{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,7]],"date-time":"2026-10-07T03:15:10Z","timestamp":1791342910582,"version":"4.3.1"},"reference-count":66,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2002,6,27]],"date-time":"2002-06-27T00:00:00Z","timestamp":1025136000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["American J Hematol"],"published-print":{"date-parts":[[2002,7]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Over the past 40 years, we observed 284 subjects with hemoglobin SC disease (Hb SC) for 2,837 person\u2010years. We examined the association of the course of clinical events with hematologic and genetic factors. The mean entry age was 21 years, although 15% entered before one year of age. The mean Hb concentration was 11.3 g\/dL, the mean fetal hemoglobin was 2.5%, and the mean MCV was 84.4 fL. Twenty\u2010five subjects died at a median age of 37 years. Chronic organ\u2010specific complications occurred in 112 subjects (39.4%), with advanced retinopathy in 65 subjects (22.9%) and osteonecrosis (avascular necrosis) in 42 subjects (14.8%). We identified the \u03b2\u2010globin haplotypes in 82 subjects and the \u03b1\u2010gene status in 79. Twenty\u2010nine percent had \u03b1\u2010thalassemia\u20102. The \u03b2<jats:sup>CI<\/jats:sup> haplotype was present in 85.4%. We found a decreased incidence of retinopathy in the \u03b2<jats:sup>CI<\/jats:sup> subjects compared to the non\u2010\u03b2<jats:sup>CI<\/jats:sup> subjects (33% vs. 67%; <jats:italic>P<\/jats:italic> = 0.049) with a later mean onset age (29 years vs. 21 years; log\u2010rank test, <jats:italic>P<\/jats:italic>= 0.026). We also found a consistent pattern of decreased morbidity in subjects who had \u03b1\u2010thalassemia\u20102 in comparison to those who did not. We found a reduced risk of chronic organ\u2010specific complications (log\u2010rank test, <jats:italic>P<\/jats:italic>= 0.003), lower incidence of sickle crisis (48% vs. 80%, <jats:italic>P<\/jats:italic>= 0.001), later onset of gallbladder disease (age of onset: 55 years vs. 34 years; <jats:italic>P<\/jats:italic>= 0.055), and lower risk of osteonecrosis (log\u2010rank test, <jats:italic>P<\/jats:italic>= 0.024). Our findings suggest that Hb SC subjects who have not inherited \u03b1\u2010thalassemia\u20102 might benefit from erythrocyte rehydration therapy. Am. J. Hematol. 70:206\u2013215, 2002. \u00a9 2002 Wiley\u2010Liss, Inc.<\/jats:p>","DOI":"10.1002\/ajh.10140","type":"journal-article","created":{"date-parts":[[2002,10,6]],"date-time":"2002-10-06T11:34:39Z","timestamp":1033904079000},"page":"206-215","source":"Crossref","is-referenced-by-count":103,"title":["Outcome in hemoglobin SC disease: A four\u2010decade observational study of clinical, hematologic, and genetic factors"],"prefix":"10.1002","volume":"70","author":[{"given":"Darleen R.","family":"Powars","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alan","family":"Hiti","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Emily","family":"Ramicone","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cage","family":"Johnson","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Linda","family":"Chan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2002,6,27]]},"reference":[{"key":"e_1_2_1_2_2","doi-asserted-by":"crossref","first-page":"385","DOI":"10.1182\/blood.V18.4.385.385","article-title":"S\u2010C hemoglobin: a clinical study","volume":"18","author":"River GL","year":"1961","journal-title":"Blood"},{"key":"e_1_2_1_3_2","doi-asserted-by":"publisher","DOI":"10.1002\/(SICI)1096-8652(199704)54:4<313::AID-AJH9>3.0.CO;2-Y"},{"key":"e_1_2_1_4_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2141.1973.tb01675.x"},{"key":"e_1_2_1_5_2","volume-title":"Sickle cell disease","author":"Serjeant GR","year":"2001"},{"key":"e_1_2_1_6_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-3476(86)80217-7"},{"key":"e_1_2_1_7_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-3476(95)70062-5"},{"key":"e_1_2_1_8_2","first-page":"163","article-title":"Chest infections and syndromes in sickle cell disease of childhood","volume":"11","author":"Dryer ZE","year":"1996","journal-title":"Sem Respir Infect"},{"key":"e_1_2_1_9_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-3476(05)83171-3"},{"key":"e_1_2_1_10_2","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM198407053110102"},{"key":"e_1_2_1_11_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-3476(97)70284-1"},{"key":"e_1_2_1_12_2","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2257.2000.00304.x"},{"key":"e_1_2_1_13_2","doi-asserted-by":"publisher","DOI":"10.1016\/0009-8981(70)90305-0"},{"key":"e_1_2_1_14_2","first-page":"113","article-title":"Hemoglobin SC disease causing ischemic necrosis","volume":"47","author":"Larson P","year":"1986","journal-title":"North Carolina Med J"},{"key":"e_1_2_1_15_2","doi-asserted-by":"publisher","DOI":"10.2105\/AJPH.87.8.1317"},{"key":"e_1_2_1_16_2","series-title":"The clinical features of sickle cell disease","author":"Serjeant GR","year":"1974"},{"key":"e_1_2_1_17_2","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.2830130106"},{"key":"e_1_2_1_18_2","doi-asserted-by":"publisher","DOI":"10.7326\/0003-4819-115-8-614"},{"key":"e_1_2_1_19_2","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.2830430430"},{"key":"e_1_2_1_20_2","doi-asserted-by":"publisher","DOI":"10.1097\/00000441-199603000-00006"},{"key":"e_1_2_1_21_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-3476(00)90054-4"},{"key":"e_1_2_1_22_2","doi-asserted-by":"publisher","DOI":"10.3109\/03630269009046967"},{"key":"e_1_2_1_23_2","first-page":"1","article-title":"The variable expression of sickle cell disease is genetically determined","volume":"27","author":"Powars D","year":"1990","journal-title":"Sem Hematol"},{"key":"e_1_2_1_24_2","doi-asserted-by":"publisher","DOI":"10.3109\/03630268909003397"},{"key":"e_1_2_1_25_2","doi-asserted-by":"publisher","DOI":"10.1002\/(SICI)1096-8652(199809)59:1<15::AID-AJH4>3.0.CO;2-2"},{"key":"e_1_2_1_26_2","doi-asserted-by":"publisher","DOI":"10.1001\/archopht.1977.04450090065005"},{"key":"e_1_2_1_27_2","first-page":"251","volume-title":"Current protocols in molecular biology. 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