Abstract
Cerebral gigantism (Sotos syndrome) is characterized by a large birth size, excessive statural growth, advanced bone age, mental retardation and dysmorphic features. In this study 30 plasma samples of 14 children with Sotos syndrome were assayed for IGF-I and II and bioassayable somatomedin activity (SM-act). Immunoreactive IGF-I was determined in unextracted plasma and compared to the Nichols Institute references. Immunoreactive IGF-II was measured with a nonequilibrium RIA, using the tyrosylated eight amino acid C-peptide region of IGF-II (CP-II) and an antiserum against this fragment (kindly donated by Dr. R. Hintz). SM-act was determined with the porcine cartilage bioassay. All but 3 values of IGF-I were within the ± 2 SD range for age. When the data were expressed as a Z-score for age, mean IGF-I decreased from -0.1 (range -1.0 to +1.0, n=6) at 0-3 years to -1.1 (range -2.0 to 0.3, n=7) at 3-5 years. IGF-II levels were generally within the reference range. SM-act was usually low between 1 and 5 years of age (mean -2.2 SD, range -5.0 to 3.6, n=11) and in the lower normal range thereafter. These IGF-I and SM-act results are in contrast to the data in constitutional tall stature. The relatively low somatomedin levels between 1 and 5 years of age concur with the deceleration of growth which is usually seen after the first year of life in children with Sotos syndrome.
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Wit, J., Opmeer, F., van Buul-Offers, S. et al. 109 PLASMA INSULIN-LIKE GROWTH FACTOR (IGF)-I AND II LEVELS AND BIOASSAYABLE SOMATOMEDIN ACTIVITY IN CHILDREN WITH CEREBRAL CIGANTISM (SOTOS SYNDROME). Pediatr Res 24, 535 (1988). https://doi.org/10.1203/00006450-198810000-00130
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DOI: https://doi.org/10.1203/00006450-198810000-00130