Related Experiment Videos
Growth hormone treatment of Russell-Silver syndrome
R Stanhope1, A Albanese, C Azcona
1Department of Endocrinology, Great Ormond Street Hospital for Children, London, UK.
Insights
Russell-Silver syndrome (RSS) is a distinct condition in children with intrauterine growth retardation (IUGR). Early growth hormone therapy may improve final height outcomes in these children.
Area of Science:
- Pediatrics
- Endocrinology
- Genetics
Background:
- Russell-Silver syndrome (RSS) is characterized by intrauterine growth retardation (IUGR) without catch-up growth and specific dysmorphic features.
- Children with RSS exhibit abnormal growth hormone pulsatility, lack of catch-down growth post-therapy, and accelerated bone age during mid-childhood.
- RSS necessitates distinct analysis separate from non-dysmorphic IUGR short stature.
Purpose of the Study:
- To evaluate the efficacy of biosynthetic human growth hormone therapy in improving final height in children with Russell-Silver syndrome.
- To investigate the potential benefits of early recognition and treatment of nocturnal hypoglycemia on educational outcomes in RSS patients.
Main Methods:
- Analysis of data from a cohort of children diagnosed with Russell-Silver syndrome.
- Administration of pharmacological doses of biosynthetic human growth hormone.
- Monitoring of growth, bone age advancement, and nocturnal hypoglycemia.
Main Results:
- Preliminary data indicate that growth hormone therapy can positively impact final height outcomes in RSS.
- The findings support the long-standing hypothesis that sufficient growth hormone availability can alter final height in IUGR children.
- Early intervention for spontaneous nocturnal hypoglycemia may enhance educational achievement.
Conclusions:
- Russell-Silver syndrome requires specific management strategies distinct from other forms of IUGR.
- Pharmacological doses of human growth hormone show promise for improving final height in RSS.
- Addressing nocturnal hypoglycemia is crucial for optimizing developmental outcomes in children with RSS.
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.