Linked Life Data

9763555

Source:http://linkedlifedata.com/resource/pubmed/id/9763555

Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
8
pubmed:dateCreated
1998-11-9
pubmed:abstractText
X-linked chronic granulomatous disease (X-CGD) is a primary immunodeficiency with complete absence or malfunction of the nicotinamide adenine dinucleotide phosphate (NADPH) oxidase in the phagocytic cells. Life-threatening infections especially with aspergillus are common despite optimal antimicrobial therapy. Bone marrow transplantation (BMT) is contraindicated during invasive aspergillosis in any disease setting. We report an 8-year-old patient with CGD who underwent HLA-genoidentical BMT during invasive multifocal aspergillus nidulans infection, nonresponsive to treatment with amphotericin-B and gamma-interferon. During the first 10 days post-BMT, the patient received granulocyte colony-stimulating factor (G-CSF)-mobilized, 25 Gy irradiated granulocytes from healthy volunteers plus G-CSF beginning on day 3 to prolong the viability of the transfused granulocytes. This was confirmed in vitro by apoptosis assays and in vivo by finding nitroblue tetrazolium (NBT)-positive granulocytes in peripheral blood 12 and 36 hours after the transfusions. Clinical and biological signs of infection began to disappear on day 7 post-BMT. Positron emission tomography with F18-fluorodeoxyglucose (FDG-PET) and computed tomography (CT) scans at 3 months post-BMT showed complete disappearance of infectious foci. At 2 years post-BMT, the patient is well with full immune reconstitution and no sign of aspergillus infection. Our results show that HLA-identical BMT may be successful during invasive, noncontrollable aspergillus infection, provided that supportive therapy is optimal.
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
AIM
pubmed:chemical
pubmed:status
MEDLINE
pubmed:month
Oct
pubmed:issn
0006-4971
pubmed:author
pubmed:copyrightInfo
Copyright 1998 by The American Society of Hematology.
pubmed:issnType
Print
pubmed:day
15
pubmed:volume
92
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
2719-24
pubmed:dateRevised
2004-11-17
pubmed:meshHeading
pubmed-meshheading:9763555-Amphotericin B, pubmed-meshheading:9763555-Antifungal Agents, pubmed-meshheading:9763555-Apoptosis, pubmed-meshheading:9763555-Aspergillosis, pubmed-meshheading:9763555-Aspergillus nidulans, pubmed-meshheading:9763555-Bone Marrow Transplantation, pubmed-meshheading:9763555-Child, pubmed-meshheading:9763555-Combined Modality Therapy, pubmed-meshheading:9763555-Drug Carriers, pubmed-meshheading:9763555-Graft Survival, pubmed-meshheading:9763555-Granulocyte Colony-Stimulating Factor, pubmed-meshheading:9763555-Granulocytes, pubmed-meshheading:9763555-Granulomatous Disease, Chronic, pubmed-meshheading:9763555-Humans, pubmed-meshheading:9763555-Itraconazole, pubmed-meshheading:9763555-Leukocyte Count, pubmed-meshheading:9763555-Leukocyte Transfusion, pubmed-meshheading:9763555-Liposomes, pubmed-meshheading:9763555-Lung Diseases, Fungal, pubmed-meshheading:9763555-Male, pubmed-meshheading:9763555-Tomography, Emission-Computed, pubmed-meshheading:9763555-Treatment Outcome
pubmed:year
1998
pubmed:articleTitle
Successful treatment of invasive aspergillosis in chronic granulomatous disease by bone marrow transplantation, granulocyte colony-stimulating factor-mobilized granulocytes, and liposomal amphotericin-B.
pubmed:affiliation
Divisions of Immunology/Hematology and Radiology, University Children's Hospital of Zurich, Switzerland.
pubmed:publicationType
Journal Article, Case Reports