Incidence of hereditary spherocytosis in a population of jaundiced neonates.
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.
Saada
Véronique
V
Laboratoire d'Hématologie, d'Immunologie et de Cytogénétique, Hôpital de Bicêtre, APHP, and Faculté de Médecine Paris-Sud, Le Kremlin Bicêtre, France.
Cynober
Thérèse
T
Brossard
Yves
Y
Schischmanoff
Pierre Olivier
PO
Sender
Alfred
A
Cohen
Henri
H
Delaunay
Jean
J
Tchernia
Gil
G
D016022:Case-Control Studies; D004907:Erythrocyte Deformability; D004913:Erythrocytes, Abnormal / Q000473:pathology; D005260:Female; D005602:France / Q000453:epidemiology; D006454:Hemoglobins / Q000032:analysis; D006801:Humans; D015994:Incidence; D007231:Infant, Newborn; D007567:Jaundice, Neonatal / Q000453:epidemiology* / Q000209:etiology; D008297:Male; D011446:Prospective Studies; D013103:Spherocytosis, Hereditary / Q000150:complications / Q000453:epidemiology*
D006454:Hemoglobins