Abstract
Patients with Marfan syndrome have disproportionate growth and often increased adult heights with progressive kyphoscoliosis. Five girls with Marfan syndrome were treated with estrogen in order to reduce their final heights. Treatment was started at a chronological age of 11.9 ± 0.5 years (mean ± S.E.M.) and bone age (Greulich-Pyle) of 12.4 ± 0,9 years, when their heights were 173.0 ± 3.1 cm (> 3 S.D.). Bayley-Pinneau and Tanner 1 height predictions were 186.2 ± 1.4 cm and 183.0 ± 1.2 cm, respectively. Estrogen treatment for 2.0 ± 0.4 years resulted in menarche at the age of 12.9 ± 0.4 years and heights at end of treatment of 179.4 ± 1.4 cm. Final heights were 180.6 ± 2.0 cm.. No side-effects were observed. The growth reductions for the two prediction methods for adult height represent 42% and 24% of the estimated growth potential before treatment. Although the methods for bone age assessment and prediction of adult height are based on data for healthy children, the present results indicate that estrogen treatment may be effective in reducing adult height in girls with Marfan syndrome. Potential beneficial effects on the development of scoliosis remain to be clarified.
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