Abstract
Circulating Sm–C reflects protein synthesis and the ability to stimulate skeletal growth in close relationship with caloric intake. The occurence of spontaneous chronic undereating in a boy with Laron type dwarfism allowed to study the effect of caloric intake on growth in primary Sm–C deficiency. The 4 yrs old boy had basal GH > 40 ng/ml, Sm–C/IGF I < 0.10 U/ml unresponsive to hGH treatment, typical features with hypoglycemia and severe dwarfism. He was studied over 2 periods. Period I (5 mo) control with spontaneously low caloric intake of 60 Kcal/Kg/d and 1.4 g/Kg/d protein. Period II (5 mo) with 130 Kcal/Kg/d and 3.8 g/Kg/d protein given by constant enteral infusion. Results were the following
Weight gain paralleled growth acceleration and hypoglycemia attacks disappeared. Refeeding with a normal diet resulted in a subtantial catch-up growth in spite of unchanged Sm–C values. In conclusion, this study provided the opportunity to demonstrate that growth may be stimulated in the absence of normal levels of circulating Sm–C/IGF I. It suggests that nutrition may act independently of circulating Sm–C and GH and plays a major role in promoting statural growth.
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Crosnier, H., Prévot, C. & Rappaport, R. 71 CALORIC SUPPLEMENTATION INDUCES GROWTH ACCELERATION WITHOUT Sm-C CHANGES IN A LARON TYPE DWARF. Pediatr Res 24, 529 (1988). https://doi.org/10.1203/00006450-198810000-00092
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DOI: https://doi.org/10.1203/00006450-198810000-00092