Pioneers in the Scientific Study of Neonatal Jaundice and Kernicterus

Abstract

Neonatal jaundice must have been noticed by caregivers through the centuries, but the scientific description and study of this phenomenon seem to have started in the last half of the 18th century. In 1785 Jean Baptiste Thimotée Baumes was awarded a prize from the University of Paris for his work describing the clinical course in 10 jaundiced infants. The work by Jaques Hervieux, which he defended for his doctor of medicine degree in 1847, was, in many respects, a landmark. He had autopsied 44 jaundiced infants and apparently had clinical observations on many others. His descriptions of pathoanatomical findings were very detailed and systematic. A number of his clinical observations are still thought to be accurate today, such as the essentially benign nature of neonatal jaundice in most cases, the appearance of neonatal jaundice during the first 2 to 4 days of life as well as its disappearance within 1 to 2 weeks, and the cephalocaudal progression of jaundice. He described jaundice of the brain in 31 of his 44 autopsied cases, with variable intensity of staining. Johannes Orth was an assistant to the famous Virchow in Berlin, when in 1875 he published the results of an autopsy of a jaundiced term infant. The brain was notable for an intense yellow staining of the basal ganglia, the wall of the third ventricle, the hippocampus, and the central parts of the cerebellum. While the contribution of Orth was limited to this single case report, in 1903 Christian Schmorl presented the results of his autopsies of 120 jaundiced infants to the German Society for Pathology. All of these infants' brains were jaundiced, but only 6 cases demonstrated a staining phenomenon similar to that previously described by Orth. Schmorl coined the term kernicterus (jaundice of the basal ganglia) for this staining pattern. Although the following century of scientific study has added an enormous amount of information about the epidemiology and pathophysiology of neonatal jaundice and kernicterus, the contributions of Hervieux, Orth, and Schmorl will undoubtedly continue to be seen as historical landmarks in our quest for understanding of these phenomena.