PEDIATRICS Vol. 18 No. 3 September 1956, pp. 369-377
This Article
Right arrow Full Text (PDF)
Right arrow P3Rs: Submit a response
Right arrow Alert me when this article is cited
Right arrow Alert me when P3Rs are posted
Right arrow Alert me if a correction is posted
Services
Right arrow E-mail this article to a friend
Right arrow Similar articles in this journal
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Add to My File Cabinet
Right arrow Download to citation manager
Right arrow reprints & permissions
Citing Articles
Right arrow Citing Articles via CrossRef
Right arrow Citing Articles via Google Scholar
Google Scholar
Right arrow Articles by Childs, B.
Right arrow Articles by Najjar, V. A.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by Childs, B.
Right arrow Articles by Najjar, V. A.

FAMILIAL NONHEMOLYTIC JAUNDICE WITH KERNICTERUS

A Report of Two Cases Without Neurologic Damage

Barton Childs M.D.1 and Victor A. Najjar M.D.1

1 Department of Pediatrics, Johns Hopkins University Medical School, and Harriet Lane Home, Johns Hopkins Hospital

Two cases of familial nonhemolytic jaundice have been described. Both patients, though severely jaundiced for periods of 5 and 2 years, have grown and developed normally and show no evidence of damage of the nervous system. This is in contradistinction to the six other patients, presented in the original report of this disease, who died after developing kernicterus. No reasons can be suggested for the escape of these two patients from kernicterus.

Some speculations concerning the nature of the genetically controlled aberration in this disease have been given.

It is suggested as a plausible hypothesis that the lesion consists of deficiency of an enzyme which controls the chelation of indirect-reacting bilirubin so as to form direct-reacting bilirubin. The enzyme, though deficient, is capable of adequate function if a sufficient load of bilirubin is presented to the liver. Under such circumstances bilirubin is excreted in normal amount, but only in the presence of a large accumulation of the pigment in the blood.

Submitted on January 9, 1956
Accepted on February 17, 1956