{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,10]],"date-time":"2026-09-10T04:59:53Z","timestamp":1789016393383,"version":"build-2803163510"},"reference-count":43,"publisher":"Wiley","issue":"12","license":[{"start":{"date-parts":[[2022,9,27]],"date-time":"2022-09-27T00:00:00Z","timestamp":1664236800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/100010269","name":"Wellcome Trust","doi-asserted-by":"publisher","award":["202800\/Z\/16\/Z"],"award-info":[{"award-number":["202800\/Z\/16\/Z"]}],"id":[{"id":"10.13039\/100010269","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100010269","name":"Wellcome Trust","doi-asserted-by":"publisher","award":["091758\/Z\/10\/Z"],"award-info":[{"award-number":["091758\/Z\/10\/Z"]}],"id":[{"id":"10.13039\/100010269","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["American J Hematol"],"published-print":{"date-parts":[[2022,12]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>It is well established that splenic dysfunction occurs in early childhood in sickle cell anemia (SCA), although the determinants and consequences of splenic injury are not fully understood. In this study, we examined spleen size and splenic function in 100 children with SCA aged 0\u201316\u2009years at King's College Hospital in London. Spleen size was assessed by abdominal ultrasound (US) and splenic function by pitted red blood cells (PIT counts). In our cohort, 5.6% of children aged 6\u201310\u2009years and 19.4% of children aged 11\u201316\u2009years had no visible spleen on US (autosplenectomy). Splenomegaly was common in all age groups, with 28% of children overall having larger spleens than the average for their age. Only one child had a PIT count suggesting preserved splenic function. We found no correlation between hemoglobin F levels and spleen size, nor was there any difference in spleen size between children treated with or without hydroxyurea. Although there was a trend toward increased spleen length in children with co\u2010inherited \u03b1\u2010thalassemia, this did not reach statistical significance. Finally, we found a strong association between erythrocyte deformability measured with oxygen gradient ektacytometry, spleen size, and PIT counts. In conclusion, our results do not agree with the general perception that most children with SCA undergo autosplenectomy within the first decade of life and indicate that loss of erythrocyte deformability contributes to loss of splenic filtration capacity in SCA, as well as phenotypical variations in spleen size.<\/jats:p>","DOI":"10.1002\/ajh.26703","type":"journal-article","created":{"date-parts":[[2022,8,28]],"date-time":"2022-08-28T10:08:47Z","timestamp":1661681327000},"page":"1520-1528","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":18,"title":["The significance of spleen size in children with sickle cell anemia"],"prefix":"10.1002","volume":"97","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-5256-7330","authenticated-orcid":false,"given":"Amina","family":"Nardo\u2010Marino","sequence":"first","affiliation":[{"name":"Danish Centre for Haemoglobinopathies, Department of Haematology Copenhagen University Hospital, Rigshospitalet  Copenhagen Denmark"},{"name":"Department of Immunology and Microbiology, Centre for Medical Parasitology University of Copenhagen  Copenhagen Denmark"},{"name":"Department of Haematological Medicine King's College Hospital  London United Kingdom"},{"name":"Comprehensive Cancer Centre, School of Cancer and Pharmaceutical Sciences King's College London  London United Kingdom"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2082-0738","authenticated-orcid":false,"given":"Andreas","family":"Glenth\u00f8j","sequence":"additional","affiliation":[{"name":"Danish Centre for Haemoglobinopathies, Department of Haematology Copenhagen University Hospital, Rigshospitalet  Copenhagen Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6129-4300","authenticated-orcid":false,"given":"John N.","family":"Brewin","sequence":"additional","affiliation":[{"name":"Department of Haematological Medicine King's College Hospital  London United Kingdom"},{"name":"Comprehensive Cancer Centre, School of Cancer and Pharmaceutical Sciences King's College London  London United Kingdom"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6116-5114","authenticated-orcid":false,"given":"Jesper","family":"Petersen","sequence":"additional","affiliation":[{"name":"Danish Centre for Haemoglobinopathies, Department of Haematology Copenhagen University Hospital, Rigshospitalet  Copenhagen Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thomas H.","family":"Braunstein","sequence":"additional","affiliation":[{"name":"Core Facility for Integrated Microscopy, Faculty of Health and Medical Sciences University of Copenhagen  Copenhagen Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2760-8713","authenticated-orcid":false,"given":"J\u00f8rgen A. L.","family":"Kurtzhals","sequence":"additional","affiliation":[{"name":"Department of Immunology and Microbiology, Centre for Medical Parasitology University of Copenhagen  Copenhagen Denmark"},{"name":"Department of Clinical Microbiology Copenhagen University Hospital, Rigshospitalet  Copenhagen Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4456-2382","authenticated-orcid":false,"given":"Thomas N.","family":"Williams","sequence":"additional","affiliation":[{"name":"KEMRI\u2010Wellcome Trust Research Programme  Kilifi Kenya"},{"name":"Department of Surgery and Cancer Institute of Global Health Innovation, Imperial College London  London United Kingdom"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3647-1050","authenticated-orcid":false,"given":"David C.","family":"Rees","sequence":"additional","affiliation":[{"name":"Department of Haematological Medicine King's College Hospital  London United Kingdom"},{"name":"Comprehensive Cancer Centre, School of Cancer and Pharmaceutical Sciences King's College London  London United Kingdom"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2022,9,27]]},"reference":[{"key":"e_1_2_10_2_1","doi-asserted-by":"publisher","DOI":"10.1111\/bjh.12950"},{"key":"e_1_2_10_3_1","doi-asserted-by":"publisher","DOI":"10.1542\/peds.76.3.392"},{"key":"e_1_2_10_4_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2010-04-278747"},{"key":"e_1_2_10_5_1","doi-asserted-by":"publisher","DOI":"10.1182\/bloodadvances.2019000106"},{"key":"e_1_2_10_6_1","doi-asserted-by":"publisher","DOI":"10.1097\/00005792-197103000-00002"},{"key":"e_1_2_10_7_1","doi-asserted-by":"publisher","DOI":"10.1097\/00000658-195208000-00006"},{"key":"e_1_2_10_8_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2141.2011.08999.x"},{"key":"e_1_2_10_9_1","doi-asserted-by":"publisher","DOI":"10.1016\/0002-9343(56)90190-5"},{"key":"e_1_2_10_10_1","doi-asserted-by":"publisher","DOI":"10.1001\/jama.1935.02760070020005"},{"issue":"3","key":"e_1_2_10_11_1","first-page":"267","article-title":"Natural history of sickle cell disease\u2010\u2010the first ten years","volume":"12","author":"Powars DR","year":"1975","journal-title":"Semin Hematol"},{"key":"e_1_2_10_12_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2141.2009.07771.x"},{"key":"e_1_2_10_13_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2009-07-233528"},{"key":"e_1_2_10_14_1","doi-asserted-by":"publisher","DOI":"10.5144\/0256-4947.1998.217"},{"key":"e_1_2_10_15_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM198206173062402"},{"key":"e_1_2_10_16_1","doi-asserted-by":"publisher","DOI":"10.1111\/bjh.17975"},{"issue":"1","key":"e_1_2_10_17_1","first-page":"79","article-title":"Minimum training requirements for the practice of medical ultrasound in Europe. Appendix 5: gastroenterological ultrasound. Ultraschall in der Medizin\/European","volume":"27","author":"EFSUMB","year":"2006","journal-title":"J Ultrasound"},{"key":"e_1_2_10_18_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF00973157"},{"key":"e_1_2_10_19_1","doi-asserted-by":"publisher","DOI":"10.2214\/AJR.10.4664"},{"key":"e_1_2_10_20_1","doi-asserted-by":"publisher","DOI":"10.1111\/bjh.16366"},{"key":"e_1_2_10_21_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V95.1.360"},{"key":"e_1_2_10_22_1","doi-asserted-by":"publisher","DOI":"10.3389\/fphys.2022.859906"},{"key":"e_1_2_10_23_1","doi-asserted-by":"publisher","DOI":"10.2214\/ajr.157.1.2048509"},{"key":"e_1_2_10_24_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM196910232811703"},{"key":"e_1_2_10_25_1","doi-asserted-by":"publisher","DOI":"10.1001\/archpedi.1994.02170080026005"},{"key":"e_1_2_10_26_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM198606193142501"},{"key":"e_1_2_10_27_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(09)61374-X"},{"issue":"3","key":"e_1_2_10_28_1","first-page":"133","article-title":"Prevalence of abdominal ultrasonographic abnormalities in patients with sickle cell disease","volume":"14","author":"Balci A","year":"2008","journal-title":"Diagn Interv Radiol"},{"key":"e_1_2_10_29_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpeds.2011.07.050"},{"key":"e_1_2_10_30_1","doi-asserted-by":"publisher","DOI":"10.7863\/ultra.15.09023"},{"key":"e_1_2_10_31_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-3476(84)80950-6"},{"key":"e_1_2_10_32_1","doi-asserted-by":"publisher","DOI":"10.15557\/JoU.2018.0001"},{"key":"e_1_2_10_33_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ultrasmedbio.2016.03.005"},{"issue":"1","key":"e_1_2_10_34_1","first-page":"40","article-title":"Malaria parasite density and splenic status by ultrasonography in stable sickle\u2010cell anaemia (HbSS) children","volume":"13","author":"Awotua\u2010Efebo O","year":"2004","journal-title":"Niger J Med"},{"key":"e_1_2_10_35_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.2830420313"},{"issue":"2","key":"e_1_2_10_36_1","doi-asserted-by":"crossref","first-page":"165","DOI":"10.4103\/1117-1936.163628","article-title":"Ultrasonographic spleen size and haematological parameters in children with sickle cell anaemia in Kano, Nigeria","volume":"21","author":"Abdullahi SU","year":"2014","journal-title":"Niger Postgrad Med J"},{"key":"e_1_2_10_37_1","first-page":"34","article-title":"Sonographic determination of splenic volume in children with sickle cell anaemia in steady state","volume":"18","author":"Yakubu CIIN","year":"2017","journal-title":"West Afr J Ultrasound"},{"key":"e_1_2_10_38_1","doi-asserted-by":"publisher","DOI":"10.4103\/ATMPH.ATMPH_393_17"},{"key":"e_1_2_10_39_1","doi-asserted-by":"publisher","DOI":"10.4103\/0331-8540.102106"},{"issue":"4","key":"e_1_2_10_40_1","first-page":"227","article-title":"Clinical and laboratory findings of cytomegalovirus infection in 115 hospitalized non\u2010immunocompromised adults","volume":"152","author":"Bonnet F","year":"2001","journal-title":"Ann Med Interne"},{"key":"e_1_2_10_41_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.clinimag.2012.10.003"},{"key":"e_1_2_10_42_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(11)60355-3"},{"key":"e_1_2_10_43_1","doi-asserted-by":"publisher","DOI":"10.1111\/ejh.12361"},{"key":"e_1_2_10_44_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V88.6.1951.bloodjournal8861951"}],"container-title":["American Journal of Hematology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/ajh.26703","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1002\/ajh.26703","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/ajh.26703","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,8,19]],"date-time":"2023-08-19T21:03:32Z","timestamp":1692479012000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/ajh.26703"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,9,27]]},"references-count":43,"journal-issue":{"issue":"12","published-print":{"date-parts":[[2022,12]]}},"alternative-id":["10.1002\/ajh.26703"],"URL":"https:\/\/doi.org\/10.1002\/ajh.26703","archive":["Portico"],"relation":{},"ISSN":["0361-8609","1096-8652"],"issn-type":[{"value":"0361-8609","type":"print"},{"value":"1096-8652","type":"electronic"}],"subject":[],"published":{"date-parts":[[2022,9,27]]},"assertion":[{"value":"2022-05-31","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2022-08-23","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2022-09-27","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}