Incidence of hereditary spherocytosis in a population of jaundiced neonates

Pediatr Hematol Oncol. 2006 Jul-Aug;23(5):387-97. doi: 10.1080/08880010600646662.

Abstract

As most of hereditary spherocytosis-affected individuals experience jaundice at birth, it seemed of interest to evaluate the proportion of hereditary spherocytosis in 402 severely jaundiced neonates with a bilirubinemia level prompting phototherapy. Red cell dehydration, a hallmark of spherocytosis whether constitutional or acquired, was demonstrated in 74 of them, among whom 23 disclosed a typical pattern of spherocytosis upon red cell deformability studies. Acquired spherocytosis of immune origin was diagnosed in 19/23 and hereditary spherocytosis in 4, making the proportion of hereditary spherocytosis-affected individuals among a severely jaundiced population of neonates amount to 1%, an incidence at least 30-fold that of the overall population.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Case-Control Studies
  • Erythrocyte Deformability
  • Erythrocytes, Abnormal / pathology
  • Female
  • France / epidemiology
  • Hemoglobins / analysis
  • Humans
  • Incidence
  • Infant, Newborn
  • Jaundice, Neonatal / epidemiology*
  • Jaundice, Neonatal / etiology
  • Male
  • Prospective Studies
  • Spherocytosis, Hereditary / complications
  • Spherocytosis, Hereditary / epidemiology*

Substances

  • Hemoglobins