title: Developmental function in toddlers with sickle cell anemia.
abstract: BACKGROUND
  Neurocognitive impairment occurs in children and adults with sickle cell anemia, but little is known about neurodevelopment in very young children. We examined the neurodevelopmental status of infants participating in the Pediatric Hydroxyurea Phase III Clinical Trial (Baby Hug) to determine relationships with age, cerebral blood flow velocity, and hemoglobin concentration.
  
  
  METHODS
  Standardized measures of infant neurodevelopment were administered to 193 infants with hemoglobin SS or hemoglobin S-β(0) thalassemia between 7 and 18 months of age at the time of their baseline evaluation. Associations between neurodevelopmental scores and age, family income, parent education, hemoglobin concentration, and transcranial Doppler velocity were examined.
  
  
  RESULTS
  Mean functioning on the baseline neurodevelopment scales was in the average range. There were no mental development scores <70 (impaired); 22 children had scores in the clinically significant range, 11 with impaired psychomotor scores and 11 with problematic behavior rating scores. Significantly poorer performance was observed with older age at baseline. Behavior rating scores were an average of 2.82 percentile points lower per month of age, with similar patterns observed with parent report using adaptive behavior scales. Parent-reported functional abilities and hemoglobin were negatively associated with higher transcranial Doppler velocities.
  
  
  CONCLUSIONS
  Whereas overall functioning was in the normal range, behavioral and adaptive function was poorer with older age, even in this very young group of children. Explanatory mechanisms for this association between poorer developmental function and older age need to be identified.
authors.affiliation: Department of Pediatrics (D-820), PO Box 016820, Miami, FL 33101, USA. darmstrong@miami.edu
authors.forename: F Daniel
authors.initials: FD
authors.lastname: Armstrong
authors.forename: T David
authors.initials: TD
authors.lastname: Elkin
authors.forename: R Clark
authors.initials: RC
authors.lastname: Brown
authors.forename: Penny
authors.initials: P
authors.lastname: Glass
authors.forename: Sohail
authors.initials: S
authors.lastname: Rana
authors.forename: James F
authors.initials: JF
authors.lastname: Casella
authors.forename: Ram V
authors.initials: RV
authors.lastname: Kalpatthi
authors.forename: Steven
authors.initials: S
authors.lastname: Pavlakis
authors.forename: Zhibao
authors.initials: Z
authors.lastname: Mi
authors.forename: Winfred C
authors.initials: WC
authors.lastname: Wang
mesh_terms: D000203:Activities of Daily Living / Q000145:classification; D000223:Adaptation, Psychological / Q000187:drug effects / Q000502:physiology; D000367:Age Factors; D000755:Anemia, Sickle Cell / Q000175:diagnosis* / Q000188:drug therapy; D000986:Antisickling Agents / Q000009:adverse effects / Q000627:therapeutic use; D001783:Blood Flow Velocity / Q000187:drug effects / Q000502:physiology; D001921:Brain / Q000098:blood supply; D001925:Brain Damage, Chronic / Q000175:diagnosis* / Q000188:drug therapy; D002653:Child Behavior Disorders / Q000175:diagnosis* / Q000188:drug therapy; D004311:Double-Blind Method; D005260:Female; D006452:Hemoglobinometry; D006801:Humans; D006918:Hydroxyurea / Q000009:adverse effects / Q000627:therapeutic use; D007223:Infant; D008297:Male; D010552:Personality Assessment; D011596:Psychomotor Disorders / Q000175:diagnosis* / Q000188:drug therapy; D012951:Socialization; D063189:Symptom Assessment; D017585:Ultrasonography, Doppler, Transcranial
chemical_list: D000986:Antisickling Agents; D006918:Hydroxyurea