Growth and symptoms in Silver-Russell syndrome: review on the basis of 386 patients.

UNLABELLED

The spontaneous growth of 386 patients (163 girls and 223 boys) with Silver-Russell syndrome (SRS) was analysed in a mixed longitudinal and cross-sectional manner. One hundred and twenty patients were seen in the two centres between 1970 and 1993, additional definite cases were added from the literature. Mean (+/- SD) length of full-term babies with SRS at birth was 43.1 +/- 3.7 cm (n = 102) in both sexes. Mean weight at birth was 1940 +/- 353 g in boys and 1897 +/- 325 g in girls. During the first 3 years of life there was poor growth with a further loss in height. Between ages 4 and 10 years there was constant growth in parallel to the 3rd percentile with a mean height SDS of -4.3. The pubertal growth spurt was reduced in the whole group. Bone age development paralleled growth, retardation increased during the first years, remained constant during prepubertal time and caught up in early puberty. Mean adult height was 151.2 +/- 7.8 cm in males and 139.9 +/- 9.0 cm in females. Head circumference for age was in the lower normal range (mean SDS for 156 prepubertal boys -1.8; mean SDS for 97 prepubertal girls -2.2).

CONCLUSION

Normative data on spontaneous growth of children with Silver-Russell syndrome are described, allowing a better counselling of patients as well as the judgement of the effects of growth promoting therapies.