Hereditary Spherocytosis in the Neonatal Period: A Case Report.

Journal Article (Journal Article)

Hereditary spherocytosis (HS) is the third most common yet most frequently underrecognized, congenitally acquired hemolytic disease of the neonate. Hereditary spherocytosis is caused by a defect of one or more erythrocyte membrane proteins, which leads to an increased rate of destruction of circulating red blood cells. The HS spectrum of symptoms is varied from asymptomatic to intrauterine hydrops. Diagnostic tests range from a complete blood count (CBC) analysis to deoxyribonucleic acid (DNA) sequencing. Management in the neonatal period focuses primarily on associated comorbidities, including the prevention of severe hyperbilirubinemia and anemia. Life span implications of HS include hemolysis, jaundice, anemia, splenomegaly, and periodic gallstones. Early identification and diagnosis of HS is essential to ensure proper monitoring and medical management throughout infancy, childhood, and adulthood.

Full Text

Duke Authors

Cited Authors

  • Will, A; Henderson, CA; Jnah, AJ; Newberry, D

Published Date

  • September 2017

Published In

Volume / Issue

  • 36 / 5

Start / End Page

  • 280 - 288

PubMed ID

  • 28847351

Electronic International Standard Serial Number (EISSN)

  • 1539-2880

International Standard Serial Number (ISSN)

  • 0730-0832

Digital Object Identifier (DOI)

  • 10.1891/0730-0832.36.5.280


  • eng