Choosing an oestrogen replacement therapy in young adult women with Turner syndrome*

Guttmann, Hadassah; Weiner, Zeev; Nikolski, Eugenia; Ish‐Shalom, Sophia; Itskovitz‐Eldor, Joseph; Aviram, Michael; Reisner, Shimon A.; Hochberg, Zèev

doi:10.1046/j.1365-2265.2001.01181.x

Cited by 70 publications

(81 citation statements)

References 18 publications

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“…On the other hand, that such lesions are associated with functional deficits cannot be excluded from our data. We saw that a short 2 month course without HRT lead to elevation of liver enzymes, readily suppressible by standard HRT, as also documented previously (3)(4)(5). Recently, the same response to HRT, albeit less pronounced, has also been found among postmenopausal women (27).…”

Section: Discussionsupporting

confidence: 58%

“…We and others have shown a normalizing effect of hormone replacement therapy (HRT), containing 17b-estradiol and a gestagen, on liver enzymes (3)(4)(5), which may point towards a protective effect on hepatocyte integrity. Marked architectural changes, including nodular regenerative hyperplasia, multiple focal nodular hyperplasia and cirrhosis are observed in some patients and are associated with a risk of liver-related complications.…”

Section: Introductionmentioning

confidence: 99%

“…Stature is short, and morbidity is increased due to risk of osteoporosis and fractures, type 2 diabetes, ischemic heart disease, hypertension, and stroke, but also the risk of cirrhosis is increased (1). Clinical studies have shown a frequent occurrence of elevated liver enzymes, primarily alanine aminotransferase (ALAT), g-glutamyl-transferase (gGT), and alkaline phosphatase (AP), while bilirubin is normal (2)(3)(4)(5).…”

Section: Introductionmentioning

confidence: 99%

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

et al. 2007

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Background: Studies have documented elevated levels of liver enzymes in many females with Turner syndrome (TS). Histology has shown a range of changes. Treatment with female hormone replacement therapy (HRT) reduces liver enzymes. Aim: To study quantitative liver functions in TS in detail with and without HRT. Design: Randomized crossover study with active treatment (HRT in TS and P-pill in controls) or no treatment. Subjects: Women with TS (nZ8, age 29.7G5.6 (meanGS.D.) years), verified by karyotype, and agematched controls (C; nZ8, age 27.3G4.9 years). Methods: We determined liver enzymes in blood, used the galactose elimination capacity to assess hepatocyte cytosol activity, plasma clearance of indocyanine green to assess excretory function, antipyrine clearance to estimate microsomal activity, and the functional hepatic nitrogen clearance (FHNC) to assess mitochondrial-cytosolic metabolic capacity for conversion of amino-nitrogen. Results: Liver enzymes were elevated in untreated TS and reduced by HRT. The hepatic capacities for conversion of galactose, indocyanine green, and antipyrine were normal and did not change by HRT.

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Section: Discussionsupporting

confidence: 58%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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

et al. 2007

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“…Additionally, we included one randomized study that compared low-dose estrogen replacement therapy with high-dose therapy, one study on the effect of timing of administration (morning or evening administration) and one randomized study on the difference between fixed and individualized dose of estrogen therapy (177,178,179).…”

Section: Preparationmentioning

confidence: 99%

Clinical practice guidelines for the care of girls and women with Turner syndrome: proceedings from the 2016 Cincinnati International Turner Syndrome Meeting

Gravholt¹,

Andersen

Conway

et al. 2017

European Journal of Endocrinology

908

1,259

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Turner syndrome affects 25-50 per 100,000 females and can involve multiple organs through all stages of life, necessitating multidisciplinary approach to care. Previous guidelines have highlighted this, but numerous important advances have been noted recently. These advances cover all specialty fields involved in the care of girls and women with TS. This paper is based on an international effort that started with exploratory meetings in 2014 in both Europe and the USA, and culminated with a Consensus Meeting held in Cincinnati, Ohio, USA in July 2016. Prior to this meeting, five groups each addressed important areas in TS care: 1) diagnostic and genetic issues, 2) growth and development during childhood and adolescence, 3) congenital and acquired cardiovascular disease, 4) transition and adult care, and 5) other comorbidities and neurocognitive issues. These groups produced proposals for the present guidelines. Additionally, four pertinent questions were submitted for formal GRADE (Grading of Recommendations, Assessment, Development and Evaluation) evaluation with a separate systematic review of the literature. These four questions related to the efficacy and most optimal treatment of short stature, infertility, hypertension, and hormonal replacement therapy. The guidelines project was initiated by the European Society of Endocrinology and the Pediatric Endocrine Society, in collaboration with the European Society for Paediatric Endocrinology, the Endocrine Society, the European Society of Human Reproduction and Embryology, the American Heart Association, the Society for Endocrinology, and the European Society of Cardiology. The guideline has been formally endorsed by the European Society of Endocrinology, the Pediatric Endocrine Society, the European Society for Paediatric Endocrinology, the European Society of Human Reproduction and Embryology and Summary of recommendationsThe recommendations (R) are worded as recommend (strong recommendation) and suggest (weak recommendation). We formally graded only the evidence underlying recommendations for therapeutic choices. The quality of evidence behind the recommendations is classified as very low (⨁◯◯◯), low (⨁⨁◯◯), moderate (⨁⨁⨁◯) and strong (⨁⨁⨁⨁). See further section 'Summary of methods used for guideline development'. Diagnosis and geneticsR 1.1. We recommend considering a diagnosis of TS in phenotypic females with a karyotype containing one X chromosome and complete or partial absence of the second sex chromosome, associated with one or more typical clinical manifestations of TS (⨁⨁⨁⨁). R 1.2. We recommend against considering a diagnosis of TS in females with one X chromosome and a deletion distal to Xq24 on the other X chromosome, and in women over the age of 50 years with less than 5% 45,X mosaicism (⨁⨁◯◯). R 1.3. We suggest that the new general surveillance management guideline applies to TS patients with any karyotype (⨁⨁◯◯). R 1.4. We recommend to consider testing for Turner syndrome (TS) in a female with typical signs (Table 2) (⨁⨁⨁⨁). R 1.5. We ...

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“…Natural estrogens are preferable to synthetic estrogens because of incomplete metabolization and a greater risk of thromboembolism and arterial hypertension of the latter. In addition to oral 17b-estradiol tablets, transdermal patches are also applicable (47,48).…”

Section: Sex Steroidsmentioning

confidence: 99%

Inducing puberty

Delemarre

Felius

Waal

2008

European Journal of Endocrinology

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Puberty is the result of increasing pulsatile secretion of the hypothalamic gonadotropin releasing hormone (GnRH), which stimulates the release of gonadotropins and in turn gonadal activity.In general in females, development of secondary sex characteristics due to the activity of the gonadal axis, i.e., the growth of breasts, is the result of exposure to estrogens, while in boys testicular growth is dependent on gonadotropins and virilization on androgens.Hypogonadotropic hypogonadism is a rare disease. More common is the clinical picture of delayed puberty, often associated with a delay of growth and more often familial occurring. Especially, boys are referred because of the delay of growth and puberty. A short course (3-6 months) of androgens may help these boys to overcome the psychosocial repercussions, and during this period an increase in the velocity of height growth and some virilization will occur.Hypogonadotropic hypogonadism may present in a congenital form caused by developmental disorders, some of which are related to a genetic disorder, or secondary to hypothalamic-pituitary dysfunction due to, among others, a cerebral tumor.In hypogonadotropic hypogonadism puberty can be initiated by the use of pulsatile GnRH, gonadotropins, and sex steroids. Sex steroids will induce development of the secondary sex characteristics alone, while combined administration of gonadotropins and GnRH may induce gonadal development including fertility.European Journal of Endocrinology 159 S9-S15

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Choosing an oestrogen replacement therapy in young adult women with Turner syndrome*

Abstract: The risk factors embodied in hyperinsulinaemia and enhanced bone turnover which, ultimately, have consequences for TS morbidity, are minimized by HRT. In the short term, neither regimen is effective for bone turnover in adult women with Turner syndrome.

Cited by 70 publications

References 18 publications

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

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

Clinical practice guidelines for the care of girls and women with Turner syndrome: proceedings from the 2016 Cincinnati International Turner Syndrome Meeting

Inducing puberty

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