Gradually increasing ethinyl estradiol for Turner syndrome may produce good final height but not ideal BMD

Hasegawa, Yukihiro; Ariyasu, Daisuke; Izawa, Masako; Igaki-Miyamoto, Junko; Fukuma, Mami; Hatano, Megumi; Yagi, Hiroko; Goto, Masahiro

doi:10.1507/endocrj.ej16-0170

Cited by 17 publications

(29 citation statements)

References 24 publications

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“…Their concept is physiologically sound, and in our study, we mainly followed their protocol while lowering the dosage even further. Although low-dose estrogen therapy, hereafter referred to as "classical therapy", sometimes reportedly causes breast development in pediatric patients with TS at the initial dose (1, 2) of 25 ng/kg/d of EE2, the ultra-low-dose regimen of 1-2 ng/kg/d of EE2 used in our study did not demonstrate similar results (3). Therefore, the dosages used in classical and our ultra-low-dose therapy may simulate the estrogen levels responsible for the early-pubertal effects of the hormone, including breast development on one hand and pre-pubertal effects, including bone maturation and growth acceleration, on the other, respectively.…”

Section: ) What Is the Background Of This New Therapy?contrasting

confidence: 60%

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Ultra-low-dose estrogen therapy for female hypogonadism

Hasegawa

Itonaga

Ikegawa

et al. 2020

Clin Pediatr Endocrinol

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In females, endogenous estrogen secretion increases gradually before pubertal development. The benefits of low-dose estrogen therapy in patients with Turner syndrome were originally discussed by Ross et al. and Quigley et al. These seminal studies used ethinyl estradiol (EE2), starting at a dose of 25 ng/kg/d. We hypothesized that the initial dosage of estrogen could be titrated to more closely mimic physiological increments of endogenous estrogen. Therefore, our recent study initiated EE2 treatment at a dosage of 1-2 ng/kg/d, an ultra-low-dose estrogen therapy in pediatric patients with Turner syndrome. The ultra-low-dose estrogen therapy in this syndrome produced a good final height outcome but achieved suboptimal bone mineral density (BMD). In the present review, we have explained our findings to clarify the merits and demerits of this new therapy and to promote further discussion and research. This type of ultra-low-dose estrogen therapy, initiated at an early age, could be ideal for estrogen replacement in female patients with hypogonadism, such as Turner syndrome.

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Section: ) What Is the Background Of This New Therapy?contrasting

confidence: 60%

“…2) What have we observed in our study of this therapy? (1) The main results of our recent study (2) The strengths of our study (3) The weaknesses of our study 3) How can the findings be applied in clinical practice? 4) What are the future directions in research on this therapy?…”

Section: Introductionmentioning

confidence: 98%

Ultra-low-dose estrogen therapy for female hypogonadism

Hasegawa

Itonaga

Ikegawa

et al. 2020

Clin Pediatr Endocrinol

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“…In addition, bone density reduction was noted even in cases where estrogen therapy, in addition to hGH, has been administered, to induce the development of secondary sex characteristics at relatively young ages. These results suggest that the application of hGH and estrogen therapies starting from early childhood might improve bone mass and density to some extent, but cannot achieve normal bone density in a reliable manner [38][39][40][41]. Relating to this, many authors are speculating a presence of endogenous bone defects in TS patients [1,38,40,42].…”

Section: Discussionmentioning

confidence: 99%

Prevalence of diverse complications and its association with karyotypes in Japanese adult women with Turner syndrome—a questionnaire survey by the Foundation for Growth Science—

et al. 2018

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The reported prevalence of complications in Turner Syndrome (TS) was highly variable because of the rarity and the limited numbers analyzed. Again, possible presence of other complications that are not described as specific for TS, is also speculated. To resolve these issues, a questionnaire survey was conducted in hGH treated 492 patients with adult TS (17-42 years). The possible association with these complications and karyotypes were also analyzed. The complications and their prevalence were as follows: chronic thyroiditis (25.2%), inflammatory bowel disease (1.8%), congenital cardiovascular anomaly (11.8%), urinary tract malformation (11.8%), low bone mineral density (BMD) (42.9%), scoliosis (8.4%), hearing loss (6.2%), epilepsy (2.8%) and schizophrenia (0.9%). The majority of prevalence of these diseases in TS was higher than in the general population. In distribution, the most frequent karyotype was 45,X monosomy (28.9%), followed by 45,X/46,X,Xi (16.9%), 46,X,Xi (9.1%), and 45,X/46,XX (6.3%), while other mosaic 45,X was noted in 29.9%. Regarding the karyotype, cardiovascular anomaly was more frequent in the 45,X group and less in the 46,X,Xi group. Urinary tract malformation and epilepsy were frequently associated with the chromosome 45,X. The prevalence of low BMD was noticed more in the chromosome 46,X,Xi and 45,X/46,X,Xi, and less in other mosaic 45,X. In conclusion, the more exact prevalence of diverse complications was clarified and it exceeded the prevalence of the majority of complications in general population. As novel findings, it was observed that the prevalence of epilepsy was significantly high, and epilepsy and low BMD were frequently associated with the specific karyotypes.

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“…This is supported by another study where initiating low-dose oestrogen therapy as early as 5 years of age as opposed to the recommended 12 years, may further increase final height by 2.1cm on top of improving cognitive and hepatic functions [2,4]. Apart from using oestrogen at low doses, transdermal or depot forms are also alternatives should growth hormone therapy interferences, suboptimal final height and thrombophilia be of concern [2,9].…”

Section: Discussionmentioning

confidence: 89%

“…Theoretically, with pubertal induction, bone matures sooner hence the final height may be reduced. However, a recent Japanese study has shown that by starting at 1% of adult dose (ultra-low dose) of oestrogen and gradually titrating it up over the years does not interfere with growth but at the expense of an ideal bone mineral density [9]. This is supported by another study where initiating low-dose oestrogen therapy as early as 5 years of age as opposed to the recommended 12 years, may further increase final height by 2.1cm on top of improving cognitive and hepatic functions [2,4].…”

Section: Discussionmentioning

confidence: 99%

Late Presentation of Turner Syndrome and Its Complications

Sani¹,

Zulkufli²

2018

JCHS

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Turner syndrome is one of the most common sex chromosome abnormalities with an estimated true prevalence of 1 in 2,000 in newborns. This case report is of a girl who presented to the adult endocrinologist at 16 years of age and subsequently diagnosed with Turner syndrome. Despite frequenting clinics for unrelated ailments, her short stature was overlooked hence not investigated for a causative pathology. The aim of this report is to explore the diagnostics of Turner syndrome, hormone treatments available and the importance of starting treatment early. KEYWORDS: Turner syndrome, short stature, primary amenorrhoea, osteoporosis

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Gradually increasing ethinyl estradiol for Turner syndrome may produce good final height but not ideal BMD

Cited by 17 publications

References 24 publications

Ultra-low-dose estrogen therapy for female hypogonadism

Ultra-low-dose estrogen therapy for female hypogonadism

Prevalence of diverse complications and its association with karyotypes in Japanese adult women with Turner syndrome—a questionnaire survey by the Foundation for Growth Science—

Late Presentation of Turner Syndrome and Its Complications

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