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28 results on '"Hillier, Kirsty"'

5. What is in a name: defining pediatric refractory ITP

Author

Nakano, Taizo A., Grimes, Amanda B., Klaassen, Robert J., Lambert, Michele P., Neunert, Cindy, Rothman, Jennifer A., Shimano, Kristin A., Amend, Christina, Askew, Megan, Badawy, Sherif M., Baker, Jillian M., Breakey, Vicky, Crary, Shelley, Davini, Monica, Fritch Lilla, Stephanie, Gilbert, Megan, Hays, Taru, Hege, Kerry, Hillier, Kirsty, Jacobson-Kelly, Amanda, Kaicker, Shipra, Kim, Taylor O., Kochhar, Manpreet, Leblanc, Thierry, Martinelli, Marie, Nunez, Mara, Remiker, Allison, Schultz, Corrina, Sharma, Ruchika, and Grace, Rachael F.

Abstract

There are no agreed upon terminology to define “refractory” pediatric immune thrombocytopenia (ITP). Guidelines are therefore limited to arbitrary and outdated definitions. The Pediatric ITP Consortium of North America held a meeting in 2023 to define this entity. With 100% agreement, the faculty established that pediatric ITP that is refractory to emergent therapy could be defined as no platelet response after treatment with all eligible emergent pharmacotherapies. With 100% agreement, the working group established that pediatric patients with ITP that continue to demonstrate high disease burden and/or no platelet response despite treatment with multiple classes of disease-modifying therapies represent a challenging subset of ITP. These patients are at higher risk of ongoing disease burden and merit additional investigation as well as consideration for clinical trials or novel therapies. Future efforts to define disease burden and disease response will be completed in collaboration with the ITP International Working Group.

Published
2024
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6. Neutrophilic dermatosis in a patient with an IKZF1 variant and a review of monogenic autoinflammatory disorders presenting with neutrophilic dermatoses.

Author

Guirguis, Justina, Iosim, Sonia, Jones, Derek, Likhite, Maryel, Chen, Fei, Kesserwan, Chimene, Gindin, Tatyana, Kahn, Philip J., Beck, David, Oza, Vikash S., and Hillier, Kirsty

Subjects
SKIN diseases, ZINC-finger proteins, AUTOIMMUNE diseases, OPPORTUNISTIC infections, DISEASE relapse, GENETIC mutation
Abstract

Monogenic diseases of immune dysregulation should be considered in the evaluation of children presenting with recurrent neutrophilic dermatoses in association with systemic signs of inflammation, autoimmune disease, hematologic abnormalities, and opportunistic or recurrent infections. We report the case of a 2‐year‐old boy presenting with a neutrophilic dermatosis, found to have a novel likely pathogenic germline variant of the IKAROS Family Zinc Finger 1 (IKZF1) gene; the mutation likely results in a loss of function dimerization defective protein based on reports and studies of similar variants. IKZF1 variants could potentially lead to aberrant neutrophil chemotaxis and development of neutrophilic dermatoses. Long‐term surveillance is required to monitor the development of hematologic malignancy, autoimmunity, immunodeficiency, and infection in patients with pathogenic IKZF1 germline variants. [ABSTRACT FROM AUTHOR]

Published
2024
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11. Standardizing the Diagnostic and Therapeutic Approach to Newly Diagnosed Children with ITP: Prospective Data from the ITP Consortium of North America (ICON) Quality Improvement Initiative

Author

Elkus, Hannah, primary, Grace, Rachael F., additional, Buissereth, Taylah, additional, Bennett, Carolyn, additional, Rifkin-Zenenberg, Stacey, additional, Gunn, Elizabeth R, additional, Lebensburger, Jeffrey D., additional, Angulo, Pablo, additional, Davini, Monica, additional, Rankin, Alexander W., additional, Nakano, Taizo A., additional, Schultz, Corinna L., additional, Pinchinat, Ashley, additional, Hillier, Kirsty, additional, and Badawy, Sherif M., additional

Published
2022
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20. A Novel ALK Fusion in Pediatric Medullary Thyroid Carcinoma

Author

Hillier, Kirsty, primary, Hughes, Amy, additional, Shamberger, Robert C., additional, Shusterman, Suzanne, additional, Perez-Atayde, Antonio R., additional, Wassner, Ari J., additional, Iafrate, Anthony John, additional, Dubuc, Adrian, additional, Janeway, Katherine A., additional, Rothenberg, S. Michael, additional, Cox, Michael C., additional, Randolph, Gregory W., additional, Wirth, Lori J., additional, Tsai, Harrison, additional, Church, Alanna, additional, and DuBois, Steven G., additional

Published
2019
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25. ASK THE DON.

Author

DON, TIM, BAIRD, MATT, WITTS, JAMES, WOOD, JOHN, and HILLIER, KIRSTY

Published
2020

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