6 results on '"Kathleen Haines"'
Search Results
2. Morbidity, Mortality, and Therapeutics in Combined Immunodeficiency: Data From the USIDNET Registry
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Jessica Durkee-Shock, Anqing Zhang, Hua Liang, Hannah Wright, Julieann Magnusson, Elizabeth Garabedian, Rebecca A. Marsh, Kathleen E. Sullivan, Michael D. Keller, Jennifer Puck, Elizabeth Secord, Javeed Akhter, Tamara Pozos, Ramsay Fuleihan, Karin Chen, Rebecca Buckley, Niraj Patel, Daniel Suez, Megan Cooper, Manish Butte, Francisco Bonilla, Kelly Walkovich, Elie Haddad, Charlotte Cunningham-Rundles, Gary Kleiner, Hey Chong, Zuhair Ballas, Burcin Uygungil, Vivian Hernandez-Trujillo, Elizabeth A. Secord, Nicholas Hartog, Morna Dorsey, Ralph Shapiro, Susan Schuval, Luigi Notarangelo, John Routes, Adina Knight, Nicholas Bennett, Fatima Khan, Jolan Walter, Christine Seroogy, Hans Ochs, Kathleen Haines, Mica Muskat, Patricia Costa Reis, and Laurence Cheng
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Primary Immunodeficiency Diseases ,Hematopoietic Stem Cell Transplantation ,Immunology and Allergy ,Guanine Nucleotide Exchange Factors ,Humans ,Lymphocyte Count ,Registries ,Morbidity - Abstract
Optimal management of patients with combined immunodeficiency, especially pertaining to hematopoietic stem cell transplantation (HSCT), remains unclear.To identify factors influencing HSCT and mortality in the population with combined immunodeficiency in North America.We identified 337 participants in the United States Immunodeficiency Network database with diverse forms of combined immunodeficiency and their characteristics, including demographic characteristics, laboratory values, infectious history, comorbidities, and treatment strategies. Univariate analysis was performed using logistic regression, whereas multivariate analysis was performed using multiple Cox proportional hazards.On univariate analysis, disseminated invasive viral infections and variants in STAT3, GATA2, and, DOCK8 were associated with increased odds of HSCT. Mucocutaneous fungal infections and variants in STAT3 were associated with increased odds of survival, whereas disseminated/invasive fungal infections, disseminated/invasive viral infections, and parasitic infections were associated with decreased odds of survival. On multiple variable Cox proportional hazards analysis, variants in ZAP70, nonspecific bacterial, and disseminated/invasive viral infections were associated with increased hazards of transplantation, whereas variants in multiple genes (RMRP, NEMO, DOCK8, CD40L, and CARD9), disseminated/invasive viral infections, autoimmune disease, and higher absolute lymphocyte count were associated with increased hazards of death. Importantly, demographic characteristics, basic lymphocyte subset counts, and absence of genetic diagnosis were not associated with HSCT or mortality.We determined that specific genetic diagnoses and infection burden impacts the decision to undergo HSCT in this cohort. In addition, certain genetic diagnoses and invasive viral infections carry an increased risk of mortality.
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- 2021
3. Consensus treatment plans for periodic fever, aphthous stomatitis, pharyngitis and adenitis syndrome (PFAPA): a framework to evaluate treatment responses from the childhood arthritis and rheumatology research alliance (CARRA) PFAPA work group
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Gil, Amarilyo, Deborah, Rothman, Kalpana, Manthiram, Kathryn M, Edwards, Suzanne C, Li, Gary S, Marshall, Cagri, Yildirim-Toruner, Kathleen, Haines, Polly J, Ferguson, Geraldina, Lionetti, Julie, Cherian, Yongdong, Zhao, Patricia, DeLaMora, Grant, Syverson, Simona, Nativ, Marinka, Twilt, Ian C, Michelow, Yuriy, Stepanovskiy, Akaluck, Thatayatikom, Liora, Harel, Shoghik, Akoghlanian, Lori, Tucker, Mariana Correia, Marques, Hemalatha, Srinivasalu, Evan J, Propst, Greg R, Licameli, Fatma, Dedeoglu, Sivia, Lapidus, and Jonathan, Hausmann
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Pediatrics ,lcsh:Diseases of the musculoskeletal system ,Childhood arthritis ,medicine.medical_treatment ,Disease ,0302 clinical medicine ,Adrenal Cortex Hormones ,Immunology and Allergy ,030212 general & internal medicine ,Child ,PFAPA ,lcsh:RJ1-570 ,Pharyngitis ,Syndrome ,Tubulin Modulators ,Histamine H2 Antagonists ,Periodic fever ,Consensus treatment plan ,Child, Preschool ,Stomatitis, Aphthous ,medicine.symptom ,Periodic fever syndrome ,Cimetidine ,Research Article ,medicine.medical_specialty ,Antipyretics ,Fever ,Advisory Committees ,Placebo ,03 medical and health sciences ,Rheumatology ,Lymphadenitis ,Internal medicine ,medicine ,Humans ,Recurrent fever ,Tonsillectomy ,030203 arthritis & rheumatology ,business.industry ,lcsh:Pediatrics ,medicine.disease ,Pediatrics, Perinatology and Child Health ,lcsh:RC925-935 ,Periodic fever, aphthous stomatitis, pharyngitis and adenitis ,business ,Colchicine ,Neck - Abstract
Background Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome is the most common periodic fever syndrome in children. There is considerable heterogeneity in management strategies and a lack of evidence-based treatment guidelines. Consensus treatment plans (CTPs) are standardized treatment regimens that are derived based upon best available evidence and current treatment practices that are a way to enable comparative effectiveness studies to identify optimal therapy and are less costly to execute than randomized, double blind placebo controlled trials. The purpose of this project was to develop CTPs and response criteria for PFAPA. Methods The CARRA PFAPA Working Group is composed of pediatric rheumatologists, infectious disease specialists, allergists/immunologists and otolaryngologists. An extensive literature review was conducted followed by a survey to assess physician practice patterns. This was followed by virtual and in-person meetings between 2014 and 2018. Nominal group technique (NGT) was employed to develop CTPs, as well as inclusion criteria for entry into future treatment studies, and response criteria. Consensus required 80% agreement. Results The PFAPA working group developed CTPs resulting in 4 different treatment arms: 1. Antipyretic, 2. Abortive (corticosteroids), 3. Prophylaxis (colchicine or cimetidine) and 4. Surgical (tonsillectomy). Consensus was obtained among CARRA members for those defining patient characteristics who qualify for participation in the CTP PFAPA study. Conclusion The goal is for the CTPs developed by our group to lead to future comparative effectiveness studies that will generate evidence-driven therapeutic guidelines for this periodic inflammatory disease.
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- 2019
4. Strategic Planning in Hennepin County—An Issues Management
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Ann M. Pflaum, Philip C. Eckhert, Timothy J. Delmont, and Kathleen Haines
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Strategic planning ,Government ,Futures studies ,Process (engineering) ,Approaches of management ,Business ,Public administration ,Private sector ,Management process - Abstract
Strategic planning has become an increasingly useful approach to public sector management. Elected and appointed government officials have adopted techniques of strategic planning from the private sector chiefly to better anticipate emerging public needs and "to govern with foresight". In adopting an issues management form of strategic planning, Hennepin County, Minnesota, has developed a process to identify and resolve issues having broad, countywide significance over the next three to five years. The chapter identifies the factors leading to the adoption of this strategic planning initiative. It describes the issues management approach in detail and the county's process of implementation from 1983 to 1986. The chapter offers an analysis of the effectiveness of the issues management process. In a climate where the raising of questions about the mission of the county, its policy and funding priorities, and the effectiveness of board leadership has been encouraged, improved morale among many managers has resulted.
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- 2018
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5. Large Splenomegaly, Mediastinal and Pulmonary Nodularity, and Elevated Transaminases Associated With Non-necrotizing Granulomatosis of Liver and Spleen in an 8-Year-Old Boy
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Orestes Sanchez, Narmer Galeano, Kathleen Haines, David S. Goldberg, and Dennis Kuo
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medicine.medical_specialty ,medicine.anatomical_structure ,Hepatology ,business.industry ,Internal medicine ,Gastroenterology ,medicine ,Elevated transaminases ,Spleen ,medicine.disease ,business - Published
- 2014
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6. Protection of uninfected human bone marrow cells in long-term culture from G418 toxicity after retroviral-mediated transfer of the NEOr gene
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Steven D. Douglas, Kathleen Haines, Saul Surrey, Eliel Bayever, Eric F. Rappaport, and Stephen Duprey
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Genes, Viral ,Biology ,Transfection ,medicine ,Humans ,Cytotoxic T cell ,Gene ,Cells, Cultured ,Selectable marker ,Kanamycin Kinase ,Phosphotransferases ,Genetic transfer ,Drug Resistance, Microbial ,Neomycin ,Cell Biology ,Hematopoietic Stem Cells ,Molecular biology ,Haematopoiesis ,Retroviridae ,medicine.anatomical_structure ,Cell culture ,DNA, Viral ,Immunology ,Bone marrow ,medicine.drug - Abstract
The long-term effect of retroviral-mediated gene transfer into human hematopoietic cells in vitro was studied in bone marrow culture. Two retroviral vectors (pN2 or pZIP NEO) were used to transfer the gene coding for neomycin phosphotransferase, which confers neomycin resistance, as a dominant selectable marker. Following infection, bone marrow cells of multiple hematopoietic lineages displayed resistance for the duration of the cultures (greater than 80 days) to normally cytotoxic doses of the neomycin analog G418. However, upon DNA analysis of cells surviving in G418, the NEOr (neomycin resistance) gene was not detected under conditions where single copy genes could readily be seen, despite the presence of NEOr RNA sequences. In order to investigate this observation further, infected and uninfected cells were separated by a filter, and cultured in the same medium containing G418. The uninfected cells continued to survive in the presence of normally toxic concentrations of G418. Medium alone from infected cells was able to protect uninfected cells the same way. Efficiency of transfer of this and perhaps other selectable marker genes to cells in the long-term culture system may consequently be overestimated if survival of cells alone is quantitated. These results indicate that long-term cultures are a useful in vitro model for the study of retroviral-mediated gene transfer to human hematopoietic cells.
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- 1988
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