REFERENCE VALUES FOR HEIGHT AND WEIGHT IN PRADER-WILLI-SYNDROME BASEDAN 315 PATIENTS

The spontaneous growth of 315 patients (109 girls and 208 boys) with P rader-Willi syndrome (PWS) was analysed in a mixed longitudinal and cr oss-sectional manner. 33 patients were seen in the department between 1970 and 1994; height and weight of 76 patients from Germany were eval uated by means of a questionnaire with detailed measuring instructions , and 206 definite cases were added from the literature. Mean (+/- SD) length of newborn babies with PWS was 50.2 +/- 2.8 cm (145 boys) and 48.9 +/- 3.3 cm (79 girls). Mean weight at birth was 2945 +/- 570 g in boys and 2782 +/- 594 g in girls. During the Ist year, the children's growth was nearly normal, thereafter short stature was present in app roximately 50% of PWS patients. Between 3 and 13 years of age, the 50t h percentile for height in PWS is roughly identical with the 3rd perce ntile in healthy controls. Body weight was normal for all boys and gir ls during the first 3 years. Thereafter, a rapid weight gain occurred; after an age of 10 years weight-for-height index in nearly all patien ts exceeded the normal range. The extent of pubertal growth was reduce d for the group. Mean adult height was 161.6 +/- 8.1 cm (23 males) and 150.2 +/- 5.5 cm (21 females). Head circumference for age was normal for boys and girls.