Jaundice with hypertrophic pyloric stenosis as an early manifestation of Gilbert syndrome
Pascale Triochea, Jacqueline Chalasb, Jeanne Francoualb, Liliane Capelb, Albert Lindenbaumb, Michel Odièvrea, Philippe Labrunea
a Service de
Pédiatrie and UPRES EA 2704, Hôpital Antoine Béclère (AP-HP),
157 rue de la Porte de Trivaux, BP 405, 92141 Clamart CEDEX, France, b Laboratoire de Biochimie, Hôpital Antoine
Béclère
Correspondence to: Dr Labrune. email: philippe.labrune{at}abc.ap-hop-paris.fr
Accepted 14 June 1999
Jaundice associated with hypertrophic pyloric stenosis was
recognised in three patients; previous reports have suggested that this
is a possible early manifestation of Gilbert syndrome. Most patients
with Gilbert syndrome are homozygous for a (TA)7TAA
polymorphism in the gene promoter coding for bilirubin
glucuronosyltransferase. Two of the reported patients were homozygous
for the (TA)7TAA polymorphism whereas the third was
heterozygous for the same polymorphism. Furthermore, no other factors
contributing to jaundice in the three patients were found. These
results suggest that jaundice associated with hypertrophic pyloric
stenosis is due to molecular defects within the gene promoter.
Keywords: jaundice; pyloric stenosis; Gilbert syndrome
© 1999 by Archives of Disease in Childhood
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