About: Outcomes of hematopoietic stem cell transplantation for Hurler's syndrome in Europe: a risk factor analysis for graft failure

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About: Outcomes of hematopoietic stem cell transplantation for Hurler's syndrome in Europe: a risk factor analysis for graft failure Goto Sponge NotDistinct Permalink

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
title	Outcomes of hematopoietic stem cell transplantation for Hurler's syndrome in Europe: a risk factor analysis for graft failure
Creator	Bertrand, Y Fischer, A Wulffraat, N Rovelli, A Boelens, J Cavazzana-Calvo, M Sedlacek, P Souillet, G Sykora, K Veys, P Wraith, J Wynn, R O'meara, A Uiterwaal, Cspm
source	PMC
abstract	Hurler's syndrome (HS), the most severe form of mucopolysaccharidosis type-I, causes progressive deterioration of the central nervous system and death in childhood. Allogeneic stem cell transplantation (SCT) before the age of 2 years halts disease progression. Graft failure limits the success of SCT. We analyzed data on HS patients transplanted in Europe to identify the risk factors for graft failure. We compared outcomes in 146 HS patients transplanted with various conditioning regimens and grafts. Patients were transplanted between 1994 and 2004 and registered to the European Blood and Marrow Transplantation database. Risk factor analysis was performed using logistic regression. ‘Survival’ and ‘alive and engrafted’-rate after first SCT was 85 and 56%, respectively. In multivariable analysis, T-cell depletion (odds ratio (OR) 0.18; 95% confidence interval (CI) 0.04–0.71; P=0.02) and reduced-intensity conditioning (OR 0.08; 95% CI 0.02–0.39; P=0.002) were the risk factors for graft failure. Busulfan targeting protected against graft failure (OR 5.76; 95% CI 1.20–27.54; P=0.028). No difference was noted between cell sources used (bone marrow, peripheral blood stem cells or cord blood (CB)); however, significantly more patients who received CB transplants had full-donor chimerism (OR 9.31; 95% CI 1.06–82.03; P=0.044). These outcomes may impact the safety/efficacy of SCT for ‘inborn-errors of metabolism’ at large. CB increased the likelihood of sustained engraftment associated with normal enzyme levels and could therefore be considered as a preferential cell source in SCT for ‘inborn errors of metabolism’.
has issue date	2007-05-28(xsd:dateTime)
bibo:doi	10.1038/sj.bmt.1705718
bibo:pmid	17529997
has license	no-cc
sha1sum (hex)	ae7d48fde4968d045abed72cd1335b86e3c4ca06
schema:url	https://doi.org/10.1038/sj.bmt.1705718
resource representing a document's title	Outcomes of hematopoietic stem cell transplantation for Hurler's syndrome in Europe: a risk factor analysis for graft failure
has PubMed Central identifier	PMC7094454
has PubMed identifier	17529997
schema:publication	Bone Marrow Transplant
resource representing a document's body	covid:ae7d48fde4968d045abed72cd1335b86e3c4ca06#body_text
is schema:about of	named entity 'registered' named entity 'sustained' named entity 'impact' named entity 'graft' named entity 'enzyme' named entity 'Patients' named entity 'Hurler' named entity 'graft' named entity 'hematopoietic stem cell' named entity 'risk factors' named entity 'targeting' named entity 'transplanted' named entity 'disease' named entity 'factor analysis' named entity 'metabolism' named entity 'Blood' named entity 'multivariable' named entity 'analyzed data' named entity 'Busulfan' named entity 'enzyme levels' named entity 'SCT' named entity 'SCT' named entity 'T-cell depletion' named entity 'odds ratio' named entity 'Hurler's syndrome' named entity 'preferential' named entity 'CD34' named entity 'multivariable analysis' named entity 'risk factor' named entity 'PBSC' named entity 'stem cell homing' named entity 'intravenously' named entity 'GvHD' named entity 'graft rejection' named entity 'busulfan' named entity 'cartilage' named entity 'RIC' named entity 'RIC' named entity 'Europe' named entity 'hearing impairment' named entity 'statistically significant' named entity 'RIC' named entity 'Prague' named entity 'SCT' named entity 'cord blood' named entity 'Prophylaxis' named entity 'Hurler's syndrome' named entity 'immunomodulating' named entity 'busulfan' named entity 'nmol' named entity 'chimerism' named entity 'hepatosplenomegaly' named entity 'SCT' named entity 'phenotype' named entity 'leukocytes' named entity 'Dublin' named entity 'regression analysis' named entity 'independent variables' named entity 'SCT' named entity 'risk factors' named entity 'enzyme levels' named entity 'infection' named entity 'busulfan' named entity 'cyclophosphamide' named entity 'ERT' named entity 'loci' named entity 'myeloablative' named entity 'chimerism'

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