GROWTH AND SYMPTOMS IN SILVER-RUSSELL-SYNDROME - REVIEW ON THE BASIS OF 386 PATIENTS

The spontaneous growth of 386 patients (163 girls and 223 boys) with S ilver-Russell syndrome (SRS) was analysed in a mixed longitudinal and cross-sectional manner. One hundred and twenty patients were seen in t he two centres between 1970 and 1993, additional definite cases were a dded from the literature. Mean (+/- SD) length of full-term babies wit h SRS at birth was 43.1 +/- 3.7 cm (n = 102) in both sexes. Mean weigh t at birth was 1940 +/- 353 g in boys and 1897 +/- 325 g in girls. Dur ing the first 3 years of life there was poor growth with a further los s 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 pub ertal growth spurt was reduced in the whole group. Bone age developmen t paralleled growth, retardation increased during the first years, rem ained 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 gi rls -2.2).