{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2023,11,13]],"date-time":"2023-11-13T05:16:04Z","timestamp":1699852564339},"reference-count":24,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2006,7,11]],"date-time":"2006-07-11T00:00:00Z","timestamp":1152576000000},"content-version":"vor","delay-in-days":9567,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["American J Hematol"],"published-print":{"date-parts":[[1980,5]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Congenital neutropenia (CN) was diagnosed in a five\u2010month\u2010old boy. A variety of studies was performed to define the pathogenesis of his disease. Opsonic antineutrophil antibodies were present in his serum. Transfused normal granulocytes circulated poorly. Incubation of the patient's serum with normal granulocytes failed to alter their metabolic or functional activity. The patient's marrow demonstrated increased numbers of colony\u2010forming units (CFUs) in vitro compared with control marrow. The patient's parents had low marrow CFU activity. The patient's serum and peripheral lymphocytes failed to inhibit normal marrow CFU activity. The patient's marrow did inhibit CFU growth of an HLA\u2010identical\u2010sibling's marrow in coculture. Histocompatibility studies demonstrated the HLA\u2010B12 antigen in this patient, a histocompatibility marker previously associated with CN. These studies suggest some cases of CN are associated with a genetically transmitted marrow factor capable of suppressing myelopoiesis in normal marrow.<\/jats:p>","DOI":"10.1002\/ajh.2830080310","type":"journal-article","created":{"date-parts":[[2007,2,21]],"date-time":"2007-02-21T05:41:10Z","timestamp":1172036470000},"page":"315-324","source":"Crossref","is-referenced-by-count":5,"title":["Congenital neutropenia: Studies of pathogenesis"],"prefix":"10.1002","volume":"8","author":[{"given":"Michael J.","family":"Chusid","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Anthony V.","family":"Pisciotta","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Rene J.","family":"Duquesnoy","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Bruce M.","family":"Camitta","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Peter A.","family":"Tomasulo","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2006,7,11]]},"reference":[{"key":"e_1_2_1_2_2","article-title":"Infantile genetic agranulocytosis","volume":"45","author":"Kostmann R","year":"1956","journal-title":"Acta Paediatr Scand"},{"key":"e_1_2_1_3_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1651-2227.1975.tb03847.x"},{"key":"e_1_2_1_4_2","doi-asserted-by":"crossref","first-page":"74","DOI":"10.1182\/blood.V38.1.74.74","article-title":"In vitro induction of myeloid proliferation and maturation in infantile genetic agranulocytosis","volume":"38","author":"Barak Y","year":"1971","journal-title":"Blood"},{"key":"e_1_2_1_5_2","first-page":"59","article-title":"Congenital neutropenia: impaired maturation with diminished stem cell 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