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

• 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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MEDICIN OCH HÄLSOVETENSKAP  -- Klinisk medicin (hsv//swe)

MEDICAL AND HEALTH SCIENCES  -- Clinical Medicine (hsv//eng)

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