Source:http://linkedlifedata.com/resource/pubmed/id/19222467
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| Predicate | Object |
|---|---|
| rdf:type | |
| lifeskim:mentions | |
| pubmed:issue |
1
|
| pubmed:dateCreated |
2009-3-18
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| pubmed:abstractText |
Chronic granulomatous disease (CGD) causes recurrent infection and inflammatory disease. Despite antimicrobial prophylaxis, patients experience frequent hospitalisations and 50% mortality by 30 years. Haematopoietic stem cell transplantation (HSCT) can cure CGD with resolution of infection and colitis. This study reports the survival and long-term outcome in 20 conditioned patients treated between 1998 and 2007, using 10 matched sibling (MSD) and 10 unrelated donors (URD). Age at HSCT, graft-versus-host disease (GvHD), growth, and outcome were analysed. Fourteen had > or = 1 invasive infection, 10 had colitis and seven had growth failure before HSCT. Median age at transplantation was 75 months (range 15 months-21 years). Eighteen (90%) were alive 4-117 months (median 61) after HSCT with normal neutrophil function. Two died from disseminated fungal infection. Two experienced significant chronic GvHD, with continuing sequelae in 1. Colitis resolved within 8 weeks of HSCT. Mean weight and height for age Z scores on recovery from HSCT rose significantly (P < 0.001). HSCT with MSD or URD gave excellent engraftment and survival, remission of colitis and catch-up growth, with low incidence of significant GvHD. Transplant-associated complications were restricted to those with pre-existing infection or inflammation, supporting the argument for early HSCT for more CGD patients with a well matched donor.
|
| pubmed:language |
eng
|
| pubmed:journal | |
| pubmed:citationSubset |
IM
|
| pubmed:status |
MEDLINE
|
| pubmed:month |
Apr
|
| pubmed:issn |
1365-2141
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| pubmed:author |
pubmed-author:AbinunMarioM,
pubmed-author:BargeDawnD,
pubmed-author:CantAndrew JAJ,
pubmed-author:CollinMatthew PMP,
pubmed-author:FloodTerence JTJ,
pubmed-author:GenneryAndrew RAR,
pubmed-author:HodgesStephenS,
pubmed-author:HughesStephenS,
pubmed-author:JacksonGraham HGH,
pubmed-author:JonesLaura B K RLB,
pubmed-author:SlatterMary AMA,
pubmed-author:SonciniElenaE,
pubmed-author:SpickettGavin PGP
|
| pubmed:issnType |
Electronic
|
| pubmed:volume |
145
|
| pubmed:owner |
NLM
|
| pubmed:authorsComplete |
Y
|
| pubmed:pagination |
73-83
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| pubmed:meshHeading |
pubmed-meshheading:19222467-Adolescent,
pubmed-meshheading:19222467-Adult,
pubmed-meshheading:19222467-Child,
pubmed-meshheading:19222467-Child, Preschool,
pubmed-meshheading:19222467-Follow-Up Studies,
pubmed-meshheading:19222467-Graft vs Host Disease,
pubmed-meshheading:19222467-Granulomatous Disease, Chronic,
pubmed-meshheading:19222467-Growth,
pubmed-meshheading:19222467-Hematopoietic Stem Cell Transplantation,
pubmed-meshheading:19222467-Histocompatibility Testing,
pubmed-meshheading:19222467-Humans,
pubmed-meshheading:19222467-Infant,
pubmed-meshheading:19222467-Proportional Hazards Models,
pubmed-meshheading:19222467-Siblings,
pubmed-meshheading:19222467-Survival Rate,
pubmed-meshheading:19222467-Tissue Donors,
pubmed-meshheading:19222467-Transplantation, Homologous,
pubmed-meshheading:19222467-Transplantation Conditioning,
pubmed-meshheading:19222467-Treatment Outcome,
pubmed-meshheading:19222467-Young Adult
|
| pubmed:year |
2009
|
| pubmed:articleTitle |
Unrelated donor and HLA-identical sibling haematopoietic stem cell transplantation cure chronic granulomatous disease with good long-term outcome and growth.
|
| pubmed:affiliation |
Department of Paediatric Immunology, Newcastle upon Tyne Hospitals Foundation Trust, Newcastle upon Tyne, UK.
|
| pubmed:publicationType |
Journal Article,
Review
|