Peer-reviewed article
Early Growth Hormone Treatment Enhances Growth and Nutritional Status in Silver-Russell Syndrome.
Eloïse Giabicani, Raphaëlle Billette de Villemeur, Mélodie Acher, Manon Cochet, Amélie Perrière et al. ·
Summary
Written by AI, not yet AICS's view · claude-haiku-4-5-20251001This study examined how growth hormone treatment affects children with Silver-Russell syndrome, a rare condition causing severe growth problems and feeding difficulties. Researchers tracked 77 children over their first two years of treatment and found that growth hormone therapy improved both their height and weight-for-height measurements. Children who started treatment before age 4 showed better height gains than those who started later. The nutritional status of treated children improved significantly, with fewer children becoming severely undernourished.
Key points
- After 2 years of growth hormone therapy, height increased by an average of 1.3 standard deviation scores
- Weight-for-height (nutritional status) improved from 81% to 86% after 2 years of treatment (P < .001)
- The proportion of children with severe malnutrition (weight-for-height below 75%) fell from 22.1% to 7.8% after 2 years
- Starting treatment before age 4 years resulted in greater height gains (1.5 vs 1.1 SDS after 2 years, P = .012)
- Mean age at treatment initiation was 3.7 years (range 1.4-10.3 years)
Limitations
This is a retrospective study from a single center with no comparison group of untreated children, so the results cannot be compared against the natural course of the condition. The study only measures outcomes during the first 2 years of treatment and does not address longer-term effects or safety.
Abstract
CONTEXT: Silver-Russell syndrome (SRS) is an imprinting disorder characterized by severe intrauterine and postnatal growth retardation, feeding difficulties, and risk of hypoglycemia. Recombinant growth hormone (rGH) therapy has shown its positive effect on adult height in SRS. OBJECTIVE: We aimed to assess the short-term effects of rGH therapy on the growth and nutritional status of these children. METHODS: Retrospective monocentric data of growth and nutritional characteristics of 77 prepubertal children with molecularly proven SRS during the first 2 years of rGH therapy were analyzed. RESULTS: The mean age at the initiation of rGH therapy was 3.7 (1.4-10.3) years. The mean height gain was 0.8 standard deviation score (SDS) after 1 year of treatment and 1.3 SDS after 2 years. The ideal weight for height (WfH), reflecting the nutritional status, increased from a mean of 81% at rGH initiation, to 84% after 1 year of treatment (P < .001) and 86% after 2 years (P < .001). The proportion of SRS children below a WfH of 75% decreased from 22.1% at rGH therapy initiation to 7.8% after 2 years of treatment. Starting rGH therapy before the age of 4 years was associated with a greater increase in height after 2 years, 1.5 vs 1.1 SDS, P = .012. CONCLUSION: In prepubertal SRS children, the first 2 years of standard-dose rGH therapy significantly enhance both height and nutritional status. Early initiation of treatment, before the age of 4 years, further optimizes height gain.
Findings the Hub has extracted
Each claim below is labelled by its type of design and links back to this report.
- Intervention
- Recombinant growth hormone (rGH) therapy at standard doses
- Population
- 77 prepubertal children with molecularly proven Silver-Russell syndrome
Key finding
Height gain of 0.8 SDS after 1 year and 1.3 SDS after 2 years of rGH therapy