Quantitative liver functions in Turner syndrome with and without hormone replacement therapy

Gravholt, Claus Højbjerg; Poulsen, Henrik E.; Ott, Peter; Christiansen, Jens Sandahl; Vilstrup, Hendrik

doi:10.1530/eje-07-0070

Cited by 49 publications

(35 citation statements)

References 45 publications

(43 reference statements)

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“…A limitation of the study was that other measurements of oestrogen action such as body composition, uterine length or bone metabolism were not recorded, although the effect of oral E 2 in these variables in TS girls had already been studied by different authors (14,26,34). The safety profile of the study is in agreement with the previous study results with oral or transdermal administration of E 2 (8,34,35,36). No adverse events detected were considered oestrogen related and treatment was safe and well tolerated.…”

Section: Discussionsupporting

confidence: 69%

Individualised vs fixed dose of oral 17β-oestradiol for induction of puberty in girls with Turner syndrome: an open-randomised parallel trial

Labarta

Moreno

López-Siguero

et al. 2012

European Journal of Endocrinology

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Context: Oestrogen induction of pubertal changes in Turner girls may reinforce their psychological well-being and may also optimise final height; however, oestrogen type, dose, and route are not well established. Objective: To induce normal pubertal development in Turner girls and ovarian insufficiency with oral 17b-oestradiol (E 2 ), either as individualised dose (ID) or as fixed dose (FD), and to determine whether growth is affected. Design: Open-label randomised, parallel groups, multicentre clinical trial in 48 GH-treated Turner girls. Oral E 2 was given in tablets, either as an ID of 5-15 mg/kg per day during 2 years or as a FD of 0.2 mg daily during the first year followed by 0.5 mg daily during the second year. Main outcome measures were the event of attaining a Tanner breast staging R4 (primary), FSH, and auxological variables (secondary). Results: Shorter median time to Tanner staging R B4 in the FD group (733 days) compared with the ID group (818 days) (PZ0.046). Higher proportion of girls with Tanner staging R B4 (65%) in the FD group compared with the ID group (42%) (PZ0.068). Bone age did not show inadequate acceleration and adult height prediction was maintained in both groups. No oestrogen-related adverse events were reported. Conclusions: Two-year treatment with oral E 2 can progressively induce normal pubertal development in Turner syndrome. Low-dose oral E 2 given as a FD produces a satisfactory pubertal development not inferior to ID. Treatment was well tolerated and did not interfere with the growth-promoting effect of GH.

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Section: Discussionsupporting

confidence: 69%

Individualised vs fixed dose of oral 17β-oestradiol for induction of puberty in girls with Turner syndrome: an open-randomised parallel trial

Labarta

Moreno

López-Siguero

et al. 2012

European Journal of Endocrinology

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“…Out of the 54 TS women in the present study, only two, by choice, did not receive estrogen treatment at the time of examination, but the calculated median estrogen insufficiency in years at follow-up was still 6 (36). All markers of bone formation were unchanged during follow-up, while we observed a rather pronounced decrease in the resorption marker, NTX/ creatinine ratio, and ICTP, which correlated closely and negatively to the observed changes in BMD, indicating that the patients with the most pronounced slowing of bone resorption had the greatest increase in BMD during the 5.9-year period of observation.…”

Section: Discussionmentioning

confidence: 66%

Long-term hormone replacement therapy preserves bone mineral density in Turner syndrome

Cleemann

Hjerrild

Lauridsen

et al. 2009

European Journal of Endocrinology

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Context: Reduced bone mineral density (BMD) and increased risk of fractures are present in many women with Turner syndrome (TS). Objective: Examine longitudinal changes in BMD in TS and relate changes to biochemical parameters. Design: Prospective, pragmatic, and observational study. Examinations at baseline and follow-up (5.9G0.7 years). Setting: Tertiary hospital. Participants: Fifty-four women with TS (43.0G9.95 years). Interventions: Hormone replacement therapy (HRT) and calcium and vitamin D supplementation. Main outcome measures: BMD (g/cm2 ) measured at lumbar spine, hip, and the non-dominant forearm. Bone formation and resorption markers, sex hormones, IGF1, and maximal oxygen uptake. Results: At follow-up, forearm BMD, radius ultradistal BMD, and hip BMD remained unchanged, radius 1/3 BMD declined (0.601G0.059 vs 0.592G0.059, PZ0.03), while spine BMD increased (0.972G0.139 vs 1.010G0.144, P!0.0005). Bone formation markers did not change over time in TS. Bone resorption markers decreased over time in TS. Testosterone, IGF1, and maximal oxygen uptake was significantly reduced in TS. Conclusion: Longitudinal changes in BMD in TS were slight. BMD can be maintained at most sites in well-informed women with TS, being encouraged to maintain a healthy lifestyle, including HRT and intake of calcium and vitamin D.

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“…Liver dysfunction in TS is likely to be a form of hepatic steatosis without functional alterations in hepatocytes, which is normalised by HRT, therefore it can be considered a state induced by the lack of estrogens [12]. In our case, liver enzymes were normalised during the second trimester of pregnancy and were again increased after delivery, before the initiation of HRT.…”

Section: Discussionmentioning

confidence: 55%

Feto-maternal risks associated with pregnancy achieved through oocyte donation in a woman with Turner syndrome

Georgopoulos¹,

Adonakis²,

Papadopoulos

et al. 2009

Gynecological Endocrinology

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We report a case of a 40-year-old primipara Caucasian woman with Turner syndrome (TS) and Hashimoto thyroiditis who had underwent a successful IVF-ET cycle with oocyte donation and single embryo transfer. All pregnancies in women with TS are considered as high risk, with cardiovascular complications being the most dangerous. Our case represents a typical case of fetal growth restriction with gradual slowing down of fetal growth after 28 weeks. At 37 + 3 gestational weeks, a healthy male newborn weighing 2240 g, with artery pH of 7.32 was delivered by cesarean section. The neonate was small for gestational age. Women with TS who become pregnant need close surveillance from a multidisciplinary team of cardiologists, obstetricians and endocrinologists. The primary goal is to prevent maternal complications and to improve perinatal outcome. In doing so, a thorough evaluation of fetal growth and uteroplacental and fetal circulation should by no means be omitted, after 26-28 weeks of gestation. The examinations should be at monthly or even shorter intervals to find early signs of growth restriction and act accordingly.

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Quantitative liver functions in Turner syndrome with and without hormone replacement therapy

Cited by 49 publications

References 45 publications

Individualised vs fixed dose of oral 17β-oestradiol for induction of puberty in girls with Turner syndrome: an open-randomised parallel trial

Individualised vs fixed dose of oral 17β-oestradiol for induction of puberty in girls with Turner syndrome: an open-randomised parallel trial

Long-term hormone replacement therapy preserves bone mineral density in Turner syndrome

Feto-maternal risks associated with pregnancy achieved through oocyte donation in a woman with Turner syndrome

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