The natural history of congenital hemolytic jaundice (congenital hemolytic anemia, hereditary spherocytosis) is well known in the medical literature. A number of reviews have pointed out the usual features of this disease, which is characterized clinically by a positive family history, dominant mode of inheritance, recurrent crises, splenomegaly, secondary cholelithiasis, microspherocytes in the blood smear, increased RBC osmotic fragility in hypotonic saline, and effective treatment by means of splenectomy.1-5
The age of onset of clinical symptoms has been variable but is stated to be most commonly in childhood or in young adulthood.4 There are a number of reports concerning symptoms in infancy, the cases usually attracting attention because of anemia.6-12 The often-quoted maxim, "children, and especially infants, with this disease are more sick than jaundiced," reflects the concepts regarding the usual findings in this age group.6
Debré 7 stated that jaundice never shows during the first years,
STAMEY CC, DIAMOND LK. Congenital Hemolytic Anemia in the Newborn: Relationship to Kernicterus. AMA Am J Dis Child. 1957;94(6):616–622. doi:10.1001/archpedi.1957.04030070028005
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