HORMONE-TREATMENT OF GIRLS WITH TURNER-SYNDROME - THE NATIONAL COOPERATIVE GROWTH STUDY EXPERIENCE

Objective. To evaluate growth rate and adult height with recombinant g rowth hormone (GH) treatment in girls with Turner syndrome (TS) and pr edictors of their growth response. Methods. Data on girls with TS who were treated with GH In the National Cooperative Growth Study (NCGS) w ere evaluated. As of January 1997, there were 2798 girls with TS in th e NCGS database, 2652 of whom had not previously received GH. Follow-u p data on growth were available for 2475 subjects, and data on adult h eight were available for 622. Results. The average age of girls with T S at enrollment in the NCGS was 10.1 +/- 3.6 years. These patients had severely short stature compared with that of unaffected American girl s (height, 118.5 +/- 16.5 cm; height standard deviation score [SDS], - 3.1 +/- 0.9), but their heights were typical of those of American girl s with TS (TS-specific height SDS, 0.01 +/- 0.9). Treatment with GH fo r an average duration of 3.2 +/- 2.0 years resulted in an increase in height SDS of 0.8 +/- 0.7 compared with unaffected girls and of 1.2 +/ - 0.8 compared with TS standards. Growth rates increased from 4.0 +/- 2.3 cm/year before treatment to 7.5 +/- 2.0 cm/year after 1 year of tr eatment. Duration of treatment with GH was the strongest predictor of change in height SDS. After 6 to 7 years of treatment with GH, there w as a cumulative change of 2.0 in mean height SDS. The 622 girls who re ached adult height were older when they began taking GH. Their mean he ight gain over pre-GH projected height was 6.4 +/- 4.9 cm after 3.7 +/ - 1.9 years of treatment. Their adult height was 148.3 +/- 5.6 cm. Con clusions. Although the response to treatment with GH varied, it was as sociated with highly significant gains in growth and adult height in g irls with TS. Duration of treatment with GH was the most important var iable predicting adult height.