{"id":"https://openalex.org/W2173075639","doi":null,"title":"Current therapy of sickle cell disease.","display_name":"Current therapy of sickle cell disease.","publication_year":2006,"publication_date":"2006-01-01","ids":{"openalex":"https://openalex.org/W2173075639","mag":"2173075639","pmid":"https://pubmed.ncbi.nlm.nih.gov/16434364","pmcid":"https://www.ncbi.nlm.nih.gov/pmc/articles/2204144"},"language":"en","primary_location":{"id":"pmid:16434364","doi":null,"is_oa":false,"landing_page_url":"https://pubmed.ncbi.nlm.nih.gov/16434364","pdf_url":null,"source":{"id":"https://openalex.org/S4306525036","display_name":"PubMed","issn_l":null,"issn":null,"is_oa":false,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":"https://openalex.org/I1299303238","host_organization_name":"National Institutes of Health","host_organization_lineage":["https://openalex.org/I1299303238"],"host_organization_lineage_names":[],"type":"repository"},"license":null,"license_id":null,"version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"Haematologica","raw_type":"Editorial"},"volume":null,"type":"editorial","is_traditional":true,"indexed_in":["doaj","pubmed"],"open_access":{"is_oa":true,"oa_status":"green","oa_url":"https://www.ncbi.nlm.nih.gov/pmc/articles/2204144","any_repository_has_fulltext":true},"authorships":[{"author_position":"first","author":{"id":"https://openalex.org/A5065078544","display_name":"Zakari Y. Aliyu","orcid":null,"observed_orcids":[]},"institutions":[{"id":"https://openalex.org/I1299303238","display_name":"National Institutes of Health","ror":"https://ror.org/01cwqze88","country_code":"US","type":"government","lineage":["https://openalex.org/I1299022934","https://openalex.org/I1299303238"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Zakari Y Aliyu","raw_affiliation_strings":["National Institutes of Health"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"National Institutes of Health","institution_ids":["https://openalex.org/I1299303238"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5065998202","display_name":"Ashaunta R. Tumblin","orcid":null,"observed_orcids":[]},"institutions":[{"id":"https://openalex.org/I1299303238","display_name":"National Institutes of Health","ror":"https://ror.org/01cwqze88","country_code":"US","type":"government","lineage":["https://openalex.org/I1299022934","https://openalex.org/I1299303238"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Ashaunta R Tumblin","raw_affiliation_strings":["National Institutes of Health, Bethesda"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"National Institutes of Health, Bethesda","institution_ids":["https://openalex.org/I1299303238"]}]},{"author_position":"last","author":{"id":"https://openalex.org/A5066832003","display_name":"Gregory James Kato","orcid":"https://orcid.org/0000-0003-4465-3217","observed_orcids":["https://orcid.org/0000-0003-4465-3217"]},"institutions":[],"countries":[],"is_corresponding":false,"raw_author_name":"Gregory J Kato","raw_affiliation_strings":[],"raw_orcid":null,"affiliations":[]}],"institutions":[],"countries_distinct_count":1,"institutions_distinct_count":1,"corresponding_author_ids":[],"corresponding_institution_ids":[],"apc_list":null,"apc_paid":null,"fwci":null,"has_fulltext":false,"cited_by_count":61,"citation_normalized_percentile":null,"cited_by_percentile_year":null,"biblio":{"volume":"91","issue":"1","first_page":"7","last_page":"10"},"is_retracted":false,"is_paratext":false,"is_xpac":false,"primary_topic":{"id":"https://openalex.org/T10554","display_name":"Hemoglobinopathies and Related Disorders","score":1.0,"subfield":{"id":"https://openalex.org/subfields/2716","display_name":"Genetics (clinical)"},"field":{"id":"https://openalex.org/fields/27","display_name":"Medicine"},"domain":{"id":"https://openalex.org/domains/4","display_name":"Health Sciences"}},"topics":[{"id":"https://openalex.org/T10554","display_name":"Hemoglobinopathies and Related Disorders","score":1.0,"subfield":{"id":"https://openalex.org/subfields/2716","display_name":"Genetics (clinical)"},"field":{"id":"https://openalex.org/fields/27","display_name":"Medicine"},"domain":{"id":"https://openalex.org/domains/4","display_name":"Health Sciences"}},{"id":"https://openalex.org/T12094","display_name":"Hemoglobin structure and function","score":0.0,"subfield":{"id":"https://openalex.org/subfields/1303","display_name":"Biochemistry"},"field":{"id":"https://openalex.org/fields/13","display_name":"Biochemistry, Genetics and Molecular Biology"},"domain":{"id":"https://openalex.org/domains/1","display_name":"Life Sciences"}},{"id":"https://openalex.org/T11660","display_name":"Blood groups and transfusion","score":0.0,"subfield":{"id":"https://openalex.org/subfields/2720","display_name":"Hematology"},"field":{"id":"https://openalex.org/fields/27","display_name":"Medicine"},"domain":{"id":"https://openalex.org/domains/4","display_name":"Health Sciences"}}],"keywords":[{"id":"https://openalex.org/keywords/sickle-cell-disease","display_name":"sickle cell disease","score":0.9990000128746033},{"id":"https://openalex.org/keywords/hemoglobin-s","display_name":"hemoglobin S","score":0.8339999914169312},{"id":"https://openalex.org/keywords/vessel-occlusion","display_name":"vascular occlusion","score":0.7860000133514404},{"id":"https://openalex.org/keywords/intravascular-hemolysis","display_name":"intravascular hemolysis","score":0.9290000200271606},{"id":"https://openalex.org/keywords/nitric-oxide","display_name":"nitric oxide","score":0.7149999737739563},{"id":"https://openalex.org/keywords/free-hemoglobin","display_name":"free hemoglobin","score":0.9769999980926514},{"id":"https://openalex.org/keywords/arginase","display_name":"arginase","score":0.652999997138977},{"id":"https://openalex.org/keywords/haptoglobin","display_name":"haptoglobin","score":0.5709999799728394},{"id":"https://openalex.org/keywords/hemopexin","display_name":"hemopexin","score":0.9779999852180481},{"id":"https://openalex.org/keywords/vaso-occlusive-crisis","display_name":"vaso-occlusive crisis","score":0.8180000185966492},{"id":"https://openalex.org/keywords/stroke","display_name":"stroke","score":0.48100000619888306},{"id":"https://openalex.org/keywords/organ-damage","display_name":"organ damage","score":0.6179999709129333}],"concepts":[{"id":"https://openalex.org/C71924100","wikidata":"https://www.wikidata.org/wiki/Q11190","display_name":"Medicine","level":0,"score":0.7350616455078125},{"id":"https://openalex.org/C2779902561","wikidata":"https://www.wikidata.org/wiki/Q471706","display_name":"Hemolysis","level":2,"score":0.600580096244812},{"id":"https://openalex.org/C2778917026","wikidata":"https://www.wikidata.org/wiki/Q43041","display_name":"Hemoglobin","level":2,"score":0.5481354594230652},{"id":"https://openalex.org/C203014093","wikidata":"https://www.wikidata.org/wiki/Q101929","display_name":"Immunology","level":1,"score":0.49186134338378906},{"id":"https://openalex.org/C2778248108","wikidata":"https://www.wikidata.org/wiki/Q5445","display_name":"Anemia","level":2,"score":0.46874454617500305},{"id":"https://openalex.org/C126322002","wikidata":"https://www.wikidata.org/wiki/Q11180","display_name":"Internal medicine","level":1,"score":0.4661974012851715},{"id":"https://openalex.org/C2778620579","wikidata":"https://www.wikidata.org/wiki/Q185034","display_name":"Sickle cell anemia","level":3,"score":0.44192373752593994},{"id":"https://openalex.org/C2776175824","wikidata":"https://www.wikidata.org/wiki/Q1145668","display_name":"Hemolytic anemia","level":2,"score":0.4210616946220398},{"id":"https://openalex.org/C206396517","wikidata":"https://www.wikidata.org/wiki/Q422025","display_name":"Haptoglobin","level":2,"score":0.41670647263526917},{"id":"https://openalex.org/C142724271","wikidata":"https://www.wikidata.org/wiki/Q7208","display_name":"Pathology","level":1,"score":0.3876919150352478},{"id":"https://openalex.org/C90924648","wikidata":"https://www.wikidata.org/wiki/Q120569","display_name":"Gastroenterology","level":1,"score":0.3541756272315979},{"id":"https://openalex.org/C2779134260","wikidata":"https://www.wikidata.org/wiki/Q12136","display_name":"Disease","level":2,"score":0.28968361020088196}],"mesh":[{"descriptor_ui":"D000077209","descriptor_name":"Decitabine","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D000081","descriptor_name":"Acetamides","qualifier_ui":"Q000627","qualifier_name":"therapeutic use","is_major_topic":false},{"descriptor_ui":"D000755","descriptor_name":"Anemia, Sickle Cell","qualifier_ui":"Q000628","qualifier_name":"therapy","is_major_topic":true},{"descriptor_ui":"D001374","descriptor_name":"Azacitidine","qualifier_ui":"Q000031","qualifier_name":"analogs & derivatives","is_major_topic":false},{"descriptor_ui":"D001374","descriptor_name":"Azacitidine","qualifier_ui":"Q000627","qualifier_name":"therapeutic use","is_major_topic":false},{"descriptor_ui":"D002087","descriptor_name":"Butyrates","qualifier_ui":"Q000627","qualifier_name":"therapeutic use","is_major_topic":false},{"descriptor_ui":"D006801","descriptor_name":"Humans","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D006918","descriptor_name":"Hydroxyurea","qualifier_ui":"Q000627","qualifier_name":"therapeutic use","is_major_topic":false},{"descriptor_ui":"D009569","descriptor_name":"Nitric Oxide","qualifier_ui":"Q000627","qualifier_name":"therapeutic use","is_major_topic":false},{"descriptor_ui":"D014320","descriptor_name":"Triphenylmethyl Compounds","qualifier_ui":"Q000627","qualifier_name":"therapeutic use","is_major_topic":false},{"descriptor_ui":"D017707","descriptor_name":"Erythrocyte Transfusion","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false}],"locations_count":5,"locations":[{"id":"pmid:16434364","doi":null,"is_oa":false,"landing_page_url":"https://pubmed.ncbi.nlm.nih.gov/16434364","pdf_url":null,"source":{"id":"https://openalex.org/S4306525036","display_name":"PubMed","issn_l":null,"issn":null,"is_oa":false,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":"https://openalex.org/I1299303238","host_organization_name":"National Institutes of Health","host_organization_lineage":["https://openalex.org/I1299303238"],"host_organization_lineage_names":[],"type":"repository"},"license":null,"license_id":null,"version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"Haematologica","raw_type":"Editorial"},{"id":"pmh:oai:europepmc.org:1372362","doi":null,"is_oa":true,"landing_page_url":"https://www.ncbi.nlm.nih.gov/pmc/articles/2204144","pdf_url":null,"source":{"id":"https://openalex.org/S4306400806","display_name":"Europe PMC (PubMed Central)","issn_l":null,"issn":null,"is_oa":false,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":"https://openalex.org/I1303153112","host_organization_name":"European Bioinformatics Institute","host_organization_lineage":["https://openalex.org/I1303153112"],"host_organization_lineage_names":[],"type":"repository"},"license":null,"license_id":null,"version":"acceptedVersion","is_accepted":true,"is_published":false,"raw_source_name":null,"raw_type":"Text"},{"id":"pmh:oai:pubmedcentral.nih.gov:2204144","doi":null,"is_oa":true,"landing_page_url":"https://pmc.ncbi.nlm.nih.gov/articles/PMC2204144/","pdf_url":null,"source":{"id":"https://openalex.org/S2764455111","display_name":"PubMed Central","issn_l":null,"issn":null,"is_oa":true,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":"https://openalex.org/I1299303238","host_organization_name":"National Institutes of Health","host_organization_lineage":["https://openalex.org/I1299303238"],"host_organization_lineage_names":[],"type":"repository"},"license":null,"license_id":null,"version":"acceptedVersion","is_accepted":true,"is_published":false,"raw_source_name":"Haematologica","raw_type":"Text"},{"id":"pmh:oai:doaj.org/article:a34e1c2f0c7143cabdbd5976d6f91e9b","doi":null,"is_oa":false,"landing_page_url":"https://doaj.org/article/a34e1c2f0c7143cabdbd5976d6f91e9b","pdf_url":null,"source":{"id":"https://openalex.org/S4306401280","display_name":"DOAJ (DOAJ: Directory of Open Access Journals)","issn_l":null,"issn":null,"is_oa":false,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":null,"host_organization_name":null,"host_organization_lineage":[],"host_organization_lineage_names":[],"type":"repository"},"license":null,"license_id":null,"version":"submittedVersion","is_accepted":false,"is_published":false,"raw_source_name":"Haematologica, Vol 91, Iss 1 (2006)","raw_type":"article"},{"id":"mag:2173075639","doi":null,"is_oa":false,"landing_page_url":"https://pubmed.ncbi.nlm.nih.gov/16434364","pdf_url":null,"source":{"id":"https://openalex.org/S4306525036","display_name":"PubMed","issn_l":null,"issn":null,"is_oa":false,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":"https://openalex.org/I1299303238","host_organization_name":"National Institutes of Health","host_organization_lineage":["https://openalex.org/I1299303238"],"host_organization_lineage_names":[],"type":"repository"},"license":null,"license_id":null,"version":null,"is_accepted":false,"is_published":false,"raw_source_name":"PubMed","raw_type":null}],"best_oa_location":{"id":"pmh:oai:europepmc.org:1372362","doi":null,"is_oa":true,"landing_page_url":"https://www.ncbi.nlm.nih.gov/pmc/articles/2204144","pdf_url":null,"source":{"id":"https://openalex.org/S4306400806","display_name":"Europe PMC (PubMed Central)","issn_l":null,"issn":null,"is_oa":false,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":"https://openalex.org/I1303153112","host_organization_name":"European Bioinformatics Institute","host_organization_lineage":["https://openalex.org/I1303153112"],"host_organization_lineage_names":[],"type":"repository"},"license":null,"license_id":null,"version":"acceptedVersion","is_accepted":true,"is_published":false,"raw_source_name":null,"raw_type":"Text"},"sustainable_development_goals":[{"id":"https://metadata.un.org/sdg/3","display_name":"Good health and well-being","score":0.9922999739646912}],"study_designs":[],"awards":[{"id":"https://openalex.org/G6052222941","display_name":"Natural History of Sickle Cell Disease and Other Hemolytic Disorders","funder_award_id":"ZIAHL006014","funder_id":"https://openalex.org/F4320337338","funder_display_name":"National Heart, Lung, and Blood Institute"}],"funders":[{"id":"https://openalex.org/F4320337338","display_name":"National Heart, Lung, and Blood Institute","ror":"https://ror.org/012pb6c26"}],"has_content":{"pdf":false,"grobid_xml":false},"content_urls":null,"referenced_works_count":35,"referenced_works":["https://openalex.org/W76712255","https://openalex.org/W88937938","https://openalex.org/W145216157","https://openalex.org/W207387586","https://openalex.org/W1751167626","https://openalex.org/W1965738657","https://openalex.org/W1970519878","https://openalex.org/W1972716918","https://openalex.org/W1989872169","https://openalex.org/W1993612737","https://openalex.org/W1996496210","https://openalex.org/W2005469488","https://openalex.org/W2022246541","https://openalex.org/W2024351946","https://openalex.org/W2033040678","https://openalex.org/W2048176236","https://openalex.org/W2049292864","https://openalex.org/W2057677658","https://openalex.org/W2066708380","https://openalex.org/W2078876535","https://openalex.org/W2111221288","https://openalex.org/W2147182596","https://openalex.org/W2150027863","https://openalex.org/W2160228508","https://openalex.org/W2168005749","https://openalex.org/W2169390962","https://openalex.org/W2195280390","https://openalex.org/W2212470184","https://openalex.org/W2313541759","https://openalex.org/W2321383376","https://openalex.org/W2323849691","https://openalex.org/W2336959523","https://openalex.org/W2338369943","https://openalex.org/W2903878519","https://openalex.org/W2914437448"],"related_works":["https://openalex.org/W7128513137","https://openalex.org/W4388165034","https://openalex.org/W2006754069","https://openalex.org/W7120407642","https://openalex.org/W2077702192","https://openalex.org/W4206787167","https://openalex.org/W2046661033","https://openalex.org/W7098651061","https://openalex.org/W2088787533","https://openalex.org/W4313730593"],"abstract_inverted_index":{"Homozygous":[0],"sickle":[1],"cell":[2,115],"disease":[3,10,179],"(SCD)":[4],"is":[5,44,99,260,325],"an":[6],"autosomal":[7],"recessive":[8],"genetic":[9],"that":[11,68,135],"results":[12,162],"from":[13,88,163],"the":[14,25,53,78,100,189,194,197,215,219,243,311,323,336],"substitution":[15],"of":[16,24,31,35,42,85,102,106,166,191,199,210,221,241,264,272],"valine":[17],"for":[18,245,315],"glutamic":[19],"acid":[20],"at":[21],"position":[22],"6":[23],"\u03b2-globin":[26],"gene,":[27],"leading":[28],"to":[29,286,327],"production":[30,247],"a":[32,103,164,253,261],"defective":[33],"form":[34],"hemoglobin,":[36],"hemoglobin":[37,109,192,212,292],"S":[38],"(HbS).":[39],"The":[40,81],"prevalence":[41],"SCD":[43,63,86,187],"about":[45],"1\u20132%":[46],"among":[47,73],"African":[48],"descendants":[49],"in":[50,60,77,108,186,214,218,256,283,297,335],"Europe":[51],"and":[52,56,75,95,111,126,142,147,157,168,170,204,259,268,276,279],"United":[54],"States":[55],"4%":[57],"or":[58,180],"higher":[59],"West":[61],"Africa.":[62],"shows":[64],"broad":[65],"phenotypic":[66],"expression":[67,271],"varies":[69],"greatly":[70],"between":[71],"regions,":[72],"patients":[74],"longitudinally":[76],"same":[79],"patient.1,2":[80],"protean":[82],"clinical":[83,133],"features":[84],"result":[87],"chronic":[89],"variable":[90],"intravascular":[91,295],"hemolysis,":[92],"microvascular":[93,122],"ischemia":[94],"organ":[96,158],"damage.":[97],"Vaso-occlusion":[98],"outcome":[101],"dynamic":[104],"combination":[105,165],"abnormalities":[107],"structure":[110],"function,":[112],"red":[113,235],"blood":[114,239],"membrane":[116],"integrity,":[117],"erythrocyte":[118],"density,":[119],"endothelial":[120,273],"activation,":[121],"tone,":[123,270],"inflammatory":[124],"mediators,":[125],"coagulation.":[127],"These":[128],"pathophysiologic":[129],"events":[130],"translate":[131],"into":[132,137,193],"manifestations":[134],"fall":[136],"four":[138],"general":[139],"categories:":[140],"anemia":[141],"its":[143,287],"sequelae;":[144],"vaso-occlusive":[145],"crises":[146],"bone":[148],"marrow":[149],"fat":[150],"embolization":[151],"syndrome;":[152],"infection":[153],"(from":[154],"functional":[155],"asplenia)":[156],"dysfunction.":[159],"Organ":[160],"damage":[161],"hemolysis":[167,185,296],"infarction":[169],"may":[171,299],"be":[172],"manifested":[173],"as":[174,333],"stroke,":[175],"retinopathy,":[176],"nephropathy,":[177],"liver":[178],"pulmonary":[181],"arterial":[182],"hypertension.":[183],"Intravascular":[184],"causes":[188],"release":[190],"plasma.":[195],"When":[196],"capacity":[198],"protective":[200],"hemoglobin-scavenging":[201],"mechanisms":[202],"(haptoglobin":[203],"hemopexin)":[205],"has":[206],"been":[207],"saturated,":[208],"levels":[209,293],"cell-free":[211,290],"increase":[213],"plasma":[216,240,291],"resulting":[217],"consumption":[220],"nitric":[222],"oxide":[223],"(NO)":[224],"by":[225,233,248,289],"hemoglobin-mediated":[226],"NO":[227,246,249,251],"scavenging.3":[228],"In":[229,305],"addition,":[230],"arginase":[231],"released":[232],"hemolyzed":[234],"cells":[236],"can":[237],"deplete":[238],"arginine,":[242],"substrate":[244],"synthase.4":[250],"plays":[252],"major":[254],"role":[255],"vascular":[257],"homeostasis":[258],"critical":[262],"regulator":[263],"smooth":[265],"muscle":[266],"relaxation":[267],"vasomotor":[269],"adhesion":[274],"molecules":[275],"platelet":[277],"activation":[278],"aggregation.5":[280],"A":[281],"deficiency":[282],"NO,":[284],"due":[285],"inactivation":[288],"during":[294],"SCD,":[298],"underlie":[300],"complications":[301],"associated":[302],"with":[303],"SCD.6":[304],"this":[306],"editorial,":[307],"we":[308],"briefly":[309],"review":[310,331],"currently":[312],"available":[313],"treatments":[314],"SCD.":[316],"For":[317],"more":[318,328],"detail":[319],"on":[320],"specific":[321],"topics,":[322],"reader":[324],"directed":[326],"extensive":[329],"recent":[330],"articles":[332],"cited":[334],"references.":[337]},"counts_by_year":[{"year":2025,"cited_by_count":1},{"year":2024,"cited_by_count":3},{"year":2023,"cited_by_count":1},{"year":2022,"cited_by_count":2},{"year":2021,"cited_by_count":3},{"year":2020,"cited_by_count":5},{"year":2019,"cited_by_count":3},{"year":2018,"cited_by_count":8},{"year":2017,"cited_by_count":2},{"year":2016,"cited_by_count":5},{"year":2015,"cited_by_count":3},{"year":2014,"cited_by_count":4},{"year":2013,"cited_by_count":5},{"year":2012,"cited_by_count":3}],"updated_date":"2026-11-10T11:39:43.021295","created_date":"2016-06-24T00:00:00"}
