{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,25]],"date-time":"2026-09-25T22:36:06Z","timestamp":1790375766430,"version":"4.1.0"},"reference-count":49,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2013,11,13]],"date-time":"2013-11-13T00:00:00Z","timestamp":1384300800000},"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":["Br J Haematol"],"published-print":{"date-parts":[[2014,2]]},"abstract":"<jats:title>Summary<\/jats:title><jats:p>Sickle cell disease (<jats:styled-content style=\"fixed-case\">SCD<\/jats:styled-content>) in <jats:styled-content style=\"fixed-case\">S<\/jats:styled-content>audi patients from the <jats:styled-content style=\"fixed-case\">E<\/jats:styled-content>astern <jats:styled-content style=\"fixed-case\">P<\/jats:styled-content>rovince is associated with the <jats:styled-content style=\"fixed-case\">A<\/jats:styled-content>rab\u2010<jats:styled-content style=\"fixed-case\">I<\/jats:styled-content>ndian (<jats:styled-content style=\"fixed-case\">AI<\/jats:styled-content>) <jats:italic><jats:styled-content style=\"fixed-case\">HBB<\/jats:styled-content><\/jats:italic> (\u03b2\u2010globin gene) haplotype. The phenotype of <jats:styled-content style=\"fixed-case\">AI SCD<\/jats:styled-content> in children was described as benign and was attributed to their high fetal haemoglobin (<jats:styled-content style=\"fixed-case\">H<\/jats:styled-content>b<jats:styled-content style=\"fixed-case\">F<\/jats:styled-content>). We conducted a hospital\u2010based study to assess the pattern of <jats:styled-content style=\"fixed-case\">SCD<\/jats:styled-content> complications in adults. A total of 104 patients with average age of 27\u00a0years were enrolled. Ninety\u2010six per cent of these patients reported history of painful crisis; 47% had at least one episode of acute chest syndrome, however, only 15% had two or more episodes; symptomatic osteonecrosis was reported in 18%; priapism in 17%; overt stroke in 6%; none had leg ulcers. The majority of patients had persistent splenomegaly and 66% had gallstones. Half of the patients co\u2010inherited \u03b1\u2010thalassaemia and about one\u2010third had glucose\u20106\u2010phosphate dehydrogenase deficiency. Higher HbF correlated with higher rate of splenic sequestration but not with other phenotypes. The phenotype of adult patients with <jats:styled-content style=\"fixed-case\">AI SCD<\/jats:styled-content> is not benign despite their relatively high HbF level. This is probably due to the continued decline in HbF level in adults and the heterocellular and variable distribution of HbF amongst F\u2010cells.<\/jats:p>","DOI":"10.1111\/bjh.12650","type":"journal-article","created":{"date-parts":[[2013,11,13]],"date-time":"2013-11-13T12:55:04Z","timestamp":1384347304000},"page":"597-604","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":85,"title":["Sickle cell disease in <scp>S<\/scp>audi <scp>A<\/scp>rabia: the phenotype in adults with the <scp>A<\/scp>rab\u2010<scp>I<\/scp>ndian haplotype is not benign"],"prefix":"10.1111","volume":"164","author":[{"given":"Abdulrahman","family":"Alsultan","sequence":"first","affiliation":[{"name":"Department of Paediatrics Sickle Cell Disease Research Centre College of Medicine King Saud University Riyadh Saudi Arabia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mohammed K.","family":"Alabdulaali","sequence":"additional","affiliation":[{"name":"King Fahad Hospital Hofuf Saudi Arabia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paula J.","family":"Griffin","sequence":"additional","affiliation":[{"name":"Department of Biostatistics Boston University School of Public Health Boston MA USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ahmed M.","family":"AlSuliman","sequence":"additional","affiliation":[{"name":"King Fahad Hospital Hofuf Saudi Arabia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hazem A.","family":"Ghabbour","sequence":"additional","affiliation":[{"name":"College of Pharmacy King Saud University Riyadh Saudi Arabia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paola","family":"Sebastiani","sequence":"additional","affiliation":[{"name":"Department of Biostatistics Boston University School of Public Health Boston MA USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Waleed H.","family":"Albuali","sequence":"additional","affiliation":[{"name":"Al\u2010Omran Scientific Chair College of Medicine King Faisal University Al\u2010Ahsa Saudi Arabia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Amein K.","family":"Al\u2010Ali","sequence":"additional","affiliation":[{"name":"Al\u2010Omran Scientific Chair College of Medicine King Faisal University Al\u2010Ahsa Saudi Arabia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David H. K.","family":"Chui","sequence":"additional","affiliation":[{"name":"Department of Medicine Centre of Excellence in Sickle Cell Disease Boston University School of Medicine Boston MA USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Martin H.","family":"Steinberg","sequence":"additional","affiliation":[{"name":"Department of Medicine Centre of Excellence in Sickle Cell Disease Boston University School of Medicine Boston MA USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2013,11,13]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.3109\/03630269.2011.617230"},{"key":"e_1_2_7_3_1","doi-asserted-by":"publisher","DOI":"10.1159\/000046529"},{"key":"e_1_2_7_4_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.10143"},{"key":"e_1_2_7_5_1","doi-asserted-by":"publisher","DOI":"10.1159\/000102151"},{"key":"e_1_2_7_6_1","doi-asserted-by":"publisher","DOI":"10.1159\/000141506"},{"key":"e_1_2_7_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-3476(84)80950-6"},{"key":"e_1_2_7_8_1","doi-asserted-by":"publisher","DOI":"10.1002\/(SICI)1096-8652(200002)63:2<68::AID-AJH2>3.0.CO;2-N"},{"key":"e_1_2_7_9_1","first-page":"1480","article-title":"The prevalence of sickle cell disease in Saudi children and adolescents. A community\u2010based survey","volume":"29","author":"Al\u2010Qurashi M.M.","year":"2008","journal-title":"Saudi Medical Journal"},{"key":"e_1_2_7_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpedsurg.2006.06.020"},{"key":"e_1_2_7_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0002-9610(96)00158-4"},{"key":"e_1_2_7_12_1","doi-asserted-by":"crossref","first-page":"53","DOI":"10.5144\/0256-4947.2005.53","article-title":"Acute chest syndrome in adult sickle cell disease in eastern Saudi Arabia","volume":"25","author":"Al\u2010Suleiman A.","year":"2005","journal-title":"Annals of Saudi Medicine"},{"key":"e_1_2_7_13_1","doi-asserted-by":"publisher","DOI":"10.5144\/0256-4947.2006.487"},{"key":"e_1_2_7_14_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.22032"},{"key":"e_1_2_7_15_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.23239"},{"key":"e_1_2_7_16_1","doi-asserted-by":"publisher","DOI":"10.1097\/MPH.0b013e3182422844"},{"key":"e_1_2_7_17_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1600-0609.1985.tb01570.x"},{"key":"e_1_2_7_18_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V84.2.643.643"},{"key":"e_1_2_7_19_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2011-02-272682"},{"key":"e_1_2_7_20_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-5347(17)38248-4"},{"key":"e_1_2_7_21_1","first-page":"611","volume-title":"Disorders of Hemoglobin: Genetics, Pathophysiology, and Clinical Management","author":"Frempong K.O.","year":"2001"},{"key":"e_1_2_7_22_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V86.2.776.bloodjournal862776"},{"key":"e_1_2_7_23_1","doi-asserted-by":"publisher","DOI":"10.1038\/198492b0"},{"key":"e_1_2_7_24_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.0804799105"},{"key":"e_1_2_7_25_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V84.9.3182.3182"},{"key":"e_1_2_7_26_1","doi-asserted-by":"crossref","first-page":"143","DOI":"10.1080\/02724936.1985.11748380","article-title":"Hypersplenism in homozygous sickle\u2010cell disease in Saudi Arabia","volume":"5","author":"Mallouh A.A.","year":"1985","journal-title":"Annals of Tropical Paediatrics"},{"key":"e_1_2_7_27_1","doi-asserted-by":"publisher","DOI":"10.1080\/02724936.1984.11748315"},{"key":"e_1_2_7_28_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.10376"},{"key":"e_1_2_7_29_1","doi-asserted-by":"publisher","DOI":"10.1159\/000072406"},{"key":"e_1_2_7_30_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM199111213252104"},{"key":"e_1_2_7_31_1","first-page":"205","article-title":"Infections and sickle cell disease in Eastern Saudi Arabian children","volume":"143","author":"el Mouzan M.I.","year":"1989","journal-title":"American Journal of Diseases of Children"},{"key":"e_1_2_7_32_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2141.2011.08916.x"},{"key":"e_1_2_7_33_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.bcmd.2012.12.005"},{"key":"e_1_2_7_34_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2141.2006.06074.x"},{"key":"e_1_2_7_35_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2141.1991.tb08013.x"},{"key":"e_1_2_7_36_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2141.1978.tb05813.x"},{"key":"e_1_2_7_37_1","first-page":"26","article-title":"Sickle beta 0 thalassemia in Eastern Saudi Arabia","volume":"32","author":"Pembrey M.E.","year":"1980","journal-title":"American Journal of Human Genetics"},{"key":"e_1_2_7_38_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(72)92592-5"},{"key":"e_1_2_7_39_1","doi-asserted-by":"publisher","DOI":"10.7326\/0003-4819-88-1-1"},{"key":"e_1_2_7_40_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM199107043250103"},{"key":"e_1_2_7_41_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.90.11.5039"},{"key":"e_1_2_7_42_1","doi-asserted-by":"publisher","DOI":"10.1172\/JCI109720"},{"key":"e_1_2_7_43_1","doi-asserted-by":"publisher","DOI":"10.1097\/01.md.0000189089.45003.52"},{"key":"e_1_2_7_44_1","first-page":"66","article-title":"Cholelithiasis in patients with major sickle hemoglobinopathies","volume":"138","author":"Rennels M.B.","year":"1984","journal-title":"American Journal of Diseases of Children"},{"key":"e_1_2_7_45_1","doi-asserted-by":"crossref","first-page":"690","DOI":"10.1001\/archinte.1974.00320160184017","article-title":"Leg ulceration in sickle cell anemia","volume":"133","author":"Serjeant G.R.","year":"1974","journal-title":"Archives of Internal Medicine"},{"key":"e_1_2_7_46_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V68.5.985.985"},{"key":"e_1_2_7_47_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.23232"},{"key":"e_1_2_7_48_1","doi-asserted-by":"crossref","first-page":"748","DOI":"10.1182\/blood.V71.3.748.748","article-title":"Effects of glucose\u20106\u2010phosphate dehydrogenase deficiency upon sickle cell anemia","volume":"71","author":"Steinberg M.H.","year":"1988","journal-title":"Blood"},{"key":"e_1_2_7_49_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V98.1.250"},{"key":"e_1_2_7_50_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2141.2002.03427.x"}],"container-title":["British Journal of Haematology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fbjh.12650","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/bjh.12650","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,10,3]],"date-time":"2023-10-03T14:44:16Z","timestamp":1696344256000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/bjh.12650"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2013,11,13]]},"references-count":49,"journal-issue":{"issue":"4","published-print":{"date-parts":[[2014,2]]}},"alternative-id":["10.1111\/bjh.12650"],"URL":"https:\/\/doi.org\/10.1111\/bjh.12650","archive":["Portico"],"relation":{},"ISSN":["0007-1048","1365-2141"],"issn-type":[{"value":"0007-1048","type":"print"},{"value":"1365-2141","type":"electronic"}],"subject":[],"published":{"date-parts":[[2013,11,13]]},"assertion":[{"value":"2013-08-22","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2013-10-04","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2013-11-13","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}