Estrogen treatment of excessively tall girls with Marfan syndrome.
Five girls with Marfan syndrome were treated with estrogens for reduction of their final heights. Treatment was started at a chronological age of 12.0 +/- 0.5 years and height of 173.0 +/- 3.1 cm (means +/- SEM). Skeletal age was 12.4 +/- 0.2 years and Bayley-Pinneau final height prediction 186.3 +/- 1.4 cm. Estrogen treatment for 2.0 +/- 0.4 years resulted in final height of 184.1 +/- 0.2 cm (p less than 0.05). The weight increased with 7.8 +/- 2.7 kg during the treatment period. The present results indicate that estrogen treatment may be effective in reducing adult height in girls with Marfan syndrome, but that the effect is probably less than that observed in normally tall girls. This may probably be explained by the rather late start of treatment. No immediate effects on aortic root diameters were observed. Estrogen treatment in Marfan syndrome is discussed with regard to beneficial effects, possible side-effects and optimal age for starting treatment.
Knudtzon
J
J
Department of Pediatrics, University of Bergen, Norway.
Aarskog
D
D
D001827:Body Height / Q000187:drug effects*; D002648:Child; D004452:Echocardiography; D004958:Estradiol / Q000031:analogs & derivatives* / Q000627:therapeutic use; D004997:Ethinyl Estradiol / Q000627:therapeutic use*; D005260:Female; D006801:Humans; D008382:Marfan Syndrome / Q000175:diagnosis / Q000188:drug therapy*; D009640:Norethindrone / Q000627:therapeutic use
D004997:Ethinyl Estradiol; D004958:Estradiol; D009640:Norethindrone