in M&MCs, which occurs at the individual attendee level and not at the organizational level. Here, we aimed to describe how organizational learning in M&MCs is optimized by particular organizational and team cultures. Methods: A prospective, multiple-case study design was used. Using purposive sampling, three cases covering different medical/ surgical specialties in North America were chosen. Data collection included direct observations of the M&MC, semistructured interviews with key M&MC members, and documentary information. Results: The role of the M&MC in all cases integrated two key concepts: recognition of system-wide trends and learning from error, at an organizational and team level. All cases provided evidence of double-loop learning and used organizational memory strategies to ensure knowledge was retained within the organization. A patient safety culture was linked to the promotion of open communication, fostering learning from adverse events. Conclusion: This study describes three cases of systems-oriented M&MCs that reflected elements of organizational learning theory. The M&MC can therefore provide a context for organizational learning, allowing optimal learning from adverse events. [ABSTRACT FROM AUTHOR]

Published
2024
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39. Systematic Review of Health‐Related Quality of Life Impact in Juvenile Localized Scleroderma.

Author

Sanchez‐Espino, Luis F., Luca, Nadia, Pope, Elena, Laxer, Ronald M., Knight, Andrea M., and Sibbald, Cathryn

Subjects
QUALITY of life, CINAHL database, SCLERODERMA (Disease), MINORITY stress, CHILD patients
Abstract

Objective: The prevalence and types of psychosocial complications of juvenile localized scleroderma (JLS), also known as morphea, an inflammatory and sclerosing disease involving the skin, fascia, muscle, and bone, are poorly understood. Methods: We performed a systematic review of literature published between 2000 and 2020 in PubMed, EMBASE, the Cochrane Skin Group Specialized Register, the Cochrane Central Register of Controlled Trials, and the Cumulative Index to Nursing and Allied Health Literature using the search terms "scleroderma, localized," "Morphea," "anxiety," "depression," "resilience," "social stigma," "quality of life," "mood," or "stress" and limited the search to pediatric patients and English language. Patient demographics, characteristics of JLS, and comorbidities were extracted. The outcomes included measures of health‐related quality of life (HRQoL), psychosocial functioning, evaluation of self‐perception, and the treatment burden of the study population. The protocol was registered with PROSPERO (CRD42021257124). Thematic synthesis generated descriptive analysis. Results: Thirteen studies fulfilled the inclusion criteria: three retrospective cohort studies, two prospective cohort studies, and eight cross‐sectional studies. A total of 690 pediatric patients with JLS were included (n = 484 with linear scleroderma). Six studies used the Children's Dermatology Life Quality Index, reporting little to no effect on HRQoL. One study used the Health‐Related Quality of Life in Children and Adolescents Questionnaire and did not find differences between children with JLS or atopic dermatitis and healthy controls. One study used a self‐perception questionnaire that showed normal self‐worth of patients with JLS. Two studies used focus groups, both reporting elevated levels of stress, decreased self‐worth, "feeling different," and bullying/teasing in patients with JLS. These emotions were associated with skin symptoms (pain, itch, and tightness), physical limitations, and treatment burden. Conclusion: Overall, quantitative studies did not report a statistically significant impairment in HRQoL in JLS. However, qualitative studies (focus groups) reported significant psychosocial impacts related to JLS. There is a need to develop a JLS‐specific tool for the HRQoL evaluation of this population. [ABSTRACT FROM AUTHOR]

Published
2024
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42. A decade of progress in juvenile idiopathic arthritis treatments and outcomes in Canada: results from ReACCh-Out and the CAPRI registry

Author

Nguyen, Kelly, primary, Barsalou, Julie, additional, Basodan, Daniah, additional, Batthish, Michelle, additional, Benseler, Susanne M, additional, Berard, Roberta A, additional, Blanchette, Nicholas, additional, Boire, Gilles, additional, Bolaria, Roxana, additional, Bruns, Alessandra, additional, Cabral, David A, additional, Cameron, Bonnie, additional, Campillo, Sarah, additional, Cellucci, Tania, additional, Chan, Mercedes, additional, Chédeville, Gaëlle, additional, Chetaille, Anne-Laure, additional, Chhabra, Amieleena, additional, Couture, Julie, additional, Dancey, Paul, additional, De Bruycker, Jean-Jacques, additional, Demirkaya, Erkan, additional, Dhalla, Muhammed, additional, Duffy, Ciarán M, additional, Feldman, Brian M, additional, Feldman, Debbie E, additional, Gerschman, Tommy, additional, Haddad, Elie, additional, Heale, Liane, additional, Herrington, Julie, additional, Houghton, Kristin, additional, Huber, Adam M, additional, Human, Andrea, additional, Johnson, Nicole, additional, Jurencak, Roman, additional, Lang, Bianca, additional, Larché, Maggie, additional, Laxer, Ronald M, additional, LeBlanc, Claire M, additional, Lee, Jennifer J Y, additional, Levy, Deborah M, additional, Lim, Lillian, additional, Lim, Lily S H, additional, Luca, Nadia, additional, McGrath, Tara, additional, McMillan, Tamara, additional, Miettunen, Paivi M, additional, Morishita, Kimberly A, additional, Ng, Hon Yan, additional, Oen, Kiem, additional, Park, Jonathan, additional, Petty, Ross E, additional, Proulx-Gauthier, Jean-Philippe, additional, Ramsey, Suzanne, additional, Roth, Johannes, additional, Rosenberg, Alan M, additional, Rozenblyum, Evelyn, additional, Rumsey, Dax G, additional, Schmeling, Heinrike, additional, Schneider, Rayfel, additional, Scuccimarri, Rosie, additional, Shiff, Natalie J, additional, Silverman, Earl, additional, Soon, Gordon, additional, Spiegel, Lynn, additional, Stringer, Elizabeth, additional, Tam, Herman, additional, Tse, Shirley M, additional, Tucker, Lori B, additional, Turvey, Stuart, additional, Twilt, Marinka, additional, Duffy, Karen Watanabe, additional, Yeung, Rae S M, additional, and Guzman, Jaime, additional

Published
2023
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44. Lengthening sleep reduces pain in childhood arthritis: a crossover randomised controlled trial

Author

Clairman, Hayyah, primary, Dover, Saunya, additional, Tomlinson, George, additional, Beebe, Dean, additional, Cameron, Bonnie, additional, Laxer, Ronald M, additional, Levy, Deborah, additional, Narang, Indra, additional, Paetkau, Susan, additional, Schneider, Rayfel, additional, Spiegel, Lynn, additional, Stephens, Samantha, additional, Stinson, Jennifer, additional, Tse, Shirley, additional, Weiss, Shelly, additional, Whitney, Kristi, additional, and Feldman, Brian M, additional

Published
2023
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