About: Maternal T-cell engraftment impedes with diagnosis of a SCID-ADA patient

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

Attributes	Values
type	Academic Article research paper schema:ScholarlyArticle
isDefinedBy	Covid-on-the-Web dataset
has title	Maternal T-cell engraftment impedes with diagnosis of a SCID-ADA patient
Creator	Beghin, Alessandra Bolda, Federica Comini, Marta Imberti, Luisa Lanfranchi, Arnalda Lougaris, Vassilios Montanelli, Alessandro Porta, Fulvio Soncini, Elena Notarangelo, Lucia
Source	Elsevier; Medline; PMC
abstract	We describe the case of a child affected by severe combined immunodeficiency (SCID) with adenosine deaminase (ADA) deficiency showing a maternal T-cell engraftment, a finding that has never been reported before. The presence of engrafted maternal T cells was misleading. Although ADA enzymatic levels were suggestive of ADA-SCID, the child did not present the classical signs of ADA deficiency; therefore, the initial diagnosis was of a conventional SCID. However, ADA toxic metabolites and molecular characterization confirmed this diagnosis. Polyethylene glycol-modified bovine (PEG) ADA therapy progressively decreased the number of maternal engrafted T cells. The child was grafted with full bone marrow from a matched unrelated donor, after a reduced conditioning regimen, and the result was the complete immunological reconstitution.
has issue date	2018-05-07(xsd:dateTime)
bibo:doi	10.1016/j.clim.2018.01.004
bibo:pmid	29355610
has license	no-cc
sha1sum (hex)	97fda0395a74e80cce5a1898b2733f42482af415
schema:url	https://doi.org/10.1016/j.clim.2018.01.004
resource representing a document's title	Maternal T-cell engraftment impedes with diagnosis of a SCID-ADA patient
has PubMed Central identifier	PMC7106042
has PubMed identifier	29355610
schema:publication	Clin Immunol
resource representing a document's body	covid:97fda0395a74e80cce5a1898b2733f42482af415#body_text
is schema:about of	named entity 'case' named entity 'Polyethylene' named entity 'SCID' named entity 'T-CELL' named entity 'CHARACTERIZATION' named entity 'DESCRIBE' named entity 'PRESENCE OF' named entity 'SEVERE COMBINED IMMUNODEFICIENCY' named entity 'CHILD' named entity 'NUMBER OF' named entity 'MATERNAL' named entity 'TOXIC' named entity 'NEVER' named entity 'CONVENTIONAL' named entity 'DID' named entity 'CASE' named entity 'INITIAL DIAGNOSIS' named entity 'ENGRAFTMENT' named entity 'MOLECULAR' named entity 'SCID' named entity 'SIGNS' named entity 'T-CELL' named entity 'MATERNAL' named entity 'ENGRAFTMENT' named entity 'ADA ' named entity 'FINDING' named entity 'DIAGNOSIS' named entity 'PATIENT' named entity 'MATERNAL T CELLS' named entity 'POLYETHYLENE GLYCOL' named entity 'AFFECTED' named entity 'THE CHILD' named entity 'COMPLETE' named entity 'SUGGESTIVE OF' named entity 'DIAGNOSIS' named entity 'REPORTED' named entity 'BOVINE' named entity 'NOT PRESENT' named entity 'T CELLS' named entity 'CONFIRMED' named entity 'THERAPY' named entity 'GRAFTED' named entity 'ADA ' named entity 'ADA DEFICIENCY' named entity 'MODIFIED' named entity 'MATCHED UNRELATED DONOR' named entity 'ADENOSINE DEAMINASE' named entity 'IMMUNOLOGICAL' named entity 'REDUCED' named entity 'METABOLITES' named entity 'RESULT' named entity 'DECREASED' named entity 'DEFICIENCY' named entity 'CONDITIONING REGIMEN' named entity 'BONE MARROW' named entity 'LEVELS' named entity 'ADA-SCID' named entity 'ENZYMATIC' named entity 'CLASSICAL' named entity 'RECONSTITUTION' covid:arg/97fda0395a74e80cce5a1898b2733f42482af415 named entity 'toxic' named entity 'ATG' named entity 'exons' named entity 'gene' named entity 'child' named entity 'child' named entity 'reduced' named entity 'T-cell' named entity 'ADA-SCID' named entity 'treosulfan' named entity 'GvHD'

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