About: Thymus transplantation for complete DiGeorge syndrome: European experience

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An Entity of Type : schema:ScholarlyArticle, within Data Space : wasabi.inria.fr associated with source document(s)

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type	Academic Article research paper schema:ScholarlyArticle
isDefinedBy	Covid-on-the-Web dataset
has title	Thymus transplantation for complete DiGeorge syndrome: European experience
Creator	Voor, Tiia Gilmour, Kimberly Adams, Stuart Bernatoniene, Jolanta Bremner, Ronald Browning, Michael Cheung, Melissa Crompton, Tessa Curry, Joe Devlin, Blythe Furmanski, Anna Halliday, Neil Hutchison, Lizzie Ifversen, Marianne Ip, Winnie Maimaris, Jesmeen Mengrelis, Konstantinos Moshous, Despina Neuling, Kim Pac, Malgorzata Papadopol, Alina Poliani, Luigi Ricciardelli, Ida Sebire, Neil Worth, Austen Bobby Gaspar, H Davies, E Erichsen, Hans Markert, M Mccarthy, Elizabeth Parsley, Kathryn Ronan Leahy, T Sansom, David Thrasher, Adrian
Source	Elsevier; Medline; PMC
abstract	BACKGROUND: Thymus transplantation is a promising strategy for the treatment of athymic complete DiGeorge syndrome (cDGS). METHODS: Twelve patients with cDGS underwent transplantation with allogeneic cultured thymus. OBJECTIVE: We sought to confirm and extend the results previously obtained in a single center. RESULTS: Two patients died of pre-existing viral infections without having thymopoiesis, and 1 late death occurred from autoimmune thrombocytopenia. One infant had septic shock shortly after transplantation, resulting in graft loss and the need for a second transplant. Evidence of thymopoiesis developed from 5 to 6 months after transplantation in 10 patients. Median circulating naive CD4 counts were 44 × 10(6)/L (range, 11-440 × 10(6)/L) and 200 × 10(6)/L (range, 5-310 × 10(6)/L) at 12 and 24 months after transplantation and T-cell receptor excision circles were 2,238/10(6) T cells (range, 320-8,807/10(6) T cells) and 4,184/10(6) T cells (range, 1,582-24,596/10(6) T cells). Counts did not usually reach normal levels for age, but patients were able to clear pre-existing infections and those acquired later. At a median of 49 months (range, 22-80 months), 8 have ceased prophylactic antimicrobials, and 5 have ceased immunoglobulin replacement. Histologic confirmation of thymopoiesis was seen in 7 of 11 patients undergoing biopsy of transplanted tissue, including 5 showing full maturation through to the terminal stage of Hassall body formation. Autoimmune regulator expression was also demonstrated. Autoimmune complications were seen in 7 of 12 patients. In 2 patients early transient autoimmune hemolysis settled after treatment and did not recur. The other 5 experienced ongoing autoimmune problems, including thyroiditis (3), hemolysis (1), thrombocytopenia (4), and neutropenia (1). CONCLUSIONS: This study confirms the previous reports that thymus transplantation can reconstitute T cells in patients with cDGS but with frequent autoimmune complications in survivors.
has issue date	2017-12-01(xsd:dateTime)
bibo:doi	10.1016/j.jaci.2017.03.020
bibo:pmid	28400115
has license	cc-by
sha1sum (hex)	8d6bcc2f1d98339588d638a0260826d7159d3049
schema:url	https://doi.org/10.1016/j.jaci.2017.03.020
resource representing a document's title	Thymus transplantation for complete DiGeorge syndrome: European experience
has PubMed Central identifier	PMC5716670
has PubMed identifier	28400115
schema:publication	J Allergy Clin Immunol
resource representing a document's body	covid:8d6bcc2f1d98339588d638a0260826d7159d3049#body_text
is schema:about of	named entity 'COMPLETE' named entity 'DiGeorge syndrome' named entity 'allogeneic' named entity 'Results' named entity 'circulating' named entity 'T cells' named entity 'patients' named entity 'European' named entity 'SECOND TRANSPLANT' named entity 'CULTURED' named entity 'OBTAINED' named entity 'CONFIRM' named entity 'STRATEGY' named entity 'OCCURRED' named entity 'COMPLETE' named entity 'OBJECTIVE' named entity 'THYMUS TRANSPLANTATION' named entity 'EXCISION' named entity 'ATHYMIC' named entity 'SINGLE' named entity 'THYMOPOIESIS' named entity 'PATIENTS' named entity 'BACKGROUND' named entity 'TREATMENT' named entity 'EVIDENCE OF' named entity '24 MONTHS' named entity 'IS A' named entity '310' named entity 'THYMUS TRANSPLANTATION' named entity 'EUROPEAN' named entity 'RESULTS' named entity 'DIGEORGE SYNDROME' named entity 'INFANT' named entity 'TRANSPLANTATION' named entity 'THYMUS' named entity 'CIRCULATING' named entity 'AUTOIMMUNE THROMBOCYTOPENIA' named entity 'RESULTING IN' named entity 'T CELLS' named entity 'ALLOGENEIC' named entity 'CDGS' named entity 'METHODS' named entity 'DIED' named entity '320' named entity 'DEATH' named entity 'VIRAL INFECTIONS' named entity 'PRE-EXISTING' named entity 'MEDIAN'

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