PEDIATRICS Vol. 108 No. 1 July 2001, pp. 25-30
A Single Dose of Sn-Mesoporphyrin Prevents Development of Severe Hyperbilirubinemia in Glucose-6-Phosphate Dehydrogenase-Deficient Newborns
Received Sep 19, 2000; accepted Nov 9, 2000.

From * Rockefeller University Hospital, New York, New York; and
Objectives. Severe neonatal jaundice
is a common clinical manifestation of glucose-6-phosphate dehydrogenase
(G-6-PD) deficiency and the most difficult to manage; kernicterus is
not an uncommon outcome. We assessed in healthy, direct Coombs
test-negative Greek newborns of Methods. The studies were conducted at Metera Maternity
Hospital in Athens, Greece. Enrolled newborns had the plasma bilirubin
concentration (PBC) determined in cord blood and daily thereafter until
a declining level was obtained and the case was closed. Intervention
with phototherapy was dictated at exact, age-specific PBC levels. In our initial study, we enrolled consecutive mature healthy
G-6-PD-deficient newborns as well as a threefold excess of
G-6-PD-normal neonates born at approximately the same time (control
group). For the SnMP trial, G-6-PD-deficient neonates were administered
SnMP as a single intramuscular dose of 6 µmol/kg birth weight within
24 ± 12 hours of age.
Results. SnMP was administered at 26.7 ± 6.1 hours
of age to 172 G-6-PD-deficient newborns (group A); 168 G-6-PD-normal
(group B) and 58 G-6-PD-deficient (group C) newborns who were enrolled
earlier provided the comparison groups. Except for the expected excess of males in the G-6-PD-deficient groups (A and C), there were no
differences in the demographic characteristics among the 3 groups. The
incremental changes in PBC from cord blood to 24 hours of age also were
similar (group A: 4.13 ± 1.32 mg/dL; group B: 4.05 ± 1.34 mg/dL; group C: 4.39 ± 1.07 mg/dL), but there were significant
differences in the next period, 24 to 48 hours of age (group A:
0.63 ± 1.44 mg/dL; group B: 1.69 ± 1.5 mg/dL; group C:
2.45 ± 1.72 mg/dL). Peak PBC was significantly different (group A: 7.81 ± 3.04 mg/dL; group B: 8.68 ± 3.1 mg/dL; group C:
11.24 ± 3.76 mg/dL) as was the age at which peak PBC was recorded
(group A: 56 ± 29 hours of age; group B: 69 ± 26 hours of
age; group C: 83 ± 29 hours of age). These differences in favor
of group A were observed despite the fact that phototherapy was used in 15% of the newborns in group B and 31% of those in group C, whereas none of those treated with SnMP required phototherapy. Finally, in one
female, who was heterozygous for G-6-PD deficiency, in group C
phototherapy failed and 2 exchange transfusions were performed.
Conclusions. In comparison with normal neonates,
G-6-PD-deficient neonates experienced a twofold increase in the
prevalence of significant hyperbilirubinemia requiring phototherapy. A
single dose of SnMP administered in the 1st day of life to the
G-6-PD-deficient newborns shifted the peak PBC distribution to the left
(lower values) even in relation to normal neonates and entirely
eliminated the need for phototherapy. Interdiction of bilirubin
production by use of a heme oxygenase inhibitor such as SnMP represents
a simple and highly effective means for the preventive management of
jaundice in G-6-PD-deficient newborns.
Metera Maternity Hospital, Athens, Greece, and New England Medical
Center, Boston, Massachusetts.
38 weeks' gestational age 1) the
current burden of G-6-PD deficiency-associated severe jaundice, and 2)
the efficacy of preventive use of Sn-mesoporphyrin (SnMP), a potent
inhibitor of heme oxygenase activity and thus of bilirubin production,
in ameliorating jaundice in G-6-PD-deficient neonates.
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