{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,19]],"date-time":"2025-10-19T05:58:18Z","timestamp":1760853498685,"version":"3.37.3"},"reference-count":21,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2010,11,5]],"date-time":"2010-11-05T00:00:00Z","timestamp":1288915200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"name":"NIH"},{"DOI":"10.13039\/100000054","name":"National Cancer Institute","doi-asserted-by":"crossref","id":[{"id":"10.13039\/100000054","id-type":"DOI","asserted-by":"crossref"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Pediatric Blood &amp; Cancer"],"published-print":{"date-parts":[[2011,8]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>The inherited bone marrow failure syndromes (IBMFS) include Fanconi anemia, dyskeratosis congenita, Diamond\u2013Blackfan anemia, and Shwachman\u2013Diamond 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.<\/jats:p><\/jats:sec><jats:sec><jats:title>Procedure<\/jats:title><jats:p>Samples from 43 IBMFS patients and 61 healthy family members were collected, shipped, and analyzed within 24\u2009hr. We also studied samples from 12 healthy control persons immediately after collection. Neutrophils were stimulated with phorbol 12\u2010myristate acetate (PMA) and <jats:italic>N<\/jats:italic>\u2010formyl\u2010methyonyl\u2010leucyl\u2010phenylalanine (fMLP), and respiratory burst analyzed by reduction of dihydro\u2010rhodamine and cytochrome <jats:italic>c<\/jats:italic>.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>There was no significant difference in the degree of fMLP or PMA\u2010driven 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.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>Neutrophil respiratory burst activity from IBMFS patients does not differ from that of healthy family members and controls. Pediatr Blood Cancer 2011; 57: 306\u2013309. \u00a9 2010 Wiley\u2010Liss, Inc.<\/jats:p><\/jats:sec>","DOI":"10.1002\/pbc.22885","type":"journal-article","created":{"date-parts":[[2010,11,7]],"date-time":"2010-11-07T13:42:37Z","timestamp":1289137357000},"page":"306-309","source":"Crossref","is-referenced-by-count":9,"title":["Neutrophil functions in patients with inherited bone marrow failure syndromes"],"prefix":"10.1002","volume":"57","author":[{"given":"Andrzej","family":"Rochowski","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Chunxiang","family":"Sun","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Michael","family":"Glogauer","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Blanche P.","family":"Alter","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2010,11,5]]},"reference":[{"key":"e_1_2_6_2_2","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2008-12-192880"},{"key":"e_1_2_6_3_2","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2002-05-1498"},{"key":"e_1_2_6_4_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.blre.2010.03.002"},{"key":"e_1_2_6_5_2","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2141.2001.02996.x"},{"key":"e_1_2_6_6_2","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.2830310413"},{"key":"e_1_2_6_7_2","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2141.2000.02109.x"},{"key":"e_1_2_6_8_2","doi-asserted-by":"publisher","DOI":"10.1159\/000040861"},{"key":"e_1_2_6_9_2","doi-asserted-by":"publisher","DOI":"10.1172\/JCI110655"},{"key":"e_1_2_6_10_2","doi-asserted-by":"publisher","DOI":"10.1016\/0145-2126(87)90026-9"},{"key":"e_1_2_6_11_2","doi-asserted-by":"publisher","DOI":"10.1615\/CritRevImmunol.v26.i1.40"},{"key":"e_1_2_6_12_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-3476(79)80578-8"},{"key":"e_1_2_6_13_2","first-page":"249","article-title":"Constant defect in neutrophil locomotion but with age decreasing susceptibility to infection in Shwachman syndrome","volume":"57","author":"Ruutu P","year":"1984","journal-title":"Clin Exp Immunol"},{"key":"e_1_2_6_14_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2141.1988.tb02525.x"},{"key":"e_1_2_6_15_2","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2004-11-4371"},{"key":"e_1_2_6_16_2","doi-asserted-by":"publisher","DOI":"10.1002\/cm.10164"},{"key":"e_1_2_6_17_2","doi-asserted-by":"publisher","DOI":"10.1002\/jlb.52.3.357"},{"key":"e_1_2_6_18_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.mrfmmm.2009.03.013"},{"key":"e_1_2_6_19_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-1759(99)00146-5"},{"key":"e_1_2_6_20_2","doi-asserted-by":"publisher","DOI":"10.1002\/jlb.59.2.270"},{"key":"e_1_2_6_21_2","doi-asserted-by":"publisher","DOI":"10.1172\/JCI108024"},{"key":"e_1_2_6_22_2","doi-asserted-by":"publisher","DOI":"10.1002\/jlb.65.2.196"}],"container-title":["Pediatric Blood &amp; Cancer"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Fpbc.22885","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Fpbc.22885","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/pbc.22885","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,10,14]],"date-time":"2023-10-14T14:57:51Z","timestamp":1697295471000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/pbc.22885"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2010,11,5]]},"references-count":21,"journal-issue":{"issue":"2","published-print":{"date-parts":[[2011,8]]}},"alternative-id":["10.1002\/pbc.22885"],"URL":"https:\/\/doi.org\/10.1002\/pbc.22885","archive":["Portico"],"relation":{},"ISSN":["1545-5009","1545-5017"],"issn-type":[{"type":"print","value":"1545-5009"},{"type":"electronic","value":"1545-5017"}],"subject":[],"published":{"date-parts":[[2010,11,5]]}}}