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  1. Article ; Online: Does shining a spotlight on XIAP deficiency bring the role of allogeneic HCT into better focus?

    Marsh, Rebecca A

    The Journal of allergy and clinical immunology

    2022  Volume 150, Issue 2, Page(s) 297–298

    MeSH term(s) Genetic Diseases, X-Linked ; Hematopoietic Stem Cell Transplantation ; Humans ; Lymphoproliferative Disorders ; X-Linked Inhibitor of Apoptosis Protein
    Chemical Substances X-Linked Inhibitor of Apoptosis Protein ; XIAP protein, human
    Language English
    Publishing date 2022-06-16
    Publishing country United States
    Document type Editorial ; Comment
    ZDB-ID 121011-7
    ISSN 1097-6825 ; 1085-8725 ; 0091-6749
    ISSN (online) 1097-6825 ; 1085-8725
    ISSN 0091-6749
    DOI 10.1016/j.jaci.2022.06.003
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  2. Article ; Online: Reply to "Radiosensitive Red Marrow: Where Is the Evidence?"

    Jones, A Kyle / Marsh, Rebecca M

    AJR. American journal of roentgenology

    2021  Volume 217, Issue 5, Page(s) 1253–1254

    MeSH term(s) Bone Marrow/diagnostic imaging ; Humans ; Radiation Tolerance
    Language English
    Publishing date 2021-09-27
    Publishing country United States
    Document type Letter ; Comment
    ZDB-ID 82076-3
    ISSN 1546-3141 ; 0361-803X ; 0092-5381
    ISSN (online) 1546-3141
    ISSN 0361-803X ; 0092-5381
    DOI 10.2214/AJR.21.26366
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  3. Article ; Online: Beyond the

    Jones, A Kyle / Marsh, Rebecca M

    AJR. American journal of roentgenology

    2021  Volume 217, Issue 5, Page(s) 1246

    Language English
    Publishing date 2021-04-21
    Publishing country United States
    Document type Journal Article ; Comment
    ZDB-ID 82076-3
    ISSN 1546-3141 ; 0361-803X ; 0092-5381
    ISSN (online) 1546-3141
    ISSN 0361-803X ; 0092-5381
    DOI 10.2214/AJR.21.25968
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  4. Article ; Online: Outcomes among racial and ethnic minority groups with X-linked agammaglobulinemia from the USIDNET registry.

    O'Toole, Dana / Groth, Daniel / Wright, Hannah / Bonilla, Francisco A / Cunningham-Rundles, Charlotte / Sullivan, Kathleen E / Ochs, Hans D / Marsh, Rebecca A / Feuille, Elizabeth / Fuleihan, Ramsay L

    The journal of allergy and clinical immunology. In practice

    2023  Volume 11, Issue 6, Page(s) 1945–1946

    MeSH term(s) Humans ; Ethnicity ; Minority Groups ; Ethnic and Racial Minorities ; Registries
    Language English
    Publishing date 2023-03-24
    Publishing country United States
    Document type Journal Article ; Research Support, N.I.H., Extramural
    ZDB-ID 2843237-X
    ISSN 2213-2201 ; 2213-2198
    ISSN (online) 2213-2201
    ISSN 2213-2198
    DOI 10.1016/j.jaip.2023.03.030
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  5. Article: Epstein-Barr Virus and Hemophagocytic Lymphohistiocytosis.

    Marsh, Rebecca A

    Frontiers in immunology

    2017  Volume 8, Page(s) 1902

    Abstract: Epstein-Barr virus (EBV) is a ubiquitous virus that infects nearly all people worldwide without serious sequela. However, for patients who have genetic diseases which predispose them to the development of hemophagocytic lymphohistiocytosis (HLH), EBV ... ...

    Abstract Epstein-Barr virus (EBV) is a ubiquitous virus that infects nearly all people worldwide without serious sequela. However, for patients who have genetic diseases which predispose them to the development of hemophagocytic lymphohistiocytosis (HLH), EBV infection is a life-threatening problem. As a part of a themed collection of articles on EBV infection and human primary immune deficiencies, we will review key concepts related to the understanding and treatment of HLH.
    Language English
    Publishing date 2017
    Publishing country Switzerland
    Document type Journal Article ; Review
    ZDB-ID 2606827-8
    ISSN 1664-3224
    ISSN 1664-3224
    DOI 10.3389/fimmu.2017.01902
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  6. Article ; Online: Diagnostic dilemmas in HLH: Can T-cell phenotyping help?

    Marsh, Rebecca A

    European journal of immunology

    2017  Volume 47, Issue 2, Page(s) 240–243

    Abstract: The diagnosis of hemophagocytic lymphohistiocytosis (HLH) can be a difficult one, and the distinction between primary versus secondary HLH can be particularly challenging during the early stages of diagnosis. This distinction is important to make as ... ...

    Abstract The diagnosis of hemophagocytic lymphohistiocytosis (HLH) can be a difficult one, and the distinction between primary versus secondary HLH can be particularly challenging during the early stages of diagnosis. This distinction is important to make as primary HLH requires allogeneic hematopoietic cell transplantation for a definitive cure. Flow cytometric screening tests for many of the genetic forms of HLH are available. However, not all patients with primary HLH are captured by these screening tests, due to the fact that no screening test is 100% sensitive, and additionally, some patients with "primary" forms of HLH may have mutations in genes which are yet to be discovered. In this issue of the European Journal of Immunology, Ammann et al. [Eur. J. Immunol. 2017. 47: 364-373] compare T-cell phenotype patterns among patients with primary and secondary HLH, and find that assessment of T-cell activation and differentiation may assist with the diagnosis of HLH. Furthermore, this phenotypic analysis has the potential to help make the important distinction between primary and secondary HLH.
    MeSH term(s) Hematopoietic Stem Cell Transplantation ; Humans ; Lymphocyte Activation/immunology ; Lymphohistiocytosis, Hemophagocytic ; Mutation ; T-Lymphocytes/immunology
    Language English
    Publishing date 2017-02-08
    Publishing country Germany
    Document type Journal Article ; Comment
    ZDB-ID 120108-6
    ISSN 1521-4141 ; 0014-2980
    ISSN (online) 1521-4141
    ISSN 0014-2980
    DOI 10.1002/eji.201646841
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  7. Article ; Online: Part 5: Allogeneic HSCT in refractory SJIA with lung disease; recent cases from centers in North America & Europe.

    Grom, Alexei A / Canna, Scott W / Abu-Arja, Rolla F / Sinha, Rashmi / Peixoto, Luciana / Cannizzaro, Elvira / Chandrakasan, Shanmuganathan / Driest, Kyla / Marsh, Rebecca / Neven, Bénédicte / Onel, Karen / Prahalad, Sampath / Prockop, Susan / Quartier, Pierre / Roth, Johannes / Schulert, Grant / Silva, Juliana M F / Wall, Donna / Zeilhofer, Ulrike

    Pediatric rheumatology online journal

    2024  Volume 21, Issue Suppl 1, Page(s) 86

    Abstract: It has been increasingly recognized that there is a subset of patients with refractory systemic JIA, who have failed all available medications and may benefit from HSCT. The increasing experience with HSCT in SJIA, suggests that despite the complicated ... ...

    Abstract It has been increasingly recognized that there is a subset of patients with refractory systemic JIA, who have failed all available medications and may benefit from HSCT. The increasing experience with HSCT in SJIA, suggests that despite the complicated post-HSCT course, short-term, the transplanted patients either achieved SJIA remission or reduced burden of disease. Longer follow-up, however, is needed to better define the long-term outcomes. The discussion at the NextGen 2022 conference was focused on the optimal timing for the procedure, the need for a good control of inflammatory SJIA activity prior to HSCT, and the role of the reduced intensity conditioning regimens as there was a remote concern that such regimens might increase the risk of SJIA relapse after the transplantation. There was unanimous agreement about the importance of long-term registries to address these questions.
    MeSH term(s) Humans ; Europe ; North America ; Registries ; Lung Diseases/therapy ; Hematopoietic Stem Cell Transplantation/adverse effects
    Language English
    Publishing date 2024-01-05
    Publishing country England
    Document type Journal Article
    ZDB-ID 2279468-2
    ISSN 1546-0096 ; 1546-0096
    ISSN (online) 1546-0096
    ISSN 1546-0096
    DOI 10.1186/s12969-023-00868-x
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  8. Article ; Online: Pediatric hemophagocytic lymphohistiocytosis.

    Canna, Scott W / Marsh, Rebecca A

    Blood

    2020  Volume 135, Issue 16, Page(s) 1332–1343

    Abstract: Hemophagocytic lymphohistiocytosis (HLH) is a syndrome describing patients with severe systemic hyperinflammation. Characteristic features include unremitting fever, cytopenias, hepatosplenomegaly, and elevation of typical HLH biomarkers. Patients can ... ...

    Abstract Hemophagocytic lymphohistiocytosis (HLH) is a syndrome describing patients with severe systemic hyperinflammation. Characteristic features include unremitting fever, cytopenias, hepatosplenomegaly, and elevation of typical HLH biomarkers. Patients can develop hepatitis, coagulopathy, liver failure, central nervous system involvement, multiorgan failure, and other manifestations. The syndrome has a high mortality rate. More and more, it is recognized that while HLH can be appropriately used as a broad summary diagnosis, many pediatric patients actually suffer from an expanding spectrum of genetic diseases that can be complicated by the syndrome of HLH. Classic genetic diseases in which HLH is a typical and common manifestation include pathogenic changes in familial HLH genes (PRF1, UNC13D, STXBP2, and STX11), several granule/pigment abnormality genes (RAB27A, LYST, and AP3B1), X-linked lymphoproliferative disease genes (SH2D1A and XIAP), and others such as NLRC4, CDC42, and the Epstein-Barr virus susceptibility diseases. There are many other genetic diseases in which HLH is an infrequent complication of the disorder as opposed to a prominent manifestation of the disease caused directly by the genetic defect, including other primary immune deficiencies and inborn errors of metabolism. HLH can also occur in patients with underlying rheumatologic or autoinflammatory disorders and is usually designated macrophage activation syndrome in those settings. Additionally, HLH can develop in patients during infections or malignancies without a known (or as-yet-identified) genetic predisposition. This article will attempt to summarize current concepts in the pediatric HLH field as well as offer a practical diagnostic and treatment overview.
    MeSH term(s) Animals ; CARD Signaling Adaptor Proteins/genetics ; Calcium-Binding Proteins/genetics ; Child ; Disease Management ; Epstein-Barr Virus Infections/complications ; F-Box-WD Repeat-Containing Protein 7/genetics ; Genetic Predisposition to Disease ; Genetic Variation ; Humans ; Lymphohistiocytosis, Hemophagocytic/diagnosis ; Lymphohistiocytosis, Hemophagocytic/genetics ; Lymphohistiocytosis, Hemophagocytic/physiopathology ; Lymphohistiocytosis, Hemophagocytic/therapy ; Signaling Lymphocytic Activation Molecule Associated Protein/genetics ; X-Linked Inhibitor of Apoptosis Protein/genetics
    Chemical Substances CARD Signaling Adaptor Proteins ; Calcium-Binding Proteins ; F-Box-WD Repeat-Containing Protein 7 ; FBXW7 protein, human ; NLRC4 protein, human ; SH2D1A protein, human ; Signaling Lymphocytic Activation Molecule Associated Protein ; X-Linked Inhibitor of Apoptosis Protein ; XIAP protein, human
    Language English
    Publishing date 2020-02-27
    Publishing country United States
    Document type Journal Article ; Research Support, Non-U.S. Gov't ; Review
    ZDB-ID 80069-7
    ISSN 1528-0020 ; 0006-4971
    ISSN (online) 1528-0020
    ISSN 0006-4971
    DOI 10.1182/blood.2019000936
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  9. Article ; Online: Review article: Peripheral intravenous catheter insertion in adult patients with difficult intravenous access: A systematic review of assessment instruments, clinical practice guidelines and escalation pathways.

    Paterson, Rebecca S / Schults, Jessica A / Slaughter, Eugene / Cooke, Marie / Ullman, Amanda / Kleidon, Tricia M / Keijzers, Gerben / Marsh, Nicole / Rickard, Claire M

    Emergency medicine Australasia : EMA

    2022  Volume 34, Issue 6, Page(s) 862–870

    Abstract: The optimal approach for peripheral intravenous catheter (PIVC) insertion in adult hospitalised patients with difficult intravenous access (DIVA) is unknown. The present study aimed to critically appraise the quality of (i) assessment instruments and (ii) ...

    Abstract The optimal approach for peripheral intravenous catheter (PIVC) insertion in adult hospitalised patients with difficult intravenous access (DIVA) is unknown. The present study aimed to critically appraise the quality of (i) assessment instruments and (ii) clinical practice guidelines (CPGs) or escalation pathways for identifying and managing patients with DIVA. Cochrane Central Register of Controlled Trials, EBSCO MEDLINE, EMBASE (OVID) and EBSCO CINAHL databases were searched on 22 March 2021. Studies describing a DIVA assessment measure, CPG or escalation pathway for PIVC insertion in adults (≥18 years of age) were included. Data were extracted using a standardised data extraction form including study design, type of resource and reported clinical outcomes. Quality of DIVA assessment instruments were reviewed using the COnsensus-based Standards for the selection of health Measurement Instruments checklist. Methodological quality of CPGs and escalation pathways was assessed using the Appraisal of Guidelines for Research and Evaluation-II (AGREE-II) instrument. Overall, 24 DIVA resources comprising 16 DIVA assessment instruments and nine CPGs or escalation pathways (including one combined assessment instrument and escalation pathway) were identified. Instruments commonly focused on vein visibility and palpability as indicators of DIVA. CPGs and escalation pathways unanimously recommended use of vessel visualisation technology for patients with or suspected of DIVA. Methodological quality of the resources was mixed. Consensus and standardisation of resources to identify DIVA and recommendations for managing patients with DIVA is limited. Adopting consistent, evidence-based CPGs, escalation pathways or DIVA assessment instruments may significantly improve clinical outcomes.
    MeSH term(s) Adult ; Humans ; Catheterization, Peripheral ; Administration, Intravenous ; Evidence-Based Practice ; Checklist ; Catheters
    Language English
    Publishing date 2022-08-29
    Publishing country Australia
    Document type Systematic Review ; Journal Article ; Review
    ZDB-ID 2161824-0
    ISSN 1742-6723 ; 1742-6731 ; 1035-6851
    ISSN (online) 1742-6723
    ISSN 1742-6731 ; 1035-6851
    DOI 10.1111/1742-6723.14069
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  10. Article: Evolution of Our Understanding of XIAP Deficiency.

    Mudde, Anne C A / Booth, Claire / Marsh, Rebecca A

    Frontiers in pediatrics

    2021  Volume 9, Page(s) 660520

    Abstract: X-linked inhibitor of apoptosis (XIAP) deficiency is a rare inborn error of immunity first described in 2006. XIAP deficiency is characterised by immune dysregulation and a broad spectrum of clinical manifestations, including haemophagocytic ... ...

    Abstract X-linked inhibitor of apoptosis (XIAP) deficiency is a rare inborn error of immunity first described in 2006. XIAP deficiency is characterised by immune dysregulation and a broad spectrum of clinical manifestations, including haemophagocytic lymphohistiocytosis (HLH), inflammatory bowel disease (IBD), hypogammaglobulinemia, susceptibility to infections, splenomegaly, cytopaenias, and other less common autoinflammatory phenomena. Since the first description of the disease, many XIAP deficient patients have been identified and our understanding of the disease has grown. Over 90 disease causing mutations have been described and more inflammatory disease manifestations, such as hepatitis, arthritis, and uveitis, are now well-recognised. Recently, following the introduction of reduced intensity conditioning (RIC), outcomes of allogeneic haematopoietic stem cell transplantation (HSCT), the only curative treatment option for XIAP deficiency, have improved. The pathophysiology of XIAP deficiency is not fully understood, however it is known that XIAP plays a role in both the innate and adaptive immune response and in immune regulation, most notably through modulation of tumour necrosis factor (TNF)-receptor signalling and regulation of NLRP3 inflammasome activity. In this review we will provide an up to date overview of both the clinical aspects and pathophysiology of XIAP deficiency.
    Language English
    Publishing date 2021-06-17
    Publishing country Switzerland
    Document type Journal Article ; Review
    ZDB-ID 2711999-3
    ISSN 2296-2360
    ISSN 2296-2360
    DOI 10.3389/fped.2021.660520
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