We have analysed growth and the major clinical manifestations of 144 patients (89 males, 55 females) with Noonan syndrome from two West German centres. Size at birth was normal in both sexes. In both males and females, the mean height followed along the 3rd per centile until puberty, but decreased transiently due to an approximately 2 year delay in onset of puberty. Final height approaches the lower limits of normal at the end of the 2nd decade of life. The mean adult height was found to be (n = 20) 162.5 cm in males and (n = 13) 152.7 cm in females, respectively. Smoothed means and standard deviations for height were derived. These data may be used for the statistical evaluation of height of Noonan syndrome patients. Except for mental retardation and microcephaly, which are more frequent in males, the relative frequencies of minor anomalies and malformations were found to be similar in both sexes. The characteristic non-cyanotic heart defects in the Noonan syndrome do not appear to have a major influence on growth. The auxological data were compared with those in the Ullrich-Turner syndrome.

's ~o s p i t a l , Tubingen, 131 FRG Centro di Endocrinologia Infantile e dell'Adolescenza, 134 Clinlca Pediatrica 3a,UniversltB degli Studl, lstlt. SOMATOMEDINS AND GROWTH HORMONE (GH) SECRETION IN ULLRICH-TURNER SYNDROME (UTS) In recent time attempts are made to treat short stature in UTS with GH. What evidence exists for disturbance of the GH-somatomedin axis causing the growth disorder in UTS ?-In a total of 56 children and adolescents with UTS (45,X;N=45) the following parameters were measured in a cross-sectional manner: somatomedin activity (Sm; porc.cart.