title: A pediatrician's practical guide to diagnosing and treating hereditary spherocytosis in neonates.
abstract: Newborn infants who have hereditary spherocytosis (HS) can develop anemia and hyperbilirubinemia. Bilirubin-induced neurologic dysfunction is less likely in these neonates if the diagnosis of HS is recognized and appropriate treatment provided. Among neonates listed in the USA Kernicterus Registry, HS was the third most common underlying hemolytic condition after glucose-6-phosphate dehydrogenase deficiency and ABO hemolytic disease. HS is the leading cause of direct antiglobulin test (direct Coombs) negative hemolytic anemia requiring erythrocyte transfusion in the first months of life. We anticipate that as physicians become more familiar with diagnosing HS in the newborn period, fewer neonates with HS will develop hazardous hyperbilirubinemia or present to emergency departments with unanticipated symptomatic anemia. We predict that early suspicion, prompt diagnosis and treatment, and anticipatory guidance will prevent adverse outcomes in neonates with HS. The purpose of this article was to review the neonatal presentation of HS and to provide practical and up-to-date means of diagnosing and treating HS in neonates.
authors.affiliation: Divisions of Neonatology and Hematology/Oncology, Department of Pediatrics, University of Utah School of Medicine, Primary Children's Hospital, Intermountain Healthcare, Salt Lake City, Utah; and robert.christensen@hsc.utah.edu.
authors.forename: Robert D
authors.initials: RD
authors.lastname: Christensen
authors.affiliation: Hematology/Oncology, Department of Pediatrics, University of Utah School of Medicine, Primary Children's Hospital, Intermountain Healthcare, Salt Lake City, Utah; and.
authors.forename: Hassan M
authors.initials: HM
authors.lastname: Yaish
authors.affiliation: Division of Neonatology, Department of Pediatrics, Yale University School of Medicine, Yale-New Haven Children's Hospital, New Haven, Connecticut.
authors.forename: Patrick G
authors.initials: PG
authors.lastname: Gallagher
mesh_terms: D017487:Ankyrins / Q000172:deficiency*; D003663:Decision Trees; D006801:Humans; D007231:Infant, Newborn; D010372:Pediatrics; D017410:Practice Guidelines as Topic; D013103:Spherocytosis, Hereditary / Q000175:diagnosis* / Q000628:therapy*
chemical_list: D017487:Ankyrins