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February 1967

Neonatal Manifestations of Hereditary Spherocytosis

Am J Dis Child. 1967;113(2):263-270. doi:10.1001/archpedi.1967.02090170127015

THOUGH the natural history of hereditary spherocytosis in adults has long been recognized,1 only recently have investigators become interested in the expression of this inborn error of red-cell metabolism in the neonate.2-9 Although almost 50% of all adults with this disease give a history of significant jaundice in the newborn period,5 the majority of cases are not diagnosed until much later. Overt hemolysis, hyperbilirubinemia and even kernicterus, have been recognized in the newborn with this defect,10,11 and it is now evident that if nurseries seek the cause of exaggerated jaundice, the discovery of this hereditary red-cell defect among infants with hyperbilirubinemia is surprisingly frequent. In our experience, a significant number of full-term infants with hyperbilirubinemia without iso-immunization are found to have hereditary spherocytosis. While hereditary spherocytosis is most common in whites, Negro infants have been diagnosed in the newborn period.4

This report reviews the natural