Growth hormone treatment of Russell-Silver syndrome

Abstract

Russell-Silver syndrome represents a special group of children with intrauterine growth retardation (IUGR) who do not experience catch-up growth and have characteristic dysmorphic features. They also have characteristics of abnormal growth hormone pulsatility, absence of catch-down growth after growth hormone therapy and inappropriate advancement of bone age during the middle childhood years. Data from children with Russell-Silver syndrome should certainly be analysed as a separate group from short children due to nondysmorphic IUGR. Initial data suggests that final height outcome will be improved by using pharmacological doses of biosynthetic human growth hormone. Indeed, the recent data supports the hypothesis of Blizzard's group in 1974 that if growth hormone became available in sufficient quantities, then final height could be altered in IUGR children. In addition, the early recognition and treatment of spontaneous nocturnal hypoglycaemia may well improve the educational achievement of such children.