GROWTH-HORMONE TREATMENT IN NOONAN-SYNDROME - THE NATIONAL COOPERATIVE GROWTH STUDY EXPERIENCE

We evaluated the response to growth hormone (GH) therapy in 150 childr en (97 boys) with Noonan syndrome (NS) by analyzing growth data from c hildren with NS who were enrolled in the National Cooperative Growth S tudy and compared those data with National Cooperative Growth Study gr owth data from children with idiopathic growth hormone deficiency (IGH D) and Turner syndrome (TS), Children with NS were significantly short er than those with IGHD and TS. The annualized growth rates for years 1, 2, 3, and 4 of therapy in patients with NS who were naive to previo us GH therapy were significantly greater than baseline, Their growth r ates for years 1, 2, 3, and 4 were intermediate between those in child ren with IGHD and TS and were significantly different from both, A sig nificant improvement occurred in height SD scores for those 42 childre n with NS who have been monitored for at least 4 years of GH therapy, Three of six boys with NS for whom adult height data were available ex ceeded their pretreatment predicted heights.