Adult Height in Patients with Turner Syndrome and Association with Lifestyle-related Diseases after Human Growth Hormone Treatment in Japan.

PEDIATRIC ENDOCRINOLOGY REVIEWS PER(2017)

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摘要
This study investigated the factors associated with adult height in Japanese women with Turner syndrome (TS) with and without human growth hormone (hGH) treatment, and the association between obesity and lifestyle-related diseases in TS based on current findings. Adult height in women with untreated TS increased from 139.1 cm to 141.3 cm between 1992 and 2008 in a secular trend. The adult height of women with untreated TS was not significantly different between those with the 45,X and non-45,X karyotypes. The adult height after (low-dose hGH treatment (0.5 IU/kg/week) was significantly lower in women with TS with spontaneous menstruation (mean, 141.9 cm) than in those women without spontaneous menstruation (mean, 145.5 cm). A recent survey reported that the mean adult height in women with TS after high dose hGH treatment (0.35 mg/kg/week) was 149.1 cm. In addition, adult height improved with low-dose estrogen treatment started at age 12-15 years, reaching a height of 140 cm. According to the questionnaire survey administered to women with TS, conducted by the Foundation for Growth Science, the prevalence of obesity (body mass index (BMI) >= 25 kg/m(2)] in women with TS aged 15-39 years (24.7%) was significantly higher than that in the general female population in the same age range (9.4%). The prevalence of complications in women with TS was 5.5% for diabetes mellitus (DM), 7.7% for hypertension, 18.9% for dyslipidemia, and 11.5% for liver dysfunction, all of which were higher than the prevalence in the general female population. BMI was higher in women with TS who were diagnosed with lifestyle-related diseases than in those without lifestyle-related diseases, and the rate of each disease increased with an increase in BMI. In addition, those with multiple complications had substantially higher BMI. These findings indicate that obesity is strongly associated with the development of lifestyle-related diseases. In addition, women with TS who had any one of the complications (except for DM) had significantly lower birth weight compared with those without complications. This is consistent with the fetal programming hypothesis, which proposes that low birth weight may be associated with obese after birth and lifestyle-related diseases in adulthood. The adult height in untreated women with TS increased by 2.2 cm over 16 years. However, adult height in women with TS treated with hGH can be further increased with early high-dose hGH treatment and low dose estrogen replacement therapy. In addition, the rate of lifestyle-related diseases among women with TS was closely associated with their weight gain. In particular, those with a BMI of >= 25 kg/m(2) or higher had a higher rate of lifestyle-related diseases. These findings offer important considerations for clinical treatment of women with TS.
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关键词
Turner syndrome,GH treatment,Adult height,Lifestyle-related disease,Obesity,DM,Hypertension
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