J Korean Med Sci.  2016 Feb;31(2):275-279. 10.3346/jkms.2016.31.2.275.

Estrogen-mediated Height Control in Girls with Marfan Syndrome

• Lee DY
• Hyun HS
• Huh R
• Jin DK
• Kim DK
• Yoon BK
• Choi D

• KMID: 2360053
• DOI: http://doi.org/10.3346/jkms.2016.31.2.275

Abstract

This study evaluated the efficacy of a stepwise regimen of estradiol valerate for height control in girls with Marfan syndrome. Eight girls with Marfan syndrome who had completed estrogen treatment for height control were included. Estradiol valerate was started at a dose of 2 mg/day, and then was increased. The projected final height was estimated using the initial height percentile (on a disease-specific growth curve for Korean Marfan syndrome [gcPFHt]), and the initial bone age (baPFHt). After the estrogen treatment, the projected final height was compared to the actual final height (FHt). The median baseline chronological and bone age were 10.0 and 10.5 years, respectively. After a median of 36.5 months of treatment, the median FHt (172.6 cm) was shorter than the median gcPFHt (181.0 cm) and baPFHt (175.9 cm). In the six patients who started treatment before the age of 11 years, the median FHt (171.8 cm) was shorter than the median gcPFHt (181.5 cm) and baPFHt (177.4 cm) after treatment. The median differences between the FHt and gcPFHt and baPFHt were 9.2 and 8.3 cm, respectively. In two patients started treatment after the age of 11, the differences between FHt and gcPFHt, and baPFHt after treatment were -4 and 1.4 cm, and -1.2 and 0 cm for each case, respectively. A stepwise increasing regimen of estradiol valerate may be an effective treatment for height control in girls with Marfan syndrome, especially when started under 11 years old.

Keyword

Marfan Syndrome; Tall Stature; Estradiol Valerate; Height Control

MeSH Terms

Body Height
Child
Contraceptive Agents/*therapeutic use
Estradiol/*analogs & derivatives/therapeutic use
Female
Growth Disorders/pathology
Humans
Marfan Syndrome/diagnosis/*drug therapy
Treatment Outcome
Contraceptive Agents
Estradiol

Figure

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  Fig. 1 Schematic treatment schedule. E2V, estradiol valerate.

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  Fig. 2 Percentile change on Korean Marfan syndrome-specific growth curve during and after estrogen treatment.

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