{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,30]],"date-time":"2026-09-30T19:20:08Z","timestamp":1790796008655,"version":"4.1.0"},"reference-count":15,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2005,6,3]],"date-time":"2005-06-03T00:00:00Z","timestamp":1117756800000},"content-version":"vor","delay-in-days":8099,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Am. J. Med. Genet."],"published-print":{"date-parts":[[1983,4]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>A sample of 354 individuals with sickle cell anemia ascertained in the city of Rio de Janeiro was studied to investigate the relationships between Hb F level, morbidity, and mortality. The mean Hb F level was 6.41 \u00b1 5.21%. The relationship between age and the proportion of Hb F can be described as a quadratic polynomial distribution, the level falling from approximately 11% in the first year to 4% at 25 years and then rising proportionally after 30 years. The correlations between Hb F level and 140 variables, including hematological values, signs and symptoms of the disease, as well as therapeutic requirements, showed that the patients with high Hb F levels are less anemic and have a more benign course. Several significant correlations (between amount of Hb F and the following clinical signs: pallor of mucous membranes, jaundice, cholelithiasis, leg ulcers, bronchial asthma, increased pulmonary vascularity, left ventricular hypertrophy, and osteomyelitis) are being reported here for the first time.<\/jats:p>","DOI":"10.1002\/ajmg.1320140410","type":"journal-article","created":{"date-parts":[[2005,6,11]],"date-time":"2005-06-11T15:34:49Z","timestamp":1118504089000},"page":"669-676","source":"Crossref","is-referenced-by-count":8,"title":["Hb F levels, longevity of homozygotes and clinical course of sickle cell anemia in Brazil"],"prefix":"10.1002","volume":"14","author":[{"given":"Mara H.","family":"Hutz","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"F. M.","family":"Salzano","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J.","family":"Adams","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"John M.","family":"Opitz","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2005,6,3]]},"reference":[{"key":"e_1_2_1_2_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2141.1970.tb01645.x"},{"key":"e_1_2_1_3_1","doi-asserted-by":"publisher","DOI":"10.1038\/1841877a0"},{"key":"e_1_2_1_4_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.2830060203"},{"key":"e_1_2_1_5_1","doi-asserted-by":"publisher","DOI":"10.1136\/jcp.29.10.898"},{"key":"e_1_2_1_6_1","doi-asserted-by":"publisher","DOI":"10.1136\/jmg.14.3.168"},{"key":"e_1_2_1_7_1","unstructured":"HutzMH SalzanoFM:Sickle cell anemia in Brazil: Demographic clinical and laboratory data(unpublished)."},{"key":"e_1_2_1_8_1","doi-asserted-by":"publisher","DOI":"10.7326\/0003-4819-88-1-1"},{"key":"e_1_2_1_9_1","doi-asserted-by":"publisher","DOI":"10.1172\/JCI109720"},{"key":"e_1_2_1_10_1","first-page":"107","volume-title":"Cellular and Molecular Regulation of Hemoglobin Switching","author":"Rucknagel DL","year":"1979"},{"key":"e_1_2_1_11_1","doi-asserted-by":"crossref","first-page":"109","DOI":"10.1016\/S0308-2261(21)00626-3","article-title":"Fetal haemoglobin in homozygous sickle cell disease","volume":"4","author":"Serjeant GR","year":"1975","journal-title":"Clin Haematol"},{"key":"e_1_2_1_12_1","first-page":"95","article-title":"Delayed skeletal maturation in sickle cell anemia in Jamaica","volume":"132","author":"Serjeant GR","year":"1973","journal-title":"Johns Hopkins Med J"},{"key":"e_1_2_1_13_1","doi-asserted-by":"crossref","first-page":"317","DOI":"10.1001\/jama.1973.03220160021005","article-title":"Mild sickle cell disease: Clinical and laboratory studies","volume":"224","author":"Steinberg MH","year":"1973","journal-title":"JAMA"},{"key":"e_1_2_1_14_1","first-page":"77","volume-title":"Proceedings of the First International Conference on Sickle Cell Disease: A World Health Problem","author":"Uy CG","year":"1979"},{"key":"e_1_2_1_15_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2141.1980.tb07163.x"},{"key":"e_1_2_1_16_1","doi-asserted-by":"publisher","DOI":"10.1093\/ajcp\/61.3.375"}],"container-title":["American Journal of Medical Genetics"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Fajmg.1320140410","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/ajmg.1320140410","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,10,19]],"date-time":"2023-10-19T17:21:41Z","timestamp":1697736101000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/ajmg.1320140410"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[1983,4]]},"references-count":15,"journal-issue":{"issue":"4","published-print":{"date-parts":[[1983,4]]}},"alternative-id":["10.1002\/ajmg.1320140410"],"URL":"https:\/\/doi.org\/10.1002\/ajmg.1320140410","archive":["Portico"],"relation":{},"ISSN":["0148-7299","1096-8628"],"issn-type":[{"value":"0148-7299","type":"print"},{"value":"1096-8628","type":"electronic"}],"subject":[],"published":{"date-parts":[[1983,4]]}}}