| Neutrophil functions in patients with inherited bone marrow failure syndromes. | |
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MedLine Citation:
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PMID: 21671367 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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BACKGROUND: The inherited bone marrow failure syndromes (IBMFS) include Fanconi anemia, dyskeratosis congenita, Diamond-Blackfan anemia, and Shwachman-Diamond syndrome (SDS). Previous studies reported decreased neutrophil chemotaxis in patients with SDS; there are no reports of neutrophil function in other IBMFS. In this study we examined neutrophil respiratory burst function in IBMFS patients. PROCEDURE: Samples from 43 IBMFS patients and 61 healthy family members were collected, shipped, and analyzed within 24 hr. We also studied samples from 12 healthy control persons immediately after collection. Neutrophils were stimulated with phorbol 12-myristate acetate (PMA) and N-formyl-methyonyl-leucyl-phenylalanine (fMLP), and respiratory burst analyzed by reduction of dihydro-rhodamine and cytochrome c. RESULTS: There was no significant difference in the degree of fMLP or PMA-driven respiratory burst activity between each of the IBMFS subgroups and their respective family members. There was also no difference in respiratory burst activity between any IBMFS, pooled group of all healthy family members and healthy controls. CONCLUSIONS: Neutrophil respiratory burst activity from IBMFS patients does not differ from that of healthy family members and controls. |
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Authors:
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Andrzej Rochowski; Chunxiang Sun; Michael Glogauer; Blanche P Alter |
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Publication Detail:
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Type: Journal Article; Research Support, N.I.H., Extramural; Research Support, N.I.H., Intramural; Research Support, Non-U.S. Gov't Date: 2010-11-05 |
Journal Detail:
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Title: Pediatric blood & cancer Volume: 57 ISSN: 1545-5017 ISO Abbreviation: Pediatr Blood Cancer Publication Date: 2011 Aug |
Date Detail:
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Created Date: 2011-06-14 Completed Date: 2011-08-23 Revised Date: 2012-03-20 |
Medline Journal Info:
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Nlm Unique ID: 101186624 Medline TA: Pediatr Blood Cancer Country: United States |
Other Details:
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Languages: eng Pagination: 306-9 Citation Subset: IM |
Copyright Information:
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Copyright © 2010 Wiley-Liss, Inc. |
Affiliation:
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Clinical Genetics Branch, Division of Cancer Epidemiology and Genetics, National Cancer Institute, National Institutes of Health, Department of Health and Human Services, Bethesda, Maryland 20852-7231, USA. |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Anemia, Hypoplastic, Congenital
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immunology* Bone Marrow Diseases / immunology Case-Control Studies Dyskeratosis Congenita / immunology* Exocrine Pancreatic Insufficiency / immunology Humans Lipomatosis Neutrophils / metabolism* Respiratory Burst* |
| Grant Support | |
ID/Acronym/Agency:
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Z99 CA999999/CA/NCI NIH HHS; //Canadian Institutes of Health Research |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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