Abstract
Duplication of the 11p15 region has been described in some patients with BW, also called Exomphalos-Macroglossia Gigantism syndrome. IGF II gene is localized at the 11p15 region. A markedly increased expression of IGF II gene was reported in Wilm's tumour, the incidence of which is increased in BW. These findings had led us to speculate for a possible overproduction of IGF II protein in this disorder. Using a specific binding assay (JCEM 1986) and a RIA for comparative determination of IGF I, we hove measured the serum levels of 11 unrelated children (aged from 1 month to 7 years) with BW. 4 had successive investigations. In 4 infants out of 8 before 1 year, IGF I was more elevated than controls. An overgrowth was present in 3. In the older children from one to seven years IGF I did not differ from normals, IGF II levels were similar to the controls whatever the age. Moreover, in three children having developed a tumour (ganglioneuroma n = 2, nephroblastoma n = 1) IGF II levels measured before surgery were not elevated. These patients had normal karyotypes. Quantification of IGF II mRNA in the tumour is under investigation.
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