{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,7,30]],"date-time":"2025-07-30T10:05:19Z","timestamp":1753869919345,"version":"3.41.2"},"reference-count":26,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2022,12,29]],"date-time":"2022-12-29T00:00:00Z","timestamp":1672272000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pediatric Blood &amp; Cancer"],"published-print":{"date-parts":[[2023,3]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Patients with homozygous sickle cell disease (HbSS) and clinical splenomegaly by 6\u00a0months of age appeared at greater risk of invasive infections after 5\u00a0years of the Jamaican Cohort Study. We determined whether this risk remained significant over a longer study period, using a more rigorous definition of infection and examining the contribution of potential confounders.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Newborn screening of 100,000 consecutive deliveries during 1973\u20131981 detected 311 births with HbSS. Age at first clinical splenomegaly was used to categorize 285 of these patients in whom this could be determined: at or before 7\u00a0months (early), after 7\u00a0months (later), or \u2018never\u2019 palpated despite repeated examinations. Infective episodes were confined to \u2018first infections confirmed by positive culture\u2019. Using a generalized linear model, the risk of septicaemia was assessed in each group, after adjusting for potential confounders.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Of 93 \u2018first infections\u2019, 42 occurred in 105 subjects in the \u2018early\u2019 group, 49 in 157 subjects in the \u2018later\u2019 group, and two in 23 subjects in the \u2018never\u2019 group; the observed to expected ratio of 1.42, 0.90 and 0.22 was highly significant (<jats:italic>p<\/jats:italic> = .003). Assessed as risk ratios, \u2018early\u2019 splenomegaly had a significantly higher risk ratio (RR) for septicaemia (RR = 7.4, confidence interval [CI]: 1.1\u201350.7, <jats:italic>p<\/jats:italic> &lt; .05) when compared to the \u2018never\u2019 group adjusting for vaccine exposure and foetal haemoglobin concentration. The most common organisms were <jats:italic>Streptococcus pneumoniae<\/jats:italic>, <jats:italic>Salmonella<\/jats:italic> species, <jats:italic>Haemophilus influenzae<\/jats:italic> and <jats:italic>Staphylococcus aureus<\/jats:italic>.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Early clinical splenomegaly in HbSS remains a predictor of septicaemia, defining a group that may require closer monitoring.<\/jats:p><\/jats:sec>","DOI":"10.1002\/pbc.30161","type":"journal-article","created":{"date-parts":[[2022,12,29]],"date-time":"2022-12-29T11:49:51Z","timestamp":1672314591000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Early splenomegaly and septicaemia in homozygous sickle cell disease: A birth cohort study"],"prefix":"10.1002","volume":"70","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-3535-0911","authenticated-orcid":false,"given":"Angela E.","family":"Rankine\u2010Mullings","sequence":"first","affiliation":[{"name":"Sickle Cell Unit, Caribbean Institute for Health Research The University of the West Indies, Kingston 7  Jamaica West Indies"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"T. M.","family":"Logan","sequence":"additional","affiliation":[{"name":"Chaplin School of Hospitality and Tourism Management Florida International University  North Miami Beach Florida USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"M.","family":"Asnani","sequence":"additional","affiliation":[{"name":"Sickle Cell Unit, Caribbean Institute for Health Research The University of the West Indies, Kingston 7  Jamaica West Indies"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"G. R.","family":"Serjeant","sequence":"additional","affiliation":[{"name":"Sickle Cell Trust (Jamaica), 14 Milverton Crescent, Kingston 6  Jamaica"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2022,12,29]]},"reference":[{"doi-asserted-by":"publisher","key":"e_1_2_10_2_1","DOI":"10.1001\/archinte.1910.00050330050003"},{"key":"e_1_2_10_3_1","first-page":"428","article-title":"Multiple osteomyelitis due to Bacillus paratyphosus B. Demonstration of the bacillus in a fresh blood preparation","volume":"36","author":"Carrington GL","year":"1925","journal-title":"Bull Johns Hopkins Hosp"},{"key":"e_1_2_10_4_1","first-page":"998","article-title":"Sickle cell anemia","volume":"36","author":"Wollstein M","year":"1928","journal-title":"Am J Dis Child"},{"doi-asserted-by":"publisher","key":"e_1_2_10_5_1","DOI":"10.1056\/NEJM196605052741806"},{"doi-asserted-by":"publisher","key":"e_1_2_10_6_1","DOI":"10.1001\/jama.211.3.467"},{"doi-asserted-by":"publisher","key":"e_1_2_10_7_1","DOI":"10.1097\/00005792-197103000-00002"},{"doi-asserted-by":"publisher","key":"e_1_2_10_8_1","DOI":"10.1097\/00007611-193410000-00003"},{"doi-asserted-by":"publisher","key":"e_1_2_10_9_1","DOI":"10.1016\/0002-9343(56)90190-5"},{"key":"e_1_2_10_10_1","first-page":"28","article-title":"Sickle cell anemia","volume":"68","author":"Ehrenpreis B","year":"1952","journal-title":"Am J Roentgenol"},{"doi-asserted-by":"publisher","key":"e_1_2_10_11_1","DOI":"10.1056\/NEJM198206173062402"},{"doi-asserted-by":"publisher","key":"e_1_2_10_12_1","DOI":"10.1111\/j.1365-2141.1970.tb01647.x"},{"doi-asserted-by":"publisher","key":"e_1_2_10_13_1","DOI":"10.4103\/ijcm.IJCM_1054_20"},{"doi-asserted-by":"publisher","key":"e_1_2_10_14_1","DOI":"10.1056\/NEJM196910232811703"},{"doi-asserted-by":"publisher","key":"e_1_2_10_15_1","DOI":"10.1136\/adc.57.5.338"},{"doi-asserted-by":"publisher","key":"e_1_2_10_16_1","DOI":"10.1016\/S0140-6736(78)92527-8"},{"doi-asserted-by":"publisher","key":"e_1_2_10_17_1","DOI":"10.1136\/bmj.288.6430.1567"},{"doi-asserted-by":"publisher","key":"e_1_2_10_18_1","DOI":"10.1111\/j.1365-2141.1986.tb04117.x"},{"doi-asserted-by":"publisher","key":"e_1_2_10_19_1","DOI":"10.1093\/clinchem\/20.6.666"},{"doi-asserted-by":"publisher","key":"e_1_2_10_20_1","DOI":"10.1038\/1841877a0"},{"doi-asserted-by":"publisher","key":"e_1_2_10_21_1","DOI":"10.1136\/bmj.307.6916.1408"},{"doi-asserted-by":"publisher","key":"e_1_2_10_22_1","DOI":"10.1002\/ajh.2830390305"},{"doi-asserted-by":"publisher","key":"e_1_2_10_23_1","DOI":"10.1136\/bmj.1.6126.1515"},{"doi-asserted-by":"publisher","key":"e_1_2_10_24_1","DOI":"10.1111\/bjh.15846"},{"doi-asserted-by":"publisher","key":"e_1_2_10_25_1","DOI":"10.1016\/S0022-3476(97)70201-4"},{"doi-asserted-by":"publisher","key":"e_1_2_10_26_1","DOI":"10.1128\/CMR.00109-13"},{"doi-asserted-by":"publisher","key":"e_1_2_10_27_1","DOI":"10.1016\/S0022-3476(84)80950-6"}],"container-title":["Pediatric Blood &amp; Cancer"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/pbc.30161","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1002\/pbc.30161","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/pbc.30161","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,8,20]],"date-time":"2023-08-20T23:47:08Z","timestamp":1692575228000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/pbc.30161"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,12,29]]},"references-count":26,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2023,3]]}},"alternative-id":["10.1002\/pbc.30161"],"URL":"https:\/\/doi.org\/10.1002\/pbc.30161","archive":["Portico"],"relation":{},"ISSN":["1545-5009","1545-5017"],"issn-type":[{"type":"print","value":"1545-5009"},{"type":"electronic","value":"1545-5017"}],"subject":[],"published":{"date-parts":[[2022,12,29]]},"assertion":[{"value":"2022-05-27","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2022-11-25","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2022-12-29","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}],"article-number":"e30161"}}