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36 results on '"Jeng, Michael R."'

2. Practical Application of Multivendor MRI‐Based R2* Mapping for Liver Iron Quantification at 1.5 T and 3.0 T

Author

Simchick, Gregory, primary, Zhao, Ruiyang, additional, Yuan, Qing, additional, Ghasabeh, Mounes Aliyari, additional, Ruschke, Stefan, additional, Karampinos, Dimitrios C., additional, Harris, David T., additional, do Vale Souza, Raphael, additional, Mattison, Ryan J., additional, Jeng, Michael R., additional, Pedrosa, Ivan, additional, Kamel, Ihab R., additional, Vasanawala, Shreyas, additional, Yokoo, Takeshi, additional, Reeder, Scott B., additional, and Hernando, Diego, additional

Published
2024
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4. Multicenter Reproducibility of Liver Iron Quantification with 1.5-T and 3.0-T MRI

Author

Hernando, Diego, primary, Zhao, Ruiyang, additional, Yuan, Qing, additional, Aliyari Ghasabeh, Mounes, additional, Ruschke, Stefan, additional, Miao, Xinran, additional, Karampinos, Dimitrios C., additional, Mao, Lu, additional, Harris, David T., additional, Mattison, Ryan J., additional, Jeng, Michael R., additional, Pedrosa, Ivan, additional, Kamel, Ihab R., additional, Vasanawala, Shreyas, additional, Yokoo, Takeshi, additional, and Reeder, Scott B., additional

Published
2023
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8. Multi-Center, Multi-Vendor Reproducibility and Calibration of MRI-Based R2* for Liver Iron Quantification

Author

Hernando, Diego, primary, Zhao, Ruiyang, additional, Yuan, Qing, additional, Aliyari Ghasabeh, Mounes, additional, Ruschke, Stefan, additional, Miao, Xinran, additional, Karampinos, Dimitrios C., additional, Mao, Lu, additional, Harris, David T., additional, Mattison, Ryan J., additional, Jeng, Michael R., additional, Pedrosa, Ivan, additional, Kamel, Ihab R., additional, Vasanawala, Shreyas, additional, Yokoo, Takeshi, additional, and Reeder, Scott B., additional

Published
2021
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9. Impact of Platelet Transfusion on Pulmonary Function of Hematology Oncology Patients: The Piper Study

Author

Wheeler, Allison P., primary, Snyder, Edward L., additional, Refaai, Majed A., additional, Cohn, Claudia S., additional, Poisson, Jessica, additional, Fontaine, Magali J., additional, Sehl, Mary, additional, Nooka, Ajay K., additional, Uhl, Lynne, additional, Spinella, Philip C., additional, Fenelus, Maly, additional, Liles, Darla, additional, Coyle, Thomas, additional, Becker, Joanne, additional, Jeng, Michael R., additional, Liu, Kathy, additional, Benjamin, Richard J, additional, and Corash, Laurence, additional

Published
2021
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19. Health Related Quality of Life and Fatigue Improve on Second Line Treatments in Pediatric Immune Thrombocytopenia (ITP)

Author

Grace, Rachael F., primary, Klaassen, Robert J, additional, Bhat, Rukhmi, additional, Neier, Michelle, additional, Rose, Melissa J., additional, Despotovic, Jenny M., additional, Bennett, Carolyn M., additional, Bussel, James B., additional, Rothman, Jennifer A., additional, Breakey, Vicky R., additional, Hege, Kerry, additional, Shimano, Kristin A., additional, Haley, Kristina M., additional, Geddis, Amy E., additional, Buchanan, George R., additional, Lorenzano, Adonis, additional, Neunert, Cindy, additional, Jeng, Michael R., additional, Pastore, Yves D., additional, Crary, Shelley, additional, Neufeld, Ellis J, additional, Neu, Nolan, additional, Forbes, Peter, additional, and Lambert, Michele P., additional

Published
2017
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20. Oxandrolone for the treatment of bone marrow failure in Fanconi anemia

Author

Rose, Susan R., primary, Kim, Mi‐Ok, additional, Korbee, Leslie, additional, Wilson, Kimberly A., additional, Douglas Ris, M., additional, Eyal, Ori, additional, Sherafat‐Kazemzadeh, Rosa, additional, Bollepalli, Sureka, additional, Harris, Richard, additional, Jeng, Michael R., additional, Williams, David A., additional, and Smith, Franklin O., additional

Published
2013
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21. The North American Chronic Immune Thrombocytopenia Registry (NACIR): Demographics and Treatment Responses

Author

Grace, Rachael F., primary, Neufeld, Ellis J., additional, Ritchey, A. Kim, additional, Kumar, Manjusha, additional, Jeng, Michael R., additional, Blanchette, Victor, additional, Klaassen, Robert, additional, Buchanan, George R., additional, Kurth, Margaret A. Heisel, additional, Nugent, Diane J., additional, Thompson, Alexis A., additional, Thornburg, Courtney, additional, Lambert, Michele P., additional, Neier, Michelle D., additional, Recht, Michael, additional, Stine, Kimo, additional, Kalish, Leslie A., additional, and Bennett, Carolyn M., additional

Published
2010
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22. Response to Steroids Predicts Response to Rituximab In Pediatric Chronic Immune Thrombocytopenia.

Author

Grace, Rachael F., primary, Bennett, Carolyn M., additional, Ritchey, A. Kim, additional, Jeng, Michael R., additional, Thornburg, Courtney, additional, Lambert, Michele, additional, Neier, Michelle, additional, Recht, Michael, additional, Kumar, Manjusha, additional, Blanchette, Victor, additional, Klaassen, Robert, additional, Buchanan, George R., additional, Kurth, Margaret A. Heisel, additional, Nugent, Diane J., additional, Thompson, Alexis A., additional, Stine, Kimo, additional, Kalish, Leslie A., additional, and Neufeld, Ellis J., additional

Published
2010
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30. Multicenter Reproducibility of Liver Iron Quantification with 1.5-T and 3.0-T MRI

Author

Hernando, Diego, Zhao, Ruiyang, Yuan, Qing, Ghasabeh, Mounes Aliyari, Ruschke, Stefan, Miao, Xinran, Karampinos, Dimitrios C., Mao, Lu, Harris, David T., Mattison, Ryan J., Jeng, Michael R., Pedrosa, Ivan, Kamel, Ihab R., Vasanawala, Shreyas, Yokoo, Takeshi, and Reeder, Scott B.

Abstract

MRI-based R2* mapping enabled reproducible quantification of liver iron overload across four centers, three vendors, and three field strengths (1.5 T, 2.89 T, and 3.0 T).

Published
2022
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31. ITRACONAZOLE-ENHANCED VINCRISTINE NEUROTOXICITY IN A CHILD WITH ACUTE LYMPHOBLASTIC LEUKEMIA.

Author

Jeng, Michael R. and Feusner, James

Subjects
*LYMPHOBLASTIC leukemia in children, *NEUROTOXICOLOGY, *DRUG side effects
Abstract

A boy with acute lymphoblastic leukemia (ALL) experienced life-threatening vincristine neurotoxicity while simultaneously exposed to itraconazole. Five pediatric and six adult cases of itraconazole-enhanced vincristine toxicity have been reported, all with ALL. Upon cessation of the itraconazole, the patient's symptoms resolved, which is similar to the outcome of the previously reported cases: 10 of 11 patients had complete resolution of symptoms. [ABSTRACT FROM AUTHOR]

Published
2001
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32. BRAF-V600E in Peripheral Mononuclear Cells and Microglia-like Brain Cells Suggest Hematopoietic Origin of Langerhans Cell Histiocytosis-Associated Neurodegeneration

Author

Zinn, Daniel, Picarsic, Jennifer, Chakraborty, Rikhia, Lin, Howard, Abhyankar, Harshal, Scull, Brooks, Shih, Albert, Phaik Har Lim, Karen, Eckstein, Olive S., Peters, Tricia L., Olea, Walter, Burke, Thomas, Ahmed, Nabil, Hicks, John, Tran, Brandon, Jones, Jeremy, Dauser, Robert, Jeng, Michael R., Baiocchi, Robert A, Schiff, Deborah E., Goldman, Stanton, Heym, Kenneth Matthew, Wilson, Harry, Carcamo, Benjamin, Kumar, Ashish, Rodriguez-Galindo, Carlos, Whipple, Nicholas, Campbell, Patrick, Murdoch, Geoffrey, Heales, Simon, Malone, Marian, Woltjer, Randy, Quinn, Joseph, Orchard, Paul, Kruer, Michael, Jaffe, Ronald, Manz, Markus G, Lira, Sergio, Parsons, Donald Will, Merad, Miriam, Man, Tsz-kwong, McClain, Kenneth L., and Allen, Carl E.

Abstract

Introduction:Langerhans cell histiocytosis (LCH) is a myeloid neoplasia characterized by inflammatory lesions with pathologic CD207+ myeloid dendritic cells (DC). Activation of ERKoccurs in all patients with the most common somatic driver mutation being BRAF-V600Ebeing found in up to 60% of patients.Mononuclear cells harboring the BRAF-V600Emutation have been found in peripheral blood of patients with involvement of the liver, spleen, or bone marrow, known as high risk organs. LCH involvement of the brain may include mass lesions in 25% of patients, most commonly the pituitary gland, or a progressive neurodegenerative syndrome (LCH-ND) that arises in approximately 5% of patients. LCH-ND may arise years after the initial LCH episode was presumed to have been cured and may lead to permanent neurologic deficits. The pathogenesis of LCH-ND is poorly understood, but is believed to be due to an autoimmune or paraneoplastic process. No standard approaches to surveillance or therapy exist. In order to define the etiology of LCH-ND, develop clinical tools for identifying patients at risk for LCH-ND, and to identify potential targets for novel therapies, we evaluated cerebral spinal fluid (CSF) proteins, extracellular BRAF- V600EDNA in CSF, and BRAF-V600E+ cells in peripheral blood and brain biopsies.

Published
2017
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33. Transfusion Dependence Is Common in Adults with Hemophagocytic Lymphohistiocytosis (HLH) and Is Associated with EBV Viremia

Author

Migdady, Yazan, Weiner, Haley, Lau, Eric, Martin, Beth A, Jeng, Michael R., and Oak, Jean

Abstract

Introduction:HLH in adults is a fulminant, often fatal hyperinflammatory disorder that often mimics sepsis and is associated with significant cytopenias, primary and secondary infections, immunosuppression, and bleeding. The etiologies in adults are typically autoimmune disease or lymphoma. As a result, HLH patients are immunocompromised hosts who represent a distinct group of high blood product users. Importantly, the need for adequate blood product support can obligate care in tertiary or quaternary settings. The literature lacks sufficient description of transfusion need patterns and the factors associated with blood product use in HLH. This IRB approved resource utilization study investigates this unique group of patients' transfusion needs and seeks to identify factors associated significant cytopenias that result in transfusion dependence.

Published
2017
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34. Resource Utilization Associated with the Management of Adults with Hemophagocytic Lymphohistiocytosis (HLH): A Benchmark Analysis for Improving Inpatient Care at a Quaternary Hospital

Author

Lau, Eric, Gong, Cynthia, Weiner, Haley, Jeng, Michael R., Purington, Natasha, and Martin, Beth A

Abstract

HLH is a highly fatal hyperinflammatory disorder that mimics sepsis in adults. Diagnostic criteria have not been validated in adults and standards for therapy have not been established. As a result, delays in diagnosis are common. Elevations in temperature, ferritin, and soluble interleukin 2 receptor (SIL-2R) are associated with HLH. Based on the Agency for Healthcare Research and Quality (AHRQ) National Inpatient Sample data, the mean cost per hospitalization of adult patients with HLH in 2014 was $70,860 in 2017 US Dollars, with a mean length of stay (LOS) of 16.8 days. In contrast, mean cost per hospitalization in patients with severe sepsis was just $30,149, with a mean LOS of 8.1 days.

Published
2017
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35. Treatment practices and response in kaposiform hemangioendothelioma: A multicenter cohort study.

Author

Borst AJ, Eng W, Griffin M, Ricci KW, Engel E, Adams DM, Dayneka J, Cohen-Cutler SJ, Andreoli SM, Wu MD, Wheeler AP, Heym KM, Crary SE, Nakano TA, Schulte RR, Setty BA, McLean TW, Pahl KS, Intzes S, Pateva I, Teitelbaum M, Zong Z, Li Y, and Jeng MR

Subjects
Child, Humans, Vincristine, Prospective Studies, Sirolimus therapeutic use, Kasabach-Merritt Syndrome drug therapy, Kasabach-Merritt Syndrome pathology, Vascular Neoplasms, Hemangioendothelioma drug therapy, Hemangioendothelioma pathology, Sarcoma, Kaposi pathology, Hemangioma, Skin Neoplasms
Abstract

Background and Objectives: Kaposiform hemangioendothelioma (KHE) and tufted angioma (TA) are rare vascular tumors in children historically associated with significant morbidity and mortality. This study was conducted to determine first-line therapy in the absence of available prospective clinical trials., Methods: Patients from 17 institutions diagnosed with KHE/TA between 2005 and 2020 with more than 6 months of follow-up were included. Response rates to sirolimus and vincristine were compared at 3 and 6 months. Durability of response and response to other treatment modalities were also evaluated., Results: Of 159 unique KHE/TA subjects, Kasabach-Merritt phenomenon (KMP) was present in 64 (40.3%), and only two patients were deceased (1.3%). Over 60% (n = 96) demonstrated treatment response at 3 months, and more than 70% (n = 114) by 6 months (no significant difference across groups). The vincristine group had higher radiologic response at 3 months compared to sirolimus (72.7% vs. 20%, p = .03), but there were no differences between these groups at 6 months. There were no differences in rates of recurrent or progressive disease between vincristine and sirolimus., Conclusions: In this large, multicenter cohort of 159 patients with KHE/TA, rates of KMP were consistent with historical literature, but the mortality rate (1.3%) was much lower. Overall treatment response rates were high (>70%), and there was no significant difference in treatment response or durability of disease comparing sirolimus to vincristine. Our results support individualized treatment decision plans depending on clinical scenario and patient/physician preferences. Response criteria and response rates reported here will be useful for guiding future treatment protocols for vascular tumors., (© 2023 Wiley Periodicals LLC.)

Published
2024
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36. Genomic analysis of bone marrow failure and myelodysplastic syndromes reveals phenotypic and diagnostic complexity.

Author

Zhang MY, Keel SB, Walsh T, Lee MK, Gulsuner S, Watts AC, Pritchard CC, Salipante SJ, Jeng MR, Hofmann I, Williams DA, Fleming MD, Abkowitz JL, King MC, and Shimamura A

Subjects
Adolescent, Adult, Aged, Bone Marrow Neoplasms classification, Child, Child, Preschool, Female, Humans, Infant, Male, Middle Aged, Myelodysplastic Syndromes classification, Phenotype, Prognosis, Young Adult, Biomarkers, Tumor genetics, Bone Marrow Neoplasms diagnosis, Bone Marrow Neoplasms genetics, Genomics methods, Mutation genetics, Myelodysplastic Syndromes diagnosis, Myelodysplastic Syndromes genetics
Abstract

Accurate and timely diagnosis of inherited bone marrow failure and inherited myelodysplastic syndromes is essential to guide clinical management. Distinguishing inherited from acquired bone marrow failure/myelodysplastic syndrome poses a significant clinical challenge. At present, diagnostic genetic testing for inherited bone marrow failure/myelodysplastic syndrome is performed gene-by-gene, guided by clinical and laboratory evaluation. We hypothesized that standard clinically-directed genetic testing misses patients with cryptic or atypical presentations of inherited bone marrow failure/myelodysplastic syndrome. In order to screen simultaneously for mutations of all classes in bone marrow failure/myelodysplastic syndrome genes, we developed and validated a panel of 85 genes for targeted capture and multiplexed massively parallel sequencing. In patients with clinical diagnoses of Fanconi anemia, genomic analysis resolved subtype assignment, including those of patients with inconclusive complementation test results. Eight out of 71 patients with idiopathic bone marrow failure or myelodysplastic syndrome were found to harbor damaging germline mutations in GATA2, RUNX1, DKC1, or LIG4. All 8 of these patients lacked classical clinical stigmata or laboratory findings of these syndromes and only 4 had a family history suggestive of inherited disease. These results reflect the extensive genetic heterogeneity and phenotypic complexity of bone marrow failure/myelodysplastic syndrome phenotypes. This study supports the integration of broad unbiased genetic screening into the diagnostic workup of children and young adults with bone marrow failure and myelodysplastic syndromes., (Copyright© Ferrata Storti Foundation.)

Published
2015
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