{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,27]],"date-time":"2026-10-27T15:30:10Z","timestamp":1793115010455,"version":"4.5.0"},"reference-count":189,"publisher":"Elsevier BV","license":[{"start":{"date-parts":[[2022,11,1]],"date-time":"2022-11-01T00:00:00Z","timestamp":1667260800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2022,11,1]],"date-time":"2022-11-01T00:00:00Z","timestamp":1667260800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"},{"start":{"date-parts":[[2022,6,19]],"date-time":"2022-06-19T00:00:00Z","timestamp":1655596800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["clinicalkey.com","clinicalkey.com.au","clinicalkey.es","clinicalkey.fr","clinicalkey.jp","elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Blood Reviews"],"published-print":{"date-parts":[[2022,11]]},"DOI":"10.1016\/j.blre.2022.100983","type":"journal-article","created":{"date-parts":[[2022,6,9]],"date-time":"2022-06-09T22:00:41Z","timestamp":1654812041000},"page":"100983","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":82,"special_numbering":"C","title":["Determinants of severity in sickle cell disease"],"prefix":"10.1016","volume":"56","author":[{"given":"David C.","family":"Rees","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Valentine A.M.","family":"Brousse","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"John N.","family":"Brewin","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1016\/j.blre.2022.100983_bb0005","doi-asserted-by":"crossref","DOI":"10.7189\/jogh.08.021103","article-title":"The global burden of sickle cell disease in children under five years of age: a systematic review and meta-analysis","volume":"8","author":"Wastnedge","year":"2018","journal-title":"J Glob Health"},{"key":"10.1016\/j.blre.2022.100983_bb0010","doi-asserted-by":"crossref","first-page":"197","DOI":"10.3181\/00379727-75-18144","article-title":"Studies on the destruction of red blood cells. VIII. Molecular orientation in sickle cell hemoglobin solutions","volume":"75","author":"Harris","year":"1950","journal-title":"Proc Soc Exp Biol Med"},{"key":"10.1016\/j.blre.2022.100983_bb0015","doi-asserted-by":"crossref","DOI":"10.1136\/bmj.g1765","article-title":"Management of sickle cell disease in the community","volume":"348","author":"Brousse","year":"2014","journal-title":"BMJ"},{"key":"10.1016\/j.blre.2022.100983_bb0020","doi-asserted-by":"crossref","first-page":"149","DOI":"10.1111\/bjh.16504","article-title":"Emerging therapies in sickle cell disease","volume":"190","author":"Nardo-Marino","year":"2020","journal-title":"Br J Haematol"},{"key":"10.1016\/j.blre.2022.100983_bb0025","doi-asserted-by":"crossref","first-page":"11","DOI":"10.1056\/NEJM199107043250103","article-title":"Pain in sickle cell disease. Rates and risk factors","volume":"325","author":"Platt","year":"1991","journal-title":"N Engl J Med"},{"key":"10.1016\/j.blre.2022.100983_bb0030","doi-asserted-by":"crossref","first-page":"1130","DOI":"10.1182\/blood-2010-06-293514","article-title":"Impact of early transcranial Doppler screening and intensive therapy on cerebral vasculopathy outcome in a newborn sickle cell anemia cohort","volume":"117","author":"Bernaudin","year":"2011","journal-title":"Blood"},{"key":"10.1016\/j.blre.2022.100983_bb0035","doi-asserted-by":"crossref","first-page":"905","DOI":"10.3324\/haematol.10937","article-title":"Clinical outcomes in children with sickle cell disease living in England: a neonatal cohort in East London","volume":"92","author":"Telfer","year":"2007","journal-title":"Haematologica"},{"key":"10.1016\/j.blre.2022.100983_bb0040","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pone.0014699","article-title":"Mortality in sickle cell anemia in Africa: a prospective cohort study in Tanzania","volume":"6","author":"Makani","year":"2011","journal-title":"PLoS One"},{"key":"10.1016\/j.blre.2022.100983_bb0045","doi-asserted-by":"crossref","first-page":"1108","DOI":"10.3324\/haematol.2014.120030","article-title":"Environmental determinants of severity in sickle cell disease","volume":"100","author":"Tewari","year":"2015","journal-title":"Haematologica"},{"key":"10.1016\/j.blre.2022.100983_bb0050","doi-asserted-by":"crossref","first-page":"1197","DOI":"10.1038\/ng2108","article-title":"A QTL influencing F cell production maps to a gene encoding a zinc-finger protein on chromosome 2p15","volume":"39","author":"Menzel","year":"2007","journal-title":"Nat Genet"},{"key":"10.1016\/j.blre.2022.100983_bb0055","doi-asserted-by":"crossref","first-page":"205","DOI":"10.1056\/NEJMoa2029392","article-title":"Post-transcriptional genetic silencing of BCL11A to treat sickle cell disease","volume":"384","author":"Esrick","year":"2021","journal-title":"N Engl J Med"},{"key":"10.1016\/j.blre.2022.100983_bb0060","doi-asserted-by":"crossref","first-page":"252","DOI":"10.1056\/NEJMoa2031054","article-title":"CRISPR-Cas9 gene editing for sickle cell disease and beta-thalassemia","volume":"384","author":"Frangoul","year":"2021","journal-title":"N Engl J Med"},{"key":"10.1016\/j.blre.2022.100983_bb0065","doi-asserted-by":"crossref","first-page":"1771","DOI":"10.1073\/pnas.81.6.1771","article-title":"Evidence for the multicentric origin of the sickle cell hemoglobin gene in Africa","volume":"81","author":"Pagnier","year":"1984","journal-title":"Proc Natl Acad Sci U S A"},{"key":"10.1016\/j.blre.2022.100983_bb0070","doi-asserted-by":"crossref","first-page":"1742","DOI":"10.1182\/blood.V69.6.1742.1742","article-title":"Fetal hemoglobin levels and beta (s) globin haplotypes in an Indian populations with sickle cell disease","volume":"69","author":"Kulozik","year":"1987","journal-title":"Blood"},{"key":"10.1016\/j.blre.2022.100983_bb0075","doi-asserted-by":"crossref","first-page":"853","DOI":"10.1073\/pnas.81.3.853","article-title":"Origin of the beta S-globin gene in blacks: the contribution of recurrent mutation or gene conversion or both","volume":"81","author":"Antonarakis","year":"1984","journal-title":"Proc Natl Acad Sci U S A"},{"key":"10.1016\/j.blre.2022.100983_bb0080","doi-asserted-by":"crossref","first-page":"4431","DOI":"10.1073\/pnas.85.12.4431","article-title":"Structural analysis of the 5\u2019 flanking region of the \u03b2-globin gene in African sickle cell anemia patients: Further evidence for three origins of the sickle cell mutation in Africa","volume":"85","author":"Chebloune","year":"1988","journal-title":"Proc Natl Acad Sci U S A"},{"key":"10.1016\/j.blre.2022.100983_bb0085","first-page":"53","article-title":"The origin of the hemoglobin S gene: Clinical, genetic, and anthropological consequences","volume":"2","author":"Nagel","year":"1984","journal-title":"Einstein Q J Biol Med"},{"key":"10.1016\/j.blre.2022.100983_bb0090","doi-asserted-by":"crossref","first-page":"547","DOI":"10.1016\/j.ajhg.2018.02.003","article-title":"Whole-genome-sequence-based haplotypes reveal single origin of the sickle allele during the holocene wet phase","volume":"102","author":"Shriner","year":"2018","journal-title":"Am J Hum Genet"},{"key":"10.1016\/j.blre.2022.100983_bb0095","doi-asserted-by":"crossref","first-page":"145","DOI":"10.1002\/ajh.2830440214","article-title":"Senegal haplotype is associated with higher HbF than benin and cameroon haplotypes in African children with sickle cell anemia","volume":"44","author":"Green","year":"1993","journal-title":"Am J Hematol"},{"key":"10.1016\/j.blre.2022.100983_bb0100","doi-asserted-by":"crossref","first-page":"1197","DOI":"10.1001\/archpedi.1993.02160350071011","article-title":"Sickle cell anemia: \u03b2s gene cluster haplotypes as genetic markers for severe disease expression","volume":"147","author":"Powars","year":"1993","journal-title":"Am J Dis Child"},{"key":"10.1016\/j.blre.2022.100983_bb0105","doi-asserted-by":"crossref","first-page":"1","DOI":"10.7326\/0003-4819-88-1-1","article-title":"Natural history of sickle cell anemia in Saudi Arabs. A study of 270 subjects","volume":"88","author":"Perrine","year":"1978","journal-title":"Ann Intern Med"},{"key":"10.1016\/j.blre.2022.100983_bb0110","doi-asserted-by":"crossref","first-page":"2111","DOI":"10.1073\/pnas.82.7.2111","article-title":"Common haplotype dependency of high G gamma-globin gene expression and high Hb F levels in beta-thalassemia and sickle cell anemia patients","volume":"82","author":"Labie","year":"1985","journal-title":"Proc Natl Acad Sci U S A"},{"key":"10.1016\/j.blre.2022.100983_bb0115","doi-asserted-by":"crossref","first-page":"880","DOI":"10.1056\/NEJM198504043121403","article-title":"Hematologically and genetically distinct forms of sickle cell anemia in Africa. The Senegal type and the Benin type","volume":"312","author":"Nagel","year":"1985","journal-title":"N Engl J Med"},{"key":"10.1016\/j.blre.2022.100983_bb0120","doi-asserted-by":"crossref","first-page":"66","DOI":"10.1016\/j.bcmd.2017.06.004","article-title":"Variability of homozygous sickle cell disease: The role of alpha and beta globin chain variation and other factors","volume":"70","author":"Serjeant","year":"2018","journal-title":"Blood Cells Mol Dis"},{"key":"10.1016\/j.blre.2022.100983_bb0125","doi-asserted-by":"crossref","first-page":"783","DOI":"10.1182\/blood.V66.4.783.783","article-title":"DNA sequence variation associated with elevated fetal G gamma globin production","volume":"66","author":"Gilman","year":"1985","journal-title":"Blood"},{"key":"10.1016\/j.blre.2022.100983_bb0130","doi-asserted-by":"crossref","first-page":"1463","DOI":"10.1182\/blood.V66.6.1463.1463","article-title":"The -158 site 5\u2019 to the G gamma gene and G gamma expression","volume":"66","author":"Labie","year":"1985","journal-title":"Blood"},{"key":"10.1016\/j.blre.2022.100983_bb0135","doi-asserted-by":"crossref","first-page":"419","DOI":"10.1097\/00000441-194804000-00008","article-title":"The significance of the paucity of sickle cells in newborn Negro infants","volume":"215","author":"Watson","year":"1948","journal-title":"Am J Med Sci"},{"key":"10.1016\/j.blre.2022.100983_bb0140","doi-asserted-by":"crossref","first-page":"1639","DOI":"10.1056\/NEJM199406093302303","article-title":"Mortality in sickle cell disease. Life expectancy and risk factors for early death","volume":"330","author":"Platt","year":"1994","journal-title":"N Engl J Med"},{"key":"10.1016\/j.blre.2022.100983_bb0145","series-title":"Disorders of Hemoglobin: Genetics, Pathophysiology, Clinical Management","author":"Steinberg","year":"2009"},{"key":"10.1016\/j.blre.2022.100983_bb0150","doi-asserted-by":"crossref","first-page":"11346","DOI":"10.1073\/pnas.0611393104","article-title":"Intergenic variants of HBS1L-MYB are responsible for a major quantitative trait locus on chromosome 6q23 influencing fetal hemoglobin levels in adults","volume":"104","author":"Thein","year":"2007","journal-title":"Proc Natl Acad Sci U S A"},{"key":"10.1016\/j.blre.2022.100983_bb0155","doi-asserted-by":"crossref","first-page":"455","DOI":"10.1111\/j.1365-2141.2009.07650.x","article-title":"Discovering the genetics underlying foetal haemoglobin production in adults","volume":"145","author":"Thein","year":"2009","journal-title":"Br J Haematol"},{"key":"10.1016\/j.blre.2022.100983_bb0160","doi-asserted-by":"crossref","first-page":"11869","DOI":"10.1073\/pnas.0804799105","article-title":"DNA polymorphisms at the BCL11A, HBS1L-MYB, and beta-globin loci associate with fetal hemoglobin levels and pain crises in sickle cell disease","volume":"105","author":"Lettre","year":"2008","journal-title":"Proc Natl Acad Sci U S A"},{"key":"10.1016\/j.blre.2022.100983_bb0165","first-page":"342","article-title":"Genetic influences on F cells and other hematologic variables: a twin heritability study","volume":"95","author":"Garner","year":"2000","journal-title":"Blood J Am Soc Hematol"},{"key":"10.1016\/j.blre.2022.100983_bb0170","doi-asserted-by":"crossref","first-page":"1264","DOI":"10.1038\/ng.3307","article-title":"Genome-wide association analyses based on whole-genome sequencing in Sardinia provide insights into regulation of hemoglobin levels","volume":"47","author":"Danjou","year":"2015","journal-title":"Nat Genet"},{"key":"10.1016\/j.blre.2022.100983_bb0175","doi-asserted-by":"crossref","first-page":"188","DOI":"10.1111\/j.1749-6632.1985.tb17188.x","article-title":"Hb F production in stressed erythropoiesis: observations and kinetic models","volume":"445","author":"Stamatoyannopoulos","year":"1985","journal-title":"Ann N Y Acad Sci"},{"key":"10.1016\/j.blre.2022.100983_bb0180","doi-asserted-by":"crossref","first-page":"253","DOI":"10.1126\/science.1242088","article-title":"An erythroid enhancer of BCL11A subject to genetic variation determines fetal hemoglobin level","volume":"342","author":"Bauer","year":"2013","journal-title":"Science (New York, NY)"},{"key":"10.1016\/j.blre.2022.100983_bb0185","doi-asserted-by":"crossref","first-page":"4935","DOI":"10.1182\/blood-2010-11-317081","article-title":"A 3-bp deletion in the HBS1L-MYB intergenic region on chromosome 6q23 is associated with HbF expression","volume":"117","author":"Farrell","year":"2011","journal-title":"Blood"},{"key":"10.1016\/j.blre.2022.100983_bb0190","doi-asserted-by":"crossref","first-page":"235","DOI":"10.1182\/bloodadvances.2017009811","article-title":"g(HbF): a genetic model of fetal hemoglobin in sickle cell disease","volume":"2","author":"Gardner","year":"2018","journal-title":"Blood Adv"},{"key":"10.1016\/j.blre.2022.100983_bb0195","doi-asserted-by":"crossref","first-page":"985","DOI":"10.1182\/blood.V68.5.985.985","article-title":"Alpha-thalassemia in blacks: genetic and clinical aspects and interactions with the sickle hemoglobin gene","volume":"68","author":"Steinberg","year":"1986","journal-title":"Blood"},{"key":"10.1016\/j.blre.2022.100983_bb0200","doi-asserted-by":"crossref","first-page":"253","DOI":"10.1111\/j.1365-2141.1986.tb04117.x","article-title":"Haemoglobin gene frequencies in the Jamaican population: a study in 100,000 newborns","volume":"64","author":"Serjeant","year":"1986","journal-title":"Br J Haematol"},{"key":"10.1016\/j.blre.2022.100983_bb0205","doi-asserted-by":"crossref","first-page":"626","DOI":"10.1182\/bloodadvances.2017014555","article-title":"Biological impact of alpha genes, beta haplotypes, and G6PD activity in sickle cell anemia at baseline and with hydroxyurea","volume":"2","author":"Bernaudin","year":"2018","journal-title":"Blood Adv"},{"key":"10.1016\/j.blre.2022.100983_bb0210","doi-asserted-by":"crossref","first-page":"1441","DOI":"10.1056\/NEJM198206173062402","article-title":"The interaction of alpha-thalassemia and homozygous sickle-cell disease","volume":"306","author":"Higgs","year":"1982","journal-title":"N Engl J Med"},{"key":"10.1016\/j.blre.2022.100983_bb0215","doi-asserted-by":"crossref","first-page":"63","DOI":"10.1016\/S0065-3233(08)60287-9","article-title":"Sickle cell hemoglobin polymerization","volume":"40","author":"Eaton","year":"1990","journal-title":"Adv Protein Chem"},{"key":"10.1016\/j.blre.2022.100983_bb0220","doi-asserted-by":"crossref","first-page":"479","DOI":"10.1111\/j.1365-2141.1983.tb02163.x","article-title":"Effect of alpha thalassaemia on the rheology of homozygous sickle cell disease","volume":"55","author":"Serjeant","year":"1983","journal-title":"Br J Haematol"},{"key":"10.1016\/j.blre.2022.100983_bb0225","doi-asserted-by":"crossref","first-page":"116","DOI":"10.1172\/JCI111181","article-title":"Concurrent sickle cell anemia and alpha-thalassemia. Effect on pathological properties of sickle erythrocytes","volume":"73","author":"Embury","year":"1984","journal-title":"J Clin Investig"},{"key":"10.1016\/j.blre.2022.100983_bb0230","doi-asserted-by":"crossref","first-page":"189","DOI":"10.1056\/NEJM198307213090320","article-title":"O-thalassemia reduces the hemolytic rate in homozygous sickle-cell disease","volume":"309","author":"de Ceulaer","year":"1983","journal-title":"N Engl J Med"},{"key":"10.1016\/j.blre.2022.100983_bb0235","doi-asserted-by":"crossref","first-page":"622","DOI":"10.1097\/00043426-200308000-00007","article-title":"Alpha Thalassemia is associated with decreased risk of abnormal transcranial Doppler ultrasonography in children with sickle cell anemia","volume":"25","author":"Hsu","year":"2003","journal-title":"J Pediatr Hematol Oncol"},{"key":"10.1016\/j.blre.2022.100983_bb0240","doi-asserted-by":"crossref","first-page":"4314","DOI":"10.1182\/blood-2008-03-143891","article-title":"G6PD deficiency, absence of alpha-thalassemia, and hemolytic rate at baseline are significant independent risk factors for abnormally high cerebral velocities in patients with sickle cell anemia","volume":"112","author":"Bernaudin","year":"2008","journal-title":"Blood"},{"key":"10.1016\/j.blre.2022.100983_bb0245","doi-asserted-by":"crossref","first-page":"516","DOI":"10.3109\/03630269.2010.526003","article-title":"Coinheritance of \u03b1-thalassemia decreases the risk of cerebrovascular disease in a cohort of children with sickle cell anemia","volume":"34","author":"Belis\u00e1rio","year":"2010","journal-title":"Hemoglobin."},{"key":"10.1016\/j.blre.2022.100983_bb0250","doi-asserted-by":"crossref","first-page":"699","DOI":"10.1111\/bjh.12791","article-title":"Haptoglobin, alpha-thalassaemia and glucose-6-phosphate dehydrogenase polymorphisms and risk of abnormal transcranial Doppler among patients with sickle cell anaemia in Tanzania","volume":"165","author":"Cox","year":"2014","journal-title":"Br J Haematol"},{"key":"10.1016\/j.blre.2022.100983_bb0255","first-page":"288","article-title":"Cerebrovascular accidents in sickle cell disease: rates and risk factors","volume":"91","author":"Ohene-Frempong","year":"1998","journal-title":"Blood"},{"key":"10.1016\/j.blre.2022.100983_bb0260","doi-asserted-by":"crossref","first-page":"1770","DOI":"10.3324\/haematol.2020.265827","article-title":"Genome wide association study of silent cerebral infarction in sickle cell disease (HbSS and HbSC)","volume":"106","author":"Brewin","year":"2021","journal-title":"Haematologica"},{"key":"10.1016\/j.blre.2022.100983_bb0265","doi-asserted-by":"crossref","first-page":"570","DOI":"10.1111\/j.1365-2141.2006.06074.x","article-title":"Sickle cell leg ulcers: associations with haemolysis and SNPs in Klotho, TEK and genes of the TGF-beta\/BMP pathway","volume":"133","author":"Nolan","year":"2006","journal-title":"Br J Haematol"},{"key":"10.1016\/j.blre.2022.100983_bb0270","doi-asserted-by":"crossref","first-page":"1014","DOI":"10.1681\/ASN.V1051014","article-title":"Evidence that microdeletions in the \u03b1 globin gene protect against the development of sickle cell glomerulopathy in humans","volume":"10","author":"Guasch","year":"1999","journal-title":"J Am Soc Nephrol"},{"key":"10.1016\/j.blre.2022.100983_bb0275","doi-asserted-by":"crossref","first-page":"154","DOI":"10.1016\/j.bcmd.2010.06.003","article-title":"Alpha-thalassemia is associated with a decreased occurrence and a delayed age-at-onset of albuminuria in sickle cell anemia patients","volume":"45","author":"Nebor","year":"2010","journal-title":"Blood Cells Mol Dis"},{"key":"10.1016\/j.blre.2022.100983_bb0280","doi-asserted-by":"crossref","first-page":"63","DOI":"10.3233\/CH-131772","article-title":"Alpha thalassemia protects sickle cell anemia patients from macro-albuminuria through its effects on red blood cell rheological properties","volume":"57","author":"Lamarre","year":"2014","journal-title":"Clin Hemorheol Microcirc"},{"key":"10.1016\/j.blre.2022.100983_bb0285","doi-asserted-by":"crossref","DOI":"10.1002\/pbc.26455","article-title":"Alpha-thalassaemia promotes frequent vaso-occlusive crises in children with sickle cell anaemia through haemorheological changes","volume":"64","author":"Renoux","year":"2017","journal-title":"Pediatr Blood Cancer"},{"key":"10.1016\/j.blre.2022.100983_bb0290","doi-asserted-by":"crossref","first-page":"155","DOI":"10.1034\/j.1600-0609.2000.90210.x","article-title":"Acute clinical events in 299 homozygous sickle cell patients living in France. French Study Group on Sickle Cell Disease","volume":"65","author":"Neonato","year":"2000","journal-title":"Eur J Haematol"},{"key":"10.1016\/j.blre.2022.100983_bb0295","doi-asserted-by":"crossref","first-page":"4382","DOI":"10.1182\/blood.V86.11.4382a.bloodjournal86114382a","article-title":"Paradoxical increase of painful crises in sickle cell patients with alpha-thalassemia [letter; comment]","volume":"86","author":"Billett","year":"1995","journal-title":"Blood"},{"key":"10.1016\/j.blre.2022.100983_bb0300","doi-asserted-by":"crossref","first-page":"776","DOI":"10.1182\/blood.V86.2.776.bloodjournal862776","article-title":"Clinical events in the first decade in a cohort of infants with sickle cell disease. Cooperative Study of Sickle Cell Disease [see comments]","volume":"86","author":"Gill","year":"1995","journal-title":"Blood"},{"key":"10.1016\/j.blre.2022.100983_bb0305","doi-asserted-by":"crossref","first-page":"735","DOI":"10.1016\/0140-6736(91)90322-G","article-title":"Is the painful crisis of sickle-cell disease due to sickling?","volume":"337","author":"Bailey","year":"1991","journal-title":"Lancet"},{"key":"10.1016\/j.blre.2022.100983_bb0310","doi-asserted-by":"crossref","first-page":"286","DOI":"10.1016\/j.jpeds.2011.07.018","article-title":"Markers of severe vaso-occlusive painful episode frequency in children and adolescents with sickle cell anemia","volume":"160","author":"Darbari","year":"2012","journal-title":"J Pediatr"},{"key":"10.1016\/j.blre.2022.100983_bb0315","doi-asserted-by":"crossref","first-page":"649","DOI":"10.3109\/03630268908998842","article-title":"The prevalence of avascular necrosis in sickle cell anemia: correlation with alpha-thalassemia","volume":"13","author":"Ballas","year":"1989","journal-title":"Hemoglobin"},{"key":"10.1016\/j.blre.2022.100983_bb0320","doi-asserted-by":"crossref","first-page":"1476","DOI":"10.1056\/NEJM199111213252104","article-title":"Sickle cell disease as a cause of osteonecrosis of the femoral head","volume":"325","author":"Milner","year":"1991","journal-title":"N Engl J Med"},{"key":"10.1016\/j.blre.2022.100983_bb0325","doi-asserted-by":"crossref","first-page":"563","DOI":"10.1111\/j.1365-2141.1991.tb08628.x","article-title":"Acute splenic sequestration in Jamaican adults with homozygous sickle cell disease: a role of alpha thalassaemia","volume":"77","author":"De Ceulaer","year":"1991","journal-title":"Br J Haematol"},{"key":"10.1016\/j.blre.2022.100983_bb0330","doi-asserted-by":"crossref","first-page":"1436","DOI":"10.1182\/blood-2016-05-716910","article-title":"Survival in adults with sickle cell disease in a high-income setting","volume":"128","author":"Gardner","year":"2016","journal-title":"Blood"},{"key":"10.1016\/j.blre.2022.100983_bb0335","doi-asserted-by":"crossref","first-page":"589","DOI":"10.1111\/j.1365-2141.2008.07375.x","article-title":"The presence of alpha-thalassaemia trait blunts the response to hydroxycarbamide in patients with sickle cell disease","volume":"143","author":"Vasavda","year":"2008","journal-title":"Br J Haematol"},{"key":"10.1016\/j.blre.2022.100983_bb0340","doi-asserted-by":"crossref","first-page":"341","DOI":"10.1111\/ejh.12245","article-title":"Alpha-thalassaemia and response to hydroxyurea in sickle cell anaemia","volume":"92","author":"Darbari","year":"2014","journal-title":"Eur J Haematol"},{"key":"10.1016\/j.blre.2022.100983_bb0345","doi-asserted-by":"crossref","first-page":"571","DOI":"10.1002\/ajh.23457","article-title":"Genetic modifiers of sickle cell anemia in the BABY HUG cohort: influence on laboratory and clinical phenotypes","volume":"88","author":"Sheehan","year":"2013","journal-title":"Am J Hematol"},{"key":"10.1016\/j.blre.2022.100983_bb0350","doi-asserted-by":"crossref","first-page":"206","DOI":"10.1002\/ajh.10140","article-title":"Outcome in hemoglobin SC disease: a four-decade observational study of clinical, hematologic, and genetic factors","volume":"70","author":"Powars","year":"2002","journal-title":"Am J Hematol"},{"key":"10.1016\/j.blre.2022.100983_bb0355","doi-asserted-by":"crossref","DOI":"10.3324\/haematol.2018.209221","article-title":"Co-inheritance of a-thalassemia dramatically decreases the risk of acute splenic sequestration in a large cohort of newborns with hemoglobin SC","volume":"104","author":"Rezende","year":"2019","journal-title":"Haematologica"},{"key":"10.1016\/j.blre.2022.100983_bb0360","doi-asserted-by":"crossref","first-page":"487","DOI":"10.1111\/j.1365-2141.1983.tb02164.x","article-title":"The effects of alpha-thalassaemia in HbSC disease","volume":"55","author":"Steinberg","year":"1983","journal-title":"Br J Haematol"},{"key":"10.1016\/j.blre.2022.100983_bb0365","doi-asserted-by":"crossref","first-page":"15","DOI":"10.1002\/(SICI)1096-8652(199809)59:1<15::AID-AJH4>3.0.CO;2-2","article-title":"Genetic and hematological studies in a group of 114 adult patients with SC sickle cell disease","volume":"59","author":"Lee","year":"1998","journal-title":"Am J Hematol"},{"key":"10.1016\/j.blre.2022.100983_bb0370","doi-asserted-by":"crossref","first-page":"1669","DOI":"10.1016\/j.ebiom.2015.09.026","article-title":"Cation homeostasis in red cells from patients with sickle cell disease heterologous for HbS and HbC (HbSC Genotype)","volume":"2","author":"Hannemann","year":"2015","journal-title":"EBioMedicine"},{"key":"10.1016\/j.blre.2022.100983_bb0375","doi-asserted-by":"crossref","first-page":"2401","DOI":"10.1182\/blood.V101.6.2401","article-title":"Stroke risk in siblings with sickle cell anemia","volume":"101","author":"Driscoll","year":"2003","journal-title":"Blood."},{"key":"10.1016\/j.blre.2022.100983_bb0380","article-title":"Heritability and predictability of sickle cell anemia subphenotypes","volume":"87","author":"Sebastiani","year":"2012","journal-title":"Am J Hematol"},{"key":"10.1016\/j.blre.2022.100983_bb0385","doi-asserted-by":"crossref","first-page":"932","DOI":"10.1046\/j.1365-2141.2003.04276.x","article-title":"Transcranial Doppler ultrasonography in siblings with sickle cell disease","volume":"121","author":"Kwiatkowski","year":"2003","journal-title":"Br J Haematol"},{"key":"10.1016\/j.blre.2022.100983_bb0390","doi-asserted-by":"crossref","first-page":"649","DOI":"10.1038\/s41397-021-00242-8","article-title":"G6PD distribution in sub-Saharan Africa and potential risks of using chloroquine\/hydroxychloroquine based treatments for COVID-19","volume":"21","author":"da Rocha","year":"2021","journal-title":"Pharmacogenomics J"},{"key":"10.1016\/j.blre.2022.100983_bb0395","doi-asserted-by":"crossref","first-page":"1771","DOI":"10.1096\/fj.00-0893fje","article-title":"Glucose-6-phosphate dehydrogenase deficiency promotes endothelial oxidant stress and decreases endothelial nitric oxide bioavailability","volume":"15","author":"Leopold","year":"2001","journal-title":"FASEB J"},{"key":"10.1016\/j.blre.2022.100983_bb0400","doi-asserted-by":"crossref","first-page":"742","DOI":"10.1182\/blood-2009-04-216861","article-title":"Glucose 6 phosphate dehydrogenase deficiency is not associated with cerebrovascular disease in children with sickle cell anemia","volume":"114","author":"Rees","year":"2009","journal-title":"Blood"},{"key":"10.1016\/j.blre.2022.100983_bb0405","doi-asserted-by":"crossref","first-page":"1046","DOI":"10.1002\/pbc.25924","article-title":"Glucose-6-phosphate dehydrogenase deficiency in brazilian children with sickle cell anemia is not associated with clinical ischemic stroke or high-risk transcranial doppler","volume":"63","author":"Belis\u00e1rio","year":"2016","journal-title":"Pediatr Blood Cancer"},{"key":"10.1016\/j.blre.2022.100983_bb0410","doi-asserted-by":"crossref","first-page":"331","DOI":"10.1002\/ajh.21958","article-title":"G6PD deficiency and stroke in the CSSCD","volume":"86","author":"Miller","year":"2011","journal-title":"Am J Hematol"},{"key":"10.1016\/j.blre.2022.100983_bb0415","doi-asserted-by":"crossref","first-page":"646","DOI":"10.1111\/bjh.12590","article-title":"Impact of glucose-6-phosphate dehydrogenase deficiency on sickle cell anaemia expression in infancy and early childhood: a prospective study","volume":"163","author":"Benkerrou","year":"2013","journal-title":"Br J Haematol"},{"key":"10.1016\/j.blre.2022.100983_bb0420","doi-asserted-by":"crossref","first-page":"404","DOI":"10.1111\/ejh.12607","article-title":"G6PD deficiency and absence of \u03b1-thalassemia increase the risk for cerebral vasculopathy in children with sickle cell anemia","volume":"96","author":"Joly","year":"2016","journal-title":"Eur J Haematol"},{"key":"10.1016\/j.blre.2022.100983_bb0425","doi-asserted-by":"crossref","first-page":"530","DOI":"10.1002\/ajh.23683","article-title":"Factors associated with survival in a contemporary adult sickle cell disease cohort","volume":"89","author":"Elmariah","year":"2014","journal-title":"Am J Hematol"},{"key":"10.1016\/j.blre.2022.100983_bb0430","doi-asserted-by":"crossref","first-page":"1285","DOI":"10.1007\/s00467-011-1857-2","article-title":"Glomerular hyperfiltration and albuminuria in children with sickle cell anemia","volume":"26","author":"Aygun","year":"2011","journal-title":"Pediatr Nephrol"},{"key":"10.1016\/j.blre.2022.100983_bb0435","first-page":"749","article-title":"Prevalence and clinical correlates of glomerulopathy in children with sickle cell disease","volume":"136","author":"Wigfall","year":"2000","journal-title":"J Pediatr"},{"key":"10.1016\/j.blre.2022.100983_bb0440","doi-asserted-by":"crossref","DOI":"10.1097\/HS9.0000000000000002","article-title":"Early markers of sickle nephropathy in children with sickle cell anemia are associated with red cell cation transport activity","volume":"1","author":"Brewin","year":"2017","journal-title":"HemaSphere"},{"key":"10.1016\/j.blre.2022.100983_bb0445","doi-asserted-by":"crossref","first-page":"345","DOI":"10.1007\/s00439-010-0861-0","article-title":"Missense mutations in the APOL1 gene are highly associated with end stage kidney disease risk previously attributed to the MYH9 gene","volume":"128","author":"Tzur","year":"2010","journal-title":"Hum Genet"},{"key":"10.1016\/j.blre.2022.100983_bb0450","doi-asserted-by":"crossref","first-page":"841","DOI":"10.1126\/science.1193032","article-title":"Association of trypanolytic ApoL1 variants with kidney disease in African Americans","volume":"329","author":"Genovese","year":"2010","journal-title":"Science (New York, NY)"},{"key":"10.1016\/j.blre.2022.100983_bb0455","doi-asserted-by":"crossref","first-page":"386","DOI":"10.1111\/j.1365-2141.2011.08832.x","article-title":"MYH9 and APOL1 are both associated with sickle cell disease nephropathy","volume":"155","author":"Ashley-Koch","year":"2011","journal-title":"Br J Haematol"},{"key":"10.1016\/j.blre.2022.100983_bb0460","doi-asserted-by":"crossref","first-page":"323","DOI":"10.1111\/bjh.14842","article-title":"Roles of APOL1 G1 and G2 variants in sickle cell disease patients: kidney is the main target","volume":"179","author":"Kormann","year":"2017","journal-title":"Br J Haematol"},{"key":"10.1016\/j.blre.2022.100983_bb0465","doi-asserted-by":"crossref","first-page":"e1","DOI":"10.3324\/haematol.2016.154153","article-title":"APOL1, \u03b1-thalassemia, and BCL11A variants as a genetic risk profile for progression of chronic kidney disease in sickle cell anemia","volume":"102","author":"Saraf","year":"2017","journal-title":"Haematologica"},{"key":"10.1016\/j.blre.2022.100983_bb0470","doi-asserted-by":"crossref","first-page":"1275","DOI":"10.3324\/haematol.2015.124875","article-title":"Genetic variants and cell-free hemoglobin processing in sickle cell nephropathy","volume":"100","author":"Santosh","year":"2015","journal-title":"Haematologica"},{"key":"10.1016\/j.blre.2022.100983_bb0475","doi-asserted-by":"crossref","first-page":"2183","DOI":"10.1056\/NEJMoa1310345","article-title":"APOL1 risk variants, race, and progression of chronic kidney disease","volume":"369","author":"Parsa","year":"2013","journal-title":"N Engl J Med"},{"key":"10.1016\/j.blre.2022.100983_bb0480","doi-asserted-by":"crossref","first-page":"1422","DOI":"10.1681\/ASN.2010070730","article-title":"The apolipoprotein L1 (APOL1) gene and nondiabetic nephropathy in African Americans","volume":"21","author":"Freedman","year":"2010","journal-title":"J Am Soc Nephrol"},{"key":"10.1016\/j.blre.2022.100983_bb0485","doi-asserted-by":"crossref","first-page":"2129","DOI":"10.1681\/ASN.2011040388","article-title":"APOL1 genetic variants in focal segmental glomerulosclerosis and HIV-associated nephropathy","volume":"22","author":"Kopp","year":"2011","journal-title":"J Am Soc Nephrol"},{"key":"10.1016\/j.blre.2022.100983_bb0490","doi-asserted-by":"crossref","first-page":"448","DOI":"10.1097\/00043426-200110000-00011","article-title":"Influence of bilirubin uridine diphosphate-glucuronosyltransferase 1A promoter polymorphisms on serum bilirubin levels and cholelithiasis in children with sickle cell anemia","volume":"23","author":"Passon","year":"2001","journal-title":"J Pediatr Hematol Oncol"},{"key":"10.1016\/j.blre.2022.100983_bb0495","doi-asserted-by":"crossref","first-page":"160","DOI":"10.1034\/j.1399-0004.2003.00113.x","article-title":"UDP-glucuronosyltransferase 1 gene promoter polymorphism is associated with increased serum bilirubin levels and cholecystectomy in patients with sickle cell anemia","volume":"64","author":"Fertrin","year":"2003","journal-title":"Clin Genet"},{"key":"10.1016\/j.blre.2022.100983_bb0500","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pone.0034741","article-title":"A genome-wide association study of total bilirubin and cholelithiasis risk in sickle cell anemia","volume":"7","author":"Milton","year":"2012","journal-title":"PLoS One"},{"key":"10.1016\/j.blre.2022.100983_bb0505","doi-asserted-by":"crossref","first-page":"778","DOI":"10.1001\/archinte.1924.00120060041003","article-title":"A case of sickle cell anemia with necropsy","volume":"34","author":"Graham","year":"1924","journal-title":"Arch Intern Med"},{"key":"10.1016\/j.blre.2022.100983_bb0510","doi-asserted-by":"crossref","first-page":"165","DOI":"10.1111\/bjh.12950","article-title":"The spleen and sickle cell disease: the sick(led) spleen","volume":"166","author":"Brousse","year":"2014","journal-title":"Br J Haematol"},{"key":"10.1016\/j.blre.2022.100983_bb0515","doi-asserted-by":"crossref","first-page":"1364","DOI":"10.1016\/S0140-6736(09)61374-X","article-title":"Bacteraemia in Kenyan children with sickle-cell anaemia: a retrospective cohort and case-control study","volume":"374","author":"Williams","year":"2009","journal-title":"Lancet"},{"key":"10.1016\/j.blre.2022.100983_bb0520","doi-asserted-by":"crossref","first-page":"215","DOI":"10.1182\/blood-2009-07-233528","article-title":"Malaria in patients with sickle cell anemia: burden, risk factors, and outcome at the outpatient clinic and during hospitalization","volume":"115","author":"Makani","year":"2010","journal-title":"Blood"},{"key":"10.1016\/j.blre.2022.100983_bb0525","doi-asserted-by":"crossref","first-page":"1663","DOI":"10.1182\/blood-2010-01-265249","article-title":"High mortality from Plasmodium falciparum malaria in children living with sickle cell anemia on the coast of Kenya","volume":"116","author":"McAuley","year":"2010","journal-title":"Blood"},{"key":"10.1016\/j.blre.2022.100983_bb0530","doi-asserted-by":"crossref","first-page":"2329","DOI":"10.1182\/blood-2009-12-260836","article-title":"Pandemic influenza A (H1N1) virus infections in children with sickle cell disease","volume":"115","author":"Inusa","year":"2010","journal-title":"Blood"},{"key":"10.1016\/j.blre.2022.100983_bb0535","article-title":"Clinical outcomes of COVID-19 in patients with sickle cell disease and sickle cell trait: A critical appraisal of the literature","volume":"100911","author":"Hoogenboom","year":"2021","journal-title":"Blood Rev"},{"key":"10.1016\/j.blre.2022.100983_bb0540","doi-asserted-by":"crossref","first-page":"2651","DOI":"10.3324\/haematol.2020.259440","article-title":"Real-time national survey of COVID-19 in hemoglobinopathy and rare inherited anemia patients","volume":"105","author":"Telfer","year":"2020","journal-title":"Haematologica."},{"key":"10.1016\/j.blre.2022.100983_bb0545","doi-asserted-by":"crossref","first-page":"2473","DOI":"10.3201\/eid2610.202792","article-title":"Coronavirus disease among persons with sickle cell disease, United States, March 20-May 21, 2020","volume":"26","author":"Panepinto","year":"2020","journal-title":"Emerg Infect Dis"},{"key":"10.1016\/j.blre.2022.100983_bb0550","doi-asserted-by":"crossref","first-page":"186","DOI":"10.1016\/j.jiph.2019.07.015","article-title":"Dengue in hospitalized children with sickle cell disease: a retrospective cohort study in the French departments of America","volume":"13","author":"Elenga","year":"2020","journal-title":"J Infect Public Health"},{"key":"10.1016\/j.blre.2022.100983_bb0555","series-title":"Sickle cell disease","author":"Serjeant","year":"2001"},{"key":"10.1016\/j.blre.2022.100983_bb0560","doi-asserted-by":"crossref","first-page":"111","DOI":"10.1042\/cs0940111","article-title":"Peripheral vascular response to mild indirect cooling in patients with homozygous sickle cell (SS) disease and the frequency of painful crisis","volume":"94","author":"Mohan","year":"1998","journal-title":"Clin Sci"},{"key":"10.1016\/j.blre.2022.100983_bb0565","doi-asserted-by":"crossref","first-page":"37","DOI":"10.1002\/ajh.23341","article-title":"Patients with sickle cell disease have increased sensitivity to cold and heat","volume":"88","author":"Brandow","year":"2013","journal-title":"Am J Hematol"},{"key":"10.1016\/j.blre.2022.100983_bb0570","doi-asserted-by":"crossref","first-page":"1191","DOI":"10.1182\/blood.2020005045","article-title":"Progressive vasoconstriction with sequential thermal stimulation indicates vascular dysautonomia in sickle cell disease","volume":"136","author":"Veluswamy","year":"2020","journal-title":"Blood"},{"key":"10.1016\/j.blre.2022.100983_bb0575","doi-asserted-by":"crossref","first-page":"666","DOI":"10.3324\/haematol.2016.154245","article-title":"Associations between environmental factors and hospital admissions for sickle cell disease","volume":"102","author":"Piel","year":"2017","journal-title":"Haematologica"},{"key":"10.1016\/j.blre.2022.100983_bb0580","doi-asserted-by":"crossref","DOI":"10.1097\/MD.0000000000000280","article-title":"Environmental influences on daily emergency admissions in sickle-cell disease patients","volume":"93","author":"Mekontso Dessap","year":"2014","journal-title":"Medicine (Baltimore)"},{"key":"10.1016\/j.blre.2022.100983_bb0585","doi-asserted-by":"crossref","first-page":"530","DOI":"10.1111\/j.1365-2141.2005.05799.x","article-title":"Windy weather and low humidity are associated with an increased number of hospital admissions for acute pain and sickle cell disease in an urban environment with a maritime temperate climate","volume":"131","author":"Jones","year":"2005","journal-title":"Br J Haematol"},{"key":"10.1016\/j.blre.2022.100983_bb0590","doi-asserted-by":"crossref","first-page":"433","DOI":"10.1111\/j.1365-2141.2008.07354.x","article-title":"Association between wind speed and the occurrence of sickle cell acute painful episodes: results of a case-crossover study","volume":"143","author":"Nolan","year":"2008","journal-title":"Br J Haematol"},{"key":"10.1016\/j.blre.2022.100983_bb0595","doi-asserted-by":"crossref","first-page":"559","DOI":"10.1016\/j.jemermed.2010.05.006","article-title":"Pediatric vasoocclusive crisis and weather conditions","volume":"41","author":"Rogovik","year":"2011","journal-title":"J Emerg Med"},{"key":"10.1016\/j.blre.2022.100983_bb0600","doi-asserted-by":"crossref","first-page":"159","DOI":"10.1016\/0035-9203(80)90236-9","article-title":"Relationship between meteorological changes and occurrence of painful sickle cell crises in Kuwait","volume":"74","author":"Ibrahim","year":"1980","journal-title":"Trans R Soc Trop Med Hyg"},{"key":"10.1016\/j.blre.2022.100983_bb0605","doi-asserted-by":"crossref","first-page":"1003","DOI":"10.1016\/S0140-6736(65)91224-9","article-title":"Sicklaemic human hygrometers","volume":"1","author":"Konotey-Ahulu","year":"1965","journal-title":"Lancet"},{"key":"10.1016\/j.blre.2022.100983_bb0610","doi-asserted-by":"crossref","first-page":"66","DOI":"10.1136\/bmj.1.6001.66","article-title":"Climate and painful crisis of sickle-cell disease in Jamaica","volume":"1","author":"Redwood","year":"1976","journal-title":"Br Med J"},{"key":"10.1016\/j.blre.2022.100983_bb0615","doi-asserted-by":"crossref","first-page":"449","DOI":"10.1067\/mpd.2003.102","article-title":"Thrombosis and gangrene in a patient with sickle cell disease and dactylitis","volume":"142","author":"Michaels","year":"2003","journal-title":"J Pediatr"},{"key":"10.1016\/j.blre.2022.100983_bb0620","doi-asserted-by":"crossref","first-page":"53","DOI":"10.1002\/ajh.20761","article-title":"Limb amputation in hemoglobin SC disease after application of ice and elevation","volume":"82","author":"Saleem","year":"2007","journal-title":"Am J Hematol"},{"key":"10.1016\/j.blre.2022.100983_bb0625","doi-asserted-by":"crossref","first-page":"349","DOI":"10.1097\/MOP.0000000000000756","article-title":"Asthma in children with sickle cell disease","volume":"31","author":"Willen","year":"2019","journal-title":"Curr Opin Pediatr"},{"key":"10.1016\/j.blre.2022.100983_bb0630","doi-asserted-by":"crossref","first-page":"750","DOI":"10.1172\/JCI89741","article-title":"Intravascular hemolysis and the pathophysiology of sickle cell disease","volume":"127","author":"Kato","year":"2017","journal-title":"J Clin Invest"},{"key":"10.1016\/j.blre.2022.100983_bb0635","doi-asserted-by":"crossref","first-page":"893","DOI":"10.1001\/jama.2011.235","article-title":"Nitric oxide for inhalation in the acute treatment of sickle cell pain crisis: a randomized controlled trial","volume":"305","author":"Gladwin","year":"2011","journal-title":"JAMA"},{"key":"10.1016\/j.blre.2022.100983_bb0640","doi-asserted-by":"crossref","first-page":"844","DOI":"10.1111\/j.1365-2141.2007.06493.x","article-title":"The associations between air quality and the number of hospital admissions for acute pain and sickle-cell disease in an urban environment","volume":"136","author":"Yallop","year":"2007","journal-title":"Br J Haematol"},{"key":"10.1016\/j.blre.2022.100983_bb0645","doi-asserted-by":"crossref","first-page":"529","DOI":"10.1007\/s00277-008-0598-1","article-title":"The effects of air quality on haematological and clinical parameters in children with sickle cell anaemia","volume":"88","author":"Mittal","year":"2009","journal-title":"Ann Hematol"},{"key":"10.1016\/j.blre.2022.100983_bb0650","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pone.0205194","article-title":"Oral carbon monoxide therapy in murine sickle cell disease: beneficial effects on vaso-occlusion, inflammation and anemia","volume":"13","author":"Belcher","year":"2018","journal-title":"PLoS One"},{"key":"10.1016\/j.blre.2022.100983_bb0655","doi-asserted-by":"crossref","first-page":"265","DOI":"10.1590\/0102-311X00013214","article-title":"Air pollution and children\u2019s health: sickle cell disease","volume":"31","author":"Barbosa","year":"2015","journal-title":"Cad Saude Publica"},{"key":"10.1016\/j.blre.2022.100983_bb0660","doi-asserted-by":"crossref","DOI":"10.1016\/j.envres.2020.109292","article-title":"Ambient air pollution and sickle cell disease-related emergency department visits in Atlanta, GA","volume":"184","author":"Blumberg","year":"2020","journal-title":"Environ Res"},{"key":"10.1016\/j.blre.2022.100983_bb0665","doi-asserted-by":"crossref","first-page":"897","DOI":"10.1001\/jama.285.7.897","article-title":"Impact of changes in transportation and commuting behaviors during the 1996 Summer Olympic Games in Atlanta on air quality and childhood asthma","volume":"285","author":"Friedman","year":"2001","journal-title":"JAMA"},{"key":"10.1016\/j.blre.2022.100983_bb0670","doi-asserted-by":"crossref","first-page":"585","DOI":"10.1002\/jha2.248","article-title":"Splenic infarction in sickle cell trait: a comprehensive systematic review of case studies","volume":"2","author":"Jefferson","year":"2021","journal-title":"EJHaem"},{"key":"10.1016\/j.blre.2022.100983_bb0675","doi-asserted-by":"crossref","first-page":"203","DOI":"10.1016\/S0022-3476(77)80630-6","article-title":"Splenic sequestration syndrome at mountain altitudes in sickle\/hemoglobin C disease","volume":"90","author":"Githens","year":"1977","journal-title":"J Pediatr"},{"key":"10.1016\/j.blre.2022.100983_bb0680","doi-asserted-by":"crossref","first-page":"431","DOI":"10.1177\/000992287901800711","article-title":"Sickling crises and altitude. Occurrence in the Colorado patient population","volume":"18","author":"Mahony","year":"1979","journal-title":"Clin Pediatr"},{"key":"10.1016\/j.blre.2022.100983_bb0685","first-page":"342","article-title":"Sickle cell disease in permanent residents of mountain and low altitudes in Saudi Arabia","volume":"42","author":"Addae","year":"1990","journal-title":"Trop Geogr Med"},{"key":"10.1016\/j.blre.2022.100983_bb0690","first-page":"364","article-title":"Risk of altitude exposure in sickle cell disease","volume":"135","author":"Claster","year":"1981","journal-title":"West J Med"},{"key":"10.1016\/j.blre.2022.100983_bb0695","doi-asserted-by":"crossref","first-page":"407","DOI":"10.1016\/S0022-3476(05)82156-0","article-title":"Cold water exposure and vaso-occlusive crises in sickle cell anemia","volume":"118","author":"Resar","year":"1991","journal-title":"J Pediatr"},{"key":"10.1016\/j.blre.2022.100983_bb0700","doi-asserted-by":"crossref","first-page":"971","DOI":"10.7326\/0003-4819-105-6-971_1","article-title":"Water sports and sickle cell anemia","volume":"105","author":"Ratner","year":"1986","journal-title":"Ann Intern Med"},{"key":"10.1016\/j.blre.2022.100983_bb0705","doi-asserted-by":"crossref","DOI":"10.1016\/S2352-3026(18)30163-7","article-title":"Moderate-intensity endurance-exercise training in patients with sickle-cell disease without severe chronic complications (EXDRE): an open-label randomised controlled trial","volume":"5","author":"Gellen","year":"2018","journal-title":"Lancet Haematol"},{"key":"10.1016\/j.blre.2022.100983_bb0710","doi-asserted-by":"crossref","first-page":"1011","DOI":"10.1016\/S0140-6736(12)61228-8","article-title":"WHO European review of social determinants of health and the health divide","volume":"380","author":"Marmot","year":"2012","journal-title":"Lancet"},{"key":"10.1016\/j.blre.2022.100983_bb0715","doi-asserted-by":"crossref","first-page":"189","DOI":"10.1080\/16078454.2018.1549801","article-title":"Risk factors for hospitalizations and readmissions among individuals with sickle cell disease: results of a U.S. survey study","volume":"24","author":"Cronin","year":"2019","journal-title":"Hematology"},{"issue":"5","key":"10.1016\/j.blre.2022.100983_bb0720","first-page":"1","article-title":"Food insecurity, housing instability, and dietary quality among children with sickle cell disease: assessment from a single urban center","volume":"69","author":"Fernandez","year":"2021","journal-title":"Pediatr Blood Cancer"},{"key":"10.1016\/j.blre.2022.100983_bb0725","doi-asserted-by":"crossref","DOI":"10.3390\/ijerph13111131","article-title":"Secondhand smoke is an important modifiable risk factor in sickle cell disease: a review of the current literature and areas for future research","volume":"13","author":"Sadreameli","year":"2016","journal-title":"Int J Environ Res Public Health"},{"key":"10.1016\/j.blre.2022.100983_bb0730","doi-asserted-by":"crossref","first-page":"488","DOI":"10.3324\/haematol.2011.041988","article-title":"The rationale for using hydroxycarbamide in the treatment of sickle cell disease","volume":"96","author":"Rees","year":"2011","journal-title":"Haematologica"},{"key":"10.1016\/j.blre.2022.100983_bb0735","doi-asserted-by":"crossref","first-page":"1663","DOI":"10.1016\/S0140-6736(11)60355-3","article-title":"Hydroxycarbamide in very young children with sickle-cell anaemia: a multicentre, randomised, controlled trial (BABY HUG)","volume":"377","author":"Wang","year":"2011","journal-title":"Lancet"},{"key":"10.1016\/j.blre.2022.100983_bb0740","doi-asserted-by":"crossref","first-page":"2524","DOI":"10.1056\/NEJMoa2000146","article-title":"Hydroxyurea dose escalation for sickle cell anemia in Sub-Saharan Africa","volume":"382","author":"John","year":"2020","journal-title":"N Engl J Med"},{"key":"10.1016\/j.blre.2022.100983_bb0745","doi-asserted-by":"crossref","first-page":"3982","DOI":"10.1182\/bloodadvances.2019000882","article-title":"End points for sickle cell disease clinical trials: patient-reported outcomes, pain, and the brain","volume":"3","author":"Farrell","year":"2019","journal-title":"Blood Adv"},{"key":"10.1016\/j.blre.2022.100983_bb0750","doi-asserted-by":"crossref","first-page":"e440","DOI":"10.3324\/haematol.2019.237602","article-title":"Mortality in children with sickle cell disease in mainland France from 2000 to 2015","volume":"105","author":"Desselas","year":"2020","journal-title":"Haematologica"},{"key":"10.1016\/j.blre.2022.100983_bb0755","doi-asserted-by":"crossref","first-page":"3447","DOI":"10.1182\/blood-2009-07-233700","article-title":"Improved survival of children and adolescents with sickle cell disease","volume":"115","author":"Quinn","year":"2010","journal-title":"Blood"},{"key":"10.1016\/j.blre.2022.100983_bb0760","doi-asserted-by":"crossref","first-page":"886","DOI":"10.1056\/NEJMoa035477","article-title":"Pulmonary hypertension as a risk factor for death in patients with sickle cell disease","volume":"350","author":"Gladwin","year":"2004","journal-title":"N Engl J Med"},{"key":"10.1016\/j.blre.2022.100983_bb0765","doi-asserted-by":"crossref","first-page":"263","DOI":"10.1146\/annurev-pathmechdis-012418-012838","article-title":"Pathophysiology of sickle cell disease","volume":"14","author":"Sundd","year":"2019","journal-title":"Annu Rev Pathol"},{"key":"10.1016\/j.blre.2022.100983_bb0770","doi-asserted-by":"crossref","first-page":"796","DOI":"10.1001\/archpedi.1994.02170080026005","article-title":"Reference values and hematologic changes from birth to 5 years in patients with sickle cell disease. Cooperative Study of Sickle Cell Disease","volume":"148","author":"Brown","year":"1994","journal-title":"Arch Pediatr Adolesc Med"},{"key":"10.1016\/j.blre.2022.100983_bb0775","doi-asserted-by":"crossref","first-page":"433","DOI":"10.1111\/j.1365-2141.2011.08961.x","article-title":"Biomarkers in sickle cell disease","volume":"156","author":"Rees","year":"2012","journal-title":"Br J Haematol"},{"key":"10.1016\/j.blre.2022.100983_bb0780","doi-asserted-by":"crossref","first-page":"2727","DOI":"10.1182\/blood-2007-04-084921","article-title":"A network model to predict the risk of death in sickle cell disease","volume":"110","author":"Sebastiani","year":"2007","journal-title":"Blood"},{"key":"10.1016\/j.blre.2022.100983_bb0785","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1016\/j.bcmd.2018.05.001","article-title":"Biomarker signatures of sickle cell disease severity","volume":"72","author":"Du","year":"2018","journal-title":"Blood Cells Mol Dis"},{"key":"10.1016\/j.blre.2022.100983_bb0790","doi-asserted-by":"crossref","first-page":"772","DOI":"10.1177\/1535370216640150","article-title":"Minireview: Multiomic candidate biomarkers for clinical manifestations of sickle cell severity: Early steps to precision medicine","volume":"241","author":"Goodman","year":"2016","journal-title":"Exp Biol Med (Maywood)"},{"key":"10.1016\/j.blre.2022.100983_bb0795","doi-asserted-by":"crossref","first-page":"37","DOI":"10.1016\/S0022-3476(81)80529-X","article-title":"Fetal hemoglobin and clinical severity of homozygous sickle cell disease in early childhood","volume":"98","author":"Stevens","year":"1981","journal-title":"J Pediatr"},{"key":"10.1016\/j.blre.2022.100983_bb0800","doi-asserted-by":"crossref","first-page":"83","DOI":"10.1056\/NEJM200001133420203","article-title":"Prediction of adverse outcomes in children with sickle cell disease","volume":"342","author":"Miller","year":"2000","journal-title":"N Engl J Med"},{"key":"10.1016\/j.blre.2022.100983_bb0805","doi-asserted-by":"crossref","first-page":"544","DOI":"10.1182\/blood-2007-07-100719","article-title":"Prediction of adverse outcomes in children with sickle cell anemia: a study of the Dallas Newborn Cohort","volume":"111","author":"Quinn","year":"2008","journal-title":"Blood"},{"key":"10.1016\/j.blre.2022.100983_bb0810","doi-asserted-by":"crossref","first-page":"525","DOI":"10.1002\/ajmg.1320270305","article-title":"The relationship between fetal hemoglobin and disease severity in children with sickle cell anemia","volume":"27","author":"Odenheimer","year":"1987","journal-title":"Am J Med Genet"},{"key":"10.1016\/j.blre.2022.100983_bb0815","doi-asserted-by":"crossref","first-page":"E125","DOI":"10.1002\/pbc.24588","article-title":"Association between baseline fetal hemoglobin levels and incidence of severe vaso-occlusive pain episodes in children with sickle cell anemia","volume":"60","author":"Bhatnagar","year":"2013","journal-title":"Pediatr Blood Cancer"},{"key":"10.1016\/j.blre.2022.100983_bb0820","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pone.0070794","article-title":"Increased reticulocytosis during infancy is associated with increased hospitalizations in sickle cell anemia patients during the first three years of life","volume":"8","author":"Meier","year":"2013","journal-title":"PLoS One"},{"key":"10.1016\/j.blre.2022.100983_bb0825","doi-asserted-by":"crossref","first-page":"675","DOI":"10.1002\/pbc.24037","article-title":"Influence of severity of anemia on clinical findings in infants with sickle cell anemia: analyses from the BABY HUG study","volume":"59","author":"Lebensburger","year":"2012","journal-title":"Pediatr Blood Cancer"},{"key":"10.1016\/j.blre.2022.100983_bb0830","doi-asserted-by":"crossref","first-page":"1411","DOI":"10.1002\/ajh.25260","article-title":"Prognostic factors of disease severity in infants with sickle cell anemia: a comprehensive longitudinal cohort study","volume":"93","author":"Brousse","year":"2018","journal-title":"Am J Hematol"},{"key":"10.1016\/j.blre.2022.100983_bb0835","doi-asserted-by":"crossref","first-page":"643","DOI":"10.1002\/pbc.21612","article-title":"MRI abnormalities of the brain in one-year-old children with sickle cell anemia","volume":"51","author":"Wang","year":"2008","journal-title":"Pediatr Blood Cancer"},{"key":"10.1016\/j.blre.2022.100983_bb0840","doi-asserted-by":"crossref","first-page":"605","DOI":"10.1056\/NEJM199202273260905","article-title":"The use of transcranial ultrasonography to predict stroke in sickle cell disease","volume":"326","author":"Adams","year":"1992","journal-title":"N Engl J Med"},{"key":"10.1016\/j.blre.2022.100983_bb0845","doi-asserted-by":"crossref","first-page":"360","DOI":"10.1016\/S0022-3476(05)80897-2","article-title":"Stroke in a cohort of patients with homozygous sickle cell disease","volume":"120","author":"Balkaran","year":"1992","journal-title":"J Pediatr"},{"key":"10.1016\/j.blre.2022.100983_bb0850","doi-asserted-by":"crossref","first-page":"385","DOI":"10.1067\/mpd.2001.117580","article-title":"Silent infarction as a risk factor for overt stroke in children with sickle cell anemia: a report from the Cooperative Study of Sickle Cell Disease","volume":"139","author":"Miller","year":"2001","journal-title":"J Pediatr"},{"key":"10.1016\/j.blre.2022.100983_bb0855","doi-asserted-by":"crossref","first-page":"904","DOI":"10.1002\/ajh.23777","article-title":"Reticulocytosis and anemia are associated with an increased risk of death and stroke in the newborn cohort of the Cooperative Study of Sickle Cell Disease","volume":"89","author":"Meier","year":"2014","journal-title":"Am J Hematol"},{"key":"10.1016\/j.blre.2022.100983_bb0860","doi-asserted-by":"crossref","first-page":"116","DOI":"10.1002\/pbc.22680","article-title":"High reticulocyte count is an independent risk factor for cerebrovascular disease in children with sickle cell anemia","volume":"56","author":"Silva","year":"2011","journal-title":"Pediatr Blood Cancer"},{"key":"10.1016\/j.blre.2022.100983_bb0865","doi-asserted-by":"crossref","first-page":"829","DOI":"10.1182\/blood-2015-09-618579","article-title":"Central nervous system complications and management in sickle cell disease","volume":"127","author":"DeBaun","year":"2016","journal-title":"Blood"},{"key":"10.1016\/j.blre.2022.100983_bb0870","doi-asserted-by":"crossref","first-page":"640","DOI":"10.1542\/peds.103.3.640","article-title":"Silent cerebral infarcts in sickle cell anemia: a risk factor analysis. The Cooperative Study of Sickle Cell Disease","volume":"103","author":"Kinney","year":"1999","journal-title":"Pediatrics"},{"key":"10.1016\/j.blre.2022.100983_bb0875","doi-asserted-by":"crossref","first-page":"699","DOI":"10.1056\/NEJMoa1401731","article-title":"Controlled trial of transfusions for silent cerebral infarcts in sickle cell anemia","volume":"371","author":"DeBaun","year":"2014","journal-title":"N Engl J Med"},{"key":"10.1016\/j.blre.2022.100983_bb0880","doi-asserted-by":"crossref","first-page":"3684","DOI":"10.1182\/blood-2011-05-349621","article-title":"Associated risk factors for silent cerebral infarcts in sickle cell anemia: low baseline hemoglobin, sex, and relative high systolic blood pressure","volume":"119","author":"Debaun","year":"2012","journal-title":"Blood"},{"key":"10.1016\/j.blre.2022.100983_bb0885","doi-asserted-by":"crossref","first-page":"1653","DOI":"10.1182\/blood-2014-09-599852","article-title":"Chronic and acute anemia and extracranial internal carotid stenosis are risk factors for silent cerebral infarcts in sickle cell anemia","volume":"125","author":"Bernaudin","year":"2015","journal-title":"Blood"},{"key":"10.1016\/j.blre.2022.100983_bb0890","doi-asserted-by":"crossref","first-page":"1136","DOI":"10.3324\/haematol.2018.187815","article-title":"Proteomic analysis of plasma from children with sickle cell anemia and silent cerebral infarction","volume":"103","author":"Tewari","year":"2018","journal-title":"Haematologica"},{"key":"10.1016\/j.blre.2022.100983_bb0895","doi-asserted-by":"crossref","DOI":"10.1002\/ajh.26323","article-title":"The International Hemoglobinopathy Research Network (INHERENT): an international initiative to study the role of genetic modifiers in hemoglobinopathies","volume":"96","author":"Kountouris","year":"2021","journal-title":"Am J Hematol"},{"key":"10.1016\/j.blre.2022.100983_bb0900","doi-asserted-by":"crossref","DOI":"10.1136\/bmjopen-2020-048208","article-title":"Sickle Cell Disease Genomics of Africa (SickleGenAfrica) Network: ethical framework and initial qualitative findings from community engagement in Ghana, Nigeria and Tanzania","volume":"11","author":"Anie","year":"2021","journal-title":"BMJ Open"},{"key":"10.1016\/j.blre.2022.100983_bb0905","doi-asserted-by":"crossref","first-page":"559","DOI":"10.1089\/omi.2020.0153","article-title":"The sickle cell disease ontology: enabling collaborative research and co-designing of new planetary health applications","volume":"24","author":"Nembaware","year":"2020","journal-title":"OMICS"},{"key":"10.1016\/j.blre.2022.100983_bb0910","doi-asserted-by":"crossref","first-page":"435","DOI":"10.1038\/ng1533","article-title":"Genetic dissection and prognostic modeling of overt stroke in sickle cell anemia","volume":"37","author":"Sebastiani","year":"2005","journal-title":"Nat Genet"},{"key":"10.1016\/j.blre.2022.100983_bb0915","doi-asserted-by":"crossref","first-page":"6681","DOI":"10.1182\/blood-2011-01-332205","article-title":"Genetic predictors for stroke in children with sickle cell anemia","volume":"117","author":"Flanagan","year":"2011","journal-title":"Blood"},{"key":"10.1016\/j.blre.2022.100983_bb0920","doi-asserted-by":"crossref","first-page":"1869","DOI":"10.1007\/s00277-016-2789-5","article-title":"Reticulocyte count is the most important predictor of acute cerebral ischemia and high-risk transcranial Doppler in a newborn cohort of 395 children with sickle cell anemia","volume":"95","author":"Belis\u00e1rio","year":"2016","journal-title":"Ann Hematol"},{"key":"10.1016\/j.blre.2022.100983_bb0925","doi-asserted-by":"crossref","first-page":"3237","DOI":"10.1182\/blood-2012-10-464156","article-title":"Genetic mapping and exome sequencing identify 2 mutations associated with stroke protection in pediatric patients with sickle cell anemia","volume":"121","author":"Flanagan","year":"2013","journal-title":"Blood"},{"key":"10.1016\/j.blre.2022.100983_bb0930","doi-asserted-by":"crossref","first-page":"1259","DOI":"10.1182\/blood-2015-05-645176","article-title":"Association between ENPP1 K173Q and stroke in a newborn cohort of 395 Brazilian children with sickle cell anemia","volume":"126","author":"Belis\u00e1rio","year":"2015","journal-title":"Blood"},{"key":"10.1016\/j.blre.2022.100983_bb0935","doi-asserted-by":"crossref","first-page":"2391","DOI":"10.1182\/blood-2003-09-3015","article-title":"Gene interactions and stroke risk in children with sickle cell anemia","volume":"103","author":"Hoppe","year":"2004","journal-title":"Blood"},{"key":"10.1016\/j.blre.2022.100983_bb0940","doi-asserted-by":"crossref","first-page":"2241","DOI":"10.1161\/STROKEAHA.107.483115","article-title":"Confirmation of an association between the TNF(-308) promoter polymorphism and stroke risk in children with sickle cell anemia","volume":"38","author":"Hoppe","year":"2007","journal-title":"Stroke"},{"key":"10.1016\/j.blre.2022.100983_bb0945","doi-asserted-by":"crossref","first-page":"531","DOI":"10.1161\/CIRCGEN.120.003025","article-title":"Genetic analysis of patients with sickle cell anemia and stroke before 4 years of age suggest an important role for apoliprotein E","volume":"13","author":"Brewin","year":"2020","journal-title":"Circ Genomic Precis Med"}],"container-title":["Blood Reviews"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0268960X22000571?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0268960X22000571?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2025,10,2]],"date-time":"2025-10-02T20:29:01Z","timestamp":1759436941000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S0268960X22000571"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,11]]},"references-count":189,"alternative-id":["S0268960X22000571"],"URL":"https:\/\/doi.org\/10.1016\/j.blre.2022.100983","relation":{},"ISSN":["0268-960X"],"issn-type":[{"value":"0268-960X","type":"print"}],"subject":[],"published":{"date-parts":[[2022,11]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Determinants of severity in sickle cell disease","name":"articletitle","label":"Article Title"},{"value":"Blood Reviews","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.blre.2022.100983","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2022 The Authors. Published by Elsevier Ltd.","name":"copyright","label":"Copyright"}],"article-number":"100983"}}