title: Weighing the evidence for newborn screening for Hemoglobin H disease.
abstract: OBJECTIVE
  To conduct a systematic review to assist the United States Secretary of Health and Human Services Advisory Committee on Heritable Disorders in Newborns and Children (SACHDNC) to determine whether Hemoglobin H screening should be included among the core recommended conditions for newborn screening.
  
  
  METHODS
  We identified 21 articles in MEDLINE from 1989 to March 2010 that provided evidence regarding screening, treatment, and outcomes associated with Hemoglobin H disease.
  
  
  RESULTS
  In California, newborn screening has identified 9 cases per 100 000 of deletional hemoglobin H disease and 0.6 cases per 100 000 of nondeletional hemoglobin H disease. Five cases of hemoglobin Bart's hydrops fetalis syndrome were also identified in over ten years of screening for Hemoglobin H disease. Although Hemoglobin H disease is associated with a wide range of morbidity, no studies were found that evaluated the benefits of early identification and treatment.
  
  
  CONCLUSIONS
  The SACHDNC found the data insufficient to recommend that states adopt newborn screening for Hemoglobin H disease.
authors.affiliation: Duke Clinical Research Institute, Duke University, Durham, NC 27705, USA. alex.kemper@duke.edu
authors.forename: Alex R
authors.initials: AR
authors.lastname: Kemper
authors.forename: Alixandra A
authors.initials: AA
authors.lastname: Knapp
authors.forename: Danielle R
authors.initials: DR
authors.lastname: Metterville
authors.forename: Anne Marie
authors.initials: AM
authors.lastname: Comeau
authors.forename: Nancy S
authors.initials: NS
authors.lastname: Green
authors.forename: James M
authors.initials: JM
authors.lastname: Perrin
mesh_terms: D006801:Humans; D015994:Incidence; D007231:Infant, Newborn; D015997:Neonatal Screening / Q000379:methods*; D014481:United States / Q000453:epidemiology; D017085:alpha-Thalassemia / Q000453:epidemiology*