{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,7]],"date-time":"2026-10-07T03:15:06Z","timestamp":1791342906245,"version":"4.3.1"},"reference-count":22,"publisher":"Wiley","issue":"10","license":[{"start":{"date-parts":[[2024,7,25]],"date-time":"2024-07-25T00:00:00Z","timestamp":1721865600000},"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":[[2024,10]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Objective<\/jats:title><jats:p>To delineate the natural history of splenic complications other than loss of splenic function in children with sickle cell disease (SCD), we performed a retrospective chart review of patients with SCD treated at the Texas Children's Hospital.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>We determined the dates of diagnoses of splenic complications, the number of acute splenic sequestration crises (ASSC), and hydroxyurea treatment in pediatric patients with SCD. We also examined the association of hydroxyurea therapy with the onset and severity of ASSC.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>The cumulative prevalence of splenic complications was 24.7% for splenomegaly, 24.2% for ASSC, 9.6% for hypersplenism, and 5.6% for splenectomy. The cumulative prevalence of splenic complications was highest in patients with hemoglobin S\u03b2<jats:sup>0<\/jats:sup> (69.2%), intermediate in hemoglobin SS (33.3%), low in hemoglobin SC (9.0%), and non\u2010existent in hemoglobin S\u03b2<jats:sup>+<\/jats:sup>. The overall event rate of ASSC was 8.3 per 100 patient\u2010years. The event\u2010rate was 28.4 for hemoglobin S\u03b2<jats:sup>0<\/jats:sup>, 10.9 for hemoglobin SS, and 3.5 for hemoglobin SC. Patients with hemoglobin SS and hemoglobin S\u03b2<jats:sup>0<\/jats:sup> on hydroxyurea therapy had a significantly higher occurrence of ASSC than those who were not, with event rates of 14.2 and 3.1, respectively. The event rate was also higher for children who started hydroxyurea before age 2\u00a0years than for those who started after this age (19.8 and 9.2, respectively).<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>The prevalence and severity of splenic problems vary widely between different sickle cell genotypes, with hemoglobin S\u03b2<jats:sup>0<\/jats:sup> having the most severe complications. Hydroxyurea therapy is associated with increased incidence of ASSC, particularly when initiated before 2\u00a0years of age.<\/jats:p><\/jats:sec>","DOI":"10.1002\/pbc.31219","type":"journal-article","created":{"date-parts":[[2024,7,26]],"date-time":"2024-07-26T05:24:28Z","timestamp":1721971468000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":4,"title":["Splenic complications in pediatric sickle cell disease: A retrospective cohort review"],"prefix":"10.1002","volume":"71","author":[{"given":"Alex","family":"George","sequence":"first","affiliation":[{"name":"Department of Pediatrics Section of Hematology\/Oncology Wake Forest University School of Medicine and Brenner Children's Hospital  Winston\u2010Salem North Carolina USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shannon E.","family":"Conneely","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Division of Hematology\/Oncology Baylor College of Medicine\/Texas Children's Hospital  Houston Texas USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2288-042X","authenticated-orcid":false,"given":"Ross","family":"Mangum","sequence":"additional","affiliation":[{"name":"Center for Cancer and Blood Disorders Phoenix Children's Hospital  Phoenix Arizona USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Titilope","family":"Fasipe","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Division of Hematology\/Oncology Baylor College of Medicine\/Texas Children's Hospital  Houston Texas USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0978-5863","authenticated-orcid":false,"given":"Philip J.","family":"Lupo","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Division of Hematology\/Oncology Baylor College of Medicine\/Texas Children's Hospital  Houston Texas USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael E.","family":"Scheurer","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Division of Hematology\/Oncology Baylor College of Medicine\/Texas Children's Hospital  Houston Texas USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2024,7,25]]},"reference":[{"key":"e_1_2_9_2_1","doi-asserted-by":"publisher","DOI":"10.1111\/bjh.12950"},{"key":"e_1_2_9_3_1","doi-asserted-by":"publisher","DOI":"10.1080\/17474086.2019.1627192"},{"key":"e_1_2_9_4_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2010-04-278747"},{"key":"e_1_2_9_5_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V85.8.2238.bloodjournal8582238"},{"issue":"9","key":"e_1_2_9_6_1","first-page":"819","article-title":"Spleen function in children with sickle \u03b2+ thalassemia","volume":"83","author":"Barrios NJ","year":"1991","journal-title":"J Natl Med Assoc"},{"key":"e_1_2_9_7_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2141.2011.08999.x"},{"key":"e_1_2_9_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(78)92527-8"},{"key":"e_1_2_9_9_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2257.2000.00304.x"},{"key":"e_1_2_9_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.bcmd.2019.102374"},{"key":"e_1_2_9_11_1","doi-asserted-by":"publisher","DOI":"10.1111\/bjh.17179"},{"key":"e_1_2_9_12_1","doi-asserted-by":"publisher","DOI":"10.1111\/bjh.14592"},{"key":"e_1_2_9_13_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.21550"},{"key":"e_1_2_9_14_1","doi-asserted-by":"publisher","DOI":"10.1002\/pbc.27352"},{"key":"e_1_2_9_15_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.25698"},{"issue":"9","key":"e_1_2_9_16_1","article-title":"Hydroxyurea (hydroxycarbamide) for sickle cell disease","volume":"9","author":"Rankine\u2010Mullings AE","year":"2022","journal-title":"Cochrane Database Syst Rev"},{"key":"e_1_2_9_17_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(11)60355-3"},{"key":"e_1_2_9_18_1","doi-asserted-by":"publisher","DOI":"10.1111\/ejh.12361"},{"key":"e_1_2_9_19_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jss.2022.11.026"},{"key":"e_1_2_9_20_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.27214"},{"key":"e_1_2_9_21_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(86)92205-1"},{"key":"e_1_2_9_22_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2141.1991.tb08013.x"},{"key":"e_1_2_9_23_1","doi-asserted-by":"publisher","DOI":"10.5144\/0256-4947.1998.217"}],"container-title":["Pediatric Blood &amp; Cancer"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/pbc.31219","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,1,18]],"date-time":"2025-01-18T05:39:16Z","timestamp":1737178756000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/pbc.31219"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,7,25]]},"references-count":22,"journal-issue":{"issue":"10","published-print":{"date-parts":[[2024,10]]}},"alternative-id":["10.1002\/pbc.31219"],"URL":"https:\/\/doi.org\/10.1002\/pbc.31219","archive":["Portico"],"relation":{},"ISSN":["1545-5009","1545-5017"],"issn-type":[{"value":"1545-5009","type":"print"},{"value":"1545-5017","type":"electronic"}],"subject":[],"published":{"date-parts":[[2024,7,25]]},"assertion":[{"value":"2023-12-17","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2024-07-04","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2024-07-25","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}],"article-number":"e31219"}}