| pubmed-article:19222467 | rdf:type | pubmed:Citation | lld:pubmed |
| pubmed-article:19222467 | lifeskim:mentions | umls-concept:C0037047 | lld:lifeskim |
| pubmed-article:19222467 | lifeskim:mentions | umls-concept:C0013018 | lld:lifeskim |
| pubmed-article:19222467 | lifeskim:mentions | umls-concept:C0018956 | lld:lifeskim |
| pubmed-article:19222467 | lifeskim:mentions | umls-concept:C1504389 | lld:lifeskim |
| pubmed-article:19222467 | lifeskim:mentions | umls-concept:C0018203 | lld:lifeskim |
| pubmed-article:19222467 | lifeskim:mentions | umls-concept:C1274040 | lld:lifeskim |
| pubmed-article:19222467 | lifeskim:mentions | umls-concept:C0018270 | lld:lifeskim |
| pubmed-article:19222467 | lifeskim:mentions | umls-concept:C1880198 | lld:lifeskim |
| pubmed-article:19222467 | lifeskim:mentions | umls-concept:C0205170 | lld:lifeskim |
| pubmed-article:19222467 | lifeskim:mentions | umls-concept:C0445356 | lld:lifeskim |
| pubmed-article:19222467 | lifeskim:mentions | umls-concept:C0443252 | lld:lifeskim |
| pubmed-article:19222467 | pubmed:issue | 1 | lld:pubmed |
| pubmed-article:19222467 | pubmed:dateCreated | 2009-3-18 | lld:pubmed |
| pubmed-article:19222467 | 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. | lld:pubmed |
| pubmed-article:19222467 | pubmed:language | eng | lld:pubmed |
| pubmed-article:19222467 | pubmed:journal | http://linkedlifedata.com/r... | lld:pubmed |
| pubmed-article:19222467 | pubmed:citationSubset | IM | lld:pubmed |
| pubmed-article:19222467 | pubmed:status | MEDLINE | lld:pubmed |
| pubmed-article:19222467 | pubmed:month | Apr | lld:pubmed |
| pubmed-article:19222467 | pubmed:issn | 1365-2141 | lld:pubmed |
| pubmed-article:19222467 | pubmed:author | pubmed-author:AbinunMarioM | lld:pubmed |
| pubmed-article:19222467 | pubmed:author | pubmed-author:CantAndrew... | lld:pubmed |
| pubmed-article:19222467 | pubmed:author | pubmed-author:JacksonGraham... | lld:pubmed |
| pubmed-article:19222467 | pubmed:author | pubmed-author:HughesStephen... | lld:pubmed |
| pubmed-article:19222467 | pubmed:author | pubmed-author:GenneryAndrew... | lld:pubmed |
| pubmed-article:19222467 | pubmed:author | pubmed-author:SpickettGavin... | lld:pubmed |
| pubmed-article:19222467 | pubmed:author | pubmed-author:FloodTerence... | lld:pubmed |
| pubmed-article:19222467 | pubmed:author | pubmed-author:HodgesStephen... | lld:pubmed |
| pubmed-article:19222467 | pubmed:author | pubmed-author:SlatterMary... | lld:pubmed |
| pubmed-article:19222467 | pubmed:author | pubmed-author:CollinMatthew... | lld:pubmed |
| pubmed-article:19222467 | pubmed:author | pubmed-author:BargeDawnD | lld:pubmed |
| pubmed-article:19222467 | pubmed:author | pubmed-author:SonciniElenaE | lld:pubmed |
| pubmed-article:19222467 | pubmed:author | pubmed-author:JonesLaura... | lld:pubmed |
| pubmed-article:19222467 | pubmed:issnType | Electronic | lld:pubmed |
| pubmed-article:19222467 | pubmed:volume | 145 | lld:pubmed |
| pubmed-article:19222467 | pubmed:owner | NLM | lld:pubmed |
| pubmed-article:19222467 | pubmed:authorsComplete | Y | lld:pubmed |
| pubmed-article:19222467 | pubmed:pagination | 73-83 | lld:pubmed |
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| pubmed-article:19222467 | pubmed:meshHeading | pubmed-meshheading:19222467... | lld:pubmed |
| pubmed-article:19222467 | pubmed:meshHeading | pubmed-meshheading:19222467... | lld:pubmed |
| pubmed-article:19222467 | pubmed:meshHeading | pubmed-meshheading:19222467... | lld:pubmed |
| pubmed-article:19222467 | pubmed:meshHeading | pubmed-meshheading:19222467... | lld:pubmed |
| pubmed-article:19222467 | pubmed:year | 2009 | lld:pubmed |
| pubmed-article:19222467 | pubmed:articleTitle | Unrelated donor and HLA-identical sibling haematopoietic stem cell transplantation cure chronic granulomatous disease with good long-term outcome and growth. | lld:pubmed |
| pubmed-article:19222467 | pubmed:affiliation | Department of Paediatric Immunology, Newcastle upon Tyne Hospitals Foundation Trust, Newcastle upon Tyne, UK. | lld:pubmed |
| pubmed-article:19222467 | pubmed:publicationType | Journal Article | lld:pubmed |
| pubmed-article:19222467 | pubmed:publicationType | Review | lld:pubmed |
| http://linkedlifedata.com/r... | pubmed:referesTo | pubmed-article:19222467 | lld:pubmed |
