Claus H Gravholt

..A putative DHEA receptor in vascular endothelium has been described and in vitro studies have shown involvement of DHEA in NO dependent pathways...

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..Turner syndrome (TS) is characterized by growth retardation, hypogonadism and a high risk of cardiovascular complications and atherosclerosis; case reports suggest that thrombo-embolic complications may be present...

..Despite the lack of randomized controlled studies, we recommend testosterone treatment in case of increased LH or low serum testosterone, and weight reduction programmes if overweight...

..Recommendations for clinical practice are given, including life-long surveillance of cardiac function, aortic diameter and blood pressure...

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..Ovarian failure necessitates substitution with female hormone replacement therapy (HRT), and HRT induces favourable changes in body composition. It is unknown how HRT affects protein metabolism...

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..The peak glycerol increments after GH were higher in abdominal adipose tissue, perhaps due to a higher basal rate of lipolysis in this region...

..Body composition in Turner syndrome (TS) is altered with final height of TS decreased; anthropometry and bone mass distinctly changed...

..Women with Turner's syndrome have an increased risk of congenital cardiac malformations, ischaemic heart disease, hypertension and stroke. Aortic dissection seems to occur with increased frequency...

..Currently no firm guidelines for diagnosis exist. In conclusion, TS is a condition associated with a number of diseases and conditions that are reviewed in the present paper. Individuals with TS need life-long medical attention...

..Girls with Turner syndrome (TS) receive GH treatment during childhood, and in adolescence this treatment may be combined with oestradiol. We have studied the effects of this combined treatment on metabolism and body composition...

..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...

..In summary, Turner syndrome is a condition associated with a number of disease and conditions which are reviewed in present paper...

..We hypothesized that altered heart rate variability is present in TS in comparison with controls, and can be influenced by hormonal replacement therapy (HRT)...

..Since the risk of cardiovascular and endocrinological disease is clearly elevated, proper care during adulthood is crucial. Cognition and social functioning are altered in Turner syndrome...

..To investigate glucose homeostasis in detail in Turner syndrome (TS), where impaired glucose tolerance (IGT) and type 2 diabetes are frequent...

..Replacement therapy with female sex hormones lowered Lp(a), HDL cholesterol and apolipoprotein A-I...

..This study emphasizes the need for prospective unbiased studies...

..During sex steroid treatment, IGFBP-3 proteolysis normalized, without any change in free IGF-I...

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..To assess muscle fiber composition and capillary density in Turner syndrome, a condition linked with insulin resistance and increased frequency of type 2 diabetes, and link these findings with insulin sensitivity and physical fitness...

..Since general physicians only encounter these patients infrequently, it is recommended that the care and treatment of Turner's syndrome be centralized...

..Since general physicians encounter Turner patients infrequently, it is recommended that the care and treatment of Turner syndrome is centralised...

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..The aim of this study was to assess levels of inhibin A and B, FSH and LH in Turner's syndrome (TS) without signs of spontaneous ovarian activity...

..The role of cortisol in the regulation of lipolysis is not clear. This study was undertaken to explore whether a standard dose of prednisolone for 1 week would influence lipolysis in abdominal and femoral tissue...

..Most girls with Turner syndrome (TS) receive growth hormone (GH) treatment during childhood and adolescence, but controlled data on the effects on body composition and glucose metabolism are lacking...

..We analyzed differences between patients with untreated Klinefelter's syndrome and control subjects and subsequently analyzed differences between testosterone-treated and untreated Klinefelter's syndrome patients...

..Unfortunately, risk stratification is inadequate due to a lack of insight into the natural course of the syndrome-associated aortopathy. Therefore, this study aimed to prospectively assess aortic dimensions in TS...

..To investigate aortic dimensions in women with Turner syndrome (TS) in relation to aortic valve morphology, blood pressure, karyotype, and clinical characteristics...

..We investigated the effect of HRT on liver enzymes, lipid levels and measures of insulin sensitivity 26 in hypopituitary women...

..In this review, we will discuss the effects of estrogen and androgen insufficiency as well as the effects of sex HRT on morbidity and mortality with special emphasis on evidence based research and areas needing further studies...

..In summary, TS is a condition associated with a number of diseases and conditions which need the attention of a multi-disciplinary team during adulthood...

..Cortisol is an important catabolic hormone, but little is known about the metabolic effects of acute cortisol deficiency...

..These effects may contribute to the gender differences observed in the GH-IGF axis in healthy adults as well as in the responsiveness of hypopituitary patients to GH substitution...

..In summary, Turner syndrome is a condition associated with a number of diseases and conditions which are reviewed in the present paper...

..CONCLUSIONS: Short-term oral DHEA replacement in women with adrenal insufficiency was not associated with measurable changes in total or regional substrate metabolism...

..Morbidity and mortality from congenital and acquired cardiovascular (CV) disease is increased in Turner syndrome (TS), where traditional indices of CV risk are widely present but the single most common feature remains estrogen deficiency...

..Our current knowledge of morbidity in KS is based on observational studies and case reports and therefore is limited...

..Klinefelter syndrome (KS) may involve a number of abnormalities besides the characteristic testicular insufficiency. Some studies have suggested that thyroid abnormalities may be common, but this has not been clarified...

..Adrenal derived androgens are low in women with adrenal failure. The physiological consequences of substitution therapy are uncertain...

..01), 166 +/- 21(CTR) (P < 0.01)]. These results suggest that femoral and abdominal adipose tissue contribute equally to the excessive rate of lipolysis in hyperthyroidism and that both tissues are resistant to the actions of insulin...

..Data on incidence rates are scarce in GH deficiency (GHD). Here, we estimate the incidence rate in childhood onset (CO) and adult onset (AO) GHD in Denmark...

..Sex chromosomal abnormalities are relatively common, yet many aspects of these syndromes remain unexplored. For instance epidemiological data in 47,XYY persons are still limited...

..There are indications that this effect of estrogens may be immediate...

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..Whether this increase is caused by the syndrome per se (i.e. hypogonadism) or other factors, e.g. socioeconomic, are involved is presently unknown...

..The average intellectual performance is within the normal range. New insight into genetics, epidemiology, cardiology, endocrinology and metabolism from a number of recent studies will be included in this review...

..CONCLUSION: Both morbidity and mortality are significantly increased in Klinefelter syndrome with a 50% increase in mortality risk and a 70% increase in risk of being admitted to hospital...

..Our findings suggest that insulin increases arterial glycolysis, but treatment with insulin, propranolol, or both, is not associated with enhanced coronary vasodilation during hypoxia...

..Despite the lack of such studies in KS, we recommend testosterone treatment to KS patients with low serum testosterone or increased LH and change in body composition...

..The lactate concentration may affect, but is not essential for, hypoxia-induced vasodilatation in porcine coronary arteries...

..Furthermore, differences in mortality rates in females and males have been reported...

..There is a marked delay in diagnosis of the syndrome. A delay in treatment with testosterone may lead to decreased muscle and bone mass with subsequent risk of osteoporosis...

..Taken together, our findings suggest that differences in GH-binding protein concentrations, which possibly reflect GHR expression, determine GH pharmacokinetics rather than age or body composition per se...

..This supports the view that holo-TC is a marker of long-term vitamin B(12) status, and that samples may also be obtained from non-fasting subjects...

..Better delineation of the clinical phenotype of 47,XXX is needed; available information does not readily explain the increased mortality...

..In women, GH secretion is strongly influenced by oestrogen status, whereas the role of androgens is unclear. We, therefore, examined GH secretory dynamics during low vs. normalized androgen levels in women with adrenal failure...

..036). In conclusion, our data demonstrate that circulating MBL concentrations are significantly elevated in patients with type 1 diabetes and suggest a possible role of MBL in the pathogenesis of renovascular complications in diabetes...

..To estimate the mortality in Denmark in patients suffering from GH deficiency (GHD)...

..In conclusion, LV systolic function seems significantly related to fasting levels of glucose and LDL cholesterol in normal subjects...

..To estimate morbidity in Denmark in all patients with GH deficiency (GHD)...

..TS was diagnosed with a considerable diagnostic delay. Prevalence is increasing, but incidence of TS was stable...

..A total of 322 patients (51%) and 1169 controls (62%) returned the questionnaire...

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..The reduced magnitude of glucose excursions in the brain suggests that different mechanisms of glucose transport are operative in CNS compared with subcutaneous adipose tissue and muscle...

..We conclude that hypoxia-induced coronary artery dilatation is not mediated by increased adenosine produced within the artery wall but might be facilitated by increased adenosine sensitivity at the A(2A) receptor level...

..Mannan-binding lectin (MBL), a protein of the innate immune system, may constitute a connection between these fields...

..Morbidity and mortality are increased and the causes behind remain unknown. Is it the chromosome aberration or is it caused by postulated poorer socioeconomic status?..

..Ectatic aortopathy and arterial abnormalities cause excess morbidity and mortality in Turner syndrome, where a state of vasculopathy seemingly extends into the major head and neck branch arteries...

..Spontaneous pregnancy rarely occurs in women with Turner syndrome, and poor maternal outcomes are prominent in these women...