Explanipedia

Functional genomics deciphers pathogenic B cell dynamics in lupus bone marrow 3662 Open

Mariasilvia Colantuoni, Marcos Chiñas, Holly Wobma, Peter A. Nigrović, Lauren A. Henderson , et al. · 2025

Description Systemic lupus erythematosus (SLE) is an autoimmune disease involving immune and non-immune cells, with complex pathogenesis and limited therapies. Dysregulated B cells, particularly double-negative 2 (DN2) B cells (CD19?, CD27…

Treating and triggering hyperinflammation: tackling hemophagocytic lymphohistiocytosis and HLH-like syndromes in the pediatric cell therapy and critical care setting Open

Holly Wobma, Lauren A. Henderson, Christine Duncan, Susan E. Prockop, Adrienne G. Randolph , et al. · 2025

Hemophagocytic lymphohistiocytosis (HLH) describes a severe, hyperinflammatory syndrome that can originate from diverse etiologies, often requiring critical care level management. Primary HLH, initially described in the 1940s, derives from…

Precision Editing of Cyclophilin A Generates Cyclosporine and Voclosporin Resistant Cellular Therapies Open

Holly Wobma, Francesca Alvarez Calderon, Jiayi Dong, Kayleigh Ingersoll Omdahl, Xianliang Rui , et al. · 2025

Patients who are recipients of allogeneic transplants or have underlying autoimmune disease require immune suppression, often with calcineurin inhibitors (CNI). There is an expanding repertoire of immune effector cell (IEC) therapies, incl…

Sex Differences in B Cells From the Joints of Children With Oligoarticular Juvenile Idiopathic Arthritis Open

Ki Pui Lam, Claudia R. Harris, Maria L. Taylor, Daniela Fernandez‐Salinas, Jing Cui , et al. · 2025

Objective Disordered T peripheral helper (Tph)–B cell interactions have been implicated in several forms of inflammatory arthritis, including oligoarticular (oligo) juvenile idiopathic arthritis (JIA). We sought to evaluate the Tph–B cell …

Severe Features of Systemic Juvenile Idiopathic Arthritis in Patients With Congenital Heart Disease Open

Bárbara Pontes Aires, Holly Wobma, Aaida Samad, Mia T. Chandler, Margaret H. Chang , et al. · 2024

Objective To describe the clinical features of patients with congenital heart disease (CHD) who subsequently developed systemic juvenile idiopathic arthritis (sJIA). Methods We conducted a retrospective review of patients diagnosed with CH…

Innovations in the Care of chILD Associated with Connective Tissue Disease and Immune Mediated Disorders Open

Aaida Samad, Holly Wobma, Alicia Casey · 2023

Childhood interstitial lung disease associated with connective tissue disease and immune mediated is the second most common chILD diagnostic category. As knowledge of the molecular and genetic underpinnings of these rare disorders advances…

Type I interferon signature and cycling lymphocytes in macrophage activation syndrome Open

Zhengping Huang, Kailey Brodeur, Liang Chen, Du, Holly Wobma , et al. · 2023

BACKGROUNDMacrophage activation syndrome (MAS) is a life-threatening complication of Still's disease (SD) characterized by overt immune cell activation and cytokine storm. We aimed to further understand the immunologic landscape of SD and …

Development of a Screening Algorithm for Lung Disease in Systemic Juvenile Idiopathic Arthritis Open

Holly Wobma, Ronny Bachrach, Joseph B. Farrell, Margaret H. Chang, Megan Day‐Lewis , et al. · 2023

Objective Lung disease (LD) is an increasingly recognized complication of systemic juvenile idiopathic arthritis (sJIA). As there are no currently available guidelines for pulmonary screening in sJIA, we sought to develop such an algorithm…

Real-world experience with low-dose IL-2 for children and young adults with refractory chronic graft-versus-host disease Open

Holly Wobma, Malika Kapadia, Haesook T. Kim, Francesca Alvarez‐Calderon, Susanne H.C. Baumeister , et al. · 2023

The majority of patients with chronic graft-versus-host disease (cGVHD) are steroid refractory (SR), creating a need for safe and effective therapies. Subcutaneous low-dose interleukin-2 (LD IL-2), which preferentially expands CD4+ regulat…

Incidence and Risk Factors for Eosinophilia and Lung Disease in <span>Biologic‐Exposed</span> Children With Systemic Juvenile Idiopathic Arthritis Open

Holly Wobma, Sage R. Arvila, Maria L. Taylor, Ki Pui Lam, M. Ohashi , et al. · 2023

Objective Although interleukin‐1 (IL‐1)/IL‐6 inhibitors are effective therapies for systemic juvenile idiopathic arthritis (JIA), some patients develop eosinophilia and lung disease during treatment. This study was undertaken to retrospect…

Disordered T cell-B cell interactions in autoantibody-positive inflammatory arthritis Open

Amélie M. Julé, Ki Pui Lam, Maria L. Taylor, Kacie J. Hoyt, Kevin Wei , et al. · 2023

T peripheral helper (Tph) cells, identified in the synovium of adults with seropositive rheumatoid arthritis, drive B cell maturation and antibody production in non-lymphoid tissues. We sought to determine if similarly dysregulated T cell-…

Dysregulation of the cGAS-STING Pathway in Monogenic Autoinflammation and Lupus Open

Holly Wobma, Daniel S. Shin, Janet Chou, Fatma Dedeoğlu · 2022

One of the oldest mechanisms of immune defense against pathogens is through detection of foreign DNA. Since human DNA is compartmentalized into the nucleus, its presence in the cytosol heralds a potential threat. The cGAS-STING pathway is …

Genetic diagnosis of immune dysregulation can lead to targeted therapy for interstitial lung disease: A case series and single center approach Open

Holly Wobma, R. Perkins, Lisa M. Bartnikas, Fatma Dedeoğlu, Janet Chou , et al. · 2022

In recent years, a growing number of monogenic disorders have been described that are characterized by immune dysregulation. A subset of these “primary immune regulatory disorders” can cause severe interstitial lung disease, often recogniz…

Features predicting colchicine efficacy in treatment of children with undefined systemic autoinflammatory disease: A retrospective cohort study Open

Mariana Correia Marques, Buğra Han Egeli, Holly Wobma, Claudio Henrique T. Ribeiro, Edwin L. Anderson , et al. · 2022

Objective: Patients with undefined systemic autoinflammatory diseases (uSAIDs) are challenging to manage, as there are no guidelines or recommendations for targeted therapy. We aimed to evaluate the efficacy of empiric treatment with colch…

Patients with monogenic immune dysregulation show improvement in childhood interstitial lung disease following targeted therapy -- a case series and single center approach Open

Holly Wobma, R. Perkins, Lisa M. Bartnikas, Fatma Dedeoğlu, Janet Chou , et al. · 2021

In recent years, a number of monogenic disorders have been described that are characterized by immune dysregulation. A subset of these ‘primary immune regulatory disorders’ can cause severe interstitial lung disease, often recognized in la…

Risk Factors, Clinical Outcomes, and Cost-of-Care Related to Graft Failure in Pediatric Allogeneic Hematopoietic Cell Transplant Recipients Open

Holly Wobma, Zhezhen Jin, Susana Moscoso, Monica Bhatia, Larisa Broglie , et al. · 2020

Allogeneic hematopoietic cell transplantation (HCT) has the capacity to cure numerous malignant and nonmalignant disorders. A dreaded complication is graft failure (GF), as it puts patients at high risk of infection and disease relapse. Th…

When Local Bone Pain Is Just the Tip of the Iceberg—A Case Report of Three Patients With Chronic Multifocal Recurrent Osteomyelitis and Some Red Flags to Help Make the Diagnosis Open

Holly Wobma, Diego Jaramillo, Lisa F. Imundo · 2019

Chronic recurrent multifocal osteomyelitis (CRMO) is an uncommon cause of chronic inflammatory bone pain in children that can be disabling. Often, this diagnosis is considered only after a prolonged workup, leading to frustration for famil…

Engineering of human cardiac muscle electromechanically matured to an adult-like phenotype Open

Kacey Ronaldson-Bouchard, Keith Yeager, Diogo Teles, Timothy Chen, Stephen Ma , et al. · 2019

The application of tissue-engineering approaches to human induced pluripotent stem (hiPS) cells enables the development of physiologically relevant human tissue models for in vitro studies of development, regeneration, and disease. However…

Regeneration of severely damaged lungs using an interventional cross-circulation platform Open

Brandon A. Guenthart, John D. O’Neill, Jin-Ho Kim, Dawn Queen, Scott Chicotka , et al. · 2019

The number of available donor organs limits lung transplantation, the only lifesaving therapy for the increasing population of patients with end-stage lung disease. A prevalent etiology of injury that renders lungs unacceptable for transpl…

Dual IFN-γ/hypoxia priming enhances immunosuppression of mesenchymal stromal cells through regulatory proteins and metabolic mechanisms Open

Holly Wobma, Mariko Kanai, P. Stephen, Ying Shih, Hao Wei Li , et al. · 2018

Interferon-gamma/Hypoxia Primed Mesenchymal Stem Cells for an Improved Immunosuppressive Cell Therapy Open

Holly Wobma · 2018

Mesenchymal stem cells (MSCs) are promising candidates for treating diverse inflammatory disorders due to their capacity to be immunosuppressive. This phenotype is not present at baseline but develops in response to instructive cues. To da…

Functional vascularized lung grafts for lung bioengineering Open

N. Valerio Dorrello, Brandon A. Guenthart, John D. O’Neill, Jin-Ho Kim, Katherine Cunningham , et al. · 2017

We propose a new approach to lung regeneration by replacement of damaged epithelium with full preservation of lung vasculature.

909 Developing a 3D microfluidic model of immune-competent human skin using autologous cells Open

Hasan Erbil Abaci, Holly Wobma, Y. Doucet, Jung U Shin, J. Jackow , et al. · 2017

Preconditioning Mesenchymal Stem Cells to Improve Transplant Tolerance Open

Holly Wobma, Mariko Kanai, P. Stephen, Kenneth R. Nakazawa, Raimon Duran‐Struuck , et al. · 2016

Mesenchymal stem cells (MSCs) are being increasingly recognized for their hypoimmunogenic and immunosuppressive properties. These include the expression of a wide array of surface and secreted proteins, which inhibit CD4+, CD8+, and NK cel…

Protection of Organ Vasculature By Endothelial Overexpression of HLA-G Open

Holly Wobma, P. Stephen, Jennifer S. Fang, Hema Vasavada, Raimon Duran‐Struuck , et al. · 2016

Solid organ transplantation is a definitive treatment for organ failure. While great progress has been made in preventing and treating acute rejection, transplantation is still limited by chronic rejection. Extending the donor pool to orga…

Tissue Engineering and Regenerative Medicine 2015: A Year in Review Open

Holly Wobma, Gordana Vunjak-Novaković · 2015

This may be the most exciting time ever for the field of tissue engineering and regenerative medicine (TERM). After decades of progress, it has matured, integrated, and diversified into entirely new areas, and it is starting to make the pi…

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