LONG-TERM TREATMENT WITH GROWTH-HORMONE IN NOONANS-SYNDROME

Five children (four boys, one girl) with Noonan's syndrome were treate d with growth hormone for a mean of 2.9 (range 1.8 to 4.6) years. Trea tment was commenced at a mean age of 3.9 (range 2.5 to 6.0) years. Aft er one year of treatment, height velocity SDS increased from a mean of -2.1 (range -4.1 to -0.3) to a mean of +3.1 (range +2.0 to +3.5). Sub sequently, height SDS increased significantly from -3.3 (range -4.2 to -2.2) to -2.4 (range -3.3 to -1.6). Although in all patients a marked decrease in height velocity was observed in the following years of tr eatment, compared to the first year, height SDS continued to improve i n comparison to pretreatment (mean height SDS in the third year for fo ur patients was -2.1) (range -3.1 to -1.4). In the four patients who h ad completed their second year of treatment, height SDS for bone age h ad increased from -1.8 (range -2.8 to -0.9) to -1.2 (range -1.6 to -0. 6).