{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,21]],"date-time":"2025-10-21T14:57:11Z","timestamp":1761058631006},"reference-count":32,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2006,7,11]],"date-time":"2006-07-11T00:00:00Z","timestamp":1152576000000},"content-version":"vor","delay-in-days":6919,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["American J Hematol"],"published-print":{"date-parts":[[1987,8]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>The hematology and pathophysiology of sickle cell disease during the postnatal development of younger hemoglobin (Hb) S homozygotes (SS) could be considerably affected by a variability of \u03b1 globin gene numbers. We have documented longitudinal developmental changes of hematological values and hemoglobin composition on 147 patients with SS (\u03b1\u03b1\/\u03b1\u03b1), 64 with SS (\u2010\u03b1\/\u03b1\u03b1), and 9 with SS (\u2013\u03b1\/\u2013\u03b1) between the ages of 1 and 15 years. Non\u2010steady\u2010state data were excluded from these analyses. The number and organization of \u03b1 globin genes was established by gene mapping. As anticipated, mean corpuscular volume and erythrocyte counts correlated with \u03b1 globin gene numbers throughout the 15\u2010year age interval. On the other hand, SS children with \u03b1\u03b1\/\u03b1\u03b1, \u2013\u03b1\/, \u2013\u03b1 had similar hemoglobin concentrations up to the ages of 5\u201310 years. Around the age of 7, the SS patients with \u2013\u03b1\/\u2013\u03b1 developed a higher Hb concentration than that of the SS (\u2013\u03b1\/\u03b1\u03b1), which in turn was higher than that of the SS (\u03b1\u03b1\/\u03b1\u03b1). The emergence of this difference coincided with a developmental increase of the mean corpuscular hemoglobin concentration (MCHC) in patients with SS (\u03b1\u03b1\/\u03b1\u03b1) and the decline of Hb F % under 15%. This newly observed developmental change of the MCHC could lead to increased hemolysis and anemia after the age of 5\u201010 years. It occurs to a smaller extent among SS (\u2013\u03b1\/\u03b1\u03b1) or not at all among SS (\u2013\u03b1\/\u03b1\u03b1) such that these two categories of patients have less severe hemolysis and higher hemoglobin levels at older ages. Although the proportion of Hb F was independent of \u03b1 globin gene numbers, the absence of Hb Bart's suggested that \u03b1\u2010thalassemia promotes the intracellular assembly of Hb F over Hb S tetramers. Thus, the interaction of \u03b1\u2010thalassemia and Hb F in young SS patients may be more complex than revealed by Hb F levels in cell lysates. Among older SS children (&gt; 7 years) \u03b1\u2010thalassemia and Hb F levels exceeding 15% appear to have additive effects in diminishing the rate of hemolysis.<\/jats:p>","DOI":"10.1002\/ajh.2830250405","type":"journal-article","created":{"date-parts":[[2007,2,21]],"date-time":"2007-02-21T11:07:03Z","timestamp":1172056023000},"page":"389-400","source":"Crossref","is-referenced-by-count":18,"title":["Effects of \u03b1\u2010thalassemia\u20102 on the developmental changes of hematological values in children with sickle cell disease from georgia"],"prefix":"10.1002","volume":"25","author":[{"given":"A. E.","family":"Felice","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"K. M.","family":"McKie","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"M. P.","family":"Cleek","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"E. M.","family":"Marino","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"A.","family":"Kutlar","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"V. C.","family":"McKie","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","volume-title":"Clinical Studies","author":"Serjeant GR","year":"1974"},{"key":"e_1_2_1_3_2","first-page":"361","volume-title":"The Management of Genetic Disorders","author":"Lehmann HR","year":"1979"},{"key":"e_1_2_1_4_2","first-page":"250","volume-title":"Hematology of Infancy and Childhood","author":"Keit AS","year":"1981"},{"key":"e_1_2_1_5_2","doi-asserted-by":"publisher","DOI":"10.1093\/ajcp\/61.3.375"},{"issue":"1","key":"e_1_2_1_6_2","first-page":"47a","article-title":"Deletions and duplications of \u03b1 or globin genes in children with sickle cell anemia","volume":"64","author":"Felice AE","year":"1984","journal-title":"Blood"},{"key":"e_1_2_1_7_2","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM198202043060504"},{"key":"e_1_2_1_8_2","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM198206173062402"},{"key":"e_1_2_1_9_2","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.2830060202"},{"key":"e_1_2_1_10_2","first-page":"93","volume-title":"The Red Cell","author":"Sewell A","year":"1977"},{"key":"e_1_2_1_11_2","doi-asserted-by":"crossref","first-page":"1353","DOI":"10.1182\/blood.V63.6.1353.1353","article-title":"the Cooperative Study of Sickle Cell Disease: Effects of thalassemia and microcytosis on the hematological and vasoocclusive severity of sickle cell anemia","volume":"63","author":"Steinberg MH","year":"1984","journal-title":"Blood"},{"issue":"1","key":"e_1_2_1_12_2","first-page":"54a","article-title":"Alpha thalassemia and the Hematology of Homozygous Sickle Cell Disease in Childhood","volume":"64","author":"Stevens MCG","year":"1984","journal-title":"Blood"},{"key":"e_1_2_1_13_2","doi-asserted-by":"crossref","first-page":"411","DOI":"10.1182\/blood.V67.2.411.411","article-title":"\u03b1\u2010thalassemia and the hematology of homozygous sickle cell disease in childhood","volume":"67","author":"Stevens MCG","year":"1986","journal-title":"Blood"},{"key":"e_1_2_1_14_2","unstructured":"McKieVC FeliceAE McKieKM BlanchardB KutlarA HuismanTHJ:An outreach clinical program for the delivery of medical care and clinical research in rural Georgia. National Sickle Cell Meeting Los Angeles CA (abstr) March 1985."},{"key":"e_1_2_1_15_2","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.2830060210"},{"issue":"5","key":"e_1_2_1_16_2","doi-asserted-by":"crossref","first-page":"1253","DOI":"10.1182\/blood.V63.5.1253.1253","article-title":"The rare \u03b1\u2010thalassemia\u20101 of Blacks is associated with deletion of all \u03b1 and globin genes","volume":"63","author":"Felice AE","year":"1984","journal-title":"Blood"},{"key":"e_1_2_1_17_2","doi-asserted-by":"publisher","DOI":"10.1007\/BF00401231"},{"key":"e_1_2_1_18_2","first-page":"4643","article-title":"Gamma thalassemia resulting from the deletion of a \u03b3\u2010globin gene","volume":"11","author":"Sukumaran PK","year":"1983","journal-title":"Nucleic Acids Res"},{"key":"e_1_2_1_19_2","volume-title":"The Hemoglobinopathies: Techniques of Identification","author":"Huisman THJ","year":"1977"},{"key":"e_1_2_1_20_2","doi-asserted-by":"publisher","DOI":"10.3109\/03630267908997539"},{"key":"e_1_2_1_21_2","first-page":"174","article-title":"A new high performance liquid chromatographic procedure for the separation and quantitation of various hemoglobin variants in adults and newborn babies","volume":"102","author":"Wilson JB","year":"1983","journal-title":"J Lab Clin Med"},{"issue":"3","key":"e_1_2_1_22_2","first-page":"189","article-title":"\u03b1\u2010thalassemia reduces the hemolytic rate in homozygous sickle\u2010cell disease","volume":"309","author":"DeCuellar K","year":"1984","journal-title":"N Engl J Med"},{"key":"e_1_2_1_23_2","first-page":"75","article-title":"Monovalent cation changes in sickle erythrocytes: A direct reflection of \u03b1 globin gene number","volume":"106","author":"Embury SH","year":"1985","journal-title":"J Lab Clin Med"},{"issue":"2","key":"e_1_2_1_24_2","doi-asserted-by":"crossref","first-page":"506","DOI":"10.1182\/blood.V68.2.506.506","article-title":"Accurate and independent measurement of volume and hemoglobin concentration of individual red cells by laser light scattering","volume":"68","author":"Mohandas N","year":"1986","journal-title":"Blood"},{"issue":"1","key":"e_1_2_1_25_2","first-page":"30","article-title":"Cell heterogeneity in sickle cell disease: Quantitation of the erythrocyte density profile","volume":"106","author":"Rodgers GP","year":"1985","journal-title":"J Lab Clin Med"},{"key":"e_1_2_1_26_2","doi-asserted-by":"publisher","DOI":"10.1172\/JCI111870"},{"key":"e_1_2_1_27_2","doi-asserted-by":"publisher","DOI":"10.1172\/JCI111181"},{"key":"e_1_2_1_28_2","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V65.1.183.bloodjournal651183"},{"issue":"2","key":"e_1_2_1_29_2","first-page":"449A","article-title":"Dense cell formation in pediatric and adolescent patients with sickle cell disease","volume":"34","author":"Abraham EC","year":"1986","journal-title":"Clin Res"},{"key":"e_1_2_1_30_2","unstructured":"AbrahamEC StallingsM McKieVC McKieKM FeliceAE:Influence of age and alpha thalassemia on dense cell formation in children with sickle cell disease(in preparation)."},{"issue":"1","key":"e_1_2_1_31_2","first-page":"64a","article-title":"\u03b1\u2010Thalassemia and cholelithiasis in children with SS disease","volume":"35","author":"McKie VC","year":"1987","journal-title":"Clin Res"},{"key":"e_1_2_1_32_2","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.2830020104"},{"key":"e_1_2_1_33_2","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.2830210407"}],"container-title":["American Journal of Hematology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Fajh.2830250405","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/ajh.2830250405","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,10,21]],"date-time":"2023-10-21T04:01:34Z","timestamp":1697860894000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/ajh.2830250405"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[1987,8]]},"references-count":32,"journal-issue":{"issue":"4","published-print":{"date-parts":[[1987,8]]}},"alternative-id":["10.1002\/ajh.2830250405"],"URL":"https:\/\/doi.org\/10.1002\/ajh.2830250405","archive":["Portico"],"relation":{},"ISSN":["0361-8609","1096-8652"],"issn-type":[{"value":"0361-8609","type":"print"},{"value":"1096-8652","type":"electronic"}],"subject":[],"published":{"date-parts":[[1987,8]]}}}