title: Association between baseline fetal hemoglobin levels and incidence of severe vaso-occlusive pain episodes in children with sickle cell anemia.
abstract: The ameliorating effect of high fetal hemoglobin (HbF) levels on the incidence of pain episodes in sickle cell anemia (SCA) is well-known; however, in children this relationship is less clearly established. We hypothesized that higher HbF levels in children with SCA are associated with fewer severe pain episodes. A meta-analysis of data from the Silent Infarct Transfusion Trial (n = 456) and the Cooperative Study of Sickle Cell Disease (n = 764), demonstrated that baseline HbF levels were associated with the incidence of severe pain, commonly defined across studies as an event requiring hospitalization (P-value = 0.02).
authors.affiliation: McKusick-Nathans Institute of Genetic Medicine, School of Medicine, Johns Hopkins University, Baltimore, MD 21205, USA.
authors.forename: Pallav
authors.initials: P
authors.lastname: Bhatnagar
authors.forename: Jeffrey R
authors.initials: JR
authors.lastname: Keefer
authors.forename: James F
authors.initials: JF
authors.lastname: Casella
authors.forename: Emily A
authors.initials: EA
authors.lastname: Barron-Casella
authors.forename: Christopher J
authors.initials: CJ
authors.lastname: Bean
authors.forename: Craig W
authors.initials: CW
authors.lastname: Hooper
authors.forename: Amanda B
authors.initials: AB
authors.lastname: Payne
authors.forename: Dan E
authors.initials: DE
authors.lastname: Arking
authors.forename: Michael R
authors.initials: MR
authors.lastname: Debaun
mesh_terms: D000293:Adolescent; D000755:Anemia, Sickle Cell / Q000097:blood* / Q000150:complications / Q000188:drug therapy*; D002648:Child; D002675:Child, Preschool; D003251:Constriction, Pathologic / Q000097:blood / Q000150:complications / Q000188:drug therapy; D005260:Female; D005319:Fetal Hemoglobin / Q000378:metabolism*; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D010146:Pain / Q000097:blood* / Q000188:drug therapy* / Q000209:etiology
chemical_list: D005319:Fetal Hemoglobin
keywords: fetal hemoglobin; sickle cell anemia; vaso-occlusive pain