ESPE2023 Poster Category 2 Growth and Syndromes (32 abstracts)
Height control using estradiol valerate considering chronological and bone age in patient with Marfan Syndrome
Insung Kim 1 , JiHoon Hwang 1 , Juyoung Sung 1 , Sung Eun Kim 2 , Dong-Yun Lee 2 , Min-Sun Kim 1 , Sung Yoon Cho 1 , Dong-Kyu Jin 1 & Doo Seok Choi 2
1Department of Pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea, Republic of. 2Department of Obsterics and Gynecology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea, Republic of
Introduction: Estrogen treatment can be used for pediatric patients with Marfan syndrome who wish to control the rate of excessive height growth. However, the appropriate timing of treatment initiation is controversial and studies were limited. In this study, the authors aimed to find out when the initiation of estradiol therapy is most appropriate for controlling height growth rate in patients with Marfan syndrome.
Methods: This study was a retrospective study targeting patients who started estradiol treatment for height control between 2000 and 2020 and completed the treatment within this period. Before the start of treatment, the expected final height was first calculated through the growth curve and bone age suitable for the patient with Marfan syndrome. After treatment with estradiol valerate, the height at the end of treatment was compared with the height predicted before the start of treatment. The difference between actual height and predicted height was analyzed according to the time fo treatment initiation.
Results: There was a significant difference between predicted height according to chronological age and bone age before treatment and actual height after treatment, based on the age of 10.5 years at the start of treatment. When treatment was started before the age of 10.5 years, the actual height was 10.6 cm (interquartile range [IQR] 10.2, 13.5) smaller than predicted by the growth curve and 10.1 cm (IQR 7.31, 11.42) smaller than predicted by bone age. On the other hand, when treatment was started after 10.5 years of age, the difference between the final height and the height predicted through the growth curve was 0.6 cm (IQR -3.65, 5.85), and the difference between the height predicted through bone age was 3.83 (IQR 0.84, 6.4).
Conclusion: When a pediatric female patient with Marfan's syndrome wants to adjust her height, treatment with estradiol should be performed before reaching the age of 10.5 to expect a great effect.
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