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  1. Article ; Online: Transcranial doppler velocity in iron-deficient Nigerian children with sickle cell anemia.

    Abdullahi, Shehu Umar / Sunusi, Surayya / Aminu, Hauwa / Umar, Rashida / Abba, Mohammed Sani / Jibir, Binta W / Sani, Saifuddeen / Gambo, Safiya / Bello-Manga, Halima / Galadanci, Najibah A / Covert Greene, Brittany / Kassim, Adetola A / Jordan, Lori C / Aliyu, Muktar H / Rodeghier, Mark / DeBaun, Michael R / Volanakis, Emmanuel J

    American journal of hematology

    2024  Volume 99, Issue 4, Page(s) 797–799

    Abstract: Oral iron supplementation in iron deficient children with sickle cell anemia and normal transcranial Doppler ultrasound (TCD) velocities does not reduce arterial flow in the middle cerebral artery. ...

    Abstract Oral iron supplementation in iron deficient children with sickle cell anemia and normal transcranial Doppler ultrasound (TCD) velocities does not reduce arterial flow in the middle cerebral artery.
    MeSH term(s) Child ; Humans ; Stroke ; Blood Flow Velocity ; Anemia, Sickle Cell/complications ; Anemia, Sickle Cell/diagnostic imaging ; Ultrasonography, Doppler, Transcranial ; Cerebrovascular Circulation
    Language English
    Publishing date 2024-02-07
    Publishing country United States
    Document type Letter
    ZDB-ID 196767-8
    ISSN 1096-8652 ; 0361-8609
    ISSN (online) 1096-8652
    ISSN 0361-8609
    DOI 10.1002/ajh.27230
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    Zs.A 1251: Show issues Location:
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  2. Article: Long-Term Health Effects of Curative Therapies on Heart, Lungs, and Kidneys for Individuals with Sickle Cell Disease Compared to Those with Hematologic Malignancies.

    Fitzhugh, Courtney D / Volanakis, Emmanuel J / Idassi, Ombeni / Duberman, Josh A / DeBaun, Michael R / Friedman, Debra L

    Journal of clinical medicine

    2022  Volume 11, Issue 11

    Abstract: The goal of curing children and adults with sickle cell disease (SCD) is to maximize benefits and minimize intermediate and long-term adverse outcomes so that individuals can live an average life span with a high quality of life. While greater than 2000 ... ...

    Abstract The goal of curing children and adults with sickle cell disease (SCD) is to maximize benefits and minimize intermediate and long-term adverse outcomes so that individuals can live an average life span with a high quality of life. While greater than 2000 individuals with SCD have been treated with curative therapy, systematic studies have not been performed to evaluate the long-term health effects of hematopoietic stem cell transplant (HSCT) in this population. Individuals with SCD suffer progressive heart, lung, and kidney disease prior to curative therapy. In adults, these sequalae are associated with earlier death. In comparison, individuals who undergo HSCT for cancer are heavily pretreated with chemotherapy, resulting in potential acute and chronic heart, lung, and kidney disease. The long-term health effects on the heart, lung, and kidney for children and adults undergoing HSCT for cancer have been extensively investigated. These studies provide the best available data to extrapolate the possible late health effects after curative therapy for SCD. Future research is needed to evaluate whether HSCT abates, stabilizes, or exacerbates heart, lung, kidney, and other diseases in children and adults with SCD receiving myeloablative and non-myeloablative conditioning regimens for curative therapy.
    Language English
    Publishing date 2022-05-31
    Publishing country Switzerland
    Document type Journal Article ; Review
    ZDB-ID 2662592-1
    ISSN 2077-0383
    ISSN 2077-0383
    DOI 10.3390/jcm11113118
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  3. Article: COVID-19 pneumonia in a pediatric sickle cell patient requiring red blood cell exchange.

    Walker, Shannon C / Murphy, Meghan L / Hendricks, Hope / Dulek, Daniel E / Volanakis, Emmanuel J / Borinstein, Scott C

    Clinical case reports

    2021  Volume 9, Issue 3, Page(s) 1367–1370

    Abstract: Patients with sickle cell disease are already at high risk for respiratory complications, which SARS-CoV-2 can rapidly worsen. The case emphasizes the importance of efficiently maximizing standard therapies in sickle cell patients with COVID-19. ...

    Abstract Patients with sickle cell disease are already at high risk for respiratory complications, which SARS-CoV-2 can rapidly worsen. The case emphasizes the importance of efficiently maximizing standard therapies in sickle cell patients with COVID-19.
    Language English
    Publishing date 2021-01-12
    Publishing country England
    Document type Case Reports
    ZDB-ID 2740234-4
    ISSN 2050-0904
    ISSN 2050-0904
    DOI 10.1002/ccr3.3774
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  4. Article ; Online: A feasibility randomized controlled trial of an mHealth app vs booklets for patient-facing guidelines in adults with SCD.

    Cronin, Robert M / Quaye, Nives / Liu, Xin / Landes, Kristina / Crosby, Lori E / Kassim, Adetola A / Volanakis, Emmanuel J / Schnell, Patrick M / DeBaun, Michael R

    Blood advances

    2023  Volume 7, Issue 20, Page(s) 6184–6190

    Abstract: Despite the increased number of evidence-based guidelines for sickle cell disease (SCD), dissemination of evidence-based guidelines in lay language for individuals or families with SCD has not been evaluated. We conducted a feasibility randomized ... ...

    Abstract Despite the increased number of evidence-based guidelines for sickle cell disease (SCD), dissemination of evidence-based guidelines in lay language for individuals or families with SCD has not been evaluated. We conducted a feasibility randomized controlled trial to determine the acceptability of a mobile health (mHealth) app with patient-facing guidelines to improve the knowledge of individuals with SCD about SCD-specific knowledge and reduce hospitalizations. Primary outcome measures include recruitment, retention, and adherence rates. Adults with SCD were enrolled at 2 sickle cell centers between 2018 and 2022. Participants were randomized to receive either an mHealth app + booklet with patient-facing guidelines or a booklet with the guidelines alone. Participants completed surveys at baseline and a final 6-month visit. Approximately 67 of 74 (91%) agreed to participate and were randomized, with 50 of 67 (75%) completing all the study components. All participants who completed the study in the treatment arm used the app. Our results demonstrated high recruitment, retention, and adherence rate for the first randomized trial for an mHealth app with patient-facing guidelines in adults with SCD. This clinical trial was registered at https://www.clinicaltrials.gov/ as #NCT03629678.
    Language English
    Publishing date 2023-08-18
    Publishing country United States
    Document type Journal Article
    ZDB-ID 2915908-8
    ISSN 2473-9537 ; 2473-9529
    ISSN (online) 2473-9537
    ISSN 2473-9529
    DOI 10.1182/bloodadvances.2023010676
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    In stock of ZB MED Cologne/Königswinter

    Zs.A 7153: Show issues Location:
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  5. Article ; Online: Use of drug challenge in a case of possible deferoxamine allergy.

    Vethody, Chandra / Houser, Ashley / Hall, Laura / Volanakis, Emmanuel J / Norton, Allison E

    The journal of allergy and clinical immunology. In practice

    2019  Volume 8, Issue 5, Page(s) 1737–1739

    MeSH term(s) Deferoxamine/therapeutic use ; Humans ; Hypersensitivity ; Pharmaceutical Preparations
    Chemical Substances Pharmaceutical Preparations ; Deferoxamine (J06Y7MXW4D)
    Language English
    Publishing date 2019-12-30
    Publishing country United States
    Document type Case Reports ; Journal Article
    ZDB-ID 2843237-X
    ISSN 2213-2201 ; 2213-2198
    ISSN (online) 2213-2201
    ISSN 2213-2198
    DOI 10.1016/j.jaip.2019.12.018
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    Zs.A 7086: Show issues Location:
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  6. Article ; Online: Long-Term Health Effects of Curative Therapies on Heart, Lungs, and Kidneys for Individuals with Sickle Cell Disease Compared to Those with Hematologic Malignancies

    Courtney D. Fitzhugh / Emmanuel J. Volanakis / Ombeni Idassi / Josh A. Duberman / Michael R. DeBaun / Debra L. Friedman

    Journal of Clinical Medicine, Vol 11, Iss 3118, p

    2022  Volume 3118

    Abstract: The goal of curing children and adults with sickle cell disease (SCD) is to maximize benefits and minimize intermediate and long-term adverse outcomes so that individuals can live an average life span with a high quality of life. While greater than 2000 ... ...

    Abstract The goal of curing children and adults with sickle cell disease (SCD) is to maximize benefits and minimize intermediate and long-term adverse outcomes so that individuals can live an average life span with a high quality of life. While greater than 2000 individuals with SCD have been treated with curative therapy, systematic studies have not been performed to evaluate the long-term health effects of hematopoietic stem cell transplant (HSCT) in this population. Individuals with SCD suffer progressive heart, lung, and kidney disease prior to curative therapy. In adults, these sequalae are associated with earlier death. In comparison, individuals who undergo HSCT for cancer are heavily pretreated with chemotherapy, resulting in potential acute and chronic heart, lung, and kidney disease. The long-term health effects on the heart, lung, and kidney for children and adults undergoing HSCT for cancer have been extensively investigated. These studies provide the best available data to extrapolate the possible late health effects after curative therapy for SCD. Future research is needed to evaluate whether HSCT abates, stabilizes, or exacerbates heart, lung, kidney, and other diseases in children and adults with SCD receiving myeloablative and non-myeloablative conditioning regimens for curative therapy.
    Keywords sickle cell disease ; hematologic malignancies ; hematopoietic stem cell transplant ; heart ; lung ; kidney ; Medicine ; R
    Subject code 610
    Language English
    Publishing date 2022-05-01T00:00:00Z
    Publisher MDPI AG
    Document type Article ; Online
    Database BASE - Bielefeld Academic Search Engine (life sciences selection)

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  7. Article ; Online: COVID‐19 pneumonia in a pediatric sickle cell patient requiring red blood cell exchange

    Shannon C. Walker / Meghan L. Murphy / Hope Hendricks / Daniel E. Dulek / Emmanuel J. Volanakis / Scott C. Borinstein

    Clinical Case Reports, Vol 9, Iss 3, Pp 1367-

    2021  Volume 1370

    Abstract: Abstract Patients with sickle cell disease are already at high risk for respiratory complications, which SARS‐CoV‐2 can rapidly worsen. The case emphasizes the importance of efficiently maximizing standard therapies in sickle cell patients with COVID‐19. ...

    Abstract Abstract Patients with sickle cell disease are already at high risk for respiratory complications, which SARS‐CoV‐2 can rapidly worsen. The case emphasizes the importance of efficiently maximizing standard therapies in sickle cell patients with COVID‐19.
    Keywords hematology ; infectious diseases ; pediatrics and adolescent medicine ; Medicine ; R ; Medicine (General) ; R5-920
    Language English
    Publishing date 2021-03-01T00:00:00Z
    Publisher Wiley
    Document type Article ; Online
    Database BASE - Bielefeld Academic Search Engine (life sciences selection)

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  8. Article ; Online: Developmental strategies for evasion of Arf tumor suppression.

    Volanakis, Emmanuel J / Sherr, Charles J

    Cell cycle (Georgetown, Tex.)

    2010  Volume 9, Issue 1, Page(s) 14–15

    MeSH term(s) Animals ; CD4-Positive T-Lymphocytes/metabolism ; CD8-Positive T-Lymphocytes/metabolism ; Cyclin-Dependent Kinase Inhibitor p16/genetics ; Cyclin-Dependent Kinase Inhibitor p16/physiology ; Epigenesis, Genetic/genetics ; Epigenesis, Genetic/physiology ; Hematopoietic Stem Cells/metabolism ; Hematopoietic Stem Cells/physiology ; Humans ; Precursor T-Cell Lymphoblastic Leukemia-Lymphoma/genetics ; Precursor T-Cell Lymphoblastic Leukemia-Lymphoma/immunology ; Precursor T-Cell Lymphoblastic Leukemia-Lymphoma/metabolism ; Receptor, Notch1/genetics ; Receptor, Notch1/metabolism
    Chemical Substances Cyclin-Dependent Kinase Inhibitor p16 ; Receptor, Notch1
    Language English
    Publishing date 2010-01-21
    Publishing country United States
    Document type Editorial
    ZDB-ID 2146183-1
    ISSN 1551-4005 ; 1538-4101 ; 1554-8627
    ISSN (online) 1551-4005
    ISSN 1538-4101 ; 1554-8627
    DOI 10.4161/cc.9.1.10390
    Database MEDical Literature Analysis and Retrieval System OnLINE

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    In stock of ZB MED Cologne/Königswinter

    Zs.A 5881: Show issues Location:
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  9. Article ; Online: A significant proportion of children of African descent with HbSβ

    Day, Melissa E / Rodeghier, Mark / Driggers, Jennifer / Bean, Christopher J / Volanakis, Emmanuel J / DeBaun, Michael R

    British journal of haematology

    2018  Volume 185, Issue 1, Page(s) 153–156

    MeSH term(s) Africa/ethnology ; Anemia, Sickle Cell/diagnosis ; Anemia, Sickle Cell/ethnology ; Anemia, Sickle Cell/genetics ; Child ; Diagnostic Errors ; False Positive Reactions ; Heterozygote ; Humans ; Phenotype ; Sequence Deletion ; Sickle Cell Trait/diagnosis ; Sickle Cell Trait/ethnology ; Sickle Cell Trait/genetics ; alpha-Globins/genetics ; beta-Thalassemia/diagnosis ; beta-Thalassemia/ethnology ; beta-Thalassemia/genetics
    Chemical Substances alpha-Globins
    Language English
    Publishing date 2018-05-24
    Publishing country England
    Document type Letter
    ZDB-ID 80077-6
    ISSN 1365-2141 ; 0007-1048
    ISSN (online) 1365-2141
    ISSN 0007-1048
    DOI 10.1111/bjh.15400
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  10. Article ; Online: Epigenetic regulation of the Ink4a-Arf (Cdkn2a) tumor suppressor locus in the initiation and progression of Notch1-driven T cell acute lymphoblastic leukemia.

    Volanakis, Emmanuel J / Boothby, Mark R / Sherr, Charles J

    Experimental hematology

    2012  Volume 41, Issue 4, Page(s) 377–386

    Abstract: Activating mutations of NOTCH1 and deletion of the INK4A-ARF (CDKN2A) tumor suppressor locus are two of the most frequent genetic alterations in T cell acute lymphoblastic leukemia (T-ALL). In a murine model of T-ALL induced by the intracellular domain ... ...

    Abstract Activating mutations of NOTCH1 and deletion of the INK4A-ARF (CDKN2A) tumor suppressor locus are two of the most frequent genetic alterations in T cell acute lymphoblastic leukemia (T-ALL). In a murine model of T-ALL induced by the intracellular domain of Notch1 (ICN1), the genetic interaction between ICN1 signaling and Arf inactivation is developmentally stage-specific, with a more pronounced requirement for Arf deletion in thymocytes than in bone marrow precursors targeted for transformation. In the thymus, the target cell for transformation is a CD4 and CD8 double-negative progenitor that undergoes T cell receptor beta-chain rearrangement, a cell type in which polycomb silencing of Ink4a-Arf is normally requisite. Epigenetic remodeling during tumor progression licenses Arf as a tumor suppressor and in turn provides the selective pressure for Ink4a-Arf deletion in clonal T-ALLs that emerge.
    MeSH term(s) Animals ; Binding Sites/genetics ; Bone Marrow Cells/cytology ; Bone Marrow Cells/metabolism ; Cell Line ; Cell Transformation, Neoplastic/genetics ; Cells, Cultured ; Coculture Techniques ; Cyclin-Dependent Kinase Inhibitor p16/genetics ; Cyclin-Dependent Kinase Inhibitor p16/metabolism ; Disease Progression ; Dogs ; Epigenesis, Genetic ; Flow Cytometry ; Gene Expression ; Mice ; Mice, Inbred C57BL ; Mice, Knockout ; Mice, Transgenic ; Mutation ; Precursor T-Cell Lymphoblastic Leukemia-Lymphoma/genetics ; Precursor T-Cell Lymphoblastic Leukemia-Lymphoma/metabolism ; Precursor T-Cell Lymphoblastic Leukemia-Lymphoma/pathology ; Receptor, Notch1/genetics ; Receptor, Notch1/metabolism ; Reverse Transcriptase Polymerase Chain Reaction ; Signal Transduction/genetics ; T-Lymphocytes/cytology ; T-Lymphocytes/metabolism ; Thymocytes/cytology ; Thymocytes/metabolism ; Tumor Suppressor Protein p14ARF/genetics ; Tumor Suppressor Protein p14ARF/metabolism
    Chemical Substances Cyclin-Dependent Kinase Inhibitor p16 ; Receptor, Notch1 ; Tumor Suppressor Protein p14ARF
    Language English
    Publishing date 2012-11-23
    Publishing country Netherlands
    Document type Journal Article ; Research Support, Non-U.S. Gov't
    ZDB-ID 185107-x
    ISSN 1873-2399 ; 0531-5573 ; 0301-472X
    ISSN (online) 1873-2399
    ISSN 0531-5573 ; 0301-472X
    DOI 10.1016/j.exphem.2012.11.006
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    Zs.A 922: Show issues Location:
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