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  1. Article ; Online: An infant with severe neonatal immune thrombocytopenia and gradual resumption of breastfeeding.

    Inam, Zaina / Nickel, Robert Sheppard

    Pediatric blood & cancer

    2024  Volume 71, Issue 3, Page(s) e30839

    MeSH term(s) Infant ; Infant, Newborn ; Female ; Humans ; Thrombocytopenia, Neonatal Alloimmune ; Breast Feeding ; Purpura, Thrombocytopenic, Idiopathic/drug therapy ; Infant, Newborn, Diseases
    Language English
    Publishing date 2024-01-03
    Publishing country United States
    Document type Letter
    ZDB-ID 2131448-2
    ISSN 1545-5017 ; 1545-5009
    ISSN (online) 1545-5017
    ISSN 1545-5009
    DOI 10.1002/pbc.30839
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    Zs.A 1131: Show issues Location:
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    Jg. 1995 - 2021: Lesesall (1.OG)
    ab Jg. 2022: Lesesaal (EG)

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  2. Article ; Online: RIC does not do the trick to prevent the high infertility risk in females with sickle cell disease after hematopoietic cell transplantation.

    Nickel, Robert Sheppard / Maher, Jacqueline Y

    Transplantation and cellular therapy

    2023  Volume 29, Issue 9, Page(s) 537–538

    MeSH term(s) Female ; Humans ; Anemia, Sickle Cell/complications ; Anemia, Sickle Cell/therapy ; Hematopoietic Stem Cell Transplantation/adverse effects ; Infertility
    Language English
    Publishing date 2023-08-25
    Publishing country United States
    Document type Journal Article ; Comment
    ZDB-ID 3062231-1
    ISSN 2666-6367
    ISSN (online) 2666-6367
    DOI 10.1016/j.jtct.2023.08.003
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    Zs.A 5082: Show issues Location:
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  3. Article ; Online: Preventing antibody positive delayed hemolytic transfusion reactions in sickle cell disease: Lessons learned from a case.

    Rankin, Alexander / Webb, Jennifer / Nickel, Robert Sheppard

    Transfusion medicine (Oxford, England)

    2022  Volume 32, Issue 5, Page(s) 433–436

    Abstract: Introduction: Red blood cell (RBC) transfusions are important in the management of patients with sickle cell disease (SCD). However, a potentially catastrophic complication of transfusion in this population is the delayed hemolytic transfusion reaction ( ...

    Abstract Introduction: Red blood cell (RBC) transfusions are important in the management of patients with sickle cell disease (SCD). However, a potentially catastrophic complication of transfusion in this population is the delayed hemolytic transfusion reaction (DHTR). The pathophysiology of all DHTRs is not understood, but some are known to be caused by an anamnestic resurgence of RBC alloantibodies.
    Case presentation: A child with SCD transfused for acute chest syndrome re-presented a week after hospital discharge with severe anaemia, hemolysis, and a newly detected anti-E. This patient had been previously transfused years ago at an outside institution and the anti-E had not been previously documented.
    Discussion: The presented case of an antibody positive DHTR illustrates several concepts critical to the prevention of this complication. RBC alloantibodies must be detected and this information must be shared. Prophylactic C/c, E/e, K antigen matching is helpful for patients with SCD, but systems must be in place to identify these patients. Patients transfused at multiple different hospitals are especially at risk for this complication and efforts are needed to prevent them from suffering a DHTR.
    MeSH term(s) Anemia, Sickle Cell ; Blood Group Antigens ; Child ; Erythrocyte Transfusion/adverse effects ; Hemolysis ; Humans ; Isoantibodies ; Transfusion Reaction/epidemiology
    Chemical Substances Blood Group Antigens ; Isoantibodies
    Language English
    Publishing date 2022-03-22
    Publishing country England
    Document type Case Reports
    ZDB-ID 1067989-3
    ISSN 1365-3148 ; 0958-7578
    ISSN (online) 1365-3148
    ISSN 0958-7578
    DOI 10.1111/tme.12862
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    Zs.A 3177: Show issues Location:
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  4. Article ; Online: Ethical Challenges in Hematopoietic Cell Transplantation for Sickle Cell Disease.

    Nickel, Robert Sheppard / Kamani, Naynesh R

    Biology of blood and marrow transplantation : journal of the American Society for Blood and Marrow Transplantation

    2017  Volume 24, Issue 2, Page(s) 219–227

    Abstract: Hematopoietic cell transplantation (HCT) using an HLA-identical sibling donor offers a very high likelihood of cure with good outcomes for patients with sickle cell disease (SCD), and alternative donor HCT for SCD is an area of active clinical research. ... ...

    Abstract Hematopoietic cell transplantation (HCT) using an HLA-identical sibling donor offers a very high likelihood of cure with good outcomes for patients with sickle cell disease (SCD), and alternative donor HCT for SCD is an area of active clinical research. Thus, HCT is a potential option for a growing number of patients with SCD. This expanded use of HCT has raised several ethical questions. Who is eligible for HCT, in terms of both disease severity and psychosocial factors? Should affected children with matched sibling donors undergo HCT only when they have declared themselves as having significant symptomatology? Regarding donors, special ethical challenges include the use of preimplantation genetic diagnosis to conceive an HLA-identical sibling. In this review, we critically analyze various ethical challenges related to HCT for SCD, and offer recommendations to guide clinical care.
    MeSH term(s) Adolescent ; Adult ; Anemia, Sickle Cell/therapy ; Child ; Hematopoietic Stem Cell Transplantation/ethics ; Hematopoietic Stem Cell Transplantation/methods ; Humans ; Preimplantation Diagnosis ; Psychology ; Tissue Donors/ethics ; Young Adult
    Language English
    Publishing date 2017-09-01
    Publishing country United States
    Document type Journal Article ; Review
    ZDB-ID 1474865-4
    ISSN 1523-6536 ; 1083-8791
    ISSN (online) 1523-6536
    ISSN 1083-8791
    DOI 10.1016/j.bbmt.2017.08.034
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    In stock of ZB MED Cologne/Königswinter

    Zs.A 5082: Show issues Location:
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    ab Jg. 2022: Lesesaal (EG)
    Zs.MO 462: Show issues

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  5. Article: Fertility after Curative Therapy for Sickle Cell Disease: A Comprehensive Review to Guide Care.

    Nickel, Robert Sheppard / Maher, Jacqueline Y / Hsieh, Michael H / Davis, Meghan F / Hsieh, Matthew M / Pecker, Lydia H

    Journal of clinical medicine

    2022  Volume 11, Issue 9

    Abstract: Curative therapy for sickle cell disease (SCD) currently requires gonadotoxic conditioning that can impair future fertility. Fertility outcomes after curative therapy are likely affected by pre-transplant ovarian reserve or semen analysis parameters that ...

    Abstract Curative therapy for sickle cell disease (SCD) currently requires gonadotoxic conditioning that can impair future fertility. Fertility outcomes after curative therapy are likely affected by pre-transplant ovarian reserve or semen analysis parameters that may already be abnormal from SCD-related damage or hydroxyurea treatment. Outcomes are also likely affected by the conditioning regimen. Conditioning with myeloablative busulfan and cyclophosphamide causes serious gonadotoxicity particularly among post-pubertal females. Reduced-intensity and non-myeloablative conditioning may be acutely less gonadotoxic, but more short and long-term fertility outcome data after these approaches is needed. Fertility preservation including oocyte/embryo, ovarian tissue, sperm, and experimental testicular tissue cryopreservation should be offered to patients with SCD pursing curative therapy. Regardless of HSCT outcome, longitudinal post-HSCT fertility care is required.
    Language English
    Publishing date 2022-04-21
    Publishing country Switzerland
    Document type Journal Article ; Review
    ZDB-ID 2662592-1
    ISSN 2077-0383
    ISSN 2077-0383
    DOI 10.3390/jcm11092318
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  6. Article ; Online: Parents of Children with Sickle Cell Disease Are Interested in Preimplantation Genetic Testing.

    Attia, Monica / Kripalani, Shawn / Darbari, Isha / Nickel, Robert Sheppard

    The Journal of pediatrics

    2020  Volume 223, Page(s) 178–182.e2

    Abstract: Objective: To evaluate awareness of and attitudes toward preimplantation genetic testing (PGT) for sickle cell disease (SCD) among parents of children with SCD.: Study design: Parents of children with SCD were given an educational handbook on PGT ... ...

    Abstract Objective: To evaluate awareness of and attitudes toward preimplantation genetic testing (PGT) for sickle cell disease (SCD) among parents of children with SCD.
    Study design: Parents of children with SCD were given an educational handbook on PGT before a routine SCD clinic visit. After their clinic visit, parents were asked to complete an anonymous survey.
    Results: Of 83 patents approached, 67 (81%) completed the survey. Only 16 of the 67 parents (24%) were previously aware of PGT for SCD. After our clinic-based education, 65 of the 67 parents (97%) indicated that it was important or very important for parents of children with SCD to know about PGT. Among parents interested in having more children, 29 of 32 (91%) would personally consider using PGT if covered by insurance.
    Conclusions: Parents of children with SCD are generally not aware of PGT. When educated in clinic, parents viewed information on PGT as valuable. Pediatricians and other health care professionals should inform parents of children with SCD about this reproductive option.
    MeSH term(s) Adult ; Anemia, Sickle Cell/diagnosis ; Anemia, Sickle Cell/genetics ; Child ; Female ; Genetic Testing/methods ; Humans ; Male ; Parents ; Preoperative Care/methods ; Stem Cell Transplantation ; Surveys and Questionnaires
    Language English
    Publishing date 2020-06-22
    Publishing country United States
    Document type Journal Article
    ZDB-ID 3102-1
    ISSN 1097-6833 ; 0022-3476
    ISSN (online) 1097-6833
    ISSN 0022-3476
    DOI 10.1016/j.jpeds.2020.04.027
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    In stock of ZB MED Cologne/Königswinter

    Um IV Zs.116: Show issues Location:
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  7. Article ; Online: Impact of universal irradiation on chronic transfusion for sickle cell disease.

    Salinas Cisneros, Gabriel / Webb, Jennifer / Luban, Naomi L C / Nickel, Robert Sheppard

    Transfusion

    2021  Volume 61, Issue 8, Page(s) 2290–2294

    Abstract: Background: Irradiation of blood products prevents transfusion-associated graft-versus-host disease, but most patients do not require this modification which could have an adverse impact on transfusion outcomes. We hypothesized that irradiation may ... ...

    Abstract Background: Irradiation of blood products prevents transfusion-associated graft-versus-host disease, but most patients do not require this modification which could have an adverse impact on transfusion outcomes. We hypothesized that irradiation may increase transfusion requirements for patients with sickle cell disease (SCD) receiving chronic transfusion.
    Study design and methods: Our pediatric hospital implemented a new policy of universal blood product irradiation in May 2018. We conducted a retrospective chart review of patients with SCD receiving chronic red blood cell (RBC) transfusion throughout the year before and after institution of this policy. The primary outcome was the change in RBC transfusion volume per patient weight transfused during the pre- vs. post- universal irradiation period. Secondary outcomes were the change in median pretransfusion laboratory values.
    Results: Among 17 patients, 8 (47%) received more RBCs the year before irradiation and 9 (53%) received more the year after irradiation. Implementation of universal irradiation did not significantly increase transfusion volumes needed to clinically manage this population (median change +1.7 ml/kg/year, p = .54). Additionally, there were no significant changes in absolute reticulocyte count, hemoglobin, hemoglobin S%, white blood cell count, lactate dehydrogenase, total bilirubin, serum potassium, and ferritin during the two time periods.
    Conclusion: In a cohort of patients with SCD receiving simple chronic transfusion, irradiation did not impact transfusion requirements or pertinent pretransfusion laboratory values. Irradiation does not appear to have clinically significant consequences for SCD chronic transfusion management.
    MeSH term(s) Adolescent ; Anemia, Sickle Cell/blood ; Anemia, Sickle Cell/therapy ; Child ; Child, Preschool ; Erythrocyte Transfusion/adverse effects ; Erythrocyte Transfusion/methods ; Female ; Gamma Rays ; Humans ; Male ; Retrospective Studies ; Treatment Outcome
    Language English
    Publishing date 2021-07-12
    Publishing country United States
    Document type Journal Article ; Research Support, N.I.H., Extramural ; Research Support, Non-U.S. Gov't
    ZDB-ID 208417-x
    ISSN 1537-2995 ; 0041-1132
    ISSN (online) 1537-2995
    ISSN 0041-1132
    DOI 10.1111/trf.16531
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    Zs.A 284: Show issues Location:
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  8. Article ; Online: Fertility after Curative Therapy for Sickle Cell Disease

    Robert Sheppard Nickel / Jacqueline Y. Maher / Michael H. Hsieh / Meghan F. Davis / Matthew M. Hsieh / Lydia H. Pecker

    Journal of Clinical Medicine, Vol 11, Iss 2318, p

    A Comprehensive Review to Guide Care

    2022  Volume 2318

    Abstract: Curative therapy for sickle cell disease (SCD) currently requires gonadotoxic conditioning that can impair future fertility. Fertility outcomes after curative therapy are likely affected by pre-transplant ovarian reserve or semen analysis parameters that ...

    Abstract Curative therapy for sickle cell disease (SCD) currently requires gonadotoxic conditioning that can impair future fertility. Fertility outcomes after curative therapy are likely affected by pre-transplant ovarian reserve or semen analysis parameters that may already be abnormal from SCD-related damage or hydroxyurea treatment. Outcomes are also likely affected by the conditioning regimen. Conditioning with myeloablative busulfan and cyclophosphamide causes serious gonadotoxicity particularly among post-pubertal females. Reduced-intensity and non-myeloablative conditioning may be acutely less gonadotoxic, but more short and long-term fertility outcome data after these approaches is needed. Fertility preservation including oocyte/embryo, ovarian tissue, sperm, and experimental testicular tissue cryopreservation should be offered to patients with SCD pursing curative therapy. Regardless of HSCT outcome, longitudinal post-HSCT fertility care is required.
    Keywords fertility ; infertility ; sickle cell disease ; bone marrow transplant ; Medicine ; R
    Language English
    Publishing date 2022-04-01T00:00:00Z
    Publisher MDPI AG
    Document type Article ; Online
    Database BASE - Bielefeld Academic Search Engine (life sciences selection)

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  9. Article ; Online: Combination dose-escalated hydroxyurea and transfusion: an approach to conserve blood during the COVID-19 pandemic.

    Nickel, Robert Sheppard / Margulies, Stefanie / Frazer, Brittany / Luban, Naomi L C / Webb, Jennifer

    Blood

    2020  Volume 135, Issue 25, Page(s) 2320–2322

    MeSH term(s) Adolescent ; Adult ; Anemia, Sickle Cell/complications ; Anemia, Sickle Cell/drug therapy ; Anemia, Sickle Cell/therapy ; Blood Donors/supply & distribution ; Blood Safety ; Blood Transfusion/statistics & numerical data ; COVID-19 ; Chelation Therapy ; Child ; Child, Preschool ; Combined Modality Therapy ; Coronavirus Infections ; Dose-Response Relationship, Drug ; Drug Monitoring ; Humans ; Hydroxyurea/administration & dosage ; Hydroxyurea/adverse effects ; Hydroxyurea/therapeutic use ; Pandemics ; Pneumonia, Viral ; Risk ; Secondary Prevention/methods ; Social Isolation ; Stroke/prevention & control ; Telemedicine ; Young Adult
    Chemical Substances Hydroxyurea (X6Q56QN5QC)
    Keywords covid19
    Language English
    Publishing date 2020-05-08
    Publishing country United States
    Document type Clinical Trial ; Journal Article ; Research Support, Non-U.S. Gov't
    ZDB-ID 80069-7
    ISSN 1528-0020 ; 0006-4971
    ISSN (online) 1528-0020
    ISSN 0006-4971
    DOI 10.1182/blood.2020006582
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  10. Article ; Online: Neonatal Transfusion Medicine: Five Major Unanswered Research Questions for the Twenty-First Century.

    Nickel, Robert Sheppard / Josephson, Cassandra D

    Clinics in perinatology

    2015  Volume 42, Issue 3, Page(s) 499–513

    Abstract: Blood component transfusions are important to the care of preterm neonates; however, their use in clinical practice often is not based on high levels of evidence. Five major questions for neonates are discussed: (1) What is the optimal red blood cell ( ... ...

    Abstract Blood component transfusions are important to the care of preterm neonates; however, their use in clinical practice often is not based on high levels of evidence. Five major questions for neonates are discussed: (1) What is the optimal red blood cell (RBC) transfusion threshold? (2) What is the optimal platelet transfusion threshold? (3) Does the storage age of an RBC unit affect outcomes? (4) Does RBC transfusion contribute to the pathogenesis of necrotizing enterocolitis? and (5) Which new practices should be used to prevent transfusion-transmitted infections? Although definitive answers to these questions do not exist, future research should help answer them.
    MeSH term(s) Biomedical Research ; Blood Transfusion/methods ; Humans ; Infant, Newborn ; Neonatology/methods ; Transfusion Medicine/organization & administration
    Language English
    Publishing date 2015-09
    Publishing country United States
    Document type Journal Article ; Review
    ZDB-ID 193116-7
    ISSN 1557-9840 ; 0095-5108
    ISSN (online) 1557-9840
    ISSN 0095-5108
    DOI 10.1016/j.clp.2015.04.006
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