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Adult height in individuals with Silver-Russell Syndrome treated with growth hormone from childhood-correlations to pubertal growth.

Conference contribution
Authors Jovanna Dahlgren
C. Velander
C. Ladaki
Published in 9th joint meeting of paediatric endocrionology, 19-22 september 2013, Milan, ESPE. Hormone Research in Paediatrics.
Volume 80
Issue suppl 1
Pages 302
ISSN 1663-2818
Publisher Horm Res in Ped
Place of publication Milan, Italy
Publication year 2013
Published at Institute of Clinical Sciences, Department of Pediatrics
Pages 302
Language en
Subject categories Clinical Medicine


Background: Silver-Russell syndrome (SRS) is characterized by intra- and extrauterine growth retardation. Growth hormone (GH) treatment has a positive outcome if started several years before puberty. Objective: To determine the effect of GH treatment in SRS on pre- and pubertal growth, as well as on adult height (AH). Methods: 20 prepubertal children (12 males) with SRS followed at a national centre at Queen Silvia Children’s Hospital, were treated with GH from a mean (SDS) age of 3.7 (1.1) years (age range between 2.0-7.0) until achieved AH (age ranged between 13-18 years). All were born SGA with a birth weight of 1800 (617) gram and birth length of 41 (5.5) cm. Ten children were born preterm (six due to maternal preeclampsia). Midparental height ranged from -1.85 to 1.6 SDS with mean maternal height -0.4 (0.9) SDS and paternal height -0.3 (1.0) SDS. GH dose ranged between 0.03-0.05 mg/kg/day. GnRH analogue treatment was given due to early puberty in 4 girls and 4 boys. GH treatment was stopped when velocity was < 1 cm/yr and AH was measured at 18 years of age. Results: Mean height SDS at start of treatment was -3.8 (1.4) and within two years a rapid catch-up was seen of 1.6 (1.4) SDS, giving a height SDS of -2.2 (1.4), P< 0.01. Height at start of puberty was -1.2 (1.4) SDS, P < 0.01 and the total prepubertal height gain was 2.6 (1.4) SDS. During the puberty, an earlier deceleration of growth was seen compared to the normal population. When AH was reached, 6 males had heights above -1 SDS (mean -0.7 (0.5)), and the group of the other 6 males had a mean height of -1.9 (0.6) SDS and the female group -3.2 (1.6) SDS. Bone age development could not predict the early fusion of bone plate. Conclusion: Treatment with GH leads to a substantial height gain during prepubertal years, whereas pubertal years lead to a premature termination of growth spurt. Further investigation of the effect of sex steroid levels during pubertal years on bone maturation in SRS patients is needed.

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