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Subject:
From:
"Lawrence M. Gartner" <[log in to unmask]>
Reply To:
Lactation Information and Discussion <[log in to unmask]>
Date:
Fri, 25 Apr 1997 16:52:28 -0500
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From my chapter on "Disorders of Bilirubin Metabolism" (Volume 1, Chapter
4)in Hemtology of Infancy and Childhood, Edited by Nathan and Oski, 4th
Edition, W.B. Saunders, 1993, page 90:

        Transient Familial Neonatal Hyperbilirubinemia (Lucey-Driscoll
Syndrome)

                "This is an extremely rare disorder characterized by
development of unconjugated hyperbilirubinemia in the absence of hemolysis
during the first week of life.  Exchange transfusion is needed to prevent
kernicterus in many cases.  A potent but unidentified inhibitor of
glucuronyl transferase [the liver bilirubin conjugating enzyme] can be
found in the circulations of both the mother and the infant.  By the end of
second week postpartum, the inhibitor disappears from both mother and
child, and serum bilirubin concentrations in the infant decline to normal.
The mother has no hyperbilirubinemia.

        References:
(1) Lucey JF, Driscoll JJ.  Physiologic jaundice re-examined.  In
Kernicterus.  Sass-Kortsak, A. Editor.  Toronto, University of Toronto
Press, 1961, P.29.
(2) Lucey JF, Arias IM et al.  Transient familial neonatal
hyperbilirubinemia.  Am J Dis Child 100:787, 1960
(3) Arias IM, Wolfson, S et al.  Transient familial neonatal
hyperbilirubinemia.  J Clin Invest 44:1442, 1965

        The Lucey-Driscoll Syndrome diagnosis should not be made without
laboratory evidence of the presence of a circulating inhibitor of
glucuronyl transferase activity in maternal and newborn blood, although it
may be suspected on clinical grounds.  Since it is an early onset
exaggerated hyperbilirubinemia, it could be confused with breastfeeding
jaundice, which is caused by insufficient breastfeeding - or the equivalent
of starvation jaundice.  It also could be confused with breastmilk
jaundice, which starts in the second week of life, but breastmilk jaundice
usually reaches its peak level of hyperbilirubinemia around days 10 to 14
of life, whereas in the Lucey-Driscoll Syndrome the hyperbilirubinemia has
usually resolved by this time.  Breastmilk Jaundice is present in about
two-thirds of ALL breastfed infantsI strongly suspect that the diagnosis of
Lucey-Driscoll Syndrome is being abused and overused, but without specific
clinical and laboratory data, it is not possible to know which diagnosis is
the correct one in each child.  Keep in mind that many of the
Lucey-Driscoll Syndrome babies developed kernicterus because of the rapid
and extreme rise of serum bilirubin concentrations.  That pattern of rapid
bilirubin rise not seen in the breastfeeding or breastmilk types of jaundice.
                                        Larry Gartner


PLEASE NOTE NEW AREA CODE FOR PHONE & FAX

Lawrence M. Gartner, M.D.
Professor of Pediatrics and Obstetrics/Gynecology
The University of Chicago
MC6060
5841 S. Maryland Avenue
Chicago, IL 60637
Phone: (773) 702-0389
FAX: (773) 702-0764
E-Mail: [log in to unmask]

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