Incidence of hereditary spherocytosis in a population of jaundiced neonates.

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.

• Authors: Véronique Saada, Thérèse Cynober, Yves Brossard, Pierre Olivier Schischmanoff, Alfred Sender, Henri Cohen, Jean Delaunay, Gil Tchernia
• Journal: Pediatric hematology and oncology (Pediatr Hematol Oncol) 2006 Jul-Aug Vol. 23 Issue 5 Pg. 387-97 ISSN: 1521-0669 [Electronic] England
• PMID: 16728359 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)

Chemical References

• Hemoglobins

Topics

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